Beruflich Dokumente
Kultur Dokumente
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Textbook of
Editors
Ravikiran Ongole BDS, MDS
Professor
Department of Oral Medicine and Radiology
Manipal College of Dental Sciences, Manipal University
Mangalore, India
ELSEVIER
A division of
Reed Elsevier India Private Limited
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Dedicated to
My Wife, Parents and Brother
Ravikiran Ongole
My Parents, Wife and Daughter (Aadya)
Praveen BN
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Foreword I
The current academic scenario in our country has witnessed an abundance of postgraduate courses and postgraduate
teachers. An offshoot of this has been the burgeoning demand for reliable sources of knowledge for undergraduates,
postgraduates and the dental practitioners.
The mushrooming number of textbooks is a welcome sign of enterprise and effort on the part of our teachers. However
all are not of acceptable quality. One needs to separate the wheat from the chaff and restrict ones interest to the textbooks
with quality content.
Once such gem is the Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology brought out by Dr Ravikiran Ongole
and Dr Praveen BN, committed academicians with a number of scientific publications and a previous textbook to their
credit; they have succeeded in creating a tome of oral medicine and radiology. It is a matter of great pride to me personally
since Dr Ravikiran is a faculty member and a gifted teacher in my institution.
Their task has been a Herculean one since they have not only contributed their own knowledge to this book; they have
also used the combined experience of other dental and medical professionals from diverse fields.
I am happy that the first edition of the book was received well and I am confident that the second edition of the book
with an additional section on dental radiology would help in imparting further knowledge to students in the field of
Oral Medicine and Radiology.
A brief overview of the book reveals the amount of effort put in by the editors and their contributors. I wish them all
the best in their endeavor to spread knowledge in Oral Diagnosis and Radiology and whole heartedly recommend this
book to all those who seek the same.
Prof (Dr) V Surendra Shetty
Pro Vice Chancellor
Manipal University, Mangalore
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Foreword II
Dentistry has always been an evolving science and every effort is to be made to keep up pace with the advancement. This
textbook has received wide recognition for its contribution to the practice of dentistry. I would like to congratulate the
editors in their continuing effort to present the second edition with attention to detail, written principally for a student
in an attempt to penetrate some depth towards newer research.
The edition has maintained its firm commitment in assuring a thorough and complete text. The examination of a case
confronts every clinician. I think this book gives a well-reasoned explanation of fundamental aspects, emphasized on
clinical scenario with illustrations, which are of value to a practicing dentist.
Launching a subsequent edition of a warmly received text is more of a challenge. I whole heartedly congratulate their
feat for their truly admirable skills and the contributors for this splendid work of knowledge.
Prof (Dr) Srivatsa G
Principal
KLE Societys Institute of Dental Sciences
Bengaluru
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List of Contributors
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List of Contributors
xii
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List of Contributors
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List of Contributors
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Preface to the
Second Edition
The first edition of the book was published two years ago. Since then we have received very encouraging feedback from
students and faculty alike. It was also heartening to note that this book was included by various health universities in their
recommended list of textbooks for undergraduate and postgraduate students.
We also received suggestions for including a section on dental radiology with a special request to include chapters on
implant imaging and cone beam computed tomography. Taking these views into consideration, we included a section on
radiology that covers varied topics from radiation physics to specialized imaging techniques. The oral medicine section has
also been updated with the latest concepts in the diagnosis and medical management of various orofacial disorders.
We wish this comprehensive Textbook of Oral Medicine, Oral Diagnosis and Oral Radiology gains wider popularity
amongst the student community and the faculty and continues to cater to the needs of students pursuing the specialty of
Oral Medicine and Radiology.
Ravikiran Ongole
Praveen BN
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Preface to the
First Edition
Oral Medicine in India is still in its nascent stages of growth. Consequently, there is not much literature that is easily
available to the student community. While there are many excellent textbooks available outside the Indian shores, in the
editors opinion, there is not much emphasis on diseases that tend to occur in the Indian subcontinent. Further, content
prescribed by the Dental Council of India, which is very useful to undergraduate and postgraduate students of dentistry,
is rarely available.
Many popular textbooks that are available to students of dentistry in India are either exam-oriented (not sufficient
background material; not suitable for reference beyond a particular examination) or focus only on oral medicine or on
oral radiology.
Our goal was two-fold: publish content that would appeal to undergraduate students because the content corresponded
to DCI curriculum; to postgraduate students and practitioners to serve as reference for diseases that seem to occur very
frequently in India. We have tried our best to combine oral medicine, diagnosis and radiological aspects of various orofacial
diseases and oral manifestations of systemic disorders. Chapters such as maxillofacial trauma, Lab investigations, Mental
illness and Syndromes of the head and neck have been specially written for postgraduate students.
We have tried our best to provide up to date references. Another unique feature of this book is the contributions made
from more than 60 authors from various dental colleges all over India and from countries such as USA, England, Canada,
Mexico, Brazil and Nigeria. We have made a conscious effort to tap into the expertise of authors from various fields of
medicine such as plastic surgery, dermatology and psychiatry and from various dental specialties apart from oral medicine
such as oral pathology, oral surgery, conservative dentistry, orthodontics, prosthodontics and periodontics.
We sincerely hope that this textbook will stimulate minds and satiate the intellectual appetite of the students of oral
medicine, diagnosis and radiology.
Ravikiran Ongole
Praveen BN
xvii
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Acknowledgments
The editors would whole heartedly like to acknowledge contributions from various individuals, especially the authors to
this book.
We would like to acknowledge the encouragement, guidance and support rendered by Dr V Surendra Shetty, Pro Vice
Chancellor, Manipal University, Dr Dilip G. Nayak, Dean, Manipal College of Dental Sciences, Manipal University, Mangalore
and Dr Srivatsa G, Principal, KLE Societys Institute of Dental Sciences, Bengaluru. We would also like to thank them for
graciously permitting us to use photographs from the department archives.
We would like to thank the teaching faculty of the Departments of Oral Medicine and Radiology, MCODS (Mangalore)
and KLE Societys Institute of Dental Sciences (Bengaluru) for their help and suggestions provided during the preparation
of this book.
Our heartfelt thanks to Dr Saranya B, Dr Richa Gaba, Dr Bijina, Dr Sumsum P Sunny, Dr Sushma CN and Dr Pramila
Mendonca who helped us at every step during the preparation of the book.
Our sincere sense of gratitude go out to Mr Mark Dirlam, Supervisor and Graphic Artist and Mr Timothy Centers,
Photographer, Department of Illustrations, Indiana University School of Dentistry, Indianapolis, Indiana, USA and
Dr Jaideep Shekhar for helping us in preparing illustrations for the book.
We are extremely indebted to Dr John O Keefe, Editor, Journal of the Canadian Dental Association, Dr Foluso Owotade
and Dr Carol Stewart for their words of strength and encouragement and for lending us photographs.
We appreciate the support and constant encouragement received from the ever enthusiastic publishing team of Elsevier
India, especially Ms Ritu Sharma, Ms Nimisha Goswami and Mr Anand K Jha.
Our sincere appreciation and gratitude to our patients, some of who were terminally ill, for enduring pain and discomfort
in the hope of relief and cure. We hope that this textbook will in some small way alleviate their sufferings.
Above all our deepest gratitude to our families for their affection, unconditional support and encouragement.
We sincerely apologize to individuals whose names have been inadvertently not mentioned.
xix
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Contents
Dedication
Foreword I
Foreword II
List of Contributors
Preface to the Second Edition
Preface to the First Edition
Acknowledgments
v
vii
ix
xi
xv
xvii
xix
Chapter 1
History of Oral Medicine Oral and Maxillofacial Radiology Oral Medicine and Radiology in India
SECTION II
Chapter 2
Developmental Disturbances
11
Fordyces Granules (Fordyces Spots/Disease) Lingual Tonsils Leukoedema Retrocuspid Papillae Prominent Palatal
Rugae Circumvallate Papillae or Vallate Papillae Parotid Papilla Racial Pigmentation/Physiological Pigmentation
Mandibular and Maxillary Tori Developmental Disorders Affecting Tongue Developmental Disorders Affecting
the Lip Developmental Disorders Affecting Buccal Mucosa and Gingiva Developmental Disturbances of the Jaws
Developmental Disturbances Affecting Teeth
Chapter 3
61
82
Scarlet Fever Diphtheria Tularemia (Rabbit Fever, Deer-fly Fever, Francis Disease, Tick-Borne Disease, Oharas
Disease) Erysipelas Impetigo Melioidosis Tetanus Actinomycosis Noma (Cancrum Oris, Gangrenous or Necrotizing
Stomatitis) Botryomycosis (Bacterial Pseudomycosis) Rhinoscleroma (Respiratory Scleroma) Cat-scratch Disease
Infectious Mononucleosis (Monoglandular Fever, Kissing Disease) Acute Lymphonodular Pharyngitis Measles (Rubeola)
German Measles (Rubella) HIV and AIDS Acquired Immune Deficiency Syndrome Sinusitis Histoplasmosis
Blastomycosis (Gilchrist Disease) Mucormycosis (Zygomycosis, Phycomycosis) Aspergillosis Cryptococcosis (European
Blastomycosis, Torulosis, Busse-Buschke Disease)
Chapter 5
Orofacial Pain
111
Pain Physiology Classification of Orofacial Pain Clinical Assessment of Pain Pain from Orodental Structures
Barodontalgia Paranasal Sinus-related Pain Myofascial Pain Neuralgias Atypical Odontalgia Atypical Facial
Pain Burning Mouth Syndrome
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Contents
133
Description of Red and White Lesions Etiologic Classification of Red and White Lesions White Lesions of the Oral
Cavity Red Lesions of the Oral Cavity Red Lesions of the Tongue
Chapter 7
Vesiculobullous Disorders
174
Classification of Vesiculobullous Lesions Predominantly Vesicular Lesions Herpes Simplex Virus (HSV) Infections
Herpetic Whitlow and Herpes Gladiatorum Recurrent Herpes Infections Varicella Zoster Infections Hand, Foot and
Mouth Disease Herpangina Dermatitis Herpetiformis Predominantly Bullous Lesions Bullous Lichen Planus
Erythema Multiforme StevensJohnson Syndrome and Toxic Epidermal Necrolysis (Lyells Syndrome) Bullous
Impetigo Epidermolysis Bullosa
Chapter 8
196
Classification of Oral Ulcers Traumatic Ulcers Primary Herpetic Gingivostomatitis Recurrent Herpes Infection
Varicella Zoster Infection Acute Necrotizing Ulcerative Gingivitis (Trench Mouth, Vincents Disease, Vincents
Gingivostomatitis) Tuberculosis Syphilis Deep Fungal Infections Drug-induced Oral Ulcers Erythema Multiforme
Blood Disorders Causing Oral Ulcers Immunologic Disorders Dermatological Disorders Pemphigoid Gastrointestinal
Disorders Associated with Oral Ulcers Neoplastic Ulcers Ulcers of Unknown Etiology Syndromes Associated with
Oral Ulcers Diagnostic Protocol
Chapter 9
Dermatological Diseases
218
Lichen Planus Epidermolysis Bullosa Psoriasis Ectodermal Dysplasia EhlersDanlos Syndrome Pachyonychia
Congenita Dyskeratosis Congenita Pityriasis Rosea Xeroderma Pigmentosum Acanthosis Nigricans GoltzGorlin
Syndrome Acrodermatitis Enteropathica HaileyHailey Disease (Familial Benign Chronic Pemphigus) Dariers
Disease (Keratosis Follicularis) Reiters Syndrome Incontinentia Pigmenti (BlochSulzberger Syndrome) Kawasaki
Disease (Mucocutaneous Lymph Node Syndrome) Tuberous Sclerosis Complex (Epiloia, Bournevilles Disease)
Graft-versus-Host Disease
Temporomandibular Disorders
239
265
Developmental Disturbances Saliva, Xerostomia, Hyposalivation and Sialorrhea Inflammatory Conditions of Salivary
Glands Viral-induced Salivary Gland Pathology Non-inflammatory Conditions of Salivary Glands Salivary Gland
Tumors Benign Tumors Malignant Tumors
SECTION V
Chapter 12
303
Classification of Cysts of Orofacial Region Theories of Cyst Expansion Odontogenic Cysts Odontogenic Keratocyst
Gingival Cysts of Adults Calcifying Epithelial Odontogenic Cyst (Gorlin Cyst) Glandular Odontogenic Cyst Nonodontogenic Cysts Nasolabial Cyst Mid-palatal Raphe Cyst of Infants Cysts of Maxillary Antrum and Salivary
Glands Inflammatory Cysts Pseudocysts Cysts of Soft Tissues of Mouth, Face and Neck Nasopharyngeal Cysts
Thyroglossal Duct Cysts Lymphoepithelial Cysts (Branchial Cleft Cysts) Cystic Hygroma Dermoid, Epidermoid and
Teratoid Cysts Parasitic Cysts
Chapter 13
331
Benign Odontogenic Tumors Odontogenic Carcinomas Odontogenic Sarcomas Epithelial Malignant Tumors
Connective Tissue Malignant Tumors
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Contents
Chapter 14
Oral Cancer
380
Incidence of Cancer of Head and Neck Etiology and Risk Factors for Oral and Maxillofacial Cancer Tobacco Alcohol
Systemic Health Molecular Basis of Cancer Clinical Signs of Cancer Clinical Examination of a Patient with
Suspected Malignancy TNM Staging Nodal Metastasis Diagnosis of Oral Cancer
405
Definition and Etiology Contributory Factors in Dental Caries Classification of Dental Caries Microbiology of
Dental Caries Fascial Space Infection Ludwigs Angina Osteomyelitis
Chapter 16
440
Classification System in Periodontal Disease Gingival Diseases HostMicrobial Interaction Periodontal Diseases
Syndromes Sex Hormones Stress and Psychosomatic Factors Nutritional Factors Radiographic Evaluation of
Periodontal Diseases
Chapter 17
458
Classification of Regressive Alterations Affecting Teeth Tooth Surface Loss Attrition Abrasion Erosion (Corrosion)
Abfraction Classification of Tooth Wear Resorption of Teeth
SECTION VII
Chapter 18
SYSTEM REVIEW
Systemic Disorders and their Clinical Implications
473
Symptoms Suggestive of Cardiovascular Disease Common Cardiovascular Disorders and their Dental Considerations
Ischemic or Coronary Heart Disease Myocardial Infarction Congenital Heart Disease Rheumatic Fever Infective
(Bacterial) Endocarditis Heart Failure Red Blood Cell Disorders Polycythemia Anemia Thalassemia White Blood
Cell Disorders Qualitative Disorders Non-neoplastic Disorders Neoplastic Disorders Bleeding Disorders Vascular
Disorders (Vessel Wall) Platelet Disorders Thrombocytopathic Disorders Thrombocytopenic Disorders Disorders of
Coagulation Inherited Coagulation Disorders Acquired Coagulation Disorders Upper Respiratory Tract Infections
Lower Respiratory Tract Infections Granulomatous Diseases Malignant Disorders Other Respiratory Diseases
Renal Diseases Gastroesophageal Reflux Disease Inflammatory Bowel Disease Ulcerative Colitis Crohns Disease
Hiatal Hernia Peptic Ulcer Disease Eating Disorders Liver Diseases Bells Palsy Epilepsy Parkinsonism
Multiple Sclerosis Muscular Dystrophy Oromandibular Dystonia Myasthenia Gravis Growth Hormone Thyroid
Gland Parathyroid Glands HypothalamusPituitaryAdrenal Axis Pregnancy Saliva and Monitoring of Hormone
Levels Interesting Interface between Dentistry and Psychiatry Management of Psychiatric Disorders
Chapter 19
568
Autoimmune Disorders
590
Concepts of Immunity and Autoimmunity Pemphigus Epidermolysis Bullosa Acquisita Systemic Lupus Erythematosus
Autoimmune PolyendocrinopathyCandidiasisEctodermal Dystrophy Diabetes Mellitus Type I (IDDM) Systemic
Sclerosis Myasthenia Gravis
Chapter 21
Granulomatous Diseases
605
Tuberculosis Leprosy (Hansens Disease) Syphilis Deep Fungal Infections Foreign Body Granulomas Wegeners
Granulomatosis Sarcoidosis Orofacial Granulomatosis Crohns Disease (Regional Ileitis, Regional Enteritis)
Chapter 22
625
Fellatio Syndrome Traumatic Lesions of Lingual Frenum Syphilis or Lues Human Immunodeficiency Virus Infection
Intraoral Molluscum Contagiosum Condyloma Acuminatum Oropharyngeal Gonorrhea Oropharyngeal Chlamydial
Infection Oropharyngeal Trichomonal Infection
Chapter 23
633
Nutritional Requirements of Indians Carbohydrates Proteins Lipids Vitamins Metabolic Disorders Lipid
Reticuloendothelioses
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Contents
SECTION VIII
Chapter 24
FORENSIC DENTISTRY
653
Introduction, History and Relevance Dental Identification Challenges in Postmortem Examination Postmortem
Alterations to the Teeth and Oral Tissues Social Profiling Identifying the Edentulous Craniofacial Identification
Facial Approximation Age Estimation Methods Bite Mark Procedures Lip Print Investigation
685
Pioneers in Dental Radiology Fundamentals of Radiation Physics Intraoral X-Ray Units Interaction of X-Rays with
Matter
Chapter 26
Radiation Biology
697
Effects on Living Systems Molecular and Cellular Radiobiology Deterministic and Stochastic Effects Sources of
Radiation Dose and Risk in Radiography Radiation Detection and Measurement Film Exposure and Processing
SECTION X
RADIOGRAPHIC METHODOLOGY
Chapter 27
719
Radiographic Techniques
724
801
803
Radiographic Faults
812
Errors in Film Storage and Handling Errors in Film Placement and Projection Technique Errors in Exposure Parameters
and Processing Technique Artifacts
SECTION XII
Chapter 32
RADIOGRAPHIC LANDMARKS
Intraoral Radiographic Anatomical Landmarks
825
Landmarks Common to both the Maxillary and Mandibular Radiographs Landmarks Unique to the Maxillary Intraoral
Periapical Radiograph Landmarks Unique to the Mandibular Intraoral Periapical Radiograph
Chapter 33
837
Chapter 34
842
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Contents
SECTION XIII
APPENDICES
Appendix 1
Terminologies
861
Appendix 2
867
Appendix 3
871
Appendix 4
872
Appendix 5
873
References
879
Index
885
e1
e23
e78
IV. Halitosis
e147
e154
References
e236
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PART
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SECTION
Introduction
and Approach
to Diagnosis
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CHAPTER
Mission
Training and Scope of Practice
Future
Mission
The mission of the AAOM is stated in Box 1.
The AAOM is the heart and pulse of oral medicine in
the United States and is also internationally recognized
through the excellent educational training programs and
scientic symposia hosted, and the educational literature
and treatment guidelines published for medically complex
patients.
5
Box 1
Future
Healthy collaborations have been developed between
AAOM and the European Academy of Oral Medicine
(EAOM). EAOM was founded in 1998 based on representation from European countries including Austria, Croatia,
Denmark, England, Estonia, Finland, France, Germany,
Greece, Hungary, Iceland, Ireland, Israel, Italy, Latvia,
Netherlands, Norway, Portugal, Romania, Scotland, Serbia,
Slovenia, Spain and Sweden.
Future research will move further into genetics and proteomics to define etiopathogenesis of these complex diseases as well as find targeted treatment approaches. The
potential for using saliva as a diagnostic tool is growing as
well. As the life span of patients continues to lengthen due
to polypharmacy, advancement in diagnostic technologies
and treatment strategies, the demand for the oral medicine
specialist, both as a primary provider and consultant, will
grow exponentially. Medicine and dentistry are coming
closer together as they gain an appreciation of what each
can provide to enhance patient care. The quality of patient
care will continue to improve in parallel with these synergistic interactions.
SECTION
II
Oral and
Maxillofacial
Disturbances
2
3
4
5
Developmental Disturbances
Orofacial Pigmentation Disorders
Bacterial, Viral and Fungal Infections
Orofacial Pain
11
61
82
111
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Developmental Disturbances
CHAPTER
Lingual Varices
Lingual Thyroid
Fordyces Granules
Lingual Tonsils
Fissured Tongue
Leukoedema
Retrocuspid Papillae
Lingual Varices
Circumvallate Papillae
Parotid Papilla
Physiological Pigmentation
Hairy Tongue
Exostoses/Tori
DEVELOPMENTAL DISTURBANCES
FORDYCES GRANULES
(Fordyces Spots/Disease)
Fordyces granules are named after an American dermatologist, John Addison Fordyce. This is an ectopic/heterotopic collection of sebaceous glands seen in more than
80% of the normal population.
They are considered ectopic because sebaceous glands
are typically appendages of the skin. When they are present in the oral mucosa they seldom have hair follicles.
This condition is characterized by the presence of
multiple discrete minute yellow colored dots, spots or
granules involving various sites in the oral cavity such as
11
Figure 1
Figure 2
Duct
opening
into
epithelium
Sebaceous
glands
LINGUAL TONSILS
Lingual tonsils are the lymphoid aggregates present in the
oral cavity that are part of the Waldeyers ring. It can occur
unilaterally or bilaterally on the posterolateral border of
the base of the tongue. Lymphoid follicles of the lingual
tonsil are irregular in shape and size and vary in number
from 30 to 100.
12
Figure 3
A
(A) Fissured tongue with central median groove. (B) Fissured tongue with multiple grooves in irregular fashion.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Fissured Tongue
Management
LEUKOEDEMA
Leukoedema is a common alteration of the oral mucosa,
which appears as a diffuse grayish white opalescent area on
the oral mucosa. It is believed that the intercellular edema
of the superficial epithelial cells and the parakeratinized
epithelium produces the typical grayish white appearance
in this condition.
Axell et al (1981) studied the inuence of tobacco habits and leukoedema among 20,333 individuals in Sweden.
In his study 48.9% of the individuals exhibited leukoedema. Males were more commonly affected. Leukoedema
was more commonly found in the second and third decades
of life. The condition was signicantly more prevalent in
individuals with any form of tobacco habit (60%) and relatively less common in individuals without any tobacco
habit (36.3%).
Van Wyk (1985) studied the association between leukoedema and smoking. He examined 1996 high school students.
He concluded that smoking does not cause leukoedema
but may aggravate it. He also suggested that the etiology
for leukoedema is multifactorial.
13
Clinical features
Leukoedema presents as an asymptomatic, grayish white
diffuse opalescent region on the buccal mucosa and occasionally extending into the vestibule, floor of the mouth
and soft palate. The surface appears spongy and usually
comprises folds or grooves. It has been shown that it is
more common in blacks (almost 90%) compared to whites
(Figure 4).
Clinically, this condition can be differentiated from
other white lesions occurring in the oral cavity by stretching the mucosa. Grayish white areas of leukoedema usually disappear at least partially on stretching. Some authors
call this technique the stretch test.
Histopathology and ultrastructural features
Histopathological studies reveal increased epithelial thickness. Parakeratinized epithelium with broad and elongated
rete pegs are typically seen. Intracellular edema within the
spinous layer is a characteristic feature in leukoedema. The
edematous/vacuolated cells are large with a pyknotic nuclei.
Histopathologically it is believed to mimic lesions of white
sponge nevus.
Van Wyk and Ambrosio (1983) studied the ultrastructural and histochemical features of leukoedema in 12 individuals and compared it with normal buccal mucosa. They
concluded that the intracellular edema of the epithelial
cells in leukoedema is due to vacuolation in the cytoplasm
of cells. Toward the surface of the epithelium, the vacuolated cells collapsed into a compact layer of attened cells.
The outer cells of this layer abruptly swelled again to form
the characteristic supercial layer of ballooning cells of
leukoedema.
Figure 4
14
They proposed that the vacuolation in the cell cytoplasm represents a reversible form of cellular degeneration
resulting from cell damage. They believed that the vacuolation was caused because of reduced mitochondrial
function. The supercial ballooning cells are degenerated
cells. They stated that the presence of a compact layer of
vacuolated cells, keratohyalin granules and keratohyalinlike structures in the supercial cells are features of an
aborted form of keratinization.
Treatment
No active treatment is necessary as it is considered as a
variation in the normal anatomy of the oral cavity. However, it is believed that the condition becomes less prominent with the cessation of tobacco habit.
RETROCUSPID PAPILLAE
The retrocuspid papilla is a normal variation in anatomy
characterized by the presence of pink colored, soft to
firm, generally sessile papule or nodule (Figure 5), located
on the gingiva on the lingual surface of the mandibular
canines (cuspid).
The size of the nodule may vary in size from 1 to 5 mm in
diameter. It is very commonly seen in children and believed
to regress with age.
DAoust et al (1991) studied the distribution of retrocuspid papillae among three groups (Ecuador, Honduras and
Nicaragua) of Latin American patients. He found that the
retrocuspid papilla was most prevalent in children below the
age of 5 years. He also found a signicantly higher female
Figure 5
more number of palatine rugae and the left side of the palate shows slightly more number of rugae in both males
and females. No bilateral symmetry is seen in the number
of rugae. As age advances, the length of the rugae and the
transverse palatal rugal region width increases.
Luke (1988) studied the development of palatal rugae in
mice. He showed that the rugae develop as localized regions
of epithelial proliferation and thickening prior to the elevation of the palatal shelves. Later on, the broblasts and
collagen bers accumulate within the connective tissue
beneath the thickened epithelium and then assume a characteristic orientation. The direction of the collagen bers
running across the base of the palatine rugae determines
their orientation.
In the human embryos the palatal rugae are usually
prominent and present throughout the length of the palatal
shelves at the time of their elevation. At about the 550 mm
stage of the embryo there are about ve to seven symmetrical ridges. The anterior ridges originate at the midpalatine
raphe. Other ridges are seen laterally.
However, toward the end of the intrauterine life, the
posterior ridges almost disappear completely and the anterior ridges become compressed and prominent.
It is believed that the role of rugae in humans is more or
less vestigial. However, in animals palatine rugae help in
suckling and feeding.
Lingual Varices
Palatal rugae. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
Lingual varices are characterized by the presence of tortuous dilated veins on the ventral surface of the tongue. It is
estimated that approximately 10% of the patients in the
15
Figure 7
Etiopathogenesis
Various theories have been proposed to explain the occurrence of these varices. Ettinger et al (1974) showed that the
incidence of varicosities increases with age. Koscard et al
(1970) reported that the elastic support to capillaries significantly diminishes with advancing age. This reduction in
the elastic support of the connective tissue supporting the
blood vessels leads to dilation of capillaries and formation of
varicosities. It is also suggested that these are due to abnormally dilated and tortuous veins (varices), as they are not
protected by surrounding tissues against hydrostatic pressure.
Eddy et al (1977) studied the role of vitamin C in 22
elderly vegetarians. He showed that vegetarians had high
levels of ascorbic acid values in plasma (10.2 0.4 mg/l),
compared to other elderly individuals. He concluded that
there was a lower incidence of sublingual petechiae and
varicosities in the vegetarian group.
Clinical features
In this condition multiple, bluish-purple papular blebs are
seen on the ventral and lateral borders of the tongue and
occasionally seen on the lips and buccal mucosa. Ventral
surface of tongue reveals the presence of tortuous and
dilated veins (Figure 7). Susmita et al (2006) reported a
case with palatal varicosities. These are usually seen in the
elderly and are not symptomatic unless the varices are
thrombosed. Jassar et al (2000) reported a symptomatic
case of sublingual varices in a patient with portal hypertension secondary to liver cirrhosis.
Figure 8
Histopathologic features
Microscopically, dilated veins are seen with little smooth
muscle and elastic tissue. Thrombosis may be seen as
concentric zones of platelets and erythrocytes (lines of
Zahn). Older thrombi show dystrophic calcification and/or
phleboliths.
Management
Treatment is usually unnecessary. Surgical treatment is
indicated for cosmetic purposes or when there is thrombosis.
project out onto the surface of the tongue. They are surrounded by a marginal groove. Each of the papillae are
attached to the tongue via their slender bases.
Roughly eight to 12 large (35 mm in diameter) mushroom shaped papillae are arranged all along the V-shaped
sulcus terminalis that divides the tongue into the body and
base (Figure 8). However, occasionally they tend to grow
in size when inamed.
Figure 9
Figure 10
Keratinized
stratified
squamous
epithelium
Nonkeratinized
epithelium
Circular
trench
Taste bud
Von Ebner
gland
PAROTID PAPILLA
Stensens duct of the parotid salivary gland opens into the
oral cavity in the buccal mucosa opposite to the maxillary
first and second molars.
In many instances the orice is hardly noticeable.
However in some individuals a small, triangular raised
pink to red color papule or nodule is readily visible. This
ap of tissue covering the orice of the Stensens duct is
called parotid papilla (Figure 10). Parotid papillae are usually seen bilaterally. As a result of their anatomic location
adjacent to the occlusal plane, the parotid papillae are
common sites for formation of traumatic ulcers.
RACIAL PIGMENTATION/PHYSIOLOGICAL
PIGMENTATION
Pigmentation of the oral mucosa can occur due to a wide
variety of endogenous and exogenous agents. Most of these
are due to five basic pigments, namely, melanin, melanoid,
oxyhemoglobin, reduced hemoglobin and carotene.
Physiologic oral pigmentations are genetically determined. Various stimuli, such as trauma, hormonal changes,
medication and radiation may result in an increased production of melanin. An age-related increase of oral melanocytes has also been observed.
In dark-skinned people, oral pigmentation increases,
but there is no difference in the number of melanocytes
between fair-skinned and dark-skinned individuals.
17
Figure 11
Figure 12
Hairy Tongue
This condition has also been referred to as black hairy
tongue. Etiology is unknown, however certain predisposing
factors include poor oral hygiene, frequent use of mouthwashes, smoking and alcohol consumption, radiation therapy
Figure 13
According to some authors, micro-damage and inflammation in periodontal tissue in genetically susceptible individuals causes exostoses. Abnormal loads during mastication
have also been implicated in inducing exostoses. Buccal
exostoses may result from lateral pressure of the adjacent
teeth.
Palatal tori may be a result of chronic periosteal ischemia due to nasal septum pressure. The torquing action of
the arch of the mandible during mandibular movements is
said to produce mandibular tori.
Clinical classification
Although a formal classification system does not exist,
based on the clinical occurrence, exostoses may be categorized as those that are commonly seen and the rarer forms.
Commonly occurring exostoses
Palatal torus
Mandibular torus
Rarer forms of exostoses
Buccal exostoses
Palatal exostoses
Subpontine exostoses
Torus/Tori
The word torus is derived from Latin, which means a
swelling or bulge. Although etiology is unknown, a hereditary basis is suspected. A torus located along the midline
of the hard palate is called a palatal torus, or torus palatinus, and a torus in the lingual aspect of the mandible is
called a mandibular torus, or torus mandibularis.
Clinical classification of tori based on
morphology
Flat torus: Occurs as a slightly convex bony protuberance with a smooth surface for mandibular tori. However
19
Figure 14
Figure 15
Figure 16
Nodular torus: These are seen as multiple bony protuberances each arising from individual bases. As they enlarge
these may coalesce forming grooves between them.
Spindle torus: Occurs along the length of the midpalatal
raphe region for palatal tori. Elongated tori are evident
bilaterally in the mandible.
Torus palatinus
This condition exhibits exostoses in the midline of the
hard palate. It may be inherited as an autosomal dominant
trait. In individual studies the incidence of palatal tori
among the population of United States has been reported
as high as 2035%. It is believed to be twice as prevalent
in females as in males.
The tori in the palatal vault may vary in size from a few
centimeters to larger sized lesions and are usually lobulated and dome shaped with a smooth surface (Figure 14).
These exostoses generally tend to enlarge with age. Histopathologically smaller lesions are composed of compact
bone. However larger tori may exhibit a central core of
cancellous bone covered by compact bone.
Buccal exostoses
Torus mandibularis
Torus mandibularis is the term used to describe exostoses
occurring on the lingual surface of the mandible. It is
estimated that 710% of the population of United States
exhibit torus mandibularis. However no specific male or
20
Figure 17
Figure 18
Palatal Exostoses
They appear as bilateral bony nodular protuberances arising on the palatal cortical plate usually corresponding to
the maxillary tuberosities.
Figure 19
Microglossia/Hypoglossia
This rare developmental condition is characterized by an
abnormally small tongue. It may occur as an isolated anomaly or, more commonly, as part of oromandibular limb hypogenesis syndrome (hypoglossia-hypodactylia syndrome) or
in association with micrognathia, hypodontia, situs inversus, asplenia, absence of lower incisors, or enamel hypoplasia. Prognosis depends on nature and severity of the
condition. Treatment is directed toward improvement of
oral function with an understanding of the changes in the
mechanisms of oral suction, mastication, swallowing, speech,
as well as dental occlusion.
Macroglossia
An abnormally enlarged tongue is one that protrudes beyond
the teeth or the alveolar ridge in the resting position
(Figure 19). Macroglossia can be broadly categorized as true
macroglossia and pseudo macroglossia (Table 1). In true
macroglossia enlarged tongue is associated with histological
abnormalities. In pseudo macroglossia (relative macroglossia)
the enlargement is apparent; though histology does not
provide a pathologic explanation (e.g. Downs syndrome).
22
Table 1
Classication of macroglossia
Pseudo macroglossia
Tongue posture
Habitual (tongue thrust)
Poor neuromuscular control
Edentulousness
Maxillofacial skeletal deficiencies
Shallow palatal vault
Deficient maxilla and mandible
Effects of surrounding structures
Enlarged adenoids
Cysts, tumors, space infections displacing the tongue
True macroglossia
Congenital causes
Muscular hypertrophy
Vascular malformations (hemangioma, lymphangioma)
Down syndrome
BeckwithWiedemann syndrome
Mucopolysaccharidoses I and II
Congenital hypothyroidism
Behmel syndrome
Transient neonatal diabetes mellitus
Trisomy 22
Laband syndrome
Lethal dwarfism of Blomstrand
Skeletal dysplasia of Urbach
Tollner syndrome
Ganglioside storage disease type I
Lipoid proteinosis
Acquired causes
Endocrinal disturbances
Acquired hypothyroidism
Acromegaly
Pituitary gigantism
Myxedema
(Contd...)
Table 1
Continued
Infections
Tuberculosis
Actinomycosis
Traumatic injuries
Self-inflicted (self-harm, injury during epileptic seizure)
Presurgical (intubation)/surgical trauma/postsurgical (anesthesia/
hemorrhage)
Neoplasms
Lymphangioma
Hemangioma
Carcinoma
Sarcoma
Solitary plasmacytoma
Neurofibroma
Granular cell tumor
Nutritional and metabolic disorders
Amyloidosis
Scurvy
Pellagra
Autoimmune disorders
Sarcoidosis
Giant cell arteritis
Miscellaneous
Angioneurotic edema
Modified from Richard D Thrasher III (2007)
Figure 20
Clinical features
Clinical features include, crenated lateral border of tongue,
open bite, mandibular prognathism and airway obstruction.
There may be ulceration, secondary infection and necrosis.
In infants it leads to lisping speech, noisy breathing, drooling and difficulty in eating. The tongue has a pebbly surface with multiple vesicle like blebs in lymphangioma; in
hypothyroidism there is diffuse smooth enlargement; the
tongue is multinodular in amyloidosis and neurofibromatosis, papillary appearance of the tongue is seen in MEN type
II and tongue is fissured in Downs syndrome. A unilateral
enlargement of tongue is seen in hemihyperplasia.
Management
Macroglossia, unless causing a functional disturbance
need not be corrected. It is treated with surgical techniques
chosen in accordance with the functional results that one
wants to achieve. It must be the most conservative technique to preserve the vascular nerve bundle. Speech therapy may be required in some cases. Tracheostomy is indicated
in cases of airway obstruction.
Ankyloglossia/Tongue-tie
Ankyloglossia or tongue-tie, is the result of a short, tight,
thick, lingual frenulum causing tethering of the tongue
Cleft Tongue
Cleft tongue or bifid tongue is caused due to lack of merging of lateral lingual swellings of tongue. Partial cleft is
more common than the total cleft and is characterized by
a deep groove in the midline of the dorsal surface. It is often
found as one of the features of orofacial digital syndrome
with thick fibrous bands in the lower anterior mucobuccal
fold and clefting of hypoplastic mandibular alveolar process. Food and microorganisms may collect in the base of
the cleft and cause irritation.
Fissured Tongue
Described on page 13.
Geographic Tongue
Geographic tongue has also been referred to as benign migratory glossitis, wandering rash of the tongue, annulus migrans,
stomatitis areata migrans and erythema areata migrans. It
is a common benign condition that is seen in almost 3% of
the population. Geographic tongue is seen in males and
females equally. However, few articles in literature describe
a slightly higher female predilection (2:1). However, the
tongue changes are more prominent in adults compared to
children.
Etiopathogenesis and predisposing factors
The etiopathogenesis of the condition is still not understood.
Histologically it is said to be an inflammatory condition
associated with human leukocyte antigen (HLA)-DR5,
HLA-DRW6, and HLA-Cw6. Eidelman et al (1976) reported
that many of the parents and siblings of individuals with
geographic tongue also presented with the condition. This
substantiated the possibility of heredity being an etiological factor.
Guimaraes et al (2007) in their investigations found that
the polymorphism3954 interleukin (IL)-1B is associated
with an increased risk of developing geographic tongue.
Redman et al (1966) and Bnczy (1975) et al suggested
that emotional stress was associated with the occurrence
and severity of geographic tongue.
There are many studies with regard to the association
of geographic tongue and diabetes mellitus. Wysocky et al
(1987) reported a four-fold increase in the presence of
geographic tongue in diabetics. However, Guggenheimer
et al (2000) reported no signicant correlation between
geographic tongue and insulin-dependent diabetes mellitus.
Waltimo (1991) in his study on the severity of geographic tongue in a patient taking oral contraceptive pills
24
Figure 21
Figure 23
Figure 22
Management
All patients of geographic tongue need to be reassured of the
harmless nature of the condition. In symptomatic patients
palliative therapy with topical or systemic antihistaminics
have proved beneficial due to their local anesthetic effect
(Sigal et al, 1992). Presence of associated fissured tongue
with superimposed candidal infection can be effectively
managed with topical clotrimazole. Benzydamine hydrochloride mouthrinse can be used to manage burning sensation. Gibson et al (1990) in their study showed that zinc
supplements proved effective for managing geographic
tongue.
Lingual Varices
Described on page 15.
Lingual Thyroid
Neutrophil infiltrations are seen in the thick layer of keratin and to a lesser degree in other portions of the epithelium. These infiltrations produce microabscess (Munros
abscess) in the keratin and spinous layers.
Failure of the thyroid tissue to descend from its developmental origin at the foramen caecum to its normal pretracheal location leads to its presence in the tongue at the
foramen caecum. Van Der Gaag et al (1985) postulated
that maternal antithyroid immunoglobulins may arrest the
descent of the thyroid in some individuals.
Thyroid tissue may be deposited ectopically along this
early thyroglossal tract. Apart from tongue, ectopic thyroid tissue has also been reported at other midline locations of the neck such as below the level of hyoid bone,
larynx and trachea, mediastinum and esophagus.
It is estimated that the incidence of lingual thyroid varies
between 1:3,000 and 1:100,000 (Williams et al, 1989).
The lingual thyroid is seen as a nodular mass in the
midline about 23 cm in diameter with a smooth surface.
It appears erythematous when highly vascular. Depending
25
Figure 24
Tongue Pits
The authors report a case of lingual pits on the dorsal surface of the tongue in a 24-year-old male (Figure 24). The
patient was asymptomatic and reported that these pits
were present since childhood. Review of literature shows
no mention of this condition.
Figure 25
Double Lip
Here a mucosal fold appears on the mucosal side of the lip,
commonly the upper lip. The congenital form is due to
Figure 26
Achondroplasia
BeckwithWiedemann syndrome
DiGeorge syndrome
Fetal alcohol syndrome
Goldenhar syndrome
Gorlin syndrome
Treacher Collins syndrome
Van der Woude syndrome
Waardenburg syndrome
BeckwithWiedemann syndrome
Cleidocranial dysplasia
Crouzon syndrome (craniofacial dysostosis)
EhlersDanlos syndrome
Fetal alcohol syndrome
Figure 27
Cleft lip
Figure 28
Figure 29
Figure 30
Figure 31
Figure 32
Figure 33
Fibromatosis Gingivae
This is an autosomal dominant disorder affecting the
gingiva of one or both arches and characterized by noninflamed, non-painful smooth or nodular diffuse overgrowth (Figure 34). The overgrowth may prevent eruption
of teeth or may be seen covering a large portion of the
29
Figure 34
Figure 35
crown in erupted teeth. It may be associated with hypertrichosis, corneal dystrophy, craniofacial deformities, nail
defects, deafness, epilepsy and mental retardation. Histopathologically dense non-inflamed collagenous connective
tissue with the overlying epithelium showing elongated
rete ridges are seen. Gingivectomy is the treatment of
choice. Recurrences may be seen but can be prevented by
extraction of teeth.
Micrognathia
Congenital micrognathia is usually seen in association
with other congenital abnormalities like congenital heart
disease and Pierre Robin syndrome. Occasionally, they
may follow a hereditary pattern. Micrognathia of maxilla
is usually due to deficiency in the premaxillary area.
The middle third of face appears retracted. Associated
maldevelopment of nasal and nasopharyngeal structures
can predispose to mouth breathing. Mandibular micrognathia is commonly due to agenesis of the condyles.
Normal growth of mandible depends on the development
of condyles as well as muscle function. If there is ankylosis of the joint due to trauma, infection of mastoid, middle
ear or the joint, it causes acquired micrognathia of mandible. Clinically, severe retrusion of chin, a steep mandibular angle, and a deficient chin button are observed
(Figure 35). Table 4 lists few of the syndromes associated
with micrognathia.
Macrognathia
Agnathia
Though the term agnathia refers to absence of jaws,
usually there is incomplete development of either maxilla
30
Hemifacial Hyperplasia
Although this is known more commonly as hemifacial
hypertrophy, there is actually hyperplasia of the tissues.
Some degree of facial asymmetry is common. Hemifacial
hyperplasia refers to significant unilateral enlargement of
the face including eyes, ears, nose and intraoral tissues
(Figure 37). It is often noted at birth and sometimes at
puberty. The disproportionate growth continues until the
patients overall growth ceases, resulting in permanent
asymmetry.
Table 4
Cohen syndrome
Intraoral findings
DiGeorge syndrome
Radiographic features
RubinsteinTaybi syndrome
The facial skull bones (mandible, maxilla, zygomatic, temporal and frontal) are enlarged on the affected side (Figure 38).
Figure 36
A
(A) Lateral cephalogram showing mandibular macrognathia. (B) Orthopantomograph showing increased ramal length and
mandibular body in macrognathia
31
Figure 37
A
(A) A child with hemifacial hyperplasia affecting the right side. (B) Right side of the tongue showing enlargement.
(C) PA view showing enlargement of the mandible and soft tissues on the right side. (D) Enlarged body and
ramus of the mandible on the right side
Figure 38
The roots and crown of the teeth particularly the permanent teeth are often enlarged and may erupt prematurely.
Primary teeth are shed prematurely on the affected side.
The mandibular canal can appear enlarged.
Management
Functional and cosmetic improvements may be achieved
through orthodontic tooth alignment and serial staged
surgeries. Because enlargement is related to all tissue levels, perfect symmetry cannot be obtained.
32
Condylar Hyperplasia
Condylar hyperplasia refers to a unilateral enlargement of
the mandibular condyle. Though the etiopathogenesis is
still unclear, various causes have been proposed such as
trauma, endocrinal disturbances and local deficiency of
circulation.
Clinical features
Condylar Hypoplasia
Bifid/Trifid Condyle
Condylar hypoplasia results from congenital or developmental disturbances or due to acquired causes. In this condition the condyle usually retains its shape but appears
smaller. Unilateral involvement of the condyles is more
common than bilateral involvement.
Most of these patients also present with a proportionately smaller ramus and body of the mandible. A prominent antigonial notch may be seen. The acquired causes
that result in hypoplasia of the condyle include traumatic
Condylar Aplasia
33
Figure 39
34
Figure 40
Coronoid Hyperplasia
Hyperplasia of the coronoid process is an uncommon
developmental disturbance affecting the jaws. The hyperplasia of the coronoid process can either be unilateral or
bilateral.
This condition is characterized by a progressive limitation in mandibular movement, due to impingement of the
elongated coronoid processes on the posterior surface of
the zygomas. The shape of the coronoid process usually
does not change, however it only increases in size.
On clinical examination there is no apparent facial
asymmetry or pain. It usually begins at puberty. Males
are more commonly affected than females (5:1). Apart
from genetic inheritance other causes for coronoid hyperplasia to occur have been proposed such as trauma,
increased activity of the temporalis muscle and endocrinal
stimulus.
Waters view and orthopantomograph are usually sufcient to evaluate coronoid hyperplasia. It is believed
that the projection of the tips of the coronoid processes
at least 1 cm over the inferior rim of the zygomatic arch is
pathognomonic of coronoid hyperplasia. The impingement
Figure 41
Exostoses/Tori
Described on page 19.
DEVELOPMENTAL DISTURBANCES
AFFECTING TEETH
Macrodontia It refers to teeth that are larger than normal. However, the term macrodontia should not be applied
to teeth that appear large due to fusion or gemination.
Localized macrodontia is seen in hemihyperplasia. All the
teeth are larger than normal in generalized macrodontia.
35
Figure 43
Figure 45
Figure 44
A
Figure 46
Figure 48
Figure 49
Figure 47
There are two crowns with a vertical groove. The cervical portion of both crowns is joined along with the pulp
chambers.
Fusion occurs when there is union of two tooth buds
with the conuence of dentin, resulting in single tooth
Figure 50
Figure 52
Figure 51
this may have to be resolved by radiography. Some authors
have reported abnormal patterns in concrescence such as
concrescence between an impacted third molar and an
erupted second molar (Romito, 2004), concrescence between
a third molar and a supernumerary fourth molar in the
mandible (Gunduz et al, 2006) and concrescence of the
crown of an impacted tooth and the roots of the erupted
tooth as a result of the deposition of acellular cementum
on the crown (Sugiyama et al, 2007).
Presence of geminated and fused teeth in deciduous
dentition can cause crowding, abnormal spacing, delayed
or ectopic eruption of underlying permanent teeth. Extraction may be necessary to prevent an abnormality in eruption. If the permanent teeth are affected, treatment of choice
is determined by patients needs. Surgical division followed
by endodontic treatment may be done. Selective shaping is
done with/without placement of full crowns. In some cases,
surgical removal of tooth with prosthetic replacement is
done.
Concrescence (fusion of the teeth via the cementum).
Courtesy: Department of Oral Pathology, MCODS, Mangalore
Dilaceration
It refers to a sharp bend or curve anywhere along the length
of the tooth, most commonly at the root (Figures 5355).
Literature review reveals interesting names that have
been used to describe dilaceration such as scorpion tooth
(Moreau, 1985) and hand of a traffic policeman (Stewart,
1978).
Figure 53
Figure 55
Figure 54
Talon Cusp
Mitchell in 1892 first described talon cusp as a prominent
accessory cusp on the lingual surface of a maxillary incisor.
39
Figure 56
Dens Invaginatus
Dens invaginatus has also been referred to as dens in dente
and gestant odontome.
Dens invaginatus is an enamel-lined surface invagination of the crown or root. Based on the site the condition may be subdivided into coronal and radicular forms.
Cementum-lined invaginations of the root are considered
variations in the root morphology and are not a type of
invaginatus.
The coronal form is formed by the infolding of the
enamel organ into the dental papilla. The radicular form of
dens invaginatus is produced because of the invagination
of the Hertwigs epithelial root sheath.
The invagination can be of varying lengths. It may be
restricted to the coronal portion or it may extend into the
root. When it extends into the root, it may or may not communicate with the pulp. If the invagination is too extensive it gives the appearance of a tooth within a tooth (dens
in dente) (Figure 57). Some of the invaginations can get
Figure 57
Figure 58
Coronal dens in dente
Type I
Type II
Type III
Dens Evaginatus
Dens evaginatus has also been referred to as occlusal
enamel pearl, Leongs premolar, tuberculated cusp, accessory tubercle, occlusal tuberculated premolar and evaginatus odontomas.
Dens evaginatus is a rare developmental anomaly characterized by the presence of an accessory cusp or enamel
pearl on the occlusal surface of the premolars occurring
between the cusps. The tubercle consists of enamel, dentin
and pulp. The condition occurs as a result of proliferation and
evagination of an area of the inner enamel epithelium and
the underlying dental papilla into the enamel organ during
early stages of tooth development.
It is believed that the prevalence of this condition
ranges between 1 and 4%. It occurs most commonly in the
Mongoloids, Chinese, Thai and Caucasians. The premolars
are most commonly affected followed by the molars,
canines and incisors. The mandibular teeth are ve times
more frequently affected than the maxillary teeth.
Presence of the occlusal tubercle can lead to occlusal
disharmony, attrition and tendency to fracture thereby
resulting in pulpal exposure. The involved tooth can turn
non-vital. Some authors have reported fascial space infections and osteomyelitis.
Grinding of the accessory cusp along with indirect pulp
capping is recommended. Non-vital teeth are best treated
endodontically.
Shovel-shaped incisors
Shovel-shaped incisors may be seen in association with dens
evaginatus. Marginal ridges of the incisors are prominent
41
Figure 59
Taurodontism
Taurodontism originated from the Greek words tauros
meaning bull and odontos meaning tooth. Like the name
suggests the tooth resembles the tooth of a bull. The term
taurodontism was first used by Keith in 1913. He defined
taurodontism as a tendency for the body of the tooth to
enlarge at the expense of the roots. According to Witkop
taurodont teeth have pulp chambers in which the bifurcation or trifurcation is displaced apically, so that the chamber
has a greater apico-occlusal height than in cynodont teeth
and lacks a constriction at the level of the cementoenamel
junction. The distance from the bifurcation or trifurcation
of the roots to the cementoenamel junction is greater than
the occlusal-cervical distance.
In this condition the body of the tooth (usually the
molars) is enlarged and rectangular in shape at the expense
of the roots. The teeth thus resemble bulls teeth. The pulp
chamber is large, with a greater apico-occlusal height. It
also lacks the constriction at the cervix. Shaw in 1928 classied the affected tooth into three subtypes based on the
degree of apical displacement of the pulpal oor, namely
hypotaurodontism, mesotaurodontism and hypertaurodontism (Figure 59).
Taurodontism is more frequently seen in permanent
dentition compared to deciduous dentition (Figure 60). The
rst molar is the most frequently affected followed by the
second and the third molar (Figure 61). Premolars may
also be affected (Figure 62).
Taurodontism may be associated with multiple syndromes. A few of the syndrome associated with this condition have been listed in Table 5.
The condition needs no specic dental management.
However there is difculty in locating, instrumenting and
obturating pulp canals during endodontic treatment.
Figure 60
Figure 61
Ectopic Enamel
It is a term used to describe the enamel present in unusual
locations like the root. Enamel pearls and cervical enamel
extensions are two types of ectopic enamel seen (Figure 63).
Figure 62
Figure 63
Figure 64
The complications associated with the presence of the ectopic enamel in deciduous teeth are delayed exfoliation of
the primary tooth and deviation in the eruption path of
the succedaneous tooth.
surface of the crown of the mandibular molar. The protostylid is usually seen on the rst or third permanent molars
or in deciduous lower second molars. It is seen in almost
40% of the population. The appearance can vary from a
simple pit in the buccal groove to a furrow or a prominent
cusp (Figure 69).
The Uto-Aztecan upper premolar is known to occur only
in native Americans, with its highest frequency in Arizona.
It occurs in the permanent upper rst premolar. In this
trait the buccal cusp may bulge out to the buccal aspect
with a marked fossa in its distal shoulder (Figure 70).
Figure 66
Figure 67
Figure 65
44
Figure 70
Lingual
Figure 68
Mesial
Distal
Carabelli cusp
Distal
Buccal
Mesial
Figure 71
Figure 69
Buccal
Mesial
Distal
Lingual
45
Figure 72
Figure 73
Figure 74
Figure 75
Figure 76
Transposition of teeth
Peck et al described dental transposition as the positional
interchange of two adjacent teeth, or the development or
eruption of a tooth in a position normally occupied by a
non-adjacent tooth. The transposition can either be complete (crowns and the roots of the involved teeth exchange
positions in the dental arch) and incomplete (crowns are
transposed, but the roots remain in their original positions).
It is estimated that approximately 1% of the population
exhibit transposition of teeth. It can be seen in the maxillary or mandibular teeth. Transposition can either be unilateral or bilateral. The common teeth that show transposition
are canine-rst premolar in the maxilla (Figure 77) and
the mandibular canine-lateral incisor. Though literature
review reveals almost all cases of transpositions affecting
the permanent dentition, Duncan et al (1996) reported the
fusion and transposition of the maxillary right central and
lateral primary incisors.
Etiology Some authors believe that dental transposition
occurs because of interchange of developing tooth buds.
47
Figure 77
Several theories have been proposed to account for dental transposition, including the interchange of developing
tooth buds, inherited condition, altered eruption paths,
trauma and the presence of retained primary teeth. It is
also believed that root dilacerations of the adjacent teeth
may be a potential etiological factor of canine-premolar
transpositions.
Peck and Peck classied dental transpositions based on
the teeth involved as:
Impacted Teeth
Impaction of a tooth occurs when its eruption is impeded
by a physical barrier. Impaction of deciduous teeth is rare;
it generally involves the second molars, probably due to
ankylosis. In the permanent dentition, third molars are
impacted most frequently (mandibular commoner than
maxillary). This is followed by maxillary cuspids and
mandibular premolars. In permanent teeth, the causes for
impaction include insufficient maxillofacial development,
overlying cysts or tumors, trauma, thickened overlying
bone or soft tissue. Impacted teeth may erupt partially or
completely encased within the bone. The former may be
associated with pericoronitis and the latter may cause
resorption of roots of adjacent tooth, periodic pain or trismus; dentigerous cysts and adenomatoid odontogenic
tumors are common in these. Multiple impacted teeth may
be related to syndromes and metabolic disorders such as
cleidocranial dysostosis, Gardner syndrome, YunisVaron
syndrome, tricho-dento-osseous syndrome, GAPO [growth
retardation, alopecia, pseudoanodontia (failure of tooth
eruption) and progressive optic atrophy] syndrome and
mucopolysaccharidoses. However literature review also
shows reports of multiple impacted teeth in non-syndromic
individuals (Figure 78).
Impaction may be classied according to angulation of
tooth in relationship to the remaining dentition: mesioangular impaction (Figure 79), distoangular, vertical (Figure
80) and horizontal (Figure 81). There can be variation of
angulation in sagittal plane, the impacted third molars
may be deected buccally or lingually (Figure 82). When
the crown points toward the inferior border of mandible or
when it is completely within the ramus of mandible it is
referred to as inverted impaction (Figure 83). Occasionally
interesting patterns of impacted teeth are seen such as sleeping molars (Figure 84) and kissing molars (Figure 85).
Figure 78
Figure 80
Figure 79
Cropped orthopantomograph showing vertically impacted
mandibular third molar. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
Figure 81
Figure 82
49
Figure 83
Figure 84
2.
The entire process of amelogenesis is influenced by a number of environmental influences and genetic mutations,
causing aberrant enamel formation. The further categorization of enamel hypoplasia is done on the basis of the
cause as follows:
Orthopantomograph showing bilateral sleeping molars.
Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
1.
2.
Figure 86
Figure 85
50
2.
Localized causes:
a. Local infection or trauma
b. Irradiation
Systemic causes: Systemic causes may have an effect
on permanent teeth if they occur between the time
soon after birth and before the age of 6 years, as this
Figure 87
51
Figure 88
Figure 89
Amelogenesis Imperfecta
Amelogenesis imperfecta (AI) represents a group of inherited, congenital defects that primarily affect only enamel
formation and are not accompanied by morphologic or
metabolic defects in other body systems other than tooth
form or eruption.
Amelogenesis imperfecta has also been known as hereditary enamel dysplasia; hereditary brown enamel and
hereditary brown opalescent teeth.
Figure 90
Etiology
The trait of AI can be transmitted by either an autosomal
dominant, autosomal recessive, or X-linked mode of
inheritance.
Mutations in the amelogenin gene (AMELX) and enamelin gene (ENAM) are believed to cause X-linked AI and
autosomal inherited forms of AI respectively.
Recent reports have mentioned the presence of mutations
in the KLK4, MMP-20 and DLX3 genes in the etiology
of AI.
Clinical features
The incidence varies between 1:718 (Sweden) and 1:14,000
(Michigan, USA); the data varies widely depending on the
geographical clustering of patients.
Affects both dentitions (deciduous and permanent);
sometimes only a part of the dentition may be affected.
Teeth exhibit a yellow to dark brown discoloration (Figure
89); the consistency varying from cheesy to hard. The
teeth exhibit pits and grooves and in some cases, enamel
may be entirely absent (Figure 90).
Classification
Amelogenesis imperfecta has been subjected to numerous
classifications but the one proposed by Witkop Jr (1989) is
the most popular and widely used classification to date.
It is based on the predominant clinical and radiographic
appearance of the defect and on the mode of inheritance
of the trait (Table 6).
Table 6
Type
Clinical appearance
Type I
Hypoplastic
Type IA
Type IB
Type IC
Type ID
Type IE
Type IF
Type IG
Hypoplastic, pitted
Hypoplastic, local
Hypoplastic, local
Hypoplastic, smooth
Hypoplastic, smooth
Hypoplastic, rough
Enamel agenesis
Type II
Hypomaturation
Type IIA
Type IIB
Type IIC
Type IID
Hypomaturation, pigmented
Hypomaturation
Snow-capped teeth
Snow-capped teeth
Type III
Hypocalcified
Type IIIA
Type IIIB
Hypocalcified
Hypocalcified
Type IV
Hypomaturation-hypoplastic
with taurodontism
Type IVA
Hypomaturation-hypoplastic with
taurodontism
Hypoplastic-hypomaturation with
taurodontism
Type IVB
Mode of inheritance
Autosomal dominant
Autosomal dominant
Autosomal recessive
Autosomal dominant
X-linked dominant
Autosomal dominant
Autosomal recessive
Autosomal recessive
X-linked recessive
X-linked
Autosomal dominant
Autosomal dominant
Autosomal recessive
Autosomal dominant
Autosomal dominant
its normal counterpart. Each child has a 50% risk of inheriting the faulty gene and the disorder. In other words, one
copy of the altered gene in each cell is able to cause the
disorder.
In an X-linked disorder, one normal copy of a gene on
the X chromosome is generally sufcient for normal function. Women who have a defective gene on one of their
two X chromosomes are protected by the normal copy of
the same gene on the second chromosome. This benet is
not available to men since they have one X and one Y
chromosome. Each male child of a mother who carries the
defect has a 50% risk of inheriting the faulty gene and the
disorder. Each female child has a 50% chance of being a
carrier like her mother. Males with an X-linked form of
disease are generally more severely affected than females
with similar mutations.
The Lyonization effect/Lyon hypothesis/X chromosome
inactivation Since the female has two X chromosomes,
a mechanism exists to ensure that only one of them
(even if both are normal) will remain active for functioning, and the other X chromosome gets switched-off or
inactivated.
A recessive disorder is thought to arise from a defect in
genes that code for enzymes, while a dominant disorder
results from mutation in a gene that codes for a structural
protein.
54
Type I/Hypoplastic AI
Clinical features There is a deciency of enamel matrix
with subsequent normal mineralization.
The enamel does not develop to normal thickness; at
places the enamel is so thin that crowns do not meet at
contact points. The decreased crown height leads to anterior open bite (in about 50% of cases).
Enamel matrix defects vary from pinpoint to pinhead
size pits arranged in rows or columns on labial or buccal
surfaces of permanent teeth. Sometimes pits and grooves
of hypoplastic enamel are seen in a horizontal fashion
across the middle-third of teeth.
In type IE, the carrier females have alternating bands
of normal thick and abnormal thin enamel (Lyonization
effect).
Enamel agenesis is seen in Type IG where the tooth has
a rough granular surface and has no contact with adjacent
teeth.
Radiographic features The enamel, although thin, shows
normal contrast from dentin. Square-shaped crowns.
Type II/Hypomaturation AI
Clinical features Enamel is of normal thickness but has
a mottled appearance. It is slightly softer than normal
Figure 91
Management
The three principles of management of patients with AI
involve:
1.
2.
3.
2.
3.
2.
3.
The differentiation of odontoblasts from ectomesenchymal cells of the dental papilla following an organizing
influence of the inner enamel epithelium.
Formation of organic matrix
Mineralization of the formed matrix to the extent of
nearly 70%.
Dentinogenesis Imperfecta
Dentinogenesis imperfecta (DI) is a hereditary developmental defect of dentin formation resulting in the appearance of opalescent teeth and occurring in the absence
of any systemic disorder. It is the most common disorder
affecting the structure of dentin.
Dentinogenesis imperfecta has also been referred to as
Capdeponts teeth and hereditary opalescent dentin.
Etiopathogenesis
Table 7
Classification by
Shields et al
Classification by Witkop
Gene mutation
DI type I
Dentinogenesis imperfecta
Mutation in
COL1A1 and
COL1A2 genes
DI type II
Mutation in DSPP
gene at 4q 21.3
DI type III
Mutation in DSPP
gene
Figure 92
Figure 93
Shell teeth are so called because they appear like thin shells
of dentin covering large pulps. The enamel may be of normal thickness. The deciency in thickness of dentin is due
to insufcient or defective dentin formation and it may
extend to involve even the crown.
Histological features
Other than a thin layer of mantle dentin, the rest of the dentin has fewer tubules, which are wide and irregular. Areas of
atubular dentin are also seen partly or totally obliterating
the pulp chamber and root canals. The dentinoenamel
junction is smooth instead of being scalloped. Vascular
inclusions are sometimes seen in the dentin (probably remnants of pulp tissue). Biochemical analysis of dentin shows
increased water content and a decreased mineral content
when compared to normal dentin. Following the loss of
enamel, the defective dentin rapidly wears away because
of the low micro hardness.
57
Figure 94
Management
The rapid loss of enamel and wearing away of dentin has
to be compensated in order to maintain vertical dimension
for function. However, like many other structural defects
in teeth, it is increasingly difficult to retain the affected
teeth. The risk of pulp exposure is high in such teeth, and the
cervical constriction renders it liable to cervical fracture
when teeth are covered by crowns or used as abutments.
Dentin Dysplasia
The term dentin dysplasia was given by Rushton to
describe a rare hereditary condition affecting dentin formation that was first described by Ballschmiede in 1920 as
rootless teeth. The enamel is normal, while the dentin is
atypical and there is abnormal pulpal morphology. It has
also been referred to as rootless teeth.
Dentin dysplasia is of two types; Type I/radicular dentin dysplasia (rootless teeth) is so called because the
crowns of the primary and permanent teeth seem normal
while the roots appear short/stunted. Type II/coronal dentin
dysplasia shows a defect in the crowns of primary teeth,
but the lengths of the roots seem normal.
Etiopathogenesis
Hereditary (autosomal dominant trait).
Mutations in the DSPP gene (that encodes for dentin
sialophosphoprotein) may be responsible for some cases of
type II dentin dysplasia. (This is similar to the mutation
seen in DI type II).
58
Dentinogenesis
imperfecta
Color
Yellow to brown to
bluish
Yellow to brown to
bluish
Crown morphology
Obliteration of pulp
chamber
Occurs 56 years
after eruption of the
tooth (type II)
Thistle tube-shaped
pulp chamber
Mostly present
Absent
Root morphology
Normal appearing
roots (type II)
No roots (type I)
Management
Meticulous maintenance of oral hygiene is the key to prolonged retention of teeth affected by dentin dysplasia,
although the presence of short roots and periapical lesions
may make it seem a daunting task for the dentist. Endodontic
and periodontal treatment is accomplished reasonably well
in teeth affected by coronal dentin dysplasia.
Regional Odontodysplasia
Regional odontodysplasia is a rare, localized, developmental disorder of dental origin with affected teeth having
characteristic clinical and radiographic appearances. This
condition has been described under various names by different authors, but Zegarelli (1963) is credited with coining the term odontodysplasia to describe a peculiar dental
anomaly of unknown cause. Later, Pindborg (1970) preferred the term regional odontodysplasia to describe the
segmental and localized nature of this condition.
Regional odontodysplasia has also been termed as ghost
teeth, odontogenesis imperfecta, odontogenic dysplasia, unilateral dental malformation, AI non-hereditaria segmentalis
and localized hypoplasia (Turner teeth).
The term regional odontodysplasia best describes the
segmental and localized nature of this condition that is of
unknown etiology and affects enamel, dentin and pulp.
Etiology
The cause of this rare dental developmental disorder is not
precisely known, but a few theories have been proposed.
1.
2.
3.
A somatic mutation in early tooth development (probably in the developing dental lamina) that disrupts
odontogenesis. This may explain the involvement of
both deciduous and permanent dentitions.
A disturbance in vascular supply that creates local
ischemia, thereby affecting odontogenesis. Many cases
of odontodyplasia have been reported to be associated
with hemangiomas or vascular nevi adjacent to the
affected teeth. (Some researchers have even proposed
that the irregularities in the dental hard tissues are a
result of the blood overflow from vessels that affects
the local concentration of magnesium and sodium
around the crystals).
Activation of a latent viral infection of the tooth germ
during odontogenesis.
Other theories that have been proposed but are unsatisfactory to explain the occurrence of ghost teeth are a hereditary basis, medications taken during pregnancy, irradiation,
and failure of migration of neural crest cells, systemic
disorders, Rh incompatibility, nutritional and metabolic
disorders.
59
Clinical features
Age at presentation is variable (423 years); the condition
is often diagnosed at the time of eruption of primary and
permanent teeth. Affects both primary and permanent
dentitions.
Generally, the disturbance is localized to one arch, most
often the maxillary left quadrant (incisors and canines).
Females are affected more often than males (1.7:1); there
is no racial predilection observed.
Pulp or periapical pathology may be associated with
the affected teeth even in the absence of gross dental caries (the pathogens may gain access to the pulp through the
clefts in the defective enamel and dentin). Patient may
present with delayed/incomplete eruption of teeth and
unesthetic appearance of affected teeth.
Affected teeth appear hypoplastic and small, discolored
yellow to brown with the enamel surface having shallow
furrows to severe pits and grooves. The gingiva surrounding the affected teeth may be enlarged.
Figure 95
Differential diagnosis
Radiographic appearance
The affected teeth have a ghost-like appearance because
of a decreased radiopacity and lack of distinction between
the enamel and dentin.
The teeth have an irregular shape with hypoplastic
crowns. Affected teeth have reduced thickness of dentin with
large pulp chambers, presence of pulp calcications, incomplete root formation and wide open apices (Figure 95).
Unerupted teeth may be surrounded by a pericoronal
radiolucency (representing an enlarged follicle) with abnormal foci of calcication interspersed. The trabeculae of the
alveolar bone adjacent to the affected teeth may show rarefaction. Periapical abscesses may be seen in relation to
the affected teeth.
Histological features
Undecalcified ground sections of the affected tooth have
thin, pitted enamel with disorganized/altered prismatic
structure. The dentin is thinner than normal, and contains
large areas of interglobular dentin, containing clefts that
may communicate with the pulp. Areas of amorphous dentin and cellular dentin may also be seen with occasional
capillaries. The dentin has fewer tubules than normal.
The predentin zone is very wide. The pulp chambers are
enlarged and contain pulp stones. Dental follicles are often
enlarged and contain epithelial rests and calcications.
Scanning electron microscopy reveals thin enamel with
irregular enamel prisms and irregular crystalline aggregates.
60
Regional odontodysplasia
(RO)
Dentinogenesis
imperfecta
Hereditary basis
Absent
Present
Enamel hypoplasia
Present
Absent
Involvement of
teeth
The teeth affected by RO may also be mistaken for odontomes or as being involved by dental caries.
Management
Depending upon the vitality of the pulp, pulpotomy or
apexification may be performed to stimulate root formation. Affected primary teeth may be treated by pulpotomy
and restored with steel crowns.
However, most dentists prefer to extract the affected
teeth for esthetic reasons and rehabilitate the patient with
prosthesis. This seems a safer option as the longer the
affected teeth are retained; the higher is the risk of development of pathology. However, the clinician must weigh
the choice against the psychological effects of a youngster
having to remove teeth with a resultant decrease in alveolar bone height, potentially decreasing the ability to optimally restore/rehabilitate the affected area. The possibility
of the future use of implants may be discussed with the
patient.
CHAPTER
Orofacial Pigmentation
Disorders
Ajit Auluck, Manuel Thomas
Pigmentation of Teeth
Extrinsic Discoloration
Intrinsic Discoloration
61
Table 1
Source
Color
Etiology
Silver, amalgam
Gray, black
Iatrogenic implantation,
trauma
Graphite
Gray, black
Tattoo, trauma
Lead, mercury,
bismuth
Gray
Ingestion of paints,
medicines, poisoning
Chromogenic
bacteria
Black, brown,
green
Superficial colonization
Table 2 Type of endogenous pigments, the discoloration caused by them and disease process they indicate
62
Pigment
Color
Disease process
Hemoglobin
Hemosiderin
Brown
Melanin
Brown/black/gray
Bilirubin
Yellow
Jaundice/liver disorder
Carotene
Yellow
Precursor of vitamin A
Metal Pigmentation
Pigmentation of the oral cavity caused by foreign materials or metals is called exogenous pigmentation. The importance of recognizing oral mucosal pigmentation caused by
heavy metals lies primarily in the identification and treatment of the cause to avoid severe systemic toxic effects.
Depending on the type of metal implicated, a number of
systemic signs and symptoms may be associated with
chronic exposure of metals.
Amalgam tattoo
Amalgam tattoo is the most common solitary focal pigmentation lesion of the oral mucosa. These lesions are generally less than 1 cm (rarely amalgam tattoos can be large)
and appear as flat, gray-black to blue-black color macules.
They are usually found in close approximation to a restoration. Majority of the lesions are located on the buccal
mucosa, gingiva and alveolar mucosa with mandibular
region being more affected as compared to the maxillary
region (Figure 1). All these lesions are asymptomatic and
are discovered during routine dental examination. If particles are large enough they can be viewed with help of a
radiograph (taken with reduced exposure parameters for soft
tissues). The amalgam granules and fragments are found
mainly in the lamina propria but were sometimes also seen
Figure 1
63
Figure 2
Lead
Lead (plumbism) is an occupational hazard seen in plumbers due to acute or chronic exposure because of inhalation
of lead vapors or dust. It is also seen among the children who
chew wood painted with lead.
Clinical features include gastrointestinal symptoms like
nausea, vomiting and constipation. Patients may have encephalitis or peripheral neuritis characterized by wrist drop or foot
drop. Patients may have hypochromic anemia with basophilic stippling in red blood cells.
Oral manifestations include linear bluish black discoloration seen in the gingival margin called Burtonian line.
Gingivitis, ulcerative stomatitis, excessive salivation, metallic taste, or rarely bismuth may get deposited in the deciduous teeth too.
Silver
Argyria (silver poisoning) is caused due to chronic exposure
due to occupational hazard by silver nitrate. It can result
in pigmentation of both the skin as well as the mucous
membrane. Exposure to silver causes a violet marginal line,
often is accompanied by a diffuse bluish-gray discoloration
throughout the oral mucosa. It can also be associated with
neurologic and hearing damage. Histologically we can
observe silver particles staining the reticular fibers.
Figure 4
Bleomycin
Busulphan
Clofazimine
Chloroquine
Chlorpromazine
Cyclophosphamide
Doxorubicin
Estrogen
5-Fluorouracil
Gold
Hydroxychloroquine
Ketoconazole
Minocycline
Tetracycline
Quinacrine hydrochloride
Zidovudine
Figure 5
Figure 3
usually occur due to the hormonal changes which inuence melanocyte stimulation.
Smokers melanosis
Tobacco smokers have more intense pigmentation of oral
mucosa as compared to non-smokers. Smokers melanosis
is more common in females. Women are more commonly
affected than men because of synergistic effect between
the female sex hormones and smoking. Smoking may
cause oral pigmentation in light-skinned individuals and
Figure 6
Figure 7
Melanin pigment can be seen in the basal layer of the epithelium. Management involves treatment of the underlying cause and corticosteroid replacement therapy.
A tumor of the posterior pituitary or certain small cell
carcinomas can also secrete excessive amounts of ACTH
which can cause pigmentation of oral tissues. In ACTH
secreting tumors (paraneoplastic syndromes) the patient
manifests features of the Cushings syndrome. In both these
conditions there occurs diffuse pigmentation of oral tissues
along with associated systemic features.
Caf au lait pigmentation
It manifests as bronze or tan diffuse multifocal macular
pigmentations that appear on the skin as well as the oral
mucosa. These pale brown macules may vary considerably
in size. They have widespread distribution and can occur on
the face, neck or the oral cavity. Because of the pale brown
color these lesions are called as caf au lait spots. It is usually associated with neurofibromatosis (von Recklinghausens
syndrome), Albrights syndrome (polyostotic fibrous dysplasia) and PeutzJeghers syndrome.
Caf au lait pigmentations in neurobromatosis have
smooth borders and are associated with axillary freckling.
It is an autosomal dominant inherited disease with multiple skin nodules.
In PeutzJeghers syndrome patients have intestinal polyposis along with oral macular pigmentations that appear
around the mouth (Figure 7) and on the ngers. When
such lesions are observed, a detailed history of the patient
should be taken about gastrointestinal complaints as well
as the family history for intestinal polyps. These pigmented melanotic spots do not require any treatment and
are not associated with any risk for malignant transformation. However, the patient should be monitored for the
Figure 8
Figure 9
Figure 10
Hemochromatosis
67
Figure 11
Figure 12
Figure 13
Yellowish discoloration of palate in a patient with jaundice.
Courtesy: Dr Ajit Auluck
the melanocytes and accumulation of melanin laden macrophages in the superficial connective tissue.
Cyanosis
Rarely generalized discoloration of oral mucosa can also
be associated with cyanosis in which entire oral mucosa may
become blue black in color when reduced hemoglobin level
reaches above the critical value of 5 g/100 ml.
Beta carotene
Yellowish discoloration of oral mucosa can also be due to
consumption of large amounts of beta carotene in patients
having metabolic disorders that impairs conversion of
beta carotene to vitamin A.
Liver disease
Yellowish discoloration of oral mucosa can also be due to
liver diseases (jaundice) when bilirubin level increases
more than 23 mg/dl (Figure 11). To distinguish between
the two conditions we must examine the sclera of the eyes.
In jaundice sclera will also be yellow in color as bilirubin
pigments stain the reticular fibers of sclera whereas betacarotene will not stain the sclera (Figure 12).
Figure 14
Figure 15
Figure 16
Table 4
AAsymmetry: One half of the lesion does not match the other half
BBorder irregularity: The edges are ragged, notched, or blurred
CColor variegation: Pigmentation is not uniform and may display
shades of tan, brown, or black; white, reddish, or blue discoloration is
of particular concern
DDiameter: A diameter greater than 6 mm is characteristic, although
some melanomas may have smaller diameters; any growth in a nevus
warrants an evaluation
EEvolving: Changes in the lesion over time are characteristic; this
factor is critical for nodular or amelanotic (non-pigmented) melanoma,
which may not exhibit the classic criteria above
Figure 17
These are relatively uncommon cause of oral mucosal pigmentation and may appear as blue or black focal pigmented
areas. Histologically, there is increase in the number of
nevus cells in the epithelium, connective tissue or both.
Accordingly, nevi are classified as junctional nevi, intradermal nevi or intramucosal or compound nevi.
Usually melanocytic nevi develop during childhood.
Most nevi originate from the basal layer of melanocytes and
proliferate in the epithelium along the junction with the
connective tissue and are called as junctional nevi. Later,
these melanocytes drop off into the connective tissue to
form islands or cluster of melanocytes and are called as
compound nevi. Eventually all the cells leave the surface
epithelium and reside in the dermis or submucosa so called
as intradermal or intramucosal nevi.
Blue nevi arise from dermal or mucosal melanocytes that
persist in the connective tissue during embryonic neural
crest migration and therefore do not arise from junctional
activity at the epithelium and connective tissue interface
(Figure 18). In blue nevi there are spindle-shaped melanocytes which synthesize huge amounts of melanin pigment.
Blue nevi can also occur along with ocular pigmentation
along the distribution of trigeminal nerve and is called
nevus of Ota (Figure 19).
Malignant melanoma
Described in Chapter 13 on page 368.
Clinical evaluation of pigmented lesions
When a patient presents with a pigmented oral lesion a
detailed medical and dental history must be recorded.
A complete extraoral and intraoral examination should be
done and relevant laboratory tests must be advised.
Figure 18
PIGMENTATION OF TEETH
Figure 19
Discoloration of the tooth is one of the most frequent reasons why a patient seeks dental care. Tooth discolorations
are usually esthetically displeasing and psychologically
traumatizing. There are many factors that could steal the
sparkle from a smile. An understanding of the etiology
of tooth discoloration is important to a dentist in order to
come to the correct diagnosis, which allows the dental
practitioner to explain to the patient the exact nature of
the condition. In some instances, the mechanism of staining may have an effect on the outcome of treatment and
influence the treatment options the dentist will be able to
offer to patients.
The causes for tooth discoloration can be classied
according to the location of the stains, either as extrinsic
or intrinsic. Extrinsic discoloration lies on the tooth surface or in the acquired pellicle. The intrinsic discoloration
occurs when the chromogens are deposited within the bulk
of the tooth, which may be of local or systemic origin.
The coronal aspect of the tooth consists of enamel, dentin and pulp. Any change to these structures during odontogenesis or post eruption can cause an alteration in the
outward appearance of the tooth because of the change in
the light transmitting and reecting properties.
Extrinsic Discoloration
Extrinsic discolorations are defined as discolorations located
on the outer surface of the tooth structure and caused by
topical or extrinsic agents. This can be divided into two
groups; direct staining by the compounds incorporated into
the pellicle layer and producing the stain as a result of the
basic color of the chromogen, and indirect staining were
71
Flowchart 1
Any discoloration of the oral mucosa
Blue/black melanin
pigmentation
Blanch on
pressure
Do not blanch
on pressure
Tumor masses
Diffuse
pigmentation
Focal
pigmentation
Hemangioma
Varices
Early hematoma
Telangiectasia
Kaposis sarcoma
Petechiae
Ecchymosis
Hemochromatosis
Neuroectodermal
tumor of infancy
Malignant
melanoma
Physiologic
pigmentation
Endocrine disorders
Smokers melanosis
Drug-induced
pigmentation
Post inflammatory
pigmentation
HIV infection
Melanotic macule
Nevi
Amalgam tattoo
Nathoo type 1: The chromogen binds to the tooth surface. The color of the chromogen is similar to that of dental stains caused by tea, coffee, bacteria, and metals.
Nathoo type 2: The colored material changes color after
binding to the tooth. The stains actually are Nathoo type 1
food stains that darken with time.
Nathoo type 3: The colorless material or prechromogen
binds to the tooth and undergoes a chemical reaction to
cause a stain. These stains are caused by carbohydrate-rich
foods, stannous uoride, and chlorhexidine.
Factors responsible for extrinsic discoloration
Diet factors Deposition of tannins found in tea, coffee,
and other beverages cause brown stains on the surface
of the teeth. Commercially available soft drinks and food
products containing permitted synthetic food colors (red
color: Ponceau 4R, carmoisine, erythrosine, yellow color:
tartrazine pyrazolone, sunset yellow FCF, blue color:
indigo carmine, brilliant blue FCF, green color: fast green
FCF) and additives can temporarily cause discoloration of
the teeth and the oral mucosa (Figure 20).
Flowchart 2
Bluish/black discoloration of oral mucosa
Usually due to metals/melanin pigment
Localized pigmentation
Diffuse pigmentation
Physiological pigmentation
Present since birth
Smokers melanosis
Melanotic macule
Small size, mostly on lips, increase
in melanin synthesis
History of smoking
Endocrine disorders like Addisons
disease/Cushings syndrome
Nevi
Increased proliferation of
melanocytes, usually from birth
Malignant melanoma
Dark, irregular borders, asymmetric
and rapid growth
Algorithm for differential diagnosis of pigmented lesions in the mouth with characteristic features which help in diagnosis
*Biopsy must be advised if there is increase in size, change in color or any proliferative changes associated in
pigmented lesions
Table 5
Classification
Factors responsible
Examples
Color
Non-metallic stains
Direct stains
Diet
Brown to black
Oral hygiene
Yellow/brown
Brown/black/green, orange
Habits
Tobacco smoking/chewing
Pan chewing
Dark brown/black
Red-black
Non-metallic stains
Indirect stains
Medications
Yellow brown
Yellow
Green-gray
Metallic stains
Indirect stains
Medications
Black
Green
Violet to black
Golden brown
Gray
Occupation
Black
Blue green
Green
Deep orange
Figure 20
Figure 21
74
Occupation related Industrial exposure to iron, manganese, and silver may stain the teeth black. Mercury and
lead dust can cause a blue-green stain; copper and nickel,
Figure 22
Figure 24
Figure 23
Figure 25
Intrinsic Discoloration
There are several causes of intrinsic tooth discolorations
which have either an endogenous or exogenous origin.
These changes may occur during or after odontogenesis.
During odontogenesis, teeth may become discolored from
the changes in the quality or quantity of enamel or dentin,
or from the incorporation of discoloring agent into the
hard tissues. Post-eruption discolorations occur when the
discoloring agent enter the hard tissues. They may originate from the pulp cavity or the tooth space.
Pre-eruptive causes of intrinsic
discoloration (Table 6)
Metabolic causes The diseases that have the potential
to cause neonatal hyperbilirubinemia may cause the
Figure 26
Table 6
76
incorporation of bilirubin into developing teeth, producing jaundice like yellow-green tint within the dental hard
tissue known as chlorodontia. These diseases include sickle
cell anemia; thalassemia; hemolytic disease of the newborn due to Rhesus factor, ABO, or other erythrocyte antigen incompatibility (erythroblastosis fetalis/icterus gravis
neonatorum); biliary atresia; bile duct obstruction; biliary
hypoplasia; and cholestasis associated with sepsis.
Congenital erythropoietic porphyria (Gnthers disease) is
a rare, autosomal recessive disorder of porphyrin metabolism,
resulting in an increase in the formation and excretion or
porphyrins. The porphyrin pigments have an afnity for calcium phosphate and are incorporated into teeth during dental formation and this causes a characteristic reddish-brown
discoloration of the teeth, called erythrodontia. The affected
tooth shows a red uorescence under ultraviolet light.
Alkaptonuria, also known as phenylketonuria or ochronosis is an inborn error of metabolism of tyrosin and phenylalanine causing a build-up of homogentisic acid. This
results in a brown discoloration of the permanent dentition.
Disturbance during development of a tooth Enamel hypoplasia may result due to the disturbance of the developing
tooth germ following trauma, infection or nutritional deficiency giving rise to localized or generalized enamel defects
(Figure 27).
Periapical odontogenic infections of the primary teeth
can disrupt normal amelogenesis of the underlying permanent successors and can cause localized enamel hypoplasia. Trauma to developing, yet unerupted, teeth can also disturb
amelogenesis and may result in enamel hypoplasia, which
is visualized as a localized opacity on the erupted tooth.
Such teeth commonly are referred to as Turners teeth.
Classification
Factors responsible
Examples
Color
Pre-eruptive
Metabolic disorders
Hyperbilirubinemia
Porphyria
Alkaptonuria
Yellow-green
Reddish brown
Brown
Localized
Turner tooth
Generalized
Infection (maternal or childhood)
Nutritional deficiency
Molar incisor hypomineralization (MIH)
White to
yellow to
brownish
Genetic disorder
Amelogenesis imperfecta
Dentinogenesis imperfecta
Dentin dysplasia
Systemic syndrome
e.g. epidermolysis bullosa
Yellow brown
Blue brown
Yellow
Yellow
Medication
Tetracycline
Minocycline
Ciprofloxacin
Fluoride
Figure 27
Crown formation begins in utero; therefore, the potential for extensive intrinsic discoloration of the primary
dentition may be present throughout pregnancy. Although
rare, maternal rubella or cytomegalovirus infection, maternal vitamin D deciency, drug intake during pregnancy
and toxemia of pregnancy can lead to tooth discoloration,
which generally manifests as a focal opaque band of
enamel hypoplasia. Such defects will be chronologically
laid down in the teeth depending on the state of development at the time of interference and the effect is directly
related to the degree of systemic upset. There may be pitting or grooving which predisposes to extrinsic staining of
the enamel in the region of tooth disturbed, often then
becoming internalized.
Crown formation of the permanent dentition occurs until
the child is aged approximately 8 years. Systemic postnatal
infections (e.g. measles, chicken pox, streptococcal infections,
scarlet fever) can also cause enamel hypoplasia. The band
like discolorations on the tooth are visualized where the
enamel layer has variable thickness and becomes extrinsically stained after tooth eruption.
Vitamins C and D, calcium, and phosphate are required for
healthy tooth formation. Deciencies can result in exposurerelated or dose-related enamel hypoplasia.
Molar-incisor hypomineralization (MIH) is an idiopathic condition characterized by severe hypomineralized
enamel affecting incisors and permanent rst molars.
The enamel defects can vary from white to yellow to
brownish areas but they always show a sharp demarcation
between sound and affected enamel. The nature of the
enamel is porous and brittle, breaking down shortly after
eruption under masticatory forces, often resembling
enamel hypoplasia, but distinguished by having irregular
Figure 28
enamel has chipped away. The teeth have a typical amberlike translucency or opalescence against reected light,
and there color varies from different shades of yellow to
bluish-brown. DI type III (brandywine isolate hereditary
opalescent dentin) is a very rare autosomal dominant disorder which occurs in a racial segregate in Maryland,
United States. It is similar in appearance to type I and II
but with radiographic appearance of shell teeth with multiple pulpal exposure in the primary dentition.
Dentin dysplasia occurs in two types. In type I DD the
primary and permanent dentition are of normal shape and
form but may have an amber translucency. Teeth with
type II DD are characterized by thistle shaped pulp chamber and pulp stones with brown tooth discoloration.
Defects in enamel formation may also occur in a number of systemically involved clinical syndromes such as
vitamin D dependent rickets, epidermolysis bullosa, Ehlers
Danlos syndrome and pseudohypoparathyroidism. Patients
with epidermolysis bullosa may have enamel hypoplasia
and pitting, which produce a yellowish tint and the
patients are at risk for caries.
Figure 29
Figure 30
Medications
Tetracycline, a broad spectrum antibiotic, is known to
cause intrinsic discoloration when prescribed during tooth
development. Tetracycline staining results from systemic
administration of the drug, which chelates with the calcium ions on the surface of the hydroxyapatite crystals as
a stable orthophosphate complex. Dentin has been shown
to be more heavily stained than enamel. The severity of
the discoloration produced depends on the type of tetracycline used, the dosage and the period of time it was taken
for, as well as the age at the time of administration.
Tetracycline should be avoided from 29 weeks in utero
until full term, in breast feeding mothers and in children
up to the age of 12 years to avoid discoloration of the
developing teeth. It has been shown that the discoloration
occurs with the greatest frequency in the developing dentition when total administration is over 3 g, or the treatment exceeds 10 days. The various analogs of tetracycline
produce different color changes, for instance, chlortetracycline produces a slate gray color and oxytetracycline
causes a creamy discoloration.
Teeth affected by tetracycline have a yellowish or browngray appearance which is worse on eruption and diminishes with time (Figure 29). The affected teeth also uoresce
under ultraviolet light, giving off a bright yellow color.
Exposure to the sunlight can change the color to brown; the
anterior teeth are particularly susceptible to this photooxidation induced color change. In severe cases of tetracycline involvement enamel hypoplasia may result.
Tetracycline discoloration has been classied according
to the extent, degree and the location of the tetracycline
involvement (Jordan and Boksman).
78
DENTAL FLUOROSIS
Dental fluorosis is characterized by enamel discoloration
resulting from subsurface hypomineralization due to the
excessive ingestion of fluoride during the early maturation
stage of enamel formation. Fluoride sources are numerous
and include naturally or artificially fluoridated drinking
water, commercially available beverages, foods prepared
in fluoridated water, chewable vitamins, oral healthcare
products (e.g. toothpastes, mouthrinses, oral fluoride supplements), and professional fluoride products prescribed
by dentists. The severity is related to age and dose, with
the primary and permanent dentitions both being affected
by endemic fluorosis. The enamel is often affected and may
vary from areas of flecking to diffuse opaque mottling
superimposed on to chalky white or dark brown/black
appearance (Figure 31). The dark discoloration thought to
be post-eruptive by a process of internalization of the
extrinsic stains into the porous enamel.
Figure 31
Table 7
Classification
Factors responsible
Examples
Color
Post-eruptive
Dental conditions
Dental caries
Incipient
Active
Arrested
Tooth wear
Aging
Chalky white
Yellowish brown
Dark brown to black
Yellowish
Yellowish
Pulpal causes
Gray-brown
Yellowish to yellowish brown
Pinkish
Dental materials
Amalgam
Composite glass ionomer cement (GIC)
Intracanal medicaments (e.g. iodoform, ledermix)
Obturating materials and sealers
Blue-gray
Yellowish brown
Brownish gray
Grayish
Figure 32
Pulpal causes
Bacterial, mechanical, or chemical irritation to the pulp may
result in tissue necrosis and the release of disintegration
by-products that might penetrate the tubules and discolor
the surrounding dentin. Trauma that occurs to erupted teeth
also causes discoloration. After acute trauma, intrapulpal hemorrhage will give the tooth a reddish tinge. Occasionally, in
younger patients, the color may return to normal as the
inammation subsides. More often, the discoloration changes
to gray-brown in a matter of days as the pulp becomes
necrotic. Hemolysis of the red blood cells would follow and
release the heme group to combine with the putrefying
pulpal tissue to form black iron sulde. In vitro studies
have recently shown that the major cause of discoloration of
80
Figure 33
Dental Materials
Clinical examination
Dental restorations most commonly cause intrinsic discoloration. Amalgam restorations can generate corrosion
products, leaving a blue-gray color in the tooth, especially
in large cavity preparations with undermined enamel
known as amalgam blue.
Metal pins and prefabricated posts are sometimes used
to reinforce composite restoration on the anterior dentition.
Discoloration from inappropriately placed pins and posts
is caused by the metal seen through the composite or the
tooth structure.
Microleakage around composite or glass ionomer restoration causes staining. Open margins may allow chemicals
to enter between the restoration and the tooth structure
and discolor the underlying dentin.
Several intracanal medicaments are liable to cause
internal staining of the dentin. Phenols or iodoform based
medicaments sealed in the root canal and the chamber
are in direct contact with dentin, allowing penetration and
oxidation. These compounds discolor dentin gradually.
Tetracyclines (e.g. ledermix-triamcinolone acetonide and
demethylchlortetracycline) used within the tooth for endodontic therapy may also cause dark gray-brown discoloration.
Obturating materials are frequent cause for single tooth
discoloration. Incomplete removal of obturating material
and sealer remnants in the pulp chamber, mainly those
containing metallic components, often result in dark
discoloration.
Diagnosis
History The patients history of tooth discoloration provides useful information regarding the etiology. The history includes the following:
Management
The treatment of tooth discoloration consists of identifying the etiology and implementing the required therapy.
Scaling and polishing of the teeth remove many extrinsic
stains. For more stubborn extrinsic discoloration and
intrinsic stain, various bleaching techniques may be
attempted. Tooth bleaching can be performed externally,
termed night guard bleaching or vital tooth bleaching, or
intracoronally in root-filled teeth, called non-vital tooth
bleaching.
Teeth discolored by dental caries or dental materials
require the removal of the caries or restorative materials,
followed by proper restoration of the tooth. Partial (e.g.
laminate veneers) or full-coverage dental restorations may
be used to treat generalized intrinsic tooth discoloration in
which bleaching is not indicated or in which the esthetic
results of bleaching fail to meet the patients expectations.
81
CHAPTER
Bacterial Infections
Viral Infections
Scarlet Fever
Infectious Mononucleosis
Diphtheria
Tularemia
Measles (Rubeola)
Erysipelas
Impetigo
Melioidosis
Sinusitis
Tetanus
Actinomycosis
Histoplasmosis
Noma
Blastomycosis
Botryomycosis
Mucormycosis
Rhinoscleroma
Aspergillosis
Cat-scratch Disease
Cryptococcosis
BACTERIAL INFECTIONS
SCARLET FEVER
Scarlet fever is also known by the names scarlatina, scarlatinella and scarlatiniform rash. It is caused by an infection
with a pyogenic exotoxin-producing group A -hemolytic
streptococci. The upper respiratory tract is the usual portal
of entry. Most cases occur between 1 and 10 years of age
and may occasionally be seen in adults.
The organism expresses an erythrogenic toxin that acts
on the blood vessels to produce the typical skin rash.
Clinical features
The incubation period of the bacteria ranges from 1 day
up to a week. Patients will present with fever and skin rashes
82
within the first 2 days of the infection. The fever and rash
usually resolve in about 7 days. Other associated clinical
features include headache, tonsillitis, pharyngitis and
lymphadenopathy.
The scarlet fever rash appears bright red and mimics a
sunburn appearance. Normal pin head-sized areas of the skin
may project through the rash giving rise to a sandpaper
like surface texture, which is popularly referred to as sandpaper rash or sunburn with goose bumps or goose pimples.
The skin rashes are commonly seen over the trunk,
extremities, neck, groin and specically along Pastias lines
(darkening of the normal skin fold/creases, such as the
axillary crease, anticubital crease).
Once the rash resolves, a 38 months phase of desquamation begins, which is characterized by peeling away of
the skin in large akes.
Oral manifestations
Patients may present with circumoral pallor. The soft palate, pharynx, tonsillar region and the tongue are commonly
affected. The oral cavity appears extensively erythematous
and edematous (stomatitis scarlatina). Occasionally a yellowish-white exudate may be seen in the tonsillar crypts.
During the rst 2 days of the infection the dorsal surface
of the tongue exhibits a white coat through which the
Management
A single episode of scarlet fever will usually confer permanent antitoxin immunity. However, recurrences are not
unusual. This is due to the fact that toxin produced by other
strains is not neutralized and the bacterial immunity is
temporary.
Penicillin is the drug of choice. Erythromycin may be used
in patients who are allergic to penicillin. Acetaminophen
or ibuprofen may be used to alleviate pain and manage
fever. Analgesic mouthrinses (benzydamine hydrochloride) may be used for stomatitis.
DIPHTHERIA
Diphtheria is an acute infectious disease caused by toxinproducing Corynebacterium diphtheriae or Klebs-Lffler
bacillus (after Klebs who discovered the bacillus and Lffler
who isolated the bacillus in pure culture).
The bacteria reside in the upper respiratory tract of the
infected individual and cause local infection of the upper
respiratory tract and occasionally the skin and the heart,
kidneys, and peripheral nerves.
It spreads through droplet infection and direct contact.
The incubation period lasts for a few days, following
which the bacterium expresses an exotoxin that causes
tissue necrosis that subsequently spreads peripherally.
General clinical and oral manifestations
Tonsillitis is usually the first clinical finding. Patients
present with fever, malaise, headache, sore throat, foul
taste/breath and cervical lymphadenopathy. The exotoxin
causes necrosis of the soft tissues producing a thick, grayish white membrane. As the infection progresses patients
may complain of difficulty in speech, swallowing and
breathing.
Diphtheria involving the skin causes excoriation of the
skin of the nasal and perinasal regions.
The grayish pseudo membrane (diphtheritic membrane
covers necrotic ulcerated areas of the mucosa and contains
Table 1
Type of tularemia
Mode of transmission
Clinical features
Ulceroglandular tularemia
Glandular tularemia
Pneumonic tularemia
Oropharyngeal tularemia
Oculo-glandular form
Management
Tularemia responds well to antibiotics. The drugs of choice
include aminoglycosides (gentamicin, streptomycin) fluoroquinolones (ciprofloxacin, gatifloxacin) and chloramphenicol.
ERYSIPELAS
Erysipelas is an acute inflammation of the skin, with marked
involvement of cutaneous lymphatic vessels. It is usually
caused by -hemolytic Streptococcus pyogenes of group A.
Erysipelas has to a lesser extent been caused by group B, C, or
G streptococci, and occasionally by Staphylococcus aureus.
It has also been referred to as St. Anthonys re after
the name of the Egyptian monk who is believed to have had
the powers to cure this disease.
Clinical features
The typical lesion of erysipelas is evident as an erythematous, well defined area that may be warm to touch. Occasionally a butterfly rash mimicking lupus erythematosus
may be seen when the bridge of the nose is involved. Fever
of sudden onset is a typical feature. Erysipelas is usually
seen in young or old patients and in systemically compromised patients.
The typical rash of erysipelas can affect skin on any
part of the body. However previous sites of trauma are the
most commonly affected. The common sites affected
include the legs, cheeks, eyelids and bridge of the nose.
The infection causes destruction of the cutaneous lymphatic vessels. This in turn increases the susceptibility for
future recurrences (30% recurrence rate).
Management
Long-term antibiotic therapy with narrow spectrum penicillins (benzylpenicillin) or macrolides may help in preventing recurrent erysipelas infection.
84
IMPETIGO
Impetigo is a highly contagious superficial skin infection
caused by -hemolytic streptococci and Staphylococcus
aureus. Impetigo is considered as the most common bacterial
dermal infection in children. It is more common in children receiving dialysis.
Clinical features
Impetigo usually affects children in the age group of 26
years. It spreads via direct skin contact. The incidence of
impetigo is greatest in the summer months, and the infection most often occurs in areas with poor hygiene and in
crowded living conditions.
There are two types of impetigo: non-bullous (impetigo
contagiosa) and bullous.
The host response to the infection results in the nonbullous type of impetigo. On the other hand, the bullous
form is not dependent on the host response but results
from the direct action of the staphylococcal toxin.
Non-bullous impetigo The skin of the face and the
hands and legs are commonly affected sites. The infection
begins as a solitary red macule or papule that almost
immediately turns into a vesicle. The vesicle ruptures to
form an erosion, and the contents dry to form characteristic honey-colored crusts that may be pruritic. These lesions
may spread to surrounding areas by autoinoculation.
Bullous impetigo Bullous impetigo is described in detail
in Chapter 7 on Vesiculobullous Disorders.
MELIOIDOSIS
Meliodosis is a potentially fatal bacterial infection caused
by exposure to soil or water contaminated with the bacterial species Burkholderia pseudomallei. The causative
TETANUS
The word tetanus comes from the Greek tetanos, which is
derived from the term teinein, meaning to stretch. Tetanus,
commonly called lockjaw, is an acute neurologic disease
that results from wound contamination with Clostridium
tetani, an anaerobic, gram-positive, motile, spore-forming
rod characterized by generalized muscle rigidity and spasm,
sometimes associated with autonomic dysfunction.
Pathophysiology
It is believed that although most wounds may be contaminated with the spores of Clostridium tetani, the germination and toxin production occurs only in wounds with
low oxidation reduction potential, such as those with devitalized tissue, foreign bodies, or active infection. The bacteria produce two exotoxins: tetanolysin (role still unclear)
and tetanospasmin. Tetanospasmin is the neurotoxin responsible for the clinical manifestations of the disease. The toxin
spreads hematogenously to the peripheral nerves and travels in a retrograde fashion along the nerve fibers to reach
the central nervous system where it blocks the release of
gamma-aminobutyric acid (GABA) from presynaptic
inhibitory neurons. This loss of inhibitory impulses results
in the cardinal clinical manifestations of reflex irritability
and autonomic hyperactivity.
Clinical features
Tetanus is usually seen in young adults who are prone to
traumatic injuries. Four types of tetanus are recognized
based on the clinical presentation: localized, generalized,
cephalic and neonatal.
The localized form of tetanus is characterized by a limited area of muscular spasm that is conned to the area of
the entry of the bacilli.
The cephalic subtype of tetanus is characterized by cranial nerve palsies that often precede trismus. Patients present with cranial nerve palsy (usually facial nerve is
affected). Approximately two thirds of cases progress to
generalized tetanus. The incidence of cephalic tetanus
ranges from 0.9 to 3.0%. The cephalic form results from
injuries sustained to the head and neck region, tooth
extraction or chronic tympanitis. Some patients may present with incomplete Bells palsy.
Generalized tetanus is the most common form of the
disease and carries the highest mortality. Incubation periods for the generalized form range from a few hours to
greater than 1 month.
Trismus or lockjaw is the initial presentation in 75% of
cases. Facial muscle spasm may cause the classic sneering
grin of risus sardonicus. Motor ndings progress to involve
the neck, trunk and extremities, eventually leading to abdominal rigidity and opisthotonus. The muscle spasms may be
sustained or paroxysmal. In severe cases the spasm of
85
ACTINOMYCOSIS
Actinomycosis is subacute to chronic, suppurative granulomatous disease that tends to produce draining sinus tracts.
It is caused by anaeroboic gram-positive, non-acid fast
bacilli. The common isolates in humans include Actinomyces
naeslundii, A. israelii, A. meyeri, A. viscosus and rarely
A. odontolyticus. Kalfas et al (2001) identified a new species, Actinomyces radicidentis that was isolated from apical
periodontitis.
Von Langenbeck noted the rst case of human actinomycosis in 1845. Bollinger and Harz in 1877, named the
genus Actinomyces when they described the etiologic
agent of bovine actinomycosis (lumpy jaw) and called it
Actinomyces bovis.
Clinical features
Actinomycosis is mostly found in young adults. Women
are less frequently affected than men. Based on the site of
involvement, actinomycosis can be grouped into the cervicofacial (55%), pulmonary (15%), abdominal and pelvic
(25%) and cutaneous and genitourinary actinomycosis
(5%). Cutaneous actinomycosis is extremely rare and these
are said to arise from wounds contaminated with saliva or
as a consequence of hematogenous dissemination following a dental procedure. However primary cutaneous actinomycosis have also been reported. The genitourinary
form has been reported in patients using intrauterine contraceptive devices.
The presenting symptoms of pulmonary actinomycosis
are fever, cough, thoracic pain and dyspnea. The sputum is
mucopurulent or even sanguineous. With the appearance
of stulae, the disease spreads to the mediastinum, the pericardium, and nally to the skin of the chest.
Actinomycosis is believed to be acquired by endogenous implantation into deep tissues where anaerobic conditions prevail. Actinomyces israelii is an anaerobic normal
inhabitant of the mouth, especially in the teeth and tonsils.
In the cervicofacial region, puncture wounds, dental
extractions, or compound fractures are some of the routes
of infection. The cervicofacial variant is characterized by
the appearance of solid sub- or supramandibular nodules
or swellings and the overlying skin becoming purple to
violet.
Clinical presentation of cervicofacial actinomycosis is
characterized by the presence of suppurative or wooden
indurated mass with discharging sinuses. Pus from the discharging sinuses contains tiny yellow sulfur granules.
Common initial symptoms of infection including pain,
fever, erythema, edema, and suppuration may be absent.
Radiographic features
Actinomycotic osteomyelitis affecting the maxilla and
mandible have been reported. Radiographs reveal ill-defined
radiolucencies with a radiopaque periphery.
Periapical actinomycosis is believed to be a non-resolving periapical lesion associated with actinomycotic infection and has been suggested as a contributing factor in the
perpetuation of periapical radiolucencies after root canal
treatment. A diagnosis is usually made by identifying the
typical actinomycotic colonies in a surgical specimen.
Occasionally, the periapical actinomycotic lesion may
appear radiopaque mimicking condensing osteitis.
Investigations
Sinus tracts may reveal the presence of yellowish granules
(16 mm in diameter) referred to as sulfur granules. On
histological examination, the sulfur granules consist of a
central tangled mass of gram-positive mycelia surrounded
at the periphery by gram-negative, club-shaped rods.
The hematoxylin and eosin stained specimen shows the
ray phenomenon. The periphery of the granule shows laments that are radially oriented and embedded in eosinophilic material.
Clinical features
Noma is usually seen in children between the age of 3 and
12 years mainly in the developing countries especially
sub-Saharan Africa. Children at risk for noma have been
seen to have low plasma concentrations of zinc, retinol,
ascorbate, and essential amino acids with increased plasma
and saliva levels of free cortisol.
Many authors believe that noma, occurs secondary to
the extension of necrotizing ulcerative gingivitis.
In the initial stages ulcerative areas from the gingiva
extend to involve the adjacent soft tissues. Subsequently
the necrotic areas spread both into deeper tissue planes
and supercially. The overlying skin turns deep blue to
black and eventually sloughs away. Extensive necrosis can
lead to exposure of bone and osteomyelitis. Patient may
present with pain, fever, malaise, foul odor and regional
lymphadenopathy. The differential diagnosis for noma
must include mucocutaneous leishmaniasis, lupus erythematosus, leprosy, agranulocytic ulcerations, injuries associated with physical trauma (including burns), syphilis,
oral cancer and yaws.
Other variants of Noma
Management
87
BOTRYOMYCOSIS (Bacterial
Pseudomycosis)
Botryomycosis arises from chronic infections produced by
low-virulence organisms in an altered host environment.
Staphylococci have been the most common organisms
implicated, but various other bacteria have also been identified in lesions of human botryomycosis.
The disease, later referred to as botryomycosis, was rst
described involving the lung of a horse by Bollinger in
1870. Sebastiano Rivolta in 1884 coined the term botryomycosis (botryos from Greek for bunch of grapes) after
he found globular granules in a tumor from the cut spermatic cord of a horse.
Clinical types and features
Winslow (1959) categorized botryomycosis into two types
based on their site of involvement: integumental and
visceral.
The integumental botryomycosis affects the exposed
body surfaces such as the hands, feet or the head. It occurs
in the site of a contaminated wound, foreign body or
trauma and manifests as a localized granulomatous infection. Occasionally it causes osteomyelitis.
The visceral form is relatively rare. It is usually seen in
immunocompromised individuals. It affects the lung, liver,
kidney, spleen, brain, prostate, bowel and the lymphatic
tissues (tonsil, lymph node).
The typical botryomycotic lesions are indurated brotic
masses that may form draining sinuses and stulas.
Literature review reveals a few reports of botryomycosis
affecting the orofacial region. Small and Kobernick (1967)
reported a patient with botryomycosis of the tongue. Rawal
and Rawal reported a patient with gingival botryomycosis
and Alavandar (1979) reported botryomycosis affecting
the mandible.
Histopathologic features
Hematoxylin and eosin stained specimens show chronic
suppurative and granulomatous inflammation with giant
cells, epithelioid macrophages and scattered microabscesses.
Within the areas of the purulent inammation, Bollingers
granules are seen. These are relatively small, but frequently microscopically visible, pale yellow or yellow-white
granules consisting of irregular aggregates or colonizations
of gram-positive cocci, usually staphylococci.
Bollingers granules are surrounded by an amorphous,
eosinophilic, refringent matrix called the Splendore-Hoeppli
phenomenon (this phenomenon is also seen around colonies of certain bacteria, fungi, helminthes, actinomyces,
mycetoma, nasofacial and subcutaneous phycomycosis and
around silk sutures).
88
Management
Surgical resection along with antibiotic therapy is the treatment of choice.
RHINOSCLEROMA (Respiratory
Scleroma)
Rhinoscleroma is an endemic, chronic, slowly progressive
granulomatous disease caused by Klebsiella rhinoscleromatis (a gram-negative rod-shaped bacteria, 2.5 m in
length).
In 1882, von Frisch identied K. rhinoscleromatis as
the etiologic agent. In 1870 Ferdinando Von Hebra, a dermatologist described the disease for the rst time. It was
later named respiratory scleroma. The word skleroma in
Greek meaning hard tumefaction, was adopted in 1932 at
the International Clinical Otorhinolaryngology Conference
(in Madrid), emphasizing involvement of upper and lower
airways.
Rhinoscleroma is contracted by direct inhalation of
droplets or contaminated material.
Clinical stages
Humans are the only identified host of K. rhinoscleromatis.
It usually affects individuals in the 2nd to 4th decades of
life. It affects people living in crowded conditions with poor
hygienic and nutritional conditions (including iron deficiency
anemia). Rhinoscleroma affects women more commonly
than men (13:1).
The nose is the most common site of infection, although
the nasopharynx, paranasal sinuses, and pharynx may also
be involved. Other affected organs include the paranasal
sinuses, eustachian tubes, middle ear, orbital tissues and
the brain.
Rhinoscleroma occurs in three overlapping stages:
catarrhal-atrophic (sometimes called ozaena), granulomatous (proliferative or nodular) and sclerotic (cicatricial or
brotic).
In the catarrhal stage, patient may complain of foul
smelling purulent nasal discharge and nasal obstruction. On
clinical examination atrophy and crusting of the nasal
mucosa or hyperemia and exudates in the respiratory tract
mucosa are evident.
In the granulomatous stage, epistaxis, nasal deformity
and destruction of the nasal cartilage (Hebra nose), hoarseness of voice, anosmia and anesthesia of the soft palate
are common signs and symptoms. Clinical examination
may reveal a bluish red and rubbery granulomatous lesion.
These lesions over a period of time turn into a pale hard
granulomatous mass.
In the sclerotic stage, clinical examination shows granulomatous lesions surrounded by dense brotic tissue.
Diagnosis
The diagnosis of rhinoscleroma is made by the bacterial
isolation by culture on blood or MacConkey agar. Histopathological specimens can be stained with periodic acidSchiff, Giemsa and WarthinStarry stain. The presence of
Mikulicz cells (clear cytoplasm vacuolated histiocytes
containing the bacillus) and degenerated plasmocytes in
Russel bodies are diagnostic of rhinoscleroma.
The hypertrophic stage of rhinoscleroma has characteristic mild to marked high signal intensity on both T1- and
T2-weighted MR images.
Management
Nasal or pharyngeal obstruction is best managed surgically along with antibiotic therapy. Many authors recommend the use of cephalosporins and clindamycin. Shaer
et al (1981) have shown that the topical application of
2% acriflavine solution is an effective and safe treatment
option for rhinoscleroma. Tracheostomy may be required
if laryngeal scarring causes airway obstruction.
CAT-SCRATCH DISEASE
VIRAL INFECTIONS
INFECTIOUS MONONUCLEOSIS
(Monoglandular Fever, Kissing Disease)
Infectious mononucleosis (IM) is a clinical syndrome caused
by EpsteinBarr virus-4 (EBV, human herpes virus-4).
EBV replicates primarily in beta-lymphocytes but also
may replicate in the epithelial cells of the pharynx and
parotid duct.
Children and young adults are usually affected. The virus
is transmitted via intimate contact. Children may acquire
the virus through sharing of saliva contaminated ngers,
toys and serving spoons. Direct transfer of contaminated
saliva may occur in adults following kissing (hence the
name kissing disease) or sharing of straws. The incubation
period is 48 weeks.
Clinical features
The characteristic clinical features of IM include, malaise,
fatigue and anorexia. These symptoms are immediately
followed by high fever (about 104F) which lasts for almost
2 weeks.
The most striking feature of IM is the presence of
lymphadenopathy. Any or all lymphatic chains may be
enlarged. Lymphadenopathy is always bilateral and symmetrical in all patients. Bilateral posterior and anterior
cervical lymphadenopathy is highly suggestive of EBV
infectious mononucleosis.
Other clinical features include the presence of tonsillar
enlargement, hepatosplenomegaly, jaundice, rhinitis and
pharyngitis.
Hoaglands criteria (1975) for the diagnosis of IM
include: at least 50% lymphocytes and at least 10% atypical lymphocytes in the presence of fever, pharyngitis, and
adenopathy, and conrmed by a positive serologic test.
Oral manifestations include hard and soft palate
petechiae, necrotizing ulcerative mucositis, necrotizing
ulcerative gingivitis and pericoronitis. Occasionally, the
parotid gland may be affected along with facial nerve
palsy.
90
Warwick et al (2003) reported a patient with IM, ruptured spleen and Cullens sign (periumblical ecchymosis).
They also suggest that the presence of abdominal pain is an
uncommon symptom in infectious mononucleosis and its
occurrence is therefore a danger sign that may forewarn a
potentially life threatening complication of ruptured spleen.
The complications of IM include myocarditis and cardiac conduction abnormalities, neurologic abnormalities,
meningitis, encephalitis, cranial nerve palsies, retrobulbar
neuritis, acute interstitial nephritis, hemolytic anemia,
thrombocytopenia and upper airway obstruction.
Syndrome association
Chronic fatigue syndrome and Lemierres syndrome have
been reportedly associated with IM. The association of IM
and chronic fatigue syndrome is still very questionable
and debatable.
Diagnosis
The Paul-Bunnell test is a serological test that detects heterophile antibodies by agglutination of sheep or horse red
blood cells. However, in the 1st week of infection, the falsenegative rate is as high as 25%.
VCA-IgG and VCA-IgM tests are useful in diagnosing
patients who have highly suggestive clinical features but
negative heterophile antibody test results.
Antibody to EpsteinBarr nuclear antigen (EBNA),
while typically not detectable until 68 weeks after the
onset of symptoms, can help distinguish between acute
and previous infections.
Elevated hepatic transaminase levels may be seen in
about 50% of the IM patients.
Management
Infectious mononucleosis usually resolves in about 6 weeks.
IM is best managed with extensive palliative and supportive
healthcare. Patient should be adequately hydrated. Fever
and malaise may be managed with acetaminophen and
NSAIDs. Though steroids have been used frequently, they
are best used only in an emergency to relieve the patient
of respiratory compromise secondary to pharyngeal edema.
Antibiotics like penicillins are best avoided as these
patients have a higher risk of developing a morbilliform
skin rash.
Clinical features
Acute lymphonodular pharyngitis is usually seen in children and young adults. Clinically, the hyperplastic lymphoid aggregates are evident as discrete yellow to dark
pink colored nodules or white to yellow papules surrounded
by an erythematous ring, generally 15 in number. These
lesions are typically found on the tonsillar pillars, uvula,
soft palate and oropharynx.
Histopathological evaluation of these nodules may reveal
epithelial necrosis and inclusion bodies (intranuclear or
cytoplasmic) and multinucleated cells.
Management
The condition is self-limiting and the management is
aimed at supportive care. Non-aspirin containing analgesics are beneficial.
MEASLES (Rubeola)
Measles is caused by an RNA virus, paramyxovirus.
Measles is an acute and highly communicable disease and
infection confers lifelong immunity. The disease process
is marked by the presence of prodromal fever, cough, conjunctivitis and coryza (profuse discharge from the nasal
mucous membrane).
Measles may often occur as an epidemic. The virus is
transmitted via direct contact or by droplet infection and
the respiratory tract forms the portal of entry.
Measles is usually considered as a serious disease in
malnourished, immunocompromised or vitamin decient
individuals.
Clinical features
Following a 14-day incubation period skin rashes begin to
appear. About 23 days before the typical skin rash
appears the patient may exhibit prodromal upper respiratory symptoms and conjunctivitis along with lymphadenopathy. The skin rash fades away in about 7 days. Pale
skinned individuals may exhibit areas of altered pigmentation following resolution of the skin rash.
During this stage of the infection the pathognomonic
Kopliks spots begin to appear on the buccal mucosa. Kopliks
spots are small, irregular, red spots with a minute bluish
white speck in the center of each seen on the buccal
mucosa and lingual mucosa. They are named after Henry
Koplik (18581927), an American pediatrician who rst
described them in 1896.
Patients are usually irritable and photophobic at this
stage. These spots represent sites of virus replication and
sites of inammation of the mucous glands.
As the disease progresses, bacterial superinfection
may lead to diarrhea, bacterial pneumonia, cancrum oris,
Mothers suffering from rubella during the rst trimester are likely to have children who exhibit pitting and
enamel hypoplasia and rarely complete aplasia. Eruption
of teeth may be retarded. Some authors have reported cleft
lip and palate in some of these children.
Prevention and management
Rubella is best prevented by mumps-measles and rubella
vaccination. The disease is self-limiting. As individuals are
highly contagious in the first 1 week, following appearance of the skin rash, they should ideally abstain from
group activities during this period. Maintenance of hydration and bed rest is generally sufficient. Non-aspirin containing NSAIDs may be given for managing the prodromal
symptoms.
Discovery of HIV
Disease syndromes similar to the clinical manifestation of
AIDS have been described in the ancient Ayurvedic literature. Sushrutha in 800 BC and later Charaka and Vagbhatta
describes a conditions with loss of muscle mass, fever, skin
eruptions and ulcers, complexion changes, neurological
disorders, exhaustion, coma and death, and stated that in
irremediable stages treatment should be given up.
In 1981, homosexual men with symptoms of a disease
that now are considered typical of the acquired immunodeciency syndrome (AIDS) were rst described in Los
Angeles and New York. The men had an unusual type of
lung infection (pneumonia) called Pneumocystis carinii
(now known as Pneumocystis jiroveci) pneumonia (PCP)
and rare skin tumors called Kaposis sarcoma. The patients
were noted to have a severe reduction in CD4 cells. These cells,
often referred to as T cells, help the body ght infections.
Shortly thereafter, this disease was recognized throughout
the United States, western Europe, and Africa. In 1983,
researchers in the United States and France described the
virus that causes AIDS, now known as the human immunodeciency virus (HIV) and belonging to the group of viruses
called retroviruses. In 1985, a blood test became available
92
HIV-2
Group M
Group N
Table 2
Region
Predominant type
Other subtypes
South America
Australia
C, F
Western Europe
A, C, D
Eastern Europe
B, C, G, D
Africa
A, B, D, C, E, F, G, H, O
Uganda
A, B, C, D, G
Zaire
A, D, H
Kenya
Nigeria, Gabon
G, H
Cameroon
A, B, E, G, H, F
Central Africa
A, C, D, E
Zambia, Malawi
(Southeast Africa)
China
Bb
Southeast Thailand,
Myanmar
Bb, E
Indonesia
India
A, B, F
Group O
Step 1: Entry of the HIV virion into the host T-cell, is triggered by the binding of membrane proteins to receptors
on the T-cell surface.
Step 2: The membrane proteins on the virus are units
called gp120. These gp120 unit binds to the receptors
called CD4 on the T-cell. The union of gp120 and CD4
initiates another event.
93
Figure 1
Figure 3
gag p17
gag p24
HIV
RNA
4
CD
gp 40
gp 120
Envelopment of
virus into host cell
T-cell
Figure 4
Binding of HIV virion into host T cell
Figure 2
HIV
Endocytosis
T-cell
CD
T-cell
5
CR
Figure 5
Capsid
T-cell
Figure 6
Figure 8
Enzymes
Double stranded
viral DNA
RNA
Capsid (shedding the capsid)
Shedding of capsid
Figure 7
Figure 9
Viral RNA
Viral DNA
Reverse
transcriptase
Viral DNA
Host cell nuclear
envelope
Reverse transcription
Host DNA
Figure 10
Viral DNA
Host DNA
Retroviral integrase
[PROVIRUS]
95
Figure 11
Figure 13
Ribosome
Viral RNA
Figure 12
Figure 14
Viral RNA
Host cell
T-cell nucleus
Figure 15
Membrane proteins
Host cell
Figure 16
Plasma membrane
Budding
Host cell
Figure 17
Transmission
Transmission of HIV-1 occurs mainly through one or more
of three routes:
1.
2.
3.
Sexual contact
Passage of the virus from infected mothers to the
newborn
Through blood and parenteral inoculation.
Sexual contact
It is the predominant mode of transmission. The rate of
infection is increased with the number of sexual partners
2.
98
500
A1
B1
C1
200 to 400
A2
B2
C2
200
A3
B3
C3
Category A
Category B
Bacillary angiomatosis
Candidiasis
Cervical dysplasia
Constitutional symptoms (fever, diarrhea 1 month)
Oral hairy leukoplakia
Herpes zoster
Idiopathic thrombocytopenic purpura
Listeriosis
Pelvic inflammatory disease
Peripheral neuropathy
Cervical cancer
Coccidiodomycosis
Cryptosporidiosis
Cytomegalovrius
Encephalopathy
Herpes simplex (chronic, esophageal)
Histoplasmosis
Isosporiasis
Kaposis sarcoma
Lymphoma
Mycobacterium avium/Mycobacterium kansasii
Pneumocystis carinii
Recurrent pneumonia
Progressive multifocal leukemia.
Children
Kaposis sarcoma
Pneumocystis carinii
pneumonia
Toxoplasma encephalitis
Esophageal candidiasis
Cytomegalovirus retinitis
Diarrhea (continuous or
intermittent more than
1 month)
Generalized extrainguinal
lymphadenopathy
Skin infection (severe or
recurrent)
Cough for more than
1 month dermatitis
Stages
Oral thrush
Acute infection
1,000750
750200
215 weeks
Early symptomatic
500100
15 years
Late symptomatic
50200
14 years
Advanced disease
500
02 years
CD4 Strata
Complication
500/mm
200/m3
(usually
100/m3)
Fever (continuous or
intermittent more
than 1 month)
100
Children
Neurologic findings
(dementia)
Focal motor deficits
14 weeks
Associated findings
Adults
Duration
Asymptomatic
Characteristic findings
Children
200500/mm3
Adults
Progressive headache
50/mm3
5.
6.
Oral manifestations
In 1986 the European community took the initiative to
establish a classification system for oral manifestations
in HIV infection. The classification was presented by
Pindborg. He classified the oral manifestations into six
categories based on associated agents like fungal, bacteria,
viral, neoplasm, neurological and unknown cause.
1.
2.
3.
4.
Fungal infection
a. Candidiasis
i. Pseudomembranous
ii. Erythematous
iii. Hyperplastic
iv. Angular cheilitis
b. Histoplasmosis
c. Cryptococcosis
d. Geotrichosis
Bacterial infection
a. HIV Necrotizing gingivitis
b. HIV Gingivitis
c. HIVPeriodontitis
Caused by: Mycobacterium avium intercellulare,
Klebsiella pneumoniae, Enterobacterium cloacae,
Escherichia coli
d. Actinomycosis
e. Cat-scratch disease
f. Sinusitis
g. Exacerbation of apical periodontitis
h. Submandibular cellulitis
Viral infection
a. Herpes simplex
b. Cytomegalovirus
c. EpsteinBarr
Hairy leukoplakia
d. Varicella zoster
i. Herpes zoster
ii. Varicella
e. Human papilloma virus
i. Verruca vulgaris
ii. Condyloma acuminatum
iii. Focal epithelial hyperplasia
Neoplasms
a. Kaposis sarcoma
Diagnostic criteria
In 1989, WHO Collaboration Center for Oral Manifestation
of the HIV under WHO Global Program on AIDS and
European Clearing House of Oral Problems related to HIV
defined the diagnostic criteria for oral manifestations.
These diagnostic criteria were proposed for epidemiological surveys. The diagnostic criteria were given for the most
common oral manifestations. Following are the diagnostic
criteria suggested.
1. Candidiasis
i.
b.
c.
Gingivitis: They defined gingivitis as the disease characterized by fiery red edematous attached gingiva and
may affect the alveolar mucosa. No ulceration must be
present.
Necrotizing gingivitis: This is characterized by gingival pain, swelling, ulcerations, necrosis or as distribution of interdental papillae covered with a fibrous
slough. The patient suffers from fever and halitosis
may be present.
Periodontitis: This is characterized by aggressive
irregular bone destruction. Any infection that gives
the impression of affecting periodontal structure other
than gingiva.
101
3. Hairy leukoplakia
A characteristic macroscopic appearance of either erythematous or violaceous plaque-like lesions, or a bulky tumor
predominantly seen in palate or on the gingiva.
Candidiasis: Erythematous
Pseudomembranous
Hairy leukoplakia
Kaposis sarcoma
Non-Hodgkins lymphoma
Periodontal disease
Linear gingival erythema
Necrotizing (ulcerative) gingivitis
Necrotizing (ulcerative) periodontitis
102
Bacterial infections
Mycobacterium avium intercellulare
Mycobacterium tuberculosis
Melanotic hyperpigmentation
Necrotizing (ulcerative) stomatitis
Salivary gland disease
Dry mouth due to decreased salivary flow rate, unilateral or bilateral swelling of the major salivary
glands
Thrombocytopenic purpura
Ulceration NOS (not otherwise specified)
Viral infections
Herpes simplex virus
Human papilloma virus (warty-like lesions)
Condyloma acuminatum
Bacterial infections
Actinomyces israelii
Escherichia coli
Klebsiella pneumoniae
Cat-scratch disease
Drug reactions (ulcerative, erythema multiforme, lichenoid, toxic epidermolysis)
Epithelioid (bacillary) angiomatosis
Fungal infections other than candidiasis
Cryptococcus neoformans
Geotrichum candidum
Histoplasma capsulatum
Mucoraceae (mucormycosis/zygomycosis)
Aspergillus flavus
Neurologic disturbances
Facial palsy
Trigeminal neuralgia
Recurrent aphthous stomatitis
Viral infections
Cytomegalovirus
Molluscum contagiosum
Laboratory diagnosis
There are two main approaches for the diagnosis of HIV
infection, the direct and the indirect.
The direct method seeks information on the presence of
virus itself by classical isolation method and identifying the
presence of virus specic genes by molecular biology techniques. George Babu in his review states that the classical
method of isolation for HIV is time consuming, expensive,
requires special containment laboratory and highly trained
personnel.
He also states that molecular biology technique such as
polymerase chain reaction (PCR) is expensive and should
be done by highly trained personnel but it is less expensive than classical virus isolation and less time consuming. He further states that PCR nds application under
special circumstances such as the presence of early stages
of infection.
The indirect methods are based upon the observation
that infected persons make antibody eventually. In most
cases, the IgG class of antibody can be detected on the
serum 68 weeks after infection.
ELISA Test
1. The first system to have been introduced is ELISA
(enzyme linked immunosorbent assay) for screening
and western blot test for confirmation of HIV antibody.
2.
3.
Management
The management of HIV disease is two pronged: one aimed
at managing the conditions arising out of the immunosuppression and opportunistic infections and the other targeted at the virus itself.
I. Management of the viral infection
Antiviral therapy is instituted in patients with AIDS
regardless of the CD4 count and in patients who are
asymptomatic but with CD4 count less than 200 cells/
mm3 (Table 3).
Four groups of drugs can be used to combat the virus.
A combination of these drugs may be used in the management of HIV infection. These regimens are referred to
as highly active antiretroviral therapy (HAART).
The commonly employed regimens used include:
2.
a. 1 NNRTI 2 NRTI
b. 1 or 2 PI 2 NRTI
c. Triple NRTI.
3.
Table 3 Antiviral therapy for management of HIV infection
Drug
Examples
Mechanism of action
Fusion inhibitors
Enfuvirtide
It is an anti-HIV peptide
that inhibits entry of the
virus into host cells
Nucleoside reverse
transcriptase
inhibitors (NRTIs)
Abacavir
Didanosine
Lamivudine
Stavudine
Zalcitabine
Zidovudine
Non-nucleoside
reverse transcriptase
inhibitors (NNRTIs)
Nevirapine
Efavirenz
Delavirdine
Protease inhibitors
(PIs)
Indinavir
Ritonavir
Atazanavir
Sterilization
Steam: Autoclave instruments at a temperature of 121C,
at 15 lb/sq in pressure for 1520 minutes on specially
modified cooker.
Flame: Heating with ame until red-hot to sterilize instruments such as knives and other skin piercing instruments.
Disinfection
a.
III. Neoplasia
Kaposis sarcoma
1. Chemotherapy: Wet Velban (Vinblastine sulfate) 0.1 ml
of a 0.2 mg/ml solution for each 0.5 cm lesion. Drug is
injected 0.2 intralesionally after local anesthesia.
2. Localized radiation therapy: Fractionated radiotherapy of approximately 800 to 1,500 cGy
3. Intro A (Interferon): 35 million IU injected into lesion
3 times per week
4. Sotradecol (sodium tetradecyl sulfate): Intralesional
injection of a 3% solution at 0.2 cc/cm
5. CO2 laser.
VI. Aphthous ulcers
1.
2.
3.
4.
5.
6.
b.
Universal precautions
WHO (1997) recommended Universal Safety Precautions
for preventing the spread of HIV infection. They stated
that Universal Safety Precaution means that all body fluids and blood of patients should be considered as infections and all precautions should be taken since it is not
known who is infected with HIV.
The universal precaution starts with:
104
Hand washing.
Creating appropriate barrier by use of gloves, masks,
gowns, eye protectors.
Careful handling of sharp objects.
Proper sterilization and disinfection.
Disposal of instruments after use/decontamination of
instruments including syringes, needles and equipment.
Proper disposal of infected waste.
2.
3.
SINUSITIS
Inflammation of the paranasal sinus mucosal lining is
referred to as sinusitis. When all the paranasal sinuses
Acute Sinusitis
Sinusitis occurs when the mucous membranes of the upper
respiratory tract namely the nares, pharynx, sinuses and
larynx become inflamed. The swelling obstructs the sinus
openings and prevents mucus from draining normally.
This creates a moist environment which aids in harboring
infection.
The most common cause for sinusitis is a viral infection. However sinusitis can also occur as a result of a bacterial or fungal (secondary to aspergillosis, mucormycosis,
candidiasis, histoplasmosis and coccidiomycosis) infection. When the upper respiratory tract infection persists
for longer than 2 weeks, a bacterial etiology is more likely.
Other causes for sinusitis include allergies, deviated nasal
septum, nasal polyps, antroliths (Figure 18) and as complications of systemic conditions such as cystic brosis, gastroesophageal reux or immunodeciency diseases. The
severity of sinusitis may exacerbate when exposed to polluted air or when an individual smokes.
Clinical features
Patients suffering from sinusitis include facial pain (especially in the periorbital region, maxillary sinus region and
forehead), fever, fatigue, nausea, nasal congestion, erythema over the sinus region (owing to increased blood
Figure 18
Chronic Sinusitis
Chronic maxillary sinusitis is defined as sinusitis lasting
longer than 12 weeks. Patients may report of chronic
facial pressure in the maxillary region, headache, rhinorrhea, postnasal drip, light headedness, decreased sense of
smell, or toothache.
In children, the symptoms of sinusitis are less specic
than in adults. Symptoms include persistent nasal congestion and cough lasting for more than 10 days, high fever
and purulent nasal discharge. Children are less likely to
present with facial pain or headache.
Clinical and radiographic diagnosis
Apart from history, clinical examination of the sinuses
will help in diagnosing sinusitis. In many individuals the
sinuses are tender in palpation. Another simple chair side
test that can be employed is transillumination to assess the
maxillary and frontal sinuses. The paranasal sinuses can
be assessed using plain radiographs such as Waters view
(facilitates viewing of the maxillary, frontal sinuses and
sphenoid sinuses), Caldwell view (visualization of the
frontal and ethmoid sinuses), lateral view (to visualize the
sphenoid sinus and the posterior frontal sinus wall) and
the submentovertex view (sphenoid sinuses are visualized).
The typical radiographic ndings in a paranasal
sinus view include: localized thickening of the mucosal
lining at the base of the sinus, generalized thickening of
the mucosal lining (Figure 19), partial lling of the sinus
(uid level appearance) and a complete cloudy or hazy
sinus (Figure 20).
CT of the osteomeatal complex has been used to assess
pansinusitis. It has been frequently reported that sinus CT
scanning has a high sensitivity but a low specicity for
demonstrating acute sinusitis. CT scanning may be used to
assess signicant mucosal thickening, air-uid levels
(Figure 21), osteomeatal complex obstruction, polyposis
(Figure 22), or calcication suggestive of fungal sinusitis.
Management
Figure 19
Figure 21
Figure 20
Figure 22
106
Disseminated Histoplasmosis
The disseminated form of histoplasmosis is a rare disease
and occurs primarily in immunocompromised persons
(patients with HIV infection, lymphoreticular neoplasms,
corticosteroid therapy, cytotoxic therapy and immunosuppressive agents).
The spectrum of illness in disseminated disease ranges
from a chronic, intermittent course in immunocompetent persons to an acute and rapidly fatal infection that usually occurs
in infants and severely immunosuppressed persons. Fever
is the most common symptom; however, headache, anorexia,
cough, weight loss, and malaise are frequent complaints.
Hepatosplenomegaly, lymphadenopathy and oropharyngeal ulcerations are typically encountered.
The buccal mucosa, tongue and palate are the most
common sites affected. Solitary painful ulcers are seen.
These erythematous ulcers exhibit rm rolled-out margins
that resemble malignant ulcers. The disseminated form of
the disease also affects the nervous system, gastrointestinal system and the renal system.
Diagnosis
Histoplasmosis can be diagnosed by culture, fungal stains,
serologic tests for antibodies, and antigen detection.
In order to demonstrate the yeast of H. capsulatum special stains such as PAS and Grocott-Gomori methenamine
silver have to be used.
Prognosis and management
Histoplasmosis is a self-limiting disease. However the
untreated disseminated form may result in death in almost
90% of the individuals. Analgesics and antipyretics may be
used to manage fever and myalgias. Intravenous amphotericin B is used as the drug of choice. Other drugs that have been
used with good results are ketoconazole and itraconazole.
It is caused by Blastomyces dermatitidis, a dimorphic fungus. It is believed to thrive in organic matter and soil
which have high moisture content.
Following inhalation of the spores, three forms of the
disease are manifested: acute, chronic and a disseminated
form.
Clinical manifestations
Blastomycosis is typically seen in men engaged in outdoor
activities usually in the 3rd and 4th decades of life. The
acute form of blastomycosis is characterized by fever,
malaise, myalgias, weight loss, cough, and pleuritic chest
pain.
The chronic form is much more commonly seen than
the acute form. It is characterized by symptoms mimicking
those of tuberculosis such as night sweats, low-grade fever,
productive cough and weight loss.
Rarely, dissemination to other body sites such as the
skin, bone, CNS, genitourinary system and oral cavity may
be seen. Cutaneous lesions may appear as erythematous
nodules that subsequently ulcerate.
Oral features
Intraoral lesions of blastomycosis appear as irregular pink
or white areas. The ulcers may be painful and appear
irregular with rolled borders.
Histologic findings
On histological section blastomyces can be easily discernible with PAS stan, H and E stain, PAP and methenamine
silver stains. It can be cultured using Sabourauds agar.
Management
For mild cases itraconazole and ketoconazole can be used
effectively. Patients who are refractory to ketoconazole
can be treated with amphotericin B.
MUCORMYCOSIS (Zygomycosis,
Phycomycosis)
Mucormycosis is an opportunistic deep fungal infection
caused by bread mold fungi of the genera Mucor, Absidia,
Rhizopus and Cunninghamella, also collectively known as
Phycomycetes.
Zygomycosis was rst described by Platauf in 1885 as
Mycosis mucorina. The class Zygomycetes is subdivided
into two orders, which contain the agents of human
Zygomycosis, the Mucorales and the Entomophthorales.
Among the Mucorales, Rhizopus (most common), Mucor,
Absidia, Rhizomucor, Cunninghamella, Saksenaea, Cokeromyces and Apophysomyces have been implicated in
causing human disease.
108
ASPERGILLOSIS
Aspergillosis was first described in 1729 by a priest botanist, Micheli. In 1893, Morrel Mackenzie published the
first case of aspergillosis affecting the maxillary sinus. It is
a saprophyte that belongs to the class of myocetes.
Aspergillus avus, A. niger and A. fumigatus are
pathogenic to humans. It is believed that aspergillosis is
the second most common opportunistic infection to affect
immunocompromised individuals.
Aspergillus is usually present in decaying matter, soil
and water. It is contracted via inhalation. Aspergillus may
cause hypersensitivity reactions and in some individuals it
exhibits direct angioinvasion. Aspergillus primarily affects
the lungs resulting in allergic bronchopulmonary aspergillosis, chronic necrotizing Aspergillus pneumoniae, aspergilloma (fungus ball/mycetoma) and invasive aspergillosis.
However, in immunocompromised host the fungi is no
longer restricted to the lung. This disseminated form causes
endophthalmitis, endocarditis, and abscesses in the myocardium, kidney, liver, spleen, soft tissue, and bone and
oral involvement.
Oral findings
Involvement of the oral cavity may be seen in the disseminated form of the disease. The tongue, soft and hard palate
and an occasional report of pulp and periodontal tissue
involvement have been described in literature. Involvement
of the maxillary sinus may result in an extension to the
adjacent structures and the palate to form a very painful
ulcer surrounded by a zone of necrotic black tissue.
Diagnosis
Aspergillus may be identified in branched septate hyphae
about 4 m in diameter in potassium hydroxide preparations. Aspergillus may be cultured in Sabourauds agar
media. The hyphae demonstrate a tendency to invade
adjacent blood vessels.
Management
The ulcerated lesion should be debrided, systemic amphotericin B therapy is the drug of choice.
Types
There are two varieties of C. neoformans: C. neoformans
var neoformans consisting of serotypes A and D (causes
disease in immunocompromised hosts) and C. neoformans
var gatti consisting of serotypes B and C (causes disease in
normal hosts).
The yeast is typically found in pigeon feces. Some of
these organisms are restricted to tropical and subtropical
areas, and are isolated from certain species of eucalyptus
trees and the air beneath them. Infections occur through
inhalation of yeast like organisms which enter smaller respiratory passages and then remain dormant depending on the
host reaction. Organisms are then reactivated from such
previous dormant infections in the lung or lymph node.
Clinical features
Cryptococcosis is seen more commonly in men due to
their occupational exposure or a lack of estrogens. The
infection usually affects the respiratory system and the
nervous system. However a wide dissemination into multiple body sites may also occur.
It is estimated that 4085% of the patients present
with involvement of the brain parenchyma. Patients may
initially complain of headache, fever and nuchal pain suggesting meningeal irritation. As the disease progresses,
stupor, coma and dementia may be seen. Papilloedema may
be seen. Complications of CNS involvement include internal hydrocephalus, focal motor decits, and symptoms of
raised intracranial pressure.
When the lung is involved, a primary pulmonary
complex, similar to TB may be seen. Symptoms of acute
pneumonia with cough, fever, and lobar pulmonary inltrates affecting alveoli may be seen. Pleural effusion without parenchymal lesions are rare.
Cutaneous involvement results in the formation of papules, abscesses, cellulitis, acneiform lesions, draining sinuses,
109
110
CHAPTER
Orofacial Pain
Joanna Baptist, Ajay Nayak, Ravikiran Ongole
Pain Physiology
Myofascial Pain
Neuralgias
Trigeminal Neuralgia
Glossopharyngeal Neuralgia
Postherpetic Neuralgia
Geniculate Neuralgia
Occipital Neuralgia
Atypical Odontalgia
Barodontalgia
PAIN PHYSIOLOGY
2.
3.
4.
5.
6.
7.
8.
Properties of Pain
Weber and Fechners law
Weber and Fechner proposed that gradation of stimulus
strength is discriminated approximately in proportion to
the logarithm of stimulus strength.
This law can be mathematically expressed as
R log S
where R intensity of the reaction (i.e. the pain perceived),
constant and
S the intensity of the stimulus.
111
Nociceptors
Pain receptors are called nociceptors (from Latin, nocereto
hurt). All nociceptors are free nerve endings (however
other cutaneous receptors when stimulated excessively
can result in pain). Either present in skin or any other tissues. They are more concentrated in superficial layers of
skin, periosteum, arterial walls, joint surfaces, the falx and
the tentorium of the cranial vault. Deep tissues are sparsely
supplied with nociceptors.
Two general types of nociceptors are characterized by the
neurons associated with them.
112
1.
2.
A fiber
C fiber
16 m diameter
1.5 m diameter
Myelinated
Non-myelinated
Stimulation of nociceptors
Mechanical, chemical and thermal stimuli excite pain
receptors. Fast pain is conducted by A fibers which is
elicited by the mechanical and thermal stimuli. The slow
dull pain is conducted by the C fibers which are elicited by
all three types of stimuli. It is almost always caused by
release of chemicals liberated by the injured tissue. These
are endogenous chemicals called algogenic (pain producing) substances. Algogenic substances stimulate nociceptors to produce pain.
These chemicals are pain producing peptides, bradykinins, serotonin (5 HT), potassium ions, prostaglandins,
acetylcholine and proteolytic enzymes.
It is interesting to note that commonly used NSAIDs suppresses pain by inhibiting prostaglandin synthesis. These
prostaglandins by themselves cannot excite the nociceptors,
i.e. prostaglandins are not algogenic but they enhance the
sensitivity of the nociceptors toward the algogenic power of
bradykinins and other chemical mediators of pain.
Sequelae of Pain
Apart from nociception there are various other sequelae of
pain that alter other systems of the body. Pain affects an
individual psychologically as well as physically.
Psychological sequelae
An uninhibited individual would react to acute pain by
mourning and crying as seen in young children and animals. However, most individuals develop frustration, mental irritation or depression in response to long standing
pain. A patient with long standing atypical facial pain will
most often come to the physician with a frowning face
and would often get irritated with prolonged history taking.
Thus such patients should be treated both by medicines
and psychological counseling.
Muscular sequelae
Injury or disease causing pain results in spasm of skeletal
muscle in the vicinity of the affected region. This is protective as it immobilizes the affected region and thereby puts
it to forcible rest which is most essential for rapid healing.
Figure 1
Thalamus
Neospinothalamic
tract
Hypothalamus
Reticular
formation
Paleospinothalamic
tract
Gasserian ganglion
Motor nucleus (v)
Reflex
Withdrawal from painful stimuli is the reflex action exhibited by the individual. It is an important sequela of pain
that protects the individual from further injury.
For example, biting on a stone during mastication
would immediately initiate a reex action to keep the
mouth open until an individual realizes the presence of the
injurious agent (stone). Such reex action would protect
further injury to the periodontium.
of the neospinothalamic tract. These fibers cross immediately to the opposite side through the anterior commissure
and then travel upward in the anterolateral column of the
spinal cord.
Few of these second order neurons terminate in the
reticular areas of the brain stem (the reticular areas when
stimulated causes excessive alertness and increases an
individuals sense perception), while most of the others
travel up to the thalamus terminating in the ventrobasal
complex along with the dorsal columnmedial lemniscal
tract. The remaining second order neurons terminate in
the posterior nuclear group of the thalamus.
From these areas third order neurons relay signals to other
basal areas of the brain and to the somatic sensory cortex.
Paleospinothalamic tract (for slow pain)
This pathway transmits pain, which is carried via the
peripheral slow conducting C pain fibers (it also transmits
very few A fibers). These peripheral fibers terminate in
the laminas I and II (together called substantia gelatinosa)
of the dorsal horns. Most of the signals then pass through
one or more additional short fiber neurons within the dorsal
horns themselves before entering laminas V through VIII,
also in the dorsal horn.
The next series of neurons gives rise to long axons that
mostly join the bers from the neospinothalamic tract,
passing rst through the anterior commissure to the opposite side of the spinal cord and then upward to the brain in
the same pathway.
113
Figure 2
Dorsal horn
Spinal
cord
A or A fiber
(Non-nociceptive)
C fiber
(nociceptive)
+
+
+
Interneuron
Projection
neuron
To spinothalamic tract
2.
3.
2.
2.
3.
4.
5.
Intracranial structures
Neoplasm
Aneurysm
Hematoma
Hemorrhage
Abscess
Edema
Extracranial structures
Teeth
Ears
Eyes
Nose
Throat
Sinuses
Tongue
Glands
Musculoskeletal disorders
TMJ disorders
Masticatory muscle disorders
Fibromyalgia
Cervical disorders
Generalized polyarthritides
Neurovascular disorders
Migraine headaches
Cluster headaches
Tension type headaches
Cranial arteritis
Neurologic disorders
Paroxysmal neuralgias
Trigeminal neuralgia
Glossopharyngeal neuralgia
Continuous neuralgias
Atypical odontalgia
Traumatic neuroma
Neuritis
Postherpetic neuralgia
Somatic pain
Superficial somatic pain (cutaneous, mucogingival)
Deep somatic pain
Musculoskeletal pain (muscle, TMJ, osseous and
periosteal, soft connective tissue, periodontal)
Visceral pain (pulpal, vascular, neurovascular,
visceral mucosal, glandular, ocular and auricular)
Neuropathic pain
Episodic (trigeminal, glossopharyngeal, geniculate, nervous intermedius neuralgias and neurovascular pains)
Continuous (neuritis, deafferentation pain and
sympathetically maintained pain)
Mood disorders
Anxiety disorders
Somatoform disorders
Other conditions
Types of Pain
Acute pain
It is generally a physiologic response to an injury. It persists as long as the noxious stimulus is present. Acute pain
almost always subsides within the time period required for
the process of normal healing.
Chronic pain
Merskey and Bogduk (1994) described chronic pain as a
persistent pain that is not amenable, as a rule, to treatments
based on specific remedies, or to the routine methods of pain
control such as non-narcotic analgesics.
In common parlance chronic pain is regarded as pain
that persists way beyond the normal time required for the
process of normal healing. Pain is said to be chronic in nature
when it lasts over 3 months. It is generally associated/
inuenced by psychological, emotional, social and cultural
factors.
Figure 3
Figure 4
None
(0)
Mild
(1)
Moderate
(2)
Severe
(3)
Throbbing
Shooting
Stabbing
Sharp
Cramping
Gnawing
Hotburning
Aching
Heavy
Tender
Splitting
Tiringexhausting
Sickening
Punishingcruel
Figure 5
The interview is followed by a complete oral and head
and neck examination, appropriate chair side investigations,
radiographic imaging and lab studies.
Assessment of pain can be achieved by certain subjective
and objective methods. Some widely accepted subjective
methods include the use of McGill Pain Questionnaire (long/
short), visual analog pain scale (VAS), brief pain inventory.
The faces pain scale and the pain diagram.
During the patient interview the clinician should obtain
the following data: mode of onset, duration, location of the
pain, quality or character of the pain, intensity of the pain,
frequency of the painful episodes, aggravating and relieving factors if any, radiation or referral patterns, any other
associated symptoms and history of any medical consultations/use of medications for the same.
The location of pain and referral patterns can be identied
by the patient using a pain diagram (Figure 3).
The pain quality or character can be expressed using
the widely accepted McGill Pain Questionnaire (Figure 4).
This questionnaire will help the patient to describe how
exactly he/she feels about the pain by selecting an appropriate adjective from a list in the questionnaire.
The intensity of pain can be quantied using the visual
analog scale (VAS). The VAS is a 10 cm long line with 0
marked on one end (represents no pain) and 10 at the other
end (represents worst possible pain). The linear scale has
markings from 0 to 10 at 1 cm intervals. The patient is
encouraged to mark a point along this scale that correlates
with the intensity of pain experienced. For convenience pain
116
10
No
pain
Worst
possible
pain
Figure 6
10
Faces pain scale. Score the chosen face 0, 2, 4, 6, 8 or 10, counting left to right, so 0 = no pain and 10 = very much pain.
Do not use words like happy and sad. This scale is intended to measure how children feel inside, not how their face looks.
From PAIN, 2001, 93, 173183 The Faces Pain ScaleRevised: toward a Common Metric in Pediatric Pain Measurement,
by CL Hicks, CL von Baeyer, PA Spafford, I van Korlaar and B Goodenough. Reprinted with permission
of the International Association for the study of Pain.
Periodontal Pain
Pulpal pathology leading to necrosis of the pulp after a while
spills into the periodontium via the apical foramen and/or
the accessory foramina. The discussion here pertains to the
apical portion of the periodontium via the pulp being
affected rather than the progressive effects of the gingival
or periodontal diseases. The periodontal ligament behaves
and responds to noxious stimulation in a manner similar
to other ligaments of the body. Therefore periodontal pain
is felt as a deep somatic pain of the musculoskeletal type.
The receptors of the periodontal ligament can precisely locate
stimulus due to the property of proprioception. The causes
of pain originating in the periodontal ligament are as varied
for pulpal pain. Inflammatory reaction, trauma, endodontic
procedures, improperly contoured restorations, faulty occlusal contacts, orthodontic appliances and surgical procedures are the most common. Parafunctional oral habits
may also be a contributing factor.
Clinical features
The periodontal ligament pain is described by patients as
sharp jabs or pricking type of pain. The pain is usually
intermittent in nature and occurs whenever there is loading of the ligament, such as during chewing. The functional action causes movement of the tooth that compresses
the ligament, leading to displacement of the edema fluid
and irritation of the free nerve endings causing pain. This
feature can be observed clinically by percussing the suspect tooth, which simulates the functional action. Any
tenderness if present during percussion indicates periodontal ligament involvement. The patient will usually
precisely identify the tooth and mention it to be sore or a
feeling of elongation being present. Clinically the tooth
may be seen to be slightly out of occlusion. There may be
associated tenderness in the vestibule and at times a frank
swelling may be present in the gingival region or in the
vestibule.
Diagnosis
The exact location being pointed out by the patient is the
first clue in suspecting periodontal ligament involvement.
Clinical test of percussion along with presence of any of the
contributing factors further helps in diagnosis. Vitality testing can be of help if the cause is related to pulpal pathology,
because a necrotic or dead pulp definitely favors a diagnosis
of apical periodontal pathology of some degree. Radiographic
findings of a widening of the periodontal space with or
118
without a breach of lamina dura and altered trabecular pattern are a definitive indication of periodontal pathology.
Dental pain
Dental hypersensitivity
May be intermittent or
continuous, depending upon the
stage of pathology
BARODONTALGIA
Barodontalgia is the pain or injury associated with teeth as a
result of alteration in the pressure gradients. Historically, the
term aerodontalgia was used to describe pain experienced by
air crew in flight. Over a period of time it was noticed that
deep sea divers also suffered tooth pain due to pressure
changes, prompting the use of the term barodontalgia.
Barodontalgia can be explained by Boyles law, which
states that at a given temperature, the volume of a gas is
inversely proportional to the ambient pressure. As an individual descends deeper and deeper below the water surface,
pressure exerted by water on the diver increases and
reduces the volume of gases in enclosed spaces such as
teeth and the paranasal sinuses.
Similarly during a high altitude ight the atmospheric
pressure outside the aircraft decreases thereby permitting the
volume of gases to increase. These changes in pressure affect
air crew and passengers of a non-pressurized aircraft.
Barodontalgia arises when gases that are conned within
closed spaces are unable to contract to adjust the internal
pressure to correspond to the outside pressure. The phenomenon begins to occur at an altitude of approximately
3,000 m and at a water depth of 10 m where the ambient
pressures are 0.75 and 1 atm, respectively.
Individuals may experience a simple sharp or squeezing
tooth pain. Occasionally the pressure change may cause the
rupture of the alveolar mucosa.
Strohaver (1972) categorized barodontalgia into direct
and indirect types. In the direct type, reduced atmospheric
pressure contributes to a direct effect on a given tooth.
However in the indirect type, dental pain is secondary to
stimulation of the superior alveolar nerves by a maxillary
barosinusitis.
Direct barodontalgia is generally manifested by moderate to severe pain, which usually develops during ascent, is
well localized, and the patient can frequently identify the
Etiology
Kollmann (1933) proposed three hypotheses to explain the
occurrence of barodontalgia: expansion of trapped air
bubbles under a root filling or against dentin that activates
nociceptors; stimulation of nociceptors in the maxillary
sinuses, with pain referred to the teeth; and stimulation of
nerve endings in a chronically inflamed pulp.
In most cases of barodontalgia the individuals tooth is
already affected by some sort of pathology such as acute or
chronic periapical infection, caries, deep restorations, residual dental cysts, sinusitis and a history of recent surgery.
The term barosinusitis has been used in literature to
describe pain in the tooth caused by congestion of the
maxillary sinus and most often the pain is experienced
during descent. Whereas barodontalgia is usually experienced during ascent and the tooth is affected usually by
periapical pathology.
Underwater Diving
During underwater diving the air from the pressurized tanks
may be forced into the tooth via the carious lesions or defective margins of restorations. With the fall in atmospheric
pressure during ascent, trapped gases may expand and enter
dentin tubules, thereby stimulating nociceptors in the pulp
or causing the movement of pulp chamber contents through
the apex of the tooth, resulting in pain.
Calder and Ramsey (1983) suggest that the physical
properties of the gas mixture used during deep sea diving
may contribute to barodontalgia. In scuba tanks, oxygens
natural diluent gas, nitrogen, is replaced by helium, resulting in a gas of lower viscosity. This gas can enter tissues,
including teeth, and can sometimes become trapped in
closed spaces, such as the pulp chamber and root canal.
There are two mechanisms by which gases can be trapped
in spaces: if there is a space between a tooth and its restoration, gas may be forced into it during an increase in
pressure; and dissolved gas may diffuse from tissues into
spaces as pressure decreases.
Occasionally, the trapped gas will expand and the resulting stress may cause tooth fracture. This process has been
called odontecrexis (Greek word meaning tooth explosion).
The Fdration Dentaire Internationale (FDI) recommends an annual check-up for divers and pilots, with oral
hygiene instructions. Also it recommends that the patients
should not dive or y in non-pressurized cabins within
24 hours of a dental treatment requiring anesthetic or for
a week following a surgical treatment.
Some authors suggest that when treating people who
are subjected to large pressure changes it is best to avoid
MYOFASCIAL PAIN
Pains of muscular origin are one of the frequent sources of
chronic pain anywhere in the body and the head and neck
region is not bereft from it. The pain originating from the
skeletal muscles, tendons and fascia surrounding these constitute the term myofascial pain (MFP). The intimate relation of the muscles in and around the oral cavity, both
anatomically and functionally accords the orofacial musculature an important consideration in the differential diagnosis of chronic orofacial pain. Pathology originating in the
dental structures can cause effects manifested in the musculature. The group of muscles involved, their number and their
relative location determine the severity of pain felt by the
patient. According to Bell (1989), it is a good rule to follow
119
NEURALGIAS
Neuralgia is a clinical condition involving a pain of a severe
intensity, with a throbbing or stabbing character in the course
or distribution of a specific nerve.
Trigeminal Neuralgia
Trigeminal neuralgia (TN) is frequently encountered neuropathy in dental practice affecting the fifth cranial nerve.
Though it has been known by various names in the literature such as tic douloureux, trifacial neuralgia, Fothergills
disease, the currently accepted terminology is trigeminal
neuralgia. It is a well-recognized disorder characterized by
a severe, paroxysmal burst of pain in one or more branches of
the trigeminal nerve often induced by touching trigger points,
in or about the oral cavity. TN is diagnosed primarily on
the basis of the history of characteristically unilateral pain
attacks that are consistent with specific clinical criteria for
the diagnosis. However the typical sequence of events that
occur in a patient manifesting TN begins with the initial
symptoms of pain that may be misdiagnosed as pain of
odontogenic origin. A careful history-taking is of paramount
importance stressing upon the onset (whether insidious or
sudden), the duration and frequency of painful episodes, and
other associated symptoms.
The exact cause of TN pain attacks is not known in
spite of accurate description of the condition over the
years by several researchers. However, an interesting nding, that some benign tumors in the brain and/or vascular
anomalies leading to compression and possible demyelination of the trigeminal nerve root at the entry into the
pons, produce symptoms clinically characteristic of classic
TN. This nding strongly implies that injury to the nerve
root may be an important initiating factor. When such a
pathologic component is present, it is known as symptomatic TN, but in the absence of any such causative factor,
the condition is known as idiopathic TN. Oral causes, such
as periodontal lesions and traumatic lesions have been
long discarded.
Clinical features
Management
Management of MFP depends upon the identification of
the trigger point and the referred zone. Conservative
methods based upon physiotherapy principles should be
applied that can be supplemented with drugs. Basic muscle
120
5.
The IHS has defined classic TN as a unilateral disorder characterized by brief electric shock-like pains, abrupt in onset
and termination, limited to the distribution of one or more
divisions of the trigeminal nerve. Pain is commonly evoked
by trivial stimuli including washing, shaving, smoking,
talking and/or brushing the teeth (trigger factors) and frequently occurs spontaneously. Small areas in the nasolabial fold and/or chin may be particularly susceptible to the
precipitation of pain (trigger areas). The pains usually remit
for variable periods. This definition covers most essential
clinical presentations of TN.
Differential diagnosis
There are few other conditions that can mimic such a characteristic condition. However, in some cases, especially in
the initial stages, may need other conditions to be ruled out.
Chief among these are odontogenic causes, TMJ-related disorders, atypical facial pain, glossopharyngeal neuralgia,
multiple sclerosis.
The specic age group and sex distribution usually helps
differentiate TN from other causes like atypical facial pain
and TMDs that usually begin at a much younger age. A valuable clue to the diagnosis of TN is the occurrence of the
pain attack with certain activities, such as rubbing the face
or shaving a trigger area, whereas in other facial pain syndromes, they massage the face or apply heat or ice, which
is strikingly missing out in the history reported by TN
patients.
Investigations
Usually all laboratory values are seen to be within normal
limits. Any kind of electrophysiological testing in the
affected area of the face also usually does not reveal any
profound abnormalities. However some clinicians insist
on an elective MRI scan of the brain to detect any missed
out clues, intracranially, and only then proceed with the
treatment.
Treatment
Given the older age manifestation of TN, medical therapy
is usually better tolerated than any form of surgical intervention, which can be attempted only if and when the pharmacologic therapy fails.
Two broad groups of pharmacologic agents, anticonvulsants and skeletal muscle relaxants, are usually employed
in the management of TN. Among the anticonvulsants,
carbamazepine is the drug of choice for TN. A 100-mg
tablet usually accords substantial relief within 2 hours, and
therefore it is used as a rst choice agent. So predictable
and powerful is the relief that if the patient does not respond
at least partially to carbamazepine, it should lead to a
121
Glossopharyngeal Neuralgia
Glossopharyngeal neuralgia (GPN) is a neuropathy similar
to TN, but occurs due to affliction in the distribution of the
122
ninth cranial nerve. Harris (1926) coined the term glossopharyngeal neuralgia. This condition though less frequently
seen in comparison to TN, is nonetheless equally painful
and causes a great deal of deterioration in the quality of
life in affected persons. The exact cause of this condition
is unknown as yet, with some reports of nerve compression
leading to the condition. However, this still remains to be
ascertained as the definitive cause of GPN.
Clinical features
Glossopharyngeal neuralgia characteristically manifests in
the older age group usually beyond 60 years of age. It does
not exhibit any specific gender predilection. A patient suffering from GPN will give a history of severe shooting
pain occurring in episodes. The attacks have no specific
pattern and may vary in frequency from a few per day to
rarely once in few months. The severity too can oscillate
from between intolerable to relatively milder attacks. The
pain is usually localized to areas around the posterior
parts of the oral cavity and the pharynx in a typical unilateral manifestation. The pain attacks may be initiated by
some specific functions like speech, swallowing, yawning
or coughing (associated with tongue movements).
Clinical examination usually yields no specic information, but careful probing may reveal certain sensitive areas
or trigger points, that are usually located in the oropharyngeal walls or the peritonsillar area (including the soft palate). Thus palpation of the lateral aspect of the throat can
easily provoke an attack conrming the diagnosis. Rarely,
the pain from GPN may be felt in the ear region only, caused
due to involvement of the tympanic plexus of the ninth
cranial nerve. The close proximity of the glossopharyngeal
nerve to the vagus nerve may cause accompanying vagal
manifestations like syncope, arrhythmia and at times,
even cardiac arrest.
Differential diagnosis
Since the trigger zones of GPN lie around areas of jaw
usage, the clinician may easily confuse it with odontogenic
causes, TMJ-related problems or masticatory muscle disorders. Masticatory pain may be easily differentiated by
avoiding movements of the mandible and allowing free
movements of the tongue. GPN will not be prevented as
tongue movements would precipitate the attack. Another
definitive method is to apply a topical anesthetic on to the
pharyngeal mucosa that would completely stop the pain
impulses arising from the stimulation of trigger zones. Any
relief by this method would certainly point to a diagnosis
of GPN.
Eagles syndrome, associated with an elongated styloid
process compressing the glossopharyngeal nerve may produce symptoms similar to those of GPN. A radiographic
examination of the neck can help rule out calcic enlargement of the styloid process. Also pain on rotation of the head
Flowchart 1
Trigeminal neuralgia
New case
Refractory cases
Resistance
Carbamazepine
(Mazetol, Tegretol)
100 mg b.i.d.
Phenytoin sodium
(Eptoin, Dilantin)
100 mg
1. Percutaneous radiofrequency
thermocoagulation
2. Microvascular decompression
Sustained release
carbamazepine
(Mazetol SR, C-Lep SR)
400 mg b.i.d./day
Note: All available medications have been enlisted. however, the physician should
choose the appropriate mode of treatment/drug based on the clinical situations
Postherpetic Neuralgia
The virus herpes virus varicellae or human herpes virus-3
(HHV-3) causes the infections varicella and herpes zoster
in man. The primary infection with this virus leads to the
clinical manifestation of varicella or chicken pox. Once
the primary infection resolves, the virus enters into the
sensory nervous system and can remain latent there for
many years. In the head and neck, the trigeminal ganglion
is the preferred site that harbors the virus during its
latency. Due to some reason, activation of such latent viral
particles causes the secondary infection called herpes
zoster.
Spontaneous pain, pain provoked by trivial stimuli, and
altered sensation accompany herpes zoster and which may
continue long after its characteristic rash has healed is
known as postherpetic neuralgia (PHN). Herpes zoster infection by itself is a painful condition and the pain continues
up to a month in most patients. But when the lesions have
clinically healed and the pain still persists beyond a period
of 3 months, the diagnosis points to PHN. The exact cause
of PHN is not known, as it does not occur in every patient
who manifests zoster infection. The sensory alterations
and pain that occur in PHN have been thought to occur as
a result of both peripheral and central disturbances in the
nervous pathways. Many risk factors have been noted in
clinical studies that predispose for development of PHN,
such as advancing age, the site of zoster rashes (a particularly higher risk for trigeminal manifestation), a severe
prodromal pain before the zoster manifestation and a very
severe degree of rashes.
Clinical features
Postherpetic neuralgia usually affects the elderly group of
patients over 60 years. As the age advances, the risk for
developing PHN increases. The general debilitation of the
health and/or any accompanying immunocompromised
124
Occipital Neuralgia
Paroxysmal sharp pain occurring in the region supplied by
the greater and lesser occipital nerves constitutes occipital
neuralgia. The pain is felt in the posterior region of the skull
up to the vertex. Rarely the patient may manifest temporal
or at times even retro-orbital pain. Usually this condition
arises due to the compression of the nerves, following trauma,
infection or neoplasms. Given the frequency of musculoskeletal pains occurring in the same region the clinician needs
to carefully differentiate these from each other. The presence
of trigger points in the cervical region causing radiating
pain up to the occipital region favors a musculoskeletal disorder. Local anesthetic blocking of the taut trigger points
helps in easy differentiation. No satisfactory management
strategies have been outlined in literature due to the paucity of true occipital neuralgia cases.
ATYPICAL ODONTALGIA
Atypical odontalgia (AO) is a clinical condition that poses
quite a challenge to the dental clinician. As the name suggests it is toothache that cannot be attributed to any cause
that are usually suspected. It is therefore also known as
phantom pain. Various other terminologies have been
applied to this condition in the past, such as, atypical
facial neuralgia, migrainous neuralgia, idiopathic toothache, etc. that have only added to confusion rather than
clear many of the impending doubts regarding this condition. Therefore the use of these alternative terminologies is
best avoided. AO is a frequently encountered condition in
clinical practice. Patients usually end up getting various
dental treatment procedures performed for the problem
and often seek exacting therapy from the clinician. The
multiple procedures performed previously may at times
mislead the dentist into assuming the diagnosis provided
by the previous clinician. It is still a poorly understood
phenomenon with unclear pathophysiology and as a consequence diagnostic confirmation is achieved by excluding other known causes or those with a somatic etiology.
Some authors have suggested a deafferentation pain model
for explaining the clinical behavior, however till date,
this has not been conclusively proven. Another school of
thought, that too has not been validated, has believed that
some form of trauma has to be involved that may lead to
some unexplained changes in either transmission or recognition of the pain impulse. The association of AO with
psychological disorders has led to some belief that the pain
may be a component of the spectrum of the psychosomatic
disorders.
Clinical features
Atypical odontalgia is a very frequently encountered clinical situation and patients present with symptoms of
toothache. The patient is usually a middle aged lady in her
early forties but may manifest in significantly older
women too. The exact reason for this age and gender predilection has not been understood. The patient usually
points out specifically to a single tooth or very rarely a
group of adjacent teeth. The teeth most commonly affected
are the premolars and molars of the maxillary jaw, AO is
very rarely reported in the mandibular teeth. On examination, there is no abnormality detected in the complaint
tooth. Many a times, the tooth may have been treated endodontically, but does not otherwise show up any cause for
concern. The tooth may have been extracted and then in
such cases, the patients insist that the procedure has not
alleviated the pain and the pain still persists, as if the tooth
is still present, giving rise to the term, phantom pain of the
tooth. The pain described is of a dull, aching, throbbing or
burning and persistent type. The patient will usually
describe a feeling of unpleasant sensation being present
throughout the day that does not increase during functions
such as eating or swallowing. AO does not cause any disturbance in sleep and patients report of occurrence of pain
immediately after awakening. The presence of the pain is
usually not altered by local provocation such as during
pulp testing, percussion, etc. Very rarely the pain may
spread to the adjoining structures within the oral cavity,
such as jaw bones and even rarely may involve the broader
facial region.
Graff-Radford and Solberg have outlined the following
criteria that may help in identifying AO:
1.
2.
3.
4.
5.
All investigative modalities such as laboratory or radiographic tests turn up with negative results. The patient usually undergoes a lot of attempted therapies without having
accorded a definitive diagnosis.
Differential diagnosis
Other pathologies manifesting similar symptoms need
to be ruled out before arriving at a diagnosis of AO, as
125
be of no value in causing any pain relief. The patients usually agree to this after having suffered through numerous
procedures not having provided relief. Any psychologic
association if suspected should be dealt with in conjunction with a psychologist.
Atypical odontalgia does not respond to opioid as well
as non-opioid analgesics. The tricyclic antidepressants are
the most favored drug employed in managing AO. The
exact mode of action of these medications is not known,
but it has been suggested that the analgesic properties of
these drugs are more likely acting than their antidepressant
effects. Amitriptyline is prescribed in doses ranging from
25 to 75 mg per day initially and if needed may be used up
to 200 mg per day. The benecial effect of the drug needs
to be carefully titrated against the risk of adverse effects
such as dizziness, drowsiness, headache, xerostomia, constipation, appetite and weight changes, nausea, weakness,
hypotension. But patients not achieving relief with high
doses are unlikely to have any satisfactory remission of the
condition. In those cases that do achieve relief, complete
elimination of pain is rare and therapy needs to be continued for a prolonged duration, a minimum of 3 months.
Other tricyclic antidepressants such as imipramine, nortriptyline, and dothiepin have also been used. However,
their effects are not very different from amitriptyline.
Phenothiazines have also been used with some success in
some clinical trials, however, their role is more supplementary to the tricyclic drugs. Tardive dyskinesia is a major
potential side effect of phenothiazines that has not allowed
for a major role of these drugs in AO. Once pain control of
a desirable degree is achieved, all these drugs should be
tapered off gently and then discontinued.
Flowchart 2
Burning mouth syndrome
Initial visit
Chronic cases
Topical:
A. Capsaicin gel 0.025% (Capsain-p)
B. Alpha-lipoic acid as mouthrinse
b.i.d./12 months (ALA-100, Aladin
100 mg)
Systemic:
A. Multivitamins B12, methyl
cobalamin and folic acid (MCBM-69,
Neurokind-G and Nuvolt-G t.i.d.;
Nurokind plus, Nuvolt: o.d.)
Systemic:
A. Amitriptyline 25 mg b.i.d. (Amitone, Amitryn, Amitrol,
Tadamit) for 15 days, o.d. for 15 days
B. Gabapentin 300 mg (Gabantin, Gabapin) 1st day 300 mg,
2nd day 300 mg b.i.d., 3rd day 300 mg t.i.d. and then maintain
the dosage. It can be given to a maximum of 2400 mg/day
C. Alprazolam 0.25 mg b.i.d. (Restyl, Alcalm, Anxit SR-0.5 mg)
for a week and slowly withdraw the drug
D. Clonazepam 2 mg o.d. at bed time for 15 days (Clozep,
Lonazep MD, Melzap)
E. Nortriptyline 25 mg o.d. for not more than 3 months (trip)
Note: All available medications have been enlisted. However, the physician should choose
the appropriate mode of treatment/drug based on the clinical situations
128
Management
Any treatable cause of the burning sensations in the oral
mucosa should be identified and treated comprehensively
before labeling any patient to be suffering from BMS.
However, once all such conditions have been ruled out
and a diagnosis of BMS has been arrived at, the situation
should be explained to the patient and counseling regarding the benign nature of the condition should be offered.
The decrease in the quality of life should be explained as
in some patients the conditions undergoes spontaneous
remission while in the majority of cases it persists as a
chronic condition. Due to an unclear etiopathogenesis,
definitive pharmacologic therapy is not available for the
condition and all modalities are essentially palliative in
nature. Topical, systemic and psychologic therapies have
been used with varying degrees of success. Topical therapies with anesthetic products such as lignocaine and benzydamine hydrochloride seem to be effective in a handful
129
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SECTION
III
Mucocutaneous
Disorders
6
7
8
9
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174
196
218
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CHAPTER
In day-to-day clinical practice oral physicians often encounter a wide spectrum of oromucosal lesions. These lesions
range from harmless mucosal alterations like change in
the color and texture of the oral mucosa, needing simple
therapeutic remedies and patient counseling to lesions of
a life-threatening nature. Red and white colored alterations
of the oral mucosa are commonly seen in dental practice.
Recognizing and differentiating these mucosal alterations
from normal anatomic variations is imperative for the
effective management of these lesions.
Formation of pseudomembranes
Pigmentation producing cells like melanocytes.
from various factors like, trauma, infections, immunologic injury to the mucosa or other genetically determined
factors.
5.
Red Lesion
Red lesion refers to an area of reddened mucosa that may
appear smooth and atrophic or exhibits a granular, velvety
texture (e.g. erythroplakia, median rhomboid glossitis,
erythematous candidiasis, etc). These lesions may occur
alone or in combination with a white lesion. Such lesions
may be termed as a mixed lesion or a red and white lesion
(e.g. speckled leukoplakia, erosive lichen planus, etc).
Individual variations in the color of the oral mucosa are
probably an expression of one or more genetically controlled factors.
Healthy masticatory mucosa (gingiva, palate, dorsal
surface of the tongue) is light pink in color. The lining
mucosa (mucosa over the vestibule, cheeks, lips, oor of
the mouth, and ventral surface of the tongue) is reddish
pink in color. Palatoglossal arch region is dusky red in
color due to increased vascularity and often misdiagnosed
as sore throat.
The histological reason behind the appearance of a red
lesion may be due to dilated blood vessels, inux of new
blood vessels, hemorrhage under the epithelium or a relatively thin outer epithelium.
2.
3.
4.
134
6.
7.
Premalignant lesions
Leukoplakia
Lichen planus
Lichenoid reactionsdrug induced, graft-versushost disease
Erythroplakia
Actinic keratoses
Discoid lupus erythematosus
Chronic hyperplastic candidiasis
Premalignant conditions
Oral submucous fibrosis
Oral psoriasiform lesion
Dyskeratosis congenita
Syderopenic dysphagia
Syphilitic glossitis
Miscellaneous
Intraoral skin grafts (people of Afro-Asian origin) will
not generally exhibit a white coloration of the skin
graft. The graft will appear black or brown depending
on the extent of melanin pigmentation (Figure 1).
Figure 1
Figure 2
Table 1
Keratotic lesions
(non-scrapable)
Non-keratotic lesions
(scrapable)
Management
Removal of the etiologic agent will generally result in complete resolution of the lesion within 2 weeks. Biopsy may
be considered in lesions that persist for a longer duration,
to rule out any dysplastic changes.
Figure 3
A
(A) Whitish macerated appearance of the upper labial mucosa. (B) Whitish thickened and shredded area on the lower labial
mucosa. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Figure 4
Figure 5
Management
1.
2.
3.
Nicotine Stomatitis
(Stomatitis Nicotine Palatinus, Smokers Palate)
Thoma in 1941 was the first to use the term stomatitis nicotine because he noticed this lesion to almost exclusively
136
Figure 6
A
(A) Diffuse grayish white pigmentation of the palate with red pinpoint areas characteristic of smokers palate.
(B) Multiple papules on the palate with red pinpoint areas in smokers palate. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
Leukoplakia
Oral leukoplakia (OL) is the most common precancerous
lesion of the oral mucosa. The term leukoplakia was first
used by Schwimmer in 1877 to describe a white lesion on
the dorsum of the tongue, which probably represented
syphilitic glossitis. He proposed the term leukoplakia for
a diffuse patch on the dorsum of tongue. Since then it has
evolved as a clinicopathologic concept over many years;
sometimes representing an innocent hyperkeratosis and
sometimes dysplastic features.
Definition
The requirement for a clear definition for oral white
lesions has long been recognized. Similar requirements
also apply to red lesions or the red component of preponderantly white lesions. The definition of leukoplakia has
often been confusing and controversial. Currently the WHO
definition and definition given by Axell are widely used.
Auluck et al conducted a survey in 10 different dental colleges in India among 153 specialists including oral surgeons,
oral physicians and oral pathologists to check the prevalence of confusion regarding the definition of leukoplakia
and its application. It was found that 33.33% of the specialists preferred to follow WHO definition (1978), while 65.35%
preferred to follow Axell (1984) definition. The authors
described the current ambiguity regarding the accepted
definition of oral leukoplakia and emphasized the need for
an international collaboration to reach a consensus on the
use of the term leukoplakia.
WHO definition of leukoplakia (1978) Leukoplakia is a
white patch or plaque that cannot be characterized clinically
or pathologically as any other disease. The definition indicates that the term leukoplakia does not carry a histologic
connotation and should be used only in descriptive clinical
context.
Axell et al definition of leukoplakia (1984) Leukoplakia
is a white patch or plaque that cannot be characterized
clinically or pathologically as any other disease and not
associated with any physical or chemical causative agent
except the use of tobacco.
Axell et al definition of leukoplakia (1996) Leukoplakia
as a predominantly white lesion of the oral mucosa that
cannot be characterized as any other definable lesion;
some oral leukoplakia will transform into cancer. This definition is most widely accepted in the western world.
Pindborg et al definition of leukoplakia (1997) A predominantly white lesion of the oral mucosa that cannot be
characterized as any other definable lesion.
137
Epidemiology
The frequency of leukoplakia is highly variable among
geographical areas and demographic groups. The prevalence in the general population varies from less than 1 to
more than 5%. The prevalence of leukoplakia increases to
8% in men over the age of 70 and the prevalence in women
past the age of 70 is approximately 2%.
Mehta et al (1961) reported the prevalence of 3.5% in
4,734 Indian population. Manghi et al (1965) reported a
prevalence of 6.5% among 2,004 persons. Smith et al (1975)
reported a prevalence of 11.7% among 57,518 persons.
Yang et al (2001) reported the prevalence of leukoplakia
as 24.4% among the aborigines in southern Taiwan. Chang
et al (2005) reported the prevalence of 7.44%. In a 10-year
follow-up study, a random sample of 30,000 villagers in
three areas in India, the annual incidence rates varied
from 1.1 to 2.4 per 1,000 men and 0.03 to 1.3 per 1,000
women.
In India the prevalence of oral leukoplakia among betel
quid chewers with tobacco ranged from 0.4 to 1.8% and
among betel quid chewers without tobacco ranged from
0.3 to 0.7%.
Table 2 gives the overall impression of the prevalence of
oral leukoplakia with a geographic emphasis.
Classification and staging system for
oral leukoplakia
Van der Waal (2000) designed and proposed the classification and staging system. The present classification and
staging system is primarily designed for the purpose of
uniform reporting of treatment or management results and
requires the availability of a biopsy report. It also could
serve as means for epidemiological studies.
L (size of the leukoplakia)
L1 Size of single or multiple leukoplakia together 2 cm
L2 Size of single or multiple leukoplakias together 24 cm
L3 Size of single or multiple leukoplakias together 4 cm
Lx Size not specied
Table 2
138
P (pathology)
P0 No epithelial dysplasia (includes no or perhaps mild
epithelial dysplasia)
P1 Distinct epithelial dysplasia (includes mild to moderate and moderate to possibly severe epithelial dysplasia)
Px Absence or presence of epithelial dysplasia not
specied in the pathology report.
OLEP (Oral leukoplakia) staging system
Stage I
L1P0
Stage II
L2P0
Stage III
L3P0 or L1L2P1
Stage IV
L3P1
General guidelines for oral leukoplakia
staging system
1.
2.
3.
Prevalence of leukoplakia
Country
Prevalence (%)
Pindborg et al (19651966)
India
30,000
India
2,03,249
0.7
Mehta et al (1972)
India
1,01,761
0.7
Axel (1976)
Sweden
20,333
3.6
USA
23,616
2.9
Netherlands
1,000
1.4
Hungary
7,820
1.3
Ikeda et al (1991)
Japan
3,131
2.5
Schepman et al (1996)
Netherlands
1,000
0.6
1.53.3
Figure 7
Figure 8
Clinical features
1.
2.
3.
140
4.
Early or thin leukoplakia appears as a slightly elevated grayish-white plaque that may be either well
defined or may gradually blend into the surrounding
normal mucosa (Figure 11). As the lesion progresses, it
becomes thicker and whiter, sometimes developing a
leathery appearance with surface fissures. Some leukoplakias develop surface irregularities and are referred
to as granular or nodular leukoplakias. Other lesions
develop a papillary surface and are known as verrucous or verruciform leukoplakia.
Figure 9
Figure 11
Figure 10
Figure 12
Figure 13
Malignant transformation
White lesion in the oral cavity were thought to be precancerous as early as 1870 by Paget, who gave them such appellations as ichthyosis, smokers patch and leukokeratosis.
Leukoplakia is an example for precancerous lesion.
The frequency of dysplastic or malignant alterations in
oral leukoplakia has ranged from 15.6 to 39.2% in several
studies. Although leukoplakia is more common in men
than women, several studies have shown that women with
leukoplakia have a higher risk of developing carcinoma.
A wide range of rates for the malignant transformation
of leukoplakia has been reported from 0.13 to 19.8% but it
is estimated that the annual transformation rate should
not exceed 1%. Up to 10% of leukoplakia may be malignant at the time of initial examination. The potential for
malignancy appears higher in certain risk sites like oor of
the mouth and ventral surface of the tongue, where the
lesion is associated with Candida species, or where the lesion
is verrucous or mixed with red lesions (erythroleukoplakia
or speckled leukoplakia).
Cawson (1966), Einhorn and Wersall (1967) and Banoczy
(1977) inferred that certain features of leukoplakia have
been reported to be associated with an increased risk of
malignant transformation. These are:
142
Non-homogeneous type of OL
Presence of Candida albicans
Presence of epithelial dysplasia.
Longitudinal studies of the rate of malignant transformation in leukoplakia were first reported by Sugar and Banoczy
(1959) in Hungary.
In a study conducted by Gupta et al, 12,212 tobacco
users were followed up annually to assess the malignant
potential of precancerous lesions in Ernakulam district in
Kerala, India. They observed that out of a total of 19 new
oral cancers over period of 8 years, 15 (79%) originated
from leukoplakia.
The location of oral leukoplakia has a signicant correlation with the frequency of nding dysplastic or malignant changes at biopsy. In the study by Waldron and
Shafer (1975), the oor of the mouth was the highest risk
site, with 42.9% of leukoplakias showing some degree of
epithelial dysplasia, carcinoma in situ, or unsuspected invasive squamous cell carcinoma. The tongue and lips were also
identied as high risk sites, with dysplasia or carcinoma
present in 24.2% and 24% respectively.
The study by Silverman and colleagues (1984) showed
an overall malignant transformation of 17.5%. In their
study only 6.5% of homogeneous leukoplakia underwent
malignant change, however, 23.4% of speckled leukoplakia and 36.4% of leukoplakias with microscopic evidence
of dysplastic changes transferred into cancer.
Among the different clinical varieties of leukoplakias,
proliferative verrucous leukoplakia has got highest risk of
malignancy. In a follow-up study of 54 cases of proliferative
verrucous leukoplakia, Silverman and Gorsky found that
70.3% of the patients subsequently developed squamous
cell carcinoma.
Holmstrup and Besserman (1983) and Silverman (1990)
inferred that proper use of antifungal therapy might result
in a shift from high risk nodular or speckled leukoplakia to
low risk homogeneous leukoplakia. Hernandez et al (2003)
have suggested that in patients who display oral premalignant conditions like leukoplakia, immunosuppression must
be considered as an important risk factor for oral cancer.
Investigations
Although an experienced oral physician may be able to
diagnose and manage majority of the leukoplakias, it is
always prudent to follow a systematic investigative protocol to diagnose leukoplakia. The investigative procedures
includes the following:
Figure 14
Figure 15
Biopsy: When a suspicious lesion is identified, an incisional biopsy using a scalpel or a biopsy forceps is
recommended. When the lesion is very small excisional
biopsy is performed as an investigative procedure and
as a treatment modality.
In homogeneous leukoplakia, the value of histological
examination to some extent is questioned. The occurrence of epithelial dysplasia is rather low in this type
as is the risk of future malignant transformation. However, even the experienced clinician will occasionally
be surprised by the histopathological findings of a
clinically innocent looking homogeneous leukoplakia.
Therefore a biopsy should be performed in homogeneous leukoplakia. In non-homogeneous leukoplakia,
i.e. usually symptomatic, epithelial dysplasia or even
carcinoma in situ or early squamous cell carcinoma is
rather common. The biopsy should be taken at the site
of symptoms, if present, and or a site of redness or
induration. Biopsies of exophytic, verrucous or papillary lesions should be taken deep enough to include a
sufficient amount of underlying connective tissue, and
preferably from the margins.
This lesion represents various degrees of epithelial dysplasias. Some lesions exhibit carcinoma in situ with top
to bottom basilar hyperplasia, loss of polarity, increased
mitosis, hyperchromatism, dyskaryosis and alteration
in nuclear cytoplasmic ratio without any evidence of
thickening of epithelial layer or without any evidence
of disturbance of keratinization process (Figure 15).
The frequency of epithelial dysplasia in leukoplakia
varies between less than 1% and more than 30%. The
presence of epithelial dysplasia is generally accepted
as one of the most important predictors of malignant
development in premalignant lesions.
Markers of proliferation in leukoplakia: There are
markers for determining future cancer development in
oral premalignant lesions. These markers are divided
into genomic markers and differentiation markers.
The genomic markers include DNA aneuploidy, loss of
heterozygosity and changes in expression of oncogenes and tumor suppressor genes (p53), whereas the
proliferative markers include keratins and carbohydrate
antigens.
Differential diagnosis
The keratotic lesions that could be considered in the differential diagnosis of homogeneous leukoplakia includes:
Treatment (Flowchart 1)
General considerations As a standard rule all possible
agents leading to white keratotic lesions should be eliminated
such as sharp teeth, candidal infection, etc. so as to rule out
other definable lesions. In persisting lesions or in the absence
of possible causative factors, a biopsy should be taken to
143
Flowchart 1
Leukoplakia
Topical antifungal agents
Candid cream (clotrimazole),
thrice/day for a week
No response
Reduction in size
Less than 1 cm
More
Morethan
thanor
or
equal
equal to
to 11cm
cm
Excisional biopsy
Incisional biopsy
Dysplasia present
Total
Totalexcision
excisionof
ofthe
the
lesion
lesionwith
withgraft
graft
Follow
Followup
uponce
oncein
in
66months
monthsfor
for33years
years
Continue
Continuethe
thetopical
topical
antifungal
antifungalagent
agentfor
for
11month
month
Dysplasia absent
IfIfexcision
excisionisisnot
notpossible
possible
A.
Capsules of
of lycopene
lycopene 44 mg
A. Capsules
mg b.i.d.
bid or
8 mg
3 months
8ormg
ODo.d.
for for
3 months
B.
Capsules antioxidants
of antioxidants
with
B. Capsules
with
selenium bid
b.i.d.
6 months
selenium
forfor
6 months
C.
Topical bleomycin
bleomycin 1%
1% w/v
w/v thrice
thrice
C. Topical
a day
days
day
forfor
1515
days
Retinol
A
RetinolA
(VitaminAAanalogue)
analog)
(Vitamin
ointment
ointmentapplication
application
b.i.d./1
b.i.d./1month
month
Note: All available medications have been enlisted. However, the physician should choose
the appropriate mode of treatment/drug based on the clinical situation
Management of leukoplakia
form of photodynamic therapy (PDT) using hematoporphyrins has been found to be effective in animal models
and has been used to treat head and neck cancers and
premalignant lesions in man. PDT was first used in 1990,
when acridine and light were combined to kill paramecia
and the first oncologic use of PDT was in 1903, when
eosin and light were employed in the treatment of skin
cancer. PDT involves using specific wavelength of laser
light to activate a photosensitizing drug which is administered systemically and is retained selectively in the lesion
and this triggers a cold photochemical reaction resulting
in the generation of reactive products such as singlet oxygen that damages tissue. Advantages of this type of treatment as reported by Sciubba (1995) includes inactivation
of clinically subtle or undetectable alteration, sparing of
normal tissue, minimal morbidity and its use as an adjunctive tool to more traditional modalities. However it was
pointed out that the major disadvantage of PDT is the
cutaneous photosensitivity which can persist for several
months after administration of the photosensitizer which
can be a major problem in the Indian subcontinent, where
oral cancer is most common.
Topical chemotherapy of oral leukoplakia Topical treatment of leukoplakia with podophyllin solution (Kovacs
et al, 1962) or bleomycin (Hammersley et al, 1985;
Malmstrom, 1988; Wong et al, 1989) has induced some
regression or even total resolution of dysplasia and of
clinical lesions. Hayasaki et al (1977) described the use of
bleomycin with iontophoresis in the treatment of cancer,
leukoplakia and papillomas of the head and neck region.
Their results showed that this method of application was
not effective for malignant lesions but was effective at
removing leukoplakia of the oral mucosa.
Newer treatments
Gene therapy Patients with head and neck cancer including oral cancer are more susceptible to chromosome damage when their cells are exposed to mutagens (Schantz et al,
1990) and there are a number of genetic changes now
described in oral carcinoma (Scully et al, 1993). Synthetic
antisense oligonucleotides complementary to the start
codons of human papilloma virus (HPV) type 18 E6 and E7
genes can significantly inhibit growth in vitro of oral carcinoma cell lines (Steele et al, 1992, 1993).
Even though laboratory and animal data for the use of
gene therapy is very incomplete, many investigators have
begun clinical trials in human patients. Tests of several
types of gene therapy have begun in various types of cancer, and for oral cancer; the trials include the testing of
recombinant p53, the expression of suicide genes and the
use of conditionally competent adenoviruses. Since the
scientic basis for these trials is rather weak, it can hardly
be expected that impressive results are imminent. There
are as yet no trials in oral potentially malignant lesions
145
Tea polyphenols are effective in reducing the accumulation of free radicals by inducing the production of
superoxide dismutase (SOD), a free radical scavenger
(Das et al, 2002).
Tea inhibits formation of mutagens in a dose dependaet manner (Weisburger et al, 2002) and reduces lipid
peroxidation (Fadhel et al, 2002).
In UV induced responses, epigallocatechin gallate
(EGCG) prevents the formation of UVB induced cyclobutane pyrimidine dimers (Katiyar et al, 1999).
EGCG is also a strong inducer of the detoxifying
enzyme glutathione-s-transferase.
These reports strongly point toward antimutagenic activity of green tea. The gallated flavonoids (especially gallated EGCG and the aflavins) act Khafif et al (1998)
reported that green tea extract has been shown to have a
chemopreventive or inhibitory role in the treatment of oral
leukoplakia. Li et al (1999) conducted a double blind, placebo-controlled trial in 59 patients with oral leukoplakia,
and found that oral and topical administration with a
black and green tea mixture resulted in a partial regression
of the lesion in 37.9% of the treated patients compared to
placebo control. The treatment reduced cell proliferation
and the rate of chromosome aberration in peripheral blood
lymphocytes. Weisburger et al (1999) have proposed that
catechins, in particular EGCG are believed to be responsible for the chemopreventive effects of green tea, which act
by antioxidant and free radical scavenging activity.
Oral lycopene Oral lycopene in the dose of 8 mg/day is
beneficial in the treatment of oral leukoplakia.
Surgical line of treatment If the lesion is very small, it
should be excised as a part of investigation and as a treatment option.
Any leukoplakia could transform into a carcinoma,
even those which did not show epithelial dysplasia initially (or in which dysplasia happened to be absent from
the biopsy taken). The main problem is that the malignant
transformation cannot be reliably predicted yet. Regular
check-up of these patients is essential both in treated and
untreated patients.
146
Lichen Planus
The word lichen planus is derived from the Greek literature; lichen meaning tree moss and planus refers to flat.
In 1869 Erasmus Wilson, a dermatologist first reported the
condition. Oral lesions of lichen planus were first described
by Thiebergie. Wickham (1895) described the characteristic appearance of whitish striae and punctuations that
develop atop the flat-surfaced papules.
Definitions
Oral lichen planus (OLP) is a common chronic immunological inflammatory mucocutaneous disorder that varies
in appearance from keratotic (reticular or plaque like) to
erythematous and ulcerative.
Eisen (2005) dened OLP as a relatively common chronic
inammatory disorder affecting the stratied squamous
epithelia. It is a skin disease common with in the oral cavity, where it appears as either white reticular, plaque, or
erosive lesions with a prominent T-lymphocyte response
in the immediate underlying connective tissue.
Etiopathogenesis
In spite of extensive research, exact etiology is still unknown.
The most accepted and current data suggest that OLP is
T cell mediated disorder in which there is production of
cytokines which leads to apoptosis. Autocytotoxic CD8 and
T cells trigger apoptosis of oral epithelial cells (Eversole,
1997; Porter et al, 1997). The immune system is triggered
due to the interactions among genetic, environmental, and
lifestyle factors.
Other possible theories include the genetic background,
where the weak association between HLA antigen and
lichen planus was found by Powell et al (1986) and
Roston (1994). Dental materials and infectious agents like
Gram negative aerobic bacillus, spirochetes and increased
prevalence of Candida species were suggested by Simon
and Hornstein (1980). Vincent et al (1990), Soto Araya
et al (2004) reported the strong association of psychological factors like higher level of anxiety, greater depression,
and psychic disorders in patients with erosive lichen
planus.
Clinical features
Lichen planus commonly affects 12% of the general population, prevalence rate being 0.14%. Forty percent lesions
occur on both oral and cutaneous surfaces, 35% occur on
cutaneous surfaces alone, and 25% occur on oral mucosa
alone.
Lichen planus commonly affects the adults. However
it has also been observed in children as recently reported
by Sharma (1999). The age range usually varies from 40 to
70 years.
Figure 16
A
Violaceous papular lesions of cutaneous lichen planus on the upper and lower extremity. Courtesy: Dr Ashok
Figure 17
A
(A) Reticular lichen planus on the buccal mucosa. (B) Reticular lichen planus on the buccal mucosa.
(C) Lichen planus on the palate. (D) Reticular lichen planus on the labial mucosa of the upper lip.
Courtesy: Dr Ashok
Figure 18
Figure 19
Figure 20
A
differentiated from other forms like pemphigus, pemphigoid and linear IgA disease.
Erythematous lesions of OLP can be excluded from
atrophic candidiasis by the presence of whitish striations
at the periphery.
Investigations
Diagnosis is generally achieved by the characteristic clinical presentation and the complete history and the extraoral
manifestations. Sometimes biopsy may be complementary.
Gingival lichen planus is often difficult to diagnose and
149
Figure 21
A
(A) Diffuse red patches surrounded by white striae in atrophic lichen planus involving the buccal mucosa.
(B) Diffuse erythematous areas in atrophic lichen planus involving the tongue. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
Figure 22
Prognosis
Cutaneous lesions are self-limiting and pigmentation may
fade after few years or remain permanent. Complete remission
occurs in 70% of cases after 1 year. Oral lesions are chronic,
rarely undergo spontaneous remission, furthermore, erosive
oral lesions are difficult to palliate. The spontaneous remission of OLP is much less than 5% of patients over 7.5 years
follow up. The reticular form of LP has best prognosis because
spontaneous remission occurs in 40% of cases. The erosive
form of the lesion can persist for 1520 years.
Management (Flowchart 2)
Treatment should be considered after the evaluation of
clinical type, associated symptoms, and age. Reticular
lesions are asymptomatic and require no therapy but only
Flowchart 2
Oral lichen planus
Symptomatic
Relief in
symptoms
Asymptomatic
No relief in
symptoms
Regular follow-up
once in 3 months
New cases
Recalcitrant cases
Intralesional steroids:
Injection triamcinolone 0.5 ml
(Amcort) with once/week
for 4 weeks
Immunomodulation:
chloroquine (Lariago, Laquin)
250 mg b.i.d. for 3 months
Immunosuppression:
Azathioprine (Imuzat, Azoran)
1 mg/kg/day (50 100 mg) for
one month. Dosage can be
increased up to 2 mg/kg/day
and should not be given
more than 3 months
No relief in
symptoms
Combination therapy (systemic + topical)
1. Tab prednisolone 10 mg (Wysolone, Emsolone)
t.i.d. for 2 weeks; 10 mg b.i.d. for 1 week; 10 mg o.d.
for 1 week; 5 mg o.d. for 1 week & 5 mg once in
2 days (thrice)
2. Topical triamcinolone acetonide 0.1% (Tess gel)
t.i.d./day till symptoms improve or cyclosporine
solution (Sandimmune) as mouthrinse twice/day
for 15 days
PUVA therapy
(Psoralen ultraviolet A rays)
Note: All available medications have been enlisted. however, the physician should
choose the appropriate mode of treatment/drug based on the clinical situations
Figure 23
A
(A) Erythematous area on the buccal mucosa in relation to an amalgam restoration. (B) Lichenoid reaction on the buccal
mucosa in relation to a tooth that was previously restored with silver amalgam restoration. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
anti-inflammatory drugs, oral hypoglycemic agents, uricosuric agents. Lichenoid reaction may develop after months
or even years after taking the drug.
Dental materials like amalgam compounds, cobalt,
gold, acrylic and casting alloys have been known to cause
lichenoid reaction.
Some authors report that a low frequency of sensitization to mercury and no benecial effects from the removal
of silver amalgam llings, whereas others suggest that
sensitization to mercury is an important cause. In cases
where patch test negative patients improve with amalgam,
mercury may be acting as an irritant in the pathogenesis
of oral lichenoid reaction. Patch testing and biopsies however cannot accurately predict the response to removal of
amalgam llings with those of other material.
Betel quid lichenoid lesion
A quid-induced lichenoid oral lesion has been reported
among betel quid users. It resembles OLP but there are
specific differences. It is characterized by the presence of
fine, white, wavy parallel lines that do not overlap or crisscross, are non-elevated, and in some instances radiate from
a central erythematous area. The lesion generally occurs at
the site of placement of the quid. These lesions may regress
with decrease in frequency, duration, or change in site of
placement of the quid. There may be complete regression
of the lesion when the habit is given up.
Candidiasis
Candidiasis is a disease caused by infection with a yeast
like fungus, Candida (Monilia) albicans, although other
154
Xerostomia
Antibiotic therapy
Poor oral or denture hygiene
Malnutrition/gastrointestinal malabsorption
Iron, folic acid, or vitamin deficiencies
Acidic saliva/carbohydrate-rich diets
Heavy smoking
Oral epithelial dysplasia
Clinical presentation
Acute pseudomembranous candidiasis Pseudomembranous candidiasis is the most common form of oral candidiasis. The most common sites include buccal mucosa,
dorsal tongue and palate. It usually follows antibiotic
therapy or immunosuppression. A burning sensation usually precedes the appearance of as soft, creamy white to
yellow, elevated plaques, that are easily wiped off from the
affected oral tissues and leave an erythematous, eroded, or
ulcerated surface which may be tender (Figure 24). Thrush
may be seen in neonates and among terminally ill patients,
particularly in association with serious underlying conditions such as leukemia and other malignancies and in HIV
disease. A possible complication of oropharyngeal thrush
is the involvement of the adjacent mucosa, particularly
those of the upper respiratory tract and the esophagus. The
combination of oral and esophageal candidiasis is particularly prevalent in HIV infected patients.
Any mucosal surface may be involved and erythematous
or white areas often develop beneath the partial or complete
dentures. The lesions may involve the entire oral mucosa
or may relatively localized areas where normal cleansing is
poor. A prodromal symptom of rapid onset of a bad taste
and the loss of taste discrimination is described in adults.
Differential diagnosis Differential diagnosis of thrush
include food debris, habitual cheek biting, burns and
rarely, a genetically determined epithelial abnormality like
white sponge nevus.
Chronic hyperplastic candidiasis (candida leukoplakia)
Hyperplastic candidiasis is seen as chronic, discrete raised
Figure 24
Figure 25
Figure 26
tongue and other mucosal surfaces. The most common etiology is poor denture hygiene, and/or continuous denture
insertion, but it may also be caused by immunosuppression, xerostomia, or antibiotic therapy.
The most common appearance is that of a red patch or
velvet textured plaque. When atrophic candidiasis occurs on
the hard palate in association with a denture, it is frequently
associated with papillary hyperplasia. Patient may complain
of a burning sensation associated with this type of candidiasis; 1565% of cases are usually associated with angular cheilitis. And lesions of chronic atrophic candidiasis
have also been frequently reported in HIV-positive and
AIDS patients. Three progressive clinical stages of denture
sore mouth have been described in the literature.
Median rhomboid glossitis Median rhomboid glossitis is
a form of chronic atrophic candidiasis characterized by an
asymptomatic, elongated, erythematous patch of atrophic
mucosa of the posterior mid-dorsal surface of the tongue
due to a chronic Candida infection (Figure 27). In the past,
median rhomboid glossitis was thought to be a developmental defect resulting from a failure of the tuberculum
impart to retract before fusion of the lateral processes of
the tongue.
A concurrent kissing lesion of the palate is sometimes
noted (Figure 28). Specic predisposing etiologic factor(s)
for median rhomboid glossitis have not been clearly
established.
Angular cheilitis (perleche) Clinical appearance is that
of red, eroded, fissured lesions which occur bilaterally in
the commissures of the lips and are frequently irritating
and painful (Figure 29). The most common etiology is loss of
vertical occlusal dimension, but it may also be associated
with immunosuppression.
155
Figure 27
Figure 28
Figure 29
immune defects, in which there is persistent mucocutaneous candidiasis that responds poorly to topical antifungal
therapy. The main types of this rare disorders include
familial CMC, diffuse CMC, candidiasis endocrinopathy
syndrome, candidiasis thymoma syndrome.
Role of candida in oral carcinogenesis
Flowchart 3
Oral candidiasis
Systemic candidiasis
Physician opinion
sought
Parenteral route
Note: All available medications have been enlisted. However, the physician should
choose the appropriate mode of treatment/drug based on the clinical situations
Management of candidiasis
158
Complementary and alternative medicine Garlic capsules. Garlic may have antifungal and antibacterial properties. One study found that ajoene, a compound obtained
from garlic, was as effective in treating the fungus that
causes athletes foot.
Caprylic acid capsules. This fatty acid, derived from
coconut oil, has been shown to have antifungal properties.
These are generally safe, but should not be used in patients
with ulcerative colitis.
They found that chewing betel nut releases copper into the
saliva that stimulates lysyl oxidase enzyme lead to brosis.
b.
c.
d.
Collagen is the major structural component of the connective tissues and its composition within each tissue needs to
be maintained for proper tissue integrity. The synthesis of
collagen is influenced by a variety of mediators, including
growth factor, hormones, cytokines and lymphokines. A
prominent mediator is transforming growth factor-beta
(TGF-). The growth factor has also been implicated in the
development of many fibrotic diseases. It causes the deposition of extracellular matrix by increasing the synthesis
of matrix proteins like collagen and decreasing its degradation by stimulating various inhibitory mechanisms. So
transforming growth factor beta signaling pathway might
be critical for pathogenesis of OSMF.
Figure 30
Clinical features
Oral submucous fibrosis is very commonly seen among
the Indians. Indians who have settled in other countries
and to lesser extent in other Asiatic people. Several
Europeans in Europe, India and Africa have also been
mentioned as victims of the disease. Epidemiological surveys of Indian population have revealed that incidence
varied in the range of 0.040.4% and in urban population
0.181.2%. Overall prevalence of up to 0.4% in Kerala. The
common age of occurrence varies from 1262 years with
the mean age being 40 years but there are reports of OSMF
even in as younger as 4-year old. There is female predilection, with the ratio being 3:2.
First and the foremost feature of OSMF is burning sensation and pallor or blanching of oral mucosa. Intraoral
sites of involvement include the buccal mucosa (Figure 30),
retromolar area, followed by soft palate, palatal fauces,
uvula, tongue (Figure 31) and labial mucosa (Figure 32A, B).
There may be stiff and small tongue, blanched and leathery oor of the mouth (Figure 33), brotic and depigmented gingiva, rubbery soft palate with decreased
mobility and blanched and atrophic tonsils, and shrunken
bud like uvula. Mouth opening may become progressively
reduced (Figure 34).
Other symptoms include increased salivation, change of
gustatory sensation, hearing loss due to stenosis of the
Eustachian tubes, dryness of the mouth, nasal tonality to
the voice. Dysphagia to solids (if the esophagus is involved),
impaired mouth movements (e.g. eating, whistling, blowing, sucking) (Figure 35).
Figure 31
Staging of OSMF
Stage 1: Early OSMF
a.
160
Mild blanching
Figure 32
B
Pallor of the upper and lower labial mucosa in OSMF. Courtesy: Dr Ashok
Figure 33
Figure 34
c.
d.
e.
Figure 35
Unilateral posterior cheek involvement with only ipsilateral involvement of the faucial pillars and soft palate and
opening reduced to 1518 mm.
Group 4: Advanced cases
Stiffness/inelasticity of oral mucosa
Trismus
Mouth opening 215 mm (interincisal opening)
Fauces thickened, shortened and firm on palpation
Uvula was seen to be involved, shrunken, small, and
fibrous band
Tongue movement restricted
Papillary atrophy (diffuse)
162
Figure 36
melanocytes, a feature which explains the clinically observable loss of pigment. Absence of fibroblasts within the
hyalinized zones, total loss of epithelial rete pegs, and
extensive degeneration of muscle fibers.
Investigations
Diagnosis of the disease is by clinical findings and confirmed by incisional biopsy. Many investigations have been
suggested by various authors, that include hematological,
serological, immunological and biochemical factors. Other
laboratory ndings include a raised ESR, slight eosinophilia, microcytosis and hyperchromic indicative of anemia.
Cytologic smears may be performed.
A neural network-based oral precancer stage detection
method has been proposed. This new technique uses wavelet coefcients from transmission electron micrography
images of subepithelial brillar collagen in normal oral
submucosa and in OSF tissues. These wavelet coefcients
are used to choose the feature vector, which, in turn, can be
used to train an articial neural network. This trained network is able to classify normal and oral precancer stages
(less advanced and advanced) after obtaining the image as
an input. It may be used as an adjunct to hematoxylin and
eosin histologic evaluations in the near future.
Differential diagnosis
Khanna and Andrade (1995) have classified the histological findings of OSMF into four groups.
Treatment (Flowchart 4)
Since the exact etiology is unknown, various treatment
modalities have been tried from time to time. The treatments of the condition include avoidance of habits but
there is no reversal of fibrosis.
Management includes the following medications:
Corticosteroids These agents can be used in pharmacologic doses for their anti-inflammatory and immunosuppressant properties and their effects on blood and lymphatic
systems in the palliative treatment. In patients with moderate OSMF, weekly submucosal intralesional injections or
topical application of steroids may help prevent further
damage.
163
Flowchart 4
OSMF
Stage I
Stage IV
Systemic:
1. Iron supplements (Dexorange Syrup, Haemup Syrup/Capsules)
2. Antioxidant capsules b.i.d. for 3 months (Antoxid, Altomin Xl, Lynet, Revup, Oxyace, Oxidix)
Topical:
1. Benzydamine 0.15% mouth
rinse (Tantum)
2. Triamcinolone gel (Tess) or
crushed dexamethasone
tablets in 20 ml of water &
use as mouthrinse
Immunomodulators:
Levamisole 150 mg o.d. for
3 days twice in a month
for 3 months (Vermisol,
Levazole)
Intralesional injections:
1. Dexamethasone +
Hyaluronidase 1,500 IU
(Hylase) + Lignocaine
2 ml; multiple site
injections once/week
for 6 weeks
2. Betamethasone +
Hyaluronidase 1,500 IU +
Lignocaine + Placentrex
= 3 ml multiple site
injections once/week
for 6 weeks
Newer drugs:
1. Pentoxifylline 400 mg b.i.d. for 15 days
(Flexital, Flowpent)
2. Interferon gamma injections
No medicinal treatment
can be given
1. Surgical relieving of
fibrous bands with
buccal pad of fat
covering the wound
2. Laser surgery
Physiotherapy:
1. Mouth opening exercises
2. Mouth opening appliance
3. Ice cream stick
4. Ultrasound
Note: All available medications have been enlisted. However, the physician should
choose the appropriate mode of treatment/drug based on the clinical situations
164
Figure 37
Psoriasis
Description is given in Chapter 9 (Dermatological
Diseases).
165
Figure 38
Dyskeratosis Congenita
Clinical features
Figure 39
Figure 40
Figure 41
The most common site of occurrence of the erythematous macules are on the anterior and lateral borders of
the tongue, oor of the mouth, posterior palate, buccal
mucosa, and the mucosal surface of lips. Clinical congurations of these depend on the offending agent. This could
be either elicited by the history or by clinical examination.
The caustic drugs (aspirin) or hot foods or beverages
result in the coagulation necrosis of the supercial tissue
and appear as whitish scrapable membrane over an erythematous base.
Erythematous macules at the junction of hard and soft
palate should be differentiated from the purpuric macule
of oral sex, palatal bruising because of severe cough or
severe vomiting, allergic manifestations, macular hemangioma, atrophic candidiasis, infectious mononucleosis and
herpangina.
The lesions usually regress after the removal of the
causative factors. In case of multiple numbers, investigations are needed to rule out the underlying hemostatic
disorders.
Reddish ulcers or ulcers with red halo Ulcerative conditions like recurrent herpes and recurrent aphthous stomatitis are first manifested as erythematous macules.
Deficiency States
Soft tissue odontogenic infection (cellulitis) Odontogenic infections may originate in canals and periapex of
the teeth, gingival and periodontal pockets, and the gingival operculum over an erupting tooth and may spread to
the surrounding soft tissues like oral mucosa appear red,
swollen and tender to palpate.
Certain deficiency states can produce a glossitis of a completely bald or patchy bald tongue. These include iron
deficiency anemias, pernicious anemia, Plummer-Vinson
syndrome; sprue and vitamin B complex deficiencies, especially those of thiamine, riboflavin, nicotinic acid, pyridoxine, pantothenic acid, and vitamin B12.
Clinical features
Macular hemangiomas and telangiectasias Red macular hemangiomas occur as both syndromic and non-syndromes associated are readily differentiated from
erythemas by the history of long duration, non-tenderness, absence of inflammatory components, characteristic
emptying of the lesions.
Polycythemia Polycythemia also called erythremia is a
chronic and sustained elevation of erythrocytes and level
of hemoglobin. Primary polycythemia is a neoplastic condition of the hematopoietic system. Secondary polycythemia
results from stimulation of bone marrow at high altitudes
or by chronic pulmonary diseases like emphysema. In this
condition the entire oral mucosa appears deep red and
gingival and soft tissues easily bleed and seen as multiple
petechiae over the palate. Laboratory investigations
including elevated levels of erythrocyte count, raised hemoglobin concentration, hematocrit values quickly establish
the diagnosis.
168
Lupus erythematosus Lupus erythematosus is a connective tissue disease of unknown cause in which antibodies
to nuclear constituents are produced to result in the involvement of various organs. Two forms of the disease include
discoid and systemic lupus erythematosus. In Schiodts
study of 32 patients of lupus erythematosus with oral lesions,
early lesions were characterized by erythema without the
striae.
Foliate Papillitis
Sometimes enlarged foliate papillae appear red in color
due to inflammatory enlargement of the lymphoid tissue
or due to upper respiratory tract infections or mechanical
irritation and may be mistaken for erythroplakic lesions
on the tongue.
Erythroplakia
Erythroplakia is a precancerous lesion occurring in the
oral cavity, The term erythroplakia (erythroplasia) was
coined to describe red lesions of the oral mucosa in contrast to oral leukoplakia.
The term erythroplasia was originally used by Queyrat
to describe a red, precancerous lesion of the penis. The term
erythroplakia is used for a clinically and histopathologically
In a house-to-house survey in Burma among 6,000 villagers over the age of 15 years, ve cases of oral erythroplakia were diagnosed, with a prevalence of 0.83%.
Feller et al from South Africa studied 138 cases of oral
precancerous lesions, of which eight were oral erythroplakia.
A recently published case-control study from Kerala,
India, included 100 cases of erythroplakia among 47,773
controls, with a prevalence of 0.2%.
With these few data available it was observed that presently erythroplakia has a range of prevalence between
0.02% and 0.83%.
Classification
Shear suggested a classification of erythroplakia in 1972. He
differentiated between clinical and microscopic variations
and neoplastic from inflammatory changes.
Clinical variations
Homogeneous erythroplakia
Erythroplakia interspersed with patches of leukoplakia
Granular or speckled erythroplakia (embracing the
lesion described as speckled leukoplakia)
Microscopic variations
Neoplastic
Squamous carcinoma
Carcinoma in situ (intraepithelial carcinoma) and
less severe forms of epithelial atypia
Inflammatory
Candida albicans infections (including denture stomatitis)
Tuberculosis
Histoplasmosis
Miscellaneous specific, non-specific and nondiagnosable lesions
Etiopathogenesis
While erythroplakia does not seem to have a known geographic incidence, studies from India have shown that
erythroplakia may be associated with tobacco smoking
and chewing habits and that the risk to develop erythroplakia was strongly associated with these.
Etiology and pathogenesis of erythroplakia are poorly
understood. Predisposing factors are widely unknown, but
it was suggested that tobacco and alcohol use are probably
involved in most cases.
Reports of large case-control study in Kerala, India,
shed more light on some of the factors involved in the
etiology of erythroplakia. One of these studies evaluated
the risk of erythroplakia in relation to chewing tobacco,
smoking, alcohol drinking, body mass index (BMI), and
vegetable, fruit, and vitamin/iron intake. It was concluded
that tobacco chewing and alcohol drinking are strong risk
factors for erythroplakia in the Indian population.
169
Figure 42
C.
II.
A.
B.
C.
D.
E.
F.
G.
H.
Chronic cutaneous LE
1. Classic discoid LE
a. Localized DLE
b. Generalized DLE
2. Hypertropic/verrucous DLE
3. Lupus profundus
4. Mucosal DLE
a. Oral DLE
b. Conjunctival DLE
5. Chilblain DLE
6. Lichenoid DLE
LENon-specific skin disease
Cutaneous vascular disease
1. Vasculitis
2. Vasculopathy
3. Livedo reticularis
4. Thrombophlebitis
5. Raynauds phenomenon
Non-scarring alopecia
Sclerodactyly
Rheumatoid nodules
Calcinosis cutis
Urticaria
Erythema multiforme
Lichen planus
Early lesionsmucosal hemorrhage, erosion, superficial erythematous patches with dilated blood vessels
on the borders. The center is depressed or superficially
ulcerated.
Chronic lesionscentral atrophic areas with small white
dots, surrounded by a keratinized border composed of
radiating white striae.
Different types of DLE
2.
3.
4.
Laboratory findings
The laboratory findings for DLE are not specific. Patients
can present with anemia, leukopenia, thrombocytopenia,
elevated ESR levels, elevated serum globulin levels, high
IgG levels, presence of antithyroid antibodies and reduced
T-cell counts.
Urine examination and blood urea nitrogen has to be
done to rule out SLE and to know patients renal function.
Antinuclear antibodies are rarely present.
172
5.
Systemic lupus erythematosus (SLE): Systemic manifestations are present, LE cells are seen and antinuclear antibodies are present.
Polymorphous light eruption (PLE): Absence of antinuclear factor from the serum and of dermal-epidermal
immunoglobulin deposits.
Lupus vulgaris: Lesions usually occur at an early age,
and are rarely symmetrical, may be ulcerated and
usually show characteristic apple jelly nodules.
Lichen planus: The presence of concentrations of
lymphocytes in the immediate underlying lamina propria, deep focal accumulations of lymphocytes with
germinal centers and perivascular infiltrates of lymphocytes is helpful in differentiating lesions of LE and
those of LP. In more chronic lesions the presence of
hyperorthokeratosis and surface depressions containing keratin (keratin plugging) suggests LE than LP.
Seborrheic dermatitis, actinic keratosis and drug
eruptions.
Prognosis
The untreated skin lesions tend to be persistent, usually
heals with scarring. Less than 5% of the cases may convert
into SLE. Squamous cell and less commonly basal cell carcinomas occasionally occur in scars of DLE, particularly
on the scalp, ears, lips and nose.
Management
General measures Patient can be advised against excessive exposure to sunlight, UV light and heat. They can
be instructed to use umbrellas or broad brimmed hats.
Sunscreen creams or lotions can be used regularly.
Topical therapy 0.025% fluocinolone cream or 0.1% triamcinolone acetonide cream has shown to be effective.
Intralesional corticosteroid injections (triamcinolone acetonide 510 mg/ml) at 6 weekly interval are helpful in
resistant cases.
Oral therapy Oral prednisolone 0.5 mg/kg rapidly tapered
over 6 weeks or hydroxychloroquine, initially 200 mg
twice daily, reducing to 200 mg/day after response.
Alternatively chloroquine sulfate 200 mg twice daily
can be used. Other drugs that have been used include
173
CHAPTER
Vesiculobullous Disorders
Nagamani Narayana, Ravikiran Ongole
Pemphigus Vulgaris
Paraneoplastic Pemphigus (Paraneoplastic
Autoimmune Multiorgan Syndrome)
Bullous Pemphigoid
Mucous Membrane Pemphigoid or Cicatricial
Pemphigoid
Erythema Multiforme
Recurrent Erythema Multiforme
Dermatitis Herpetiformis
Bullous Impetigo
Epidermolysis Bullosa
Pemphigus
Herpangina
CLASSIFICATION OF VESICULOBULLOUS
LESIONS
I. Acute and chronic vesiculobullous lesions
(Table 1)
II. Based on the clinical presentation
1.
174
Predominantly vesicular
HSV infection
Varicella infection
Hand, foot and mouth disease
2.
Herpangina
Dermatitis herpetiformis
Predominantly bullous
Pemphigus vulgaris
Bullous pemphigoid
Benign mucous membrane pemphigoid
Bullous lichen planus
Erythema multiforme
StevensJohnson syndrome
Bullous impetigo
Epidermolysis bullosa
Linear IgA disease
Table 2
Name of virus
AcuteVB lesions
ChronicVB lesions
Duration
Short
Long
Age
Young
Middle agedolder
Etiology
Autoimmune
Examples
Pemphigus
Chicken pox
Bullous pemphigoid
Herpes zoster
Cicatricial pemphigoid
Herpangina
Erythema multiforme
Site of latency
CMV
EBV
HHV-6, HHV-7
CD4 lymphocytes
HHV-8
Epidermolysis bullosa
Mucosal erythema multiforme
Subepithelial vesiculobullous lesions
Bullous pemphigoid
Cicatricial pemphigoid
Epidermolysis Bullosa
Dermal Erythema multiforme
Dermatitis herpetiformis
Linear IgA disease
Infectious VB lesions
Herpes simplex infections
Varicella infections
Herpangina
Hand, foot and mouth disease
Non-infectious VB lesions
Pemphigus
Paraneoplastic pemphigus
Bullous pemphigoid
Cicatricial pemphigoid
Erythema multiforme
Dermatitis herpetiformis
Epidermolysis bullosa acquisita
Linear IgA disease
Clinical features
Primary HSV infection Only 1% of the population in
USA exhibit all signs and symptoms of primary infection.
It usually affects young children and adolescents, and occasionally young adults. The primary HSV infection seldom
occurs in the first 6 months of life as the infant is protected
by the maternal antibodies that are still circulating in the
newborn.
Primary herpetic gingivostomatitis is characterized by
fever, malaise, anorexia, irritability and regional lymphadenopathy (especially, submandibular and supercial cervical group of nodes are involved). Subsequently the mouth
becomes sore and the individual may complain of burning
sensation in the mouth and difculty in swallowing. Apart
from the gingiva, the buccal mucosa, palate, tongue, tonsillar and pharyngeal region may be affected.
The gingiva is erythematous, boggy and bleeds spontaneously or on the slightest of provocation. As the disease
advances multiple tiny to moderately large (few mm to
almost a centimeter in size) yellow colored vesicles develop
that rupture to form shallow painful ulcers that are usually
covered by a grayish colored membrane. These ulcers are
bound by an erythematous halo.
Generally in about 2 weeks the lesion heals without
scarring. Immediately after the resolution of the primary
lesion, the virus travels along the nerve pathway and lies
dormant in the nerve ganglia regional to the site of the
primary infection. Within the regional ganglion the virus
incorporates its DNA into the hosts DNA thereby establishing the beginning of a life-long afiction. The trigeminal ganglion is usually the site for dormancy for HSV-1
and HSV-2.
Herpetic Labialis
This is the most common secondary herpetic lesion,
usually following exposure to UV light or extreme cold.
Figure 1
Figure 3
Figure 2
Table 3
Immunosuppressed
Treatment
The aims of managing HSV infections are to inhibit autoinoculation and transmission. The management is targeted
at symptomatic relief. Topical and systemic antivirals like
acyclovir can be used (Table 3).
To be effective all treatment should be started within
72 hours of initial disease presentation. Patient compliance is shown to be better with fewer and shorter doses
(Whitley, 2006).
Prognosis
The main question arises should you treat these patients
when they have recurrent lesions. Universal precautions
including use of gloves enable us to do proceed with treatment. If the lesions are oozing and will transfer virus to
other sites and patient has no emergency it is advisable to
postpone treatment. If in an emergency application of
rubber dam may help in decreasing transmission of virus
to other sites.
Herpes simplex virus infections are described in detail
in Chapter 4 on Bacterial, Viral and Fungal Infections.
Figure 4
Figure 5
Differential diagnosis
Recurrent herpetic lesions and aphthous ulcers should be
considered in the differential diagnosis of intraoral lesions.
Treatment and prognosis
In healthy patients if the diagnosis occurs within 72 hours
of initiation of the disease a course of acyclovir or valacyclovir can be administered. If the patient is seen later during the course of the disease symptomatic relief in the form
of magic mouthwash can be prescribed. In immunosuppressed patients a prescription of acyclovir or valacyclovir
can be administered. It is some belief that a prescription for
antiviral and corticosteroid therapy prevents postherpetic
neuralgia.
The term hand, foot and mouth (HFM) disease was used for
the first time in 1960 following an outbreak of a relatively
mild febrile condition associated with papular and vesicular
lesions on the dermis and the oral cavity in Birmingham,
England.
Etiology
HERPANGINA
Herpangina was first described by John Zahorsky in 1920.
Herpangina is primarily caused by coxsackie virus A 1-10,
16, or 22. Other viruses that may cause herpangina are
coxsackie virus B 1-5, enterovirus 71 and echovirus 3, 6,
9, 11, 16, 17, 22, 25, and 30. Herpangina occurs in epidemics and usually occurs in the summer months. It usually
affects young children and adolescents. The mode of spread
is the fecal-oral route. The incubation period varies from
1 to 10 days and usually lasts 4 days.
Clinical features
Compared to hand, foot and mouth disease the disease
process is usually mild and patients may not exhibit overt
prodromal symptoms. Patients may present with mild rise
in temperature, malaise, anorexia, abdominal pain, sore
throat and cervical lymphadenopathy.
Oral manifestations
Oral manifestations are very typical of this condition.
Multiple minute vesicles and ulcers roughly measuring
12 mm in diameter are seen. The ulcers are surrounded by
an erythematous halo. These lesions are typically confined
to the posterior part of the oral cavity such as the faucial
pillars, tonsillar region, posterior pharyngeal region, soft
palate and uvula. Very rarely the posterior parts of the
buccal mucosa and tongue are involved. Patients may
complain of sore throat and difficulty in swallowing.
Management
Herpangina is a self-limiting disease. The fever associated
with this condition usually subsides within 34 days. The
oral ulcers heal within a week. The condition can be managed symptomatically with antipyretics and analgesic/
anesthetic mouthrinses.
DERMATITIS HERPETIFORMIS
Dermatitis herpetiformis (DH) is a rare, chronic, pruritic
immunobullous disorder of the skin that is associated with
gluten hypersensitivity. The condition is characterized by
the subepidermal deposition of IgA antibody against the
tissue transglutaminase. Antibody deposition leads to the
formation of dense neutrophilic abscesses, subepidermal
vesicles and bullae. Spurkland et al (1997) described the
genetic basis for DH. They showed that DH and celiac
disease are associated with class II HLA alleles A1*0501
and B1*02, which encode the HLA-DQ2 heterodimer.
Salmela et al (2001) showed that there was an upregulation of metalloelastase in both intestinal and lesional
skin.
Clinical features
Dermatitis herpetiformis usually affects individuals in the
2nd and 3rd decade of life. Dermal lesions are characterized by blisters over an erythematous or urticarial base.
The lesions are pruritic.
The common sites of involvement include the knees,
elbows, scalp, back and buttocks. It is believed that about
10% of the patients present with symptoms of celiac disease. DH is believed to be associated with some of the
autoimmune and connective tissue diseases such as Type I
diabetes mellitus, pernicious anemia, autoimmune thyroiditis, Sjgrens syndrome, lupus erythematosus, sarcoidosis,
scleroderma and psoriasis. DH has also been frequently associated with hypopigmentation.
Oral manifestations
Dermatitis herpetiformis can involve any part of the oral
mucosa. It manifests as multiple vesicles are bullae. These
fragile vesicles rupture immediately to form shallow painful ulcers.
Investigations
Histopathologically, presence of microabscesses in the
dermal papillae and subepithelial blister formation are
some of the findings associated with DH. The characteristic finding in DH is the presence of eosinophilia.
Direct immunouorescence test reveals the presence of
antibodies IgA, IgM and IgG at the junction of the dermis
and epidermis.
Dietrich et al (1999) demonstrated that the level of
immunoglobulin A autoantibodies to endomysium in patients
with DH was signicantly elevated.
Patients exhibit sensitivity to halogens.
Management
Dermatitis herpetiformis usually runs a prolonged course
and sometimes persists for life. Patients are advised to
avoid gluten in the diet. Dapsone is usually the drug of
choice. In the initial stages 50 mg/day is given and later
increased to about 300 mg/day as per the requirement.
Table 4
Antigens
Forms of pemphigus
Desmoglein 3
Predominantly mucosal
pemphigus vulgaris
Mucocutaneous pemphigus
vulgaris
Desmoglein 1
Pemphigus foliaceous
Paraneoplastic pemphigus
2.
3.
Pemphigus vulgaris
Pemphigus vegetansvariant of pemphigus vulgaris
characterized by excessive granulation and crusting
Pemphigus foliaceous
Idiopathic pemphigus foliaceous
Drug induced pemphigus foliaceous
Pemphigus erythematosus
Endemic pemphigus foliaceous/fogo selvagem
Paraneoplastic pemphigus-associated with neoplasms
Pemphigus vulgaris and vegetanspemphigus foliaceous
and erythematosus
Affects entire epithelium
Involves oral mucosaaffects upper prickle/spinous
layer of epithelium of skin
Pemphigus
Pemphigus is an autoimmune disease characterized by
intraepithelial blister formation due to a breakdown in intercellular adhesion. This breakdown process is referred to as
primary acantholysis. Prior to the advent of steroids, pemphigus was fatal due to fluid loss and electrolyte imbalance and bacterial infection. Pemphigus presents itself as
clinically characterized vesicles and bullae. The lesions are
Pemphigus Vulgaris
Epidemiology
Pemphigus vulgaris occurs equally in both sexes during the
4th and 5th decades. Studies have shown a slight increased
incidence in Ashkenazi Jews and individuals with HLA-DR,
HLA-A10, HLA-DRB1 phenotypes. Other autoimmune
181
Oral manifestations
Clinical features
Differential diagnosis
182
Investigations
Cytology Smears taken from freshly opened vesicles are
usually preferred. Tzanck cells can be seen. These are epithelial cells that are free in the vesicular space and are
Figure 6
A
(A) Shallow ulcers on the buccal mucosa in a patient with pemphigus vulgaris.
(B) Shallow ulcers on the palate in a patient with pemphigus vulgaris. Courtesy: Dr Sumanth
Figure 7
approached 90%. However in the recent times the mortality rate is said to be approximately 10%.
Topical therapy
Eroded and crusted, painful skin lesions and the associated foul odor can be effectively managed by bathing
the area with 0.01% potassium permanganate solution
or 0.5% silver nitrate solution.
Alternatively the raw surfaces can be sprayed with
corticosteroids or 2% procaine hydrochloride.
Chlorhexidine mouthrinses can be used to alleviate
discomfort and malodor.
183
Painful oral ulcerations can be managed by topical application of viscous xylocaine especially before food intake.
Systemic therapy
Corticosteroids: Systemic administration of corticosteroids comprises three phases:
Control phase: Characterized by an initial high dose
corticosteroid administration to the point of obvious clinical improvement. Therapy is initiated by
giving 60160 mg of prednisone daily. If there is no
response even after a week, the dosage is doubled.
When new lesions cease to form and old lesions
heal, the dosage is decreased slowly. Lever (1977)
suggests 180360 mg of prednisone daily for
610 weeks.
Consolidation phase: In this phase the dosage of
prednisone is reduced over a period of several
weeks. According to Arnold, once the control over
the disease is achieved, an attempt to decrease the
steroid dose by transferring the patient to intramuscular injections of triamcinolone acetonide is
highly advisable.
Maintenance phase: The corticosteroids are gradually tapered down to alternate day dose and ultimately stopped. However this reduction in dosage
is made possible by replacing steroids with immunosuppressive drugs. The dosage of immunosuppressive drugs is reduced to zero in several months.
Immunosuppressive agents: Azathioprine 100200 mg
per day in conjunction with prednisone 150200 mg
daily can be used. Fellner et al reported good results
by combining 200 mg of prednisone with 100200 mg
cyclophosphamide.
Other agents that have been used with mixed
results are dapsone, gold sodium thiomalate and
aurothioglucose.
Plasmapheresis: It is particularly useful in patients who
are refractory to corticosteroids. It involves removal of
the circulating antibodies.
Photopheresis: This modality of treatment was described
by Rook et al. It involves administration of 8-methoxypsoralen followed by exposure of peripheral blood to
ultraviolet radiation, causing photoinactivation of WBC.
Immunomodulators: Chaffins et al in 1993 reported
that drugs like levamisole (100 mg/week), combination
of nicotinamide and tetracycline and oral prostaglandins
are effective in the treatment of pemphigus.
2.
3.
4.
Paraneoplastic Pemphigus
(Paraneoplastic Autoimmune Multiorgan Syndrome)
Anhalt and coworkers in 1990 were the first to suggest the
term paraneoplastic pemphigus for a clinically and immunologically distinct condition. They reported five patients
with underlying neoplasms associated with painful mucosal
184
seen in pemphigus vulgaris, as well as basal cell vacuolation, lymphocytic exocytosis, and dyskeratotic keratinocytes typical of erythema multiforme.
Paraneoplastic pemphigus is distinguished from the
other forms of pemphigus as direct immunouorescence
reveals not only IgG and C3 deposits within the intercellular spaces but also along the basement membrane zone.
In the classic forms of pemphigus, indirect immunouorescence is positive only on stratied squamous epithelial substrates. However, in paraneoplastic pemphigus,
there is staining of other tissues, including the bladder, heart,
and liver. IgG autoantibodies are directed against desmoplakins I and II (components of the cytoplasmic plaque),
which are present in stratied squamous epithelium and
these other tissues.
Management and prognosis
There is little to offer in the treatment of paraneoplastic
pemphigus. If a benign tumor is resected, some patients
may go into remission. Unfortunately, the prognosis is
generally poor, and treatment is usually unsuccessful.
Immunosuppressive treatment and plasmapheresis have
not been effective; however, immunophoresis may be a
promising alternative.
Paraneoplastic pemphigus is a rapidly progressive bullous disease that is invariably fatal when associated with a
malignant tumor. When paraneoplastic pemphigus occurs
in the context of a benign neoplasm, the mucocutaneous
erosions will usually show gradual resolution after excision
of the tumor. It is important to remember that paraneoplastic pemphigus may precede the clinical appearance of
a neoplasm; therefore, it is mandatory that these patients
receive screening for neoplasms and regular follow-up care.
Other forms of pemphigus are described in Chapter 20,
Autoimmune Disorders.
Bullous Pemphigoid
Bullous pemphigoid (BP) was first described as a distinct
clinical entity by Lever in 1953. It is an autoimmune condition characterized by subepidermal blistering resulting
in large, tense bullae involving the skin and rarely the mucous
membrane. Autoantibodies are targeted at the components
of the basement membrane.
Pathophysiology
Kasperkiewicz et al (2007) reviewed the pathophysiology of
BP. They described that the autoimmune response in BP is
directed against two hemidesmosomal proteins within the
dermal-epidermal junction, namely BP180 and BP230 (also
known as BPAG1).
BP230 localizes intracellularly and associates with the
hemidesmosomal plaque. BP180 is a transmembrane
glycoprotein with an extracellular domain. Most of the
patients have autoantibodies binding to an immunodominant region of BP180, the non-collagenous 16A domain
(NC16A), which is located extracellularly close to the
transmembrane domain of the protein. Autoreactive T and
B cell responses to BP180 have also been found in patients
with BP.
Wohl and others (2008) studied the expression of vinculin in autoimmune cutaneous diseases. Vinculin is a cytoskeletal protein associated with cell to cell and cell to
matrix junctions, where it is thought to function as one of
several interacting proteins involved in anchoring F-actin
to the membrane.
On semiquantitative immunohistochemistry investigations they found that the expression and distribution of
vinculin are accentuated in patients with various skin
autoimmune diseases and appear to be stronger in diseases
involving the basement membrane.
Clinical features
Three distinct clinical types BP have been reported: the
commonly known adult form of BP and the relatively rare
forms of BP occurring at infancy and childhood.
The adult form of BP typically occurs between the 6th
and 8th decades of life. Males and females can be equally
affected. BP shows no ethnic or racial predilection. Dermal
lesions of BP are polymorphous in nature. Literature is
replete with reports of non-bullous forms of BP. They can
present as urticarial papules, plaques, vegetating forms,
nodular lesions, hyperkeratotic areas and erythematous and
eczematous lesions.
Initial lesions can usually present as urticarial eruptions that progress to bullae over weeks or months. The
bullae are pruritic, large and tense. However they generally persist longer due to their thick wall. Over a few days
the bullae rupture to form large and tender eroded regions.
These bullae can occur in any part of the body such as the
axillae, abdomen, legs, forearms and groin.
The bullae may contain clear uid or at occasions contain hemorrhagic content. The erosions heal without scar
formation.
Involvement of the mucous membrane is relatively
uncommon in BP compared to pemphigus vulgaris. The
oral cavity and genital and anal mucosa can be affected.
Oral bullae tend to rupture and form erosions or ulcers due
to the frequent micro trauma sustained during mastication.
Childhood BP though rare, is the most common IgG
mediated subepidermal bullous disease in children. It typically affects children below the age of 18 years and involves
the skin. The mucous membrane involvement is relatively
more common than the adult from of BP. The histopathologic
picture is similar to the adult form consisting of subepidermal bullae with variable amount of eosinophils and direct
immunouorescent studies show linear deposition of IgG
and/or C3 at the basement membrane zone.
185
Oral lesions can have two clinical presentationserosions on the non-keratinized mucosa/keratinized gingiva or desquamative gingivitis.
The common sites of involvement are the facial gingiva (64%), buccal mucosa (58%), palate (26%), edentulous alveolar ridge, especially under a prosthetic
appliance (16%), tongue (15%) and lower lip (15%).
Figure 8
Figure 10
Figure 9
Ocular findings
The classic histological feature of mucous membrane pemphigoid is separation of the epithelium from the underlying connective tissue due to subepithelial separation.
A mixed inflammatory infiltrate is present in the underlying submucosa (Figure 11). Direct immunofluorescence
demonstrates linear deposition of IgG and C3 at the basement membrane (Figures 12 and 13).
Differential diagnosis
Mucous membrane pemphigoid must be differentiated
from pemphigus vulgaris, BP, lupus erythematosus, lichen
planus and paraneoplastic pemphigus. Bullous pemphigoid
is similar to mucous membrane pemphigoid, but BP is commonly seen in older women with circulating antibodies to
BP protein 230 in addition to BP antigen 180.
Treatment and prognosis
There is no single treatment for mucous membrane pemphigoid, as it must be tailored to the individual patient similar to pemphigus vulgaris. If extensive lesions involving
the oral cavity are present, systemic prednisone may be
187
Figure 11
Figure 13
Figure 12
indicated. Normally a short course of prednisone is prescribed (40 mg per day for 7 days without tapering). Topical
steroids may be prescribed either alone or in addition
to systemic steroids, as ointments or oral rinse solutions.
188
Hebra used the term leichen ruber to describe lichen planus. However in 1869, Erasmus Wilson introduced the
term lichen planus (LP). It is believed that almost 0.5 to 1%
of the population is affected by lichen planus. Typically
lichen planus affecting the skin presents as violaceous,
polygonal flat topped papules, usually associated with
white striae on their surface.
Andreasen classied oral lichen planus into six types
namely reticular, plaque-like, erosive, papular, atrophic
and bullous lichen planus. It is believed that almost 25%
of the individuals present with oral manifestations alone
and about 50% of the patients with skin lesions have oral
ERYTHEMA MULTIFORME
Clinical features
Figure 14
190
Figure 15
Ng and others (2003) used nested PCR to detect HSVDNA in skin biopsies with histologically proven EM.
Their ndings showed that PCR was positive in 60% of
the patients with HSV-related recurrent EM and PCR
was positive in 50% of the patients with idiopathic
recurrent EM.
Differential diagnosis
Dermal lesions may mimic lesions of hypersensitivity
reactions, drug eruptions and urticarial lesions. Intraoral lesions may resemble recurrent aphthous stomatitis, contact stomatitis and ANUG when the gingiva is
involved. Other conditions that can be considered are
pemphigus and varicella infections.
Management
Patients with mild EM usually do not require any form
of treatment as the condition is self-limiting. Since EM
is usually associated with HSV, oral acyclovir will minimize the duration of the condition. Topical steroids will
provide symptomatic relief.
The incidence of recurrent EM drastically falls down
following treatment with valacyclovir (5001,000 mg/
day) or famciclovir (125250 mg/day).
Severe recurrent EM can be managed with immunosuppressive agents. Dapsone or antimalarials (hydroxychloroquine) are the primary drugs in the treatment of
recurrent EM. Azathioprine also helps in suppressing the
condition, however it has many side effects. Davis et al
(2002) showed that mycophenolate mofetil can be used
as an effective drug in managing recurrent EM.
Murata and Abe (2007) reported that the keratinocyte apoptosis is brought about by the interaction of the fas receptor
and fas ligand. They also emphasize the need to evaluate
other genetic factors that may predispose to TEN and SJS.
Lerner et al (2000) used reverse transcriptase-PCR and
immuniperoxidase staining to study the role of inducible
form of nitric oxide synthetase in generating large amounts
of nitric oxide. They proposed that a large burst of nitric
oxide in TEN and SJS may cause the epidermal apoptosis
and necrosis.
In their study, inducible nitric oxide synthase was detected
by reverse transcription polymerase chain reaction. Strong
staining for inducible nitric oxide synthase was observed
in inammatory cells in the lower epidermis and upper
dermis.
Drugs are considered to be the most common cause of
SJS/TEN. The drugs that commonly cause SJS/TEN are anticonvulsants, sulfonamides, non-steroidal anti-inammatory
drugs and antibiotics.
SJS/TEN is considered as a cytotoxic immune reaction
causing destruction of keratinocytes expressing drug-related
antigens. TNF-alpha derived from macrophages and keratinocytes may play an important role in the pathogenesis
by inducing apoptosis of epidermal cells or by attracting
cytotoxic effector cells, or both. Drug metabolites such as
hydroxylamines and arene oxides derived from sulfonamides and aromatic anticonvulsants respectively, bind to
cell constituents if they are not rapidly detoxied by epoxide hydrolase. These metabolites act as haptens and render
the keratinocytes antigenic by binding to them. A defect
in the detoxication system may be the cause of the drug
eruption.
Clinical features
Toxic epidermal necrolysis and StevensJohnson syndrome
are usually seen in adults. Both the conditions occur as a
single episode and associated with drug exposure.
Literature review reveals various age groups being
involved. The involvement of men and women also shows
inconsistency.
191
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
BULLOUS IMPETIGO
Impetigo is a bacterial infection that affects the superficial
areas of the skin. It commonly affects infants and young
children in the age group of 25 years. However it has
known to affect individuals of various age groups. It is
believed that impetigo is the most common skin disease to
affect children.
Donovan et al (1992) in their study to evaluate whether
bullous impetigo in homosexual men is a risk marker for
HIV-1 infection, concluded that bullous impetigo if seen
in an adult, it could prove to be a clinical indication that
a person is either infected with HIV-1 or is in close (possibly sexual) contact with a person with HIV-1 infection.
It is a highly contagious infection caused primarily by
Staphylococcus aureus. However some cases have been
reportedly caused by group A beta-hemolytic streptococcus (Streptococcus pyogenes) alone or in tandem with
Staphylococcus aureus. The mode of transmission is generally by direct contact.
Types
There are two forms of impetigo, namely, bullous impetigo
and non-bullous impetigo (impetigo contagiosa). The basic
difference between the two is that the non-bullous variety
represents the host response to the staphylococcal infection,
whereas in bullous impetigo the staphylococcal toxin is
responsible for the condition and not the host response.
Clinical features
Bullous impetigo is usually seen in infants. But it is seen
to affect children and adults likewise. Unlike the nonbullous form which may be caused by a combination of
staphylococcus and streptococcus. Bullous forms is always
caused by the toxins produced by Staphylococcus aureus.
Staphylococcus aureus, phage group II type 71 is the predominant causative organism. This strain of bacteria produces an exfoliatin toxin that causes subcorneal epidermal
EPIDERMOLYSIS BULLOSA
Epidermolysis bullosa (EB) comprises a group of inherited
mucocutaneous disorders characterized by blister formation due to a defect in collagen metabolism. These blisters
may arise spontaneously or as a result of mild trauma.
These inherited forms of epidermolysis bullosa should be
differentiated from epidermolysis bullosa acquisita which is
an autoimmune disorder. Some authors prefer to use the term
hereditary epidermolysis bullosa to refer to the forms that are
generally inherited. EB is a genodermatosis. The pattern of
inheritance may either be dominant or recessive. The recessive forms of EB are relatively more severe and aggressive.
Types of EB based on ultrastructural features
Jo-David Fine et al (2008) The classification of inherited
EB: Report of the Third International Consensus Meeting
on Diagnosis and Classification of EB
193
194
RDEB, pretibial
RDEB, pruriginosa
RDEB, centripetalis
RDEB, bullous dermolysis of the newborn
General clinical features and oral manifestations
Epidermolysis bullosa simplex The condition is characterized by the presence of the presence of multiple vesicles
or bullae at birth or some time after birth.
The common sites that are involved are the hand and
feet. Oral mucosa is seldom affected.
The condition is self-limiting. The vesicles heal in about
a week to 10 days time without the formation of scars.
Most of the children are free of this condition at puberty.
Junctional epidermolysis bullosa The junctional variety
of epidermolysis bullosa is a severe form that occurs at
birth and usually death ensues in the first 3 months of life.
In very severe forms the skin tends to shed increasing the
risk for superinfection and septicemia.
Children may present with hoarse cry, cough and breathing difculty. Apart from the skin, oral, ocular, pharyngeal
and genitor urinary mucosa may be affected.
The oral lesions manifest as bullae and erosions affecting
almost any part of the oral cavity. Infants may have difculty in feeding.
Dystrophic epidermolysis bullosa The dominant variety
usually occurs at birth and occasionally at adolescence. At
young age the condition has a generalized involvement.
As the age advances the condition tends to be confined to
a particular region.
Bullae are seen on the feet, ankles, knees and elbows.
Nails are typically dystrophic. Other features that may be
seen are palmoplantar hyperkeratosis and hypertrichosis.
The bullae rupture and heal with extensive scarring.
Milia (tiny keratin lled cysts) may be seen on the face.
Compared to the recessive form of the condition, the oral
involvement is limited. Occasionally bullae may be seen in
the oral cavity. Teeth are seldom affected.
The recessive form of the condition is usually seen at
birth or in neonates. Bullae are seen in the sites predisposed
to pressure or mild trauma such as the buttocks, knees,
elbows, feet and ngers. The bullae subsequently rupture to
reveal raw erosive areas. These heal by extensive scarring.
Scarring of the ngers may result in club like sts.
Like the dystrophic variety, nail may be absent altogether
or may be defective. Oral manifestations are commonly
seen. Bullae may be seen affecting any part of the oral
mucosa. With the rupture of the bullae, painful erosions are
seen which heal by scarring. Extensive scarring of the oropharyngeal and esophageal tissues may lead to hoarseness
of voice and difculty in feeding and swallowing. Dental
tissues are usually affected. Congenitally missing teeth,
malformed or hypoplastic teeth are usually seen.
195
CHAPTER
Stomatitis Medicamentosa
Stomatitis Venenata
Traumatic Ulcers
Erythema Multiforme
Chicken Pox
Herpes Zoster
Herpangina
Hand, Foot and Mouth Disease
Tuberculosis
Syphilis
Immunologic Disorders
Aphthous Ulcers
Dermatological Disorders
Pemphigus
Bullous Pemphigoid
Cicatricial Pemphigoid
Neoplastic Ulcers
Ulcerative lesions affecting the oral cavity can be categorized based on the etiology or based on the mode of onset
and clinical presentation.
196
Traumatic
Infectious (bacterial, viral and fungal infections)
Drug induced
Single ulcers
Traumatic ulcer
Tuberculous ulcer
Syphilitic ulcer
Histoplasmosis
Blastomycosis
Mucormycosis
Neoplastic ulcers
Multiple ulcers
Acute multiple ulcers
Herpes virus infections
Primary herpes simplex virus infections
Coxsackievirus infections
Varicella zoster virus infection
Erythema multiforme
Contact allergic stomatitis
Oral ulcers secondary to cancer chemotherapy
and/or radiotherapy
Acute necrotizing ulcerative gingivitis
Chronic multiple ulcers
Pemphigus
Subepithelial bullous dermatoses
Herpes simplex virus infection in immunosuppressed patients
Recurring oral ulcers
Recurrent aphthous stomatitis
Behets syndrome
Recurrent herpes simplex virus infection
Cyclic neutropenia
Chronic idiopathic neutropenia.
Flowcharts 1 and 2 summarize acute ulcers that are recurring in nature and those that occur as an isolated episode.
TRAUMATIC ULCERS
Traumatic injuries involving the oral cavity may lead to
the formation of surface ulcerations. Although the exact
incidence of these ulcerations is not known they are one
of the most common ulcers seen affecting the oral cavity.
Types of Trauma
Mechanical (sharp tooth, overextended denture, orthodontic brackets, toothbrush, ill-fitting dental prosthesis, etc.)
Chemical (aspirin, concentrated clove oil, sodium hypochlorite, hydrogen peroxide, root canal medicaments,
chemotherapy, etc.)
Thermal (extremely hot or cold insults such as hot
cheese, hot beverages, popsicle) (Figure 1)
Radiation (used in treatment of head and neck cancer)
Self-inflicted (self-harm)
Iatrogenic (injuries caused by high speed rotary instruments, cotton rolls, etc.)
Flowchart 1
Recurrent episodes
Systemic symptoms
Absent
Trauma
Present
IBD
Location
Keratinized
Cyclic neutropenia
Nonkeratinized
Sprue
Behcets
RAS
Culture
+
HSV
Atypical RAS
HIV
Cyclic neutropenia
HIV
FAPA
Magic
Flowchart 2
Single episodes
Infection
Drugs
Iatrogenic
Trauma
Radiation
Bacterial
Viral
Chemotherapy
ANUG
HSV
Syphilis
VZV
Gonorrhea
Coxsackie
Tuberculosis
Rubeola
Rhinoscleroma
EBV
Algorithm to evaluate acute oral ulcers that occur as an isolated episode. (Reprinted with minor modifications from
Bruce AJ (2003), Acute oral ulcers, Dermatologic Clinics, Vol. 21, p. 2, with permission from Elsevier)
Figure 1
Clinical features
In most cases, the source of the injury is identified. The
patients usual complaint is pain or a painful ulceration.
Individual lesions usually appear as shallow or deep ulcers
198
The diagnosis of traumatic ulcerations is based on the history of trauma or insult (hot/cold or radiation therapy) prior
to onset of ulcer. Mechanical trauma induced ulcers often
have linear configuration. The depth of ulcer depends on
Figure 2
Histologic features
Histopathologically an area of surface ulceration covered
by a fibrinopurulent membrane consisting of acute inflammatory cells intermixed with fibrin is seen. The stratified
squamous epithelium from the adjacent surface may be
hyperplastic and exhibit areas of reactive squamous atypia.
The ulcer bed is composed of a proliferation of granulation
tissue with areas of edema and an infiltrate of acute and
chronic inflammatory cells.
Management
Figure 3
Complications
nature of trauma. Generally the area surrounding the ulcer
is inflamed. A traumatic factor will often be evident in the
vicinity of the ulcer (e.g. sharp edge of tooth).
Differential diagnosis
Carcinomatous ulcer, recurrent aphthous ulcerations, ulcers
associated with deep fungal infections can be considered
in the differential diagnosis of traumatic ulcers.
Prognosis
The outcome of traumatic ulcerations is excellent, provided
the etiological factor is eliminated. Healing of the ulcerated
199
Table 1
Viral
Specific
Non-specific
Mycobacterium tuberculosis
Tuberculous ulcer
Tuberculosis cutis orificialis
Coxsackie virus
Herpangina
Hand, foot and mouth disease
Treponema pallidum
Chancre
Snail track ulcer
Gumma
EpsteinBarr virus
Infectious mononucleosis
Gonococci
Gonococcal stomatitis
Fungal
Histoplasmosis
Mucormycosis
Blastomycosis
Cryptococcosis
Coccidiodomycosis
Prodromal systemic symptoms (fever, malaise, myalgia) precede oral lesions by 23 days.
The skin, mucous membranes, eyes and central nervous system are the most commonly affected sites.
Multiple oral ulcers affecting all parts of the mouth.
Generalized erythema of gingiva usually associated
with multiple vesicles or ulcers (Figure 4).
Cervical lymphadenopathy occurs as a rule.
Food intake becomes difficult and dehydration may
ensue.
Self-limiting condition in normal children. However,
it may become disseminated in immunocompromised
children or adults.
Diagnosis
Primary herpetic gingivostomatitis is caused by herpes simplex virus (double-stranded DNA virus which is a member
of the human herpes virus family). Most orofacial and ocular infections are caused by HSV-1. Infections involving the
genitalia and the skin surface of the lower part of the body
are caused by HSV-2. It has been reported that HSV-2 has a
greater virulence. Almost 95% of the cases have a subclinical infection, only about 5% manifest symptoms. The infection confers resistance against another primary infection
for lifetime.
Clinical features
Treatment
Symptomatic management Ensure that the patient is
adequately hydrated and electrolyte balance is maintained.
Antipyretic and analgesic medication like acetaminophen, 500 mg given thrice daily is effective (3 times a day).
Figure 4
2.
Precipitating Factors
Clinical presentation
Patients present with cluster of tiny fluid filled vesicles (Figure 5AC) which rupture to form pinpoint
ulcers. These ulcers may coalesce to form larger areas
of ulceration.
Lesions may be preceded by burning, itching, tingling
sensation or pain in the region.
The lesions last for 57 days and subside and subsequently recur. Now the frequency of recurrence may
be varied.
Occasionally associated with fever and pharyngitis.
As a consequence to healing an area of pigmentation
is noticed at the site of the lesion (however this pigmented area is readily visualized only in fair skinned
individuals).
Diagnosis
Recurrent herpes infection can be identified by their typical location and clinical presentation. In addition, biopsy
and Tzanck smear can be performed.
Treatment
The management of recurrent lesions is generally symptomatic. Patients can be advised to stay indoors to minimize exposure to sunlight. Sunscreen lotion can be used
when venturing out. However in individuals where there is
an increased frequency of recurrence, acyclovir therapy
can be instituted as a prophylactic measure (400 mg twice
a day for 10 days).
Chicken Pox
1.
201
Figure 5
Figure 6
B
Vesicular lesions involving the face and neck in
chicken pox. Courtesy: Dr Ceena Denny
202
Figure 7
Management
Vaccination against varicella zoster should be given to
infants between 12th and 18th months of life. The management of the disease is mainly symptomatic. Acyclovir can
be administered in the early phases of the disease.
Herpes Zoster
Herpes zoster is caused by reactivation of varicella zoster
virus that is inactive in dorsal root or cranial nerve ganglion, after primary infection.
It is estimated that only in about 0.30.5% of the
population, the virus is reactivated after the primary
infection.
Clinical features
Herpangina
Herpangina is caused by coxsackie virus (A 1-10, 16, or 22).
It occurs generally in epidemics. It predominantly affects
posterior parts of oral cavity.
Clinical findings
Oral lesions
Topical application of anesthetics like viscous lidocaine and diphenhydramine may be used to manage
painful oral ulcers. Sucralfate suspension can also be
effectively used.
Fever and arthralgias can be treated using antipyretics
and analgesics.
Literature review reveals that there is a reported quicker
resolution of symptoms with the use of oral acyclovir.
A low intensity laser ablation of the oral ulcers is said
to shorten the duration of painful oral ulcers.
Prognosis
The prognosis of the condition is generally excellent
owing to the self limiting nature of the disease. However,
complications like meningoencephalitis, myocarditis, pulmonary edema, and death have been reported.
Figure 8
Table 2
Acute herpetic
gingivostomatitis
Viral etiology
Non-contagious
No viral antibodies
TUBERCULOSIS
Tuberculosis is a chronic infectious granulomatous bacterial disease generally acquired by inhaling droplets contaminated by Mycobacterium tuberculosis. However, it can
also be acquired by consuming unpasteurized cows milk
that is infected by Mycobacterium bovis or by other atypical
mycobacteria.
Oral manifestations
It is hypothesized that tubercle bacilli enter the oral
mucosa through a break in the mucosal surface. Abbot
et al in an independent study were able to isolate tubercle
bacilli from the mouthwashings of 44.9% of the individuals suffering from pulmonary tuberculosis. This study
underlines the importance of intact mucosal lining in providing protection against tuberculosis affecting the oral
cavity.
The oral manifestations of tuberculosis are rare. It is
estimated that approximately 1.4% of the patients suffering
from tuberculosis exhibit oral manifestations. The tuberculous lesions may present as ulcer, ssure, tuberculoma
or as a generalized enlargement of the tongue. In the initial
stages the lesion can be in the form of a nodule or opalescent vesicle which progresses to form an ulcer. The most
common oral manifestation is ulcer (Figure 9). Oral tuberculous lesions typically present with severe, unremitting
and progressive pain.
Sites
Soft tissues are more frequently affected than hard tissues.
The sites of involvement include the tongue, soft palate,
hard palate, gingiva, lips, floor of the mouth, vestibular
and retromolar regions and recent tooth extraction sockets. Maxilla and the mandible have also been affected and
referred to as tuberculous ostemyelitis. Mandible is more
commonly involved. Tuberculous osteomyelitis generally
205
Figure 9
Figure 10
Oral manifestations
occurs due to a hematogeneous spread of the bacilli or
through a direct extension through a fresh extraction
socket.
Typical lesion
The ulcer is irregular with ragged, undermined edges, minimal induration and yellowish granular base. The ulcer is
surrounded by sentinel tubercles. Primary oral tuberculous
lesions are usually associated with caseation of the regional
draining group of lymph nodes (Figure 10).
Differential diagnosis
Infected traumatic ulcer, major aphthous ulcer, syphilitic
ulcer, sarcoidosis, lymphogranuloma venereum, foreign
body granuloma, histoplasmosis and malignant ulcer can
be considered as differential diagnosis for tuberculous
ulcer.
Management
The pain associated with the ulcer can be treated symptomatically. Antitubercular therapy for 18 to 24 months
should be instituted.
SYPHILIS
Syphilis is a sexually transmitted disease that is caused by
Treponema pallidum.
206
Oral findings
Histoplasmosis
Mucormycosis
Aspergillosis
Aspergillosis is reportedly the second most commonly seen
fungal infection affecting the oral cavity after Candida.
Aspergillosis in humans is caused primarily by Aspergillus
fumigatus and A. niger. The spore is found in rotting vegetation. The transmission of fungal spores to the human
being is by inhalation.
It primarily affects the lungs. Aspergillosis has a tendency for a hematogeneous invasion causing thrombosis
and infarction of perivascular tissues.
Histoplasmosis is caused by Histoplasma capsulatum. Following inhalation of this dimorphic fungus it can cause an
acute pulmonary histoplasmosis or can assume a disseminated form in immunocompromised individuals.
The typical oral lesion, though very rare appears as an
erosion or ulcer commonly involving the tongue, palate
and buccal mucosa.
Cryptococcosis
Cryptococcosis is an opportunistic fungal infection caused
by Cryptococcus neoformans. It is usually present in bird
droppings, rotting wood and soil.
C. neoformans preferentially affects the meninges, basal
ganglia and cortical gray matter. Oral mucosal lesions appear
as supercial ulcers or as deep ulcers with indurated borders and rolled out edges.
blastomycosis has a localized form that affects the respiratory system and a disseminated form. However, unlike other
fungal infections immunosuppression does not increase the
risk of disseminated disease.
Blastomycosis is endemic to the Mississippi and Ohio
River basins in the United States and certain areas in Africa
and India.
Disseminated form of blastomycosis commonly involves
the skin, CNS and the genitourinary tract. Papulopustular
or verrucous, grayish lesions are seen on the skin surface.
Oral mucosa may exhibit ulcerations.
Paracoccidioidomycosis
The paracoccidioidomycosis is one of the most prevalent
deep systemic mycoses. It is more frequently observed
in patients from South America or Central America and
is an important expression of the spectrum of pathology in Latin America. Brazil accounts for approximately 70% of the cases reported in the literature.
Immigrants from these regions may carry it elsewhere
or people visiting these endemic areas may acquire the
infection.
Knowing paracoccidioidomycosis is important to
the dentist, since the oral mucosa provides an important substrate to the saprophytic life of the fungus, and
the disease has frequent oral and dermatological manifestations, usually the first to be detected. In spite of
that, few cases have been reported in the English language literature. Although a primarily lung infection,
208
Stomatitis Medicamentosa
Adverse effects of drugs are generally manifested over the
skin surface. However, literature review reveals that the
oral cavity can sometimes be the only site of involvement.
It is a known fact that no medication is safe and that every
drug has the potential to cause damage to the body including the oral cavity. These drug reactions affecting the oral
mucosa can be in the form of stomatitis, lichenoid reactions, erosions and ulcers.
Table 3
Activation of immunologic
Activation of memory T-cells
mediators without involvement
of memory T-cell function
209
Box 1
Table 4
RBC disorders
WBC disorders
Anemia
Quantitative
disorders
Qualitative
disorders
Lazy leukocyte
syndrome
Iron deficiency
Pernicious
Sickle cell anemia
Thalassemia
Leukemia
Agranulocytosis
Cyclic neutropenia
Multiple myeloma
WBC Disorders
WBC disorders can cause oral ulcerations and necrotizing
lesions which are commonly seen on the gingiva, floor of
the mouth, buccal mucosa and pharynx. These ulcers are
characterized by the lack of the inflammatory halo and
generally associated with necrosis and foul smell. Cyclic
neutropenia is associated with recurring oral ulcers. The
treatment is directed at the cause of the ulceration.
IMMUNOLOGIC DISORDERS
Patients are instructed to avoid smoking. Toothpaste and
mouthwashes with strong flavoring agents are best avoided
(baking soda can be an effective alternative to tooth pastes).
Topical triamcinolone acetonide or flucinonide 0.05% gel
can be used.
ERYTHEMA MULTIFORME
Described in Chapter 7 on Vesiculobullous Disorders.
RBC Disorders
Iron deficiency anemia is characterized by the presence
of glossitis and glossodynia, angular cheilitis and atrophic
areas on the tongue due to papillary atrophy. Patients with
pernicious anemia exhibit glossitis, burning tongue, angular cheilitis, papillary atrophy, and recurrent oral ulcerations. Sickle cell anemia and thalassemia exhibit oral
ulcerations. These patients are relatively more likely to
develop osteomyelitis.
210
Clinical features
Figure 11
Figure 12
A
(A) A minor aphthous ulcer on the lower labial mucosa. (B) A minor aphthous ulcer on the lateral margin of the tongue.
The ulcer is surrounded by the characteristic erythematous halo. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
211
Table 5
Non-specific etiology
Viral etiology
Middle age
Attached mucosa
No antibody titers
Corticosteroids used
Corticosteroids contraindicated
Table 5 compares clinical features of recurrent aphthous stomatitis and recurrent intraoral herpes.
Diagnosis
The diagnosis of RAS is done by exclusion. Hematological
examination can be performed to rule out blood dyscrasias. Ocular, genital, skin, or rectal lesions should not be
present to make a diagnosis of aphthous stomatitis.
Management
The primary goals of therapy for RAS are relief of pain,
reduction of ulcer duration, and restoration of normal oral
function. Secondary goals include reduction in the frequency
and severity of recurrences and maintenance of remission.
Topical medications, such as antimicrobial mouthwashes
and topical corticosteroids, can achieve the primary goals
but have not been shown to alter recurrence or remission
rates. Systemic medications can be tried if topical therapy
is ineffective.
Levamisole has shown variable efcacy in reducing
ulcer frequency and duration in patients with minor recurrent aphthous ulcer (RAU). Dose: 150 mg per day for 3 consecutive days followed by a gap of 2 weeks. Then repeat
for 3 days. This is to be done 6 times (total therapy time is
3 months and total number of tablets is 18). Thalidomide
is effective but, because of its toxicity and cost, should be
used only as an alternative to oral corticosteroids.
DERMATOLOGICAL DISORDERS
Pemphigus
Pemphigus is a chronic, autoimmune skin disorder which is
considered to be the most dreaded, dramatic and devastating
of all dermatologic disorders. Pemphigus vulgaris, vegetans,
212
PEMPHIGOID
Bullous pemphigoid and benign mucous membrane pemphigoid cicatricial pemphigoid are the two variants of
pemphigoid.
Bullous Pemphigoid
Bullous pemphigoid is commonly seen in women in the 6th
and 7th decades of life. It is characterized by the appearance of bullae over the skin, which rupture to give rise to
erosive areas, which heal spontaneously.
Not all cases of bullous pemphigoid involve the oral
cavity. Intraorally small bullae are seen especially on the
Figure 13
A
(A) Ulcers in the vestibular region in pemphigus vulgaris. (B) Erosions on the hard palate in pemphigus vulgaris.
Courtesy: Dr Sumanth
Figure 14
GASTROINTESTINAL DISORDERS
ASSOCIATED WITH ORAL ULCERS
Cicatricial Pemphigoid
Cicatricial pemphigoid is a chronic subepidermal blistering
autoimmune disease. It is characterized by the presence
of vesicles involving the oral, genital, and conjunctival
mucosa. The oral lesions are generally erosions or shallow
ulcers on the facial gingiva, buccal mucosa, palate, tongue,
and lower lip. The ulcers exhibit a distinct outline and heal
with scarring in about one month.
Patients complain of pain burning sensation extending from epigastrium to the neck (heart burn) which is
commonly felt after a meal.
Chest pain that mimics anginal pain.
213
Clinical features
Oral manifestations
Crohns Disease
Crohns disease is a chronic inflammatory bowel disease
that can affect any part of the GI tract. It causes fissures,
transmural inflammation, and non-caseating granulomas of
the GI tract.
It generally affects males and females equally, however
it affects white males more frequently.
Clinical features
Oral findings
Oral mucosal involvement is characterized by the presence
of ulcers, angular stomatitis and superficial hemorrhagic
ulcers. It is estimated that approximately 510% of the
patients with ulcerative colitis exhibit oral manifestations.
Celiac Disease
Celiac disease is an autoimmune gluten-dependent enteropathy characterized by intestinal malabsorption and subtotal or total atrophy of intestinal villi which improves
after gluten-free diet.
Oral findings
Clinical features
1.
Ulcerative Colitis
Ulcerative colitis is a chronic inflammatory bowel disorder
sharing the similar clinical features of Crohns disease
except that the involvement is restricted to the mucosa
and submucosa of the colon.
214
2.
3.
4.
5.
Oral findings
Patients may present with oral ulceration that may mimic
ulceration of recurrent aphthous stomatitis.
NEOPLASTIC ULCERS
Neoplastic lesions that exhibit oral ulcerations are squamous cell carcinoma, adenocarcinoma, mucoepidermoid
carcinoma and metastatic carcinoma.
Clinical features
Diagnosis
Based on clinical appearance alone necrotizing sialometaplasia cannot be diagnosed. Generally, a histopathological
evaluation of the lesion is necessary to establish the correct diagnosis. Malignant ulcers, Wegeners granulomatosis, and extranodal lymphoma can be considered in the
differential diagnosis.
2.
3.
4.
5.
MAGIC Syndrome
Literature review reveals that the symptom complex of
mouth and genital ulcerations associated with inflamed
cartilage (polychondritis) was first described in 1995. Apart
from the typical ulcers involving the oral cavity and the
genital regions, polychondritis is present. It involves the
auricles, lungs, heart, and the vascular system. Patients may
also complain of associated symptoms such as fatigue, malaise, and fever. Use of immunosuppressive agents such as
azathioprine, methotrexate, or cyclophosphamide is the
modality of choice to manage symptoms and signs associated with MAGIC syndrome.
Reiters Syndrome
Behets Syndrome
Behets syndrome is named after Hulusi Behet, a Turkish
dermatologist who described a triad of RAUs, genital ulcerations, and uveitis leading to blindness, in 1937.
There are two widely used diagnostic criteria for
Behets disease, namely, the International Study Group
Criteria for diagnosis of Behets syndrome and ODuffy
criteria.
International Study Group Criteria for
diagnosis of Behets syndrome
Oral ulcers (major/minor/herpetiform RAUs) occurring at
least thrice in a year and the presence of at least two of the
following:
Genital ulcers
Uveitis
Cutaneous pustular vasculitis
Synovitis
Meningoencephalitis.
216
StevensJohnson Syndrome
StevensJohnson syndrome is characterized by the presence of minute blisters on the skin. Detachment of the
skin is limited to about 10% of the body surface area. Skin
detachment involving 30% or more of the body surface
area associated with epidermal necrosis is referred to as
toxic epidermal necrolysis. Clinically another variant is
considered which is characterized by skin detachment
between 10 and 29% of the body surface area and is termed
StevensJohnson syndrometoxic epidermal necrolysis
overlap.
Clinical findings
Patients complain of fever and myalgia. The initial presentation of the condition is an erythematous rash on the face
and trunk that rapidly spreads to involve other parts of the
body. Occasionally blisters are seen within the rash.
StevensJohnson syndrome and toxic epidermal necrolysis usually begin with fever, headache, cough, and body
aches, which may last from 1 to 14 days. This is followed
by the appearance of a at red rash on the face and trunk,
that often spreading later to the rest of the body in an
irregular pattern. The areas of rash enlarge and spread,
often forming painful blisters in the center. The skin over
the blisters can be easily slided off.
Blisters are seen on oral, ocular and genital mucosa that
subsequently ruptures to form ulcers. Patients will complain
Chronic
Single
Multiple
Single
Multiple
Recurrent
Traumatic ulcer
Aphthous ulcer
Herpetic gingivostomatitis
Recurrent intraoral herpes
Herpangina
Hand, foot and mouth disease
Chicken pox
Herpes zoster
Infectious mononucleosis
HIV infection
ANUG
Stomatitis medicamentosa
Stomatitis venenata
Herpetiform aphthous ulcers
Minor aphthous ulcers
Leukemia
Cyclic neutropenia
Aphthous ulcers
Cyclic neutropenia
Behets syndrome
Reiters syndrome
Lichen planus
Pemphigus
Pemphigoid
Chronic
Single
Multiple
Single
Multiple
Recurrent
Generally no need
of investigations
Routine hemogram
may be performed
Biopsy
In case of TB, Mantoux
test, ESR, lymphocyte
count, chest X-ray, PCR
In case of syphilis, VDRL
test, FTA-ABS test or TPI
assay
Special stains in case of
suspected fungal infections
For cyclic
neutropenia, TC
and DC thrice in a
week for 68 weeks
Hemogram
Pathergy test in
Behets syndrome
DIAGNOSTIC PROTOCOL
Step 1: Determine whether ulcers are acute or chronic,
single or multiple or recurrent (by history) (Table 6).
Step 2: Note the features of the ulcer(s) and associated
symptoms:
1. Size, shape, location, surrounding area, tissue tags at
periphery, tenderness, foul smell, bleeding, induration
of base, edges, margins, floor
2. Presence of skin lesions (e.g. lichen planus, pemphigus,
pemphigoid, erythema multiforme, StevensJohnson
syndrome, etc.)
3. Systemic symptoms (fever, malaise, etc.) seen in viral
infections, ANUG, TB, erythema multiforme, etc.
217
CHAPTER
Dermatological Diseases
K Srinivas, Sarita Dimri, Ravikiran Ongole
Lichen Planus
Psoriasis
Ectodermal Dysplasia
Reiters Syndrome
EhlersDanlos Syndrome
Pachyonychia Congenita
Dyskeratosis Congenita
Pityriasis Rosea
Xeroderma Pigmentosum
Acanthosis Nigricans
GoltzGorlin Syndrome
Graft-versus-host disease
Acrodermatitis Enteropathica
Epidermolysis Bullosa
LICHEN PLANUS
Lichen planus (LP) has also been known as lichen ruber
planus. It is one of the most common dermatologic, immunopathological diseases to affect the oral mucous membrane. The management of oral lichen planus continues to
challenge even the most experienced oral physician.
The term lichen planus is derived from a Greek word
lichen which means tree moss and a Latin word planus
which means at. The strange name of the condition was
provided by the British physician Erasmus Wilson, who
rst described the lesion in 1869. Thibierge rst described
the oral lesions systematically in 1885.
Fitzpatrick et al (1993) described LP as a unique cutaneous entity consisting of an eruption of papules distinct in
color and conguration, in patterns and location of appearance and in microscopic as well as gross structure.
Andreasen categorized oral LP into six types, namely,
reticular, papular, plaque-like, atrophic, erosive, and bullous.
Lichen planus has been associated with various diseases
such as hepatitis C, oral cancer, and diabetes mellitus.
218
Etiopathogenesis
An interplay of host, lifestyle, and environmental factors
has been implicated in the etiopathogenesis of LP. It is
believed that LP is caused due to cell-mediated immunity
initiated by endogenous or exogenous factors.
Clinical features
The onset of LP occurs most commonly during the 5th or
6th decade. No sexual predilection is evident. The typical
cutaneous lesions of LP present as flat topped, purple, polygonal, pruritic papules and plaques most commonly occurring
on the flexor surfaces of the arms, wrists, ankles, and legs.
Oral lesions may be observed in up to 75% of patients
with cutaneous LP and in approximately 25% of cases it
can be the only manifestation of the disease. Conversely,
only 1020% of patients whose initial presentation is oral
LP will develop cutaneous LP. The oral lesions have been
observed in up to 14% of the population.
Oral LP almost invariably occurs as a bilateral disease
and it involves the posterior buccal mucosa followed less
commonly by the tongue, gingiva, hard palate, and the
Figure 1
Keratinocyte damage
Apoptosis of keratinocytes
Investigations
Colloid bodies (underlying dermis)
Biopsy of the lesion should be done to confirm the diagnosis. In situations where histopathology does not confirm
the diagnosis then immunofluorescence studies of biopsy
specimens should be done. Direct immunofluorescence
demonstrates a shaggy band of fibrinogen in the basement
membrane zone in 90100% of cases. Specimens for immunofluorescence should be stored in Michels/Bouins solution or normal saline and then sent to histopathology.
Differential diagnosis
Reticular form
Plaque form
Lichenoid reactions
Leukoplakia, hyperplastic candidiasis, traumatic keratosis
Atrophic form
Speckled leukoplakia, anemic
stomatitis, systematic lupus
erythematosus and discord
lupus erythematosus
Erosive and bullous form Vesiculobullous lesions
Annular form
Erythema circinata migrans.
Management
The lesions of oral LP appear, regress and reappear in some
what unpredictable fashion. Asymptomatic LP need not be
treated. The treatment of symptomatic LP is necessary.
The role of Candida in the causation of oral LP has been
debated, therefore a smear for candida needs to be made and
219
EPIDERMOLYSIS BULLOSA
Epidermolysis bullosa (EB) is a diverse group of disorders
that have as a common feature blister formation with tissue
separation occurring at variable depths in the skin and/or
mucosa depending on the specific EB type. There may be
marked oral involvement, potentially creating devastating
alterations in the soft and hard tissues. Oral tissue fragility
and blistering is common to all EB types.
Classification
Dental management
Etiopathogenesis
PSORIASIS
Psoriasis is a non-contagious skin disorder that most commonly appears as inflamed, edematous skin lesions covered
with a silvery white scale.
Types
Guttate psoriasis
Pustular psoriasis
Inverse psoriasis
Erythrodermic psoriasis.
Psoriasis is more common in whites and in women. Approximately 1015% of new cases begin in children younger than
10 years. The median age at onset is 28 years. Typical skin
lesions of psoriasis appear as well-circumscribed erythematous patches with overlying thick silvery scales (Figure
2A, B). Lesions may occur in any location, but most commonly involve the scalp and the anterior hairline, torso,
bony prominences of the extremities, nails, perianal and
perineal areas. The course of the disease is unpredictable
and characterized by spontaneous episodes and relapses.
If the deep scales are removed, one or more tiny bleeding points are disclosed, which is popularly referred to as
Auspitzs sign.
Figure 2
A
Erythematous patches on the forearms along with silvery scales in psoriasis. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
221
The oral counterpart of psoriasis is rare. The rst descriptions of oral lesions of psoriasis have been attributed to
Oppenheim and Thimm (1903).
Van der Waal and Pindborg described four types of oral
psoriatic lesions.
Clinical features
The condition is characterized by hypodontia, hypotrichosis, and hypohidrosis. Neonates exhibit excessive scaling
of the skin and unexplained pyrexia. Patients present with
sparse hair and eyebrows (Figure 3A, B).
As the age progresses frontal bossing, saddle nose, sunken
cheeks, thick/everted lips wrinkled and hyperpigmented
Figure 3
A
Histopathology
Histopathologically, intraepithelial microabscesses (Munros
abscesses) are common. However, these are not specific for
the disease.
Management
Oral lesions should be treated with topical corticosteroids.
The topical steroids that are available in India are triamcinolone acetonide0.1% with orabase (Kenacort oral paste,
TESS cream), clobetasol propionate0.05% (Clobetamil
cream, Tenovate skin cream).
Psoriatic arthritis
Psoriatic arthritis is a systemic disorder and inflammatory
condition and this disease may be the major contributing
factor to temporomandibular dysfunction (TMD) symptoms
and signs. TMD signs and symptoms are found more frequent and more severe in the patients with psoriatic arthritis
of other joints than in the patients with psoriasis without
arthritis. TMD signs and symptoms in psoriasis are mainly
caused by the related joint involvement that directly affects
the masticatory system.
ECTODERMAL DYSPLASIA
The ectodermal dysplasias comprise a large and heterogeneous group of disorders (about 170) characterized by a
variety of congenital defects in structures of ectodermal
origin including skin, hair, teeth, nails, and sweat glands.
Out of these the most common and best studied disease is
anhidrotic or hypohidrotic ectodermal dysplasia (Christ
SiemensTouraine syndrome).
Etiopathogenesis
It is an inherited X-linked recessive trait associated with
the repressed expression of a gene on the X-chromosome
in the positions from q13 to q21.
222
Figure 4
Figure 5
Figure 6
skin around the eyes are seen. Patients may present with
fever of unknown origin because of the inability to sweat.
Oral manifestations
Anodontia/oligodontia is often seen. The remaining teeth are
usually malformed. Both deciduous and permanent teeth are
affected. Most common missing teeth are molars. Malformed teeth have truncated and conical crowns and shortened roots (Figures 4 and 5).
Dry mouth, high palatal arch and cleft palate may be
seen in some individuals. The alveolar ridges are usually
EHLERSDANLOS SYNDROME
The term EhlersDanlos syndrome (EDS) was coined after
the names of a Danish dermatologist, Edvard Ehlers (1901)
and a French dermatologist, Henri Alexandre Danlos
(1908) who reported patients exhibiting thin, hyperplastic
skin, loose jointedness, and hemorrhagic tendencies.
It is a group of inherited disorders characterized by
excessive looseness (laxity) of the joints, hyperelastic skin
that is fragile and bruises easily, and/or easily damaged
blood vessels. The diagnosis of EDS encompasses any of
six types of connective tissue disorders that are hereditary
in nature and exhibit a characteristic defect in collagen
metabolism.
There are six major types of EDS that are characterized
by distinctive features.
223
Figure 7
Etiopathogenesis
Different forms of EDS have different modes of inheritance.
Family history is a risk factor in some cases. Various subtypes are inherited as autosomal dominant/recessive and
X-linked traits. A variety of genetic mutations cause abnormality in collagen that will result in the disease.
Clinical features
Symptoms
Joint dislocation/subluxation/joint pain, increased
joint mobility, joints popping, early arthritis, doublejointedness and flat feet
Easily damaged, bruised, and stretchy skin which is
very soft and velvety
224
Signs
Excessive joint laxity and hypermobility
Soft, thin, or hyperextensible skin
Mitral valve prolapse
Signs of platelet aggregation failure
Rupture of intestines, uterus, or eyeball (seen only in
vascular EDS, which is rare)
Deformed cornea.
Oral manifestations
Scarring on the chin and forehead, a history of repeated
luxations of the TMJ, epicanthus, hypertelorism, a narrow
curved nose, sparse hair and hyperelasticity of the skin.
Fragile mucosa and gingivaearly-onset generalized
periodontitis leading to premature loss of deciduous and
permanent teeth. Tooth mobility is often encountered.
Hypoplasia of the enamel is commonly seen. Premolar
and molar teeth can present with deep ssures and long
cusps. Microdontia is sometimes present. Irregularities in
the dentin structure and dentinal tubules may also be seen.
Radiographic examination often reveals pulp stones
and roots that are short and deformed.
The tongue is very supple. Approximately, 50% of those
with the syndrome can touch the end of their nose with their
tongue (Gorlins sign), compared to 810% of the normal
population and the palate is commonly vaulted.
Investigations
Differential diagnosis
Marfans syndrome, generalized familial joint hypermobility syndrome, cutis laxa, pseudoxanthoma elasticum and
Larsens syndrome.
Management
There is no specific cure for EDS, so individual problems
and symptoms must be evaluated and cared for appropriately. Genetic counseling is recommended for prospective
parents with a family history of EDS.
Dental considerations
PACHYONYCHIA CONGENITA
Pachyonychia congenita (PC) is a rare autosomal dominant keratin disorder that typically affects the nails and
palmoplantar skin, and often the oral mucosa, tongue,
larynx, teeth, and hair.
Mller made the rst documented observation in 1904.
Jadassohn and Lewandowsky published the next reports
in 1906. In the dermatologic literature, PC is better known
as JadassohnLewandowsky syndrome. PC is also known
as Touraines polykeratosis congenita, palmoplantar keratoderma.
Etiopathogenesis
The disease results from mutations in the genes encoding
epidermal keratinocyte keratins. The mutation is likely to
have a deleterious effect on protein structure as it interferes with the assembly of polypeptides forming the keratin
skeleton of epidermal cells (Bowden, 1995).
Clinical features
Onychodystrophy, palmoplantar keratoderma and follicular keratosis in areas of friction are characteristic features. Epidermal inclusion cysts and pilosebaceous cysts
are often seen in PC. Thickened or coarse curly hair is
usually seen. Laryngeal involvement is usually present
DYSKERATOSIS CONGENITA
Dyskeratosis congenita, also known as ZinsserEngman
Cole syndrome and HoyeraalHreidarsson syndrome, is a
rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation,
nail dystrophy, and oral leukoplakia.
It is a pre-malignant condition. It is genetically heterogeneous, with X-linked recessive, autosomal dominant
and autosomal recessive subtypes. It is related to telomerase dysfunction. Telomeres are repeat structures found at
the ends of chromosomes that function to stabilize chromosomes. With each round of cell division, the length of
telomeres is shortened and the enzyme telomerase compensates by maintaining telomere length in germline and
stem cells. Because telomeres function to maintain chromosomal stability, telomerase has a critical role in preventing cellular senescence and cancer progression.
Rapidly proliferating tissues with the greatest need for
telomere maintenance (e.g. bone marrow) are at greatest
risk for failure.
Clinical features
Mucocutaneous features The mucocutaneous features are
the most consistent features of the disease. Reticulated skin
hyperpigmentation affecting the neck, face, chest, and
arms is the most common finding occurring in approximately 90% of patients. Telangiectasia, atrophy (pokilodermia) dystrophic nails and palmoplantar keratoderma,
hyperhidrosis, blepharitis, conjunctivitis, epiphora include
other signs which are less frequently seen.
Leukoplakia, which is the third feature of the classic
clinical triad, has been reported in 80% of the affected
225
PITYRIASIS ROSEA
Pityriasis rosea (PR) is an acute self-limiting disease, probably infective in origin, affecting mainly children and
young adults, and characterized by a distinctive skin eruption and minimal constitutional symptoms.
Etiology
The cause of PR is uncertain, but many epidemiological and
clinical features suggest that an infective agent may be
226
Seborrheic dermatitis
Guttate psoriasis
Secondary syphilis
Pityriasis lichenoides.
XERODERMA PIGMENTOSUM
Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by photosensitivity, pigmentary
changes, premature skin aging, neoplasia and abnormal
Diagnosis
The unscheduled DNA synthesis assay (following UV irradiation of the cells in culture) is the standard laboratory
method for diagnosis of XP. Prenatal diagnosis by amniocentesis and molecular genetic techniques allow for earlier
and more reliable results.
Treatment
Patients must be protected from sunlight by every possible
meansby using sunblock creams, sunglasses with side
shields, two layers of clothing and broad brimmed hat.
Early and adequate excision of all tumors is essential.
Topical 5-uorouracil may be useful for early or premalignant lesions. A recent clinical trial by Yarosh et al
used the microbial enzyme T4 endonuclease V applied
regularly as a topical liposome lotion for over a period of
1 year, signicantly reduced the onset of both new basal
cell carcinoma and actinic keratoses.
The prognosis is poor. Most patients die 30 years earlier
than the normal population, either directly from cutaneous malignancy or from complications associated with the
treatment of the cancer.
ACANTHOSIS NIGRICANS
Acanthosis nigricans (AN) is an acquired dermatologic
condition characterized by the development of a velvety,
brownish alteration of the skin. The cutaneous lesions itself
is benign, yet it is significant because it represents a cutaneous marker for internal malignancy.
Etiology
Acanthosis nigricans has a variety of known causes
whose common mechanism is likely to be stimulation of
tyrosine kinase growth factor receptor signaling pathway
in epidermis.
In insulin resistance syndrome, high levels of circulating insulin directly or indirectly activate the insulin-like
growth factor 1 receptor (IGF1R), which is a transmembrane protein related to the insulin receptor.
Tumor-derived growth factors are preserved to be involved
in malignant AN.
Clinical features
Earliest changes are pigmentation, dryness and roughness
of the skin which in the affected areas is grayish brown or
black, palpably thickened and covered by small papillomatous elevations, which give it a velvety texture. As the
thickening increases, the skin lines are further accentuated
and the surface becomes mammillated or rugose and larger
warty excrescences develop. Most common sites are axillae,
227
Management
Treatment is of the underlying cause or is otherwise symptomatic and of little help. Removal of the tumor in the
malignant form may allow some improvement. A case of
hereditary benign AN improved dramatically with etretinate.
Although AN itself is a harmless process, the patients should
be evaluated to ascertain which form of disease is present.
GOLTZGORLIN SYNDROME
GoltzGorlin syndrome is also referred to as focal dermal
hypoplasia syndrome or Goltz syndrome. We should be
aware that this syndrome is not the same as Gorlin syndrome or GorlinGoltz syndrome, which is basal cell nevus
syndrome.
This uncommon genetic condition is transmitted as an
autosomal dominant trait. It is characterized by typical
skin defects and widespread involvement of various organ
systems. It affects the eyes, skeletal system, urinary system, cardiovascular and central nervous system and the
gastrointestinal system.
Clinical features
Almost 90% of the individuals who present with this syndrome are females. When it occurs in males the condition
is lethal. The clinical features are usually evident at birth
and the signs and symptoms progress with advancing age.
Patients are usually short statured with sparse hair over
the scalp, pubic region, eyebrows and eyelashes. Patients
may complain of hypohidrosis. Other features include nail
dystrophy and syndactyly, polydactyly and lobster-claw
deformity of the hands. Diffuse cortical cerebellar atrophy
and recurrent respiratory infections may be seen.
228
Skin manifestations
As the name suggests there is a focal loss of the dermis
characterized by the outpouching or herniation of the subcutaneous fat. Based on the degree of melanin pigmentation, the lesion has varied appearances. Erythematous
macules are evident in fair skinned individuals whereas
areas of hypo- or hyperpigmentation are seen in darker
individuals. The lesions are typically confined to the lines
of Blaschko. The common sites that are affected are the
forearms, thighs and cheeks.
Some authors describe the presence of raspberry like
papillomas. Papillomas are usually present at the skin and
mucosal junction such as the perioral, periocular, perianal
and perivulvar regions.
Facial features
Patients may have an asymmetrical face with a pointed chin.
The eyes are usually sunken and ears may appear to be
protruded and asymmetric. Colobomas of the iris, choroid,
retina, or optic disk are seen in almost 35% of the patients.
Occasionally, hypertelorism and blue sclera may be seen.
Oral features
Occasionally, cleft lip and palate associated with this syndrome have been reported. Both the deciduous and permanent
dentition may be affected. Teeth are usually hypoplastic
and microdontic. Oral papillomas may be seen in any part
of the oral mucosa. However, the lips, gingiva, tongue and
buccal mucosa are the common sites.
Other relatively rare ndings include split/double lingual
frenum, high palatal vault and cleft lip/palate.
Radiographic findings
The characteristic finding is the presence of osteopathia striata (longitudinal striations) in the metaphysis of the long
bones and the sacral bone. Orthopantomograph may reveal
the presence of multiple taurodonts.
Treatment and prognosis
No definitive treatment is possible for the condition. The
prognosis and severity of the condition depends on the
organ system that is involved.
ACRODERMATITIS ENTEROPATHICA
Acrodermatitis enteropathica (AE) is a rare disease transmitted as an autosomal recessive trait. It was first recognized in 1936 by Thore Brandt and further investigated by
Danboly and Closs. Although deficiency dermatitis caused
by low dietary zinc has the exact clinical and histological
features but the term should be reserved only for genetic
causes of zinc deficiency.
Etiology
Clinical features
Clinical features
The disease typically starts after weaning or earlier if the
infant is not given breast milk. The child turns peevish,
withdrawn and photophobic and develops a vesicobullous
dermatitis on the hands, feet and periorificial areas. The
scalp hair is lost. Diarrhea is often present. Growth is stunted
and there is a decreased resistance to infection. Wound
healing is poor and skin lesions do not heal.
Differential diagnosis
Atopic dermatitis
Cutaneous candidiasis
Epidermolysis bullosa
Seborrheic dermatitis.
Diagnosis
Low serum of zinc and alkaline phosphatase (a zinc
dependent enzyme) may aid in the diagnosis of zinc
deficiency.
Treatment and prognosis
Zinc sulfate for AE was introduced between 1973 and
1974. Oral zinc in a dose of about 2 mg/kg per day was
found to clear all clinical manifestations. Prolonged therapy up to adult age is necessary.
HAILEYHAILEY DISEASE
(Familial Benign Chronic Pemphigus)
HaileyHailey disease is a rare autosomal dominant
intraepidermal blistering disease which is characterized by
recurrent vesicles and erosions usually affecting the neck,
axilla and groin. The condition was described by Hailey
brothers in 1939.
Differential diagnosis
Pemphigus vegetans
Dariers disease
Impetiginized eczema
Candidal intertrigo.
DARIERS DISEASE
(Keratosis Follicularis)
Dariers disease, described independently by White and
Darier in 1889, is an autosomal dominant condition characterized by a persistent eruption of hyperkeratotic papules,
histological examination with a distinctive overlying dyskeratosis.
Etiology
It is caused by mutations in ATP2A2 gene at chromosome
12q24.1, which encodes the sarco and endoplasmic reticulum calcium ATPase type 2 (SERCA2) which is a member of
a family of ion pumps that maintain high calcium concentration in endoplasmic reticulum.
Clinical features
Etiology
HaileyHailey disease is caused by mutations in ATP2C1,
a gene on chromosome 3q21 that encodes a P-type calcium
transport adenosine triphosphate (ATPase). The cellular
process that may be affected by mutation includes gene
transcription, post-translational modifications and trafficking of adhesion proteins and the assembly of adhesion
junctions.
Characteristic lesion of Dariers disease is a firm rough papule, which is skin colored, yellow brown or brown seen on
seborrheic areas which exacerbate on sun exposure,
coalescent papules form irregular warty fissured plaques
or papillomatous plaque masses which, in the flexures,
become vegetating and malodorous. On the scalp, heavy
crusting has a characteristic spiny feel on palpation
with loss of hair. The external auditory meatus may be
229
REITERS SYNDROME
Reiters syndrome is characterized by the presence of
a non-suppurative polyarthritis exceeding a duration of
1 month associated or preceded closely by a lower urogenital or enteric infection in young men who carry the
HLA-27 antigen. It was named after Professor Hans Reiter,
who in 1916 reported a German officer who developed
urethritis conjunctivitis and arthritis following an episode
of bloody diarrhea.
Inammatory eye involvement and mucocutaneous
manifestations are common. The classical triad of arthritis,
conjunctivitis, and urethritis is observed only in 33% of the
cases. The less stringent criteria of American College of
Rheumatology, comprising of peripheral arthritis of more
than 1 month duration, occurring in association with urethritis or cervicitis exhibit a sensitivity of 84.3% and specicity of 98.2% when compared with other arthropathies.
Etiology
The etiopathogenesis of Reiters disease centers on the
determination of the role of probable infective triggering
agents and the proneness of certain individuals to develop
the condition due to genetic susceptibility (Table 1).
Clinical features
The earliest features of Reiters disease usually presents
within 14 weeks of exposure. Fever, constitutional complaints and features of urethritis or enteritis usually precede arthritis.
230
Table 1
Urogenital pathogens
Enteric pathogens
Chlamydia trachomatis
C. psittaci
C. pneumoniae
Ureaplasma urealyticum
Neisseria gonorrhoeae
INCONTINENTIA PIGMENTI
(BlochSulzberger Syndrome)
Incontinentia pigmenti (IP) is a rare X-linked dominant
disease that affect skin, eyes, hair, teeth and central nervous
system and manifest itself during early neonatal period.
The disease was first described by Garrod in 1903, its pathogenesis in 1926 by Sulzberger and localization of rash in
1985 by Happel.
Clinical features
Four stages are differentiated according to the changes in
the skin are shown in (Table 2).
Nail dysplasia is found in 4060% of cases. Eye
changes are seen in one-third of the cases as speckled diffuse hypopigmentation in the retina (a pathognomonic feature), microphthalmia, lenticular hemorrhage, retrolental
broplasia, cataract and atrophy of the optic nerve.
Table 2
Stages
Clinical features
Stage 1 vesicular
Stage 2 verruciform
Stage 3 pigmented
Stage 4 depigmented
Hairless streaks
Alopecia at vertex
Anomalous dentition
Woolly hair
Retinal disease
Multiple male miscarriages.
Differential diagnosis
In acute stage of disease, herpes simplex infection, impetigo, candidosis, epidermolysis bullosa congenita. In later
stage, differentiated from post-inflammatory pigmentation
and hypomelanosis of Ito.
Treatment and prognosis
The vesicles should not be touched and the skin must be kept
clean to avoid infection. Local anti-inflammatory treatment with steroids may be applied.
Timely diagnosis of IP prior to pregnancy and early
genetic consultation of pregnant women with IP to evaluate
the risk of damage for the children of such women are
essential.
KAWASAKI DISEASE
(Mucocutaneous Lymph Node Syndrome)
This condition is usually seen in children, often affecting
those below 2 years of age, characterized by fever and generalized exanthem with lymphadenitis. The disease was
first described in 1967 by Kawasaki from Japan.
Time of manifestation
Clinical features
The onset is acute, with a high fever, which lasts for at least
57 days. The mucosa and conjunctiva are injected. In the
mouth, the lips are dry and fissured, the tongue appears red
with prominent papillae (strawberry tongue) and the throat
is injected. After 34 days there is generalized exanthema.
The area affected on the limbs becomes edematous followed
by scaling. There is accompanying cervical lymphadenitis,
although not always present and may only involve one
node. Fever resolves in 12 weeks.
Complications
In about one-fourth of cases there is accompanying
myocarditis which may be followed by symptomatic coronary artery disease and in 12% by myocardial infarction.
Other complications include arthralgia, arthritis, severe erythema multiforme, iritis, proteinuria, hepatitis and aseptic
meningitis.
Diagnosis
Abnormalities on investigation includes leukocytosis and
thrombocytosis with a raise ESR. The raised platelet count
is most often seen in the post-acute phase.
Treatment
Intravenous gammaglobulin in high doses (2 g/kg in a single effusion over 10 hours) is very helpful in reducing the
overall mortality and complication of the disease. Shortterm use of aspirin is also helpful in reducing the risk of
platelet aggregation.
Etiology
Various hypotheses have been advanced to account for its
symptoms. These include rickettsial illness, exposure to
house-dust mite. It has been suggested that bacterial
superantigens cause disease through wide scale activation of immune mechanism with cytokine release bringing
other cell types, including vascular endothelium, into an
uncontrolled immunological reaction.
232
Skin lesions
Skin lesions are found in 6070% cases. Lesions of four
types are pathognomonic:
Mental deficiency
Mental deficiency is present in 6070% of cases and may
be progressive, but if mental development has been normal throughout the childhood subsequent deterioration is
uncommon.
Epilepsy is seen in almost all mentally retarded patients
and in some 70% of those with average intelligence. It
usually begins in infancy or early childhood, thus often
preceding the skin lesions by many years.
Ocular signs
Ocular signs occur in 50% of the cases but are hard to
detect. Retinal phacomas, pigmentary and other retinal
abnormalities and hypopigmented spots on iris can occur.
Cardiac and renal tumors, pulmonary changes, gastrointestinal tumors and endocrine and other metabolic disturbances can occur.
Oral manifestations
Oral manifestations of tuberous sclerosis include developmental enamel pitting on the facial aspect of the anterior
permanent dentition in 50100% of patients. These pits
are readily appreciated after applying a dental plaquedisclosing solution to the teeth.
Multiple brous papules affect 1156% of patients seen
predominately on the anterior gingival mucosa. Diffuse
gingival enlargement and radiolucencies of the jaws that
represents dense brous tissue proliferation is also seen.
Treatment and prognosis
The cosmetic appearance may be improved by removing
angiofibromas with pulsed dye vascular laser (585 nm).
233
Neurosurgery should be considered when epilepsy is controlled by drugs and there is fixed, circumscribed, electroencephalographic focus. The treatment of lesions in other
organs is unsatisfactory and surgical procedure may be
required for relief of symptoms.
Patients affected by this condition have a slightly reduce
life span compared with the general population, with deaths
usually related to CNS or kidney disease.
GRAFT-VERSUS-HOST DISEASE
Graft-versus-host disease (GVHD) occurs when immunocompetent cells from a donor recognize and react against
foreign tissue antigen in an immunocompromised host.
Moderate to severe acute GVHD affects 935% of patients
undergoing standard allogenic bone marrow transplantation, despite using HLA matched sibling donors and
immunosuppression after grafting.
followed by a faint red maculopapular rash leading to desquamation or even toxic epidermal necrolysis.
Chronic GVHD occurs 314 months after the transplant. When the condition is localized it occurs as hypopigmented nodular areas which eventually soften and atrophy.
Generalized GVHD starts as erythematous rash and
becomes lichenoid. With advancing time scleroderma like
changes appear.
The oral mucosal manifestations of GVHD depend on
the duration and severity of the attack and the target
oral tissue. Sometimes the oral lesions of GVHD are the
only signs of disorder. It is estimated that 3375% of
patients suffering from acute GVHD and about 80% of
patients suffering from chronic GVHD will have oral
involvement. Patients will present with ne reticular network of white striae that resembles oral lichen planus
involving tongue, labial mucosa and the buccal mucosa.
Atrophy, ulceration and xerostomia of the oral mucosa
can be present.
Treatment and prognosis
Etiology
Billingham described the original criteria for development
of a GVHD in 1966:
Prophylactic use of cyclosporine, methotrexate, prednisolone combination therapy has reduced incidence of acute
GVHD. T-cell depletion by using anti-T cell receptor antibodies is successful.
Granulocyte colony stimulating factor, used to enhance
engraftment may reduce the incidence of GVHD. Once the
disease is established, treatment with high dose steroids or
cyclosporine is of value symptomatically and antilymphocyte globulin may be of additional benet.
The prognosis depends on the extent to which the disease progresses and whether it can be controlled. Mortality
is caused by the disease itself and severe superinfection
due to immunosuppressive therapy. It is believed that about
55% of the patients with mild GVHD survive compared to
the 15% survival rate in severe GVHD.
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236
SECTION
IV
Diseases of
Specific Structures
10 Temporomandibular Disorders
11 Diseases of Salivary Glands
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CHAPTER
Temporomandibular
Disorders
10
Osteoarthrosis
Osteoarthritis
Juvenile Idiopathic Arthritis
Polyarthritides
Traumatic Arthritis
Infectious Arthritis/Septic Arthritis
Rheumatoid Arthritis
Psoriatic Arthritis
Hyperuricemia
TMJ Ankylosis
Condylar Fractures
COMPONENTS OF TEMPOROMANDIBULAR
JOINT
Glenoid Fossa and Articular Eminence/
Protuberance
The glenoid fossa or mandibular fossa is a well-defined hollow area on the inferior portion of the squamous temporal
Mandibular Condyle
The condyle is elliptically shaped with its long axis oriented
mediolaterally. The dimension of the condyle is roughly
20 mm in the mediolateral direction and approximately
810 mm in the anteroposterior direction. The articulating
surface of the condylar head is covered by fibrocartilage
(Figure 1).
Figure 1
Figure 2
240
Synovial Fluid
The main functions of the synovial fluid are to nourish the
avascular articulating cartilage and provide lubrication
between the articulating surfaces during function.
Synovial uid is a clear straw-colored thixotropic uid
which is composed of mucin with some albumin, fat, epithelium, and leukocytes. Synovial uid also contains lubricin
secreted by synovial cells. It is mainly responsible for socalled boundary layer lubrication, which reduces friction
between opposing surfaces of cartilage. Synovial uid is
made of hyaluronic acid and lubricin, proteinases and collagenases.
During movement, the synovial uid held within the
cartilage is squeezed out mechanically to maintain a layer
of uid on the cartilage surface (so-called weeping lubrication). Boundary lubrication is a function of water physically bound to the cartilaginous surface by a glycoprotein.
Discal Ligaments
The medial and lateral portions of the articular disk are
attached to the corresponding poles of the condyles via nonelastic, short discal ligaments. These ligaments are vascularized and innervated.
These ligaments restrict the movement in the lower joint
to hinge or rotatory action when viewed in a sagittal plane.
Discal ligaments cause the disk to move passively with
the condyle in an anterior and posterior direction during
condylar translation. These ligaments permit very little
lateral excursion.
Muscles of Mastication
Various mandibular movements such as opening, closing, protrusion and retrusion occur under the coordinated movements of the muscles of mastication, namely,
the masseter, temporalis, medial pterygoid and lateral
pterygoid.
Masseter muscle
Masseter muscle arises from the lower border and inner
surface of the anterior two-thirds of the zygomatic arch,
passes inferiorly and posteriorly, and inserts on the outer
surface of the mandibular ramus (Figure 3). The deep fibers
of the masseter muscle (pars profunda) are vertically oriented
whereas the superficial fibers (pars superficialis) are more
oblique. This muscle is responsible for elevating the mandible to aid in jaw closure and for clenching and crushing
action.
Temporalis muscle
The temporalis is a broad, fan-shaped muscle that arises
from the temporal fossa (superior and inferior lines of the
temporal bone). Via a strong tendon it inserts into the
upper anterior border and the medial aspect of the coronoid
241
Figure 3
Temporal muscle
Masseter muscle
Figure 4
A
242
Figure 5
CLINICAL EVALUATION OF
TEMPOROMANDIBULAR JOINT
Temporomandibular joints are located about 1.5 cm anterior to the tragus of the ear. The two TMJs, considered
together, compromise only one part of the total articulation between the lower jaw and the skullfacial skeleton
complex. The other important contribution is made by the
interdigitations of the mandibular and maxillary dentition,
and function and health of the joint is directly related to
condition of the teeth.
The history of presenting illness should include the onset
and course of signs and symptoms. Past history should
include the details regarding arthritis, infections, degenerating diseases, parotitis, ear disorders, muscular disorders,
trauma, past dental treatment, diet/nutritional adequacy
and habits like clenching, gum chewing, etc. and the individual lifestyle.
Figure 6
nger inside the external auditory meatus. During mandibular movement the posterior poles of the condylar head
can be palpated with the pulp of the little nger. Intraauricular palpation may also be used to elicit capsular
tenderness.
Extra-auricular examination of the TMJ is achieved by
placing the index ngers in the pre-auricular region about
1.5 cm medial to the tragus of the ear. The lateral pole of
the condyle is accessible during this examination.
Palpatory examination of the TMJ should include
the assessment of mouth opening, range of mandibular
movements, joint tenderness, detection of clicks and/or
crepitus.
244
Cervical Examination
Temporomandibular disorders/myofascial pain disorders
often have musculoskeletal problems in other regions that
are particularly associated with neck. Check for mobility
of the neck and examine for range and symptoms.
Patient is rst asked to look to the right and then to the
left. There should be at least 70 rotation in each direction.
Next patient is asked to look upward as far as possible
(extension) and then downward (exion). Any pain is
recorded and any limitation of the movement determines
muscular or vertebral problem. Sternocleidomastoid/trapezius/posterior cervical muscles are often part of neck
disorder and may refer pain to face and head.
Figure 7
Figure 8
Occlusal Evaluation
Sternocleidomastoid
Palpation is done bilaterally near its insertion on the outer
surface of the mastoid fossa behind the ear. The entire
245
Imaging Protocol
Clinical situation
Cost
Radiation dose.
These depict the osseous structures of the joint with varying degrees of bony detail.
Disorders of TMJ
Dimitroulis in 1998 described temporomandibular disorders as a collective term used to describe a number of
related disorders involving the TMJ, masticatory muscles
and occlusion with common symptoms such as pain,
restricted movement, muscle tenderness and intermittent
joint sounds.
The disorders of the TMJ may exhibit a wide variety
of symptoms and signs. The symptoms and signs that are
frequently associated with temporomandibular disorders
include: pain on mouth opening, limitation of mouth
opening, pain on chewing, joint noises (clicking and/or
popping, grating), pain in the region of the joint and/or
muscles, pain around the region of the ear, temporal region
and cheeks, subjective hearing loss, occlusal irregularities,
attrition of teeth, headache (frontal, temporal, suboccipital), tinnitus, muscle hypertonicity and hypertrophy of jaw
muscles, neck pain and difculty in swallowing.
TMJ disorders may arise from macro trauma such as
in road trafc accident (RTA), excessive mouth opening
(yawning, biting onto a large chunk of food) or from
repeated micro trauma such as in parafunctional habits
(bruxism), uneven occlusal loading (malocclusion, high
points in restorations, poorly contoured crowns). Other
causes include stress, underlying systemic diseases, arthritis and developmental abnormalities. See Box 1 for classication of TMJ disorders.
246
Clinical features
Patient is usually asymptomatic. Over a period of time the
patient is accustomed to a new pattern of mouth opening,
thereby avoiding pain during mandibular movements.
Occasionally, a click may be evident during the opening
and closing movements. What is interesting about the
clicks associated with condition is that the click is evident
at the same point both during opening and closing. Whereas
in click associated with disk displacement, the opening
click is usually evident after 20 mm of mouth opening and
the closing click is felt just short of occlusion of teeth.
This condition can be managed by instructing the
patient to develop a path of mandibular movement that
avoids the interference and to chew on the affected side.
This will minimize the intra-articular pressure in the ipsilateral joint.
Box 1
Figure 9
Disk Displacement
Disk displacements are also termed as internal derangement. The internal derangements could include disk displacement with reduction and disk displacement without
reduction.
Anterior disk displacement is common and it usually
occurs when there is elongation of the disk attachment and
deformation or thinning of the posterior border of the disk,
which in turn permits the articular disk to get displaced in
an anterior direction on the surface of the condyle. In normal conditions, when the teeth are in occlusion, the posterior band of the disk ends at the apex of the condyle. In
anterior disk displacement, the posterior band of the articular disk terminates ahead of the condylar apex. The most
common causes for internal derangement include trauma,
clenching and biting on hard substances.
Figure 10
Resting phase
Open click
248
Synovitis and capsulitis are clinically considered as a single disorder. Synovitis refers to inflammation of the synovial tissues and inflammation of the capsular ligaments is
referred to as capsulitis. Various causes have been attributed to these inflammatory conditions such as trauma,
opening the mouth excessively, chronic condylar displacement in a posterior direction and sometimes from a direct
spread of inflammatory products from the surrounding
structures.
Clinical features
Types of dislocation
Depending upon the position the condyle occupies, Heslop
in 1956, described the anterior dislocation in which the
condyle moves anterior to the articulating eminence. It is
one of the most common type of dislocation. It represents
the pathological forward extension of normal translatory
movement of head of condyle. The anterolateral variant
was described by Morris and Hutton in 1957.
Helmy in 1957 described the posterior variant in which the
head of condyle is displaced posterior to its usual position.
It is usually associated with a fracture of base of skull or
the anterior wall of bony meatus.
Continuous pain that exacerbates during function is characteristic of this condition. Limitation in jaw movements is
another common finding. In some individuals malocclusion
in the posterior teeth is seen due to inferior displacement
of the condyle resulting from the edema.
Retrodiscitis
Inflammation of the retrodiscal tissues (retrodiscitis) results
from a traumatic injury which may indirectly cause the
condylar head to impinge on the retrodiscal tissues.
Figure 11
Resting phase
Late translatory
249
Figure 12
A
(A) Elongated face in bilateral condylar dislocation. (B) OPG showing bilateral condylar dislocation.
Courtesy: Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore
Figure 13
A
(A) Normal facial appearance following reduction of the dislocated condyles. (B) Normal positions of the condyle in the glenoid
fossa after reduction. Courtesy: Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Mangalore
Clinical features
Inflammation of the retrodiscal tissues may lead to a forward and downward placement of the condyle thereby
producing a same-sided malocclusion of posterior teeth
and heavy contact in the anterior teeth of the opposite
side. Continuous pain is felt in the TMJ region, which
exacerbates on clenching.
250
Figure 14
Ely cyst in the mandibular condyle. Courtesy: Department of Oral Medicine and Radiology,
Manipal College of Dental Sciences, Mangalore
Osteoarthritis
Osteoarthritis is characterized by pain secondary to TMJ
synovial inflammation. It is usually seen in elderly and
relatively more frequently in women.
Clinical features
Almost all the patients report of a gradual onset of symptoms. The condition is self-limiting and even in the absence
of active treatment the symptoms subside over a period of
time, and the TMJ movements revert back to an acceptable
level.
Clinical features
Figure 15
Polyarthritides
Polyarthritides represent a group of disorders characterized
by inflammation of the articular surfaces of the joint. It
resembles osteoarthritis as it exhibits degenerative changes
in the articular cartilage and underlying bone along with
inflammation of the capsule and synovial tissues.
Clinically, tenderness may be elicited on TMJ palpation.
The TMJ area may exhibit swelling and erythema. The
patients may have limited function. Crepitus is a characteristic nding. Patients symptoms may aggravate with parafunctional habits. Radiographs reveal surface changes in
the glenoid fossa and attening of the articular eminence.
Traumatic Arthritis
Occasionally a major traumatic episode can result in inflammatory changes that ultimately lead to articular surface
changes. Patients may complain of restricted mouth opening and pain. On palpation a soft end feel is typically
evident.
Swelling, tenderness and rise in local temperature are characteristic of septic arthritis. Limitation in mouth opening
and deviation of the jaw to the affected side are other ndings (Figure 15). Affected side may reveal tender cervical
lymphadenopathy.
Synovial uid and blood studies will aid in the diagnosis. The commonly isolated organisms from a previously
normal TMJ are gonococcal species and from TMJ with
previous history of arthritis, Staphylococcus aureus has
been isolated. Untreated cases of septic arthritis can lead
to brain abscess, ankylosis and osteomyelitis of temporal
bone. Ankylosis and facial asymmetry may be a common
complication in children.
Rheumatoid Arthritis
Infectious Arthritis/Septic Arthritis
This form of arthritis is generally seen in patients with
previously existing joint disease or with underlying systemic illness. The highest incidence of septic arthritis is
seen in individuals on long-term immunosuppressive drugs
or corticosteroids.
Otherwise sterile articular surfaces and joint spaces
may become infected secondary to blood borne bacterial
infection or extension of infection from adjacent sites such
as the molar teeth, middle ear and parotid gland.
Clinically patient gives a history of constant pain in
the TMJ region which typically aggravates with function.
252
Rheumatoid arthritis (RA) is a chronic inflammatory, autoimmune disorder, which may involve many of the diarthrodial joints (usually in a symmetrical fashion) in the
body characterized by persistent synovitis. Women are three
to four times more affected than men. The age of onset
varies between 25 and 55 years.
Though the exact nature of RA is still unknown, it is
believed that the inammation of the synovial membranes
extends into the surrounding connective tissues and articular surfaces (this reactive macrophage laden broblastic
proliferation from the synovium that extends to the joint
surface is called pannus), which then become thickened
and tender. The cells of the synovial membrane express
Figure 16
Clinical features
TMJ afflicted with RA may produce pain, joint stiffness,
limited mouth opening, joint sounds and open bite. The
reported prevalence of TMJ involvement by RA varies
widely from 4.7 to 88%.
The American Rheumatism Association (1987) laid down
guidelines for diagnosis of RA. According to these guidelines, the patient must present three or four of the following symptoms for more than 6 months: morning stiffness
for more than 1 hour; arthritis in three or more joints; arthritis in the hands; symmetrical arthritis; rheumatoid nodes;
presence of rheumatoid factor; radiographic alterations.
Laboratory investigations
The level of rheumatoid factor, erythrocyte sedimentation
rate, C-reactive protein, thrombocyte count and plasma
tumor necrosis factor- will aid in the diagnosis.
Radiographic features
The characteristic radiographic findings include generalized decreased density of bone, severe erosion of the condylar head (occasionally only the neck of the condyle may
be remaining), subchondral sclerosis, flattening of the condylar head, subchondral cysts and osteophyte formation.
In some individuals, the condyle may assume a sharpened
pencil shape owing to the erosion of the anterior and posterior condylar surfaces (Figure 16).
A modied grading system for the evaluation of TMJ
abnormalities based on the degree of bony destruction of
the mandibular condyle can be summarized as:
Psoriatic Arthritis
Psoriatic arthritis (PA) is present in about 57% of the
patients suffering from psoriasis. The diagnosis can be
made when patients present with erosive polyarthritis with
negative rheumatoid factor and psoriatic skin lesions are
seen long before TMJ is affected. The skin lesions are seen
long. This condition affects the fingers and spine along
with the TMJ. Eighty-five percent of the patients present
with pitting of the nails.
Clinical features
Unlike rheumatoid arthritis, the TMJ symptoms associated
with PA are unilateral. Patients present with pain over the
TMJ, limited mandibular movement and deviation of the
mandible to the affected side. Radiographically, the TMJ
changes are generally similar to those that are seen in
rheumatoid arthritis, however in some patients extensive
sclerotic changes may be evident.
Hyperuricemia
Gout is considered a true crystal deposition disease. Gout
may be described as a pathological response of the periarticular tissues to the presence of monosodium urate monohydrate crystals. Though a chronic state of hyperuricemia
is required for the development of gout, it alone is not sufficient to predispose to gout. It is estimated that 95%
of hyperuricemic individuals may not develop gout. Gout
commonly affects the first metatarsophalangeal joint (50%)
and other joints such as the ankle, knee, wrist, elbow and
the TMJ.
Clinical features
Crystal deposition may be seen in tissues adjacent to the TMJ.
Occasionally, monosodium urate crystals may be evident
in the synovial fluid aspirate.
TMJ ANKYLOSIS
Ankylosis is an intra-articular condition where there is
fusion between the bony surfaces of the joint, the condyle
and the glenoid fossa. The term ankylosis is derived from
253
Type II: Bony fusion on the outer edge of articular surface, but no fusion on the deeper aspect of the joint.
Type III: A bridge of bone exists between the ramus
and zygomatic arch. The upper articular surface and
the articular disk on the deeper aspect are still intact.
Medially, a displaced atrophic condyle still exists and
which is functional. Type III ankylosis results from a
fracture-displaced condyle, compared to the crushing
types of condylar injuries as in types I and II.
Type IV: Total TMJ obliteration between ramus and
skull by large bony mass. It is the most common type.
Figure 17
Clinical features
The clinical features of ankylosis depends on:
Intraoral features
Occlusal cant with deviation of maxillary and mandibular midlines toward affected side.
Class II Angle malocclusion present on the affected
side with unilateral crossbite on the opposite side.
The mouth opening is restricted: amount of opening
depends upon degree of ankylosis.
Clinical features of bilateral ankylosis
Facial features
Symmetrical defect.
Retrognathic mandible with a short ramus and a small
body.
Often microgenia, small chin.
Bird-face deformity (Figure 17) or Andy Gump facies.
Convex profile.
Intraoral features
Figure 18
Management
The goals of management should include restoration of
mouth opening and joint function, facilitation of condylar
growth, correction of facial profile and to relieve upper
airway obstruction.
Surgical correction of ankylosis is best achieved by
condylectomy, gap arthroplasty, coronoidectomy, interpositional arthroplasty (with autogenous or alloplastic grafts)
and secondary procedures such as orthognathic surgery
and distraction osteogenesis. Surgical correction should be
followed by active physiotherapy.
When ankylosis is left untreated it may result in abnormal facial growth and development, speech defects, nutritional impairment, respiratory distress syndrome, conditions
related to poor oral hygiene and psychological impact on
the patient.
Figure 19
Usually removal of the cause and resting the affected muscle is sufficient. Moist heat fomentation will aid in hastening the recovery process. Patient should be advised not to
overuse the affected the muscle. Muscle relaxants can be
used for a short period of time.
Myositis
Inflammation of the muscle that results from a local cause
is referred to as myositis. The causes of myositis include
traumatic injuries, muscular strain and orodental infections.
Chronic Conditions
Myofascial Pain
Secondary findings
Possible findings
TMJ pain
Joint sounds
Inflammation
Restricted range of motion unmodified by assisted
opening/vapocoolant
Hypertrophy
Myalgia secondary to systemic disease.
Other reported symptoms include: musculoskeletal symptoms (fatigue, stiff joints and swelling), neurological symptoms (tingling, numbness, blurred vision, muscle twitching
and excessive lacrimation), and otologic symptoms (ear
pain, tinnitus, diminished hearing, dizziness and vertigo).
Other symptoms that patients report of include: dentinal
hypersensitivity and cutaneous hypersensitivity.
Referral pattern for myofascial pain
1.
2.
3.
4.
Management
The management of MFP requires a multi-pronged
approach. In the short term, the aim is to abolish the TB,
TrP and TS for pain relief. In the long-term, achieving
muscle flexibility and eliminating associated precipitating
factors is the objective.
Counseling Patient should be counseled about the nature
of the condition and encouraged to discontinue parafunctional habits and consume soft diet.
Physiotherapy Physical modalities are useful supplementary treatments that help in controlling muscle pain and
spasm. Moist heat application to the affected site increases
blood flow and increases vascularity, resolution of inflammation and fibrosis. It also increases flexibility of connective tissue and decreases muscle spasm and pain. However
moist heat fomentation provides superficial heat with limited
subcutaneous penetration. Ultrasound, on the other hand,
provides deep heat with higher subcutaneous penetration.
Trigger point therapy Spray and stretch technique and
local injections are used.
Refrigerant spray such as ethyl chloride or uoromethane
can be sprayed onto the skin surface from a distance of
about 18 inches at an angle of 30. The skin surface should
be stretched manually during the spray. The spray anesthetizes the site and facilitates the patient to stretch the
258
Muscular Hypertrophy
Enlargement or hypertrophy of the masticatory muscles is
a relatively rare condition. The hypertrophy is clinically
evident as a localized firm non-tender swelling along the
body of the affected muscle.
Legg was the rst to describe bilateral enlargement of
the masseter and temporalis muscles. However, literature
review reveals the description of muscular hypertrophy of
the masseter, temporalis and the pterygoid.
Masticatory muscle enlargement can be either congenital
or acquired. Usually, the acquired form is a result of parafunctional jaw habits such as bruxism or masticatory
hyperfunction (chewing hard food, chewing gum). Some
authors believe that emotional stress may result in chronic
forceful clenching of the jaws and bruxism, resulting in
hypertrophy of the muscle (Figure 20).
Muscular hypertrophy should be differentiated from conditions such as lipomatosis, vascular tumors, liposarcoma,
rhabdomyosarcoma and inltrative leukemia and lymphoma.
MRI and ultrasound will help in revealing the homogeneous
enlargement of the muscles.
Figure 20
Fibromyalgia
Fibromyalgia is a syndrome characterized by chronic widespread musculoskeletal pain, stiffness, non-restorative sleep,
and fatigue. Occasionally, many of the patients may mimic
patients of chronic fatigue syndrome. The characteristic feature of fibromyalgia (FM) is the presence of non-inflammatory muscle and connective tissue tender points that are
widespread, involving all four quadrants of the body.
Figure 21
Figure 22
Back
Front
Occiput
Trapezius
Lower cervical
Supraspinatus
2nd rib
Gluteal
Lateral
epicondyle
Greater
trochanter
Knee
Malignant tumors such as chondrosarcoma, synovial sarcoma, brosarcoma, osteogenic sarcoma and multiple
myeloma have been reported to affect the TMJ structures.
Distant metastasis to the TMJ have been reported to occur
from primaries in the breast, lungs, prostate, liver, uterus,
pancreas and rectum.
The clinician needs to be aware of these uncommon
neoplasms as they tend to mimic the clinical features of
other common temporomandibular disorders such as limited
jaw movements, restricted mouth opening, deviation or
deection during mouth opening and joint sounds. As the
condition advances it may result in extension of the tumor
mass into the middle cranial fossa or invade posteriorly
through the glenoid fossa and present as a lesion of the
middle ear or external auditory canal. When the tumor mass
extends laterally it can produce a preauricular swelling mimicking a parotid gland tumor. Cranial nerves may be involved
if the tumor mass extends into the infratemporal and pterygopalatine fossae.
Clinical features
Figure 23
A
Figure 24
Multiple myeloma and occasionally a solitary plasmacytoma may affect the TMJ in males over the 6th decade of
life. Patients usually present with pain and limited mouth
opening. Large tumors may exhibit pre-auricular swelling.
Radiographs reveal well-dened punched-out radiolucent
areas without a sclerotic rim. Occasionally pathological
fractures may be seen.
Metastatic tumors Metastasis to the jaws is rare and the
condyle is almost never a location. However, isolated
reports have described TMJ dysfunction and pathologic
fracture as the presentation features. There have been various theories suggested to explain the cause for very less
chances of metastasis to the condyle. Some of these include
lack of abundant red marrow, relatively sluggish pace of
blood flow, separate vascular supply from the circular penetrating branches of the maxillary and superficial temporal
arteries and an osseous plate that cuts off the condylar marrow cavity from the spongiosa of the rest of the mandible.
Synovial chondromatosis is an idiopathic condition,
which has been one of the more commonly reported conditions associated with loose joint bodies. It is thought to
represent a cartilaginous metaplasia associated with an
abnormal synovium, rather than a true neoplasia. It presents with multiple cartilaginous nodules located within
the joint space. Symptoms include joint swelling, pain,
limited movement and joint noise.
CONDYLAR FRACTURES
Condylar fractures are the most common among fractures
involving the mandible. It is estimated that of all the mandibular fractures approximately 2552% involve the condyle (Figure 25).
Lindhal in 1977 described three major sources of condylar injuries:
1.
2.
3.
262
Figure 25
Figure 26
Intracapsular
fracture
Extracapsular
fracture
Subcondylar
fracture
Figure 27
Figure 28
264
Developmental Disturbances
Non-inflammatory Conditions of
Salivary Glands
Benign Tumors
Pleomorphic Adenoma (Benign Mixed Tumor)
Canalicular Adenoma
Basal Cell Adenoma
Papillary Cystadenoma Lymphomatosum
(Warthins Tumor)
Oncocytoma (Oxyphilic Adenoma)
11
Sialadenosis
Anorexia/Bulimia-related Sialadenosis
Sialadenosis Associated with Alcoholic Cirrhosis
Diabetes Mellitus
Medication-induced Sialadenosis
Orofacial Granulomatosis
Sarcoidosis
CHAPTER
Malignant Tumors
Mucoepidermoid Carcinoma
Intraosseous Mucoepidermoid Carcinoma
Acinic Cell Adenocarcinoma
Malignant Mixed Tumor (Carcinoma Ex Pleomorphic
Adenoma)
Metastasizing Mixed Tumor
Adenoid Cystic Carcinoma
Polymorphous Low-Grade Adenocarcinoma
Salivary glands and saliva play a critical role in maintenance of oral and systemic health. Salivary glands are frequently involved in a wide array of conditions which
result in glandular dysfunction. These may be subdivided
into five categories which include:
1.
2.
3.
Developmental disturbances
Saliva and salivary flow alterations
Inflammatory conditions
4.
5.
Non-inflammatory conditions
Tumors.
Many of these conditions result in salivary gland hypofunction, enlargement, pain and facial nerve paresthesia
that will prompt the patient to seek evaluation and treatment from the dentist. This chapter will address each of
these five subgroups focusing on the etiology, clinical features, diagnosis and treatment of these conditions.
265
DEVELOPMENTAL DISTURBANCES
Aplasia/Agenesis and Related Aberrancy
of Salivary Glands
Congenital aplasia or agenesis of the major salivary glands
is an uncommon finding, characterized by partial or total
lack of development of the gland. Agenesis may involve
one gland, pairs, or multiple glands, and be unilateral or
bilateral. It may be a single independent finding or be
associated with other developmental anomalies.
Congenital agenesis of major salivary glands was rst
reported by Gruber in 1985. A recent search of the world
literature reported 30 documented cases. Three cases of
unilateral aplasia of the parotid have been reported. As it
may be asymptomatic and go unnoticed, the true incidence
is unknown. Bilateral agenesis is more common, with
10 cases reported in the English literature. Sometimes congenital absence of one parotid or submandibular gland is
associated with hypertrophy of the contralateral gland.
Aplasia may occur as a single event or with autosomal
dominant inherited developmental conditions such as rst
branchial arch anomalies, hemifacial microsomia and mandibulofacial dysostosis. Parotid gland aplasia may be associated with malformations of the lacrimal apparatus as
well. A combination of multiple developmental anomalies
is found in the lacrimo-auriculo-dento-digital (LADD)
syndrome (LevyHollister syndrome). This is an autosomaldominant multiple congenital anomaly disorder characterized by hypoplasia, aplasia or atresia of the lacrimal and
salivary systems, ear anomalies, hearing loss, digital malformations and dental alterations. As the parotid gland
develops during the 4th week of uterine life, and the submandibular and sublingual, and minor glands develop
between 6th and 12th weeks, association of salivary gland
aplasia with other congenital abnormalities is easily
understood. Bilateral aplasia of the parotid gland has also
been reported in a patient with Downs syndrome. In ectodermal dysplasia, aplasia of the submandibular glands and
alterations in salivary gland function have been reported
as well.
Clinical features and diagnosis
Salivary gland agenesis may be asymptomatic if only partial agenesis occurs or if the condition is isolated to one
gland. If more extensive involvement occurs, agenesis can
produce profound hyposalivation in children resulting in
advanced dental caries, candidiasis, ascending sialadenitis, and even laryngitis and pharyngitis. A child with subjective xerostomia and functional hyposalivation should
be carefully examined for salivary gland dysfunction to
include partial or complete salivary gland agenesis. Familial parotid gland aplasia has been reported, hence examination of the siblings of a child with agenesis, might be
266
staining for cell proliferative activity demonstrated no statistically significant differences among adenomatoid hyperplasia and a matched group of normal palatal salivary
glands. The conclusion was that adenomatoid hyperplasia
had limited growth potential.
Treatment
To determine the true nature of a clinical enlargement, a
biopsy is necessary. After the diagnosis of adenomatoid
hyperplasia has been established via histopathologic examination, no further treatment is indicated.
Figure 1
Table 1
Parotid
gland
Sublingual
gland
Submandibular
gland
Glands
Type of secretion
Parotid
Purely serous
Submandibular
Mixed, however
predominantly serous
Sublingual
Mixed, however
predominantly mucous
Primarily mucous
Purely serous
Mixed
Purely mucous
267
Box 1
Medications
Radiation to head and neck
Systemic diseases
Sjgrens syndrome
Sarcoidosis
Systemic lupus erythematosus
Primary biliary cirrhosis
Viral infections
HIV
Hepatitis C
Box 2
Tricyclic antidepressants
269
The mucositis will slowly resolve 23 weeks after cessation of the treatment. A loss of taste has been reported
which generally is recovered after 6 months.
Minimizing the probability of osteoradionecrosis (ORN)
includes a dental examination at least 2 weeks prior to the
initiation of radiation therapy to address or remove teeth
that have a hopeless long-term prognosis. Daily uoride
treatments in custom carriers and close follow-up aid in
reducing the incidence of xerostomia-induced dental caries.
Due in part to more efcient radiation techniques, the incidence of ORN has been declining in radiation patients over
the last two decades. Advances in radiation techniques,
including the use of fractionated radiation doses, have
minimized the incidental damage to adjacent tissues. Furthermore, use of three-dimensional dosimetric intensitymodulated radiation therapy (IMRT) has been shown to
reduce late salivary toxicity, since the portion of tissue
exposed to low radiation doses has a potential for repair.
Based on recent publications, the prevention of ORN remains
controversial. A recent report compiled by Chang et al after
reviewing a large series of ORN studies stated that extraction of teeth with poor prognosis before radiation therapy
did not appear to reduce the risk of ORN. The investigation
of IMRT by Wu et al to achieve sparing of the parotids and
yet achieve higher tumor control appears to show promise.
Until additional evidence is available to dene guidelines,
a pre-radiation referral for a dental evaluation is necessary.
To facilitate prevention of ORN, irradiated dental patients
should maintain a high level of oral health. Pre- and posttherapy close collaboration by a multidisciplinary team
can be invaluable for patients receiving head and neck
radiation therapy.
Sjgrens Syndrome
Sjgrens syndrome (SS) is a chronic autoimmune disease
affecting the exocrine glands, primarily the salivary and
lacrimal glands. Patients most commonly complain of a
subjective persistent feeling of dry mouth (xerostomia)
and of dry eyes (keratoconjunctivitis sicca). This is due
to lymphocytic infiltrates and destruction of salivary and
lacrimal glands and systemic production of autoantibodies. In 1933, Henrik Sjgren, a Swedish ophthalmologist,
presented his doctoral thesis entitled Zur Kenntnis der
Keratoconjunctivitis Sicca, and described the clinical
and histopathological aspects of the disease.
Sjgrens syndrome occurs worldwide and while it
may occur at any age, the peak incidence is between 40
and 50 years. Sjgrens syndrome has one of the highest
female-to-male ratio (9:1) of any autoimmune rheumatic
disease. In addition to ocular and oral dryness, a wide
spectrum of extraglandular manifestations may occur as
well. The musculoskeletal, hematological, vascular, pulmonary, gastrointestinal, dermatological, renal and nervous
systems may be involved. Patients with SS have an increased
Box 3
diseases and co-association of multiple autoimmune diseases has been reported by Becker et al. Reports have
also indicated that a SS proband may have relatives with
other autoimmune diseases in approximately 3035% of
the cases. Assessing human leukocyte antigen (HLA)-DR
and HLA-DQ gene segments in patients with SS reveals an
increased use of haplotypes B, Drw52 and DR3. Correlations
271
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 8
A
Sjgrens syndrome. CT images showing diffuse sialadenitis affecting the submandibular and parotid glands bilaterally.
Courtesy: Dr Madhu Nair
274
Figure 9
A
Chronic Sjgrens syndrome. Contrast CT demonstrating a granular appearance of parotid glands that have been
reduced in size. Courtesy: Dr Madhu Nair
Figure 10
Figure 11
A
Sjgrens labial salivary gland histopathology (5 and 40). Courtesy: Dr Carol Stewart.
(A) Magnification 5; (B) magnification 40
includes minimizing carbonated drinks and avoiding snacking on sticky sugary processed foods. Salivary stimulants
such as sugar-free gums and lozenges will assist in enhancing the salivary ow. If available, xylitol sweetened gums
and lozenges are benecial due to their reported anticariogenic effects. Prescription sialogogues, such as pilocarpine
and cevimeline can be very helpful as long as functional
salivary gland tissue remains. However, prescription sialogogues are not recommended for all SS patients. These
should not be used in patients with uncontrolled asthma
or narrow-angle glaucoma, and should be used with caution with certain types of cardiovascular disease, eye, lung,
and liver conditions. Consultation with the patients rheumatologist before prescribing these medications is prudent
to conrm the lack of contraindications. Not only are SS
patients more susceptible to dental decay and candida,
their periodontal status should be monitored as well. While
some reports indicate no signicant difference between
the periodontal status of SS patients and controls, others
report more severe periodontitis in SS patients. Patients
should be given all assistance possible to maintain their
dentition in the optimal condition. As many of these
patients are taking bisphosphonates for osteoporosis, the
option of implant replacements for lost teeth, or implant
supported dentures is one that should be approached with
utmost care and patients informed consent. The potential of
bisphosphonate-associated osteonecrosis should be reviewed
with the patient prior to extractions, implants, or any oral
surgical procedures.
While SS often follows an indolent course, of critical
importance is the concern for development of lymphoma.
Kassan et al reported that SS patients have 40 times higher
risk of development of lymphoma than the normal population. Lymphoma has been reported by Voulgarelis et al
Figure 12
Mikulicz actually reported the first case of MALT lymphoma, based on the published histopathology. The authors,
Ihrler and Harrison further urged that the terms Mikuliczs
disease and Mikuliczs syndrome should no longer be used.
Conversely, a recent report by Yamamoto et al concluded
that Mikuliczs disease is a distinct entity and different
from SS both clinically and histopathologically. Additionally, the authors reported that Mikuliczs disease was an
IgG4-related systemic disease.
Treatment
The affected gland must be surgically removed. Fortunately,
most MALT lymphomas are low-grade tumors that tend to
remain localized with good survival rates. Occasionally,
tumors transform to high-grade lymphomas with aggressive
behavior.
Sialorrhea
Clinical features
Sialorrhea or ptyalism is a condition characterized by
increased salivary flow. Sialorrhea can occur with various
neurologic disorders, infections, the secretory phase of
the menstrual cycle, heavy metal poisoning, Wilson disease, paroxysmal sialorrhea, and rabies. Older aged individuals in chronic care facilities and chronically debilitated
with cerebrovascular accident may demonstrate chronic
drooling.
Parkinsons disease (PD), amyotrophic lateral sclerosis
(ALS), and cerebral palsy are neurodegenerative diseases
associated with sialorrhea. The appearance of excess saliva
in neuropathologic conditions may be due to excessive
saliva, but is usually related to impaired cerebral control
of orofacial function. Weakness of the facial and perioral
muscle tone inhibits the normal retention, movement,
and/or swallowing of saliva. Excessive salivation has been
reported in familial dysautonomia (FD) due to submandibular and sublingual salivary gland hyperactivity. These
changes may be the result of ongoing parasympathetic
denervation characteristic in FD. The consequences of
drooling are not restricted to medical issues, but can
cause major social handicaps. Severe psychosocial consequences and social stigmatization may be emotionally
devastating for patients and families. Drug-induced sialorrhea has been reported as well. Major medication groups
associated with drooling are anti-psychotics, particularly
clozapine, and direct and indirect cholinergic agonists that
are used to treat dementia of the Alzheimers type and
myasthenia gravis. Other drugs cited include risperidone,
lithium and digoxin. Heavy metal toxins, such as mercury
and thallium produce sialorrhea as does exposure to irreversible acetylcholinesterase inhibitors such as insecticides
and nerve agents.
278
INFLAMMATORY CONDITIONS OF
SALIVARY GLANDS
Inflammatory conditions are the most common pathology affecting the salivary glands. Dentists should be familiar with their clinical manifestations and recommended
treatment.
Mucocele
The mucocele is a common lesion that results from rupture
of a salivary gland duct and spillage of mucin into the
surrounding tissues. For that reason, the term mucus extravacation phenomenon is used to describe this lesion. The
rupture of the gland or duct may be due to local trauma,
but many cases develop without a history of trauma. These
will be found most frequently in the lower lip.
Clinical features
Mucoceles typically present as fluctuant, non-ulcerated
dome-shaped mucosal swellings that range from 2 mm to
several centimeters in size. Mucoceles have been reported
in patients of all ages. The lesions typically have a bluish
translucent hue due to the spilled mucin under the tissue
surface (Figure 13). Deep mucoceles may appear normal in
color, and may feel firmer to palpation than superficial
ones. Duration may be days to years. Patients will often
report that the lesion intermittently gets larger, and then
shrinks. This history is consistent with the nature of the
lesion which will enlarge, sometimes during eating, and
then spill contents into the surrounding tissue, and eventually shrink in size. The most common location is the
lower lip, but mucoceles may also be found in the buccal
mucosa, anterior ventral tongue and floor of the mouth
(ranula).
Ranula
Clinical features
Ranula is the term used for mucoceles that occur in the
floor of the mouth in association with ducts from the submandibular or the sublingual gland. Generally these are
larger than mucoceles occurring in other locations and can
elevate the tongue. The ranula is usually located lateral
to the midline and appears as a dome-shaped fluctuant
swelling in the floor of the mouth as seen in Figure 14.
The color may be translucent blue or normal in color if
deep seated. A rare plunging type that has herniated
through the mylohyoid muscle has been described by
Davison et al.
Histopathology
Histopathology
Figure 13
Treatment
Treatment of a ranula includes unroofing the lesion, excision of the lesion, or removal of the sublingual gland.
Figure 14
279
Figure 15
Clinical features
Sialoliths are calcified bodies that develop within the salivary gland or ductal system. These are one of the most
common salivary gland conditions. These are believed to
develop from deposition of calcium salts around a focus
of material within the duct lumen. Solitary or multiple sialoliths can form. The initiating focus may consist of desquamated epithelial cells, bacteria, foreign bodies, or mucus.
Sialoliths most commonly occur in the submandibular
gland (8090%), but may develop in the parotid and sublingual gland as well. Multiple stones are more common in
the parotid glands than other major glands. Minor gland
calculi are occasionally seen in labial glands and buccal
mucosa. These usually form in young and middle-aged
adults, but may develop at any age. The classic presentation is an intermittent postprandial salivary gland swelling that gradually subsides over the next 23 hours.
Patients report moderately severe pain, just before, during
and after meals due to stimulation of salivary flow and the
pressure produced against the occluded duct. Recurrent
and chronic obstruction causes stasis, inflammation, and
infection, which can result in persistent enlargement.
Sialoliths may be round or elongated and measure from
2 mm to 2 or more centimeters in diameter. The involved
duct may contain a single stone or multiple stones. Upon
excision and gross examination, these appear yellow in
color. The cause is uncertain, but sialolith formation can
be promoted by chronic sialadenitis and partial duct
obstruction.
Figure 16
Diagnostic procedures
Conventional radiographs can successfully image most
sialoliths as they appear as radiopaque masses. Smaller
sialoliths that are not fully calcified pose a diagnostic challenge. Underexposed radiographs can sometimes demonstrate the presence of the sialolith. Occlusal films help
identify stones in the submandibular gland as shown in
Figures 15 and 16. If a panoramic film is generated, multiple calcifications may be identified as shown in Figure 17.
It is possible for multiple calcifications to appear superimposed on the mandible and mimic a bony lesion. A sialograph of Whartons duct depicts ductal enlargement
proximal to the sialolith in Figure 18. However, Rabinov
and Weber report that up to 20% of salivary calculi are
radiolucent. If not calcified, the stones may not be evident
on these films. Non-contrast CT is often considered the
best single modality for the diagnosis of calculi. Most stones
not seen on conventional radiographs can be detected by
CT. Sialographs are useful in detecting non-calcified sialoliths, but sialography is an invasive procedure with potential to dislodge the stone deeper into the gland, as well as
280
facilitate retrograde spread of any existing bacterial infection as a result of stasis. In addition, sialography can cause
increased pain when acute sialadenitis exists. Dormant
infections can get worse after sialography; thus necessitating the use of antibiotics. Contrast CT examination, on
the other hand, can image non-calcified sialoliths if these
are larger. In Asia and Europe, ultrasound imaging is the
mainstay of imaging.
Figure 17
Sialadenitis
Figure 18
Non-specific Sialadenitis
Histopathology
The histopathologic features include a calcified mass that
exhibits concentric laminations surrounding a central focus
of amorphous debris. The associated duct will demonstrate
squamous, oncocytic or mucous cell metaplasia.
Treatment
Small stones may be removed by massaging and manipulation to move the stones toward the duct orifice or peripheral/
transoral ductotomy. Large stones may require excison of
gland and/or associated duct. Lithotripsy has been tried with
limited success. Sialoendoscopy provides a new minimally
Non-specific sialadenitis may manifest as chronic parotitis, also called chronic recurrent parotitis. The cause of the
parotid gland enlargement may be multifactorial and
include decreased salivation either from decreased production or decreased secretion, stasis and an ascending
retrograde infection. Sometimes, no predisposing factor is
identified. This entity is usually unilateral, painful (mild to
severe), and characterized by intermittent exacerbations of
swelling and remission. Massaging or milking the gland
will reveal cloudy or purulent secretions. In a large series,
Bhatty et al reported that the mean age was 46 years,
with mean duration of symptoms of 4.6 years. The swelling may last for several hours or several weeks. The condition establishes a cycle of blockage and infection, which
becomes self-perpetuating. Fever and malaise may be
present. Remissions last from weeks to years. The extent of
the gland destruction may increase over time with each
episode as does the pain intensity. The disease tends to
progress and may lead to the formation of a fibrous mass
in the affected gland.
Diagnostic imaging
Sialography will demonstrate the parotid duct system. A
sausage-like pattern reflects areas of duct wall dilatations
and stricturing that result from the effects of the ascending bacterial infection. Because sialography may result in
retrograde spread of infection into the gland, other imaging modalities are preferred. CT scan may show increased
density due to the normal parotid gland fat being replaced
by inflammation and fibrosis. Contrast CT will show
enlargement of the gland and its duct in the absence of
281
a causative agent such as a sialolith. Figure 19 shows unilateral gland and duct enlargement of the right submandibular gland. Figure 20AC show non-specific sialadenitis
in the right parotid and submandibular glands.
Histopathology
The histopathology is consistent with non-specific sialadenitis with marked infiltrate of inflammatory cells. The
histology may show mild chronic sialadenitis or widespread
Bacterial Sialadenitis
Figure 19
Figure 20
A
Non-specific sialadenitis noted in the right parotid and submandibular glands. Courtesy: Dr Madhu Nair
282
Histopathology
Treatment
Diagnosis
A history of preceding events may aid in making the diagnosis. Predisposing factors can include dehydration, malnutrition, immunosuppression, dental infection, anticholinergic
medications, tracheotomy, sialectasis, ductal obstruction,
neurosurgical, and abdominal surgical procedures.
Treatment
Treatment for acute sialadenitis includes appropriate antibiotic therapy based on culture and sensitivity findings.
Rehydration is important to improve salivary flow. If an
abscess has formed, surgical drainage may be required. If
the condition is severe, surgical removal of the gland may
be necessary. In debilitated patients, the condition may be
fatal due to spread of infection and sepsis. Prompt recognition by the dentist with appropriate referral otorhinolaryngologist for definitive treatment is essential for a
good outcome.
Cheilitis Glandularis
The term cheilitis glandularis was first used by Volkmann
in 1870 to describe a disorder that presented with a
chronic, suppurative inflammation of the lower lip characterized by swelling of the mucous glands, dilated openings, and mucopurulent discharge. Cheilitis glandularis is
an uncommon inflammatory condition of the minor salivary glands, and most commonly affects the lower lip of
adult males. The etiology is unknown, however associated
factors have been suggested. These factors include tobacco,
poor oral hygiene, bacterial infections, possibly heredity,
and actinic damage.
Clinical features
Cheilitis glandularis most commonly affects the lower lip,
but it has been reported in the upper lip, palate, and buccal
mucosa. Affected individuals experience swelling and
eversion of the lower lip as a result of hypertrophy and
inflammation of the glands as seen in Figure 21. The openings of the minor salivary ducts are inflamed and dilated,
and pressure on the glands may produce mucopurulent
secretions emanating from the ductal openings. The condition most frequently occurs in middle-aged men, but
women and children have been reported as well.
Historically, cheilitis glandularis has been classied into
three types, based on the severity of the disease. These are
(i) simple, (ii) supercial suppurative (Baelzs disease), and
(iii) deep suppurative (cheilitis glandularis apostematosa).
The supercial suppurative type demonstrates painless
crusting, swelling, and induration of the lip with supercial and deep ulceration. This type seems to be the result of
secondary infection of the simple type. The deep suppurative type, also known as cheilitis glandularis apostematosa,
myxadenitis labialis, or cheilitis glandularis suppuritiva
profunda, is a deep-seated infection associated with abscess
formation and spontaneous expression of suppurative material from the ducts. The latter two types represent progressive stages of the diseases with bacterial involvement
and demonstrate increased inammation, suppuration, and
ulceration of the lip.
283
Figure 21
Histopathology
Histopathologic features consist of non-specific chronic
inflammation, dilated secretory ducts, dilated ducts containing mucin, areas of fibrosis, and areas of chronic sclerosing sialadenitis. The ductal lining may show oncocytic
metaplasia and atrophy of the acini. Concomitant dysplastic changes may be seen in the surface epithelium.
Diagnosis
The differential diagnosis for cheilitis glandularis would
include orofacial granulomatosis and multiple mucoceles.
Orofacial granulomatosis, which will be described in the
next section, is a non-tender, persistent swelling of the lips
usually with oral ulcerations. Histologic findings would
include non-caseating giant cell granulomas. Definitive
diagnosis of cheilitis glandularis requires a biopsy along
with the clinical picture.
Treatment
The treatment for cheilitis glandularis may vary depending on the severity of the condition. It may be treated with
lip balms, sunscreens, topical steroids, intralesional steroids, systemic antihistamines, and/or antibiotics. If conservative therapy fails, surgical resection or vermillionectomy
may be indicated. A significant percentage of cases of deep
suppurative type have been associated with the development
of squamous cell carcinoma of the overlying epithelium.
Because actinic damage has been implicated in many cases of
cheilitis glandularis, malignant degeneration could be associated with susceptibility to environmental factors, especially sun damage versus considering cheilitis glandularis
284
Figure 22
Necrotizing Sialometaplasia
Clinical features
Necrotizing sialometaplasia is an uncommon, benign, but
locally aggressive inflammatory lesion of salivary gland
tissue which both clinically and histologically may mimic
a salivary gland malignancy. In 1973, it was described by
Abrams and colleagues as a reactive necrotizing inflammatory process of the minor salivary glands of the hard
palate. While the etiology is unknown, the prevailing theory is that local ischemia of the salivary tissue leads to
local infarction. Most patients are in the 4th or 5th decade,
but the lesion has been reported in all ages, except children. In a large series as reported by Brannon et al, males
were affected more often than females, and whites were
affected more commonly than African-Americans by a
ratio of 5:1. Most lesions occur in the posterior hard palate, usually unilateral (Figure 22). Bilateral and midline
lesions may occur as well. Other intraoral sites such as the
retromolar pad, buccal mucosa, lower lip, and tongue have
been affected. Early lesions may present as a non-ulcerated
swelling in the posterior palate with or without pain.
Within 23 weeks, the necrotic tissue sloughs and leaves
a crater-like ulcer ranging from 1 cm to more than 5 cm.
Commonly, patients do not seek treatment until the ulcer
occurs. They may report a feeling of fullness in the area,
prior to the ulceration. Pain may or may not be a complaint
even though the ulcer may be quite large.
Diagnostic imaging
Diagnostic imaging
A palatal soft tissue attenuation focus with no characteristic appearance may be noted on CT and MRI. Osseous
changes are not noted. Lesions in the parotid are also sometimes seen. Ultrasonography may detect multiple foci of
hypoechoic nature within the parotids. Contrast CT demonstrates high attenuation areas within the parotids while
MRI can clearly show well-delineated masses that are hypointense on T1 and isointense on T2 weighted contrasted
images. Findings could resemble those of benign tumors
such as pleomorphic adenomas. Alternatively, a more diffuse margin on CT can be misconstrued for a malignant
tumor. The condition can also appear in other locations
within the sinonasal and upper aerodigestive tracts.
Histopathology
Biopsy is necessary to confirm the diagnosis and rule out
malignant disease. The histopathology includes ulcerated
mucosa, pseudoepitheliomatous hyperplasia of the epithelium, acinar necrosis, and squamous metaplasia of salivary
ducts. Coagulation necrosis has been seen in early lesions.
Inflammatory cells may be found with fibrosis and granulation tissue. While mucous cells are necrotic, the lobular
architecture of involved glands is preserved. The lesion
can be misdiagnosed as squamous cell carcinoma or
mucoepidermoid carcinoma in the absence of an adequate
representative section of the lesion.
Treatment
The treatment is surgical removal.
Clinical features
When the disease affects the adult male, orchitis, inflammation of the testicles, is a complication approximately
20% of the time. The orchitis is usually unilateral, but can
occur bilaterally. Even with involvement of both testicles,
sterility is only a rare complication of orchitis. In adults,
dyspnea secondary to severe swelling of the salivary gland
which required a tracheostomy, has been reported by
Ishida et al. The mumps vaccine has been available since
1968, which has resulted in a marked decline in the incidence of the disease.
Treatment
Once the diagnosis has been established, no specific treatment is indicated. The lesion will heal by secondary intention with no intervention within 410 weeks.
Histopathology
The histolopathologic features consist of chronic sclerosing
sialadenitis, specifically chronic inflammation and fibrosis. It may be seen with sialolithiasis.
Histopathology
Treatment
The AIDS-related swellings reflect the lymphoid hyperplasia or lymphoepithelial cyst formation. The cyst wall may
contain germinal centers and a dense infiltrate of lymphoid cells.
Diagnostic imaging
Contrast enhanced CT or MRI is advised to help delineate
the internal architectural details. Cystic and solid tumors
can appear within the parotids. Bilateral enlargement is
noted on advanced imaging modalities with associated
cervical and nasopharyngeal lymphadenopathy. Adenoids
and tonsils may appear enlarged as well. However, reactive adenopathy may not be seen on these studies, owing
Figure 23
A
Axial and coronal T1 FLAIR MRI images showing cysts in the parotids bilaterally in an HIV-positive individual.
Courtesy: Dr Madhu Nair
286
NON-INFLAMMATORY CONDITIONS OF
SALIVARY GLANDS
Figure 24
Sialadenosis
Sialadenosis (sialosis) is a non-inflammatory, non-neoplastic, often recurrent condition which most commonly
manifests as a bilateral enlargement of the parotid and/or
submandibular glands. In a recent report of 65 cases of
sialadenosis as described by Satoh and Yoshirara, age
ranged from 19 to 71 years and the male:female ratio was
5:3. The salivary gland enlargements are usually slow
growing, may be unilateral or bilateral, and are sometimes
associated with pain. The condition is usually associated
with an underlying systemic disorder. These disorders
include diabetes, hypothyroidism, pregnancy, alcoholism,
malnutrition, obesity, anorexia nervosa, bulimia, and medications that affect the autonomic nervous system. Treatment depends upon identification of the underlying cause.
As patients with these conditions will commonly present
to the dental office, an understanding of the causes and
effective management strategies is essential for optimal
patient care.
Anorexia/Bulimia-related Sialadenosis
Clinical features
Eating disorders are not uncommon in young women who
seek to be thin or have a more ideal figure. These disorders
include anorexia nervosa and bulimia nervosa. Anorexia
involves deliberate lack of food intake and bulimia includes
binge eating with self-induced purging. It has been estimated that up to 19% of college-going women have bulimia.
Only 510% of people with bulimia are male. Sometimes
the frequent purging of acidic gastric fluids will produce a
characteristic enamel erosion on the lingual surfaces of
the maxillary anterior teeth (Figure 24). However, if measures are taken to neutralize the oral pH and maintain oral
hygiene, these signs may not be present.
Parotid sialadenosis has been reported to occur in
1066% of people with bulimia. Bilateral parotid gland
enlargement has been the presenting sign in some cases
and one report by Mignogna et al also included bilateral
palatal minor gland enlargements in addition to the parotid
gland involvement. Schlienger et al reported a case where
a 24-year-old female consulted physicians for 3 years for
treatment of a painless parotid swelling which was initially
confused with SS. Eventually, bulimia was conrmed.
Unilateral parotid gland and submandibular gland swelling may also occur in sialadenosis associated with bulimia, but less frequently. Bulimics may purge several times
per day. Emetics, diuretics, and laxatives frequently are
used as adjunctive agents for weight reduction, but may
not be revealed during a routine medical history review.
Pathogenesis
While the specific pathogenesis has not been determined,
it is generally accepted that multiple emetic episodes cause
an autonomic neuropathy. The enlargement is believed
to affect the autonomic innervation of the salivary acini,
causing disruption of the intracellular secretory cycle.
This may lead to excessive accumulation of secretory
granules, with marked enlargement of the acinar cells. Due
to sympathetic nerve dysfunction, zymogen (a precursor
of amylase) production and storage may increase. Individual acinar cells enlarge because of zymogen granule
engorgement.
Diagnosis
Because patients with bulimia and anorexia do not frequently disclose their condition, a broad medical evaluation will be required. The diagnostic assessment would
include tests to rule out SS (ANA, anti-SS-A/Ro, anti-SSB/La, rheumatoid factor, hepatitis C virus, complete blood
count [CBC], serum angiotensin-converting enzyme, immunoglobulin disorder [IgA, IgM, albumin, urine Bence Jones
protein], hepatic disorder [glutamic oxaloacetic transaminase and glutamic pyruvic transaminase], diabetes [fasting
blood glucose], and hypokalemia [serum electrolytes]).
Salivary secretion may be decreased or normal. Sialography may be helpful in establishing the diagnosis. Sialography may demonstrate a leaess tree pattern which is
thought to be caused by compression of the ner ducts by
the hypertrophic acinar cells. Splaying of the ducts within
the enlarged gland may be seen, with the ducts appearing
normal. In early stages of the disease however, there may
be no sialographic changes. CT scan of the parotids may
show a bilateral enlargement of the parotid glandular
parenchyma with increased glandular density, brosis
or fatty inltration that are characteristic of advanced
287
changes. CT is non-specic. The changes could be misconstrued for a lipoma; however, the absence of a brous
capsule is helpful in differentiating between these conditions; ultrasound and MRI are useful and non-invasive
approaches.
Histopathology
The histopathological report of a salivary gland biopsy
will reveal hypertrophy of the acinar cells, sometime 23
times greater than normal size. The nuclei are displaced to
the cell base, and the cytoplasm is engorged with zymogen
granules. Significant inflammation is not observed.
Treatment
Therapy will include psychological counseling with an
eventual goal of cessation of vomiting. This may result in
a gradual reduction of the salivary gland enlargement.
Pilocarpine hydrochloride drops have shown efficacy in
reducing parotid gland enlargement in bulimic patients. In
cases refractory to treatment, parotidectomy may be considered to improve unacceptable esthetics in patients with
bulimia.
288
Treatment
Treatment should focus on the underlying liver disease as
well as the oral complications. When the liver disease is
managed, the parotid glands may reduce somewhat in
size. In addition to sialadenosis, the dentist should also be
aware of salivary changes that increase caries risk susceptibility. The parotid gland flow rate in patients with alcoholic cirrhosis has been reported to be less than flow rates
in healthy controls. Diminished buffer capacity and less
attention to oral hygiene will put the patient at increased
risk for dental decay. In addition, patients who consume
large quantities of wine will have increased risk for dental
erosion due to the erosive potential of wines due to their
acidity. The pH of wine is reported to range from 3.0 to 3.8
and the pH at which enamel dissolves is reported to be pH
of 5.0 to 5.7. Treatment will include patient education and
complete dental evaluation.
Diabetes Mellitus
Clinical features
Diabetes mellitus is a disorder of carbohydrate metabolism mediated through decreased production of insulin
or tissue insensitivity to the effects of insulin, resulting
in hyperglycemia. Two types of presentations are recognized, although overlap is seen. Type I is characterized by
a lack of insulin production and is commonly diagnosed
during childhood, and Type II may present in obese children and adults, due to decreased sensitivity to insulin.
The complications include kidney failure, blindness, neuropathies, and atherosclerosis. Oral symptoms may include
dry or burning mouth, gingival inflammation. Sialadenosis has been reported in diabetes mellitus. The sialadenosis is believed due to an autonomic neuropathy, as in
alcoholism and nutrition-induced sialosis. With autonomic disturbances in sympathetic innervation, alteration
in protein synthesis occurs. Cytoplasmic swelling develops
from engorgement by intracytoplasmic zymogen granules. As a result, the parotid acini may double in size
(hypertrophy) resulting in clinically obvious parotid gland
enlargement.
Diagnosis
Long-standing, bilateral, painless enlargement of the
parotid glands may be observed. These are soft in consistency and follow the outline of the parotid glands, without nodularity. Milking the parotid glands may reveal
fluid of normal volume and consistency. Lower resting
and stimulated saliva flow rates in diabetics versus normal
controls have been reported as well. Levels of calcium are
increased and salivary magnesium, zinc and potassium
are reduced.
Diagnostic imaging
CT scan may show enlarged parotid glands without masses,
but demonstrating decrease in density due to significant
fat infiltration of the gland.
Histopathology
The histopathology associated with parotid gland biopsies
of diabetic patients when compared with those from alcohol associated sialadenosis is characterized by an increased
number of lipid intracytoplasmic droplets in the acinar
and ductal cells, and abundant adipose infiltration in the
stroma.
Treatment
While proper glycemic control should be the treatment goal,
this does not result in a significant reduction in the parotid
enlargement. Cosmetic improvement may be obtained with
gland removal, but the riskbenefit ratio must be considered.
Medication-induced Sialadenosis
Medication-induced sialadenosis has been reported for
a number of medications that affect the autonomic nervous system. Antihypertensive agents, psychotropic drugs,
-adrenergic agents, and bronchodilators have been implicated. Mauz et al reported a case were valproic acid was
associated with bilateral parotid and submandibular gland
sialadenosis.
Orofacial Granulomatosis
Clinical features
Orofacial granulomatosis was first introduced in 1985
by Wiesenfeld and encompass a spectrum of clinical presentations that share common histopathologic findings
of non-caseating giant cell granulomas. The conditions
included in this category are MelkerssonRosenthal syndrome, cheilitis granulomatosa, Crohns disease, sarcoidosis, and tuberculosis. Clinical presentation will vary, but
the most frequent site affected is the lips. Patients present
with a non-tender, persistent enlargement of the upper or
upper and lower lips. When this presentation is combined
with fissured tongue and facial paralysis, the clinical condition is called MelkerssonRosenthal syndrome. Involvement of the lips alone is called cheilitis granulomatosa.
Signs and symptoms include edema, erosions, paresthesia
or taste alterations.
Histopathology and diagnosis
The diagnosis is confirmed upon characteristic histopathologic findings. Scattered aggregates of non-caseating
granulomatous inflammation are found, often surrounding vessels. Granulomas contain histiocytes and multinucleated giant cells. Special stains for fungal organisms and
acid-fast bacteria are negative. No foreign bodies or inclusions are identified in the tissue.
Treatment and prognosis
Upon receiving the histopathologic findings, the clinician
must determine the initiating cause. Treatments have included
intralesional corticosteroids, radiotherapy, sulfasalazine,
hydroxychloroquine sulfate, azathioprine, cyclosporine A,
methotrexate, metronidazole, and other antibiotics. Most
clinicians treat the lips with intralesional corticosteroids
with variable success. Spontaneous remissions occur as
well. The prognosis is variable as there is no clearly effective treatment to date.
Sarcoidosis
Sarcoidosis is an immune-mediated multisystem disease
of unknown origin, characterized by the presence of epithelioid non-caseating granulomas in the involved organs.
Sarcoid granulomas are commonly encountered in the
lungs, with lymph node involvement, but may be seen in
many other sites. Sarcoidosis may also affect the heart,
liver, spleen, bones, skin, eyes, lymph nodes, parotid glands
and the oral cavity and may first manifest in the oral and
maxillofacial region. The disease occurs worldwide and
may affect either gender. Geographically, there appears
to be a higher incidence among central and northern
Europeans. It usually arises in the 2nd to 4th decades of
life, with a slight female predominance. In the United States,
sarcoidosis primarily affects African Americans, who tend
to have more acute symptoms, extrapulmonary manifestations and higher mortality rate at an earlier age compared
to whites. The most prominent manifestation of the disease involves the lungs with acute dyspnea, cough, and
chest pain being common symptoms. Chest radiographs
reveal bilateral hilar lymphadenopathy, diffuse parenchymal infiltrates or both. Less commonly, sarcoidosis develops slowly over months or years, especially among whites.
In some cases, patients have no symptoms, and the disease
is discovered on routine chest radiographs. Mortality rates
are similar among races. Approximately, one-fourth of
those with chronic sarcoidosis die of respiratory failure as
reported by Keller. Skin lesions occur nearly 25% of the
time. These often appear as chronic, violaceous indurated
lesions that are termed lupus pernio and frequent the nose,
ears, lips and face. Symmetric elevated indurated purplish
plaques are also commonly seen on the limbs, back and
buttocks. Scattered, non-specific, tender erythematous nodules known as erythema nodosum, frequently occur on
the lower legs.
289
290
BENIGN TUMORS
Figure 25
Figure 26
Diagnostic imaging
Sialography demonstrates displacement of the ducts around
the benign tumor which itself is well delineated with
definitive margins (ball-in-hand appearance). CT shows a
similar picture, with a higher degree of attenuation within
the tumor mass that demonstrates homogeneous density.
A lobulated appearance is also not unusual. The margins
may appear vague if there is associated inflammation
or hemorrhage. Differentiation from a malignant lesion
becomes difficult. Occasionally, the mass appears with lower
attenuation similar to a cyst. A mixed appearance is also
noted if cystic change or necrosis exists. Bleeding within
the tumor mass can result in the presence of increased
attenuation foci within the gland. Dystrophic calcifications are not uncommon. Figure 27A, B demonstrates the
radiographic features of a persistent pleomorphic adenoma
on contrast CT. Figure 28 demonstrates an axial plain CT
scan of a pleomorphic adenoma of the parotid gland.
Figure 29 shows a parotid gland pleomorphic adenoma via
an axial CT scan heterogeneously enhancing post contrast.
MRI, on the other hand, can clearly demonstrate the benign
nature of the lesion with distinct margins. Most often, the
apparent lobulation noted in CT is not noted, with the
mass appearing solitary. The mass presents with a low T1
weighted and high T2 weighted intensity consistent with
291
Figure 27
A
Persistent but stable, known pleomorphic adenoma in the left prestyloid parapharyngeal space. Courtesy: Dr Madhu Nair
Figure 28
solid contrast enhancement. Recurrent pleomorphic adenomas may be multifocal, along with malignant tumors
such as the acinic cell carcinoma and oncocytoma.
Referral to a radiologist plays an essential role in diagnosis and localization of salivary gland tumors in the major
glands. Ultrasonography results in generation of hypoechoic
areas within a well-dened homogeneous mass. Detection
of deep lobe tumors is difcult using ultrasonography.
292
Figure 29
Histopathologic features
The pleomorphic adenoma is usually a well-circumscribed,
encapsulated tumor, composed of a mix of glandular epithelium and myoepithelial cells within a hyalinized, eosinophilic, mesenchymal stroma. The epithelium may form
ducts, cystic structures, and islands or sheets of cells. Myoepithelial cells often comprise a large percentage of the
Figure 30
Canalicular Adenoma
Clinical features
The canalicular adenoma is an uncommon tumor that
occurs almost exclusively in the minor salivary glands and
has a marked predilection for the upper lip. The buccal
mucosa is the second most common location. The tumor
appears in older adults and demonstrates a female predilection. The clinical presentation is a slowly growing painless mass that ranges from several millimeters to 2 cm.
It may be firm or fluctuant to palpation. The overlying
mucosa may be bluish or normal in color. It may be multifocal and have multiple nodules in the upper lip or
buccal mucosa.
Histopathology
The tumor is named so for its histologic pattern which consists of uniform columnar or cuboidal cells forming canallike ductal structures. The cells may appear in a double row
and enclose cystic spaces of varying size. The spaces are
filled with an eosinophilic coagulum, and the supporting
stroma is loose and fibrillar with fine vascularity. Large
cystic spaces may be created and the epithelium may demonstrate papillary projections into the lumina.
Treatment and prognosis
Treatment consists of surgical excision and the outcome is
very good. Recurrence is rare.
Figure 31
A
Warthins tumor as noted on a contrast CT study reveals a multiseptated predominantly cystic mass within the tail of the
parotid gland on the right side. It is intrinsic to the gland and well below the main trunk of the facial nerve and its
major branches. Courtesy: Dr Madhu Nair
forms papillary projections into cystic spaces. The epithelium is supported by a lymphoid stroma which may demonstrate germinal centers.
Treatment and prognosis
The preferred treatment for Warthins tumor is surgical
removal. Malignant Warthins tumors, carcinoma ex papillary cystadenoma lymphomatosum, have been reported, but
are extremely rare.
MALIGNANT TUMORS
Adenocarcinomas of salivary gland origin are quite varied
in their histologic features and clinical behavior. These are
diagnosed based on their microscopic features. The parotid
is the most common site, followed by minor salivary glands.
In the parotid, the lesions are usually firm to palpation
and may produce damage to the facial nerve. The presence
of enlarged lymph nodes raises the suspicion of malignancy, but also occurs in inflammatory conditions. The
presence of nodal metastasis is a poor prognostic indicator. Intraorally, minor salivary glands of the hard palate
are the most common location for adenocarcinomas followed by buccal mucosa, lips and base of tongue.
Mucoepidermoid Carcinoma
Clinical features
Mucoepidermoid carcinoma is one of the most common
malignant salivary gland tumors. It was first described by
in 1945 by Stewart, Foote and Becker. It occurs in individuals from the second to the seventh decade. While it is
rarely seen in the first decade of life, it is the most common malignant salivary gland tumor in children. Some
295
Figure 32
Histopathology
Mucoepidermoid carcinoma is composed of cells from stratified squamous lines, mucous cell lines, and a third celltype, the intermediate cell. The mucous cells have abundant
foamy cytoplasm that stains positively with mucicarmine
stain. The epidermoid cells frequently demonstrate features
of squamous epithelium, intercellular bridges, but rarely
show keratinization. The intermediate cell is basaloid in
appearance and may be the origin of the other mucous and
epidermoid cells. A lymphocytic infiltrate is commonly seen.
Low-grade tumors show all cell types. Prominent cyst formation, a high proportion of mucous cells, and minimal
atypia are characteristic features. High-grade tumors consist of solid islands of squamous and intermediate cells,
which can show pleomorphism and mitotic activity. Few
mucus producing cells are present, which allow the
lesion to be confused with a squamous cell carcinoma. The
intermediate-grade tumor shows features that rank in
between the low- and high-grade tumors (Figure 33).
Treatment
Treatment may vary according to the tumor location, grade,
and staging. Surgical removal of the lobe or gland may be
adequate. High-grade or large tumors merit wider resection,
similar to that employed for squamous cell carcinoma. For
patients with evidence of metastatic disease or large highgrade tumors, radical neck dissection may be indicated.
Postoperative radiation therapy may be employed as well.
Prognosis
Patients with low-grade tumors and complete excision
have a good prognosis. Patients with high-grade tumors
have a poorer prognosis. In a large series reported by
Goode et al, most patients (75%) were tumor free after the
296
Figure 33
neck (43%), and lung (36%). The 5-year disease-free survival was 50%. Chemotherapy and radiotherapy were of
limited value.
298
Diagnostic imaging
Parotid lesions appear with well-defined margins while
minor gland masses have ill-defined margins. Retrograde
spread along facial or mandibular nerve is not uncommon.
Contrast MRI is the optimal imaging modality to visualize
such extension. Varying degrees of nerve involvement
Figure 34
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CHAPTER
Odontogenic Cysts
Gingival Cyst of Infants
Eruption Cyst
Dentigerous Cyst
Pseudocysts
Traumatic Bone Cyst
Aneurysmal Bone Cyst
Stafnes Bone Cyst
Odontogenic Keratocyst
Gingival Cysts of Adults
Inflammatory Cysts
Periapical Cyst
Residual Cyst
Paradental Cyst
Mandibular Infected Buccal Cyst
12
Non-odontogenic Cysts
Lymphoepithelial Cyst
Cystic Hygroma
Nasolabial Cyst
Parasitic Cysts
The word cyst is derived from Greek word kystitis meaning bladder. Kramer (1974) defined cyst as a pathological
cavity having fluid, semifluid or gaseous contents and
which is not created by the accumulation of pus. It is frequently, but not always, lined by the epithelium. Cysts may
arise due to trauma, inflammation and degeneration or
retention. They are called true cysts if lined by epithelium
and, pseudocysts (false) if not lined by epithelium. During
the initial stages, when the cysts are small they are usually
asymptomatic. Secondary infection may result in the
formation of abscess, cellulitis, osteomyelitis and subsequent sinus formation. As the cyst enlarges it may cause
Nasopharyngeal Cyst
Cysticercosis
Hydatid Cyst
Trichinosis
displacement of roots of teeth, resorption of roots, paresthesia, expansion of the cortical plates and eventually
result in pathologic fracture of the jaw.
CLASSIFICATION OF CYSTS OF
OROFACIAL REGION
1.
2.
3.
303
Figure 1
Developmental cysts
Odontogenic cysts
Gingival cyst of infants
Eruption cyst
Dentigerous cyst
Odontogenic keratocyst*
Orthokeratinized odontogenic cyst
Gingival cyst of adults
Lateral periodontal cyst and botryoid odontogenic
cyst
Calcifying odontogenic cyst
Glandular odontogenic cyst
Non-odontogenic cysts
Nasopalatine duct cyst
Nasolabial cyst
Mid-palatal raphe cyst of infants
Inflammatory cysts
Radicular cyst
Residual cyst
Paradental cyst
Buccal bifurcation cyst
Figure 2
ODONTOGENIC CYSTS
Finger-like projections which are zones of active cell
division or proliferation
The odontogenic cysts are derived from epithelium associated with the development of dental apparatus. It is estimated that odontogenic cysts make up approximately
90% of the jaw cysts. These cysts are generally lined by
stratified squamous epithelium. However, some developmental or fissural cysts in the maxilla will have respiratory
epithelium as the lining.
Eruption Cyst
The eruption cyst is a type of soft tissue cyst associated
with erupting teeth. It surrounds the crown of a tooth that
has already erupted through bone but impeded by the
overlying soft tissue. Kuczek et al (2003) reported a case
where a boy developed an eruption cyst who was administered cyclosporin A (potent immunomodulatory agent) subsequent to a cardiac transplantation. Cyclosporin A was replaced
with tacrolimus and there was no new cyst formation.
305
Figure 3
Clinical features
Eruption cysts are usually seen in childhood. These cysts
are commonly seen in the age groups of 5 and 9 years.
However, Woldenberg (2004) published a case report of
an eruption cyst in the maxilla of a 40-year-old female.
Studies by Anderson (1990), Woldenberg (2004) and
Aguil (1998) show that these cysts are more prevalent in
the maxillary arch. In a study of 24 cases of eruption cysts
by Bodner et al (2004), it was found that eruption cysts
were associated with natal teeth in two cases, with primary
teeth in 10 cases and with permanent teeth in 12 cases.
It was seen that males more commonly presented with
eruption cysts than females (2:1). The primary mandibular
central incisors and the permanent first molars were the
most common site involved. Though most of the eruption
cysts are reported as solitary entities, literature review
reveals report of a patient presenting with multiple eruption
cysts. Ramn Boj and Garca-Godoy (2000) described a
case of a 15-month-old child who had six eruption cysts
simultaneously.
On clinical examination, the cyst is visible as a soft
uctuant mass on the alveolar ridges and may vary in size
from about 1 to 1.5 cm in diameter. It may have the same
coloration of healthy oral mucosa or appear bluish or
bluish black. Eruption cyst histologically mimics dentigerous cyst.
Figure 4
Management
Usually the cysts open up spontaneously. In some children
the overlying soft tissue can be incised to facilitate the tooth
to erupt. Literature reveals that marsupialization is sufficient to manage eruption cysts.
Clinical features
It occurs frequently in association with impacted mandibular (Figure 3), maxillary third molars (Figure 4), and
maxillary canines (Figure 5). Dentigerous cyst is commonly seen in the 2nd and 3rd decades of life. Males are
306
Radiographic features
Radiographically, dentigerous cysts exhibit three different
presentations, namely, the central, lateral and circumferential
types. In central variety, the cyst symmetrically envelops
Figure 5
Figure 6
A
ODONTOGENIC KERATOCYST
Odontogenic keratocyst (OKC) was first described by
Philipsen in 1956. WHO has recently designated OKC as
keratocystic odontogenic tumor (KCOT) and is defined as a
benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized
stratified squamous epithelium and potential for aggressive, infiltrative behavior.
Keratocystic odontogenic tumor is described in Chapter 13
on Tumors of Orofacial Region.
Management
Surgical removal of cyst and tooth is recommended
(Figure 12). Marsupialization is done in case of very large
cysts. The cyst recurs if incompletely removed.
pattern. Wright et al (1981) reported 60 cases of orthokeratinized odontogenic cyst (OOC) and compared with OKC and
found that the OOC appears to be a distinct clinicopathologic entity. It was suggested that this cyst be called OKC,
orthokeratinized variant. Iamaroon et al (2004) compared
the proliferation index of the epithelial cells between OKC,
OOC, dentigerous cyst and ameloblastoma. He concluded
that OKC should be considered a benign tumor rather than
simply an odontogenic cyst and OOC as a non-aggressive
cystic lesion.
Clinical features
Orthokeratinized odontogenic cyst usually presents as a solitary cyst in the posterior part of the mandible. It is usually
seen in males commonly in the 2nd to 5th decade of life.
In comparison to OKC it is less aggressive and has a very
low recurrence rate (2.2%). Literature review reveals that
almost two-thirds of the cases of OOC appear like dentigerous cyst on clinical and radiographic examination.
Histopathologic features
Orthokeratinized odontogenic cyst has a thin epithelial lining with a luminal surface of orthokeratin. The basal cell
layer contains flattened squamous or cuboidal cells and a
well-developed granular cell layer (Figure 13).
Figure 7
A
Radiographic features
Central
Lateral
Orthokeratinized odontogenic cyst presents either as a welldefined unilocular or multilocular radiolucency. Radiographically, OOC may resemble a dentigerous cyst when it
is unilocular and resembles ameloblastoma when it is multilocular. Occasionally, it may cause cortical plate expansion and thinning of the lower border of the mandible.
Roots of teeth may be displaced (Figure 14). However,
resorption of roots is seldom seen.
The cyst should be surgically excised (Figure 15).
Figure 8
A
308
Figure 9
Orthopantomograph (OPG) showing a dentigerous cyst associated with the impacted left lower third molar causing root
resorption of the adjacent second molar. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Figure 10
Figure 11
23 layered
epithelium
Connective
tissue capsule
Cystic lumen
Figure 14
Figure 12
Figure 13
310
Figure 15
Management
Management
Histologic features
The cyst is lined by a thin non-proliferating cuboidal to
stratified squamous non-keratinizing epithelium, ranging
from 1 to 5 cell layers, and thus resembling the reduced
enamel epithelium.
The cyst wall and the lining usually show no signs of
inammation. The lateral periodontal cyst presents two
important histologic features, namely, the presence of epithelial thickenings or plaques and the presence of glycogenrich clear cells either in plaques or in the supercial
layers of the lining epithelium.
Radiographic findings
The cyst may appear as a round, oval or teardrop-like wellcircumscribed interradicular radiolucent area, usually with
a sclerotic margin, lying usually between the apex and the
cervical margin of the teeth. Resorption of adjacent teeth
though uncommon, has been reported. Occasionally, loss
of lamina dura and widening of the periodontal ligament
space may be present.
311
Figure 16
Types
In 1981, Praetorius and coworkers attempted to classify
calcifying odontogenic cyst by categorizing it into two entities: cyst and neoplasm.
Cystic entity
It was classified as:
Figure 17
Neoplastic entity
It was described as an odontogenic tumor with ghost cells.
The epithelial elements consist of numerous ameloblastomatous proliferations of tissue in the connective tissue
of the stroma. Varying amounts of ghost cells are present
within the epithelial islets. The hard tissue is composed of
different amounts of dentinoid in direct contact with the
epithelium.
Clinical features
It produces slow growing painless non-tender swelling of
jaws (Figures 16 and 17). It occurs more in 2nd decade of
life. It is commonly seen in the anterior part of the jaws. In
the maxilla, the canine region is the most commonly affected
site. In the mandible, the cyst is rarely found posterior to the
first molar. Occasionally the cyst may be seen crossing the
midline. On aspiration, it yields viscous granular yellow
fluid. According to Wood et al, 68% of cases occur in mandibular molar area. At least 52% cases are associated with
unerupted tooth, occasionally associated with pain.
Radiographic features
The radiographic features are quite variable. The central
lesion may be radiolucent with a variable margin that is
312
Figure 18
Differential diagnosis
Calcifying odontogenic tumor and ameloblastoma.
Management and prognosis
The management of calcifying odontogenic cyst is primarily by enucleation and curettage (Figure 19). Some authors
describe the possibility of malignant transformation. Only
eight cases till date have shown recurrences.
Figure 19
odontogenic cyst, although controversy still exists regarding its origin and the terminology.
Current literature review reveals the mention of only
23 cases of GOC. These appear to be odontogenic in origin
and present as a well-dened radiolucent swelling of the
jaws with a tendency to recur following conservative
treatment. These occur over a wide age range with no gender, race or ethnic predilection.
The cyst is possibly derived from rests of dental lamina
and comprises both secretory elements and stratied squamous epithelium.
Clinical and radiographic features
Glandular odontogenic cyst is commonly seen in the anterior mandible. Occasionally maxillary involvement has been
reported. The usual complaints are swelling, pain or discomfort in the involved area (Figure 20). It is usually seen
as a slow-growing swelling. Pain is very unusual but has
been reported.
Reports in literature mention the occurrence of GOC in
a wide age range (1480 years), however it is relatively
common in the 4th and 6th decades of life.
Radiographically it may be a unilocular or multilocular
well-circumscribed radiolucency usually displacing the roots
of teeth (Figures 21 and 22).
Histopathologic features
The histopathologic features of this cyst have been described
as a combination of findings from a botryoid odontogenic
cyst and a mucoepidermoid carcinoma, often causing a
diagnostic dilemma for pathologists.
313
Figure 20
Little inflammation
Occasional findings of hyperchromatic basal cells
within the cyst lining.
Figure 21
NON-ODONTOGENIC CYSTS
Non-odontogenic cysts including developmental cysts
account for about 6% of cysts in the orofacial region.
A cystic cavity lined by epithelium of varying thickness with a flat interface between the epithelium and
underlying connective tissue
Variable numbers of mucous cells in the epithelium
Eosinophilic cuboidal cells in the superficial layer
Localized plaque-like thickening of the epithelium
314
Figure 22
Orthopantomograph (OPG) showing well-defined unilocular radiolucency extending from the mesial aspect of the maxillary
right side lateral incisor to the mesial aspect of the left side lateral incisor. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
NASOLABIAL CYST
Nasolabial cyst is classified as developmental, non-odontogenic soft tissue cyst. It occurs in the nasolabial region.
It is estimated that the nasolabial cyst accounts for only
0.7% of the cysts of the jaws. The nasolabial cyst is also
Extrinsic cysts
Intrinsic cysts
a. Mucous retention cyst
b. Serous cysts
c. The mucocele.
Extrinsic Cysts
The extrinsic variety develops in structure adjacent to sinus
and as they expand they may encroach upon the sinus air
space. Extrinsic cyst may be odontogenic in origin or infrequently non-odontogenic.
Intrinsic Cysts
This cyst is referred to in literature by a plethora of names
like mucosal cyst, mucosal antral cyst, mucous cyst,
mucous retention cyst secreting or secretory cyst, mucocele serous cyst, non-secreting or secretory cyst, mesothelial cyst, lymphangiectatic cyst, interstitial cyst, pseudocyst
and false cyst. Concurrent concept permits the usage of term
mucosal cyst. There are three types of mucosal cysts:
a.
b.
c.
Figure 23
Extrinsic Cysts
Cysts like radicular, dentigerous, primodial and other
odontogenic cysts may encroach on the sinus, but rather
easily be identified by their characteristic location in the
jaws or their relationship to a tooth. Extrinsic cysts will
retain a thin curved radiopaque rim of bone which separates the cyst from the antral shadow.
Cysts associated with the salivary glands are described
in Chapter 11 on Diseases of Salivary Glands.
INFLAMMATORY CYSTS
Periapical Cyst (Radicular Cyst, Root End Cyst,
Apical Periodontal Cyst)
Periapical cyst is the most common inflammatory odontogenic cyst. It is associated with non-vital teeth. The tooth
might be deeply carious, traumatized or improperly
restored.
Etiopathogenesis
Bacteria invade the tooth followed by the death and degradation of pulp. The first line of defense in the periapical
area to seal the apex is the proliferation of epithelial rests
of Malassez in the periodontal ligament. They proliferate to
form a granulation tissue called periapical granuloma.
This consists of highly vascular tissue with immunocompetent cells like lymphocytes, macrophages, plasma cells.
As the time elapses, the central area is deprived of nourishment. The cells undergo liquefaction necrosis surrounded by epithelium to form a cyst.
Clinical features
More often (60%) these occur in the maxillary anterior
region. These might cause painless bony expansion of either
buccal or palatal cortical plate (Figure 24).
The cortex might be intact where it is hard or uctuant.
When perforated, the bone thins out and exhibits crepitus.
However, occasionally the patient may complain of pain
Figure 24
Figure 25
Figure 26
Figure 27
Figure 28
of cholesterol clefts is another important feature of radicular cysts. The brous capsule of the cyst is made up of dense
bundles of collagen bers. Usually chronic inammatory
cells are seen in the connective tissue approximating the
epithelium.
Simon (1980) described the presence of an epithelium
lined cavity that may open to the root canal in small periapical lesions. Simon termed this bay cyst. Subsequently
Nair et al (1990) studied 256 periapical lesions. Their inference was that 61% of the identied cysts fullled the criteria of true periapical cysts and the rest were apical
inammatory lesions that contained a sac-like, epitheliumlined cavity, continuous with the root canal. This epithelium formed an attachment that seemed to seal off the
infected root canal and its apical pouch from the periapical
region. It was suggested to term this a periapical pocket
cyst rather than a bay cyst.
Differential diagnosis
Paradental Cyst
Residual Cyst
Figure 29
Clinical features
It is commonly seen in young children in the 611 year
age group. The cyst causes a buccal tilting of the crown of
the involved tooth. The associated tooth is vital. On clinical examination, deep periodontal pockets may be evident
on the buccal aspect of the tooth. Occasionally, pain or
swelling may be present.
Radiographic features
Periapical radiographs, occlusal radiograph and orthopantomograph may be required to evaluate the presence of
buccal bifurcation cyst. Radiographically, the lamina dura
and the periodontal ligament space are unaffected. The cystic
radiolucency is always located on the buccal aspect of the
molar, which can be best appreciated on occlusal radiograph.
Occasionally, subperiosteal new bone may be laid down,
which may appear laminated.
Management
The cyst can be managed by curettage and extraction of the
tooth. Some authors recommend enucleation.
PSEUDOCYSTS
Clinical features
Paradental cysts are mostly seen involving the mandibular
third molars. However, maxillary third molar involvement
has also been reported in literature. Most of the cysts cover
the bifurcation of the roots and are usually attached to
the buccal root surface. Teeth are vital. Both sexes may
be affected. However, males are more commonly affected.
These cysts are commonly seen in the 2nd and 3rd decades
of life.
Radiographically, a well-dened radiolucency is seen
in relation to the distal aspect of a partially erupted third
molar. Generally the distal part of the radiolucency is separate and distinct from the distal follicular space of the
third molar. The width of the periodontal ligament space is
unaffected.
Histological features resemble that of radicular cyst.
The paradental cyst is managed effectively by surgical
enucleation. The partially erupted third molar can be
removed.
Clinical features
The cyst is usually seen in the 2nd decade of life. It is
slightly more common in males. The cyst is generally
asymptomatic and hence incidentally discovered on routine
radiographic examination. Approximately 1030% of the
patients complain of pain. Traumatic bone cysts frequently
affect the mandibular body (canine to molar region), followed by the mandibular symphysis and rarely the maxillary anterior region. The ramus and condylar regions are
hardly involved.
Other features that may be noticed are tooth sensitivity,
paresthesia and delayed eruption of permanent teeth.
Aspiration may occasionally yield a straw-colored uid or
bright blood.
Radiographic features
Most TBCs are found incidentally on routine radiographic
investigations. Radiographically, it is seen as a well-defined
radiolucent area with or without a sclerotic border. The
radiolucent area is commonly seen in the body of the mandible and the symphyseal region and the anterior region of
the maxilla. The radiolucent areas can occur unilaterally,
bilaterally or in multiple sites. The cyst may extend interdentally, between the roots of teeth giving rise to a scalloped
margin. Extensive lesions may cause expansion (usually
buccal cortical plate is expanded) and erosion of the cortical plates resulting in pathological fracture. Occasionally
the mandibular canal can be displaced.
Histopathologic features
The tissue specimen for histopathological examination can
be taken during the surgical curettage. However the amount
of tissue obtained is generally insufficient for histopathological analysis. Epithelial lining is typically absent. Other
findings include presence of fibrous connective tissue and
normal bone. The lesion may exhibit areas of vascularity,
fibrin and erythrocytes. Giant cells may be occasionally
seen adjacent to the bone surface.
Management
Literature review reveals that some cases of TBC undergo
spontaneous resolution. However, the most preferred and
widely recommended treatment modality is surgical exploration followed by meticulous curettage of the bony walls.
It is believed that curettage and surgical exploration will
induce bleeding. This induced bleeding will form a clot
which will ultimately be replaced by healthy bone. Recurrences are rare after surgical treatment.
Radiographically, it is characterized by ballooned out expansion of the periosteum which is usually outlined by a wafer
thin subperiosteum. This is, in turn, bounded by an area of
disintegrated cortex.
Clinical features
Aneurysmal bone cyst is commonly seen in the 2nd decade
of life. It is rarely seen after the 3rd decade of life. It is
believed that females are more commonly affected than
males.
Literature review reveals that the mandible is more
commonly affected than the maxilla. The molar regions of
the maxilla and mandible are the most common sites of
involvement. Extensive lesions in the mandible may involve
the angle and ascending ramus of the mandible. Rare sites
of involvement such as the coronoid process of the mandible, oor of the orbit and the zygomatic arch have been
reported in literature.
Clinically the swelling is rm on palpation. Aneurysmal
bone cysts tend to enlarge rapidly and cause thinning and
perforation of the overlying cortical plate. Some patients
complain of pain. Large lesions can cause displacement of
teeth and a progressive malocclusion. Associated teeth are
vital.
Occasionally, patients may complain of difculty in
mouth opening when the cyst involves the capsule of the
temporomandibular joint.
Differential diagnosis
Aneurysmal bone cyst may mimic other lesions such as
ameloblastoma, giant cell tumor, hyperparathyroidism,
myxoma, TBC and OKC. However, on aspiration, blood
may be expressed from a vascular lesion or aneurysmal
bone cyst. Clinically aneurysmal bone cyst may be differentiated from a vascular lesion based on the absence of bruits
or thrill and lack of pulse pressure.
Radiographic features
Radiographically, aneurysmal bone cyst resembles other
cystic lesions of the jaw. It may appear as a well-defined
unilocular radiolucent lesion or sometimes exhibit internal
septa within the radiolucent lesion giving rise to a multilocular appearance (honeycomb pattern).
Dabska and Buraczewski (1969) and Wilner (1982)
described four stages in the radiographic appearance of
aneurysmal bone cysts:
1.
2.
3.
Initial lytic phase: a well-defined area of bone resorption with no distinctive features is observed
Phase of active development: there is the typical subperiosteal, blow-out expansile appearance
Stabilization phase: there is a distinct peripheral bony
shell with internal septa and trabeculations, resulting
in the so-called soap bubble appearance
4.
Figure 30
CT scan may reveal fluid-fluid levels within the radiolucent lesion. Fluid-fluid levels are produced due to layering
of solid blood components within the cyst.
Management
Malghem et al (1989) reported three cases of spontaneous
healing of aneurysmal bone cysts in about 79 months
duration.
Aneurysmal bone cysts can be managed by surgical
curettage or by excision. Some authors have reportedly used
calcitonin injections with unpredictable results.
Szendri et al (1992) recommended direct injection of
calcitonin into the cyst as a useful non-invasive method
for the management of hypovascular aneurysmal bone
cysts.
Adamsbaum et al (2003) studied the affect of Ethibloc
(Ethnor Laboratories) injection, which is an alcoholic solution of zein (a vegetable protein dissolved in alcohol). It is
a brogenic and thrombogenic agent, on 17 patients in
the age group of 218 years. After 5 years of follow-up,
14 out of 17 patients demonstrated complete healing
manifested by increased cortical and septal thickening.
Inammatory reaction following the injection such as
pain and fever is seen. They concluded that the percutaneous direct Ethibloc injection is a safe, efcient and noninvasive treatment for aneurysmal bone cyst.
Other modalities of treatment include introduction of
demineralized bone and autogenous bone marrow that
promotes self-healing of a primary aneurysmal bone cyst.
It is estimated that 1044% of the cases exhibit recurrence. Recurrences have been reported due to difcult
access and incomplete removal of the cyst. It is advisable
to review the patients on a regular basis to evaluate for
recurrence.
NASOPHARYNGEAL CYSTS
Nasopharyngeal cysts are rare entities which may either be
congenital or acquired in nature. Most of the cysts are
asymptomatic. However, larger cysts can cause nasal
323
2.
Midline cysts
a. Congenital cysts
i. Cysts of bursa pharyngea embryonalis
ii. Cysts of Rathkes pouch
b. Acquired cysts
i. Retention cysts of the median recess
ii. Retention cysts of seromucinous glands
Lateral cysts
a. Congenital cysts
i. Branchiogenic cysts
b. Acquired cysts
i. Retention cysts of seromucinous glands
Lateral Cysts
These are usually branchiogenic in origin. The base of the
cyst is located between the posterior pillar and pharyngeal
opening of the Eustachian tube.
These cysts have more uid content and lesser cellular
debris compared to the midline cysts. Unlike the midline
cysts, the germinal centers are rarely present in the lateral
cysts. The inner lining of the cyst is made up of cylindrical
and ciliated epithelium.
Clinically these soft mucosal masses can be identied
by posterior rhinoscopy or by using a nasal berscope.
Choanal polyp, sphenoid sinus mucocele and juvenile angiobroma can mimic nasopharyngeal cysts.
Clinical features
Radiographic investigations
Midline Cysts
In the midline cysts, the retention variety is more common
than the congenital cysts.
It is believed that the retention cysts occur due to
the fusion of the median recess of the pharyngeal tonsil.
Hemorrhagic contents along with shed epithelial debris
may be evident in the cyst. The cyst wall may show lymphoid follicles with pronounced germinal centers. The inner
epithelial lining is composed of cylindrical ciliated cells. It
is believed that the presence of inammatory stimulus may
produce squamous metaplasia.
The congenital midline cysts may originate from the pharyngeal bursa (Tornwaldts bursa) or from Rathkes pouch.
The clinical and histological features of the cysts originating from the pharyngeal bursa are similar to those
of the retention cysts. However, to differentiate these cysts
a simple thumb rule can usedcysts deep to the pharyngobasilar fascia arise from pharyngeal bursa whereas cysts
originating supercial to the pharyngobasilar fascia are
more likely to be retention cysts.
The pharyngeal bursa is a tubuliform invagination lying
at the junction of the nasopharyngeal vault with posterior
pharyngeal wall and extending between the heads of the
longissimus muscles of the head just above the uppermost
bers of the superior constrictor muscle of the pharynx. It is
estimated that the pharyngeal bursa persists in about 3%
of the normal adults.
324
Management
Temporary relief of symptoms can be achieved by aspiration of the cystic contents or injection of sclerosing agents
into the cystic lumen such as ethanolamine oleate solution.
Surgical excision of the cyst is the treatment of choice.
Clinical features
Thyroglossal cysts are usually seen in the midline. However,
it is estimated that about 38% of the cysts are seen slightly
off the midline (located adjacent to the outer surface of the
thyroid cartilage, deep to the strap muscles). Almost all
cysts are present in the vicinity of the hyoid bone.
Based on the anatomic location the thyroglossal cysts
can be categorized as suprahyoid, at the level of hyoid and
infrahyoid.
About 2025% are suprahyoid, 1550% occurring at
the level of the hyoid bone, where they may be anterior or
posterior to the hyoid bone, and 2565% occurring in the
infrahyoid part of the neck.
The differential diagnosis of thyroglossal duct cysts
includes dermoid cyst, branchial cleft cyst, lymphadenopathy, and a cystic nodule arising from the thyroid gland.
Ultrasonographic features
The cyst typically appears anechoic, well-circumscribed with
increased through-transmission. However, the cyst may also
present as homogeneous or heterogeneous complex hypoechoic lesion. Some cysts may present as truly anechoic,
some as predominantly anechoic but containing internal
debris and some have a complex heterogeneous echo pattern, and few others have a uniformly homogeneous pseudosolid appearance. The coarse echo pattern is due to the
proteinaceous content of the cyst secreted by the cyst lining.
Occasionally, such a uniform echogenic appearance may
lead to a false assumption of a solid lesion. However, when
gentle but uniform pressure is applied over the cyst with the
transducer, the contents tend to shift which is suggestive of
a cystic lesion. It is estimated that about 45% of the cysts
have thick walls due to inflammation and cellular debris.
Management
Sistrunk procedure involving resection of the cyst and
tract, and excision of the middle third of the hyoid bone is
the standard procedure employed in the management of
the thyroglossal duct cyst. However, incomplete resection
will cause recurrence.
However, when a malignancy is evident, a near total or
total thyroidectomy along with the Sistrunk procedure is
recommended. The adjoining lymph nodes need to be evaluated because of the likelihood of a intrathyroidal foci of
cancer.
Straw-colored uid is aspirated from the cyst. Microscopically the aspirant may contain cholesterol crystals,
squamous cells, lymphocytes and polymorphonuclear cells.
Ultrasonographic imaging will show a circumscribed
mass of homogeneous low echogenicity. CT is a useful tool
to assess the topographical plane of the cyst and its proximity to the surrounding vital structures.
Differential diagnosis
Lipoma, cystic hygroma, thyroglossal duct cysts, lipomas,
lymphomas, and dermoid cysts should be considered in
the differential diagnosis of branchial cleft cysts.
Management
Infected cyst should be managed with appropriate antibiotics. Surgical excision of the cyst is the treatment of
choice.
CYSTIC HYGROMA
Cystic hygroma is a developmental entity characterized by
progressive dilatation of the lymphatic vessels. It was first
described by Wernher in 1843. It is estimated that 75% of
the cystic hygromas affects the head and neck region.
Cystic lymphangioma or macrocystic lymphatic malformation, rst described by Redenbacker (1828), is a term
used as a synonym for cystic hygroma.
It is believed that sequestered lymphatic rests that retain
their embryonic growth potential penetrate adjacent structures or dissect along fascial planes and eventually become
canalized. These spaces retain their secretions and develop
cystic components because of the lack of a venous outow
tract.
Clinical features
Approximately 60% of the cystic hygromas are present at
birth. By the age of 2 years 90% are apparent. The posterior triangle of the left side of the neck is most frequently
affected site. On clinical examination, these are large, soft
to doughy in consistency, painless swellings that can be
compressed. The skin overlying the swelling can occasionally show a bluish coloration. Transillumination is positive
in cystic hygromas.
Staging of cystic hygroma
de Serres et al (1995) proposed a staging system based on
the anatomical location:
1.
2.
3.
326
4.
5.
PARASITIC CYSTS
Though parasitic cysts in the head and neck region are
rare, the common cysts that may be seen are cysticercosis,
hydatid cysts and trichinosis.
Cysticercosis
Human cysticercosis is a parasitic infection caused by Taenia
solium. Other forms of Taenia include saginata and Asian
Taenia. It is believed that the Asian Taenia is an intermediate
form between solium and saginata as it is morphologically
similar to T. saginata and uses pigs as the intermediate host
like T. solium.
Ingestion of fecally contaminated food, water, fruit or
vegetables containing the ova of T. solium causes human
cysticercosis. Human cysticercosis is endemic in various
parts of the world including Mexico, Africa, South-East
Asia, Eastern Europe, Central and South America and India.
Poorly cooked meat (pork) is ingested by humans who
are the only denitive hosts. The undercooked meat includes
eggs or proglottids of the pork tapeworm. In the stomach
the larval form (Cysticercus cellulose) is released following
digestion of the outer protective layer of the proglottids.
These cysticerci are then disseminated by the arterial blood
to various tissues, preferentially to the CNS (60%), eye
(70%) and skeletal muscle (5%).
Clinical features
Clinically the condition may range from an asymptomatic
nodule to life-threatening symptoms. Individuals may complain of nausea, vomiting and severe epigastric pain. CNS
involvement may cause irritability, convulsions and loss
of consciousness.
On clinical examination, calcied nodules may be palpated anywhere in the body. In the head and neck region
the common sites for presence of calcied cysticerci include
muscles of mastication, muscles of facial expression and
cervical muscles. Some of these calcied masses may be as
large as 10 mm.
Management
Epidermoid, dermoid and teratoid cysts are best managed
by surgical excision. There is no definitive evidence for
malignant transformation of epidermoid or dermoid cysts.
However, individual case reports of such malignant changes
have been reported in literature. Dini et al reported of
Diagnosis
The diagnosis of cysticercosis should be done following
thorough evaluation of the patients lifestyle, personal
hygiene history and nature of food consumption along
with the characteristic neurological and gastrointestinal
327
Diagnosis
Hydatid Cyst
Radiographic features
Management
Clinical features
Hydatid disease primarily affects the liver (5570%) and
the lung (1835%). However, the less common sites that
are affected are the brain (common in children), heart,
kidney, ureter, spleen, uterus, pancreas, diaphragm and
muscles.
Emamy and Asadian (1976), Saxena et al (1983) and
Bickers (1970) reported cases of hydatid cyst affecting the
parotid gland.
The hydatid cyst grows slowly. It is usually diagnosed
in the 3rd and 4th decades of life. Symptoms usually
328
Figure 31
Trichinosis
Trichinosis is caused by the ingestion of undercooked meat
containing larvae of the nematode Trichinella spiralis.
These larvae enter the lymphatics and blood circulatory
system and migrate to well-vascularized striated skeletal
muscle. The parasite has a predilection for the most metabolically active muscle groups such as the tongue, diaphragm,
masseter, intercostal and pectoral muscles. The average
incubation period is between 1 and 30 days. The larvae are
estimated to have a 510 year life span.
Clinical features
The disease generally affects adults with no sex predilection. The disease process progresses through various phases
such as intestinal phase, parenteral phase and convalescent
phase. The intestinal phase of the disease presents with
diarrhea, anorexia, weakness and abdominal cramps. Some
individuals may present with macular rashes. High degree
fever (104F) is another characteristic feature. The intestinal
phase lasts for about 1 week.
In the parenteral phase the larvae migrate from the intestine into the circulation. This process may continue for many
weeks to a few months. Patients may complain of severe
myalgia. At this stage, skeletal muscles, CNS, cardiac and
respiratory system are involved.
330
CHAPTER
Traumatic Neuroma
Rhabdomyoma
Leiomyoma
Hemangiomas and Vascular Malformations
Hemangioma
Vascular Malformations
Capillary Malformations
Arteriovenous Malformations
Lymphatic Malformations
Fibroma
Giant Cell Fibroma
Fibromatoses
Myofibroblastoma
Ameloblastoma
Squamous Odontogenic Tumor
Clear-Cell Odontogenic Tumor
Calcifying Epithelial Odontogenic Tumor
Adenomatoid Odontogenic Tumor
Keratocystic Odontogenic Tumor
Odontome
Ameloblastic Fibroma, Ameloblastic Fibrodentinoma,
and Ameloblastic Fibro-odontoma
Benign Cementoblastoma
Odontogenic Myxomas
Odontogenic Fibroma
Desmoplastic Fibroma
Odontogenic Carcinomas
Squamous Papilloma
Verruca Vulgaris (Oral Warts)
Verrucous Hyperplasia
Condyloma Acuminatum
Focal Epithelial Hyperplasia/Hecks Disease
Keratoacanthoma/Self-healing Carcinoma
Oral Melanoacanthoma
Acquired Melanocytic Nevus
Ossifying Fibroma
Juvenile Ossifying Fibroma
Peripheral Ossifying Fibroma
Osteoma
Chondroma
Benign Chondroblastoma (Codmans Tumor)
Benign Osteoblastoma
Giant Cell Granuloma
Peripheral Giant Cell Granuloma
Pyogenic Granuloma
Fibrous Hyperplasia: Denture-related
Congenital Epulis of Newborn
Lipoma
Neurogenic Tumors
Schwannoma (Neurilemmoma)
Neurofibroma
13
Odontogenic Sarcomas
Ameloblastic Fibrosarcoma
Odontogenic Carcinosarcoma
331
ODONTOGENIC TUMORS
Odontogenic tumors are lesions of great interest and
importance to oral physicians, pathologists and maxillofacial surgeons alike, who for several decades have studied
and catalogued these lesions and developed modalities for
adequate treatment.
It is estimated that approximately 9% of tumors in the
oral cavity are odontogenic in nature, out of which 94.8%
are benign and 5.2% are malignant, 58.5% of benign
odontogenic tumors are ameloblastoma.
The age predilection for benign odontogenic tumors is
around 28 years and for the malignant odontogenic tumors
is more than 40 years. Most of the tumors have mandibular
predilection with the ratio of 3.2:1, with the maximum in
case of ameloblastoma showing a rate of 12.8:1.
There are different ways of dening the content of the
term tumor in its broadest sense and not restricted to
lesions that are denitively neoplastic. Benign tumor is
dened as a growth made up of normal cells that have no
signs of cancer.
These tumors have some characteristic properties such as
new uncoordinated growth that spreads by direct extension
unlimited growth potential, do not metastasize, are encapsulated and resemble tissue of origin histologically.
Muscle Origin
Leiomyosarcoma
Rhabdomyosarcoma
Nerve Origin
Neurofibrosarcoma
Complete history: Pain, loose teeth, occlusion, swellings, dysesthesia, delayed tooth eruption
Thorough physical examination: Inspection, palpation, percussion, auscultation
Plain radiographs: Panaromic radiographs and dental
radiographs at contrasting angles
Advanced imaging: CT for larger, aggressive lesions
Table 1
Obtain tissue
Fine-needle aspirationrule out vascular lesions,
inflammatory conditions
Excisional biopsysmaller cysts, unilocular tumors
Incisional biopsylarger lesions prior to definitive
therapy.
Ameloblastoma
Churchill in 1934, defined ameloblastoma as unicentric,
non-functional, intermittent growing anatomically benign
but clinically persistent. These are benign, locally aggressive polymorphic neoplasms that consist of proliferating
odontogenic epithelium.
Classification
Leon Barnes has classified ameloblastoma into four types on
the basis of behavioral pattern, anatomical location, radiographic appearances and histologic features as follows:
1.
2.
3.
4.
Unicystic
Multicystic
Desmoplastic
Peripheral.
Table 2
025 years
Table 3
Male
Female
Ameloblastoma
Ameloblastic fibroma
Ameloblastic fibro-odontoma
Ameloblastic odontoma
Calcifying epithelial odontogenic tumor
Odontoma
Benign cementoblastoma
Adenomatoid odontogenic
tumor
Squamous odontogenic
tumor
Odontogenic myxoma
Calcifying odontogenic
fibroma
Table 4
Maxilla
Mandible
Ameloblastoma
Ameloblastic odontoma
Odontoma
Benign cementoblastoma
Odontogenic myxoma
Calcifying odontogenic fibroma
Clinical features
Ameloblastomas may be present over a wide age range but
are usually diagnosed in the 4th and 5th decades of life,
although they can occur in children or the elderly. About
80% of tumors occur in the mandible, of which some 70%
arise in the molar region and ascending ramus, 20% in the
premolar region, and 10% in the incisor region. In the maxilla, most of these also occur in the molar region but about
15% involves the antrum.
The tumor is slow growing and in the early stages may
be asymptomatic and discovered as an incidental nding.
As the tumor enlarges the patient may become aware of a
gradually increasing facial deformity and expansion of the
jaw bone (Figure 1A, B). The enlargement is usually bony
hard, non-tender, and ovoid or fusiform in outline but in
advanced cases, egg-shell crackling may be elicited due to
thinning of the overlying bone. However, perforation of
333
bone and extension of the tumor into soft tissues are late
features. In the maxilla, even large tumors may produce
little expansion as the lesion can extend into the sinus and
beyond. Teeth in the area of the tumor may become loosened, but pain is seldom a feature.
Radiographically, the ameloblastoma appears most commonly as a multiloculated radiolucency (Figure 2A, B). Roots
of teeth involved by the tumor show varying degrees of
resorption. As the tumor enlarges it may become associated
with an unerupted tooth, particularly an impacted third
molar, and the appearances may mimic those of a dentigerous cyst. Less frequently, ameloblastomas present as a
single unilocular radiolucency indistinguishable from an
odontogenic cyst.
MRI findings
Characteristic findings are: multilocularity, mixed solid and
cystic components, irregularly thickened walls, papillary projections, and marked enhancement of the walls and septa.
Histopathology
There are six histologic subtypes: follicular, plexiform,
acanthomatous, granular cells, basal cell and desmoplastic. These can be found combined or isolated and not
related to prognosis of the tumor. The two main patterns
are anastomosing epithelial strands and fields or discrete
epithelial islands. The former pattern is called the plexiform (Figure 3) type, the others the follicular (Figure 4A).
Figure 1
A
(A) Ameloblastoma involving mandible. (B) Intraoral view of ameloblastoma involving mandible.
Courtesy: Dr Foluso Owotade
Figure 2
A
(A) Radiographic features of ameloblastoma (honeycomb appearance). (B) Ameloblastoma (soap bubble appearance).
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
334
Both may occur within the same lesion. The follicles consist of a central mass of loosely connected, angular cells
resembling the stellate reticulum of the normal enamel
organ, surrounded by a layer of cuboidal or columnar cells
resembling ameloblasts. The nuclei of the latter are situated away from the basal ends of the cells, and this is
described as reversed polarity. The follicles are separated
by varying amounts of fibrous connective tissue stroma. A
variety of changes can occur within the stellate area of the
follicles and these include cystic breakdown, squamous
metaplasia, and granular cell change. The tumor epithelium in the plexiform type is arranged as a tangled network of anastomosing strands and irregular masses, each
of which shows the same cell layers as for the follicular
pattern. Thus, each strand or mass is bounded by columnar or cuboidal cells resembling ameloblasts, while the
Figure 3
Ameloblast-like
columnar cells
Stellate
reticulum-like
cells
Interconnecting
strands
Box 1
Dentigerous cyst
Figure 4
A
B
Ameloblastic
follicles
Cystic
degeneration
Stellate
reticulumlike cells
Ameloblastlike cells
showing
palisading
Ameloblastic
follicles
Squamous
metaplasia
Stellate
reticulumlike cells
335
Box 2
Literature review
Ameloblastoma is a tumor with a complex biological character and presents a high recurrence rate even in patients
treated with radical therapy. It has been reported that the
aggressiveness of ameloblastoma may be related to the
histological subtype, in fact.
Reichart et al in a review of the literature, found recurrence rates for plexiform, follicular, acanthomatous and
unicystic ameloblastoma respectively of 16.7%, 29.5%,
4.5% and 13.7%. Proliferating cell nuclear antigen (PCNA)
is a cell cycle related antigen and has been used for the
evaluation of the proliferation ability of this tumor.
Determinants of biological behavior
Location Seventy-five percent in the mandible, but worse
prognosis in the maxilla due to spongier bone that facilitates dissemination of the tumor. There is an absence of the
contention effect that provides the mandibular cortical
bone.
Arquitectonic pattern This pattern of the lesion does have
a prognostic validity. Unicystic has a better prognosis than
multicystic or solid.
Unicystic Ameloblastoma
Unicystic ameloblastoma, a variant of ameloblastoma first
described by Robinson and Martinez in 1977, refers to
those cystic lesions that show clinical and radiologic characteristics of an odontogenic cyst but on histologic examination show a typical ameloblastomatous epithelium lining
part of the cyst cavity, with or without luminal and/or mural
tumor proliferation. This type of ameloblastoma typically
presents in a younger age group than other variants of
ameloblastoma (2nd to 3rd decade) and occurs predominantly in the mandibular third molar region.
Radiographically, it appears as a well-dened unilocular
radiolucency, usually associated with an unerupted tooth,
and is indistinguishable from a dentigerous cyst. The pathogenesis of the lesion is unknown. Although it may represent
ameloblastomatous change in a dentigerous or other type
of odontogenic cyst, the possibility that it was a grossly
cystic ameloblastoma from the outset cannot be excluded.
336
Histopathology
Based on the character and extent of tumor cell proliferation within the cyst wall, several histologic subtypes of
unicystic ameloblastoma are recognized, which include
those of simple cystic nature, intraluminal proliferative
nodules and infiltrative tumor islands in the cyst walls.
The lesion presents as a cyst lined by ameloblastomatous
epithelium comprising a basal layer of columnar cells with
polarization of their nuclei away from the basal lamina,
covered by a loose, vacuolated layer of stellate epithelial
cells. The diagnosis is made based on histopathologic
examination.
Treatment
This variant is believed to be less aggressive, tends to affect
patients at a younger age and its response to enucleation
or curettage is more favorable than the classic solid or
multicystic ameloblastomas.
Odontoameloblastoma (OA)
This is an extremely rare mixed odontogenic tumor
appearing within the maxillary bone, with both epithelial
and mesenchymal components. It is also known as ameloblastic odontoma, although the term odontoameloblastoma
(OA) was included in the 1971 WHO classification and
seems to be more appropriate due to the behavior of the
tumor like an ameloblastoma rather than as an odontoma.
Thoma et al described in 1944 the first case, it is an ameloblastoma in which focal differentiation into an odontoma appears to have taken place.
Histopathology
The marked histological polymorphism of odontogenic
tumors make the final diagnosis difficult and in some
cases it must be made based on clinical, radiologic and
histopathologic features. Moreover, Wachter et al could not
histologically differentiate between OA and ameloblastic
fibro-odontoma, and therefore suggest that they are the
same entity.
Treatment
Radiographic features
Histopathology
The calcifying epithelial odontogenic tumor consists of
sheets of polygonal cells with ample eosinophilic cytoplasm,
distinct cell borders, and very conspicuous intercellular
bridges. Nuclei are pleomorphic with prominent nucleoli;
cells with giant nuclei and multiple nuclei are also present.
The epithelial tumor islands as well as the surrounding
stroma frequently contain concentrically lamellated calcifications. Stromal deposits of bone and cementum may
occur (Figure 5).
Treatment
The treatment for CEOT has ranged from simple enucleation or curettage to radical and extensive resection such
as hemimandibulectomy or hemimaxillectomy. The choice
should be individualized for each lesion because the radiological and histological features may differ from one
lesion to another.
Prognosis
The prognosis of the CEOT is good with infrequent recurrence. Malignant behavior is extremely rare. Although it has
Figure 5
Polyherdal
epithelial cells
Calcifications
Clinical features
It occurs over a wide age range and is about twice as common in the mandible as in the maxilla. The chief sign is
cortical expansion and pain is not normally a complaint.
Most of the tumors arise in the molar or premolar area and
about half are associated with the crown of an unerupted
tooth. Although most tumors arise within bone, extraosseous lesions have been reported.
337
Figure 6
A
Definition
Philipsen and Birn (1971), defined it as an odontogenic
epithelial tumor with an inductive effect on the odontogenic
mesenchyme. The nature of the lesion is uncertain and it
may be hamartomatous rather than truly neoplastic.
Classification
Based on the location it can be classified as:
Follicular
Extra follicular
Peripheral.
Clinical features
The adenomatoid odontogenic tumor usually presents
during the 2nd and 3rd decades of life. The majority of
tumors arise in the anterior part of the maxilla (Figure 6A),
especially in the canine areas, and there are usually few
symptoms apart from a slowly enlarging swelling. Clinical
features generally focus on complaints regarding a missing
tooth. The lesion usually presents as asymptomatic swelling which is slowly growing and often associated with an
unerupted tooth. However, the rare peripheral variant occurs
primarily in the gingival tissue of tooth-bearing areas.
Unerupted permanent canines are the teeth most often
involved in AOT.
Histopathology
Lesion is well encapsulated and may be solid or partly
cystic; in some cases the tumor is almost entirely cystic. It
consists of sheets, strands, and whorled masses of epithelium which in places differentiate into columnar, ameloblast-like cells. The columnar cells form duct or tubule-like
structures (hence adenomatoid) with the central spaces
containing homogeneous eosinophilic material. These are
thought to represent abortive attempts at enamel organ
formation. There is very little supporting stroma. Small
foci of calcification are scattered throughout the tumor
and occasionally tubular dentin and enamel matrix may
be seen (Figure 7).
Radiographic features
On radiographs it usually appears as a well-defined radiolucency but in some cases calcification within the tumor
may produce faint radiopacities (Figure 6B). The lesion is
often associated with an unerupted tooth and may simulate
a dentigerous cyst (simulating a target like appearance).
338
Differential diagnosis
Radiologically, it should be differentiated from dentigerous cyst, which most frequently occurs as a pericoronal
radiolucency in the jaws. Dentigerous cyst encloses only
the coronal portion of the impacted tooth, whereas AOT
Figure 7
Figure 8
Duct-like space
Calcifications
Epithelial cells
(polyherdal to
spindle-shaped)
Dentinoid
Connective tissue
Histopathology
Histologically, these cysts are formed with a stratified
squamous epithelium that produces orthokeratin (10%),
parakeratin (83%), or both types of keratin (7%). The epithelial lining appears corrugated when viewed under a microscope. A well-polarized hyperchromatic basal layer is
observed, and the cells remain basaloid almost to the surface. No rete ridges are present; therefore, the epithelium
often sloughs from the connective tissue. The epithelium is
thin, and mitotic activity is frequent; therefore, OKCs grow
in a neoplastic fashion and not in response to internal pressure. The lumen frequently is filled with a foul-smelling
cheese-like material that is not pus but rather collected
degenerating keratin (Figure 9AC).
Radiographic features
Radiographically, KCOT presents predominantly as a unilocular radiolucency with well-developed sclerotic borders.
These may also present as a multilocular radiolucency or
Figure 9
A
Cystic space
Stratified
squamous
parakeratinized
epithelium
Palisaded
basal cells
Corrugated
parakeratin
layer
Connective
tissue
(A) Histopathological features of keratocystic odontogenic tumor. (B) and (C) Gross specimens of the resected tumor, showing
removal of the cystic lining and the resultant hollowing in the bone. Courtesy: Department of Oral Pathology and Microbiology,
MCODS, Mangalore. (D) Orthopantomograph showing well-defined scalloped radiolucent area extending across the midline.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
340
Treatment
A review of the literature suggests that recurrence rate is
relatively low with aggressive treatment, whereas more conservative methods tend to result in more recurrences.
Recurrence
KCOTs have a high recurrence rate, reportedly between
25% and 60% and when associated with NBCCS, the recurrence rate is about 82%.
Odontome
History
Paul Broca (1867), defined it as a tumor formed by the
overgrowth of transitory/complex dental tissues.
1946 WHO classification
1. Germinated composite odontome
2. Compound composite odontome
3. Complex composite odontome
4. Dilated odontome
5. Cystic odontome
In 1950 Gorlin eliminated the term composite odontome.
Recent WHO classification
1. Complex odontome: Malformation in which all the
dental tissues are represented, individual tissues being
mainly well formed but occurring in a more or less
disorderly pattern.
2. Compound odontome: Malformation in which all the
dental tissues are represented, in a more orderly pattern;
consists of tooth-like structures called the tooth-lets.
Clinical features
Odontomas are the most common maxillary tumors, and
according to different sources in the literature account
for 2267% of all odontogenic maxillary neoplasms. As to
their locations, most are found in the areas of the upper
Prognosis
Odontomas are benign tumors that sometimes produce
no symptoms and constitute casual findings of routine
radiological studies. However, they usually tend to cause
341
Figure 10
Orthopantomograph showing odontoma. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
strata in the palms of the hands and soles of the feet, is the
only pathognomonic data which has been described in the
literature regarding this condition.
Box 4
Figure 11
CEOT
Highly cellular
mesenchymal
stroma
Proliferating
odontogenic
epithelium
Box 3
Odontoma
COC
AFO
Benign Cementoblastoma
Cementum is a modified form of bone and, with the exception of the cementoblastoma, disorders of the jaws containing cementum-like tissue are now classified as lesions
of bone. The cementoblastoma is still classified as an
odontogenic tumor because of its unique association with
the root of a tooth. Cementoblastoma is classified as an
odontogenic tumor because of its unique association with
the root of a tooth. This is one characteristic feature that
differentiates it from osteoblastoma of bone.
Clinical features
The cementoblastoma is a rare benign neoplasm most frequently seen in patients below 25 years of age. It usually
arises in the molar or premolar area of the mandible and
is attached to the root of a tooth. Most cases involve the
mandibular first permanent molar. It presents as a slowly
enlarging swelling which sometimes gives rise to pain, but
the involved tooth is vital. Pain is main symptom, pain is
severe and not relieved by NSAIDs.
Radiographic features
Radiographic features show a well-demarcated, mottled, or
dense radiopaque mass with a radiolucent margin attached
to the root of a tooth which usually shows resorption.
Histologic features
Tumor consists of a mass of calcified cementum-like tissue
containing scattered cells lying in lacunae. Around the
periphery and in other actively growing parts of the lesion,
extensive sheets of uncalcified matrix formed by plump,
deeply staining cementoblasts may be seen.
Prognosis
Lesions which are incompletely removed may recur.
Odontogenic Myxoma
These are uncommon, benign neoplasms arising from
mesenchymal odontogenic tissue which are locally invasive neoplasm consisting of rounded, angular cells lying
in abundant myxoid stroma.
343
Clinical features
Desmoplastic Fibroma
Commonly seen in the 3rd decade of life with higher prevalence among women, and occurs mainly in the mandibular molar area. Presents as painless swelling or incidental
finding.
Radiographic features
Often multilocular with internal osseous trabeculae arranged
in tennis racket pattern.
MRI: Prolongation of T1 and T2, reecting rich myxoid
stroma. Gradual contrast enhancement is typically seen on
contrast-enhanced images.
Histopathology
Odontogenic myxomas consist of rather monotonous cells
with multipolar or bipolar slender cytoplasmic extensions
that lie in a myxoid stroma. Nuclei vary from round to
fusiform in appearance. Binucleated cells and mitotic figures are present, but scarce. Occasionally, the lesion contains
odontogenic epithelial rests. The lesion spreads into the
jaw bone without any encapsulation, thereby engulfing
neighboring cancellous bone.
Treatment
Excision or partial resection of the jaw bone with high
rate of local recurrence due to infiltrative growth pattern
enhancement is typically seen on contrast-enhanced
images.
Odontogenic Fibroma
This jaw tumor is considered a neoplasm that is derived
from periodontal ligament or pulp-related fibroblasts. It is
a tumor of adults and appears as a well-defined radiolucency in either jaw. It is not, however, particularly aggressive, and it infrequently recurs after simple curettage.
Histopathology
These lesions are more collagenous than myxomas but
may range from myxofibrous to densely fibrous. Characteristically seen in odontogenic fibromas are few to many
islands and strands of bland odontogenic epithelium.
Calcific deposits may also be found. A variant (granular
cell odontogenic fibroma), in which granular cells are seen
in the connective tissue, has been described. The behavior
of this tumor does not appear to be different from odontogenic fibroma. Abundant rest proliferation in follicular sacs
can occasionally simulate the appearance of odontogenic
fibroma or ameloblastic fibroma.
Clinicopathological correlation is important for the
diagnosis of these lesions.
344
Treatment
Ennucleation/excision or partial resection of the jaw bone.
Squamous Papilloma
Verruca vulgaris
Verrucous hyperplasia
Condyloma accuminata.
Table 7
Site
Type of lesion
Palpation characteristic
Swelling
Soft, fluctuant
Gingiva
Mucocele, ranula
Developmental cysts
Sialocysts
Gingival cysts
Parulis
Abscess
Soft non-fluctuant
Lipoma
Fibroma
Organized mucocele
Firm fixed
Mesenchymal tumors
Granulomas
Salivary adenomas
Adnexal skin tumors
Firm movable
Palate
Dorsum of the
tongue
Ventral aspect
of the tongue
Table 6
Color
Blue-purple
Red
Brown
Box 5
Black
Melanoma
Verruca vulgaris 2, 4
Yellow-orange
Squamous papilloma 6, 11
Condyloma acuminatum 6, 11
Focal epithelial hyperplasia 13, 32
Squamous cell carcinoma 16, 18, 31, 33, 35
Squamous Papilloma
A benign epithelial proliferation induced by HPV in most
cases; several subtypes have been identified, especially
HPV-6 and 11.
Clinical features
Exophytic, papillary mass, measuring less than 1 cm, usually
pedunculated and soft in texture.
Treatment
Surgical excision is the preferred modality of management.
345
Table 8
Epithelial
Papilloma
Keratoacanthoma
Nevus
Connective tissue
Fibroma
Giant cell fibroma
Peripheral ossifying fibroma
Lipoma
Vascular tissue
Hemangioma
AV fistula
Carotid body tumor
Lymphatic origin
Lymphangioma
Neural tissue
Neurilemmoma
Neuroma
Neurofibroma
Neurofibromatosis
Muscular origin
Leiomyoma
Rhabdomyoma
Bone origin
Osteoblastoma
Osteoid osteoma
Benign osteoblastoma
Desmoplastic fibroma of bone
Cartilaginous
Chondroblastoma
Histopathology
Histological changes can be in the form of surface hyperkeratosis, granulosis and koilocytosis.
AIDS-associated oral warts may appear dysplastic
microscopically.
Differential diagnosis
AV: Arteriovenous.
Treatment
Hyperplastic
epithelium
Treatment may involve surgery, laser surgery, or cryotherapy. It should be noted that HPV survives in aerosol.
Topical 5-fluorouracil treatment has been used on external
lesions, but should be avoided in fair individuals as it can
cause hyperpigmentation. It should be noted, however, that
this is a specialized treatment and should only be used by
those experienced with the use of this topical medication.
Lesions tend to recur after treatment.
Connective
tissue core
Verrucous Hyperplasia
Figure 12
Blood vessels
Differential diagnosis
Verrucous carcinoma, papillary squamous cell carcinoma
and proliferative verrucous leukoplakia can be considered
in the differential diagnosis.
Treatment
Excision or ablation (e.g. laser, electrocautery) has been
used successfully in the management of verrucous
hyperplasia.
Condyloma Acuminatum
Described in Chapter 22 on Sexually Transmitted Diseases
on page 630.
Treatment
Keratoacanthoma/Self-healing Carcinoma
Keratoacanthoma (KA) is usually encountered on the facial
skin and lips yet can also arise, albeit rarely, in the mouth.
Observation and careful follow-up local excision, cryotherapy and intralesional chemotherapy have also been
tried. Wong et al in their study reported oral isotretinoin
to be an effective treatment of multiple as well as solitary
keratoacanthomas. Canas et al reported that successful
treatment was achieved with administration of oral
isotretinoin. Spieth et al reported that intralesional methotrexate shows to be an effective, easy and inexpensive
alternative.
Etiology
Unknown, may be related to several factors, as follows:
Clinical features
Clinically these are characterized by a tumefaction with
round, mounded borders that surround a central core of hard
keratinized material that may appear pale yellow or brown.
The brown appearance is caused by extrinsic pigments that
become incorporated with the excessive keratin. Intraoral
KAs are generally non-pigmented. They can be solitary on
sun-exposed areas, including lip. They initially present as
erythematous papule with rapid growth over 48 weeks;
extremely rare intraorally.
Clinical features
Oral melanoacanthoma is seen in blacks, shows a female predilection, and occurs in the 3rd and 4th decades of life. The
buccal mucosa is the most common site of occurrence. The
lesion is smooth, flat or slightly raised, and dark-brown to
black in color.
Histopathologic features
Numerous benign dendritic melanocyte cells that are normally confined to the basal cell layer scattered throughout
the lesional epithelium. Basal layer melanocytes are also
present in increased numbers.
Treatment and prognosis
Histopathology
Histopathology shows keratin plus normal, peripheral epidermis and mature, premature keratinization; no invasion
Because of the alarming growth rate of oral melanoacanthoma, incisional biopsy is usually indicated to rule out the
possibility of melanoma.
347
348
Treatment
No active intervention is required, unless in case of clinical
suspicion and cosmetic concern.
Ossifying Fibroma
Described in Chapter 19 on Bone Diseases and Fibroosseous Lesions on page 571.
Clinical features
Acquired melanocytic nevi begin to develop during childhood and most cutaneous lesions present before 35 years
of age with no sex predilection even though racial differences are seen.
Head and neck region is a common site of involvement.
Junctional nevus is a sharply demarcated, brown or black
macule less than 6 mm in diameter. When nevus cells proliferate become slightly elevated soft papule it is called
as compound nevus. The degree of pigmentation becomes
less; most lesions appear brown or tan. Nevus gradually
loses its pigmentation, the surface may become somewhat
papillomatous, and hairs may be seen growing from the
center intradermal nevus remains less than 6 mm in diameter. Ulceration is not a feature unless placed at a region
of recurrent trauma. Intraoral melanocytic nevi are distinctly uncommon, they may arise on the palate or gingiva,
although any oral mucosal site may be affected. More than
one in ve intraoral nevi lack clinical pigmentation and
females are more affected; the average age at diagnosis is
35 years.
Histopathologic features
Pathogenesis
Chronic irritation causes an abundance of fibrous connective tissue containing immature bone. Often patients traumatize the area again, and this promotes a cycle of more
exuberant tissue formation and varying surface ulceration.
There is a close resemblance to the pyogenic granuloma.
The lighter surface color is due to hyperkeratosis produced
through trauma.
Clinical features
Peripheral odontogenic ossifying fibromas occur more
often in females and can be anywhere from 1 to 2 cm in
size. They occur most often in young adults, originate
from the periodontal ligament, and occur exclusively on
the gingiva as a sessile or pedunculated mass. The lesions
are normally found on the attached gingiva. The abundance
of tissue is usually the same color as the surrounding tissue, with a smooth surface. Sometimes the surface may be
more textured, depending upon the existing oral forces
Figure 13
Osteoma
Benign, slowly growing lesion composed of well-differentiated mature bone with a predominant lamellar structure.
It is usually an incidental finding or painless hard swelling.
Peripheral ossifying fibroma in the interdental regions of the
upper lateral and canine. Courtesy: Department of Oral
Medicine, KLE Institute of Dental Sciences, Bangalore
Etiology
Sporadic form is idiopathic or may be a component of
Gardners syndrome.
Clinical features
Figure 14
Stratified
squamous
epithelium
Connective
tissue
Histopathology
Area of
ossification
Histology reveals dense areas of normal cortical and trabeculae (Figure 16).
Diagnosis
Radiographic features
Microscopic features: Normal cortical and trabecular
bone.
Differential diagnosis
and location in the patients mouth. Additionally, the surface epithelium may be more of a dark color depending
upon the amount of trauma and inflammation that is produced with the lesion (Figure 13).
Histopathologic features
Microscopically, the tissue is composed primarily of cellular fibroblasts with various types of calcification including
bone, cementum and dystrophic calcification. The surface
epithelium may exhibit varying degrees of ulceration
(Figure 14).
Tori, exostoses
Ossifying fibroma
Osteoblastoma
Focal sclerosing osteitis.
Treatment
Local resection, if compromising.
Prognosis
Little recurrence potential when associated with Gardners
syndrome, malignant conversion of intestinal polyps is
assured.
349
Clinical features
Figure 15
Figure 16
Compact bone
Haversian system
Chondroma
Chondromas are benign tumors of mesenchymal origin
composed of multiple nodules of mature hyaline cartilage.
It is estimated that chondromas represent approximately
2.38% of osteocartilaginous tumors.
Chondromas are well-recognized tumors in the bony skeleton. However, these are relatively rare in the jaw bones.
Based on the location chondromas are classied as
enchondromas (account for 80% of these tumors, located
in the medullary cavity of the long bones, diaphysis), juxta
articular chondromas (related to extraskeletal cartilage),
extraskeletal chondroma or soft tissue chondroma (rare
cartilaginous tumor, involving hands, feet, tongue and
buccal mucosa).
350
Histopathologic features
Figure 17
Connective
tissue capsule
Chondrocytes
in lacunae
Hyaline
cartilage
Benign Osteoblastoma
Benign osteoblastoma is an osteoid and bone forming
benign tumor of bone. It is an uncommon lesion that
accounts for 1% of all bone tumors and about 3% of all
benign bone tumors.
Clinical features
Chondroblastomas are usually seen in the 3rd decade of
life. Males are slightly more commonly affected than
females (1.86:1).
In the maxillofacial and the temporal region, mandible,
maxilla and the TMJ have been reported to be affected.
Patients may complain of a local mass, auditory dysesthesia, and limitation of mouth opening.
Radiographic features
On radiographs, chondroblastoma affecting the extremities appears as round to ovoid radiolucencies which are
well-defined, expansile in nature and usually exhibit a
sclerotic margin.
Chondroblastomas affecting the condyle have been
reported to have caused resorptive defects and occasionally enlargement of the condyle.
Clinical features
Benign osteoblastoma occurs in the 2nd and 3rd decades
of life, but the age range is 578 years. As to sex incidence,
there is male predominance 23:1. Benign osteoblastoma
can arise in any bone, but there is a predilection for the
vertebra, long bones, skull and small bones of the hand
and feet. Clinically, osteoblastoma is a well-circumscribed,
solitary lesion, which measures 2 cm or more and may be
as large as 12 cm. Usually, the lesion is not painful, or if
pain is present, it is not very responsive to salicylates.
Radiographic features
Radiographically, the picture may vary in accordance with
the size of the tumor and with the extent to which the tissue
is calcified. Osteoblastomas are well-circumscribed, expansive lesions with central radiopaque areas suggesting new
351
bone formation, but it is less likely to provoke an outstanding bony sclerosis typical of osteoid osteoma.
Histopathologic features
Histologically, benign osteoblastoma consists of a highly
vascularized, fibrocellular stroma in which there are abundant newly formed trabeculae of immature bone and
osteoid. Proliferating osteoblasts are found lining the trabeculae of immature bone and osteoid. The major problem
for pathologists is the correct differentiation between
benign osteoblastoma and a number of lesions that may
have similar characteristics. Benign osteoblastoma and
osteoid osteoma have almost identical histologic features.
Based on this histologic similarity, Dahlin and Jonson in
1954 suggested a name giant osteoid osteoma for the
lesion that is denoted as the benign osteoblastoma in the
present.
Diagnosis
The diagnostic evaluation is based on the histologic features and the clinical behavior of the lesion.
Differential diagnosis
Osteoid osteoma, ossifying fibroma, condensing osteitis,
Pagets disease, fibrous dysplasia, chondroblastoma, cementoblasoma, aneurysmal bone cyst and giant cell granuloma.
Treatment and prognosis
Surgical excision, malignant transformation (osteosarcoma)
within a benign osteoblastoma is very rare.
Odontogenic lesions
Ameloblastoma
Odontogenic myxoma
Odontogenic keratocyst
Non-odontogenic lesions
Hemangioma
Aneurysmal bone cyst
Traumatic bone cyst.
Clinical features
Most cases arise in those less than 30 years of age with
female predominance. It occurs exclusivity in mandible or
maxilla; rarely in facial bones. Occurrence in mandible
352
Radiographic features
Any alveolar region may be affected and radiographs may
show either a saucerization of underlying bone, periodontitis
Inflammatory
cells
Poncet and Dor in 1897 rst described pyogenic granuloma as granuloma pyogenicum. Over the years various
authors have suggested other names such as granuloma
gravidarum, pregnancy tumor, Crocker and Hartzells disease, vascular epulis, benign vascular tumor, hemangiomatosis granuloma, epulis telangiectaticum granulomatosa,
and lobular capillary hemangioma.
Connective
tissue stroma
Etiology
Figure 18
Epithelium
Blood vessels
Giant cells
The etiology of the lesion is unknown, though it was originally believed to be a botryomycotic infection. It is theorized that pyogenic granuloma possibly originates as a
response of tissues to minor trauma and/or chronic irritation, thus opening a pathway for invasion of non-specific
microorganisms, although these microorganisms are seldom demonstrated within the lesion.
Clinical features
of underlying tissues, or an isthmus of soft tissue connecting to an intraosseous central giant cell granuloma.
Histopathology
The peripheral giant cell granuloma is comprised of an
unencapsulated aggregation of rather primitive but uniform mesenchymal cells with oval, pale nuclei and with a
moderate amount of eosinophilic cytoplasm. Mitotic activity is not unusual in the lesion and may even be pronounced in lesions developing in children and adolescents.
Stromal cells may be spindled with a background of collagenic fibers, or may be rounded with a less fibrotic background. A thin band of routine fibrovascular tissue
separates the lesion from the overlying epithelium, often
with dilated veins and capillaries. When surface ulceration
is present, the ulcer bed consists of routine fibrinoid
necrotic debris over granulation tissue (Figure 18).
Treatment
Peripheral giant cell granuloma is treated by conservative
surgical excision followed by curettage of any underlying
bony defect and careful scaling and root planing of associated teeth. A recurrence rate of 10% or more has been
reported, hence, re-excision may be necessary.
Pyogenic Granuloma
Pyogenic granuloma or granuloma pyogenicum is a wellknown oral lesion. It is a localized granulation tissue
overgrowth in reaction to mild irritation. The name pyogenic granuloma is a misnomer since the condition is not
associated with pus and does not represent a granuloma
histologically.
Figure 19
Figure 20
Stratified
squamous
epithelium
Vascular
spaces
Inflammatory
cells
Connective
tissue
Diagnosis
Although pyogenic granuloma can be diagnosed clinically
with considerable accuracy, radiographic, and histopathological investigations aid in confirming the diagnosis and
planning the treatment. Marx et al (2003) reported that
radiographs are advised to rule out bony destruction suggestive of malignancy or to identify a foreign body or sharp
restorative margin that would need to be removed with the
lesion. Long-standing pyogenic granulomas, like other irritation hyperplasias can show dystrophic calcifications. The
radiographic appearance of such calcifications varies from
barely perceptible, fine grains of radiopacities to larger,
irregular radiopaque particles that rarely exceed 0.5 cm in
diameter.
Histopathologic features
All clinically suspected pyogenic granulomas must be biopsied to rule out more serious conditions as mentioned previously. The pathology is distinct consisting of a matrix of
edematous connective tissue in which numerous thin walled
vascular channels can be seen. These vessels sometimes
are organized in lobular aggregates, and some pathologists
require this lobular arrangement for the diagnosis (lobular
capillary hemangioma). There is also moderately dense
mixed cellular infiltrate. The overlying stratified squamous
epithelium may be atrophic or hyperplastic, and is usually
degenerated or ulcerated in large areas; and the ulcer edge
may have a primitive dysplastic appearance (Figure 20).
Treatment
Treatment of pyogenic granulomas consists of conservative
surgical excision, which is usually curative. Although these
are reactive hyperplasias, they have a relatively high rate
of recurrence after simple excision, especially in pregnant
patients. Pierson and Pierson reported a recurrence rate of
354
Figure 21
Differential diagnoses
Differential diagnoses include hemangioma, fibroma, granuloma, embryonal rhabdomyosarcoma, malignant granular
cell myoblastoma, alveolar rabdomyosarcoma, chondrogenic and osteogenic sarcoma and schwannoma.
Treatment
Surgery is the only possible treatment of these tumors.
Surgery should not be radical; it minimizes the danger of
damaging underlying alveolar bone and developing tooth
buds. Delay in operation may cause airway obstruction and
feeding difficulty. The tumor should be removed during
the immediate postnatal period.
Lipoma
A lipoma is a benign neoplasm composed of fat cells, oral
and oropharyngeal lipomas are rather rare.
Etiology
Histopathology
These tumors have large cells with an eosinophilic granular cytoplasm, but the congenital epulis is covered with a
normal gingival epithelium. The congenital epulis is more
vascularized.
Differential diagnosis
Figure 22
Figure 23
Epithelium
Antoni A tissue
Connective
tissue
capsule
Verocay bodies
Adipocytes
Treatment
Surgical removal is the treatment of choice. Recurrences
have not been reported with the exception of infiltrating
and intramuscular lesions.
Neurogenic Tumors
Although the distinction between a schwannoma (neurilemmoma) and a neurofibroma may be debatable, most
authors adhere to the concept that there are indeed two
separate entities.
Neurofibroma
Schwannoma (Neurilemmoma)
Clinical features
A schwannoma is a benign neurogenic neoplasm composed of Schwanns cells. In a review from the literature
152 cases of oral schwannomas were collected (Gallo et al,
1977).
Clinical features
The tumor may occur at all ages and does not show a preference for men or women. A schwannoma is a slowly growing, rather circumscribed, submucosally located tumor that
may be painful. No characteristic clinical features appear.
It may occur at any site in the oral cavity and rarely involves
the oropharynx.
Histopathologic features
Histologically, one sees an encapsulated tumor composed
of two cell typesthe so-called Antoni type A and Antoni
type B cells. The A cells have elongated nuclei and are
often arranged in a palisade pattern, including hyalinized
356
Histopathologic features
Histopathologic features
Treatment
With a single neurofibroma, management consists of surgical removal. With multiple or massive involvement, surgical removal may be impossible to carry out and is indicated
only when malignant changes are suspected. With von
Recklinghausens disease, there is a 515% risk of malignant
degeneration. This seems especially true for deeply located
lesions (Maceri and Saxon, 1984).
Traumatic Neuroma
A traumatic neuroma is a reactive hyperplasia caused by
injury of a nerve.
Clinical features
Traumatic neuromas may occur anywhere in the oral cavity.
Occurrence in the oropharynx is exceptional. The lesion usually manifests itself as a small submucosal nodule that may
be painful at palpation. No characteristic clinical features
appear.
Treatment
Management of a rhabdomyoma consists of surgical
removal.
Leiomyoma
A leiomyoma is a benign neoplasm composed of smooth
muscle cells. The source of a smooth muscle tumor in the
oral cavity is believed to be the walls of blood vessels or
undifferentiated mesenchymal cells. Occurrence of a leiomyoma in the oral cavity is rare. In two reviews of the
literature (Natiella et al, 1982; Praal et al, 1982) approximately 80 oral leiomyomas were collected.
Histopathology
Histologically, masses of irregularly arranged nerve fibers
and Schwann cells are seen to spread diffusely throughout
the tissue, mimicking to some extent the picture of a neurofibroma.
Treatment
Management consists of conservative surgical removal, if
possible, followed by coagulation of the adherent nerve.
Recurrence is rare.
Rhabdomyoma
A rhabdomyoma is a benign neoplasm of striated muscle.
It is an exceedingly uncommon tumor.
Clinical features
The male-to-female ratio is more than 2:1. The mean age of
patients with a rhabdomyoma is about 40 years. Although
extracardiac rhabdomyomas show a preference for the
head and neck, occurrence in the oral cavity and oropharynx is rare. The floor of the mouth is the most common site.
Multifocal appearance is exceptional (Schlosnagle et al,
1983). The clinical presentation is a submucosal swelling
without any specific signs or symptoms.
Clinical features
The majority of reported leiomyomas of the oral cavity and
oropharynx were small, circumscribed, and asymptomatic
swellings, covered with an apparently intact mucosa. They
were either single or multiple.
Histopathologic features
A leiomyoma is composed of whorls of smooth muscle cells.
The diagnosis of leiomyoma can be difficult to make just
from light microscopic examination. The tumor should be
differentiated from fibromatosis and schwannoma on the
one hand and leiomyosarcoma on the other hand.
Management
Management consists of surgical removal with rare
recurrence.
tailored treatment plans. However, hemangioma still continues to be used as a clinical and pathological description
of different types of vascular anomalies.
History
Before the 1980s, the terminology that was used to describe
vascular anomalies was confusing and ambiguous. The
descriptive terminology used in the past (port-wine stain,
strawberry hemangioma, salmon patch) conjure up visual
approximation to the lesions but have no correlation with
the biological behavior or natural history of these lesions.
Mulliken and Glowacki introduced a simple classification
in 1982 that was based on the clinical, histochemical, and
cellular criteria to distinguish between the various vascular anomalies.
Hemangioma
Treatment
The natural history of hemangiomas should influence the
timing and type of intervention. Benign neglect is often
advocated. A useful approach to the management of hemangiomas can be based on the following:
In general, life-threatening and sight-threatening hemangiomas should be dealt with, regardless of the stage of the
lesion. Active intervention should be considered in all disfiguring hemangiomas, but each case should be managed
on its merits after careful discussion and counseling, to prevent potential psychosocial trauma and cosmetic deformity.
Ethunandan and Mellor stated that prednisolone is the
first-line drug of choice for the treatment of life- or sightthreatening hemangiomas.
Clinical features
This is the most common tumor in white infants, and the
head and neck region is the most commonly involved site
(60%). Most lesions are solitary (80%) and girls are more
affected than boys (3:1). Multiple cutaneous lesions (three
or more) are often associated with visceral involvement.
Facial hemangiomas have a predilection for segmental
distribution and for regions of embryological fusion
(Figure 24AC). Hemangiomas usually appear soon after
birth, typically proliferate during the 1st year of life and
then involute during the childhood years (up to 12 years).
Classification
Superficial hemangiomas originate from the papillary
dermis and present as bright red macular or papular
masses.
Deep hemangiomas originate from the reticular dermis
or subcutaneous tissues and appear as bluish or relatively
colorless masses.
Compound hemangiomas have supercial and deep components and were previously called capillary cavernous
hemangiomas. The current classication of hemangiomas
and vascular malformations is summarized in Table 9.
Histopathology
The histological features are dependent on the stage of the
lesion.
Proliferative phase: The lesion is highly cellular and contains plump proliferating endothelial cells and pericytes, with
a high mitotic activity and numerous mast cells. Vascular
channels are not prominent (Figure 24D).
Involutive phase: The endothelial cells are attened, the
cell turnover is normal and there are few mast cells.
358
Vascular Malformations
These are errors of morphogenesis that are populated by
stable mature vascular endothelium.
Clinical features
Both sexes are equally affected. Vascular malformations
are always present at birth (though some may not be
apparent until a later stage) and in contrast to hemangiomas, these never proliferate or involute. Instead, these
expand slowly and relentlessly throughout life, in pace
with the growth of the patient. Trauma, puberty, and pregnancy can cause accelerated growth. Unlike hemangiomas, vascular malformations are associated with skeletal
abnormalities in up to 35% of cases. Capillary malformations may be associated with SturgeWeber and Klippel
Trenaunay syndromes.
Classification
These lesions are subclassified according to the predominant type of vessel and characteristics of flow (Box 2).
Waner et al graded these lesions according to the degree of
ectasia of the vessels into grades IIV which correlate well
with the clinical features and outcome of treatment. The
degree of ectasia increases with age and the clinical features
depend on the depth and size of the lesion. The lesions are
usually soft, compressible, and enlarge in size when venous
pressure is increased.
Histopathology
Venous malformations are characterized by an abnormal
collection of veins, which do not have any demonstrable
mitotic activity in endothelial or pericyte cells. Capillary
Figure 24
A
Proliferating
capillaries
Epithelium
Extravasated
red blood
cells
(A, B, C) Clinical photograph of hemangioma. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
(D) Histopathological section of hemangioma. Courtesy: Department of Oral Pathology and Microbiology, MCODS, Mangalore
Capillary Malformations
Capillary malformations never regress spontaneously.
They often darken and thicken with age and the rate of
progression is probably related to the degree of absence of
the autonomic nerves in addition to other factors such as
hormonal influences and trauma. Troilius and Wrangsjo
in their recent study have emphasized the psychological
aspects of the birthmark and have suggested earlier
intervention to counteract the undesirable psychosocial
effects.
Arteriovenous Malformations
These lesions show slow but relentless growth which is the
norm and high-grade lesions present earlier in life and
expand more rapidly, whereas low-grade lesions present
Lymphatic Malformations
These lesions are the most difficult to eradicate. Microcystic
lesions in particular are diffuse, do not respect tissues planes,
and it is difficult to distinguish involved tissue from normal
tissue.
Macrocystic lesions, on the other hand, are more localized and respect tissue planes and are more easily excised.
Hancock advocated these options for treatment which
included laser ablation, excision, and sclerotherapy. Carbon
dioxide laser should be reserved for supercial mucosal
lesions. Because uid-lled vesicles are almost always
359
Fibroma
The fibroma, also referred to as irritation fibroma, is by far
the most common of the oral fibrous tumor-like growths.
While the terminology implies a benign neoplasm, most if
not all fibromas represent reactive focal fibrous hyperplasia
due to trauma or local irritation. Although the term focal
fibrous hyperplasia more accurately describes the clinical
appearance and pathogenesis of this entity, it is not commonly used.
Clinical features
A fibroma may occur at any oral site, but it is seen most
often on the buccal mucosa along the plane of occlusion of
the maxillary and mandibular teeth. It is a round-to-ovoid,
asymptomatic, smooth-surfaced, and firm sessile or pedunculated mass (Figure 25A, B). The diameter may vary from
1 mm to 2 cm. The surface may be hyperkeratotic or ulcerated due to repeated trauma. Fibromas are most often
observed in adults, but they may occur in individuals of
any age and either sex.
360
Histopathology
Histologically, a fibroma is an unencapsulated, solid, nodular mass of dense and sometimes hyalinized fibrous connective tissue that is often arranged in haphazard fascicles.
A mild chronic inflammatory infiltrate may be present. The
surface epithelium may be hyperkeratotic, either hyperplastic or atrophic, and it may be ulcerated (Figure 25C).
Differential diagnosis
The clinical differential diagnosis of a fibroma includes giant
cell fibroma, neurofibroma, peripheral giant cell granuloma, mucocele, and benign and malignant salivary gland
tumors.
Treatment
Conservative excisional biopsy is curative, and its findings
are diagnostic. Recurrence is possible however, if the offending irritant persists.
Figure 25
A
D
Epithelium
(stretched)
Bundles of
collagen fibers
(A, B) Irritational fibroma on the buccal mucosa. Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore. (C) Histopathological picture of fibroma. Courtesy: Department of Oral Pathology and
Microbiology, MCODS, Mangalore
Fibromatoses
Histopathology
Clinical features
Differential diagnosis
Table 9
Condition
Odontogenic myxoma
Honeycomb appearance
CEOC
Sunburst appearance
Osteoblastoma, osteosarcoma
Sunray appearance
Hyperparathyroidism
Eggshell appearance
Ameloblastoma
Garres osteomyelitis
Myofibroblastoma
Myofibroblasts are spindle-shaped cells with features of
both fibroblasts and smooth muscle cells. Myofibroblasts
have been identified in lesions other than myofibromas, but
when they are the predominant cell type in a tumor, the
terms myofibroma (if solitary) or myofibromatosis (if multicentric) are applied.
Clinical features
Tumors of myofibroblasts may occur in either sex and in
patients of all ages, with a mean age of 26.6 years. Solitary
myofibromas have a head and neck predilection, with the
mandible being the most common site of occurrence. The
most common oral soft tissue sites are the tongue, lips, and
buccal mucosa.
Radiographic features
Intraosseous jaw lesions most often manifest as well-defined
unilocular or multilocular radiolucencies.
Differential diagnosis
The clinical differential diagnosis for oral myofibroma
includes irritation fibroma, fibromatosis, peripheral giant
cell fibroma, neurofibroma, leiomyoma, and benign and
malignant neoplasms of the minor salivary glands.
Treatment
Treatment for oral myofibromas is conservative excision.
The recurrence rate is low, and spontaneous regression has
been reported.
362
Table 10
Odontogenic carcinomas
Odontogenic sarcomas
Malignant ameloblastoma
Ameloblastic fibrosarcoma
(ameloblastic sarcoma)
Primary intraosseous
carcinoma
Ameloblastic fibro-dentinosarcoma
Ameloblastic fibro-odontosarcoma
Malignant variants of
other odontogenic
epithelial tumors
Odontogenic carcinosarcoma
Malignant changes in
odontogenic cysts
ODONTOGENIC CARCINOMAS
(2004)
Odontogenic carcinomas
Odontogenic sarcomas
1. Metastasizing malignant
ameloblastoma
1. Ameloblastic fibrosarcoma
(ameloblastic sarcoma)
2. Ameloblastic carcinoma
a. Primary intraosseous carcinoma
b. Secondary (dedifferentiated)
intraosseous
c. Secondary (dedifferentiated)
extraosseous
2. Ameloblastic fibrodentinosarcoma
a. Ameloblastic fibroodontosarcoma
Terminology used to describe diverse odontogenic carcinomas is varied and confusing. We will discuss the important
lesions.
Ameloblastic Carcinoma
It is a malignant epithelial proliferation that is associated
with an ameloblastoma (carcinoma ex ameloblastoma) or
histologically it resembles an ameloblastoma (de novo
ameloblastic carcinoma). It is an aggressive neoplasm that
is locally invasive (extending out of bone to involve the
infratemporal fossa, parapharyngeal space, the masticator
space, or cervical soft tissue) and can spread to regional
lymph nodes or distant sites, such as lungs and bones and
myocardium.
363
carcinoma. These arise de novo or as dedifferentiated carcinomas from a pre-existing benign peripheral ameloblastoma.
Solid PIOC
These occur mainly in the posterior mandible, are more
often seen in males, may metastasize to regional lymph
nodes, and have a poor prognosis. It occurs commonly in
elderly patients (mean age 50 years). These tend to occur
in the mandibular body where they may compress the
inferior alveolar nerve causing pain and/or paresthesia.
Loosening of teeth and mandibular expansions are signs.
Radiographically, these may be well-circumscribed as well
as more diffuse with irregular moth-eaten borders. The
tumors metastasize regionally and distantly and show a
3040% 5-year survival.
Cystic PIOC (PIOC arising in an odontogenic cyst, PIOC
ex odontogenic cyst) Cystic PIOC is a squamous cell carcinoma that demonstrates a cystic component with a
lumen that contains fluid or keratin and a lining of stratified squamous epithelium that exhibits cytological atypia.
Primary intraosseous carcinomas may develop from a still
recognizable precursor lesion such as the epithelial lining
of an odontogenic cyst. The linings of residual apical periodontal cyst, OKC and dentigerous cyst have shown malignant transformation. Moreover, reduced enamel epithelium
has been documented as tissue of origin in cases in which
an impacted tooth was presented within the tumor.
Co-occurrence of squamous odontogenic tumor and ameloblastomas has been reported. Most carcinomas arising
from the cyst linings are well or moderately differentiated.
Carcinoma ex dentigerous cyst The most common
odontogenic cyst to show carcinomatous changes is the
dentigerous cyst. Few cases that lacked rigorous evidence
of dentigerous cyst formation, but showed thickening of
pericoronal soft tissue that was associated with an impacted
tooth, were reported as carcinoma ex dentigerous cyst.
ODONTOGENIC SARCOMAS
These tumors may arise de novo or from a pre-existent ameloblastic fibroma or ameloblastic fibro-odontoma. Whether
an odontogenic sarcoma displays deposition of hard dental
tissues has not shown any prognostic significance. Odontogenic sarcomas arising from pre-existing ameloblastic
365
Ameloblastic Fibrosarcoma
It is also known as odontogenic sarcoma, ameloblastic sarcoma. It is a malignant proliferation of connective tissue
cells that contains benign odontogenic epithelium that is
similar to ameloblastic fibroma. Typically, it presents in the
mandible with an age range from 8 to 83 years. In the mandible, posterior region is affected more. It demonstrates an
expansile radiolucency with indistinct margins, evidence of
extraosseous soft tissue extension, and sometimes, an associated impacted molar. Other radiographic appearances
include destructive/permeative, unilocular or multilocular.
Typically, the patients do not develop metastases; they
form the group of a locally aggressive neoplasm. The sarcoma is most often treated by a wide surgical excision and
postoperative radiation therapy without elective neck dissection. Ameloblastic brosarcoma has the histologic architecture similar to an ameloblastic broma.
Slender budding and branching epithelial cords of
bland cuboidal to columnar cells with uniform nuclei or
epithelial islands that are indistinguishable from those are
seen in follicular ameloblastomas are separate widely by
hypercellular connective tissue that exhibits plump polygonal to fusiform stromal cells that show mild to moderate
cytologic atypia and numerous mitotic gures in a pale
hypocollagenous myxoid extracellular matrix.
Ameloblastic brosarcoma can show focal evidence of
dentin formation or dentin and enamel formation, resulting
in the terms ameloblastic dentinosarcoma and ameloblastic
odontosacrcoma, respectively. The histologic differential
diagnosis includes low grade spindled ameloblastic carcinoma that demonstrates plump broblastic cells that are
associated with ameloblastomatous islands and sheets. The
distinction between the two is made by cytokeratin which is
found to be reactive by broblastic cells of spindled ameloblastic carcinoma.
Odontogenic Carcinosarcoma
It is a tumor architecturally resembling an ameloblastic
fibroma in which both the epithelial and the connective
tissue components show cytologic evidence of malignancy.
For these extremely rare lesions that combine carcinomatous and sarcomatous elements and recognizable as odontogenic if the epithelial component resembles that of an
ameloblastoma, the designations malignant ameloblastoma
and fibrosarcoma or odontogenic carcinoma with sarcomatous proliferations have been used.
The increase in the number of reported malignant odontogenic tumors will help in the future to attempt studies
366
Verrucous Carcinoma
Verrucous carcinoma has also been referred to as snuff
dippers cancer and Ackermans tumor. Rock and Fisher in
1960 coined the term oral florid papillomatosis.
Lauren Ackerman in 1947 rst described this less severe
variant of squamous cell carcinoma associated with spittobacco chewing habit. He suggested that verrucous carcinoma needs to be considered as a distinct entity as it
exhibits a characteristic morphologic appearance and specic clinical behavior. He noted that even extension lesions
had excellent prognosis with proper treatment.
This lesion has a predilection for mucous membranes of
the head and neck and is most commonly found in the oral
cavity followed by the larynx. Since its earlier description,
verrucous carcinoma has been also reported to occur in the
esophagus, paranasal sinuses, nasal fossae, genital and anal
mucosa, soles of feet, breast and axilla.
Enriquez et al (1980), Pomatto et al (2001) and Nooshin
Mohtasham et al (2008) reported verrucous carcinoma arising from an odontogenic cyst.
Predisposing and etiological factors
The exact etiology is still not understood. Chewing tobacco
is the primary etiologic factor for these tumors. Most of these
tumors originate from the site of the placement of tobacco.
It has been suggested that opportunistic viruses such as
HPV-6 and 16, act in tandem with frank carcinogenesis to
promote development of verrucous carcinomas. Few other
authors suggest that the lesions develop at sites of chronic
irritation and inammation.
Clinical features
Verrucous carcinoma is usually seen between the 5th and
7th decade of life and commonly in white men. It is estimated that these tumors account for about 110% of all
oral squamous cell carcinomas.
In the initial stages, verrucous carcinoma appears as
white, translucent patches on an erythematous base. As they
mature they begin to take on a cauliower-like papillomatous growth with a supercial pebbly surface (Figure 26).
Figure 26
Figure 27
Parakeratin plug
Cleft lined by
parakeratin
Epithelium with broad
pushing borders
Subepithelial band of
chronic inflammatory
cell infiltrate
368
Management
Neville et al (2007) studied the efficacy of 5% imiquimod
(commercially available in India as Imiquad 5% cream.
Imiquimod is a keratolytic) in the treatment of nodular
basal cell carcinoma after initial treatment with curettage.
In their study, 5% imiquimod was applied locally once a
day, 5 times per week for 6 weeks, following curettage.
They concluded that imiquimod cream was an effective
treatment modality.
Gross et al (2007) studied the potential of 5%
5-uorouracil cream for the treatment of small supercial
basal cell carcinoma. They used 5% 5-uorouracil cream
twice daily for up to 12 weeks. The results showed that the
histologic cure rate was 90% and the mean time to clinical
cure was 10.5 weeks.
Healsmith et al (1991) used intralesional interferon
alpha-2b (IF-2b) for the treatment of basal cell carcinoma. The injected nine intralesional injections of IF-2b
(1.5 million units dissolved in 0.20.5 ml water), 3 times
per week for 3 weeks. At a 3-month follow-up, out of the
11 tumors they treated, six tumors had resolved both clinically and histologically, three tumors had reduced in size
and one tumor grew larger.
Surgical excision for basal cell carcinoma is still the
most popular modality of treatment. It is recommended
that an excision margin of 4 mm around the tumor be maintained. Mohs micrographic surgery offers high cure rates for
basal cell carcinoma (5-year cure rate of 99% for primary
tumors and up to 95% for recurrent basal cell carcinoma).
During this surgical technique, serial frozen sections are
examined histologically until all margins are clear.
Figure 28
Superficial spreading
Nodular
Lentigo maligna
Acral lentiginous.
1.
Histologic features
Histologically a high concentration of melanocytes may be
seen in the biopsy specimen. These atypical melanocytes
are larger, exhibit nuclear pleomorphism and hyperchromatism in the epithelial and connective tissue junction
(Figure 29).
Based on the extent of penetration through the dermis
to the subcutaneous fat, Clark suggested ve levels of
microstaging:
2.
369
Table 12
Type of melanoma
Characteristic features
Superficial spreading
Nodular melanomas
Represent approximately 1015% of cutaneous melanomas (almost 30% of these occur in the head and neck region)
Common on the trunk of males
Usually symmetrical and uniform
Dark brown or black in color
The radial growth phase may not be evident (due to its high risk)
Amelanotic melanomas represent approximately 5% of all nodular melanomas
Lentigo maligna
melanoma
Acral lentiginous
melanoma
5.
Figure 29
3.
4.
370
Fibrosarcoma is a malignant neoplasm of the fibroblastic origin. Fibrosarcoma may occur anywhere in the body.
Extremities are the most commonly affected. However,
only about 10% involve the head and neck region.
Figure 30
Long fascicle
Short fascicle
Interlacing
fascicles
Histopathologic features
Clinical features
Radiographic features
In the head and neck region, CT image may reveal a multilocular tumor with smooth, well-defined margins and
heterogeneous enhancement after injection of contrast
medium. Occasionally, internal calcification may be
evident.
Histopathologic features
Histopathologically synovial sarcoma is divided into
biphasic, monophasic (fibrous and epithelial) and poorly
differentiated (round cell) subtypes. A myxoid variant has
also recently been described.
The biphasic subtype is composed of spindle and epithelial cell elements. The monophasic type is composed
primarily of spindle cells and rarely epithelial cells.
The poorly differentiated subtype shares features of
both the mono-and biphasic types along with poorly differentiated areas characterized by high cellularity, pleomorphism, numerous mitoses and round cell morphology.
In some patients, necrosis may be evident.
Immunohistochemically, the epithelial-like cells are
positive for cytokeratins and epithelial membrane antigen,
and the spindle cells are positive for vimentin and bronectin.
All the subtypes are characterized by a specic t(x;18)
(p11.2;q11.2) chromosomal translocation.
Synovial Sarcoma
Knox in 1936 proposed the term synovial sarcoma as the
earliest of the cases reported, showed some histological
resemblance to synovial tissue. However, in the present
times it is widely accepted that the tumor does not arise
from the synovium.
Synovial sarcoma arises from the pluripotent mesenchymal cells of the para-articular surfaces. It commonly
affects the extremities. It is estimated that about 10% of all
soft tissue sarcomas are synovial sarcomas and around
310% occur in the head and neck region.
Clinical features
Synovial sarcomas usually affect men in the 2nd to 4th
decade of life. The common sites of involvement in the head
and neck are the hypopharyngeal and retropharyngeal
regions. Involvements of the parotid gland, tongue, buccal
mucosa, soft palate, floor of mouth, mandible and TMJ
have also been reported.
Many of the patients may complain of a steadily growing
painful mass over a period of many months. Some individuals may complain of difculty in swallowing, dyspnea
and hoarseness of voice.
Almost 50% of the synovial sarcomas may metastasize
to the lung. Other sites for metastases include the lymph
nodes and bone marrow.
372
Grade I lesions: These resemble benign cartilage, having a relatively uniform, lobular histologic appearance
and no metastasis.
Grade II lesions: Higher recurrence rate than Grade I
lesions. These exhibit occasional mitotic figures. The
rate of metastasis is approximately 10%.
Grade III lesions: These are more cellular and pleomorphic in appearance, with a marked increase in the
number of mitotic figures. The rate of metastasis in
grade III lesions is more than 70%.
Radiographic features
Radiographically, chondrosarcomas can present as ill-defined
radiolucencies with few radiopaque foci (calcification/
ossification of cartilaginous matrix). Occasionally, these
may present as areas of dense radiopacification with illdefined margins. Involvement of the cortices can result in a
sunburst pattern.
Symmetric widening of periodontal ligament space and
resorption of the roots of teeth may be evident.
Wide surgical excision is the treatment of choice for liposarcoma. Lymph node dissection is not indicated unless
metastasis is strongly suspected.
Some authors feel that radiation therapy along with
surgical excision may improve the condition of the patient.
The 5-year survival rate is approximately 6070%.
Management
The treatment of choice is radical surgical excision with
negative margins. Distant metastasis is rare. However, distant metastasis to the sternum, vertebrae and lungs has
been reported.
Figure 31
It occurs most often in the long bones, with predilection for the distal femoral metaphysis, proximal tibia, and
humeral metaphysis.
Most of the osteosarcomas originate intramedullary (classic or conventional osteosarcoma). However, the other
relatively rare types of osteosarcomas are the juxtacortical
(periosteal and parosteal [Figure 31]) and extraskeletal.
Many authors have described osteosarcoma of the jaws
as a specic entity, with a clinical behavior different from
osteosarcoma of other skeletal bones.
Predisposing factors
The exact pathogenesis for the tumor is still unknown.
However, various predisposing factors have been proposed
such as trauma, virus, genetic mutations, pre-existing bone
cyst, Pagets disease, osteogenesis imperfecta, osteochondroma fibrous dysplasia and previous history of radiation
(radiation-induced sarcomas).
It is suggested that the average latent period between
radiation treatment and development of sarcoma is 12.5
years, following radiation dose of 45 Gy. It is believed that
individuals who harbor the mutation in tumor suppressor
genes like p53 and retinoblastoma gene are more prone to
develop osteosarcomas.
Clinical features
Osteosarcomas affecting the jaw bones are usually seen in
the 3rd and 4th decades of life. However, children have
also been affected. Males are slightly more commonly
affected.
The mandible and maxilla are equally affected. The
symphysis, ramus and posterior parts of the body of the
374
Ng et al (2001) in a pioneering study described the ultrasound features of osteosarcoma of the mandible. In the
mandible they recommend that the most accessible surface
is the buccal cortex. Features such as bone thinning,
Figure 32
A
(AC) 3D-reconstructed images showing changes seen in osteosarcoma. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
erosion, expansion, and the sunray appearance of the buccal cortex were all detected by ultrasound imaging.
Figure 33
Histopathologic features
Histologically, osteosarcomas are categorized as osteoblastic, chondroblastic, fibroblastic and telangiectatic.
The characteristic features of osteosarcoma are the presence of atypical osteoblasts. The osteoblasts are arranged
in a disorderly fashion (Figure 33). Irregular sheets of osteoid
may be seen. The telangiectatic type exhibits extensive
blood-lled spaces. However, this type is generally seen in
young adults.
Management
The choice of treatment for osteosarcoma is radical surgery along with adjuvant chemotherapy. As most of the
Osteoid
Trabeculae of bone
Chondroid tissue
Pleomorphic,
hyperchromatic
osteoblasts
375
Vascular Origin
Kaposis Sarcoma
Kaposis sarcoma is a vascular tumor that was first described
by Moritz Kaposi, a Hungarian dermatologist in 1872. He
described a distinct variety of skin tumors in five male
patients in their 6th and 7th decades of life. He termed these
tumors as idiopathic multiple pigmented sarcoma of the skin.
There is a strong evidence to show that Kaposis sarcoma is caused by human herpes virus 8 (HHV-8).
Kaposis sarcoma has four distinct variants: classic or
Mediterranean, endemic or African, epidemic or AIDS
associated, post-transplant or iatrogenic immunosuppression associated Kaposis sarcoma. The histopathological
and immunohistochemical features of all forms of Kaposis
sarcoma are similar.
Clinical features
Radiographic features
Extensive ill-defined destruction of the bone may be the
only finding in jaw lesions. A laminated periosteal reaction
(onion skin/peel appearance) or sunray appearance is usually a feature of long bones. Occasionally, widening of the
periodontal ligament space, loss of lamina dura, root resorption and/or displacement and destruction of unerupted
tooth follicles are seen.
376
Figure 34
Management
Angiosarcoma
Angiosarcoma is an uncommon malignancy originating
from the vascular endothelium of either the blood vessels
or lymphatic channels. It generally occurs in the skin and
subcutaneous or skeletal muscle and has been reported to
occur in the spleen, bone, liver, and breast. Angiosarcomas
are usually seen in the 5th decade of life. However, individuals in the age range from 6 to 90 years have also been
affected. More than half the angiosarcomas affect the head
and neck region. The scalp and forehead are the most
commonly affected sites.
Oral and salivary gland angiosarcomas are exceedingly
rare, comprising only about 2% of all angiosarcomas.
Angiosarcomas may involve the lip, buccal mucosa, oor
of mouth, gingiva, tongue and the mandible.
The intraoral lesions may appear as simple bruise in the
early stages. As the neoplasm advances, it tends to become
nodular and ultimately ulcerate.
The differential diagnosis for angiosarcoma includes
hemangioma, pyogenic granuloma, hemangiopericytoma,
Kaposis sarcoma and hemangioendothelioma.
Histopathologically, the tumor is characterized by the
presence of proliferation of endothelium-lined vascular
channels forming a network of anastomosis with increased
mitotic activity.
Management
Angiosarcomas are best treated with surgical excision and
radiation therapy.
Muscle Origin
Leiomyosarcoma
Leiomyosarcoma is a malignant mesenchymal neoplasm
exhibiting smooth muscle differentiation. It accounts for
377
Figure 35
Clinical features
Oral leiomyosarcomas may occur at any age. About 310%
of the leiomyosarcomas. Smooth muscle tumors in the oral
cavity may originate from the arterial tunica media, the
ductus lingualis, the circumvallate papillae, and pluripotential mesenchymal cells, which are rich sources of
smooth muscle tissue.
A leiomyosarcoma in the oral cavity may be primary or
secondary (metastatic from another location). The common
sites of metastatic involvement are the mandible for bony
depositions and the gingivae and tongue for soft tissue
deposits.
It is estimated that 45% of the reported cases affecting
the oral cavity occur in the jaw bones.
Clinically they may appear as painless or occasionally
painful well-circumscribed mass, rmly adherent to the
surrounding tissues. Some of these tumors may reveal
ulcerations.
Histopathologic features
Leiomyosarcoma is characterized by sheets of sweeping,
alternating bundles and fascicles of densely packed spindle cells with abundant fibrillar eosinophilic cytoplasm
and indistinct cytoplasmic borders. The nucleus is usually
centrally located and blunt-ended, squared-off or cigarshaped. Occasional cells have perinuclear vacuoles.
Massons trichrome staining and immunohistochemical evaluation for muscle antigens are helpful in differentiating leiomyosarcoma from other sarcomas. Positive
reactions for desmin, vimentin, smooth-muscle actin and
h-caldesmon have been demonstrated in this neoplasm.
Management
Radical surgical excision is the treatment of choice along
with chemotherapy and radiotherapy. It is estimated that
the 5-year survival rate is about 30%.
Types of rhabdomyosarcomas
Based on their anatomical location of occurrence, these
tumors are broadly categorized as orbital, parameningeal
and non-orbital non-parameningeal forms. Parameningeal
tumors are said to have the worst prognosis.
The International Classication of Rhabdomyosarcoma
in 1994, divides this tumor into four subgroups, namely,
botryoid and spindle cell RMS, embryonal RMS, alveolar
RMS, and undifferentiated sarcoma.
Oral features
These tumors are generally seen in the first decade of life.
These are also occasionally seen in adolescents and young
adults. Clinically a rapidly growing painless mass may be
seen. Palate, tongue and alveolar ridge have been reported
as the common sites affected (Figure 35). In the advanced
stages of the disease, pain, paresthesia, loosening of the
teeth, and trismus may be seen.
Management
The primary tumors are best managed with surgical resection, multidrug chemotherapy and radiation therapy. The
5-year survival rate is estimated to be about 60%.
Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is considered as the most common soft tissue sarcoma affecting children. It is a malignant
neoplasm of skeletal muscle origin. Weber in 1854 is credited for giving the first description of rhabdomyosarcoma.
It is estimated that about 35% of RMS arises in the head
and neck.
378
Radiographic features
The tumor is managed with surgery and radiation therapy. The tumors have a high recurrence rate as they tend
to spread along the involved nerve. It is estimated that the
prognosis for patients with neurofibrosarcoma arising de
novo is about 50% for 5-year survival, whereas it is 15% for
neurofibrosarcoma arising in cases of neurofibromatosis.
Radiographs may exhibit an ill-defined radiolucency suggestive of destruction of the bone. Widening of the inferior alveolar canal may also be seen.
Histopathologic features
The typical histopathological feature is the presence of fascicles of atypical spindle-shaped cells, which may resemble
Tumors of lymphoid origin, namely, Hodgkins and nonHodgkins lymphoma and hematological malignancies
including leukemia and myeloma are described in Chapter 18
on Systemic Disorders and their Clinical Implications.
379
CHAPTER
14
Oral Cancer
SV Kumaraswamy, V Jeevan Prakash,
Praveen BN, Ravikiran Ongole
Tobacco
TNM Staging
Alcohol
Nodal Metastasis
Systemic Health
Ionizing Radiation
Role of Viruses
Role of Nutrition
Oral Hygiene and Dental factors
Surgical Treatment
Radiation Therapy
Chemotherapy
It has been estimated that 43% of cancer deaths worldwide are due to tobacco, unhealthy diet, physical inactivity
and infections. Tobacco use and excessive alcohol consumption have been estimated to account for about 90% of cancers in the oral cavity; the oral cancer risk increases when
tobacco is used in combination with alcohol or areca nut.
About 96% of all oral cancers are carcinomas and the
remaining 4% are sarcomas. Majority of oral carcinomas
are squamous cell carcinomas. It is estimated that 9 out of
every 10 oral malignancies is squamous cell carcinoma. It
is a disease of increasing age with 95% of the patients
older than 40 years of age.
In India, it is estimated that there are 22.5 million cancer patients at any given point of time with about 0.7 million new cases diagnosed every year and nearly half of
them die every year. Two-thirds of the new cancers is
diagnosed at a very advanced and incurable stage. More
than 60% of these affected patients are in the age group of
35 and 65 years. Fifty percent of all male and 25% in
female are tobacco related cancers.
Genetic Susceptibility
Family history of oral cancer is considered a risk factor.
Head and neck cancer patients show an increased susceptibility to chromosome damage by mutagens. Some studies suggest that lip cancers have shown some amount of
genetic predisposition. Mork et al (1999) reported a significantly increased odds ratio for developing head and neck
squamous cell carcinoma in female patients, aged less than
45 years, who had first-degree relatives with cancer. It is
suggested that familial oral cancer may be attributed to both
shared environmental factors within families and a common
oral cancer susceptibility gene with low penetrance.
Immune Status
It has been noticed that immunosuppressed individuals
tend to show an increased incidence of oral malignancies.
It is also a well-known fact that the host response diminishes with advancing age. HIV-positive immunocompromised individuals, may usually exhibit Kaposis sarcoma
and non-Hodgkins lymphomas.
Environmental Factors
Exposure to actinic radiation There is enough evidence
in literature to show that skin and lip cancers are more
frequently seen in individuals whose occupation necessitates long working hours in the sun such as fishermen and
farmers.
It has been reported that fair skinned individuals, people
residing in high latitudes with clear atmosphere (UV light
can penetrate easily) such as Finland and Sweden, residents closer to the equator (long periods of sunshine) such
as Greece are more susceptible to develop lip cancers.
The wavelengths of the light thought to be responsible for
the actinic damage are in the range of 2,9003,200 A.
Sunscreen lotions are effective in protecting the lip from
the damaging effects of UV light. Melanin pigment acts as
a protective agent against actinic radiation.
Atmospheric pollution Air pollution arising from industrial wastes and automobile exhausts are particularly harmful. Other sources include gases emitted from burning
firewood/coal for domestic purposes. Sulfur dioxide, carbon
monoxide, nitrogen gases have been implicated in causing
pharyngeal, laryngeal and lower respiratory tract cancers.
TOBACCO
All the forms of tobacco (smoke and the smokeless/
chewable/inhaled) such as cigarettes, pipes, cigars, beedis,
paan and snuff have been implicated in the development
of oral cancers. It is believed that tobacco use is responsible for 90% of the oral cancers in males.
Figure 1
Figure 3
Figure 4
Figure 2
Tobacco is also used in the form of a powder for inhalation (Figure 4, snuff). Tobacco (Figure 5) contains nicotine
(nitrosamine), nitrosodiethanolamine, nitrosoproline, polonium and polycyclic aromatic hydrocarbon (tars). Areca nut
(Figure 6 contains cholinergic muscarinic alkaloids such
as arecholine and guavacoline) chewing is also widely
practiced in India.
382
Figure 5
Figure 7
Figure 6
Figure 8
ALCOHOL
Alcohol consumption is the most important risk factor for
development of oral cancer in non-smokers. It is also considered as the second independent major risk factor for the
development of oral cancer. It is estimated that an average
consumption of over 30 ml of alcohol per day increases the
risk of oral cancer linearly with the quantity of alcohol consumed. Though any form of alcohol when consumed in large
quantities is dangerous, it is believed that dark colored drinks
are more hazardous (as they may contain higher beverage
congeners such as nitrosamines, hydrocarbons and other
impurities which are known carcinogens). Carcinogens present in the tar are insoluble in saliva but are highly soluble
in alcohol and easily absorbed in the oropharynx.
The International Agency for Research on Cancer published a monograph in 1982, which details the various methods in which alcohol predisposes to head and neck cancers.
SYSTEMIC HEALTH
Candidiasis Autoimmune polyendocrinopathy candidiasis
ectodermal dystrophy an autosomal recessive disease
associated with a limited T lymphocyte defect, presumably
supports the growth of Candida albicans and predisposes
to chronic mucositis and oral cancer.
Diabetes In diabetic patients, alterations occur in the oxidative equilibrium of free radicals. Elevated blood glucose
levels can lead to excessive formation of free radicals.
Moreover, due to protein breakdown, the activity of antioxidant scavengers and enzymes is reduced. Both the
increase in free radicals and oxidative stress promote carcinogenesis.
It has been suggested that poor diabetic control is associated with an increased cancer risk due to enhanced
384
Ionizing Radiation
Exposure to large amounts of ionizing radiation such as
in a nuclear mishap is a known risk for causing cancers.
Radiation exposure, known to cause DNA damage, may
be a potential source of field cancerization of the upper
aerodigestive tract.
Hashibe et al (2005) evaluated the possible risk of
developing second primary cancers following radiotherapy
for head and neck cancers in 30,221 oral squamous cell
carcinoma patients. Patients treated with radiation only or
radiation with surgery had elevated risks of developing a
second primary tumor, whereas patients treated with surgery
only did not appear to be at increased risk. They also suggested that the expected latent period between radiation
exposure and tumor occurrence, radiation became a risk
factor after 10 years of follow-up for solid cancers of the oral
cavity, pharynx, esophagus, and lung, and after 15 years
of follow-up for second primary leukemia.
Role of Viruses
The role of viruses remains unclear. Varying viral genomes
have been frequently found within cancer cells. Viruses
have been known to modify the DNA and the chromosomal structures and induce proliferative changes in the
cells they infect.
Evidence of a viral carcinogenesis is perhaps strongest
for infection with human papilloma viruses (HPV). DSouza
et al (2007) in a multicenter case-control study reported that
infection with HPV-16 increased the risk of cancer of the
oral cavity and particularly oropharynx.
The role of infection with EpsteinBarr virus (EBV) and
herpes simplex viruses (HSV) remains uncertain. The role
of HSV, HSV-1 and HSV-2, as co-factors in association
with tobacco, alcohol, or HPV-16 infection has also been
proposed in causing oral cancers.
Human immunodeciency virus (HIV), due its effect on
immunosurveillance, acts as a cofactor along with other
viruses such as EBV and cytomegalovirus in predisposing
the affected individual to oral cancer.
Role of Nutrition
Various studies have shown that a diet with low vitamin A,
vitamin C, vitamin E, iron, selenium, folate and other trace
element content is associated with an increased risk of oral,
laryngeal, lung, gastric, ovarian, breast and cervical cancers. Certain dietary deficiencies may cause epithelial atrophy which renders the epithelium vulnerable to the action
of carcinogens. The relationship between sideropenic dysphagia and oral cancer is well recognized (sideropenic
dysphagia may be associated with epithelial atrophy in the
upper alimentary tract).
Garewal (1994) summarized the ndings of 54 studies
that evaluated fruit and vegetable intake in the development of cancers in the upper aerodigestive tract; he found
that 52 of the studies demonstrated a protective effect of
the antioxidant content of fruits and vegetables.
It has been shown that patients who ingest high levels
of vitamin C and ber have half the risk of developing oral
cancer as those with minimal level of consumption. Block
(1991) and Mirvish (1986) showed that a low intake of
vitamin C is associated with an increased risk of cancers of
the stomach, esophagus, oral cavity, larynx, and cervix.
Gridley et al (1992) in a study involving 2,000 individuals proposed that the use of vitamin E supplements correlated with a diminished risk for oral and pharyngeal cancers.
Cellular Kinetics
Generation time is the time required for a quiescent cell to
enter the cell cycle and give rise to two daughter cells.
Malignant cells usually have a shorter generation time than
non-malignant cells and a smaller percentage of cells in
G0 (resting phase), so a larger proliferation fraction exists.
Initial exponential tumor growth is followed by a plateau
phase when cell death equals the rate of formation of
daughter cells. Compared to large tumors, small tumors
have a greater percentage of actively dividing cells and
thus show greater rates of proliferation.
Molecular Abnormalities
Genetic mutations are largely responsible for the generation
of malignant cells. These mutations alter the quantity or
function of protein products that regulate cell growth and
division and DNA repair. Two major categories of mutated
genes are oncogenes and tumor suppressor genes.
Oncogenes are abnormal forms of normal genes (protooncogenes) that regulate cell growth. Mutation of these
genes may result in direct and continuous stimulation of the
molecular biologic pathways (e.g. intracellular signal transduction pathways, transcription factors, secreted growth
factors) that control cellular growth and division.
There are more than 100 known oncogenes that may contribute to human neoplastic transformation, for example,
the ras gene encodes the Ras protein, which regulates cell
division. Mutations may result in the inappropriate activation of the Ras protein, leading to uncontrolled cell growth
and division. In fact, the Ras protein is abnormal in about
25% of human cancers. Other oncogenes have been implicated in specic cancers. These include various protein
kinases (bladder cancer, breast cancer), bcr-abl (chronic
myelocytic leukemia, B-cell acute lymphocytic leukemia),
C-myc (small cell lung cancer), N-myc (small cell lung
cancer, neuroblastoma), and C-erb B-2 (breast cancer).
Specic oncogenes may have important implications
for diagnosis, therapy, and prognosis (see individual discussions under the specic cancer type). Oncogenes typically result from acquired somatic cell mutations secondary
to point mutations (e.g. from chemical carcinogens), gene
amplication (e.g. an increase in the number of copies of
a normal gene), or from insertion of viral genetic elements
into host DNA. Occasionally, mutation of germ cell lines
results in vertical transmission and a higher incidence of
cancer development in an offspring.
Tumor suppressor genes are inherent genes that play a
role in cell division and DNA repair and are critical for
detecting inappropriate growth signals in cells. If these
genes, as a result of inherited or acquired mutations, become
unable to function, genetic mutations in other genes can
proceed unchecked, leading to neoplastic transformation.
As with most genes, two alleles are present that encode
for each tumor suppressor gene. A defective copy of one
gene may be inherited, leaving a person with only one
functional allele for the individual tumor suppressor gene.
If an acquired mutation occurs in the other allele, the normal protective mechanisms of the tumor suppressor gene
are lost, and dysfunction of other protein products or DNA
damage may escape unregulated, leading to cancer.
386
Figure 9
Table 1
Early signs
Late signs
Indurated area
Paresthesia, dysesthesia of
the tongue or lips
Airway obstruction
Chronic earache (chronic
serous otitis media)/otalgia
Trismus and dysphagia
Cervical lymphadenopathy
Persistent pain or referred pain
Altered vision
Epiphora
Figure 10
A
387
Some cases appear to be growing outward from the surface rather than invading the tissues is called exophytic or
verrucous growth. The most common site of metastasis is
the submandibular lymph nodes as they are the primary
echelon nodes for these regions.
Carcinoma of floor of mouth
It is seen more commonly in men; reasons being cited that
they tend to smoke and abuse tobacco a lot more than
women and the chemicals that mix with the saliva tend to
pool in the floor of the mouth for a longer time. It is seen
most frequently in the anterior portion of floor. The typical
carcinoma of the floor of mouth is an indurated ulcer of
varying size, on one side of the midline. It may take the
form of wart-like growth (Figure 11), which tend to spread
superficially rather than in depth. It may spread in four
primary directions, i.e. extend to the other side crossing
the midline, may extend upward involving the base of the
tongue or may come anteriorly invading the lingual mucosa
and ultimately invade the bone causing loosening of the
anterior teeth (Figure 12). Loosening of teeth is seen only
in advanced cases. However, patients seek an early consultation because of the restriction in the movements of the
tongue often causing peculiar thickening or slurring or the
speech. There may be excessive salivation. In some cases,
there may be referred pain in the ears.
Carcinoma of oor of mouth may invade the deeper tissues and may even extend into the submaxillary and sublingual glands. Metastasis from the oor of the mouth are
found most commonly in the submandibular group of
lymph nodes and also sometimes in the facial nodes, since
the primary lesion frequently occurs near the midline where
Figure 12
Figure 11
Figure 13
388
Figure 14
Figure 16
Figure 17
Figure 15
Figure 18
Figure 19
Table 2
Stage
TNM STAGING
The tumor-node-metastasis (TNM) staging system was first
reported by Pierre Denoix in the 1940s. The International
Union Against Cancer (UICC) eventually adapted the system and compiled the first edition of the TNM staging system in 1968 for 23 body sites. It is important to realize that
the TNM staging system is simply an anatomic staging
system that describes the anatomic extent of the primary
tumor as well as the involvement of regional lymph nodes
and distant metastasis. Tumor size and the extent of spread
are considered to be the best indicators of the patients prognosis. Table 2 summarizes the most widely accepted staging
protocol, the TNM classification of oral cancer. This system
has three basic clinical features:
1.
2.
3.
N stage
M stage
Stage 0
Tis
N0
M0
Stage I
T1
N0
M0
Stage II
T2
N0
M0
Stage III
T3
T1
T3
N0
N1
N1
M0
M0
M0
Stage IV A
T4a
T4a
T1
T2
T3
T4a
N0
N1
N2
N2
N2
N2
M0
M0
M0
M0
M0
M0
Stage IV B
Any T
T4b
N3
Any N
M0
M0
Stage IV C
Any T
Any N
M1
NODAL METASTASIS
Involvement of regional lymphatic by primary squamous
cell carcinomas of the upper aerodigestive tract is dependent on various factors related to the primary tumor. These
include the site, size, T-stage and location of the primary
tumor. In addition to this, histomorphologic features of the
primary tumor also influence the risk of nodal metastases.
The risk of nodal metastasis increases in relation to location
of the primary tumor, as one progresses from the anterior
to posterior aspect of the upper aerodigestive tract meaning: the risk of the lips oral cavity oropharynx hypopharynx. For tumors of the larynx and pharynx, the risk
of nodal metastasis increases as it progresses from the
center to the periphery, meaning the risk of regional lymph
node metastasis from carcinoma of the true vocal cord is
exceedingly small. The risk, however, increases as one
progress for the vocal cords to the false vocal cords, aryepiglottic fold, pyriform sinus, and pharyngeal wall. Nearly
two-thirds of patients with primary carcinomas of the hypopharynx present with clinically palpable regional lymph
node metastasis.
Certain primary sites have a signicantly increased risk
of nodal metastases compared to the other sites in the
same region, for example, oor of mouth versus hard palate in the oral cavity. In general, T-stage usually reects
tumor burden or invasiveness and therefore the risk of
nodal metastases increase with increasing T-stage of the
primary tumor at any site. Similarly, tumor size (large versus small) increases the risk of dissemination to regional
lymph nodes.
Certain histomorphologic features of the primary tumor
also increase the risk of nodal metastasis. Endophytic tumors
392
examination of the smears. An enlarged metastatic cervical lymph node may be the only physical finding present
in some patients whose primary tumors are either microscopic or occult at the time of presentation. A systematic
search for a primary tumor should be undertaken in these
patients prior to embarking upon therapy for the metastatic nodes. If a thorough head and neck examination,
including fiberoptic nasolaryngoscopy, fails to show a primary tumor, then the diagnosis of metastatic carcinoma to
a cervical lymph node from an unknown primary (occult
primary) is established.
ViziLite Plus is another popular screening tool for detection of oral cancers. As ViziLite Plus is passed over oral
tissue that has been treated with the rinse solution, normal
healthy tissue will absorb the light and appear dark,
abnormal tissues will appear white.
Venkatakrishna et al (2003) developed a HPLC-LIF
technique to detect and record simultaneously spectra and
chromatograph of physiological samples. This system
enables the detection of multiple markers in a single physiological sample in a short time. Samples of saliva and
serum from normal and oral cancer subjects have been
studied with the set-up. Their study showed that body uids
like saliva and serum of normal, premalignant and malignant subjects had substantially different protein proles.
They suggest that by simultaneous recording of the
chromatographic peaks and corresponding uorescence
spectra, it is possible to carry out unambiguous discrimination between normal, premalignant and malignant
cases even when markers are present in femto/subfemto
mole quantities, which should assist in early diagnosis of
neoplasia.
Imaging Modalities
Plain radiographic examination may include the use of
OPG, occlusal and intraoral periapical radiographs. Paranasal sinus view is useful for assessing extent of bone
involvement in the maxillary sinus region.
Plain radiographs
Intraoral periapical radiographs are useful to assess finer
details such as subtle bone invasion, bony trabecular
architecture (Figure 20) and destruction of the lamina
dura. Occlusal radiographs (Figure 21) may help in evaluating destruction of bone in the anterior portion of the
jaws which cannot be assessed adequately in an OPG. The
mandible is more commonly affected than the maxilla.
The typical radiographic features of carcinoma invading
bone include: ill-defined destruction (moth-eaten appearance) of bone with ragged borders (Figure 22), cortical
plates may be thinned out leading to a pathological fracture (Figure 23), extensive lesions may cause destruction
of floor of the nasal fossa, maxillary sinus (Figure 24), and
the cortical lining of the mandibular canal. Lamina dura
around teeth are lost. However, roots of teeth show no
signs of resorption as seen in benign tumors. This destruction of bone around teeth gives rise to floating in space
appearance (Figure 25).
CT, MRI and PET
Cross-sectional imaging modalities such as CT and MRI
help in assessing the size and extent of a tumor in three
dimensions. MRI imaging is considered more sensitive in
evaluating the possibility of intracranial invasion. On the
395
Figure 20
Figure 22
Figure 23
Figure 21
Figure 24
Figure 25
In 1964, Niebel and Chomet first reported the use of toluidine blue (tolonium chloride) as a vital tissue stain to aid
in the early detection of oral precancerous and malignant
lesions. Though it is not cancer specific, it has been
reported to stain mitochondrial DNA, altered DNA in premalignant and malignant epithelial lesions and cells with
relatively increased amounts of DNA. Warnakulasuriya
and Johnson (1996) reported that toulindine blue-positive
lesions with minimal or no identifiable dysplasia on initial
biopsy were almost four times more likely to transform to
carcinoma than lesions found to be toluidine blue-negative.
Toluidine blue is also an useful adjunct to clinical examination and biopsy. Toluidine is a basic metachromatic dye
that stains for acidic tissue components and thus binds
more readily to DNA. In addition, it can help to determine
the most appropriate biopsy sites and to surgically delineate
margins. Meta-analysis of toluidine blue staining in oral
cancer screening found that its sensitivity ranged from
93.5 to 97.8%, and specicity from 73.3 to 92.9%.
TBlue630 is a patented, pharmaceutical-grade toluidine
blue-based metachromatic dye. It provides the deep blue
staining that allows identied lesions to be seen clearly
under normal light.
Exfoliative cytology
It is a known fact that dysplastic and cancerous cells tend
to have fewer and weaker connections to each other and to
their neighboring normal cells in the surrounding tissue.
Dysplastic and cancerous cells therefore, tend to slough off
or exfoliate preferentially and can easily be collected from
the surface of the lesion. A sample of these cells applied
to a microscope slide will often contain abnormalities if
obtained from a dysplastic or cancerous lesion.
Sample can be obtained using rm strokes with a tongue
blade, cement spatula or a newer technique using a brush
(referred to as brush biopsy).
The cytology is reported as:
Brush biopsy
This technique was introduced in 1999 as a substitute to
the conventional exfoliative cytology procedure. A commercially available kit is used (oral CDX).
This biopsy method utilizes a brush to obtain a complete
transepithelial biopsy specimen with cellular representation
from each of the three layers of the lesion: the basal, intermediate, and supercial layers. Unlike previous cytologic instruments, which collect only exfoliated supercial cells,
when used properly and rubbed against an area of suspect
tissue aggressively (to the point of minor bleeding) the
biopsy brush penetrates to the basement membrane, removing tissue from all three epithelial layers of the oral
mucosa. The oral brush biopsy does not require topical or
local anesthetic and causes minimal bleeding and pain. The
brush biopsy instrument has two cutting surfaces, the at
end of the brush and the circular border of the brush. Either
surface may be used to obtain the specimen. In a recent
study, paired, same site samples of tongue tissue were
obtained from patients, rst by brush biopsy and then by
surgical punch biopsy. The study demonstrated that the
brush biopsy technique, unlike cytology, sampled the full
thickness of oral epithelium.
Results of brush cytology specimen are classied into
one of the four categories:
1.
2.
3.
4.
Histopathologic features
All suspicious lesions should be subjected to an excisional
or incisional biopsy and the specimen should be subjected
to histopathological evaluation.
In general, all carcinomas exhibit the following features:
epithelial dysplasia, keratinization (varies with degree of
differentiation), local invasion by break in the basement
membrane and invasion and proliferation into the underlying connective tissue (clinically manifested as xation
and induration of the lesion), metastasis by blood or lymphatic channels histopathologically seen as invasion of
tumor cells into the capillaries and lymphatic ducts permeation and perineural invasionsome of the tumor cells
may have atypical invasion pattern along the nerve
sheath.
The histopathological features are graded into three
types depending upon the degree of differentiation of the
neoplastic proliferating cells. They are:
1.
398
Figure 26
Normal epithelium
Dysplastic epithelium
Islands of tumor
epithelium
Keratin pearl
2.
3.
shape. Keratin pearls may not be present. Numerous epithelial islands of prickle cells with peripheral basal cells
may be seen.
Poorly differentiated squamous cell carcinoma
This is the tumor with proliferation of anaplastic cells,
highly invasive with poor prognosis. The tumor cells bear
little resemblance to their cells of origin and often will
present diagnostic difficulties because of the primitive and
uncharacteristic histologic appearance. These cells show
lack of cohesiveness and are extremely vagarious. The
mitotic figures are extremely high.
Management
There are three recognized treatment modalities for managing head and neck cancers: surgery, radiotherapy and chemotherapy. As a thumb rule, Stage I and Stage II cancers
can be managed either by surgery or radiotherapy. However,
Stage III and Stage IV cancers are managed using a combination of radiation therapy and surgery.
Based on the nature of the tumor, the treatment modality is chosen. Factors that inuence the choice of treatment include: site and location of the primary (tongue,
oor of mouth/anteriorly or posteriorly), size of the tumor
(based on T stage), proximity to the bone, status of nodal
involvement, histological typing of the tumor (cell type
and degree of differentiation), ability to achieve adequate
surgical margins and preserve functions, physical and
mental status of patient and history of any treatment.
Surgical management Surgical treatment aims at complete removal of the primary as well as the metastatic nodes.
It is never used as a palliative mode of treatment. Criteria
for choosing surgical management are: tumors which are
non-sensitive to radiation, tumors involving bone, recurrent tumors in sites that have been previously irradiated,
to reduce bulk of tumor prior to radiation therapy (debulking), where side effects of surgery are expected to be less
significant than those associated with radiation and when
nodes are to be removed.
Surgical management aims at clearance of both the
primary lesion and the involved regional lymphatics.
The extent of resection of the primary lesion depends
upon the size and the adjacent structures that may have
been inltrated.
Neck dissection (cervical lymphadenectomy) In this procedure, all lymph nodes are removed beginning from the
lower border of the mandible superiorly to the clavicular
region inferiorly, and from the trapezius muscle posteriorly to the midline anteriorly. The sternocleidomastoid
muscle, omohyoid muscle, jugular vein and submandibular
salivary gland are resected. Based on the clinical involvement of lymph nodes, neck dissections can be categorized
as prophylactic (clinically non-palpable nodes, may or
2 Gy per fraction. The total dose for preoperative radiotherapy or radiotherapy for malignant lymphomas is usually lower.
Fractionated radiation Fractionated radiation is used
because there is a difference in the responses of tumor tissue and normal tissue. In general, normal tissue repairs
sublethal DNA damage, especially in the low-dose range,
better than tumor tissue. The sparing effect of fractionated
radiation is the largest for late-responding tissues, whereas
early-responding tissues respond more like tumor tissue.
Next to DNA-repair advantages, fractionated irradiation
allows for the re-population of tissue between fractions
(especially during the weekend, when the tumor and normal
tissues are not radiated), thereby reducing early effects.
This, however, also applies for rapidly proliferating malignant tissue. Another advantage is that fractionated irradiation allows for re-oxygenation of radio-resistant hypoxic
tumors between fractions, leading to a higher percentage
of radiosensitive oxygenated cells.
Ideal candidates for radiotherapy Small lesions less than
3 cm and superficial without necrotic areas can be readily
managed with radiation therapy. The choice of treatment
also depends on functional and cosmetic concern. If
regional excision can be accomplished without much morbidity, surgery should be the treatment of choice. On the
contrary, if the lesion involves large areas like tongue,
floor of the mouth or buccal mucosa, and if excised it
would result in morbidity, thus local irradiation would be
the treatment of choice. But if the lesion is invasive involving the bone, radiation sterilization of the tumor is not
possible without extensive normal tissue damage, therefore surgery or a combination of irradiation and surgery
may be required. Lesions that are extremely radiosensitive
such as lymphomas, radiation therapy should be the treatment of choice. If the location and clinical behavior of a
lesion indicates significant lymphatic permeation, then
radiotherapy alone or in combination with surgery is the
treatment of choice as a wider volume.
Radiation technique Radiation therapy may be administered to a localized lesion by using brachytherapy (applicators or implants) or to a region of the head and neck by
using teletherapy (external beam radiation).
External beam radiation therapy The source of radiation
can either be low energy (orthovoltage: 50300 kVp) or
high energy (cobalt-60 or linear accelerators of 4 million
electron volts). Low energy beams are used for small sized
intraoral tumors, lip, and skin cancers. The high energy
radiation provides variable penetration due to its ability to
vary the energy of the photons. It spares bone and skin.
For teletherapy, three principal eld arrangements are
planned: wedged-pair elds, parallel-opposed elds and
three-eld technique. The wedged-pair eld allows a
therapeutic dose to unilateral disease while sparing a high
400
erythematous candidiasis. In such patients, topical application of anticandidal agents may not be effective as the
salivation is compromised.
Osteoradionecrosis Radiation therapy causes endarteritis
obliterans, which in turn results in hypovascularity, hypocellularity and finally hypoxic tissue. Owing to its inherent poor vascularity, the mandible is more commonly
affected than the maxilla.
Trauma (external, iatrogenic), periodontal diseases, illtting dentures, peridontally weak or pulpally affected
teeth in the line of radiation are predisposing factors for
osteoradionecrosis.
Patients complain of pain, foul taste, paresthesia/
anesthesia and intraoral or extraoral discharging sinuses.
Lymphadenopathy may be seen. As the condition worsens,
pathological fracture occurs. Radiographically, it is difcult
to differentiate between the ill-dened patchy destruction
of bone that is seen in cancer of the bone, osteoradionecrosis and suppurative osteomyelitis.
As a preventive measure, if extractions are planned, it
is desirable to allow as much healing time as possible.
Seven to 14 days and up to 21 days have been suggested
as healing times prior to radiotherapy.
Patients should be instructed to avoid mucosal irritants,
discontinue the use of dental appliances if they contact the
area of the lesion, maintain nutritional status, stop smoking
and alcohol consumption.
Osteoradionecrosis is best managed with topical antibiotic
(tetracycline) or antiseptic (chlorhexidine) rinses. Hyperbaric oxygen (HBO) therapy increases the oxygenation of
tissue, increases angiogenesis, and promotes osteoblast and
broblast function.
HBO therapy (Figure 27A, B) is usually carried out for a
period of 2030 dives at 100% oxygen and 22.5 atmospheres of pressure.
Sequestrated bone may be managed with limited resection or may require mandibulectomy. If surgery is required,
postsurgical HBO therapy of 10 dives is recommended.
Chemotherapy It is provided as an induction therapy
before local therapies. It is also an adjunctive modality for
other cancers. The objective of induction chemotherapy is
to promote initial tumor reduction and to provide early
treatment of micrometastases. The goal of chemotherapy
is to eradicate the rapidly growing cells of the tumor or
modify their growth. Chemotherapeutic agents affect the
rapidly dividing cells of the target tumor and the lining
epithelium, the oral ecology and the vascular, inflammatory and healing responses of the oral cavity. These alterations may result in mucositis and ulceration of the oral
mucosa. Chemotherapeutic agents also target the hemopoietic cells of the bone marrow, resulting in anemia,
thrombocytopenia and leukopenia. Unfortunately, chemotherapy is often toxic to other cells that rapidly divide
normally. These include bone marrow, hair, and the
401
Figure 27
A
402
SECTION
VI
Teeth and
Periodontium
405
440
458
403
Chapter-15.indd 403
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Prelims-Ongole.indd ii
10/15/2012 2:06:46 PM
CHAPTER
15
Ludwigs Angina
Osteomyelitis
ProteolysisChelation Theory
Miller in his observations reported that many of the microorganisms in the oral cavity were capable of producing
acids and these acids were usually present in deeper carious
lesions. In many of the lesions studied, lactic acid was
identified in carbohydrate saliva combined mixtures. A
specific microorganism, Leptothrix buccalis, was isolated
from the dentinal tubules, suggesting that the microorganisms and the acids may have a synergistic action to dissolve the organic portion of the tooth.
Proteolytic Theory
The initial work on the proteolytic theory was done by
Heider and Bodecker in 1878 and Abbott in 1879. Studies
showed that the organic portion of the tooth plays an
important role in the development of dental caries.
406
Autoimmune Theory
Figure 1
Host factor
Enzymatic Theory
Diet
Dental
caries
Time
Microorganisms
Acidenzyme Theory
Diagram depicting the contributing factors for
dental caries
Host factor
a. Tooth factor
i. Morphology and position in the arch
ii. Chemical nature
b. Saliva
i. Composition, pH and antibacterial activity
ii. Quantity and viscosity of flow
2.
3.
4.
Microflora
Substrate or diet (physical nature and chemical nature)
Time.
Host Factor
Tooth Factor
Morphology and position in arch
Compared to the smooth surfaces of teeth, deep pits and
fissures are more prone to carious attack because of
food lodgment and bacterial stagnation. Owing to their,
complex occlusal morphology consisting of numerous
pits and fissures, the permanent mandibular first molars
followed by the maxillary first molars and mandibular
and maxillary second molars are more prone to carious
attack.
Apart from the morphology of the tooth, the position of
the tooth in the arch has a heavy bearing on the incidence
of carious lesions.
Irregularities in the arch form, crowding and overlapping of the teeth also favor the development of caries as these
regions provide an excellent environment for plaque accumulation.
Partially impacted third molars are more prone to caries
and so are the buccally or lingually placed teeth.
Chemical nature
The chemical components of enamel such as dicalcium phosphate dihydrate and fluorapatite make the enamel resistant
to carious attack to a certain extent.
407
Saliva
Composition
Hay et al (1982) and Lagerlof (1983) in their reports reiterated the fact that human salivary secretions are supersaturated on calcium and phosphate.
The concentrations of inorganic calcium and phosphorus show considerable variation within resting and stimulated saliva. Caries prone individuals have low calcium
and phosphorus levels. Salivary proteins such as statherin,
acidic proline-rich proteins (PRPs), cystatins, and histatins
help in the maintenance of the homeostasis of the supersaturated state of saliva. According to Hay and Moreno
(1989), statherin is present in stimulated saliva in concentrations sufcient to inhibit the precipitation of calcium
and phosphate salts. Studies by Gibbons and Hay (1988)
have shown that statherin may contribute to the early
colonization of the tooth surfaces by certain bacteria, such
as Actinomyces viscosus.
The acidic PRPs account for 2530% of all proteins in
saliva, and they have high afnity for hydroxyapatite in vitro
(Hay and Moreno, 1989). The acidic PRPs bind free calcium,
adsorb to hydroxyapatite surfaces, inhibit enamel crystal
growth, and regulate hydroxyapatite crystal structure (Hay
and Moreno, 1989).
The amount and quality of acidic PRPs and agglutinins
are found to be different in caries-free and caries-active
individuals as shown by the studies of Rosan et al (1982)
and Stenudd (1999).
The role of cystatins in the caries process is still unclear.
However, they may play a minor role in the regulation of
calcium homeostasis in saliva. Phosphorylated and nonphosphorylated cystatins bind to hydroxyapatite.
Salivary flow rate, pH and buffer capacity
Saliva has the most important function of caries prevention
by way of its flushing and neutralizing effects, commonly
referred to as salivary clearance or oral clearance capacity. As a thumb rule, the higher the flow rate, the faster the
clearance and the higher the buffer capacity. Reduced salivary flow rate and the concomitant reduction of oral
defense systems may cause severe caries and mucosal
inflammation. Though, patients with impaired saliva flow
rate often show high caries incidence (Papas et al, 1993;
408
Spak et al, 1994) or caries susceptibility, it is still a mystery as to how much saliva is adequate enough.
The pH of saliva at which it ceases to be saturated with
calcium and phosphorus is referred to as the critical pH.
Normally, the critical pH is 5.5. Below this value, the inorganic content tends to demineralize. The normal pH of
resting saliva is 67.
Buffering capacity The buffer capacity of both unstimulated and stimulated saliva involves three major buffer systems: the bicarbonate (HCO3), the phosphate, and the
protein buffer systems. These systems have different pH
ranges of maximal buffer capacity. The bicarbonate and
phosphate systems have pH values of 6.16.3 and 6.87.0,
respectively.
Since most of the salivary buffering capacity operative
during food intake and mastication is due to the bicarbonate
system, sufcient saliva ow provides the oral cavity with
the neutralizing components.
The phosphate and protein buffer systems make a minor
contribution to the total salivary buffer capacity, relative
to the bicarbonate system. The phosphate system is, in
principle, analogs to the bicarbonate system but without
the important phase-buffering capacity, and it is relatively
independent of the salivary secretion rate.
Lagerlof and Oliveby in 1994 showed that a low ow
rate combined with a low or moderate buffer effect indicated poor salivary resistance against microbial attack.
It is a well-established fact that the buffer capacity of
the saliva and the caries experience are inversely related.
The buffer effect of saliva is inuenced by the hormonal
and metabolic changes, as well as by altered general health.
It is generally accepted that the buffer effect is greater in
men than in women (Heintze et al, 1983). In women, the
buffer effect decreases gradually, independent of ow rate,
toward late pregnancy and promptly recovers after delivery. Introduction of either hormone replacement therapy
in menopausal women (Laine and Leimola-Virtanen, 1996)
or low-dose oral contraceptives (Laine et al, 1991) can
slightly increase the buffer capacity.
Carbonic anhydrases (CAs) participate in the maintenance of pH homeostasis in various tissues and biological
uids of the human body by catalyzing the reversible
hydration of carbon dioxide. Recent research suggests that
salivary CA VI plays a role in protecting the teeth from
caries (Kivela et al, 1999a, b). CA VI has been reported to
bind to the enamel pellicle and retain its enzymatic activity
on the tooth surface.
It is also believed that the urea and saline in saliva
become hydrolyzed to produce ammonia and the later can
cause rise in the salivary pH. This rise in pH can counter
the attacks on the tooth surface during the progression of
caries.
Antibacterial activity The primary oral innate defense
factors are peroxidase systems, lysozyme, lactoferrin, and
Microflora
The main etiological agent in occlusal and pit and fissure
caries is the S. mutans. Deep dentinal caries is commonly
associated with lactobacilli, certain gram-positive anaerobes and filaments such as Eubacterium and Actinomyces.
Root caries or cemental caries is predominantly associated with Actinomyces viscosus. However other species of
Actinomyces such as A. naeslundii and A. nocardia have
also been isolated.
2.
Role of heredity
Literature review reveals various studies to assess
the genetic modifications in dental enamel, genetic modification of immune response, genetic regulation of
salivary function and inherited alterations in sugar metabolism.
Bachrach and Young (1927) compared the caries incidence of monozygotic twins with same-sex dizygotic (93
pairs) and different-sex dizygotic (78 pairs) twins. Their
results showed that the monozygotic twins had a more
similar caries incidence than dizygotic twins and that different-sex dizygotic twins had the greatest variance. The
authors concluded that heredity plays a subsidiary part in
the incidence of caries. It is believed that heredity affects
the dental decay only in as much as it controls the shape
of a tooth and its pits and ssures and its position in the
dental arch.
Senpuku et al (1998) and Acton et al (1999) have correlated specic HLA-DR types with binding S. mutans
antigens and S. mutans colonization.
Acton concluded that genes within MHC modulate the
level of oral cariogenic organisms.
Mariani et al (1994) in their study of celiac disease,
enamel defects and HLA typing observed that HLA-DR3
was associated with increased enamel defects and
HLA-DR5, 7 were associated with a reduced frequency of
enamel defects. Studies have shown that the genes in the
HLA complex are associated with altered enamel development and increased susceptibility to dental caries.
Role of immunity
Salivary IgA and immunoglobulins secreted in the gingival crevicular fluid such as IgG, IgM and IgA along with
neutrophil leukocytes and macrophages play an important
role in the prevention of dental caries. It is believed that
the immune response exerted by the gingival crevicular
immune system is more potent compared to the salivary
immune mechanism.
Salivary IgA prevents S. mutans from adhering to the
tooth surface. The IgG antibodies acting as opsonins, facilitate phagocytosis and the death of S. mutans by the action
of macrophages and neutrophil leukocytes.
410
Figure 2
Figure 4
Figure 3
Figure 5
ii.
Figure 6
A
Extensive destruction of teeth in child suffering from rampant caries. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
412
Figure 7
2.
3.
4.
5.
Clinically presents as widespread destruction of deciduous teeth, most commonly the four maxillary incisors,
followed by the first molars and then the cuspids if the
habit is prolonged.
The lower teeth are not usually affected as they remain
under the cover of the tongue, so the absence of caries in
the mandibular incisors distinguishes this disease from
ordinary rampant caries.
Both the nursing bottle and rampant cause early pulp
involvement.
Figure 8
According to Rapidity
i.
ii.
413
Table 1
Extent of
carious lesion
Signs and
symptoms
Clinical findings
Clinical
diagnosis
Radiographic diagnosis
Final diagnosis
Caries involving
enamel
Asymptomatic
patient rarely
notices blackish
discoloration of
the tooth
Chalky white
discoloration of the tooth
or
blackish discoloration
of the tooth usually
seen in the pits and
fissures and proximal
aspects of teeth
Enamel
caries
May not be
evident on a
radiograph until
at least 40%
demineralization
occurs
Ill-defined radiolucency
involving the enamel cap
Enamel
caries
Caries involving
dentin
Sensitivity on
consuming hot,
cold or sweet food
Sensitivity subsides
on removal of
stimulus
Food lodgment
patient may notice
blackish
discoloration of
the tooth
Blackish discoloration
of the tooth
Catch experienced
on using an
explorer
Frank cavitation
Dentinal
caries
or
reversible
pulpitis
Ill-defined
radiolucency
involving the
dentin
(Figure 9)
Dentinal
caries
Caries involving
pulp
Frank cavitation
involved tooth
non-tender on
percussion
Grossly decayed
tooth/root stump
(Figure 10)
Chronic
irreversible
pulpitis
Radiolucency
involving the pulp
No periapical
changes (Figure 11)
Widening of the
periodontal ligament
space at the periapex
(Figure 12)
Discontinuity of the lamina
d ura at the periapex or
ill-defined periapical
radiolucency (Figure 13)
Well-defined radiopacity at
the periapex (Figure 14)
Chronic
irreversible
pulpitis
Well-defined radiolucency
measuring 1.5 cm in
diameter at the periapex
of the tooth (Figure 15)
Well-defined radiolucency
measuring 1.5 cm in
diameter at the periapex
of the tooth bounded by
a sclerotic border
Chronic apical
periodontitis
Chronic periapical
abscess
Focal sclerosing
osteomyelitis
or condensing
osteitis seen
in young
individuals with
good immune
response or
when the
virulence of the
microorganisms
is low
Periapical
granuloma
(Figure 16)
Periapical cyst
(Contd.)
414
Extent of
carious lesion
Signs and
symptoms
Clinical findings
Clinical
diagnosis
Radiographic diagnosis
Final diagnosis
Caries involving
pulp
Pain
Food lodgment
Patient usually
avoids chewing
on the affected
side
Patient may
complain of
fever and
malaise
Frank cavitation
Involved tooth
tender on percussion
Calculus build up on
the occlusal surfaces
of the teeth on the
affected side is indicative
of unilateral chewing
Intraoral or extraoral
swelling tender on
palpation (Figure 17A, B)
Vestibular tenderness
and/or obliteration
Acute apical
periodontitis
Acute apical
periodontitis
Acute
exacerbation
of chronic
apical
periodontitis
Acute periapical
abscess
Frank cavitation
Involved tooth nontender on percussion
Intraoral or extraoral
non-tender swelling
may or may not be
present (Figure 18)
Sinus opening on the
attached gingiva or
vestibule associated
with the affected tooth
(Figure 19) or in close
proximity (occasionally
sinus openings may be
seen distant from the
affected site as the sinus
tracts may follow the
path of least resistance
(through the bone)
Vestibule may be obliterated
When the involved tooth
is tender along with
the above findings
Chronic
periapical
abscess
Radiolucency involving
pulp with discontinuity of
lamina dura at the
periapex and or diffuse
radiolucency at the
periapex (Figure 20)
Chronic
periapical
abscess
Cellulitis
Caries involving
pulp
Caries with
pulpal
involvement
Pain
Diffuse extraoral swelling
(sudden onset)
Fever, malaise
Based on the fascial
space involved,
patient may
complain of
difficulty in eating,
swallowing, limited
mouth opening
and occasionally
difficulty in
breathing
Acute
periapical
abscess
Acute
exacerbation
of a chronic
periapical
abscess
(Phoenix
abscess)
Phoenix
abscess
Ill-defined radiolucency
involving pulp
Usually very minimal
periapical changes such
as widening of the
periodontal space or illdefined radiolucency at
the periapex may evident
at acute nature of the
condition is the reason
for the very minimal bone
changes
Cellulitis of the
infraorbital,
buccal,
submandibular
space, etc.
(Contd.)
415
Table 1
Continued
Extent of
carious lesion
Signs and
symptoms
Clinical findings
Clinical
diagnosis
Radiographic diagnosis
Final diagnosis
Caries with
pulpal
involvement
Patient may
complain of dull
aching pain or may
be asymptomatic
Periapical
cyst
Well-defined radiolucency
measuring 1.5 cm in
diameter at the periapex
of the tooth surrounded
by a sclerotic border
(Figures 23 and 24).
Absence of the sclerotic
border may be indicative
of an infected cyst
(Figure 25)
Periapical cyst
Flowchart 1
Enamel caries
Acute
Apical periodontitis
Periapical abscess
Cellulitis
Infected
Acute
suppurative
osteomyelitis
Followed by
chronic phase
Ludwigs angina/fascial
space infections
Death
Sequelae of pulpitis
Chronic
Periapical
abscess
Dentoalveolar
abscess
Periapical
granuloma
Periapical
cyst
Figure 9
Figure 10
A
B
Radiograph showing proximal caries
involving dentin suggestive of dentinal caries.
Courtesy: Department of Oral Medicine and Radiology,
MCODS, Mangalore
Figure 11
Figure 13
Caries
involving
pulp
No periapical changes
Figure 14
Figure 12
Widening of periodontal
ligament space at the periapex
Figure 15
Well-defined periapical
radiolucency less than 1.5 cm
Figure 16
Figure 17
A
(A) Intraoral photograph showing a well-defined swelling on the palatal surface of decayed maxillary molar suggestive of
an acute periapical abscess. (B) Tender extraoral swelling suggestive of an acute periapical abscess. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
Figure 18
Figure 19
Shern and coworkers (1990) compared sodium uorescein with potassium iodide to disclose the porosity of an
articial incipient lesion. The extent of porous (active)
white spot lesions was disclosed only by uorescein.
Van de Rijke et al (1991) presented an optical quantication of approximal in vitro caries using a uorescent dye,
Fluorol.
Figure 20
Figure 22
Figure 21
Figure 23
Well-defined periapical
radiolucency more than 1.5 cm
with sclerotic border
Figure 24
Figure 25
Thermal Test
Cold test
Adjacent or contralateral unaffected teeth should be tested
for baseline comparisons because the duration of pain may
differ among individuals.
Figure 26
Heat test
The tooth in question is isolated. A 3-inch gutta percha
stick is warmed over a flame until it becomes soft and just
begins to glisten.
The heated stick is then applied to the middle one-third
of the facial surface of the crown. Normally a response is
appreciated in about 2 seconds.
2.
3.
4.
Patient anxiety
Saliva conducting the stimulus to the gingiva
Metallic restoration conducting the stimulus to the adjacent teeth
Liquefaction necrosis conducting the stimulus to the
attachment apparatus.
Pulse Oximetry
The pulse oximeter is a non-invasive oxygen saturation monitoring device widely used in medical practice for recording
blood oxygen saturation levels during the administration
of intravenous anesthesia.
The pulp oximeter is based on the modication of the
principle of Beers law, which relates the absorption of
light, by a solute to its concentration and optical properties at
a given light wavelength. It also depends on the absorbance
characteristics of hemoglobin in the red and infrared range.
In the red region, oxyhemoglobin absorbs less light than
deoxyhemoglobin and vice versa in the infrared region.
The system consists of a probe containing a diode that
emits light in two wavelengths:
1.
2.
A silicon photo detector diode is placed on the opposing surfaces of the tooth, which is connected to a microprocessor.
The probe is placed on the labial surface of the tooth
crown and the sensor on the palatal surface. Ideal placement of the probe is in the middle third of the crown. If placed
in the gingival third, disturbances from gingival circulation
or any gingival trauma or bleeding will interfere with the
readings. Incisally, less of pulp tissue is present for adequate
detection of the pulse.
A number of clinical studies have proved that the pulse
oximetry is an effective and objective method of evaluating dental pulp vitality. Though the surrounding insulation of the enamel and dentin are hindrances to the
detection of a pulse in the pulp, it has proved to be a successful method in 70% of the clinical trials. It is also useful
in cases of impact injury where the blood supply remains
intact but the nerve supply is damaged.
424
Radiographs show radiolucent areas adjacent to a restoration. Restorative materials such as composite, silicate and
acrylic can resemble recurrent caries. These radiolucent
restorative materials can be differentiated from recurrent
caries by their well-defined and smooth outlines (Figure 27).
Rampant caries
Rampant caries usually occurs in children. There will be
extensive smooth surface caries involving many teeth.
Radiation caries
Radiation caries can be considered as a type of rampant
caries seen in patients who receive radiation therapy for
head and neck tumors. It occurs secondary to xerostomia.
Caries begins at the cervical region and may aggressively
encircle the tooth causing entire crown to be lost with
only root fragments remaining in the jaws. Radiograph
shows dark radiolucent shadows appearing at the cervical
margins of teeth.
Figure 27
426
Figure 30
Buccinator
muscle
Temporalis muscle
Tongue
Sublingual space
Pterygomandibular space
Masseteric space
Medial pterygoid muscle
Figure 29
Medial pterygoid muscle
Parotid gland
Parotid
space
Masseter
muscle
Submasseteric
space
Parapharyngeal space
Superior
constrictor
muscle
Peritonsillar
space
Figure 31
Lateral pharyngeal space
Prevertebral space
Retropharyngeal space
Superior constrictor muscle
Pterygoid muscle
Pterygomandibular
space
Mandible
Masseter muscle
Buccal space
Buccinator
muscle
Buccinator muscle
Mylohyoid muscle
427
Figure 32
Buccopharyngeal
fascia
Alar fascia
Prevertebral fascia
Retropharyngeal
space
By lymphatics to the regional lymph nodes and eventually into the blood stream
By the blood stream.
General factors
Hosts resistance or immunocompetence of the host
Virulence of microorganisms.
Local factors Intact anatomical barriers:
Alveolar bone
Periosteum
Adjacent muscles and fascia.
LUDWIGS ANGINA
Prevertebral space
Table 2
Space
Boundaries
Contents
Submental space
Submandibular space
Sublingual space
Canine space
Buccal space
Temporal space
(masticatory space)
429
Table 2
Continued
Space
Boundaries
Contents
Parotid space
Submasseteric space
(masticatory space)
Pterygomandibular space
Retropharyngeal space
Parapharyngeal space
Lateral pharyngeal and
retropharyngeal spaces
These spaces form a ring
around pharynx and
communicate with
submandibular space and
retromandibular space
mandible by periapical dental abscesses. Spread to the sublingual space is around the posterior margin of mylohyoid
muscle. It has, however, been reported as a result of mandibular fracture, submandibular sialadenitis, peritonsillar
abscess, epiglottitis and oral malignancies.
Microbiology
Causative bacteria include many gram-negative and anaerobic organisms, streptococci and staphylococci. The bacterial
430
isolates vary and are often mixed. Alpha-hemolytic streptococci, staphylococci and Bacteroides are commonly
reported. Other anaerobes such as peptostreptococci, peptococci, Fusobacterium nucleatum, Veillonella species and
spirochetes are also seen. A foul breath/odor usually indicates the presence of an anaerobe. Gram-negative organisms such as Neisseria catarrhalis, Escherichia coli,
Pseudomonas aeruginosa and Hemophilus influenzae have
also been reported.
Anatomical considerations
The submandibular space is composed of two spaces separated anteriorly by the mylohyoid muscle: the sublingual
space, which is superior, and the submaxillary space,
which is inferior. The spread of infection is halted anteriorly by the mandible and inferiorly by the mylohyoid
muscle. The infectious process expands superiorly and
posteriorly, elevating the floor of the mouth and the
tongue.
The hyoid bone limits the process inferiorly, and swelling spreads to the anterior aspect of the neck, causing
distortion and a bull neck appearance. This then evolves
to an infectious compartment syndrome of the submandibular and sublingual spaces.
Investigations
Conventional radiographs such as intraoral periapical
radiographs or orthopantomograph (OPG) may help to
identify the offending tooth. However, a contrastenhanced computed tomography (CECT) scan is helpful in
assessing the extent of spaces involved.
Treatment
The treatment plan for each patient should be individualized and based on a number of factors. The stage of the
disease and co-morbid conditions at the time of presentation and the resources available.
Treatment includes assessment and protection of the
airway, use of intravenous antibiotics, surgical evaluation
and, if necessary, operative decompression. Intravenous
dexamethasone and nebulized adrenaline have been used
to reduce upper airway edema in such cases to defer or
avoid airway instrumentation altogether. Distorted airway
anatomy, tissue immobility, and limited access to the
mouth make orotracheal intubations by direct laryngoscopy difcult. In advanced cases, induction of general
anesthesia is dangerous because this may precipitate complete airway closure and make mask ventilation and intubation impossible. Securing of the airway in the awake
state is therefore the safest option.
Blind nasal intubation is to be avoided as, besides having a high failure rate, it could cause catastrophic bleeding, laryngospasm, airway edema, rupture of pus into the
oral cavity and aspiration. Complete airway obstruction
could be precipitated, potentially necessitating an emergency cricothyrotomy. Classically, tracheostomy was considered as the standard of care for establishment of a
denitive airway.
Elective awake tracheostomy has been suggested for all
patients with deep neck infections to avoid the dangers
of emergency tracheostomy in a severely compromised
airway.
OSTEOMYELITIS
By strict definition, osteomyelitis is the inflammation of
medullary portion of bone. However, clinical evidence has
shown that it is seldom limited to the endosteum and it usually affects the cortical plates and the periosteum.
Considering the extent of the bone involvement, osteomyelitis may best be described as an inammatory condition
of bone that usually begins as an infection of the medullary cavity which rapidly involves the haversian system and
quickly extends to the periosteum of the area.
Osteomyelitis can be broadly categorized as exogenous
osteomyelitis (47%), osteomyelitis secondary to vascular
insufciency (34%) and hematogeneous osteomyelitis (19%).
The implantation of pins, plates, screws, dental implant
and articial joint can also seed infection as a nidus for
pathogens, and therefore create postoperative osteomyelitis.
431
Predisposing factors
Conditions affecting host resistance Systemic, metabolically compromised individuals (factors such as age of
patient, malnutrition, immunosuppression and congenital
or acquired pathophysiology disrupting microvascular perfusion of the calcified tissue structure and investing soft
tissue envelope).
Examples include diabetes, AIDS, use of steroids,
agranulocytosis, leukemia, severe anemia and cancer chemotherapy.
Conditions affecting jaw vascularity Conditions that
affect the vascularity of the jaw bones include radiation,
bone malignancy, osteoporosis, osteopetrosis, Pagets disease of bone, fibrous dysplasia and bone necrosis (mercury,
bismuth, arsenic poisoning, long-term chemotherapeutic
agents like bisphosphonates).
Microbiology of osteomyelitis
Microorganisms specific for different age groups
(Osteomyelitis of other bones)
Newborns (younger than 4 months): S. aureus, Enterobacter
species, and group A and B Streptococcus species.
Children (aged 4 months to 4 years): S. aureus, group A
Streptococcus species, Hemophilus inuenzae, and Enterobacter species.
Children, adolescents (aged 4 years to adult): S. aureus
(80%), group A Streptococcus species, H. inuenzae, and
Enterobacter species.
Adult: S. aureus and occasionally Enterobacter or
Streptococcus species.
Osteomyelitis of jaw bones The microorganisms responsible for osteomyelitis affecting the jaw bones reflect the
polymicrobial nature of odontogenic infections in general
and agents associated with suppurative infection and periapical abscesses in specific.
Fusobacterium nucleatum, Prevotella intermedia, Peptostreptococcus, Actinomyces and Streptococcus species (alpha
hemolytic) are the predominant isolates from osteomyelitis
affecting the jaws.
Hosts who have serious underlying illnesses may exhibit
the presence of facultative gram-negative bacilli and S. aureus.
Mycobacterium tuberculosis, Treponema pallidum and
Actinomyeces spp. produce specic forms of osteomyelitis.
Tubercular osteomyelitis of the spine is referred to as
Potts disease. Burkholderia cepacia complex have been
implicated in vertebral osteomyelitis in intravenous drug
abusers. Systemic mycotic (fungal) infections may also
cause osteomyelitis. The two most common pathogens
involved in such infections are Blastomyces dermatitidis
and Coccidioides immitis.
Osteomyelitis of jaw bones Osteomyelitis of the mandible
occurs primarily from odontogenic infection caused by
432
Pathogenesis
With the initiation of the infection, the intramedullary
pressure increases substantially, compromising the vascularity, thus heralding the beginning of a stage of bony
necrosis.
The purulent material traverses networks of Haversian
and perforating canals, eventually accumulating under the
periosteum and lifting it from the bony cortex.
As the pus accumulates, periosteal perforation can
occur ultimately forming abscesses and often stulous
tracts within mucosal and cutaneous tissues. In chronic
cases granulation tissue, dead bone (sequestrum) separated
from surrounding healthy tissue, and eventually, a reactive sleeve of new periosteal tissue (involucrum) formation
may also be seen.
Classification of Osteomyelitis
Waldvogel classification system for
osteomyelitis
Hematogeneous osteomyelitis
Osteomyelitis secondary to contiguous focus of infection
No generalized vascular disease
Generalized vascular disease
Chronic osteomyelitis (necrotic bone).
Systemic factors (Bs): Malnutrition, renal or hepatic failure, diabetes mellitus, chronic hypoxia, immune disease, extremes of age, immunosuppression or immune
deficiency.
Local factors (Bl): Chronic lymphedema, venous stasis,
major vessel compromise, arteritis, extensive scarring,
radiation fibrosis, small-vessel disease, neuropathy,
tobacco abuse.
Classification based on the clinical course and
radiographic features
Suppurative osteomyelitis
Acute suppurative osteomyelitis
Chronic suppurative osteomyelitis
Primary (no preceding acute phase)
Secondary (follows acute phase)
Infantile osteomyelitis.
Non-suppurative osteomyelitis
Focal sclerosing osteomyelitis (condensing osteitis)
Diffuse sclerosing osteomyelitis
Proliferative periostitis (periostitis ossificans)
Osteoradionecrosis.
Clinical features
Osteomyelitis may be acute, subacute or chronic, and
presents a different clinical course depending on its
nature.
Figure 33
A
Intraoral sinus opening and diffusely enlarged gingiva associated with acute osteomyelitis.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
434
Figure 34
Figure 35
Figure 36
A
Radiographs showing ill-defined radiolucent areas with moth-eaten appearance characteristic of chronic suppurative osteomyelitis.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
Histologic features
The typical histological picture shows intertrabecular areas
of bone filled with chronically or subacutely inflamed
fibrous connective tissue. Presence of scattered sequestra
and pockets of abscess are common.
Radiographic features
Radiographs will reveal a poorly defined radiolucency
(moth-eaten appearance) within the body of the mandible
435
610 inches long are placed into the bone bed through
separate skin incisions along the lateral bony surface through
holes drilled into bone. These drains are held to the skin with
sutures or tape. Alternatively, two tubes may exit from one
stab incision or a single tube may be used for instillation and
suction.
The tubes are ushed with saline solution and the irrigation solution is introduced through one tube while the other
tube is connected to low pressure suction. Various irrigating
solutions can be used often containing antibiotics, wetting
agents and proteolytic enzymes.
Antibiotics in high concentration also may be placed in
direct contact with the bone manually or with an implantable
pump.
Tobramycin or gentamicin is contained in acrylic resin
bone cement beads. Impregnated chains of beads are useful,
especially in chronically infected bone associated with fractures and in chronic sclerosing osteomyelitis refractory to
systemic antibiotics. The beads and drain are left in place
1014 days and then removed through a small incision.
Hyperbaric oxygen (HBO) therapy has been used to promote healing in refractory chronic osteomyelitis.
In the acute stage, surgery should be limited to removal
of severely loose teeth and bone fragments and incision,
drainage of uctuant areas. Deeply located or extensive
abscesses may require treatment with the patient under
general anesthesia.
Sequestrectomy Sequestra can be cortical, cancellous or
cortical-cancellous and generally are not seen until at
least 2 weeks after the onset of infection. These can persist
for several months. In the chronic state, the involucrum or
shell of bone produced by the periosteum may be perforated by tracts (cloacae) through which the pus escapes to
epithelial surfaces. Sequestra are avascular, so are poorly
penetrated by antibiotics.
Once the sequestra are formed, they can be removed
with a minimum of surgical trauma.
Saucerization Saucerization is the unroofing of the bone
to expose the medullary cavity for thorough debridement.
Saucerization is useful in chronic osteomyelitis because it
permits removal of formed and forming sequestra. The procedure can be done after resolution of the acute phase. This
decompresses the bone to allow ready extrusion of pus, debris
and avascular fragments. The patient is more comfortable.
Decortication This refers to removal of chronically
infected cortex of bone. Once the disease is in its subacute or
chronic stage, use of decortication promotes resolution based
on the premise that the affected bone is avascular and harbors microorganisms. This can be used as initial treatment
of primary and secondary chronic osteomyelitis, or when
initial regimens have failed.
Resection and reconstruction Resection of osteomyelitic
area with immediate or delayed reconstruction may be
436
necessary to resolve low-grade persistent chronic osteomyelitis. Using an extraoral approach bone is debrided
until bleeding surfaces are encountered distally and proximally. Single or multiple blocks of autologous corticocancellous bone grafts are placed for immediate reconstruction.
Infantile Osteomyelitis
Infantile osteomyelitis usually occurs few weeks after
birth and generally involves the maxilla. This uncommon
condition involves risks with ocular, intracranial spread
and facial deformities.
It is believed to occur by hematogeneous route or from
perinatal trauma.
Generalized symptoms include fever, irritability, malaise, anorexia, dehydration and even convulsions and
vomiting.
Facial cellulitis is seen centered about the orbit associated
with inner and outer canthal swelling, palpebral edema, closure of the eye and proptosis. Purulent discharge from the
nose and the medial canthus may be evident.
Radiographic features
Radiographs reveal diffuse sclerotic patchy cotton-wool
appearance of bone. Extensive involvement of the mandible bilaterally and occasionally the maxilla and mandible may be affected. The borders of the patchy sclerosis are
diffuse and cannot be differentiated from the surrounding
normal bone (Figure 37).
Management
Owing to the extensive bone involvement surgical management is not indicated. In some patients the resolution
of the causative periodontal disease results in improvement
of this condition. However, antibiotics have been employed
to combat periods of acute exacerbations.
Periostitis Ossificans
(Chronic Osteomyelitis with Proliferative Periostitis)
Wood et al (1988) in their two part series of articles titled
Periostitis ossificans versus Garrs osteomyelitis. Part I.
What did Garr really say? and Periostitis ossificans versus Garrs osteomyelitis. Part II. Radiologic analysis of
93 cases in the jaws, described their opinions and observations on the use of the terms Garrs osteomyelitis and
periostitis ossificans.
1.
2.
3.
4.
Gorman is given the credit for using the term periostitis ossificans in 1951 to describe a productive inflammatory condition of the mandibular periosteum. Lovemann (1941)
was probably the first to recognize the condition.
Tong et al (2006) reported a case of osteomyelitis with
proliferative periostitis. The authors believe that the term
chronic osteomyelitis with proliferative periostitis is the
most accurate description of periostitis ossicans.
Nortj et al (1988) conducted a radiological analysis
of 93 cases of periostitis ossicans occurring in the jaws.
In their study, the age at initial consultation ranged from
2 to 69 years with a mean of 13.3 years. Males were more
commonly affected than females (1.27:1). The causes
for periostitis ossicans were due to periapical lesions
437
Figure 37
Orthopantomograph showing diffuse sclerosis of the mandible giving rise to cotton-wool appearance.
Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
secondary to dental caries, untreated fractures and primary tuberculosis of the mandible. Two patients in their
study had lateral inammatory odontogenic cysts.
The mandibular left rst molar was most commonly
involved. The other sites involved were mandibular second
molar, mandibular premolar, mandibular primary second
molar, angle of the mandible, mandibular third molar and
maxillary premolar.
The periosteal reaction was visible at the buccal, lingual
and inferior aspects of the mandibular body. The authors
also reported of periosteal reaction occurring in the maxilla and sigmoid notch. In their study, the number of the
periosteal laminations ranged from 1 to 12. The size of most
of the sequestra ranged from 1 to 2 mm. Larger sequestra
were seen in patients with infected fractures. The size of
the sequestra was age dependent with smaller sequestra
present in younger patients (Figure 38).
In about 63% of their patients, follicles of unerupted teeth
adjacent to the site of periostitis showed destruction. Other
changes that were noted included supraeruption of teeth,
bodily movement of unerupted tooth bud and widening of
the periodontal ligament space of the teeth inciting the
infection and occasionally of the adjacent vital teeth.
The periosteal reaction can either occur as long, narrow
periosteal reaction (common in adults) or fusiform, short and
wide periosteal reaction (seen in children).
When X-ray beams interact with successive layers of
periosteal new bone on the buccal cortical plate, they get
attenuated and the resultant image will show a patchy or
granular radiopacity in the body of the mandible on lateral projections (Figure 39A, B).
Periostitis occurring in the maxilla has been referred to
as halo-shadow, which results from periosteal stripping
and subsequent bone formation in the oor of the maxillary sinus related to a rst molar tooth.
438
Figure 38
Radiographic investigations
Nortj et al (1988) recommended the use of lateral oblique
and panoramic radiographs (lateral radiographs) to demonstrate the periosteal reaction. If the periosteal reaction is not
evident on lateral radiographs, occlusal radiographs have to
Figure 39
Periosteal
new bone
X-rays
Patchy
radiopacity
Illustration showing the appearance of granular radiopacity in the body of the mandible on lateral projections
Figure 40
It is an excessive exuberant proliferation of a longstanding inflamed dental pulp. Usually affects teeth
with large carious lesions. Commonly seen in children
and young adults and often associated with the primary or permanent first molars.
Clinically it appears as red to pink mass of tissue protruding from the pulp into the large open cavity of the
carious tooth (Figure 40).
There can be progressive, diffuse sclerosis of the medullary
and cortical portions of the maxillofacial skeleton, especially
mandible, over time.
439
CHAPTER
16
Plaque Microbiology
Organisms in Various Periodontal Diseases
Periodontal diseases range from simple and early inflammation of marginal gingiva to advanced gingivitis and
subsequently periodontitis. A good classification system is
crucial in identifying and differentiating the various kinds
of periodontal diseases.
Periodontal disease classication is useful to help
establish diagnosis, determine prognosis and facilitate
treatment planning.
Different classication systems of periodontal diseases
have been used for years and also replaced by newer classication systems as the knowledge of understanding
microbiology, etiology, pathogenesis and host response
have improved vastly over the past few decades.
Currently, the most accepted classication is the 1999
World Workshop Classication System.
Periodontal Diseases
Local Contributing Factors
Iatrogenic Factors
HostMicrobial Interaction
Defense Mechanism
Plaque-induced Gingivitis
Non-plaque-induced Gingivitis
Gingival Diseases Associated with Medications
Gingival Diseases Associated with Systemic Diseases
Gingival Diseases Associated with Malnutrition
Gingival Diseases Associated with Heredity
Gingival Diseases Associated with Ulcerative Lesions
Gingival Lesions Manifested in Childhood Diseases
Gingival Lesions Associated with Chicken Pox
440
Gingival Diseases
Syndromes
Hormonal Factors
Sex Hormones
Nutritional Factors
Effects of Vitamin Deficiency
I. Gingival diseases
A. Dental plaque-induced gingival diseases: These diseases
may occur on a periodontium with no attachment loss
or on one with attachment loss that is stable and not
progressing.
1. Gingivitis associated with dental plaque only
a. Without local contributing factors
b. With local contributing factors
2. Gingival diseases modified by systemic factors
a. Associated with endocrine system
i. Puberty-associated gingivitis
ii. Menstrual cycle-associated gingivitis
iii. Pregnancy-associated gingivitis and pyogenic
granuloma
iv. Diabetes mellitus-associated gingivitis
b. Associated with blood dyscrasias
i. Leukemia-associated gingivitis
ii. Others
3. Gingival diseases modified by medications
a. Drug-influenced gingival diseases
b. Drug-influenced enlargements
c. Drug-influenced gingivitis
B.
II.
Chronic periodontitis
A. Localized (less than 30% of sites involved)
B. Generalized (more than 30% of sites involved)
C. Slight (12 mm clinical attachment loss)
D. Moderate (34 mm clinical attachment loss)
E. Severe (more than 5 mm clinical attachment loss)
441
v. Gingival excess
Pseudopocket
Inconsistent gingival display
Excessive gingival display
Gingival enlargement
vi. Abnormal color
C. Mucogingival deformities and conditions on edentulous ridges
i. Vertical and/or horizontal ridge deficiency
ii. Lack of gingival/keratinized tissue
iii. Gingival/soft tissue enlargement
iv. Aberrant frenum/muscle position
v. Decreased vestibular depth
vi. Abnormal color
D. Occlusal trauma
i. Primary occlusal trauma.
The current classification has many changes seen which is
drastically different from the previous classifications. The
most noticeable change is that periodontitis is now classified based on the rate of progression of the disease and not
on the age of onset.
Adult periodontitischronic periodontitis
The term adult periodontitis is discarded since this
form of periodontitis is seen in wide range of ages and
found both in primary and permanent dentitions. Hence,
the term chronic periodontitis was chosen because it does
not reflect the age of individual rather reflects the rate
of progression of periodontal disease, which is slow in
nature.
Early onset forms of periodontitisaggressive
periodontitis
The term early onset periodontitis has been discarded as
this form of periodontitis is seen in various ages and in
older individuals too. Thus, the term aggressive periodontitis was chosen. Progressive periodontal disease can be
either localized or generalized. The term localized aggressive periodontitis replaces localized juvenile or localized
early onset periodontitis. The term generalized aggressive
periodontitis replaces generalized juvenile or generalized
early onset periodontitis. The term pre-pubertal periodontitis has been discarded and is currently described as
localized or generalized periodontitis or periodontitis as
manifestations of systemic disease.
GINGIVAL DISEASES
Increasing evidence indicates that gingivitis is not a single
disease, but an assortment of diseases that are the end result
of a variety of different processes. Inflammation of gingiva by bacteria is most common, but pathological changes
in the gingiva can also result from systemic conditions
(e.g. puberty), drugs (e.g. amlodipine) and neoplasms (e.g.
leukemia). Hence, any disease that primarily affects gingival
tissues should be primarily classified as a gingival disease.
The gingival diseases associated with children, adolescents and young adults have several common characteristics.
Universal features include clinical signs of inammation,
signs and symptoms that are conned to the gingiva,
reversibility of the disease on removal of the etiology, and
the presence of microbial plaque to initiate or exacerbate
the severity of the lesion.
Plaque Microbiology
Oral cavity can be regarded as a single microbial ecosystem or macroenvironment because the colonization of oral
cavity starts at the time of birth. Within hours of birth the
sterile oral cavity gets colonized by facultative and aerobic bacteria, which will be followed by anaerobic bacteria
by 2nd day. By 2 years of age there will be around 400
different kinds of bacteria. After tooth eruption, a more
complex oral flora is established. However, all these species are seen to be living in harmony with the host. But,
however, there is an imbalance in this relationship between
the host and microorganisms, disease prevails which is
controlled by various other factors.
Oral environment is dominated with saliva, which is very
complex in composition. Although saliva is not a good
medium for supporting the growth of bacteria, it is likely that
organisms shed from intraoral reservoirs nd transient
residence in saliva. Oral cavity provides two types of surfaces
for colonizationsoft and hard tissue (teeth)main difference
being soft tissue desquamation; hence the colonies are shed
frequently whereas the hard surface provides a solid medium
for the bacteria to adhere and develop complex layers.
Terminology
Dental plaque: Dental plaque is clinically defined as a
structural resilient yellow-grayish substance that adheres
tenaciously to the intraoral hard surfaces, including
removable and fixed restoration.
e.
f.
5.
Generalized aggressive
a. F. nucleatum
b. Lactobacillus
c. Eubacterium
d. A. naeslundii
e. A. actinomycetemcomitans
B. forsythus
P. gingivalis
Campylobacter
6.
NUG/NUP
a. P. intermedia
b. Fusobacterium
c. F. nucleatum
d. P. gingivalis
7.
Periodontal abscess
a. F. nucleatum
b. P. micros
c. P. intermedia
d. P. gingivalis
e. B. forsythus.
Formation of plaque
It is a complex procedure initially involving the formation
of pellicle (glycoprotein, phosphoproteins) around the tooth
surface. Then the early bacterial colonization takes place
with facultative aerobic gram-positive organisms. Some of
the early colonizers are Actinomyces and Streptococcus
sanguis which adhere to the pellicle through adhesins.
Later plaque maturation takes place with co-aggregation
of secondary colonizers including Fusobacterium nucleatum, Prevotella intermedia, Porphyromonas gingivalis,
Capnocytophaga, etc. Thus in maturation of plaque there
is a transition from the early aerobic gram-positive facultative species to an anaerobic gram-negative species.
Periodontal health
a. Streptococcus
b. Actinomyces
c. Veillonella
d. Fusobacterium
2.
Gingivitis
a. Streptococcus
b. Actinomyces
c. Peptostreptococcus
d. Eubacterium
e. Capnocytophaga
f. Fusobacterium
g. Veillonella
3.
Chronic periodontitis
a. Streptococcus
b. Peptostreptococcus
c. Eubacterium
d. Actinomyces
e. Lactobacillus
f. P. gingivalis
g. Campylobacter rectus
h. F. nucleatum
i. Selenomonas
j. Actinobacillus actinomycetemcomitans
k. Eikenella corrodens
4.
Localized aggressive
a. A. actinomycetemcomitans
b. Eubacterium
c. A. naeslundii
d. F. nucleatum
C. rectus
Veillonella
HOSTMICROBIAL INTERACTION
Health is not a static condition, it is a dynamic state in which
the living and functioning individuals remain in balance
with a constantly changing environment. These changes
in environment also cause changes in tissue activity so that
normal function can continue, a process known as homeostasis. If the environmental changes over-ride this homeostasis, the normal function cannot continue and this change
is termed as disease.
Defense Mechanism
These protect the body from attack from microorganisms
and can be classified as:
Non-specific mechanism
Specific mechanism.
Non-specific mechanisms
Bacterial balances
Surface integrity
Surface fluid and enzymes
Inflammatory reaction
Neutrophil and macrophage activity.
Specific mechanisms
Plaque bacteria produce a number of factors (virulence factor), which causes disease directly, or individually by stimulating the immune and inflammatory system. It is now
known that individuals prone to periodontal disease have
an aberrant immune inflammatory response to plaque which
is genetically determined.
Plaque-induced Gingivitis
It is the inflammation of gingiva which results from the bacteria located at the gingival margin. The association of plaque
with gingival inflammation made it a frequently postulated
cause of gingivitis. The initial histologic changes from health
to plaque-induced gingivitis may not be evident clinically
(Page and Shroeder, 1976; Bimstein et al, 1985) but as
gingivitis progresses, clinical signs becomes more obvious.
Plaque-induced gingivitis begins at the gingival margin
and can spread into the deeper gingival component.
Clinical signs of gingival inammation involve the change
in color, contour, size, shape, consistency and surface texture are associated with a stable periodontium which exhibits no loss of periodontal attachment or alveolar bone. The
classic clinical indicators of signs of inammation are bleeding on probing and color change from pink/coral pink to
reddish pink or erythematous gingiva. In children, gingivitis is not intense as that found in young adults with similar
quantity of accumulation of dental plaque (Mattson and
Goldberg, 1985).
In the initial and established stages of gingivitis, dental
plaque is predominantly comprised of gram-positive aerobic
microorganisms including Streptococcus mitis, S. sanguis,
Actinomyces viscosus, A. naeslundii and Eubacterium spp.
As age advances, development and severity of gingivitis is
mainly dependent on the quality of the plaque rather than
the quantity and the other contributing factors like the host
immune response, environmental, genetic and the behavioral factors. The common clinical signs and symptoms of
gingivitis include redness, edema, bleeding on probing,
tenderness and enlargement (Loe et al, 1965; Suzuki, 1988)
(Figure 1).
Radiographically no changes will be seen as the inammation is conned only to the gingival sulcus. Histologic
NSAIDs
Prostaglandin E2 (PGE2), a product of cyclooxygenase pathway has been shown to increase the amount of alveolar bone
destruction by amplifying local inflammation. NSAIDs block
COX pathway thus decreasing PGE2 synthesis. A number of
NSAIDs have been used including ibuprofen, flurbiprofen,
indomethacin, ketoprofen and naproxen.
Figure 1
Matrix metalloproteinases
Matrix metalloproteinases are a family of proteolytic
enzymes secreted by a number of cells including leukocytes, epithelial connective tissue cells whose primary function is degradation of extra-cellular matrix components.
Tetracycline and other drugs of the same group show to
inhibit the MMPs production from the host, primarily the
neutrophil MMPs. In doing so they increase host resistance
to connective tissue destruction forms of tetracycline are
submicrobial dose of tetracycline/doxycycline and chemically modified tetracycline.
444
changes include proliferation of basal junctional epithelium leading to apical and lateral cell migration, vascular
dilatation and vasculitis of blood vessels adjacent to
the junctional epithelium, progressive destruction of collagen bers, cytopathologic alteration of resident broblasts and progressive inammatory cell inltrate (Page
and Shroeder, 1976).
Non-plaque-induced Gingivitis
Gingival diseases associated with endogenous
sex steroid hormones
Since the 19th century, evidence suggests that the tissues of
the periodontium are modulated by androgens, estrogens and
progesterone. Much of this evidence has come from observing
the changes in gingival tissues during distinct endocrinologic events (puberty, menstrual cycle, pregnancy, etc.).
The principal explanation for sex steroid hormoneinduced changes in the gingiva has pointed to changes of
microbiota in dental plaque, immune function, vascular
properties and cellular function in the gingiva. Sex steroid
hormones will affect the host by inuencing cellular function (in the blood vessels, the epithelium and the connective
tissue) and immune function, and together with hormoneselected bacterial populations occupying the gingival sulcus,
induce specic observable changes in gingival tissues
(Mariotti, 1994).
Puberty-associated gingivitis
Puberty is not a single episode but a complex process of
endocrinological events that produce changes in the physical appearance and behavior of adolescents. Average age
of menarche is 1213 years. The early onset of puberty, particularly for adolescent girls, increases the time of exposure
of periodontal tissues to steroid hormones and the possibility
of gingival disease.
The incidence and severity of gingivitis in adolescents
are inuenced by a variety of factors, including plaque levels, dental caries, mouth breathing, crowding of teeth and
tooth eruption (Stamm, 1986). A number of studies have
demonstrated an increase in gingival inammation in circumpubertal individuals of both sexes without a concomitant increase in plaque levels (Sutcliffe, 1972; Hefti et al,
1981). Although puberty-associated gingivitis has many
of the clinical features of plaque-induced gingivitis, this
disease will develop frank signs of gingival inammation
in the presence of relatively small amounts of local irritants
(plaque) during the circumpubertal period.
Phenytoin
The disfiguring overgrowth of gingiva is a significant outcome principally associated with antiepileptic drugs such as
phenytoin sodium, immunosuppressors such as cyclosporine and calcium channel blockers such as nifedipine, amlodipine, verapamil, diltiazem and sodium valproate (Hassel
and Hefti, 1991; Seymour et al, 1996).
Most common clinical features seen in drug-induced
gingival enlargement are as follows:
Figure 2
A
Enlargement of the labial and palatal aspects of the gingiva in patients using phenytoin sodium.
Gingival enlargement leads to the formation of pseudopockets. Courtesy: Dr Francisco, Mexico
446
Figure 3
A
Enlargement of the labial and palatal aspects of the gingiva in a patient on nifedipine. Courtesy: Dr Francisco, Mexico
Figure 4
B
aggregation has been designated by terms such as gingivostomatitis elephantiasis, familial elephantiasis, juvenile
hyaline fibromatosis, idiopathic gingival fibromatosis and
hereditary gingival fibromatosis. Although there were almost
100 published reports of hereditary-associated gingival
overgrowths in the 20th century, information about the
natural history of this rare disease is extremely limited and
its etiology is unknown.
Hereditary gingival bromatosis appears to be a slowly
progressive gingival enlargement which develops upon
eruption of the permanent dentition. However, gingival
enlargement can also occur in the primary dentition
(Emerson, 1965; Jorgenson and Cocker, 1974; Lai et al,
1995; Miyake et al, 1995). The disease can be localized or
generalized and may ultimately cover the occlusal surfaces
of teeth. The enlarged gingiva is non-hemorrhagic and
rm, but there can be an overlay of gingival inammation
which can augment the enlargement. The histologic features
of hereditary gingival bromatosis include dense brotic
connective tissue as well as epithelial hyperplasia with
elongated and increased rete pegs (Johnson et al, 1986;
Clark, 1987).
Hereditary gingival bromatosis can be inherited as a
simple mendelian trait, in some chromosomal disorders and
as a malformation syndrome (Witkop, 1971; Jones et al,
1977; Takagi et al, 1991; Goldblatt and Singer, 1992; Hallet
et al, 1995). Although the specic genes for this disease have
not been identied, genetic analysis supports the presence
of two different gene loci on chromosome 2p (Shashi et al,
1999). Research into the cellular responses of this disease
suggests an accumulation of specic populations of gingival broblasts resulting in an abnormal accumulation of
connective tissues (Huang et al, 1997; Tipton et al, 1997).
may be expressed. The lesion is generally self-limiting, ultimately rupturing if permitted to progress. The gingival abscess
should not be confused with the periodontal abscess, which
affects the supporting periodontal structures.
PERIODONTAL DISEASES
Local Contributing Factors
It is well known that the primary cause of gingival inflammation and periodontal destruction is bacterial plaque.
These factors that tend to accelerate the disease locally, are
termed as local contributing factors in the progression of
periodontal disease.
The local factors are:
Calculus
Iatrogenic factors
Overhanging restoration
Margins of restoration
Contours
Restorative materials
Occlusion
Restorative procedures
Pontic
Improper removable partial dentures
Malocclusion
Food impaction
Orthodontic therapy
Anatomic contributing factors
Proximal contact relationship
Cervical enamel projections and enamel pearls
Bifurcation ridges
Developmental grooves
Root anatomymorphology and length
Root fusion, cemental tears
Proximity to adjacent teeth
Endodontic lesions
Caries
Habits
Toothbrushing trauma
Mouth breathing, tongue thrusting and other habits
Factitial injuries
Smoking
Trauma from occlusion
Trauma
Physical
Chemical
Thermal
Radiation therapy
Mucogingival problems
Cysts and tumors.
Figure 5
Calculus
Calculus is mineralized dental plaque that forms on surface
of teeth and dental prosthesis. It is one of the most important
and commonly occurring local contributing factor as it is
invariably covered with bacterial plaque on its surface.
Types
Supragingival calculus It is white or whitish yellow in
color hard with clay-like consistency and is easily detached
from the tooth. It is most commonly found on lingual surface
of mandibular teeth and buccal surface of maxillary teeth.
Subgingival calculus It is located below the crest of
marginal gingiva and appears dark brown or black in
color and flint-like consistency. It is firmly attached to the
tooth (Figure 5).
Composition
Calculus consists of organic and inorganic constituents.
organic contents are mainly desquamated epithelial cells,
leukocytes and microorganisms. Inorganic constituents are
mainly calcium, calcium phosphates and calcium carbonates,
magnesium phosphates and other metals.
Significance of calculus
Calculus is a local factor in periodontal disease because it
always has a layer of plaque on its surface. Because calculus
is firmly attached to the tooth through an organic pellicle,
it resists removal through routine oral hygiene techniques.
Calculus plays an important role in the progression of
periodontal disease by keeping plaque in close contact
with the periodontal tissues in gingiva.
Iatrogenic Factors
Overhanging restorations: These contribute to periodontal disease by acting as a local plaque retentive
area causing accumulation of plaque and changing
the ecology that favors the growth of microorganisms.
Margins of restoration: A subgingival margin is associated with more plaque accumulation and more periodontal destruction than supragingival margins, margins
placed at the level of gingiva.
Contours: Overcontoured restorations tend to accumulate more plaque, and the natural self-cleansing mechanism fails.
Open contours: These are associated with increased
food impaction and papillary inflammation.
Restorative materials: Silicate cements and self-curing
acrylic resins accumulate more plaque.
Restorative procedures: Rubber dam, clamps, matrix
band wedges, gingival retraction chords are shown to
cause injury to the periodontium.
Pontics: When pontics are in contact with gingival tissue, they tend to accumulate more and oral hygiene
techniques are more difficult.
Improper removable partial denture: It causes an
increase in mobility of abutment teeth and also favors
accumulation of plaque leading to gingival inflammation and periodontal pocket.
Malocclusion: Plaque control is more difficult in individuals with malocclusion. Malocclusion is usually associated
with recession if the tooth is buccally placed and plaque
control is difficult because of lack of attached gingiva.
Food impaction: It is forceful wedging of food into the
periodontium by occlusal forces. This harbors more
microorganisms in interproximal area leading to periodontal destruction.
Orthodontic therapy: Orthodontic appliances are associated with food debris and plaque accumulation and
changing the ecosystem. These also cause increase in
forces on the periodontium.
451
Figure 6
Habits
Smoking
It is one of the etiological factors in ANUG. It is classified
as an environmental factor with local and systemic effects.
The local effects are peripheral vasoconstriction and localized ischemia. The individual harbors more pathogenic and
virulent subgingival microorganisms. The systemic effects
are decreased immunity with non-specific (decreased polymorphoneutrophils, chemotaxis, phagocytosis) and specific
(decreased IgG, IgA). There is also activation of proinflammatory cytokines including IL-1. TNF- IL-6 all lead to
periodontal diseases.
Since immunity and inammation are reduced in smokers there are lesser clinical signs of gingival inammation
with increased local deposits and periodontal destruction;
it is one of the etiological factor in NUG.
452
Figure 7
Systemic factors
It is well known that bacterial plaque is a main etiological
factor responsible for gingival inflammation and periodontal destruction. The bacterial plaque causes a marked host
response that varies from one individual to other, hence susceptibility of individual to periodontitis depends on various
factors, including systemic and genetic factors.
Genetic factors
Periodontal disease is multifactorial with plaque being
major factor, but some of these factors fail to explain the
variation of disease in different individuals with same
amount of local factor. This is attributed to genetic susceptibility. Genetic pleomorphism in IL-1 has been shown to
be associated with chronic periodontitis. Studies have
shown a link between susceptibility of aggressive periodontitis and the human leukocyte antigen of chromosome 6, which is responsible for production of IgG2, which
in turn is responsible for periodontal destruction. The
genetic polymorphism in the genes for the Fc- receptor
on the phagocytic cell is identified in localized aggressive periodontitis. Polymorphism in the gene promoter
region of chromosome 6, results in increased production
of TNF-, which has been shown to be associated with
periodontitis.
ChediakHigashi syndrome
It is an autosomal recessive disease that affects the production of organelles in many cells including melanocytes,
platelets and leukocytes. Neutrophils are characterized by
abnormal giant lysosomes containing enzymes and with
impaired ability to release them.
It is associated with severe gingivitis, periodontal disease
and loss of dentition at an early age.
Lazy leukocyte syndrome
It is a rare disease characterized by defect in PMN chemotaxis and an abnormal inflammatory response.
Leukocyte adhesion deficiency (LAD)
SYNDROMES
Downs syndrome
It is a congenital disease characterized by mental deficiency, growth retardation and severe periodontal disease.
Incidence is about 1:8001,000. Both deciduous and permanent dentitions are affected. The destruction increases
with age. The rapid periodontal destruction is commonly
attributed to immunologic defects.
It is a rare inherited genetic disorder resulting from inability to produce or express CD18, an integrin useful in leukocyte adhesion.
Both primary and secondary dentitions are affected in
LAD which presents as acute inammation and rapid
destruction of bone.
EhlersDanlos syndrome
It is an inherited condition affecting connective tissue with
reduction of collagen fiber production. The oral mucosa,
gingiva and periodontium are affected.
PapillonLefevre syndrome
It is an inherited autosomal recessive disease characterized by diffuse palmoplantar hyperkeratosis, severe destruction of periodontium and calcification of dura. Incidence
is 14:10,00,000. Deciduous teeth are usually lost by
56 years and permanent teeth a few years later (Figure 7).
Defects in PMNs adherence and chemotaxis has been
reported.
Hypophosphatasia
It is an autosomal recessive condition where there is a deficiency of the enzyme alkaline phosphatase and characterized by abnormal mineralization of bone and dental tissues.
There is premature exfoliation of deciduous teeth, loss
of alveolar bone, absence of gingival inammation and
absence of cementum. The permanent teeth are not affected.
453
Mucopolysaccharidoses (MPS)
Figure 8
Hormonal Factors
Diabetes: It is a complex metabolic disorder characterized
by glucose intolerance.
It is associated with:
Figure 9
Oral effects
Patients with diabetes have diminished salivary flow,
burning mouth and candidiasis.
Diabetes also has profound effect on periodontium resulting in gingivitis, gingival enlargement, periodontal abscess
formation, periodontitis and loss of teeth (Figures 8 and 9).
Pathogenesis
SEX HORMONES
The female sex hormones affect periodontal tissues. Estrogen
promotes keratinization and increased mucopolysaccharide content, the gingival connective tissue. Progesterone
increases the permeability and gingival blood vessels.
Changes seen are:
Oral changes include increased tooth mobility. Radiographically, it shows widening of periodontal ligament
space with absence of lamina dura, closely meshed trabaculae and cystic space known as Browns tumor.
NUTRITIONAL FACTORS
There are no nutritional factors that causes gingivitis or
periodontitis, but they produce changes in the oral cavity
including the gingiva and alveolar bone that can accelerate the process of gingivitis and periodontitis.
with pyrexia, oral ulceration and skin infections. Oral features include oral ulcerations, severe gingivitis, periodontal destruction and alveolar bone loss.
Chronic benign neutropenia of childhood There is moderate neutropenia with absolute lymphocytosis and monocytosis. Oral findings include bright red hyperplastic
edematous gingiva which bleeds easily and permanent
destruction with generalized bone loss.
Familial neutropenia It is an inherited condition which
can occur in benign and severe forms. There is moderate
to severe form of neutropenia in these conditions. Oral
findings are similar to that of chronic benign neutropenia
of childhood.
Chronic idiopathic neutropenia This occurs mainly in
females with persistent neutropenia from birth. Oral findings are similar to that of chronic benign neutropenia of
childhood.
Gingivitis
Presence of local factors like calculus and/or at least one of the
following clinical features:
Erythematous gingiva
Soft and edematous gingiva
Blunting of the interdental papilla
Loss of gingival stippling
Gingiva bleeds spontaneously on palpation or probing
Periodontitis
Gingivitis plus at least one of the following clinical features:
Gingival recession
Periodontal pocket
Mobility
Pathologic migration
Pus discharge from the gingival sulcus
Furcation involvement
Lazy leukocyte syndrome There is a defect in leukocytic chemotaxis and mobility associated with severe
gingivitis.
RADIOGRAPHIC EVALUATION OF
PERIODONTAL DISEASES
Thumb rules
Evaluate the nature and extent of the causes for periodontal destruction (subgingival calculus, overhanging
margins of proximal restorations and poorly contoured
crowns).
Assess the width of the periodontal ligament space.
Evaluate crestal bone loss.
Assessment of the pattern and extent of alveolar bone
destruction (horizontal, angular, furcation involvement).
Interpretation of radiographs
457
CHAPTER
17
Regressive Alterations
of Teeth
Ravikiran Ongole, Sumati Nagappa
Baddannavar, Praveen BN
Affecting Teeth
Attrition
Abrasion
Erosion (Corrosion)
Causes for Corrosion
Erosion Associated with Common Food Substances
Erosion Associated with Medications
Erosion Related to Deleterious Habits
Occupation-related Dental Erosion
Resorption of Teeth
Regressive Alterations of Dentin
Regressive Alterations of Pulp
Regressive Alterations of Cementum
Hypercementosis
Cementicles
CLASSIFICATION OF REGRESSIVE
ALTERATIONS AFFECTING TEETH
Tooth wear
Attrition
Abrasion
Erosion (corrosion)
Abfraction
Resorption of teeth
Internal
Internal inflammatory
Internal replacement
External
Idiopathic
458
ATTRITION
Pindborg defined attrition as the loss of enamel, dentin or
restoration by tooth-to-tooth contact. Tooth-to-tooth friction causes the form of wear called attrition. Two types of
attrition have been described. They are physiologic and
pathologic.
Physiologic attrition is referred to as the gradual and
regular loss of tooth structure as a result of normal mastication. However, pathologic attrition is conned to local areas
or specic groups of teeth caused by abnormal friction.
The physiologic causes for attrition include mastication
and deglutition. Pathologic causes that cause attrition are
abnormal occlusion, bruxism and habits such as tobacco
and betel chewing and defective tooth structure such as in
dentinogenesis imperfecta.
Attrition involves occlusal/proximal surfaces of teeth.
It occurs more frequently in males than in females due to
greater masticatory forces. It appears as a small polished
facet on cusp tips and causes attening of incisal edges in
case of anterior teeth (Figure 1). The wear will lead to
exposure of the dentin causing hypersensitivity. The worn
surfaces of opposing teeth occlude together very accurately.
Figure 1
Figure 2
459
Figure 3
Management
Patients should be educated regarding the consequences of
attrition. Sharp edges of teeth can be smoothened. Desensitizing toothpastes will help patients presenting with dentinal hypersensitivity. A soft bite guard will help break the
habit of bruxism and prevent further loss of tooth structure.
Teeth with pulpal exposure warrant endodontic treatment.
Finally, crowns may be fabricated for restoring esthetics.
ABRASION
Pathologic wearing away of tooth substance through some
abnormal mechanical process especially in the presence of
abrasive materials is known as abrasion. It usually occurs
on exposed root surfaces of teeth. Different foreign bodies
produce different patterns of abrasion. Most common type
of abrasion is toothbrush and dentifrices (toothpaste and/or
tooth powder) abrasion. Many of the residents in the villages of the Indian subcontinent use charcoal, brick and ash
to cleanse teeth. These indigenous dentifrices abrade teeth
further.
Figure 4
Clinical features
It is seen frequently on exposed root surfaces and in cervical regions of labial and buccal surfaces due to overzealous toothbrushing in horizontal manner. Maxillary teeth
are involved more than mandibular teeth, and left side is
affected commonly in case of right-handed persons and
vice versa. It appears as a V-shaped or a wedge-shaped
ditch on the root side of the cementoenamel junction in
teeth with some gingival recession (Figure 3). The exposed
dentin appears highly polished (Figure 4). Improper use of
dental floss and tooth picks may also produce such lesions
on the exposed proximal root surfaces.
In habitual pipe smokers, notching of teeth can be seen
that conforms to the shape of the pipe stem. It can also be
seen as occupation-related oral nding in carpenters and
tailors who hold objects against the teeth during work.
Exposure of dentinal tubules and the consequent irritation
of odontoblastic processes stimulates the formation of secondary dentin.
When tooth wear is accelerated by chewing an abrasive
substance between opposing teeth, the process is termed
demastication and it exhibits the feature of both attrition
and abrasion.
460
Grippo et al (2004) described a term, masticatory abrasion to refer to tooth wear on the occlusal or incisal surfaces
due to friction from the food bolus. They reported that the
masticatory abrasion can also occur on the facial and lingual
aspects of teeth as coarse food is forced against these surfaces by the tongue, lips and cheeks during mastication.
Radiographic features
Radiographically, abrasive lesions caused by toothbrush
are seen as half-moon shaped, well-defined radiolucent
areas in the cervical regions of the teeth. These defects are
usually seen involving the maxillary premolar teeth.
Figure 5
Management
Patients should be educated regarding the correct brushing and flossing technique. They should be advised to
discontinue any deleterious habits associated with their
occupations. Use of abrasive dentifrices should be strongly
discouraged.
Pulpal exposure is rarely a complication of cervical abrasion as the formation of secondary dentin protects the pulp
from being involved. Dentinal hypersensitivity when present can be managed with the use of desensitizing toothpastes
and mouthrinses.
EROSION (Corrosion)
Eccles in 1982 described erosion as the loss of dental hard
tissues by chemical action not involving bacteria. In simple
terms, erosion is the chemical or electrochemical dissolution
of teeth.
Grippo et al (2004) quoting the description of erosion by
The American Society for Testing and Materials Committee
on Standards proposed that the term erosion should be
replaced by the term corrosion. The American Society for
Testing and Materials Committee on Standards denes erosion as the progressive loss of a material from a solid surface due to mechanical interaction between that surface and
a uid, a multicomponent uid, impinging solid or liquid
particles. In order to explain this in simple terms Grippo
gave an example of river water owing forcefully against
the bridge supports leading to its erosion.
It is a common fact that no such powerful gush of oral
uids exists in the oral cavity. Thus, the term erosion may
be replaced by corrosion to describe chemical dissolution
of teeth.
Clinical features
Erosive lesions are usually smooth surface lesions evident
on the buccal and labial surface of teeth. Occasionally,
proximal surfaces may be involved.
Clinically, erosions appear as wide, polished and smooth
areas on the enamel approximating the cervical margin of
the tooth (Figure 5). The erosive areas are almost always
shallow and exhibit scooped-out architecture. The common teeth to be affected by erosion are the anterior teeth.
Flowchart 1
Erosion
Intrinsic causes
Frequent vomiting
Anorexia nervosa
Pregnancy
Bulimia
Reflux
Gastroesophageal
reflux disease
Stress reflux syndrome
Extrinsic causes
Figure 6
Table 1
Food substance
pH
Apples
2.93.5
Grapes
2.93.4
Lime
1.82.4
Oranges
2.84.0
Pineapples
3.34.1
Strawberries
3.04.2
Coffee
2.43.3
Black tea
4.2
Wines
2.33.3
7Up
3.5
2.7
2.7
Pepsi
Coke
Vinegar
2.43.4
Ketchup
3.7
Fruit jam
3.04.0
Professional swimmers
Centerwall et al (1986) studied the extent of enamel erosion
among professional swimmers at a gas-chlorinated swimming pool. In their study, the pool water sample had a pH
of 2.7. Although the recommended pH for swimming pools
is 7.28.0. They used the term swimmers erosion for the
acid erosion of dental enamel caused by inadequately maintained gas-chlorinated swimming pools.
ABFRACTION
Abfraction may be described as the microstructural loss of
tooth substance in areas of stress concentration caused by
tooth flexure. Theoretically it is believed that occlusal
forces create stresses in the cervical area of the enamel
and dentin thereby predisposing the tooth to abrasion and
erosion.
464
Parafunctional habits, malocclusion, excessive masticatory load and use of dental appliances are some known
causes for abfraction.
Abfraction commonly occurs in the cervical region of
teeth, where exure may lead to the fracture of the slender
layer of enamel rods, as well as microfracture of cementum
and dentin.
Morphologically they appear as crescent-shaped lesions
along the cervical margin of the tooth. It often affects a
single tooth with adjacent unaffected teeth. It commonly
affects the mandibular dentition because of the lingual orientation and increased susceptibility to concentration of
tensile stresses in the cervical region.
Table 2
Score
Criteria
Score between 3 and 8: Oral hygiene and dietary assessment, and advice, routine maintenance and observation, Repeat at 2-year intervals.
Score between 9 and 13: Oral hygiene and dietary
assessment, and advice, identify the main etiological
factor(s) for tissue loss and develop strategies to eliminate respective impacts. Consider fluoridation measures
or other strategies to increase the resistance of tooth
surfaces. Ideally, avoid the placement of restorations
and monitor erosive wear with study casts, photographs
or silicone impressions. Repeat at 6- to 12-month
intervals.
Score 14 and over: Oral hygiene and dietary assessment,
and advice, identify the main etiological factor(s) for
tissue loss and develop strategies to eliminate respective impacts. Consider fluoridation measures or other
strategies to increase the resistance of tooth surfaces.
Ideally, avoid restorations and monitor tooth wear
with study casts, photographs or silicone impressions.
Especially in cases of severe progression consider special care that may involve restorations. Repeat at 6- to
12-month intervals.
RESORPTION OF TEETH
It is defined as a condition or pathologic process resulting
in the loss of dentin, cementum and/or bone.
Types of resorption
1.
Management strategies
2.
Internal resorption
a. Internal replacement
b. Internal inflammatory
i. Transient
ii. Progressive
External resorption
a. External surface resorption
465
Figure 7
3.
4.
b. External inflammatory
c. Ankylosis
d. Replacement resorption
Combined internal and external
Transient apical breakdown.
Figure 8
Figure 9
Flowchart 2
Traumatic injury
Intrapulpal hemorrhage
Internal resorption
Internal resorption
It is also called chronic perforating hyperplasia of pulp,
internal granuloma, odontoclastoma and pink tooth of
Mummery. Resorption of internal type occurs from pulp
space/root canal space and is asymptomatic. It is usually
discovered during routine radiographic evaluation. A pink
colored discoloration is visible if extensive internal resorption occurs in the coronal portion of the tooth, as described
by Mummery. Most cases are found in the anterior region.
Etiological factors are mainly trauma and caries.
Internal resorption can be of two types: internal
replacement (metaplastic) and internal inammatory.
Internal replacement type occurs as a result of lowgrade irritation of the pulp such as chronic irreversible
pulpitis or pulp necrosis. This can occur because of trauma
or application of extreme heat to the tooth. There is concomitant resorption of hard tissue with frequent deposition
of hard tissue resembling bone or cementum. The resultant
External resorption
External resorption is the one which occurs primarily from
the periodontal space affecting the root surface. Four categories of external resorption have been described based
on the clinical and histological manifestations:
Figure 10
Figure 11
Figure 12
Flowchart 3
Process of formation of pulp stone
Trauma
Vascular damage
(thrombosis)
Fibrosis
Mineralization
(nidus formation)
Pulp stone
469
Figure 13
Figure 14
Figure 15
Hypercementosis
Hypercementosis is a non-neoplastic deposition of excessive
cementum which is continuous with the normal radiographic cementum. It is asymptomatic and seen in adulthood. The incidence of hypercementosis increases with
age. It may appear in one tooth or involving multiple teeth
(Figure 13). Hypercementosis is frequently seen in relation
to the premolars.
The cause for hypercementosis can be either due to
local factors like abnormal occlusion, inammation, nonfunctional tooth, tooth repair, root fracture, and cemental
tear or systemic factors like Pagets disease, hyperpituitarism (acromegaly and gigantism), arthritis, calcinosis,
rheumatic fever, thyroid goiter, idiopathic.
Radiographically, thickening or blunting of root is seen.
Enlarged root is surrounded by the radiolucent periodontal
ligament space and intact radiopaque lamina dura (Figures 14
and 15).
Histological section reveals deposition of cellular or
secondary cementum in concentric layers over the existing
thin layer of acellular or primary cementum. This cellular
cementum has also been referred to as osteocementum
owing to its histological resemblance to bone.
Treatment is not required but it may pose problem during
extraction.
470
Cementicles
Small foci of non-neoplastic calcified tissue, which lie in
periodontal ligament along the lateral and apical root
areas are called cementicles. Calcification of epithelial cell
rest of Malassez, aging and cemental tears undergoing
remodeling have all been implicated in the formation of
cementicles. These present as circular laminated structures.
When attached to root, cementum shows roughened globular
outline on the root surface.
SECTION
System Review
VII
18
19
20
21
22
23
473
568
590
605
625
633
471
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CHAPTER
Cardiovascular Disorders
Symptoms Suggestive of Cardiovascular
Disease
Chest Pain
Breathlessness or Dyspnea
Cyanotic
Acyanotic
Platelet Disorders
Thrombocytopathic Disorders
Thrombocytopenic Disorders
Rheumatic Fever
Infective (Bacterial) Endocarditis
Clinical Conditions that Warrant Use of Antibiotic
Prophylaxis
Treatment Needing Antimicrobial Prophylaxis in
Patients at Risk of Infective Endocarditis
Disorders of Coagulation
Heart Failure
Classifications of Heart Failure
Oral Disease Aspects
Hematological Disorders
Red Blood Cell Disorders
Polycythemia
Anemia
Iron Deficiency Anemia
PatersonKelly Syndrome (PlummerVinson Syndrome)
Anemia due to Vitamin B12 and Folic Acid Deficiency
Aplastic Anemia
Glucose-6-phosphate Dehydrogenase Deficiency
Sickle Cell Anemia
Thalassemia
White Blood Cell Disorders
Qualitative Disorders
Respiratory Disorders
Non-neoplastic Disorders
Leukopenia
Reactive Leukocytosis
ChediakHigashi Syndrome
Bleeding Disorders
Classification of Bleeding Disorders
Angina Pectoris
Neoplastic Disorders
Leukemia
Lymphomas
Multiple Myeloma
Hypertension
18
473
Granulomatous Diseases
Malignant Disorders
Renal Disorders
Renal Diseases
Chronic Renal Failure
Uremic stomatitis
Gastrointestinal Disorders
Gastroesophageal Reflux Disease
CARDIOVASCULAR DISORDERS
Cardiovascular disease is quite common, though more frequent and severe in later life, can also affect young individuals. It is one of the leading causes of death in the world.
A thorough knowledge of cardiovascular diseases is necessary because of its implications in dentistry and also the
initial measures taken by the dentists in case of certain
emergency conditions can be lifesaving.
474
Thyroid Gland
Pathophysiology of HypothalamicPituitaryThyroid
Axis
Parathyroid Glands
Hyperparathyroidism
Hypoparathyroidism
Tetany
HypothalamusPituitaryAdrenal Axis
Cushings Syndrome
Endocrinopathic Pigmentation
Adrenal Insufficiency
Aldosteronism
Disorders of Adrenal Medulla
Sex Hormones
Jaundice
Hepatitis
Endocrinal Disorders
Liver Diseases
Neuromuscular Disorders
Bells Palsy
Epilepsy
Parkinsonism
Multiple Sclerosis
Muscular Dystrophy
Oromandibular Dystonia
Myasthenia Gravis
Growth Hormone
Growth Hormone Deficiency
Growth Hormone in ChildrenDwarfism
Adult GH Deficiency
Growth Hormone Excess
Gigantism
Growth Hormone Excess in Adults
Bronchogenic Carcinoma
Pregnancy
Multiple Endocrine Neoplasia Type III
(Multiple Mucosal Neuroma Syndrome)
disease, diabetes mellitus, anemia, dyslipidemia, peripheral vascular disease, orthostatic intolerance and anemia.
Further an accurate record of current medication taken by
the patient, use of tobacco products, alcohol and over-thecounter and recreational drugs should be documented.
SYMPTOMS SUGGESTIVE OF
CARDIOVASCULAR DISEASE
Chest Pain
It is the common presentation of cardiac diseases, though can
be a manifestation of disease of the lungs, musculoskeletal
system or gastrointestinal system. Pain is usually felt behind
the sternum, radiates across the chest and down the arms,
it may also radiate to the back or to the mandible.
Breathlessness or Dyspnea
It is a major symptom of many cardiac disorders, particularly left heart failure. Dyspnea may vary in severity from
an uncomfortable awareness of breathing to a frightening
sensation of fighting for breath.
There are three forms of dyspnea:
Functional capacity
Functional capacity refers to an individuals capacity
to perform a spectrum of common daily tasks.
patients at the time of initial appointment and at all subsequent appointments on all patients with a history of hypertension, cardiovascular disease, diabetes mellitus, thyroid
disorders, adrenal disease, renal dysfunction and significant
use of tobacco, alcohol or coffee. The auscultatory method
of measurement of BP is recommended. In patients older
than 50 years, elevated systolic pressure may predict the
potential for cardiovascular morbidity and mortality.
Pulse rate and rhythm The pulse pressure closely correlates with systolic pressure and is a reliable cofactor that
will provide us with information on cardiovascular disease.
Rate below 60 or above 100 in adults and if associated with
symptoms such as sweating, weakness, dyspnea and chest
pain should be considered as a risk factor in association
with non-cardiac procedures. Further abnormalities in the
normal rhythm of the pulse should provoke a search for
any underlying cardiac diseases.
476
Obesity
High dietary salt intake
Excess alcohol
Smoking
Physical inactivity
Stress/anxiety.
Systolic BP
(mmHg)
Diastolic BP
(mmHg)
Normal
120
80
Pre-hypertension
120139
8090
Stage 1 hypertension
140159
9099
Stage 2 hypertension
160
100
Pharmacologic strategies
Diuretics
Beta-1 adrenergic receptor antagonists
ACE inhibitors
Angiotensin II receptor antagonists
Calcium channel blocking agents
Alpha-1 adrenergic receptor antagonists
Alpha-2 adrenergic receptor antagonists
General management
Drugs
Side effects
Diuretics
Beta blockers
ACE inhibitors
Alpha blockers
Dry mouth
Direct-acting vasodilators
Central-acting agents
Angiotensin II antagonists
Dental considerations
Patients with controlled hypertension can receive dental
care in short appointments.
Anxiety and pain should be avoided since endogenous
epinephrine release in response to pain or fear may induce
dysrhythmias.
Preoperative reassurance and sedation with 10 mg
temazepam or 5 mg diazepam may be helpful.
Raising patients from supine position may cause postural
hypertension and loss of consciousness if patient is using
thiazides, calcium channel blockers.
Aspirating syringe should be used to give local anesthesia to avoid intravenous entry of epinephrine.
Epinephrine in local anesthesia is not contraindicated
unless systolic pressure is over 200 mmHg or diastolic pressure is over 115 mmHg.
Epinephrine containing local anesthesia should not be
given in large doses in patients taking non-selective beta
blockers since interaction may induce hypertension and
cardiovascular complications.
Gingival retraction cords containing epinephrine should
be avoided.
477
Administration of two to three cartridges of local anesthesia with epinephrine 1:100,000 will not cause cardiovascular changes.
Epinephrine should be used with caution in patients taking tricyclic antidepressants and diuretics since acute hypertensive changes and dysrhythmias, respectively may occur.
Angina Pectoris
It is the most common clinical presentation of ischemic
heart disease and is infrequent before the age of 40 years.
478
MYOCARDIAL INFARCTION
It is a severe form of coronary artery disease. An anginal
pain lasting longer than 30 minutes is considered to be a
myocardial infarction. The pain may be accompanied by
nausea and vomiting, tachycardia, grossly irregular pulse,
pallor and difficulty in breathing, sweating, and restlessness.
About 10% have painless infarctions.
Diagnosis
The diagnosis of acute myocardial infarction is based on the
presence of two of the following three criteria: (1) signs and
symptoms compatible with myocardial ischemia, (2) typical
ECG changesST segment elevation at the end of the PR
segment, (3) measurement of creatinine kinase (CK) and
myocardial-bound CK (CK-MB).
Elevations in the serum troponin T and troponin 1 levels,
which are sensitive markers for myocardial injury, have
also been used to test for acute myocardial infarction.
Therapeutic goals and pharmacologic strategies for coronary
heart disease
Therapeutic goals
Pharmacological strategies
Inhibit progression of
atherosclerosis
Lipid-lowering agents
HMG-Co-A reductase inhibitors
Improve circulation in
coronary arteries
Reduce workload
Antithrombotic agents
Prevent coagulation
Anticoagulants
2.
3.
4.
5.
6.
Cyanotic
Transposition of great vessels, tetralogy of Fallot, Eisenmengers syndrome. Cyanotic defects are lethal.
Acyanotic
Atrial and ventricular septal defects, patent ductus arteriosus, coarctation of aorta, pulmonary stenosis, mitral valve
prolapse, aortic stenosis.
Causes
Idiopathic
Acquired (congenital rubella, maternal drug misuse).
Clinical features
Dental aspects
1.
Antimicrobial prophylaxis
Bleeding tendencies due to platelet dysfunction and
excessive fibrinolytic activity
Dental bacteria may cause cerebral abscess
Aspiration during local anesthetic procedure is a must
due to epinephrine in local anesthesia
Gingival retraction cord containing epinephrine should
be avoided.
Oral manifestations associated with congenital
heart diseases
RHEUMATIC FEVER
Rheumatic fever is the most common cause of cardiac
valve disorders. The mitral valve is affected more frequently. It results from an altered immunologic response to
a group A beta hemolytic streptococcal pharyngitis, leading to formation of Aschoffs nodules in the myocardium
which develops 13 weeks after the streptococcal infection.
Though, only 3% of cases of beta hemolytic pharyngitis
resulting in rheumatic disease, there is no way to forecast
which individuals will have rheumatic heart disease (RHD).
Common manifestations include new onset murmur,
carditis, polyarthritis, chorea, erythema marginatum, fever
and subcutaneous nodules (modied Jones criteria). The
beta streptococcal infection is conrmed by increased
serum antistreptolysin O (ASO) antibody titer or positive
throat culture. RHD resulting in valvular injury is conrmed by echocardiography for any patient with a history
of rheumatic fever, it is imperative to determine whether
the infection resulted in RHD.
Dental considerations
480
40%
30%
10%
10%
10%
Neurosurgical shunts
Shunts are placed in patients with hydrocephaly to help
in the drainage of cerebrospinal fluid. The ventriculoatrial
shunt allows drainage of the CSF from the lateral ventricles
to the venous circulation. The ventriculo-peritoneal shunt
helps drain the CSF into the abdominal cavity.
Indwelling catheters and stents
Antibiotic prophylaxis is indicated only in instances where
the catheters are on the right side of the heart. Only the first
2 weeks are critical and indicated for antibiotic prophylaxis
when stents are placed in cardiac patients. The risks of developing a superinfection are very minimal after the first few
weeks as an epithelial layer develops over these stents.
Patients with renal diseases undergoing
hemodialysis
Patients receiving peritoneal dialysis do not require antibiotic prophylaxis. However, patients who have an arteriovenous shunt (made up of autogenous tissue or a silastic tube)
implanted for dialysis require antibiotic coverage during
dental procedures, as these shunts are vulnerable to infection.
Extractions
Subgingival procedures (probing, cord placement or
scaling)
Oral/periodontal/implant surgery or raising mucogingival flaps
Endodontics beyond root apex
Sialography
Intra-ligamental local anesthesia
Rubber dam, matrix or wedge placement.
Dental radiography
Endodontics not beyond apex
Exfoliation of primary teeth
Impression taking
Non-surgical procedures that do not induce bleeding
Abscess incision and drainage
Suture removal
Orthodontic band removal.
Oral medications
481
Patient category
Non-oral medications
Chlorhexidine hydrochloride and povidoneiodine mouthrinses may reduce the incidence and magnitude of bacteremia before dental treatment.
Moreover, patients should be encouraged to maintain
meticulous oral hygiene.
It has been suggested by some authors that in patients
with poor oral health, antibiotic coverage should be given
for all dental procedures.
Stage D: Patients with end stage disease requiring specialized treatment such as mechanical circulatory support, continuous ionotropic infusion or cardiac
transplantation.
2. The New York Heart Association
Class one: Asymptomatic patients
Class two: Patient symptomatic with moderate exertion
Class three: Patient symptomatic with mild exertion
Class four: Patient symptomatic at rest.
Clinical manifestations
Left heart failure manifests as tachycardia, fatigue on
exertion, dyspnea on mild exercise, intolerance to cold,
paroxysmal nocturnal dyspnea and nocturnal cough.
Right heart failure is characterized by fatigue, jugular
venous distention, fullness in the abdomen, with an enlarged
liver and tenderness in the upper right quadrant. In advance
cases ascites and pitting edema of lower extremities is seen.
Management
HEART FAILURE
Heart failure is a clinical syndrome defined as chronic
inadequate contraction of the heart muscle, resulting in
insufficient cardiac output. It is basically an inability of the
heart to pump blood at a rate required by the body tissues.
It occurs frequently in the elderly population. It is a manifestation of one or more underlying conditions including:
Reduction in physical activities, stress reduction, fluid restriction, dietary sodium restriction, weight loss, subcutaneous
heparin administration, supplemental portal oxygen, medications prescribed include diuretics, cardiac glycosides,
vasodilators, ACE inhibitors and sympathomimetic drugs.
Dental considerations
Special considerations
Appointment timing Most sudden cardiac arrests may
come during peak endogenous epinephrine levels (08:00
11:00 hours) and so appointments are best for late morning
or early afternoon.
Preventive strategies Dental management plans for
patients with cardiovascular diseases should include appropriate preventive strategies.
Toothbrush The use of electromechanical brushes have
been shown to be more effective than conventional brushes
in reducing plaque and gingivitis and hence should be recommended in all patients with cardiovascular disease.
Topical rinses and fluorides The use of topical agents,
such as chlorhexidine gluconate is useful to combat gingivitis and other periodontal pathosis that result from plaque
accumulation. For patients with xerostomia and a high
incidence of dental caries, preventive modalities such as
dietary analysis and counseling, and prophylaxis combined
with home fluoride use should be implemented. A topical
fluoride, 1.1% sodium fluoride in the form of a brush-on
gel, may be preferred to a topical solution.
Sialagogues Xerostomic patients, in whom the salivary
glands respond to stimulation benefit from dietary measures
such as carrots, celery, or from chewing xylitol containing
gums. Drugs such as pilocarpine hydrochloride 2.55 mg in
26 increments per day and cevimeline hydrochloride can be
used to treat drug-induced xerostomia. However, in patients
with no residual salivary gland function, salivary substitutes,
oral moisturizer, and artificial saliva provide some relief.
Local hemostatic agents They are used for surface dressing for wounds and over extraction sockets. They arrest
bleeding by creating a mechanical matrix which facilitates
blood clotting when the absorbable hemostatic agent is
applied directly to the hemorrhagic site. Examples include
absorbable gelatin sponge, microfibrillar collagen hemostat and oxidized regenerated cellulose.
Adverse drug events Dentists must have an awareness
of the potential for adverse drug events since patients with
cardiovascular diseases are frequently treated with multiple drugs. Hence, a rational approach should be developed
toward the use of pharmacotherapeutic agents in the management of odontogenic problems.
that supports the association between periodontal infections, atherosclerosis and vascular disease. They further
recommend that irrespective of whether there is an association between periodontal diseases and cardiovascular
lesions or not, periodontal treatment must be recommended
on the basis of the value of its benefits for the oral health of
patients, recognizing that patients are not healthy without
good oral health.
Lichenoid stomatitis Cardiovascular drugs such as diuretics, beta-1 blockers, ACE inhibitors may cause development
of lichenoid lesions. Lichenoid lesions are indistinguishable
from oral lichen planus. The diagnosis is confirmed when
condition resolves after the offending drug is discontinued.
Xerostomia Cardiovascular drugs with xerostomic side
effects include diuretics, beta-1 blockers and centrally acting sympathetic agonists. Xerostomia is usually caused due
to the drugs parasympatholytic or antimuscarinic effects.
Xerostomia can lead to complications such as high incidence in dental caries. Moreover, it can contribute to difficulties in mastication, swallowing, speech. The dryness
of the oral mucosa if persistent can lead to atrophy and
susceptibility to candidiasis and other superinfections.
Gingival hyperplasia Calcium channel blockers such as
nifedipine can cause gingival enlargement primarily affecting the labial or facial interdental papilla and it is firm and
painless. However, it is usually associated with erythema
and edema resulting in pain, bleeding, and difficulty in
mastication.
Patients on antithrombotic agents
An increasing number of cardiovascular patients are prescribed antithrombotic, anticoagulant, and thrombolytic
agents and hence special precautions need to be taken in
such patients.
Aspirin is one of the commonly prescribed antithrombotic
agents. It irreversibly acetylates cyclo-oxygenase resulting
in inhibition of thromboxane A2 mediated platelet aggregation. However, it is relatively a weak antithrombotic agent,
since the action of other mediators of platelet aggregation
are not inhibited, hence the effect of aspirin therapy on
intra- and post-operative surgical and periodontal procedures are minimal.
If the patient is also taking oral anticoagulant, signicant synergism may mandate modications of drug regimen. Measurement of patients bleeding time can be helpful
to determine the degree of antithrombotic effect and its
impact on invasive dental procedures.
Considerations for patients on anticoagulant agents
For any patient taking anticoagulant the dentist and physician must assess the risk for altered bleeding after an invasive dental procedure. Assessment includes magnitude of
invasive procedure, patients bleeding history, patients
483
bleeding prole which includes prothrombin time (PT), partial thromboplastin time (PTT), and international normalized ratio (INR).
Oral anticoagulants are commonly prescribed for arterial or venous thromboembolism. This therapy is usually
monitored by prothrombin time which is converted to INR.
For most clinical indications, a moderateintensity anticoagulant effect with a target INR of 2.03.0 and PT of 1.5
2.5 is appropriate.
Warfarin is the commonly prescribed oral anticoagulant.
Most patients can undergo minor oral surgical procedures
such as extraction and alveolectomies without alteration of
their warfarin regimen. However, to prevent serious bleeding
the preoperative assessment of the patients level of anticoagulation is necessary. Warfarin has plasma half-life of
3642 hours, hence any change in dosage requires at least
2 days to be reected in INR value. Once an acceptable range
is achieved local anesthesia (LA) can be administered with
caution to minimize hematoma formation and using meticulous local measures such as minimal trauma, application
of gelfoam and placement of sutures to ensure hemostasis.
Heparin therapy
For a patient on heparin therapy requiring an oral surgical
procedure, the drug should be discontinued approximately
4 hours before the procedure. Surgery is performed using
local anesthesia, atraumatic surgical technique, application
of local hemostatic agents and careful suturing. Postoperatively heparin therapy may be re-instituted the same
day if there is no active bleeding.
Local anesthesia
The use of local anesthetic agents with vasoconstrictors in
patients with cardiovascular disease is controversial. The two
most commonly used vasoconstrictors are epinephrine and
levonordefrin. Patients receiving LA without vasoconstrictor
have impaired pain control compared to those receiving LA
with epinephrine. Hence, patients with cardiovascular disease may be at greater risk of experiencing massive endogenous epinephrine release secondary to poor LA than they
are from the small amount of vasoconstrictor used in LA.
Some studies have found that inltration anesthesia
containing 3.6 ml of lignocaine with 180,000 epinephrine
can be given safely in CVS patients who have an exercise
capacity of more than 4 MET. Most studies have shown no
signicant changes in BP or heart rate in patients with mild
to moderate cardiovascular system (CVS) disease after dental injection of 1.85.4 ml of 2% of lignocaine with 1100,000
epinephrine. Hence, it is recommended that patients with
mild to moderate CVS disease receive the smallest amount
of LA needed to provide anesthesia with an aspirating
syringe. Moreover, use of conscious sedation to decrease
stress to minimize endogenous release of epinephrine may
help in ensuring hemodynamic stability than avoiding small
484
Electric toothbrushes
Electronic apex locators
Piezoelectric ultrasonic scalers.
Diathermy units
Electronic dental analgesia units
Electrosurgical units
Ferromagnetic seaters
Lithotripsy units
MRI (magnetic resonance imaging) units
TENS (transcutaneous electric nerve stimulation) units
Ultrasonic instrument baths.
HEMATOLOGICAL DISORDERS
Hematological diseases include abnormalities involving
RBCs, WBCs and platelets. As platelet abnormalities lead
Disorder
WBCs
ChediakHigashi syndrome
Enzyme deficiencies
e.g. G-6-P dehydrogenase deficiency, pyruvate
kinase deficiency
RBCs
Hemoglobinopathies
e.g. Sickle cell anemia, thalassemia
Abnormal shape
e.g. Hereditary spherocytosis, hereditary
elliptocytosis
Quantitative defects
Cells affected
Disorder
Increase in number
e.g. Leukocytosis, leukemia, lymphoma
WBCs
RBCs
Decrease in number
e.g. Granulocytopenia, agranulocytosis,
cyclic neutropenia
POLYCYTHEMIA
Polycythemia is an abnormal increase in the erythrocyte
count. Polycythemia is of three types: (1) primary proliferative polycythemia (polycythemia rubra vera), (2) secondary polycythemia, and (3) apparent polycythemia.
Polycythemia rubra vera (PRV) is an idiopathic myeloproliferative disorder seen after 5th decade of life. PRV is
not only characterized by proliferation of erythrocytes, but
also granulocytes and platelets. While secondary polycythemia results from increased erythropoietin concentration,
apparent polycythemia results from decrease in plasma
uids rather than any increase in RBC concentration. RBC
levels may reach up to 612 million cells/mm3 leading to
increased blood viscosity and thrombosis. Cyanosis of the
face and extremities (due to deoxygenated blood), and
hemorrhagic tendency may be seen in the later stages of
the disease.
Oral manifestations
Polycythemia rubra vera will exhibit oral manifestations
such as petechiae, purpura, spontaneous bleeding of the
gingiva owing to hemorrhagic tendency and purple red discoloration on tongue, mucosa owing to cyanosis. Leukemic
manifestations may also be seen if PRV progresses to acute
myeloid leukemia, due to the proliferation of leukocytes.
Secondary polycythemia also exhibits petechiae, ecchymosis and purple red discoloration of tongue and mucosa.
Apparent polycythemia, which does not affect the quantity
of RBC, does not exhibit any appreciable oral changes.
Dental considerations
Bleeding tendency during dental treatment procedures warrants adequate hemostasis measures. Cytotoxic chemotherapy
should be administered prior to dental treatment procedures
if hemoglobin levels are not controlled.
Increase in number
e.g. Polycythemia
Treatment
Decrease in number
e.g. Anemia (sickle cell anemia, thalassemia and
anemia due to deficiency of vitamin B12, folic acid
and iron)
ANEMIA
Anemia is defined as a reduction in the oxygen carrying
capacity of the blood. It is not a disease but rather a symptom complex due to either reduction in number of circulating RBCs or due to an abnormality in the hemoglobin
contained within RBCs. RBCs with a normal range of
56 million cells/mm3 may dwindle due to loss of blood or
due to decreased production or increased destruction.
485
Extracorpuscular factors causing hemolysis, e.g. autoimmunity, infections, liver disease and Rh factor
incompatibility.
Intracorpuscular factors causing hemolysis, e.g. hereditary spherocytosis, G6PD deficiency, sickle cell anemia,
thalassemia and paroxysmal nocturnal hemoglobinuria.
Diagnosis
Causes
Inadequate ingestion/increased requirements Infants,
children and in pregnancy.
Decreased absorption or utilization
malabsorption syndrome.
Partial gastrectomy,
Blood loss
486
Figure 1
Treatment
Since oral changes would appear to precede many of the
systemic indicators of vitamin B12 deficiency, it is hoped
that the recognition of these will lead to early diagnosis
and institution of therapy. Regardless of the etiology of
vitamin B12 deficiency, high dose oral supplementation
(1,0002,000 mcg daily for 2 weeks), followed by 1,000 mcg
daily for maintenance is currently recommended. Historically pernicious anemia was treated with intramuscular
vitamin B12 supplementation. However, several studies have
demonstrated that high doses of oral vitamin B12 are just
as effective as, and are better tolerated than intramuscular
cyanocobalamin in patients with vitamin B12 malabsorption.
Management for folic acid deficiency consists of administration of oral folic acid (5 mg/day), which is given for
a period of 4 months. The differentiation of vitamin B12
deficiency and folic acid deficiency is crucial as folic acid
supplements may correct the anemia but will not stop the
neurological manifestations.
Aplastic Anemia
Aplastic anemia is a rare, potentially life-threatening failure of hemopoiesis characterized by pancytopenia and bone
marrow aplasia. Although most cases are acquired (drugs,
viruses, chemicals, toxins and radiation), some are inherited. The pathophysiology of acquired aplastic anemia is
immune mediated in most cases; autoreactive lymphocytes mediate the destruction of hemopoietic stem cells.
Fanconis anemia is an inherited aplastic anemia that
manifests in early childhood. The term anemia in aplastic
anemia is a misnomer, since all the three cellular elements
of the bone marrow are often involved (pancytopenia), i.e.
granulocytes, erythrocytes and platelets. Clinical manifestations are proportional to the peripheral blood cytopenias
and include dyspnea, fatigue and pallor (effects of anemia),
petechiae and easy bruising (effects of thrombocytopenia)
and susceptibility to infections (effects of neutropenia).
Oral manifestations
Oral manifestations like gingival bleeding, pallor, petechiae, ecchymosis; neutropenic ulcers and delayed healing
are observed in patients with aplastic anemia (effects of anemia, thrombocytopenia and neutropenia). Petechial hemorrhages were the most common intraoral finding in
patients with aplastic anemia. These petechiae might be due
to minor trauma from normal deglutition and mastication
rather than due to thrombocytopenia. Rapidly progressing
severe periodontitis, which is a feature of several quantitative and qualitative neutrophil defects like cyclic neutropenia, agranulocytosis and leukocyte adhesion deficiency
(LAD), is also evident in aplastic anemia. Bacterial sepsis
and fungal infections represent the most frequent cause of
death in this condition.
Dental considerations
Thorough oral examination should be carried out in a
patient with aplastic anemia as bleeding tendency and infections pose a serious problem. Local hemostatic measures
such as pressure pack application and systemic antifibrinolytic agents such as aminocaproic acid and tranexamic
acid should be considered. Tranexamic acid is given in
a dosage of 20 mg/kg body weight 4 times a day starting
24 hours before oral procedures and continuing for 34 days
afterwards. Chlorhexidine mouthrinses will reduce the microbial load in the oral cavity. Antibiotic prophylaxis, typically
with amoxicillin or clindamycin, and platelet transfusion
of thrombocytopenic patients, when necessary, should be
adequate to prevent serious sequelae for all dental procedures. However, intramuscular injections and nerve block
anesthesias are to be avoided because of risk of thrombocytopenia and bleeding tendency.
Management
Cause should be identified and removed. Patients with
asymptomatic cytopenias probably need no treatment.
Treatment for patients with severe cytopenias includes bone
marrow transplantation, immunosuppressive therapy and
high dose cyclophosphamide without transplantation of
bone marrow.
Diagnosis
A complete blood count, leukocyte differential, reticulocyte count, and a bone marrow aspirate and biopsy can
establish the diagnosis. Peripheral blood flow cytometry can
be done to rule out paroxysmal nocturnal hemoglobinuria.
Patients younger than 40 years should be screened for
Fanconis anemia by the use of clastogenic agents, diepoxybutane or mitomycin, which test for increased chromosomal breakage seen with this disorder. A family history of
cytopenias should raise suspicion of an inherited disorder
even when no physical abnormalities are present.
488
palpitations, weakness and jaundice may be seen. Oral manifestations common to other anemias may be encountered.
Dental considerations
Avoidance of triggering drugs such as dapsone and sulfasalazine. Blood transfusion can be considered prior to
dental treatment if anemia is very severe.
3.
Treatment
Oral manifestations
Diagnosis
Treatment
Clinical manifestations
Manifestations of sickle cell anemia are divided into three
categories.
1.
2.
Figure 2
Oral manifestations
Diagnosis
THALASSEMIA
Thalassemias are a group of congenital hemoglobinopathies characterized by a reduced rate of production of one
or more of globin chains (alpha or beta) in the hemoglobin
molecule. Alpha chain is reduced or deficient in alpha
thalassemia and beta chain is reduced or deficient in beta
thalassemia. Beta chains are commonly involved, when
compared to alpha chains. If affected individuals are
heterozygous for beta chains, it is called as beta thalassemia trait or beta thalassemia minor. If affected individuals
are homozygous for beta chains, then it is called as beta
thalassemia major or Cooleys anemia. In thalassemia,
imbalance in globin chain production leads to hemolysis
thus resulting in microcytic, hypochromic type of anemia.
The hemoglobin levels can even reach up to 23 g/dl.
While, beta thalassemias are most frequently found in
Mediterranean and black populations, alpha thalassemias
are found more frequently in South East Asia, Middle East
and Mediterranean populations.
Clinical manifestations
Systemic manifestations are mildly similar to sickle cell
anemia, which are indicative of severe anemia. Growth and
development of the child is retarded with ashen gray skin
color due to pallor and jaundice. Patients may also present
with cardiomegaly, splenomegaly and hepatomegaly.
490
Oral manifestations
Classification
Qualitative disorders
ChediakHigashi syndrome
NON-NEOPLASTIC DISORDERS
Non-neoplastic disorders
Leukocytosis
Leukopenia
Neoplastic disorders
Leukemia
Lymphoma
Multiple myeloma.
QUALITATIVE DISORDERS
Since leukocytes play a central role in host defense, it is
not surprising that defects in leukocyte function, both
genetic and acquired, lead to increased vulnerability to
infections. These qualitative defects can be:
Leukopenia
A decrease in the peripheral WBC count may occur because
of decreased numbers of any of the specific types of leukocytes, but most often it involves the neutrophils (neutropenia). A reduction in the number of granulocytes in
blood is known as neutropenia or sometimes, when severe
as agranulocytosis. Affected persons are extremely susceptible to infections, which may be severe enough to
cause death. Patients may also exhibit fever, weakness and
marked fatigability.
Causes for neutropenia
Inadequate or ineffective granulopoiesis
Aplastic anemia
Leukemia
Due to cancer chemotherapeutic agents.
Idiopathic
Drugs such as aminopyrine
Splenomegaly
Overwhelming bacterial or fungal infections.
ChediakHigashi Syndrome
It is a rare autosomal recessive disorder characterized by
abnormal granules in the granulocytes. This abnormality
results in neutrophils with decreased chemotactic and bactericidal ability. Patients develop severe neutropenia as a
result of ineffective granulopoiesis and most die in childhood from infections or advanced lymphoproliferative
syndrome. Patients exhibit neuropathy, hypopigmentation,
recurrent bacterial infections and hepatosplenomegaly.
Oral manifestations
Ulcerations and necrotizing lesions may be seen on the
gingiva, floor of the mouth, buccal mucosa, pharynx or
other sites within the oral cavity (agranulocytic angina).
Ulcers lack the surrounding inflammation and are characterized by necrosis and foul smell. All of these lesions often
show massive growth of microorganisms, with a relatively
poor leukocyte response (minimal swelling and pus).
491
Treatment
Cause must be identified and all drugs should be discontinued. Infections must be prevented or treated, if present.
Current treatment efforts also include administration of
recombinant hematopoietic growth factors, such as granulocyte CSF, which stimulate neutrophil production by the
bone marrow.
Dental considerations
Oral infections in patients with severe neutropenia should
be considered potentially life-threatening because they can
lead to bacteremia and septicemia. Ulcers can be treated with
topical anesthetics and antiseptic mouthrinses. In severe
pulpal and periodontal infections, broad-spectrum antibiotics can be advised until culture reports are available.
Cyclic neutropenia
It is a rare disorder that occurs secondary to a periodic
failure of the stem cells in the bone marrow. It is characterized by transient severe neutropenia that occurs approximately every 21 days. Neutrophil count is at its bare
minimum for a period of 37 days during which the clinical
manifestations pertaining to neutropenia are observed.
The most common signs are fever, stomatitis, pharyngitis and skin abscesses. Severity of infection is directly proportional to the severity of neutropenia. Treatment and
dental considerations are similar to that of neutropenia.
Reactive Leukocytosis
An increase in the number of WBCs is a common reaction
in the variety of inflammatory states caused by microbial
and non-microbial stimuli. Leukocytosis is relatively nonspecific and can be classified on the basis of particular
white cell series affected. In response to increased demand,
increased number of immature neutrophils called bands
enters the circulation, a process called a left shift. This is
called as leukemoid reaction, which is often secondary to
viral infections. This reaction can be distinguished from
acute leukemia, as there is an orderly maturation and
proliferation of all normal myeloid elements in the bone
marrow.
Causes of leukocytosis
Physiologic
Exercise
Pregnancy
Stress
Pathologic
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Neutrophilic leukocytosis
Acute bacterial infections
Burns
Eosinophilic leukocytosis
Allergic diseases
Parasitic infestations
Drug reactions
Certain malignancies
Basophilic leukocytosis
Malignancies
Monocytosis
Chronic bacterial infections such as tuberculosis,
systemic lupus erythematosus, etc.
Lymphocytosis
Infections due to hepatitis A, cytomegalovirus and
EpsteinBarr virus.
Oral manifestations are seen as a consequence of underlying disease process rather than the elevated leukocyte levels.
For example, in infectious mononucleosis, there is lymphocytosis and patients complain of sore throat, petechiae
and fever. Patients exhibiting these clinical signs must be
evaluated and investigated as these clinical findings can
be confused with leukemia.
NEOPLASTIC DISORDERS
Leukemia
Leukemia is a heterogeneous group of hematological disorders that arise from a hematopoietic stem cell characterized by a disordered differentiation and proliferation of
neoplastic cells. This neoplastic proliferation in marrow
results in diminished production of normal erythrocytes,
granulocytes and platelets in leukemia. These neoplastic
cells ultimately infiltrate various organs like spleen, CNS,
lymph nodes, skin and gingiva. Leukemia is categorized
according to its clinical behavior: acute or chronic; and
histogenic origin: lymphocytic or myelocytic. The classification is called as FAB (French, American, and British) and
is widely accepted. The etiology is unknown but genetic
factors, irradiation, viruses, chemicals such as benzene and
some drugs have been implicated. All types of leukemia
are more common in males compared to females.
Acute Leukemia
About 50% of all leukemias are in the acute form. Acute
leukemias are divided into two major groups: acute lymphocytic leukemia (ALL) and acute monocytic leukemia
(AML). While, ALL is more commonly seen in children,
AML is frequently seen in the adults. ALL is frequently
derived from B-lymphocytes or their precursors. The acute
leukemias have increased number of immature cells called
blasts in the peripheral circulation. As blasts accumulate
in the marrow, they suppress normal hematopoietic stem
cells.
Clinical features
The major manifestations of acute leukemia result from
the paucity of normal RBCs, WBCs and platelets. They
have the following characteristics:
Chronic Leukemia
Onset of chronic leukemia is insidious with the course
(untreated) of the disease running up to 26 years. So
Laboratory diagnosis
Treatment
Control of chronic phase is often successful when compared to blastic phase. Imatinib, a tyrosine kinase inhibitor
is the first line of treatment in the chronic phase. As the
disease enters blastic phase, other treatment options have
to be explored.
Allogenic hemopoietic stem cell transplantation can cure
approximately 70% of chronic phase CML patients but with
risk of complications and death due to graft-versus-host
disease (GVHD) and opportunistic infections.
Clinical features
Figure 3
(result of radiotherapy for cranial involvement), and lichenoid lesions, desquamative gingivitis due to graft-versus-host
reaction. The oral mucosal cells due to their high mitotic
index are frequently compromised, due to their susceptibility to chemotherapeutic agents, predisposing the patients to
mucositis and xerostomia. The dental anomalies encountered are microdontia, dental agenesis and arrested root
development.
Radiographic findings Most children with leukemia had
detectable radiographic changes of the jaws. These changes
include loss of lamina dura, displacement of teeth, loss of
the crypt outline around unerupted teeth, widened periodontal ligament and loss of cancellous bone trabeculation. The latter leads to an appearance of generalized
rarefaction. There are other reports that mention generalized bone loss, increased mobility and protrusion of teeth in
leukemic patients. Periodontal destruction, which is severe,
may have been due to infiltration of leukemic cells into alveolar bone.
The patients of chronic leukemia also exhibit oral hemorrhage, petechiae, ulcerations and gingival swellings.
Dental considerations
Dental treatment should only be carried out after consultation with the physician, as various aspects of management
and the probable life expectation may affect it. Preoperative
precautions should include screening for hepatitis B and
HIV. Preventive oral healthcare is essential and where
indicated, conservative dental treatment may be possible.
Management of chronic dental infections in patients with
hematologic malignancies ideally should be based on data
that correlates examination findings with outcomes of
treatment. One result of compromised hematologic status
among dental patients with hematologic malignancy, idiopathic or drug-induced blood dyscrasias and sickle cell
anemia is that certain clinically relevant laboratory values
have been proposed as important in management protocols.
When oral surgical procedures are anticipated, a platelet
count of at least 50 109/l and absolute neutrophil count of
at least 0.5 109/l are sought by the provider to comfortably
assure effective hemostasis and reduce the risk of postoperative bacterial infection. But, in smokers, dental extractions should not be used as a means of controlling chronic
asymptomatic periodontal and pulpal diseases, as these
patients are prone to fungal infections. Oral ulcerations in
leukemic patients should be managed by topical antibacterials along with analgesic and anesthetic rinses. Removing
irritants, applying local pressure and hemostatic agents
such as absorbable gelatin or collagen sponges should be
used to manage bleeding tendency. If the patient does not
respond, then platelet transfusions might be warranted.
Lymphomas
Non-Hodgkins Lymphoma
Hodgkins Disease
Hodgkins disease (HD) is a disorder of unknown etiology
involving primarily the lymphoid tissue. It arises almost in
a single node or chain of nodes and spreads characteristically to the anatomically contiguous nodes. There are two
peaks of incidence, one in early adulthood and one around
fifth decade of life, and males have increased incidence when
compared to females. Four subtypes are recognized based
on histologic features (Rye system): lymphocyte predominance, nodular sclerosis, mixed cellularity and lymphocyte depletion. The lymphocyte predominance has the best
prognosis and lymphocyte depletion, the worst. HD is also
staged into four stages based on the clinical features (Ann
Arbor classification). Prognosis becomes worse as the stage
increases. Histologically, HD shows multinucleated Reed
Sternberg cells.
Treatment
The outlook after aggressive radiotherapy and chemotherapy for patients with this disease, including those with
disseminated disease, is generally very good. With current
modalities of therapy, the histologic picture has very little
impact on the prognosis; instead clinical stage appears to
be an important prognostic indication. However, long-term
survivors of combined chemotherapyradiotherapy protocols are at much higher risk of developing acute leukemia.
Currently, combination chemotherapy of doxorubicin, bleomycin, vincristine and dacarbazine is used for most patients.
Clinical manifestations
The most common presentation of NHL is a painless persistent enlargement of lymph nodes (Figure 4). Unlike HD,
extranodal lesions can occur in GI tract, Waldeyers ring,
spleen, skin and bone marrow. Signs and symptoms include
fever of unknown cause, weight loss, malaise, sweating, and
abdominal or chest pain. The clinical differences between
HD and NHL are outlined below.
Clinical differences between HD and NHL
HD
NHL
Non-contiguous spread
PelEbstein fever
Non-specific fever
Clinical manifestations
Hodgkins disease usually presents as a painless enlargement of the lymph nodes (rubbery). Extranodal involvement is rare. The signs and symptoms include fever, weight
loss, sweating, pruritis and fatigue. Characteristic clinical
Treatment
Non-Hodgkins lymphoma is radiosensitive and the treatment may include a combination of radiotherapy and chemotherapy. High dose chemotherapy with autologous stem
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Figure 4
Burkitts Lymphoma
Burkitts lymphoma (BL) is a B-cell neoplasm endemic in
some parts of Africa and sporadic in other areas, including
the United States. The tumor is the human cancer most
closely linked with a virus (EpsteinBarr virus). The tumor
is considered as the fastest growing human tumor as it
doubles in 13 days.
Clinical features
Both the endemic and non-African cases mainly affect
children or young adults. In both the forms, the disease
rarely arises in lymph nodes. In African patients, involvement of maxilla or mandible is the common mode of presentation, whereas abdominal tumors are more common
in North America.
Treatment
Majority of patients can be cured with aggressive chemotherapy. BL was found to be responsive to cyclophosphamide, methotrexate, vincristine and cytarabine.
Oral manifestations and dental considerations
Painlessly enlarged cervical lymph nodes are the initial
complaint in many of the cases. Suspicion of lymphoma
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Multiple Myeloma
Multiple myeloma (MM) is a relatively uncommon malignant neoplasm of the plasma cells (which arise from the
B-lymphocytes) that often appears to have a multicentric
origin within bones. The cause of the condition is unknown,
although sometimes a plasmacytoma may evolve into
multiple myeloma. This disease makes up about 1% of all
malignancies and 1015% of hematologic malignancies.
The abnormal plasma cells in the bone marrow proliferate
from a single malignant precursor (monoclonal) and produce immunoglobulins that are not normal or functional.
This monoclonal nature of plasma cells can be differentiated from polyclonal nature of plasma cells seen in chronic
inflammation.
BLEEDING DISORDERS
The integrity of circulation is maintained by blood flowing
through intact vessels lined by endothelial cells. Efficient
mechanisms have evolved to maintain the circulation as
a transport system, which both prevent blood loss from
a damaged vessel by securing hemostasis, and also prevent the cessation of flow due to thrombosis. Hemostasis
depends upon interactions between vessel wall, platelets
and clotting factors. There are two phases of hemostasis:
primary and secondary. In the initial primary phase, the
damaged vessel constricts and platelets aggregate at the
site of damage to form a plug to arrest hemorrhage within
a few minutes. This is followed by activation of the coagulation system with secondary deposition of a fibrin mesh to
secure the platelet plug. These two phases are interlinked;
damaged endothelium and the subendothelial matrix activates platelets, which then provide the optimal surface for
the binding of the clotting factors and generation of insoluble fibrin. Fibrinolysis is the major means of disposing of
fibrin after its hemostatic function has been fulfilled, and
it can be considered the rate limiting step in clotting. The
protagonists of the hemostasis are as follows:
Vessel wall
After tissue injury, serotonin and other vasoactive substances are released, which mediate the immediate reflex
vasoconstriction. This alone might be sufficient to arrest
bleeding from small vessels.
Platelets
If the defect in the blood vessel is very smalland many
small holes develop in the vasculature each dayit is often
sealed by a platelet plug rather than by a blood clot.
Platelets are minute round or oval disks, which circulate in
the blood. When these circulating platelets are exposed to
damaged vascular surfaces (in the presence of normal
vWF and endothelial cells), they are activated to produce
physical and chemical changes. These changes produce an
environment that causes the platelet to aggregate and
release ADP and platelet factors, which cause further platelet
aggregation and promotes clotting mechanism.
Clotting mechanism
Coagulation involves a series of enzymatic reactions leading to conversion of soluble plasma fibrinogen to fibrin clot.
This process involves multiple proteins, many of which are
synthesized by liver (fibrinogen, prothrombin, factors V,
VII, IX, X, XI and XII) and many are vitamin K dependent
(factors II, VII, IX and X). The scheme of reaction is a bioamplification in which, the precursor is altered to an active
form, which, in turn, activates the next precursor in the
sequence. Beginning with an undetectable biochemical
497
reaction, the coagulation mechanism results in the formation of insoluble fibrin. The clotting of blood also requires
calcium and phospholipids.
The mechanism initially proceeds by two separate pathways (intrinsic and extrinsic) that converge by activating
a third (common) pathway. The extrinsic pathway is initiated by release of tissue thromboplastin and does not
require contact activation. Tissue thromboplastin binds to
factor VII in the presence of calcium, and this complex is
capable of activating factor X to Xa. The intrinsic pathway
is initiated when factor XII is activated by surface contact
(e.g. with collagen or subendothelium), and it involves the
interaction of factors XII and XI. The activated factor XI
with the help of divalent cation (calcium) and phospholipids
converts factor X to Xa. The factor Xa heralds the initiation of common pathway. Factor Xa converts prothrombin
to thrombin, which further converts brinogen, a soluble
plasma protein, to insoluble brin. Finally, brin polymerizes to form a gel, stabilizing the platelet plug. The clot thus
formed has to be broken down after or else it may lead to
thrombosis. This critical function is carried out by TPA
(tissue plasminogen activator), which converts plasminogen to plasmin. The plasmin thus formed degrades brinogen and brin into brin degradation products (FDPs).
This phase is the brinolytic phase, which forms the rate
limiting step of hemostasis.
Laboratory investigations
A number of procedures that are performed in dentistry
may cause bleeding. Under normal circumstances, these
procedures can be performed with little risk to the patient;
however, the patient whose ability to control bleeding has
been altered by drugs or disease may be in grave danger
unless the problem is identified before undertaking any
dental procedure. An alert clinician will get suspicion
based on the history offered by the patient, which underscores the importance of meticulous history taking. The
most commonly used laboratory screening tests for bleeding disorders are bleeding time, platelet count, prothrombin
time and activated partial thromboplastin time. In addition
to this, capillary fragility test can also be conducted.
Platelet count This is usually obtained as a part of complete blood count. Normal platelet count is 150,000
450,000 cells/mm3. Decrease in the number of platelets is
called as thrombocytopenia. If the platelet count falls
below 50,000 cells/mm3, then hemorrhage may result due
to trauma or minor surgery. Spontaneous clinical hemorrhage is usually not observed with platelet counts above
10,00020,000.
Bleeding time (BT) This is a functional test of primary
hemostasis. The ivy template method is performed using
a special device that produces two small wounds keeping
the cuff of a sphygmomanometer constantly inflated at
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in collapsing adjacent capillaries and veins leading to cessation of bleeding. The various vascular disorders that may
lead to excessive bleeding tendency are:
Vessel wall disorders
1. Congenital
a. Hereditary hemorrhagic telangiectasia
b. Connective tissue disorders
EhlersDanlos syndrome
Osteogenesis imperfecta
Marfans syndrome
Cushings syndrome
2. Acquired disorders
a. Severe infections
b. Allergic manifestations
c. Drugs
Steroids
d. Others
Scurvy
Senile purpura
EhlersDanlos Syndrome
EhlersDanlos disease is a congenital disorder of collagen
synthesis in which there is joint hyperextensibility, skin
extensibility and tissue fragility such that capillaries are
poorly supported by subcutaneous collagen leading to ecchymosis. Oral findings include bleeding after toothbrushing,
hypermobility of TMJ, fragility of oral mucosa and gingiva,
stunted teeth and pulp stones on dental radiograph.
Marfans Syndrome
The connective tissue disorder is inherited as an autosomal
dominant trait and is caused by mutations in the fibrillin
Scurvy
Ascorbic acid (vitamin C) is the most active reducing
agent in the aqueous phase of living tissues and is involved
in the hydroxylation of proline in protocollagen to
hydroxylproline in mature collagen. It has been suggested
that high dose of vitamin C improves immune function
(including resistance to common cold) and cholesterol
turnover, but such effects remain unproven in controlled
trials. The scurvy, i.e. deficiency of vitamin C, leads to
defective formation of collagen. This impairs healing of
wounds, and causes capillary hemorrhage and reduced
platelet adhesiveness (normal platelets are rich in ascorbic
acid). Hemorrhage can occur in the muscles, joints, nail
beds and gingival tissues. Gingival involvement may
include swelling, bleeding, secondary infection and loosening of teeth. For treatment, a dose of 250 mg vitamin C,
8-hourly by mouth should saturate the tissues quickly. The
general deficiencies of the patients former diet also need
to be corrected and other vitamin supplements given if
necessary.
Investigations for vascular disorders
PLATELET DISORDERS
Platelet numbers or function may be impaired by many
diseases or drugs. Decrease in the number of platelets is
called as thrombocytopenia, which can occur either due to
impaired production or excessive destruction. Defect in the
function of platelets is called as thrombocytopathy, which
can occur due to drugs, liver disease or can be inherited. For
convenience, thrombocytopenic disorders and thrombocytopathic disorders are again divided into congenital and
acquired.
THROMBOCYTOPATHIC DISORDERS
Disorders of the platelet function are usually associated
with excessive bruising and gingival bleeding and, in
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Acquired
BernardSoulier syndrome
Glanzmanns thrombasthenia
Myeloproliferative disorder
THROMBOCYTOPENIC DISORDERS
These are caused by reduced platelet production in the
bone marrow or excessive peripheral destruction of platelets. The underlying cause may be revealed by history and
examination but a bone marrow examination will show
whether the platelets are reduced, normal or increased and
will provide essential information on morphology as well.
In patients with thrombocytopenia due to failure of production, no specific treatment may be necessary but the
underlying condition should be treated if possible. If the
platelet count is very low or the risk of bleeding is very
high, then platelet concentrate administration is indicated.
Idiopathic thrombocytopenic purpura (ITP), which is due
to increased destruction of platelets and thrombotic thrombocytopenic purpura are the commonly acquired thrombocytopenic disorders. MayHegglin anomaly is a rare
hereditary condition characterized by the triad of thrombocytopenia, giant platelets and inclusion bodies in leukocytes. Clinical features and the pathogenesis of bleeding of
these are poorly defined.
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Congenital
Acquired
MayHegglin anomaly
Impaired production
Bone marrow failure
Leukemia
Aplastic anemia (drugs, chemicals)
Excessive destruction
Immune
Idiopathic thrombocytopenic purpura
Dilutional
Massive transfusion
Others
DIC
Thrombotic thrombocytopenic purpura
Hemophilia A
DISORDERS OF COAGULATION
Coagulation disorders may be inherited or acquired. The
inherited disorders are uncommon and usually involve
deficiency of one factor only. The acquired disorders occur
more frequently and almost always involve several coagulation factors. The coagulation disorders can be classified
as follows.
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The inherited disorders can result from deficiency of factors essential in the clotting cascade or deficiency of vWF.
Of the inherited coagulopathies, von Willebrands disease
is the most common. It results from the deficiency of von
Willebrands factor (vWF). Hemophilia A, caused by coagulation factor VIII deficiency, is the next most common
coagulation disorder, followed by hemophilia B, a factor IX
deficiency. Clinical bleeding can vary from mild to severe,
depending on the specific clotting factor affected and the
level of factor deficiency.
Management
Bleeding is treated by administration of factor VIII concentrate by IV infusion. Factor VIII concentrate is freeze-dried
Hemophilia B and C
Christmas disease (factor IX deficiency) is clinically identical
to hemophilia A and inherited in the same way, but it is about
one-tenth as common as hemophilia A. Factor IX replacement
is needed before surgery and desmopressin is not used.
Concentrates used to treat factors VIII and IX deficiencies
are specific for each state, and therefore a correct diagnosis
must be made to ensure effective replacement therapy.
Factor XI deciency (PTA deciency) is one of the more
common among other congenital coagulation defects and
is sometimes known as hemophilia C. It is transmitted as
an autosomal dominant trait. Bleeding symptoms do occur
but are usually mild. In the event of major surgery or
trauma, hemorrhage can be controlled with infusions of
fresh frozen plasma.
Investigations
Management
Treatment depends on the severity of the condition and may
be similar to that of mild hemophilia, including the use of
desmopressin where possible. Intermediate purity factor VIII
or vWF concentrates should be used to treat bleeding or to
cover surgery in patients who require replacement therapy.
Cryoprecipitate should be avoided because of greater risk
of transfusion transmitted infections. Aspirin and other
NSAIDs should also be avoided.
Vitamin K Deficiency
Vitamin K is necessary for carboxylation of coagulation factors II, VII, IX, X, without which these factors cannot bind
calcium. Deficiency of vitamin K may be due to inadequate stores, malabsorption and oral anticoagulants (vitamin K antagonists). PT and aPTT are prolonged and there
may be bruising, hematuria and gastrointestinal or cerebral
bleeding. Deficiency may be treated by phytomenadione
(vitamin K) 10 mg IV. Newborn babies have low levels of
vitamin K, and this may cause minor bleeding in the first
week of life (classic hemorrhagic disease of the newborn).
RESPIRATORY DISORDERS
The lungs, with their combined surface area of more than
500 m2, are directly open to the external environment.
Thus, structural functional, or microbiological changes
within the lungs can be closely related to epidemiological,
environmental, occupational, personal, and social factors.
Primary respiratory diseases are responsible for a major
burden of morbidity and ultimately deaths and lungs are
often affected in mouth system diseases.
Respiratory infections are commonly encountered among
dental patients. The anatomic proximity of respiratory
tract with the oral cavity lead to much interplay between
oral and respiratory infections and there is a growing body
of literature pointing to a direct association between oral
pathogens and respiratory diseases. Recent studies have
reported an oral bacteria as a causative pathogen in respiratory diseases and conditions associated with signicant
morbidity and mortality and furthermore some respiratory
illnesses may have an effect on orofacial morphology and
even on dentition.
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Common causes
Nature/characteristics
Pharynx
Post-nasal drip
Usually persistent
Larynx
Laryngitis, tumor
Harsh, barking
Trachea
Tracheitis
Painful
Bronchi
Bronchitis
Dry or productive
Asthma
Bronchiectasis
Productive. Changes in
posture induce sputum
production
Sputum
Sputum is matter (mucus, phlegm) that is expectorated
from the respiratory tract (usually lower respiratory tract),
that is mixed with saliva, which can then be spat from the
mouth. It is usually associated with diseases of the lower
respiratory tract.
Type
Appearance
Cause
Serous
Mucoid
Purulent
Rusty
Pneumococcal pneumonia
Hemoptysis
Hemoptysis is the expectoration of blood or of bloodstained sputum from the bronchi, larynx, trachea, or
lungs.
Causes for hemoptysis
Common causes
Uncommon causes
Others causes
Pulmonary infarction
Mitral stenosis
Bronchial carcinoma
Aspergilloma
Chest trauma
Tuberculosis
Bronchial adenoma
Iatrogenic
Lung abscess
Metastatic
pulmonary
Bronchoscopy
Transbronchial biopsy
Acute bronchitis
Malignant disease
Chronic bronchitis
Laryngeal tumors
Chronic obstructive
pulmonary disease
Chest pain
Chest pain is commonly associated with anxiety, cardiac
diseases, respiratory diseases, and disorders of the musculoskeletal and gastrointestinal system.
Chest pain associated with the respiratory system can
manifest as pleural pain (pneumonia, pulmonary infarction,
tuberculosis, malignant disease), retrosternal pain (tracheitis,
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Diagnosis
Viral Infections
Main causes for upper respiratory tract infections (RTI)
}}
Rhinoviruses
Respiratory syncytial viruses
Parainfluenza virus
Coxsackie viruses
Echoviruses
Adenoviruses
Influenza viruses
EpsteinBarr viruses
Beta-hemolytic streptococci
Common cold
Allergic Rhinitis
Rhinitis is the recurrent inflammation of the nasal membranes and is characterized by a symptom complex that
consists of a combination of any of the following: sneezing,
nasal congestion, nasal itching, and rhinorrhea. The eyes,
ears, sinuses, and throat may also be affected. When the
conjunctiva is also affected the term allergic rhinoconjunctivitis is used. It is estimated that almost 20% of the population suffer from allergic rhinitis. Seasonal, perennial,
sporadic/vasomotor and occupational allergic rhinitis are
some of the types of allergic rhinitis.
Environmental triggers such as pollen, dust mites, animal dander may incite a type I allergic hypersensitivity
reaction.
In susceptible individuals, exposure to certain foreign
proteins leads to allergic sensitization, which is characterized by the production of specic IgE directed against
these proteins. This specic IgE coats the surface of mast
cells, which are present in the nasal mucosa. When the
specic protein (pollen/dust mite, spore) is inhaled, it can
bind to the IgE on the mast cells, leading to immediate and
delayed release of a number of mediators such as histamine, chymase, kinins and heparin, which in turn produce
the symptoms of rhinorrhea.
Rhinits is present in two phases: the early or immediate
phase and the late phase. The early phase is seen within a
few minutes of exposure to the allergen and characterized
by nasal congestion, sneezing, itching, redness, tearing, and
post nasal drip. The late phase usually occurs about 6 hours
after the early phase and patients present with reduction
in the frequency of sneezing and itching. However, congestion and increased mucus production is more common. This
phase may last for a few days.
Fatigue, malaise, drowsiness or sleepiness are common systemic effects of rhinitis. It is believed that allergic
rhinitis may often occur along with asthma or atopic
dermatitis.
Orofacial features and dental considerations
The readily visible facial features in allergic rhinitis are
allergic shiners, transverse nasal crease and DennieMorgan
lines. Allergic shiners is the term used to describe the
dark circles around the eyes due to vasodilation or nasal
congestion.
Rhinorrhea prompts individuals to frequently rub the
nose in an upward direction with the palm. This is classically referred to as allergic salute. Repeated rubbing of
the nose causes the formation of a transverse crease at the
tip of the nose. DennieMorgan lines are prominent infraorbital creases. These are also seen in atopic dermatitis.
The typical intraoral ndings include tonsillar hypertrophy and streaks of lymphoid tissue on the posterior part of
the oropharynx which gives rise to a cobble-stone appearance. Other symptoms include itching sensation in the palate and tongue protrusion. A study by Elad et al (2006)
showed the patients with allergic rhinitis had lower salivary ow rates compared to healthy individuals.
Gingival inflammation
Matsson and Mller (1990) studied the degree of gingival
inflammation in children with rhinoconjunctivitis due to
birch pollinosis. Their study showed that during the pollen
season, children with allergic rhinoconjunctivitis exhibit
an enhanced degree of gingival inflammatory reaction.
Dental and skeletal abnormalities
Trask et al (1987) analyzed the effects of perennial allergic
rhinitis on dental and skeletal development. The allergic
subjects were characterized by deeper palatal vault height,
retroclined mandibular incisors, increased total anterior
facial height and lower facial height, a larger gonial angle,
and greater SN, palatal, and occlusal planes to mandibular
plane angles. Also, the allergic subjects had smaller SNB
and SN-pogonion angles and an increased overjet compared to normal controls.
Martnez Esteinou and Omaa Vidal (1988) showed that
the patients with history of allergic rhinitis and nasopharyngeal obstruction (may result in mouth breathing) of
allergic origin exhibited a high palatal vault, retroinclinable maxillary incisors and increased total anterior facial
height.
Bacterial pharyngitis
Patients with bacterial pharyngitis generally do not have
rhinorrhea, cough, or conjunctivitis. GABHS (mainly Streptococcus pyogenes) is the most common bacterial cause of
pharyngitis. Other less common bacterial causes include
Corynbacterium diphtheriae, Neisseria gonorrheae, Chlamydia
and Mycoplasma pneumoniae.
Pharyngotonsillitis caused by GABHS is characterized
by pharyngeal erythema and swelling, tonsillar exudate,
edematous uvula, palatine petechiae, and anterior cervical
lymphadenopathy. The infections last for about one week
when left untreated.
Scarlet fever is associated with GABHS pharyngitis and
usually presents as a punctate, erythematous, blanchable,
sandpaper-like exanthem. The rash is found in the neck,
groin, and axillae, and is accentuated in body folds and
creases (Pastias lines). The pharynx and tonsils are erythematous and covered with exudates. The tongue may be
bright red with a white coating (strawberry tongue).
The complications of GABHS infection are rheumatic
fever, peritonsillar abscess and poststreptococcal glomerulonephritis. Peritonsillar abscess occurs in fewer than 1%
of patients treated with antibiotics. Patients with peritonsillar abscess typically have a toxic appearance and may
present with a hot potato voice, uctuant peritonsillar
mass, and asymmetric deviation of the uvula.
Clinical scoring system for validation of
Streptococcal infection
Symptoms and points
Asthma
Asthma is a dynamic condition characterized by chronic
airway inflammation and bronchial hyperactivity resulting
in symptoms of paroxysmal wheeze, cough, chest tightness
and dyspnea.
Age
Scoring
Diagnosis
Along with the clinical findings, blood smear examination,
estimation of liver enzymes, culture for group A Streptococcus and detection of antigen will aid in the diagnosis.
Antistreptolysin O titers raise about 150 U within 2 weeks
of acute infection. They are useful for documenting recent
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Predisposing/risk factors
Asthma originates from the interaction of multiple genetic
and environmental factors. Asthma is known to run in
families. Childhood asthma usually occurs in atopic individuals who express elaborate amounts of IgE when exposed
to common allergens/antigens.
Environmental factors are: house dust mites, automobile
exhausts, industrial gaseous wastes, climatic changes,
tobacco smoke, airborne irritants (including acrylic and
aerosolized dental materials) and pollen. Other factors that
predispose to asthma are infections (viral and bacterial
infections), drugs (-blockers, salicylates/NSAIDs cause
bronchoconstriction) and anxiety.
Extrinsic asthma is precipitated by allergens in house
dust, feathers, animal fur, molds, milk, eggs, sh, fruits,
nuts and NSAIDs; whereas intrinsic is related to mast cell
instability and hyperresponsive airways.
Pathophysiology
It generally is believed that both genetic and environmental
factors, as well as allergens, are important in the initiation
and continuation of the airway inflammation.
Airway inammation in asthma has been characterized
as acute, subacute or chronic. The acute state of inammation is caused by the release of chemical mediators from
activated resident cells, such as local airway mast cells
undergoing histamine degranulation.
Subacute inammation is marked by early cellular inltrates, especially eosinophils that release mediators with
direct toxic effects on the respiratory epithelium.
The airway inammation is described as chronic when
lymphocytes and eosinophils mediate a persistent, ongoing inammation, thus resulting in a continuous cycle of
damage and repair. Long-standing chronic inammation
can lead to irreversible airway obstruction in some patients.
Bronchial smooth muscle contraction contributes markedly to the airway obstruction seen in asthma, while vasodilation, diapedesis and vascular permeability account for
the edematous changes. These changes are attributed to cellderived mediators. Mucus hypersecretion is also observed
and can result in the development of mucus plugs and
associated dyspnea.
Clinical symptoms and signs
Cough, shortness of breath, chest tightness, wheezing,
tachypnea and tachycardia, are usually noticed. Typically
these symptoms show ready reversal when bronchodilators
are used. In acute asthmatic attacks patients are extremely
distressed, flaring of the nares, sweating, central cyanosis,
use of accessory respiratory muscle, pulsus paradoxus and
silent chest may be seen.
Orofacial features
Asthmatic patients are known to have an increased incidence of carious lesions, reduced salivary flow, an increased
prevalence of oral mucosal changes, increased levels of
gingivitis and related orofacial abnormalities.
It is believed that the prolonged use of 2-agonists,
which is associated with diminished salivary production
and secretion results in increased caries development. This
reduction in salivary ow (26% drop in whole saliva) is
accompanied by concomitant increase in lactobacilli and
Streptococcus mutans in the oral cavity. Anti-asthmatic
medications containing fermentable carbohydrate and
sugar may also add to the bacterial build-up.
The use of corticosteroids via nebulizers leads to dysphonia, dryness of mouth and oropharyngeal candidiasis.
Linder et al (1995) reported tongue hypertrophy associated
with inhaled corticosteroid therapy in premature infants.
Since most of the asthmatics are habitual mouth breathers, gingival enlargement is routinely encountered. Use of
Table 1
Dos
Donts
Instruct patient to rinse mouth with water after using inhalers. Oral
hygiene measures to reduce gingivitis and periodontitis
Clinical features
Early morning productive cough, dyspnea and orthopnea
are common clinical features. Dyspnea leads to reduced
oxygen saturation, carbon dioxide build-up and respiratory failure or cor pulmonale (right sided heart failure).
Some patients with emphysema try their best to maintain normal levels of blood gases by hyperventilating. These
patients are referred to as pink panters or pink puffers.
However, patients suffering from chronic bronchitis cannot
hyperventilate and become hypoxic and hypercapneic (high
levels of carbon dioxide in blood). Such a state of hypoxemia results in central cyanosis in association with elevated
jugular venous pressure giving rise to a blue bloated appearance. These patients are referred to as blue bloaters.
Dental considerations
Patients suffering from emphysema may present with xerostomia. Occasionally, tobacco related mucosal lesions may
be seen.
510
Patients should ideally be seated upright for the treatment. Rubber dam should be avoided as it can further
compromise breathing. Drugs to be avoided include IV barbiturates, diazepam and midazolam as they cause respiratory
depression.
General anesthesia is best avoided. Wherever possible
all treatment should be performed under local anesthesia
without epinephrine. Bilateral mandibular nerve blocks
should be avoided.
Management
Patients should be advised to abstain from exposure to
smoke. Expectorants and humid environment will hasten
the recovery process. Bronchodilators (methylxanthines
theophylline, aminophyline), sympathomimetic agents
(isoproterenol, ephedrine, salbutamol) and corticosteroids
are effectively used. Acute infective phases can be managed
with antibiotics. Digitalis and diuretics are used for cor
pulmonale.
Cystic Fibrosis
Cystic fibrosis (CF) is an autosomal recessive genetic disorder that affects the functioning of nearly all of the
bodys exocrine glands. The pulmonary manifestations are
characterized by repeated endobronchial infections, an
exaggerated inflammatory response, airways obstruction,
and bronchiectasis. It is estimated that the incidence of CF
is approximately 1 in 3,500 live births.
Pathogenesis of cystic fibrosis
Felix (2009) describes the current concept in the evolution
of cystic fibrosis. The CF gene defect leads to an absent
or malfunctioning cystic fibrosis transmembrane conductance regulator (CFTR) protein, which results in abnormal
chloride conductance on the apical membrane of the epithelial cell. In the lung, this results in airway surface liquid
depletion and, since airway surface liquid is essential to
support ciliary stability and functioning, ciliary collapse
and decreased mucociliary transport. The consequence of
this is a vicious circle of phlegm retention, infection, and
inflammation.
Clinical features specific to respiratory system
Patients present with a chronic or recurrent cough, which
can be dry at the beginning and can produce mucoid (early)
and purulent (later) sputum. Prolonged symptoms of bronchiolitis occur in infants. Paroxysmal cough followed by
vomiting may occur. Recurrent wheezing, recurrent pneumonia, atypical asthma, pneumothorax, hemoptysis, and
digital clubbing are all complications and may be the initial
manifestation. Dyspnea on exertion, history of chest pain,
recurrent sinusitis, nasal polyps, and hemoptysis may occur.
These patients may have a sweat sodium concentration
in excess of 70 mmol/l. Spirometry can be used to assess
the lung function.
Dental considerations
Swelling of submandibular gland or parotid gland may be
seen. Tooth eruption may be delayed. Enamel hypoplasia is
a common feature. Pancreatin, which is used in the treatment
of CF can cause oral ulcerations. Extensive use of antibiotics and steroids can predispose to oral candidiasis. The low
fat, high carbohydrate diet will lead to carious teeth.
GRANULOMATOUS DISEASES
Clinical features and oral manifestations of tuberculosis
and sarcoidosis have been described in Chapter 21 on
Granulomatous Diseases.
MALIGNANT DISORDERS
Bronchogenic Carcinoma
It is estimated that almost 95% of all primary lung tumors
are bronchogenic carcinomas. These carcinomas may arise
from the bronchial epithelium or the mucous glands.
Cigarette smoking is considered as the single most important etiological factor in the production of lung cancer.
Other predisposing factors include: occupation related exposure to asbestos, chromium and cadmium, and exposure to
radiation.
Clinical features
The earliest clinical signs and symptoms include: cough,
breathlessness, pleural pain and hemoptysis. Local infiltration of the tumor leads to pleural effusion, brachial neuritis and recurrent laryngeal nerve palsy (produces bovine
cough). When the superior vena cava is obstructed facial
cyanosis and edema is seen. Esophageal obstruction may
cause dysphagia.
Distant metastasis is characterized by symptoms of lassitude, anorexia and weight loss.
A wide spectrum of clinical features is appreciated based
on the site of metastasis. Common metastatic sites include
bones, liver, adrenal glands, lymph nodes, brain and spinal
cord. Palpable lymphadenopathy, particularly in the supraclavicular fossa, suggests metastasis.
Hepatomegaly is seen when there is hepatic metastasis;
cerebral metastasis leads to epilepsy, hemiplegia and disturbed vision. Bone metastasis results in pain, swelling and
in severe cases pathological fracture of the bone affected.
Dental considerations
Metastasis to the jaw bones is relatively rare. On rare occasions, the metastatic lesion may appear as a soft tissue swelling or pigmented lesion of the oral mucosa. Abraham et al
(2003) described facial pain as the presenting symptom of
lung carcinoma. They reported 31 cases with a symptom of
unilateral facial pain, although there was no metastatic
lesion in the mandible. The most frequent locations of pain
were the ear, the jaw and the temporal region, 61% occurring
on the right and 39% on the left side. The pain resolved or
partially remitted in most patients following treatment of
the carcinoma. They suggested that this pattern of referred
pain from the chest to the face presumably involves either
direct tumor invasion or compression of the vagus nerve by
malignant lymph nodes. The vagus nerve, which contains
motor, visceral and somatic afferent and parasympathetic
fibers, innervates both thoracic and cranial structures.
General anesthesia is best avoided as the respiratory
function is compromised.
511
Sporadic or epidemic infection in the form of legionellosis pneumonia, described as Legionnaires disease
Pontiac fevera flu-like form having a mild course
An extra-lung form in immunosuppressed patients,
often taking a severe clinical course, with the septic
syndrome, coagulation disorders, acute cardiovascular
deficiency and nephritis.
Diagnosis
Legionellosis diagnostics is based on serological studies of
the blood serum to indicate the level of antibodies, on the
patients urinalysis to determine the presence of a specific
antigen with the immunoenzymatic ELISA and radioimmunological (RIA) tests and on the bacteriological examination of the bronchial tree secretion, bronchoalveolar
washings, lung biopsy material and sputum.
Dental considerations
High- and low-speed handpieces, ultrasonic instruments and
airwater syringes produce air-water aerosols, which may
be source of infection. Both the dental team and the patient
are exposed to the infected aerosols via inhalation. By inhaling them and choking, the airwater aerosol with the droplets of 0.25.0 m in diameter, can contain Legionella.
RENAL DISORDERS
Homeostasis, a term introduced by WE Cannon, which
means the maintenance of constant internal environment.
Functions of Kidney
1.
2.
3.
4.
5.
6.
Nephrons that are about 1 million per kidney are the functional unit of the kidney that helps to filter wastes from
blood, modulate excretion of salts and water from the body
and allow the kidney to perform its excretory, metabolic
and endocrine functions. Since, the nephrons are incapable
of regeneration, renal function is maintained until approximately half of the nephrons are destroyed. When the kidneys compensatory mechanisms are overwhelmed, signs
and symptoms of renal failure begins to manifest.
RENAL DISEASES
1.
2.
3.
4.
5.
Chronic glomerulonephritis
Traumatic loss of kidney tissue
513
2.
Pathogenesis
The various by-products of protein and amino acid metabolism in a patient with uremia exert toxic effect on every
organ systems in the body. As the kidney fails, the nephron population falls. If this progresses the glomerular filtration rate falls, and the blood urea nitrogen (BUN) rises,
which results in mild azotemia (abnormal retention of nitrogen products in blood), impaired ability to concentrate urine,
nocturia and mild anemia. If this continues, frank renal
disease follows with its associated polyuria. The anemia may
become severe and hypocalcemia, hyperphosphatemia and
metabolic acidosis may occur. When azotemia is associated with adverse clinical signs and symptoms, it is called
uremia. Advanced uremia is associated with derangement
of function in many major organ systems.
Mirahamadi et al stated that the prolongation of life in
patients with end-stage uremia by the use of maintenance
hemodialysis has resulted in the occurrence of a number
of clinical syndromes that may be related to the persistence of certain biochemical abnormalities associated with
impaired renal function.
According to Harrison, urea represents some 80% or more
of the total nitrogen excreted into urine in patients with
chronic renal failure maintained on diets containing 40 g
or more of protein per day. Creatinine may cause adverse
effects in uremic subjects following conversion to more
toxic metabolites such as sarcosine and methyl guanidine.
Davidson has said that disturbances in water, electrolyte
and acidbase balance undoubtedly contribute to the clinical picture in patients with chronic renal failure, but the
exact pathogenesis of the clinical syndrome of uremia is
unknown. Almost any substance present in abnormal concentration in the plasma has been suspected of being a
uremic toxin. It is most likely that the syndrome is caused
by accumulation in body uids of a number of substances,
among which phosphate, parathyroid hormone, urea creatinine, guanidine, phenols and indoles must be included.
Scott stated that uremic syndrome primarily results from
the retention and accumulation of excretory products and
the diminished endocrine and metabolic functions of the
kidney. He has given the following laboratory ndings in
progressive renal disease.
Systemic manifestations of renal failure
Systemic manifestations:
1.
514
Gastrointestinal
a. Anorexia
3.
4.
5.
6.
b. Hiccups
c. Nausea
d. Vomiting
e. Uremic gastroenteritis
Neuromuscular
a. Drowsiness
b. Lack of concentration
c. Insomnia
d. Loss of memory
e. Mild behavioral changes
f. Errors in judgment
g. Neuromuscular irritability
h. Seizures and coma
i. Peripheral neuropathy
Hematologic
a. Normochromicnormocytic anemia
b. Bleeding
Immunologic
a. Infection
Endocrinemetabolic
a. Loss of libido
b. Decreased thyroid function
c. Amenorrhea
Cardiovascular
a. Hypertension.
1.
2.
3.
4
5.
6.
7.
Endocrine-metabolic Number of hormonal abnormalities including loss of libido in both the sexes, are due to
the associated hyperprolactinemia. Thyroid function was
diminished. Amenorrhea was common in female.
Cardiovascular Hypertension develops in 8090% of
patients during the course of chronic renal failure. The hypertension contributes to the development of cardiomyopthy,
atherosclerosis and progression of the renal failure itself.
Pericarditis was very common in untreated end stage renal
failure. Vascular calcification may develop and be sufficiently severe to cause inadequate perfusion of the limbs.
Dermatologic Severe pruritus was one of the important
manifestations of the clinical syndrome of hyperparathyroidism in patients with advanced uremia. The skin of the uremic
patients may be dry and there may be ecchymosis resulting
from bleeding tendencies. Pruritus has been attributed to calcium and phosphate deposition in the skin and high circulating PTH levels. In advanced uremia a white, uremic frost
may evaporate on the skin surface with a fishy scale odor.
The patient frequently complains of brittle nails.
Oral manifestations
Several changes occur in the oral cavity that is associated
with chronic renal failure and uremia. It was estimated that
Erythematopultaceous stomatitis
Ulcerative stomatitis
Hemorrhage
Hyperkeratosis and generalized pallor
Poor taste perception
Infection
Peripheral giant cell lesion.
Several mechanisms have been postulated for the pathogenesis of each form of uremic stomatitis. The oral lesion
may be a reaction to toxins in the tissues or to the action
of ammonia or to irritating ammonium compounds formed
by the action of bacteria on urea. The manifestation of
uremia in the mouth and GI tract may also be caused by
the hemorrhagic diathesis common in uremia. Local hemorrhage may cause a decrease in vitality and viability of
the affected tissues allowing bacterial infection. These
infections result in ulceration and psuedomembranous
formation.
Wysocki et al presented a case of primary hyperoxaluria
with oxalosis (accumulation of related deposits in various
extra-renal sites) involving oral tissues, including bone,
gingiva, dental pulp, periodontal ligament and dentin in a
27-year-old male patient.
Goldstein (1990) quoted Baries classication of uremic
stomatitis as follows:
Type I Erythematopultaceous, initially manifests as a red
thickening of the buccal mucosa, which later includes a
gray, thick, pasty, gluey exudate and pseudomembrane
which covers the gingiva, fauces and the oral mucosa. When
the psuedomembrane is removed with tongue blade, a
swollen, dry, red but not ulcerated mucosa is found. Associated manifestations include fetor oris, dry burning sensation, excessive saliva and perversion of taste.
Type II Ulcerative form which is similar to type I but
includes loss of integrity of the mucosa with frank ulceration. The ulcers can be superficial or deep and frequently
515
Figure 5
Radiographic manifestations
Liu and Chu (1943) coined the term renal osteodystrophy,
which denotes osseous changes that occur in patients with
ESRD.
Skeletal changes are caused by the disorders in calcium
and phosphorus metabolism, abnormal vitamin D metabolism, and increased parathyroid activity. In chronic renal
failure, intestinal absorption of calcium is reduced because
the kidneys are unable to convert vitamin D into its active
form 1,25-dihydroxycholecalciferol which is required for
absorption of calcium from the digestive tract. Impaired
absorption of calcium because of defective kidney function and corresponding retention of phosphate causes a
decrease in the serum calcium level. This is associated with
a compensatory hyperactivity of the parathyroid glands.
The parathyroid hormone then extracts calcium from bone
516
Figure 6
Uremic Stomatitis
Uremic stomatitis has become relatively rare, seen mostly
in cases of undiagnosed and untreated chronic renal failure.
Painful plaques and crusts are distributed predominantly
on the buccal mucosa, the oor or dorsum of the tongue,
and the oor of the mouth. The incidence has decreased
because of readily available dialysis centers throughout the
country. The most commonly accepted mechanism behind
the development of uremic stomatitis is irritation and chemical injury of mucosa by ammonia or ammonium compounds formed by the hydrolysis of urea in saliva by urease.
This occurs when the intraoral concentration of urea exceeds
30 mmol/l. Hemorrhagic diathesis from inhibited platelet
aggregation may also play a role due to local hemorrhage,
resulting in decreased vitality and viability of the affected
tissues, thus allowing bacterial infection.
There are two predominant types of uremic stomatitis.
In Type I, there is a generalized or localized erythema of
the oral mucosa and a thick gray pseudomembranous exudate which does not leave a bleeding or ulcerated base
when removed. Additional ndings may include pain, burning, xerostomia, halitosis, gingival bleeding, dysgeusia, or
candidal infection. Type II leaves ulceration if the pseudomembranous lm is removed. This type may indicate a
more severe form of stomatitis, secondary infection, anemia or underlying systemic hematologic disturbances caused
by renal failure.
Histologically, both types of uremic stomatitis show
evidence of an intense inammatory process with heavy
polymorphonuclear leukocytic inltration and necrosis of
the oral mucosa. Bacterial colonization is most commonly
associated with Fusobacterium, Spirochaeta or Candida.
GASTROINTESTINAL DISORDERS
GASTROESOPHAGEAL REFLUX DISEASE
Gastric esophageal reflux is defined as the passage of gastric contents into esophagus. It is believed that Helicobacter
pylori, a spiral shaped bacterium located in the mucous
layer of the stomach, may inhibit or exacerbate acid reflux.
The symptoms that develop when the esophageal mucosa is
exposed to the gastric reflux are referred to as gastroesophageal reflux disease (GERD). It is a common, chronic
gastrointestinal disorder that affects approximately 30%
of the population.
Predisposing factors and pathophysiology
Genetic factors, smoking, lower esophageal sphincter abnormalities, hiatal hernia, defective esophageal peristaltic activity (common in esophagitis) and defective gastric emptying,
increased abdominal pressure (pregnancy and obesity), and
dietary behavior (diet consisting of fat, coffee, alcohol and
chocolate relax the lower sphincter and incite symptoms).
Clinical features
Gastroesophageal reflux disease is more common in adults
in their third decade of life. The common symptoms are
heartburn (mimics anginal pain, burning sensation spreading upward from the epigastrium to the neck) and regurgitation. Water brash is also a very common complaint in
patients. It is the presence of a sudden burst of salivation
in the mouth due to reflex salivary gland stimulation in
response to presence of acid in the gullet. Other clinical
findings are chest pain, cough, laryngitis and laryngeal
polyps. In severe recurrent reflux episodes, aspiration and
pulmonary fibrosis may occur.
Oral considerations
Bargen and Austin (1937) first described the association
between GERD and erosion of teeth. In the initial stages
enamel may exhibit subtle changes. As the condition progresses, complete loss of tooth structure may be seen. The
teeth typically affected are the palatal aspect of the upper
anteriors and premolars.
Grade Ierythema
Grade IIlinear non-confluent erosions
Grade IIIcircular confluent erosions
Grade IVBarretts esophagus.
ULCERATIVE COLITIS
Ulcerative colitis is an IBD affecting part or whole of the
large intestine frequently lower colon and rectum. It was
first described by a physician, Sir Samuel Wilks from
London in 1859. The precise etiology of ulcerative colitis
is not well understood. A current hypothesis suggests
that primary dysregulation of the mucosal immune system
leads to an excessive immunologic response to normal
microflora.
Clinical features
Ulcerative colitis is most common between the 2nd4th
and 5th8th decades of life. Males and females are equally
affected. The characteristic symptoms and signs include
bloody diarrhea, rectal urgency and difficulty to pass stools.
The frequency of bowel movements and the amount of
blood present reflect the activity of the disease. The diarrhea is severe, possibly five to eight bowel movements in
24 hours. Patients usually complain of pain that is in both
abdominal quadrants and that is crampy in nature and exacerbated prior to bowel movement. Along with the change
in the pattern of bowel movements, the patient may have
nocturnal diarrhea.
The extraintestinal manifestations include osteoporosis,
arthritis, primary sclerosing cholangitis, uveitis, pyoderma
gangrenosum, deep venous thrombosis, pulmonary embolism. The most serious complication of ulcerative colitis is
carcinoma of the colon.
520
Disease
Clinical characteristics
Crohns colitis
Infectious colitis
Ischemic colitis
Pseudomembranous
colitis
CROHNS DISEASE
Crohns disease was first observed by the German surgeon
Wilhelm Fabry in 1623, and was later described by and
named after physician Dr Burril B Crohn from New York.
It is an idiopathic, relapsing chronic inflammatory disease
of the GI tract categorized as IBD and is an important cause
of morbidity in children and adolescents.
The prevailing hypothesis regarding the pathogenesis of
CD is an interaction between environmental factors and an
altered immune response in genetically predisposed children,
leading to chronic inammation of the GI tract. In CD there
is a disordered regulation of mucosal and systemic immune
response resulting in the perpetuation of the inammatory
cascade. A dysregulated Th1 response (T helper 1) seems to
be crucial in the conversion of physiologic to pathologic
inammation. The immunological prole in CD is predominantly a cell-mediated response. Active mucosal inammation of the small and large intestine results in diarrhea,
protein-losing enteropathy, bleeding, abdominal pain and
stricture formation. Proinammatory cytokines and eicosanoids increase vascular permeability and cause electrolyte
secretion, and augment smooth muscle contraction. Many
cytokines promote the recruitment and activity of collagen
forming cells leading to brous tissue proliferation and
years before the intestinal symptoms. Hepatobiliary complications such as chronic hepatitis, sclerosing cholangitis,
cholelithiasis and elevated aminotransferase may precede
the active disease. The renal manifestations of IBD include
nephrolithiasis, hydronephrosis and enterovesical stula.
Other extraintestinal manifestations are thromboembolic
manifestations, vasculitis, pancreatitis, interstitial pneumonitis and pericarditis, others include weight loss, growth
failure, bone disease (due to malnutrition). Delay in sexual
maturation seen in some children with CD may have a
signicant effect on self-esteem and socialization.
Oral manifestations
Aphthous ulcerations are the most common oral manifestation of CD. The oral manifestations may occur along with
intestinal disease or precede it and may be the primary
presentation. Cobble stone appearance of the oral mucosa,
oral epithelial tags and folds, gingivitis, persistent lip swelling, lichenoid mucosal reactions, granulomatous inflammation of minor salivary gland ducts, candidiasis, and
angular cheilitis. Some patients may have asymptomatic
intestinal disease. MelkerssonRosenthal syndrome (facial
swelling, facial palsy and fissured tongue) and cheilitis granulomatosa may also be incomplete manifestation of CD.
High prevalence of caries and periodontitis in CD has also
been reported.
Differentiating CD from ulcerative colitis can be challenging, particularly early in the course of the disease,
but it is an important step because appropriate treatments
and potential complications vary for these two conditions
(Table 2).
Table 2
Feature
Ulcerative colitis
Crohns disease
Sites mainly
affected
Ileum
Colo-rectum
Abdominal pain
Variable
Common
Depth of
inflammation
Mucosal
Transmural
Distribution
Diffuse, contiguous
spread; always
involves rectum;
spares proximal
gastrointestinal tract
Segmental, non-contiguous
spread (skip lesions); less
common rectal involvement;
occurs in entire
gastrointestinal tract
Iron deficiency
Common
Common
Other
complications
Vitamin B12
deficiency
Diarrhea
Severe
Less severe
Fistula and
sinus tracts
Rare
Common
Colonic
carcinoma risk
Present
High
521
Investigations
The small bowel has been defined for many years as the
black box of the gastrointestinal system due to its inaccessibility to endoscopic exploration. The conventional
radiological methods, i.e. small bowel enteroclysis (SBE)
and small bowel follow-through (SBFT), have long been
the only imaging methods providing information on the
morphological features of the small bowel valuable in the
diagnosis and management of Crohns disease. Typical small
bowel changes which can be observed by means of these
techniques include irregular thickening and distortion of
the valvulae conniventes, loops adhesions (mass-like effect)
or separated loops because of wall thickening and mesenteric inflammatory infiltration. Transverse and longitudinal
distribution of ulcerations can separate islands of thickened
internal wall, resulting in the typical cobble stone appearance. Strictures are often separated by healthy bowel tracts
(skip lesions); impaired small bowel peristalsis is commonly
observed within rigid stenotic tracts. Extrinsic compression
may be observed, due to mesenteric lymph node enlargement. Barium meal series is useful to identify small bowel
involvement and lesions such as strictures, fistulas and
ulcerations may be identified.
Bowel ultrasonography The main ultrasonography findings in CD are represented by thickening and stiffness of
the gut wall, modifications or lack of its echo stratification, reduction of peristalsis, mesenteric fibro-fatty proliferation, lymph node enlargement; in case of complications,
narrowing of the intestinal lumen, abscesses and fistula
are usually easily detectable. The use of power Doppler
methods and of oral (polyethylene glycole, PEG) and/or
intravenous (Levovist) contrast media, have been suggested to improve the diagnostic accuracy of ultrasonography, particularly in discriminating inflammatory from
fibrotic strictures and in better defining the presence of
internal fistulas.
Computed tomography Computed tomography (CT) has
been utilized for the detection of extra-enteric complications of CD, mainly intra-abdominal abscesses, but is also
suitable in the evaluation of strictures, prestenotic dilatations and fistulas. Non-enhanced CT scan is also used in
the diagnosis of post-surgical complications (intra-peritoneal abscesses, anastomotic dehiscence, extra-abdominal
abscesses and fistulas, incisional hernias, ascites, volvolus,
bowel adhesions, etc). The main findings at CT scan observed
in CD patients, are small bowel wall stratification and/or
thickening (target or double halo appearance), with or
without contrast enhancement, edema of the mesenteric fat,
engorged ileal vasa recta (comb sign), submucosal fibrofatty infiltration and mesenteric adenopathy.
Video capsule endoscopy Video capsule endoscopy
(VCE) is a potentially safe and painless endoscopic method
522
HIATAL HERNIA
Hiatal hernia is the herniation of part of the stomach into
the thoracic cavity through the esophageal hiatus in the
diaphragm. Hiatus hernias affect anywhere from 1 to 20%
of the population. It is estimated that about 9% present
with symptoms. Hiatal hernia is usually seen in the elderly
although people of any age can be affected.
Etiology
Peptic ulcer disease of the GI tract is characterized by mucosal damage secondary to pepsin and gastric acid secretion.
It usually occurs in the stomach and proximal duodenum;
less commonly, it occurs in the lower esophagus, the distal
duodenum, or the jejunum, as in unopposed hypersecretory states such as ZollingerEllison syndrome, in hiatal
hernias (Cameron ulcers), or in ectopic gastric mucosa (e.g.
in Meckels diverticulum. It is believed to affect almost 10%
of the adults (usually between 25 and 65 years) at least
once in their lifetimes.
Clinical features
The sign and symptoms include heartburn, belching, chest
pain and nausea, which tend to become worse on leaning
forward, lifting heavy objects or lying down. In severe cases,
dysphagia and painful swallowing may occur. Infants may
regurgitate blood stained food and present with difficulty
in breathing and swallowing. Occasionally pain may radiate to the jaw and along the arm mimicking anginal pain.
Other clinical features are hiccups, dry cough and increase
in the contractile force of the heart.
There are two major types of hiatus hernia: the sliding
hiatus hernia (95%), where the gastroesophageal junction
moves above the diaphragm together with some of the
stomach. The second rare type is rolling (or paraesophageal) hiatus hernia, when a part of the stomach herniates
through the esophageal hiatus beside, and without movement of the gastroesophageal junction. Occasionally, a third
type is described, which is a combination of the rst and
second.
Investigations and management
Endoscopy is more frequently used than barium studies in
the diagnosis and assessment of hiatal hernia. Manometry
and esophageal pH testing may provide useful information.
Treatment is indicated if symptoms interfere with day to
day routine of the patient. Weight reduction, diet modification, raising the head of the bed and reduction of alcohol,
caffeine and nicotine intake are all recommended.
Routinely, antacids to H2-receptor antagonists and proton pump inhibitors are sufcient. Surgery should be considered in severe and resistant cases of hiatal hernia.
Dental considerations
Since many of the medications used for treating hiatal
hernia can cause xerostomia, cervical caries is common. If
reflux into the oral cavity is present, oral manifestations are
the same as those of GERD.
Patients can be advised to frequently sip water. Salivary
substitutes can be recommended. NSAIDs should be avoided.
Etiology
H. pylori infection and the use of non-steroidal anti-inflammatory drugs are the most common causes of peptic ulcer
disease. Other medications that have been known to cause
ulcers include steroids, bisphosphonates, potassium chloride
and chemotherapeutic agents (e.g. intravenous fluorouracil).
A variety of other infections and co-morbidities are associated with a greater risk of peptic ulcer disease (e.g. cytomegalovirus, tuberculosis, Crohns disease, hepatic cirrhosis,
chronic renal failure, sarcoidosis, myeloproliferative disorder). Critical illness, surgery or hypovolemia leading to
splanchnic hypoperfusion may result in gastroduodenal
erosions or ulcers; these may be silent or manifest with
bleeding or perforation. Smoking increases the risk of ulcer
recurrence and slows healing.
Clinical features
Typical symptoms of peptic ulcer disease include episodic
gnawing or burning epigastric pain; pain occurring 25 hours
after meals or on an empty stomach; and nocturnal pain is
relieved by food intake, antacids or antisecretory agents.
A history of episodic or epigastric pain, relief of pain after
food intake, and night time awakening because of pain with
relief following food intake are the most specific findings
for peptic ulcer.
Less common features include: indigestion, vomiting
(coffee ground vomitus), loss of appetite, intolerance of fatty
foods, heartburn and a positive family history.
The serious or alarming symptoms that necessitate further
investigations and immediate treatment include: anemia,
hematemesis, melena (black tarry stools); vomiting suggests obstruction; anorexia or weight loss suggests cancer;
persisting upper abdominal pain radiating to the back suggests penetration; and severe, spreading upper abdominal
pain suggests perforation.
Diagnosis
Patients older than 55 years and those with alarming symptoms should be referred for esophago-gastro-duodenoscopy
(EGD). It is more sensitive and specific for peptic ulcer
523
Dental considerations
Non-steroidal anti-inflammatory drugs and steroids should
be strictly avoided. Most patients present with xerostomia
secondary to the anticholinergic drugs. Anemia related
symptoms and dental considerations have to kept in mind.
2.
3.
Management
Treatment of peptic ulcer disease should include eradication
of H. pylori in patients with this infection. The recommended duration of therapy for eradication is 1014 days.
Regimen: Omeprazole 20 mg 2 times daily or lansoprazole 30 mg 2 times daily plus amoxicillin 1 g 2 times daily
or metronidazole 500 mg 2 times daily (if allergic to penicillin) plus clarithromycin 500 mg two times daily.
Surgery is indicated in patients who are intolerant of
medications or do not comply with medication regimes, and
those at high risk of complications (e.g. transplant recipients, patients dependent on steroids or NSAIDs, those with
giant gastric or duodenal ulcer, those with ulcers that fail
to heal with adequate treatment).
EATING DISORDERS
Eating disorders are associated with various medical and
psychologic consequences, including death, osteoporosis,
growth delay and developmental delay. Anorexia nervosa
and bulimia nervosa are two of the most devastating eating disorders. Eating disorders are most commonly seen
among adolescents and young adults of developed nations.
They are many times more common in females than in
males.
These disorders are seen in individuals who participate
in activities that promote thinness, such as modeling, movies
and athletics, and certain personality traits such as low selfesteem, difculty expressing negative emotions, difculty
resolving conict, and being a perfectionist. It is also seen
that up to one-third of women with type 1 diabetes may
have eating disorders.
Anorexia Nervosa
Patients with anorexia use caloric restriction or excessive
exercise to control emotional need or pain, and they are
terrified of becoming overweight.
524
4.
Refusal to maintain body weight at or above a minimally normal weight for age and height (e.g. weight
loss leading to body weight less than 85% of that
expected; or failure to make expected weight gain
during period of growth, leading to body weight less
than 85% of that expected).
Intense fear of gaining weight or becoming overweight, even though patient is underweight.
Disturbance in the way in which ones body weight or
shape is experienced, undue influence of body weight
or shape on self-evaluation, or denial of the seriousness of the current low body weight.
Amenorrhea in postmenarchal females (i.e. the absence
of at least three consecutive menstrual cycles. A woman
is considered to have amenorrhea if her periods occur
only following hormone administration.)
Bulimia Nervosa
Bulimia also has two subtypes: purging and non-purging.
Bulimia is characterized by uncontrollable binge-eating
episodes, often followed by purging behaviors such as vomiting or the use of laxatives. Patients with binge-eating/
purging-type anorexia also might binge and purge. Patients
who have bulimia may be of normal weight, or they may be
under- or overweight, whereas patients with binge-eating/
purging-type anorexia are underweight.
Diagnostic criteria for bulimia nervosa
1.
2.
3.
4.
5.
Immunological function
Acts as a detergent
Emulsifies fat
Absorption of fat-soluble vitamin
Excretion of hemoglobin by-products
Neutralizes any stomach acid
Bactericidal potential
SCOFF questions
Oral manifestations
Frequent vomiting leads to erosion of the enamel on the
lingual surfaces of the maxillary teeth. Palatal ulcerations
may be noticed. Parotid enlargement may develop as a
sequela of starvation.
LIVER DISEASES
The liver has many essential functions, and liver disease
presents a number of concerns for the delivery of medical
and dental care. A number of conditions that cause liver
dysfunction may be related to lifestyle and a variety of
diseases. This section of the chapter will deal with two
common liver conditions, namely, hepatitis and jaundice,
that have important dental implications.
Functions of liver
Functions of the liver can be categorized as follows:
Nutrient metabolism
Carbohydrate
Protein
Lipids
Protein synthesis
Albumin
Coagulation factors
Complement factors
Hepatoglobin
Ceruloplasmin
Storage
Excretion
Iron
Copper
Vitamin A
Vitamin B12
Vitamin D
Bile salts
Bilirubin
Metabolism of drugs
Imaging
Ultrasonography
CT
MRI.
Jaundice
Jaundice or icterus is a yellowish discoloration of tissue
resulting from the deposition of bilirubin. Tissue deposition
of bilirubin occurs only in the presence of serum hyperbilirubinemia and is a sign of either liver disease or less
525
Hepatitis
Findlay and colleagues in 1939 postulated that the etiology
of this particular disease was probably a virus carried in
the blood of some apparently by inadvertent inoculation
with vaccines contaminated with human serum containing
the infectious agent.
The term serum hepatitis rst came into use in the
early 1940s as a synonym for post-inoculation hepatitis. The
present diseases now labeled hepatitis A and hepatitis B
were rst described as a single entity, separate from other
causes of jaundice, called sporadic and epidemic catarrhal
jaundice was thought to be infectious, but unknown, causative agent (Cockayne, 1912). MacCallum in 1947 suggested
that to avoid confusion in terminology, the two postulated
viruses be termed virus A, which produces infectious hepatitis after a short incubation period and virus B, which
produces serum hepatitis after a long incubation period.
Hepatitis has a number of potential causes, both infectious and non-infectious. Alcohol, prescription medications,
and drug abuse are predominant non-infectious causes,
while viruses and bacteria are important infectious etiologic factors. Viral and drug-induced hepatitis are examples
of primary hepatitis. Secondary hepatitis may occur as a
sequela of other disease entities such as mononucleosis,
syphilis and tuberculosis.
Hepatitis A
Hepatitis A is caused by the hepatitis A virus (HAV), an
enterovirus of the Picornaviridae family. HAV is a singlestranded RNA virus surrounded by a protein capsid. Transmission is primarily by the oral-fecal route. Various methods
of spread are through contact of an infected person, traveling to an endemic region, and ingestion of contaminated
food or water. Hepatitis A is typically a self-limiting disease and usually causes mild illness characterized by sudden onset of non-specific symptoms. In children of age
6 years or younger, it is usually asymptomatic. In adults,
infection may present with symptoms such as fever, fatigue,
526
Hepatitis B
Hepatitis B virus is a 42 nm DNA virus of the Hepadnaviridae
family. The DNA genome is circular and comprised of two
strands, with one strand being partially incomplete. It is a
highly infectious virus that produces three distinct particles during replication:
Hepatitis E and G
Hepatitis C
Hepatitis C virus, previously known as one of the non-A
non-B hepatitis viruses, is a small, positive-sense, singlestranded RNA virus of the Flaviviridae family. Six major
genotypes of HCV and 40 related subtypes have been
identified. The virus has a core protein and two envelope
glycoproteins. The prevalence of HCV infection worldwide
is between 0.3% and 1.5%, with an estimated 300 million
carriers worldwide. The genetic diversity of HCV, and its
ability to mutate, allows the virus to avoid neutralization
and establish a chronic infection in about 8590% of
infected persons. Eighty-five percent of patients with HCV
will develop chronic hepatitis. Chronic HCV infection is
the major cause of cirrhosis and hepatocellular carcinoma.
Acute HCV infection usually presents with mild flu-like
symptoms, while chronic disease is variable in presentation. Patients may experience non-specific symptoms such
as fatigue, nausea, and/or abdominal pain. Majority of
HCV-infected patients develop chronic active hepatitis
that is characterized by persistent and intermittent viremia, fluctuating elevations of serum alanine aminotransferase (ALT) levels and slow but progressive liver damage.
The first decade is usually marked by inflammatory cell
infiltration of the portal tracts and focal liver cell necrosis.
Mild fibrosis ensues that is followed by more severe fibrosis, and bridging between portal tracts and hepatic veins.
By the second decade after infection, fibrosis progresses to
cirrhosis in at least 20% of patients with chronic HCV
infection. Progression is more likely if patients consume
excessive amounts of alcohol.
Future treatment of hepatitis C may include agents that
specically target the virus rather than current non-specic
forms of antiviral therapy. Research is focused on targeting the helicase and polymerase crucial to HCV replication.
Other therapeutic targets are the viral proteases and the
59 and 39 strands of HCV RNA.
Hepatitis D
Hepatitis D virus (HDV, delta agent) is a defective RNA
virus that uses the HBV surface antigen as a viral envelope. HDV can occur as a coinfection or superinfection in
Autoimmune Hepatitis
Autoimmune hepatitis is a chronic inflammatory disease.
Although the etiology is not clear, it is thought that environmental and viral factors may cause alterations in cellular
markers on hepatocytes leading to an autoimmune response
in genetically susceptible individuals. The etiology appears
to be related to antigen specific and generalized suppressor defects that perpetuate the autoimmune response. The
main finding in autoimmune hepatitis is IgG hypergammaglobulinemia, which may be due to chronic infections
or alteration in immune response. Autoimmune hepatitis
527
may lead to liver cirrhosis. Treatment protocols for autoimmune hepatitis patients usually include steroid therapy.
If remission is not obtained by steroid therapy alone, azathioprine or its metabolite (6-mercaptopurine) may be added
to the protocol.
Fulminant Hepatitis
Fulminant hepatitis is sudden, severe liver dysfunction
which may lead to hepatocellular necrosis as well as hepatic
encephalopathy. Signs of fulminant hepatitis include jaundice, hepatomegaly and right upper quadrant tenderness
during the inflammatory stage. As necrosis occurs and
progresses, the liver becomes atrophic and less readily palpable. With the onset of severe liver failure, liver enzymes,
bilirubin, prothrombin time and partial thromboplastin time
will be elevated, while hemoglobin and hematocrit will
decrease. The prognosis for fulminant hepatitis is poor, and
the treatment of choice is liver transplantation.
Clinical course of hepatitis B and C
Hepatitis B and C virus infections can occur at any age,
but are more common after puberty. The incubation period
for HBV is 45180 days and for HCV it is 14180 days.
Hepatitis B and C viruses produce symptoms in 10% and
2530% of patients, respectively. Symptoms initially are
flu-like and include fatigue, fever, loss of appetite, diarrhea,
headache, malaise, myalgia, nausea, vomiting and weakness. During acute HBV infection, about 50% of symptomatic persons become icteric within about 10 days of the
onset of symptoms. A minority become icteric with HCV
infection. Icterus is the stage of infection in which patients
demonstrate jaundice in the skin, conjunctiva of the eye,
oral mucosa, and urine as a result of serum bilirubin levels
rising three- to four-fold above normal levels (0.21.2 mg/dl).
About 10% of patients infected with HBV also demonstrate
serum sickness-like manifestations, including angioedema,
arthralgia, and a rash. As the disease progresses, abdominal pain increases and hepatomegaly and splenomegaly
develop. Two to eight weeks are required for recovery from
symptoms, with hepatomegaly and abnormal liver function
persisting for weeks to months. The course of the disease
varies with alcohol use and the viral strain and load. The
acute infection rarely requires medical treatment other than
rest and the avoidance of hepatotoxic drugs. Patients who
fail to produce an adequate immune response can develop
fulminant hepatitis or a chronic infection.
Investigations and diagnosis
The diagnosis of acute HBV infection is made by recognition of the clinical features, specific serologic tests for viral
antigens and antibodies, and elevated liver enzymes. The
hepatitis B surface antigen (HBsAg) is the first detectable
specific marker. Hepatitis B surface antigen appears in the
528
Prior to dental treatment appropriate laboratory investigations should be carried pout. These include:
NEUROMUSCULAR DISORDERS
BELLS PALSY
Bells palsy is a lower motor neuron disease of the facial
nerve (cranial nerve VII) characterized by acute unilateral
peripheral facial weakness involving muscles innervated
by the facial nerve. Bilateral weakness is very rare and
occurs in less than 1% of patients.
Bells palsy is named after the 19th century Scottish
anatomist and surgeon Sir Charles Bell (17741842), who
rst described the condition along with the anatomy and
function of the facial nerve.
The annual incidence of Bells palsy is about 20 per
100,000 persons, with equal numbers of men and women
affected and the incidence increases with age. There is no
predilection for either side of the face. It occurs more commonly in patients with diabetes and in pregnant women.
Patients who have had one episode of Bells palsy have an
8% risk of recurrence. About 10% of those with Bells
palsy have a family history of the condition.
Etiopathogenesis
Etiology Common condition affecting 15-to 45-year-old
age group, both sexes. The cause is unknown, but site of
damage is probably the portion of facial nerve lying within
the facial canal (stylomastoid).
Inammation of facial nerve in the canal with demyelination and edema further hazards with blood supply.
Inammation of the nerve initially results in a reversible
EPILEPSY
The word epilepsy is derived from the Greek word epilambanein meaning to take or to seize. Epilepsy can be defined
as a chronic neurological disorder characterized by frequently recurrent seizures. A seizure manifests as an episodic disturbance of movement, feeling or consciousness
caused by sudden synchronous, inappropriate and excessive
electrical discharges that interfere with the normal functioning of the brain.
Epilepsy usually affects young children (genetic predisposition to epilepsy associated with chromosome 12 anomalies) and elderly individuals who have a history of stroke,
tumors and Alzheimers disease.
Clinical types of epilepsy
Generalized seizures There are two basic forms of generalized seizures, namely, tonic-clonic (grand mal) seizures
and absence (petit mal) seizures.
Grand mal seizures are usually seen in childhood or
at teenage. These seizures begin after a brief period of aura
(warning signs like irritability, headache, nausea). The seizures begin with abrupt loss of consciousness. The tonic
phase is characterized by rigidity of the arm, leg, chest
and back muscles. The patient may fall, pupils become
dilated and the spine becomes extended (opisthotonos).
Following this phase there is a clonic phase characterized
by repetitive jerking and twitching movements of the limbs,
tongue and lips. Tongue biting, frothy salivation and
vomiting may occur. In this phase, bowel and bladder control may also be lost. The seizures usually last for about
23 minutes. Following this is the post ictal phase in
which the patient is tired and goes to sleep for as along as
12 hours.
Status epilepticus When tonic-clonic seizures last more
than 5 minutes or recur in a series of three or more seizures
without return to consciousness between attacks, a serious
neurological emergency called convulsive status epilepticus
has developed.
Absence (petit mal) seizures Absence (petit mal) seizures
are seen in childhood. These are called absence seizures as
these are not preceded by aura or warning signs. These
seizures last for about 30 seconds and may only present as
rapid eye blinking or stare, and brief loss of consciousness.
However, these recur very frequently almost up to 100 attacks
in a day.
Broadly, seizures can be classified as partial or generalized. A seizure is termed partial when the electrical
discharge causing it occurs in a specific area of the brain
or generalized when the discharge affects the entire brain
cortex.
It is estimated that about 50% of the patients using phenytoin exhibit gingival hyperplasia within 1224 months of
initiation of treatment.
Xerostomia and stomatitis have been reported rarely as
side effects of carbamazepine. Valproic acid can cause
direct bone marrow suppression, which can impair wound
healing and increase postoperative bleeding and infections.
Decreased platelet count is the most common and best
recognized hematologic effect of valproic acid.
When restoration and replacement of teeth are considered
it is best to use a xed rather than removable prosthesis
and inclusion of additional abutments should be planned
for xed partial dentures.
Rash is a common side effect of antiepileptic drugs
(almost 57%). Although most drug-associated rash is
benign, serious rashes, including StevensJohnson syndrome and toxic epidermal necrolysis do occur.
Metronidazole, antifungal agents such as uconazole
and antibiotics such as erythromycin may interfere with
the metabolism of certain antiepileptic drugs and render
them ineffective for seizure control.
PARKINSONISM
Parkinsons disease is a serious brain disorder characterized
by degeneration of the basal ganglia. Patients present with
bradykinesia (slow movements), increased muscular tone
(rigidity), tremors and loss of postural reflexes.
Etiology
The exact etiology is still unknown. Though previously no
strong genetic basis was considered, it is now believed
that genetic factors may have a role to play. Parkinsonism
may be caused by head injuries, drug abuse (phenothiazine, valproate, prochlorperazines and methyl phenyl tetrahydropyridine). Environmental exposure to toxins such
as carbon monoxide, heavy metals and herbicides/pesticides may have a crucial role in the etiology.
This led to the suggestion that many cases of apparently
idiopathic toxins such as pesticides, herbicides, manganese, heavy metals and carbon monoxide.
Clinical features
In the initial stages of the condition patients may complain
of fatigue, aching limbs, depression and mental sluggishness. It has also been seen that these patients tend to have
a tiny handwriting (micrographia). The unique feature in
parkinsonism is a unilateral presentation of the signs/symptoms initially which turns into a bilateral involvement as
the condition worsens.
Other clinical features are tremors of the hands and feet
at rest, tongue tremors, gait becomes slower, muscular
tone and rigidity increases (log wheel rigidity) and speech
MULTIPLE SCLEROSIS
Multiple sclerosis (MS) is a chronic inflammatory disease of
the CNS. The etiology is still not clear. It is thought that
genetic predisposition together with immunity and environmental factors could trigger the disease.
Clinical features
Multiple sclerosis is usually seen between the 2nd and 4th
decades of life. It is slightly more common in women than
in men. MS exhibits a wide variety of symptoms as a result
of the demyelination of the CNS, and a later slow down or
blocked transmission at the level of axons. Ectopic impulses
may also appear, causing paroxysms and convulsions.
Multiple sclerosis is categorized into four subtypes:
(i) relapsingremitting (RR), (ii) progressive-relapsing (PR),
(iii) secondary-progressive (SP) and (iv) primary-progressive
(PP). Almost 85% of the patients belong to the relapsingremitting subtype.
Clinically, MS usually appears with a number of symptoms of neurological anomalies which settle in a matter of
minutes or hours, and which frequently progress in later
days. The most common features are: visual and oculomotor
anomalies, optical neuritis, anterior internuclear ophthalmic
paralysis, palsies, paresthesia, lack of motor coordination
and tonic spasms. Other less common features are genitourinary dysfunction, increased frequency and urge of urination and urinary incontinence, lack of bladder control and
episodic retention of urine. In the later stages, paroxysmal
ataxia and dysarthria or shaking of upper limbs is frequently seen which indicate brain damage.
Dental considerations
Patients may be encouraged to sit upright on the dental chair
to avoid respiratory embarrassment. Hypothetically, nitrous
531
MUSCULAR DYSTROPHY
Muscular dystrophy (MD) is a life-threatening recessive
neuromuscular disease affecting the short arm of the
X chromosome in the p21-2 position. Of the various forms
of MD, severe generalized familial muscular dystrophy
and mild restricted muscular dystrophy have significant
orofacial findings.
Severe generalized familial muscular dystrophy is popularly referred to as Duchenne muscular dystrophy (DMD).
It is commonly seen in young male children. In the early
stages of the disease, muscle enlargement may be seen
which becomes atrophic as the disease worsens. The early
recognizable symptom is the inability to walk and run.
Muscles associated with the lower extremities, pelvis and
shoulder girdle rapidly atrophy. Similarly, muscles of the
face, larynx and pharynx are affected.
Symons et al (2002) carried out a study to assess the
dental characteristics of patients with DMD. Their study
showed that patients with DMD had high plaque and calculus accumulation, leading to gingival inammation, due
to decreased muscle function. However, the caries experience was low. Disturbances in tooth form, number and
eruption of the second premolars were observed in 39% of
patients. Anterior and posterior open bites were common,
associated with lip incompetence, mouth breathing, macroglossia and tongue thrusting. Maxillary and mandibular
arch breadths were signicantly larger, on average, in the
DMD group than in controls. Rather than a normal parabolic arch form, the dental arches in DMD patients tended
to be hyperbolic, with the posterior teeth being displaced
buccally, consistent with an imbalance between the lingual and facial musculature.
An elevated serum creatinine phosphokinase level is
typical for this condition. The disease has no known treatment as of date and usually the affected individuals do not
live beyond the 2nd decade of life.
OROMANDIBULAR DYSTONIA
Dystonia is an involuntary, repetitive, sustained (tonic)
or spasmodic (rapid or clonic) muscle contraction. Few of
the dystonias that are of interest to a dental surgeon are
cranial-cervical dystonia (CCD) and oromandibular dystonia (OMD). Multiple etiological factors are proposed for
OMD, namely, genetic predisposition, injury to the CNS,
peripheral trauma, medications, metabolic or toxic states
and neurodegenerative disease. It usually affects individuals
532
MYASTHENIA GRAVIS
Myasthenia gravis is described in Chapter 20 on Autoimmune
Disorders.
ENDOCRINAL DISORDERS
The specialty of endocrinology encompasses the study of
glands and the hormones they produce. The term endocrine
was coined by Starling to denote the action of hormones
secreted internally in contrast to exocrine glands which
secrete into a lumen. The term hormone is a Greek word
meaning to set in motion describing the dynamic actions
of hormones as they elicit cellular responses and regulate
physiologic processes through feedback mechanisms.
The classic endocrine glandspituitary, thyroid, parathyroid, pancreatic islets, adrenals and gonadscommunicate
broadly with other organs through the nervous system,
hormones, cytokines and growth factors. In addition to its
synaptic functions, the brain produces a vast array of peptide hormones, spawning the discipline of neuroendocrinology. The immune and endocrine systems are also intimately
intertwined. The adrenal glucocorticoid, cortisol, is a powerful immunosuppressant. Cytokines and interleukins have
profound effects on the functions of the endocrine organs.
Common endocrine diseases, such as autoimmune thyroid
disease and type I diabetes mellitus are caused by dysregulation of immune surveillance and tolerance.
Endocrinal disease can present with either overt disease
or subtle ndings to the oral health clinician. Patients with
hormonal dysfunctions caused by endocrinal diseases may
be found to have signicant abnormalities on head and neck
examination. Large pituitary adenomas frequently present
with changes in vision and loss of visual eld integrity,
and the oral health clinician may be the rst medical professional to note these diseases. For example, in active
Graves disease (one of the conditions causing excessive
Table 3
Hormones released
Thyrotrophs
Corticotrophs
Gonadotrophs
Somatotrophs
Lactotrophs
Prolactin
regulation of digestion, use and storage of nutrients, electrolyte and water metabolism and reproductive functions.
Hormones
Hormones function as chemical messengers moving through
the blood to distant target sites of action (pituitary hormones like growth hormone, thyroid stimulating hormone
[TSH] and adrenocorticotropic hormone [ACTH]); act more
locally as paracrine or autocrine messengers that incite more
local effects (like histamine, bradykinin and eicosanoids).
Most are present in body fluids at all times in greater or
lesser amounts as needed.
Categories of hormones according to structure
Table 4
Glucagon
Insulin
Epinephrine
Parathyroid hormone
Thyroid-stimulating hormone (TSH)
Adrenocorticotropic hormone (ACTH)
Follicle-stimulating hormone (FSH)
Luteinizing hormone (LH)
Antidiuretic hormone (ADH)
Secretin
Table 5
Estrogens
Testosterone
Progesterone
Adrenal cortical hormones
Thyroid hormones
534
Congenital defects
Glandular destruction due to infarction, hemorrhage,
infection, inflammation, autoimmune responses, neoplastic growth or surgeries
Decline in function with aging
Atrophy as result of drug therapy or unknown reasons
Mutations in a number of hormones, hormone receptors, transcription factors, enzymes and channels can
also lead to hormone deficiencies.
Autoimmune damage to the thyroid gland (Hashimotos
thyroiditis) and pancreatic islet -cells (type 1 diabetes
mellitus) are prevalent causes of endocrine disease.
Hyperfunction
An organ is in the state of hyperfunction if there is excessive hormone production.
Causes
Autoimmune disorders
Hormone-producing tumor of the gland
Excess hormone administration.
Hormone resistance
Hormone resistance is a state when the production of hormone is optimal but the end organs are resistant to
hormonal effects.
Causes
Inherited defects in membrane receptors, nuclear receptors, or in the pathways that transduce receptor signals.
Pathophysiology of HypothalamicPituitary
Axis (HPA)
The HPA axis can be considered the master of ceremony in
regulating the endocrine functions. The secretions of the
hypothalamus act on the pituitary which further releases
hormones to act on the end organs.
Hypothalamic hormones regulating the secretion
of anterior pituitary hormones
GROWTH HORMONE
The growth hormone (GH), produced by somatotropes in
the anterior pituitary is necessary for linear bone growth
in children. It stimulates cells to increase in size and divide
more rapidly by enhancing amino acid transport across cell
membranes; increases the rate at which cells use fatty acids
and decreases the rate at which cells use carbohydrates.
535
Investigations
Variants of normal
Low birth weight
Chronic illness and malnutrition
Functional endocrine disorders
Chromosomal disorders
Skeletal abnormalities
Unusual syndromes.
Orodental manifestations
In pituitary dwarfs eruption rate and shedding of teeth is
delayed for 13 years for teeth that normally erupt during
1st decade of life and for 310 years for teeth erupting
in the 2nd decade. Lack of third molar development is a
common finding. Clinical crowns are smaller because of
incomplete eruption of teeth. The dental arches are smaller
resulting in malocclusion. The anatomical crowns are normal in size. The roots of teeth are shorter and supporting
structures are retarded in growth. Mandibular growth is
more retarded than maxilla.
Evoked GH is less than 3 ng/ml, IGF-I level is low or normal, Concomitant gonadotropin, TSH, ACTH reserves may
be deficient, LDLcholesterol is increased.
Managment
Replacement therapy with GH at 0.150.3 mg/day, maximum 1.25 mg/day to maintain IGF-I levels in the midnormal range for age and gender matched controls. It is
contraindicated in the presence of an active neoplasm,
intracranial hypertension, uncontrolled diabetes and retinopathy. Replacement with adrenocortical and thyroid
hormones in panhypopituitarism may be necessary.
Gigantism
Management
Replacement therapy with recombitant GH (0.020.05 mg/
kg per day SC) GH insensitivity and in mutations of GH
receptor IGF-I is given which bypasses the dysfunctional
GH receptor.
Adult GH Deficiency
Growth hormone deficiency in adults is caused by hypothalamic or pituitary somatotrope damage. Infarction of
the pituitary leading to hypopituitarism in adults is called
Simmonds disease. Panhypopituitarism, loss of weight,
diminished sexual function, reduced basal metabolic rate,
atrophic skin, thin eyebrows, loss of eyelash, sharp features;
thin lips with immobile expressions are the features. Other
clinical features include impaired quality of life, increased
body fat mass, central fat deposition, reduced exercise
capacity, CVS risk factors with impaired cardiac structure
and function, abnormal lipid profile and atherosclerosis.
536
Laboratory findings
Investigations
Age and gender matched serum IGF levels is increased.
Single GH level estimation is not useful. Confirmation is
by the failure of GH suppression to less than 1 g/l within
12 hour of an oral glucose load. Prolactin levels are
increased.
CT and MRI play a pivotal role in the evaluation of these
patients. MRI is now the investigation of choice in diagnosis
of pituitary adenomas. About 8085% of micro-adenomas
are visible on unenhanced T1 weighted MRI and 3350%
are seen as areas of hyperintensity on T2 weighted MRI.
MRI is better than CT in assessment of sella in the presence
of bony skull base thickening due to brous dysplasia as
in cases of McCuneAlbrights syndrome. The distinction
between pituitary gland and abnormal brous bone tissue
at skull base is better made on MRI. The combination of
pre- and post-contrast images is useful in this regard.
However, CT of skull base plays a useful role in some cases
for detailing neural foraminal compression, especially if
surgery is being contemplated.
In one study (Akira Hagiwara et al) on T2-weighted MRI,
hypointensity was seen more commonly in adenomas that
produced GH than in those which did not; hypointensity
was nearly exclusive to densely granulated GH-producing
adenomas.
Octreotide scintigraphy, in combination with other imaging modalities, is useful in the diagnosis and follow-up of
pituitary tumors. It allows scar tissue to be differentiated
from tumor recurrence after surgical treatment and ensures
better selection of patients who will benet from medical
treatment with somatostatin analogs.
Radiographic features
General radiographic features
Management of acromegaly
537
Clinical features
Investigations
Diabetes Insipidus
Diabetes insipidus (DI) is caused due to the decreased
secretion or action of ADH. It is characterized by production of abnormally large volumes of dilute urine.
Etiology
Cranial DIdeficient production of ADH. It is caused by
genetic defect, such as dominant, recessive DIDMOAD syndrome (DI, diabetes mellitus, optic atrophy, deafness).
Hypothalamic or high stalk lesionhistiocytosis X, sarcoidosis, craniopharyngioma, suprasellar pituitary adenoma,
basal meningitis, head injury, surgery, encephalitis.
THYROID GLAND
The thyroid produces two hormones, thyroxin (T4) and triiodothyronine (T3). Acting through nuclear receptors,
these hormones play a critical role in maintaining thymogenic and metabolic homeostasis; help in cell differentiation influencing growth and development in children and
mental development and attainment of sexual maturity.
Thyroxin stimulates eruptive movement and tooth size, but
has little effect on alveolar growth. After diabetes mellitus,
thyroid disease is the most common endocrine problem in
the general population and most commonly seen in women
with higher rate after the age of 50 years.
Pathophysiology of HypothalamicPituitary
Thyroid Axis
It is a classic example of endocrine feedback loop.
Idiopathic causes
Nephrogenic DIrenal tubules unresponsive to vasopressin.
Causes for nephorgenic DI are:
Hypothalamus
TRH +
Anterior
pituitary
TSH +
Thyroid
()
()
T4T3
peptide most specific to thyroid function thyrotropinreleasing hormone (TRH); TSH, a larger peptide from the
pituitary, predominantly associated with thyroid function.
Together, these peptides control three major functions of
the thyroid: the production of thyroid hormone (T4), the
growth and proliferation of thyroid cells, and the production of thyroglobulin (a large protein that acts as a binding,
storage and maturation protein for T4). As a consequence
of driving thyroid hormone production with TSH, circulating systemic blood levels of T4 are elevated. The increased
blood levels of T4 result in decreased pituitary secretion of
TSH, forming a negative feedback loop. The negative feedback mechanism causes decrease in the specific releasing
and stimulating hormones of the hypothalamus and pituitary. Decreases in the pituitary production of the corresponding stimulating hormones, TSH and ACTH, result in
the downregulation of the hormone produced by the specific endocrine gland, T4.
Laboratory evaluation
Categorized as:
1.
2.
Etiology
Primary
Iodine deficiency
Autoimmune hypothyroidism: Hashimotos thyroiditis, atrophic thyroiditis
Iatrogenic: Treatment, subtotal or total thyroidectomy,
external irradiation of neck for lymphoma or cancer
Drugs: Iodine excess (including iodinecontaining
contrast media and amiodarone), lithium, antithyroid
drugs, p-aminosalicylic acid, interferon-
and other
cytokines, aminoglutethimide
Congenital hypothyroidism: Absent or ectopic thyroid
gland, dyshormonogenesis, TSH-R mutation.
Infiltrative disorders: Amyloidosis, sarcoidosis, hemochromatosis, scleroderma, cystinosis, Riedels thyroiditis
Transient hypothyroidism
Silent thyroiditis, including postpartum thyroiditis
Subacute thyroiditis.
Secondary
Hypopituitarism: Tumors, pituitary surgery or irradiation, infiltrative disorders, Sheehans syndrome, trauma,
genetic forms or combined pituitary hormone deficiencies
Isolated TSH deficiency or inactivity
Hypothalamic disease: Tumors, trauma, infiltrative
disorders, idiopathic.
Hypothyroidism in children is termed cretinism and that
manifesting in adults called myxedema.
Congenital hypothyroidismcretinism
Congenital hypothyroidism if undetected early results in
hypothyroidism in children termed cretinism.
Congenital hypothyroidism is due to thyroid gland dysgenesis or inborn errors of thyroid hormone synthesis,
TSHR antibody mediated.
Females have a 2:1 prediliction. The infant appears
normal at birth. The condition is diagnosed because of
prolonged jaundice, feeding problems, hypotonia, macroglossia, delayed bone maturation, umbilical hernia, sparse
and brittle hair and ngers, atrophic sweat glands. Diagnosis is established by heel prick blood estimation of T4.
If treatment is delayed permanent neurologic damage
occurs. T4 is instituted at a dose of 1015 g/kg per day
with close monitoring of TSH levels.
Oral manifestations
Hypothyroidism
Primary hypothyroidism is an intrinsic disorder of the thyroid gland causing low levels of thyroid hormone with
raised TSH.
539
Hyperthyroidism
Primary hyperthyroidism
1.
2.
3.
4.
5.
540
Causes of thyrotoxicosis
6.
7.
Graves disease
Toxic multinodular goiter
Toxic adenoma
Functioning thyroid carcinoma metastases
Activating mutation of the TSH receptor (autosomal
dominant)
Struma ovarii
Drugs; iodine excess (JodBasedow phenomenon).
Subacute thyroiditis
Silent thyroiditis
Other causes of thyroid destruction; amiodarone, radiation, infarction of adenoma
Ingestion of excess thyroid hormone (thyrotoxicosis
factitia) or thyroid tissue.
Secondary hyperthyroidism
1.
2.
3.
4.
Clinical features
Symptoms Hyperactivity, irritability, dysphoria, heat
intolerance and sweating, palpitations, fatigue and weakness, weight loss with increased appetite, diarrhea, polyuria, oligomenorrhea, loss of libido.
Signs Tachycardia, atrial fibrillation in the elderly, tremors, goiter, warm, moist skin, muscle weakness, proximal
myopathy, lid retraction or lag and gynecomastia.
Oral manifestations
Alveolar atrophy is seen in advanced cases as a consequence of osteoporosis. Periodontitis and dental caries
appear more rapidly. The jaws and teeth development is
accelerated and earlier shedding of deciduous teeth and
accelerated eruption of permanent teeth is seen. Euthyroid
infants of hyperthyroid infants may have natal teeth. Lingual thyroid is sometimes seen. A lingual thyroid if seen
Graves Disease
Hyperthyroidism with diffuse thyroid enlargement, ophthalmopathy and dermatopathy.
An autoimmune disorder characterized by abnormal
stimulation of the thyroid gland by thyroid-stimulating
antibodies (thyroid-stimulating immunoglobulins [TSI])
that act through the normal TSH receptors.
This is associated with human leukocyte antigen
(HLA)-DR3 and HLA-B8. Familial tendency is evident due
to immunologically mediated activation of broblasts in
extraocular muscles and skin with accumulation of lid lag
or retraction, proptosis, exophthlamos, diplopia, corneal
involvement, periorbital edema, chemosis, optic nerve
damage.
Dermatopathy in the form of pretibial myxedema, skin
appearance appears like orange and clubbing may be seen.
Radiographic features
In children early development and eruption of teeth with
premature loss of primary teeth is seen. In adults generalized decrease in bone density or loss of some areas of
edentulous alveolar bone is seen.
Investigations
Increased T3
Increased or upper limit normal rangeT4
Decreased TSH
Non-specific or increased serum glutamic oxaloacetic
transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), alkaline phosphatase.
Routine thyroid 99mTc radionuclide imaging is helpful
in identifying patients with an unsuspected cause of
hyperthyroidism other than Graves disease.
The treatment of Graves disease is aimed at
1.
2.
Clinical features
Patients present with very high fever, extreme cardiovascular effects (tachycardia, congestive failure and angina) and
severe CNS effects (agitation, restlessness and delirium).
Management
PARATHYROID GLANDS
Parathormone (PTH) is an 84 amino acid single chain peptide and is the primary regulator of extracellular calcium
concentration. It acts directly on bone inducing calcium
resorption and on the kidney where it stimulates calcium
reabsorption and synthesis of 1,25-dihydroxy vitamin D
(1,25(OH)2D) a hormone that stimulates GI calcium
reabsorption.
Continuous exposure to elevated PTH leads to increased
osteoclastic mediated bone resorption. However intermittent administration of PTH, elevating levels for 12 hour/
day leads to net stimulation of bone formation rather than
breakdown. Thus the effects of PTH are: (i) calcium removal
from bone, and (ii) bone remodeling by acting on both
osteoblasts and osteoclasts.
Hyperparathyroidism
Hyperparathyroidism (HPT) causing bone disease was first
described by von Recklinghausen in 1891 and so-called as
von Recklinghausen disease of bone. It is a generalized disorder of calcium, phosphate and bone metabolism due to
increase in PTH secretion.
Hyperparathyroidism occurs in three clinical forms:
Clinical features
It was described by Jackson and Frame (1972) as the tetrad
of bones, stones, abdominal groans and psychic moans
with fatigue overtones.
Mostly occur after 60 years of age and women are
affected 24 times more often than men. About 80% of
the cases are asymptomatic and the primary involvement
is in the kidneys and skeletal system. Stones refer to the
increased deposition of calcium in renal parenchyma and
a tendency to develop recurrent nephrolithiasis (renal calculi) with resulting complications like urinary tract obstruction, infection, loss of renal function and uremia. Metastatic
calcications are also seen in blood vessel walls, subcutaneous soft tissues, dura and the region around the joints. Band
keratopathy, where calcication occurs as a narrow band at
the limbic margin of the cornea of the eye is also seen.
Bones refer to the distinctive involvement of the bone.
Ostetitis brosa cystica, bone lesions which on histopathological examination show multinucleated giant osteoclasts in scalloped areas of the bone surface (Howships
lacunae) and replacement of normal cellular and marrow
elements with brous tissue.
Abdominal groans refer to subtle vague GI disturbances
such as nausea, vomiting, anorexia, pancreatitis, duodenal
and peptic ulcers.
Psychic moans may be due to CNS manifestation ranging from mild personality problems to severe psychiatric
disorders due to hypercalcemia.
Other manifestations include neuromuscular problems
with proximal muscle weakness, easy fatigability and
muscle atrophy (differentiated from other neuromuscular
disorders by regression after surgical removal of the
glands).
Orodental manifestations
One of the first signs is development of malocclusion
because of drifting of teeth. Giant cell tumors and pseudocysts of the jaws are the other possible lesions found.
Padbury et al demonstrated a signicantly greater incidence of torus/tori in the Caucasian population with the
incidence of tori nearly three times higher in HPT patients.
HPT leads to a preferential loss of cortical bone and preservation or increase in trabecular bone. It has been proposed that the presence of hypercalcemia in HPT patients is
preceded by longer periods of elevated PTH levels. It could
be that the tori represent an expansion of trabecular bone
at the expense of cortical bone in response to elevated PTH
levels with possible contribution from the mechanical
forces present in the oral cavity. Furthermore, PTH mediated
endosteal resorption has been shown to be compensated
by PTH-mediated periosteal apposition with an increase in
overall bone size.
Periodontal ligament (PDL) width in patients with a torus
was higher than those without a torus, and this intriguing
Serum calciumannually
24-Hour urinary calcium only during initial evaluation
Creatinine clearance only during initial evaluation
Serum creatinine annually
Bone densityannually
Abdominal radiographs with or without ultrasonograph at the time of initial evaluation
Secondary HTP
Restriction of dietary phosphate, administration of
bisphosphonates and vitamin D metabolite (calcitrol)
and renal transplantation.
Hypoparathyroidism
Hypoparathyroidism could be hereditary or acquired.
Decreased amount of PTH leads to hypocalcemia and
hyperphosphatemia.
Hereditary
Hypoparathyroidism associated with conditions like
DiGeorge syndromedefective development of thymus
and parathyroid glands; and polyglandular syndromes.
Acquired
Postoperative hypoparathyroidism Damage to parathyroid gland or inadvertent removal during thyroid surgery.
Infantile hypoparathyroidism Transient, associated with
maternal hyperparathyroidism or calcium deficiency.
Idiopathic hypoparathyroidism Autoimmune disease of
adrenal, thyroid or ovary in young people.
Tetany
Tetany is defined as an increased excitability of peripheral
nerves due to hypocalcemia or alkalosis.
Causes
Clinical features
Calcifications of basal ganglia and extrapyramidal syndromes like choreoathetotic movement and dystonia are
more common and appear earlier. Tetany as consequence
of hypocalcemia, grand mal epilepsy, psychosis, cataracts,
papilledema, increased intracranial pressure and chronic
changes in finger nails, hair, alopecia and hypomagnesemia are seen. May be associated with autoimmune polyglandularcandidiasis syndrome.
Management
544
HYPOTHALAMUSPITUITARYADRENAL
AXIS
The main function of hypothalamuspituitaryadrenal
(HPA) axis is to maintain metabolic homeostasis and to
mediate the neuroendocrine stress response. An understanding of the axis is important especially for management of
conditions which need long-term corticosteroid therapy
and management of patients on long-term corticosteroid
therapy and adrenal insufficiency.
Hormone
Released from
Type
Actions
Hypothalamus
Peptide
Anterior pituitary
Pro-opiomelanocortin (POMC)
Anterior pituitary
Pro-opiomelanocortin (POMC)
B-endorphins
Anterior pituitary
Pro-opiomelanocortin (POMC)
Mineralocorticoids (aldosterone)
Glucocorticoids (cortisol)
Cushings Syndrome
The diagnosis of the condition presents two great challenges:
1.
2.
Glucose metabolism
Protein metabolism
Fat metabolism
545
Anti-inflammatory action
Psychic effect
Permissive effect.
Adrenal Insufficiency
Primary Adrenal Cortical Insufficiency
(Addisons Disease)
Primary adrenal cortical insufficiency is caused by a progressive destruction of adrenal cortex. The etiology is idiopathic or may result from hemorrhage, sepsis, infectious
diseases, malignancies, adrenalectomy or drugs leading to
deficiency of the major hormones cortisol and aldosterone.
The original description of Addisons diseasegeneral languor and debility, feebleness of hearts action, irritability
of the stomach, and a peculiar change of the color of the
skin.
Another specic symptom of primary adrenocortical
insufciency is a craving for salt. Although hyponatremia
occurs in both primary and secondary adrenal insufciency, its pathophysiology in the two disorders differs. In
the primary condition, adrenocortical insufciency is
mainly due to aldosterone deciency and sodium wasting,
whereas in the secondary form, adrenal insufciency is
due to cortisol deciency, increased vasopressin secretion
and water retention.
Signs and symptoms of adrenal insufficiency
3.
2.
3.
548
Minor surgical stress: 25 mg of hydrocortisone equivalent (refer to Table 5) on the day of surgery. For
instance, a patient on 5 mg of prednisone every day
may be given 5 mg of prednisone or 25 mg of hydrocortisone or equivalent preoperatively.
Moderate surgical stress: 5075 mg of hydrocotisone
equivalent may have to be given for 2 days. For
instance, a patient on 10 mg of prednisone daily should
be given 10 mg of prednisone (or parenteral equivalent) preoperatively and 50 mg of intravenous hydrocortisone intraoperatively. On the first postoperative
day 20 mg intravenous hydrocortisone can be given
8 hourly (60 mg/day). On the second postoperative day,
the dosage is then tapered to the preoperative maintenance level.
Major surgical stress: 100150 mg/day of hydrocortisone equivalent may be required for 23 days. For
instance, a patient on long-term 40 mg of prednisone/
day should receive 40 mg of prednisone (or parenteral
equivalent) preoperatively and 50 mg of hydrocortisone intravenously thereafter every 8 hourly for
4872 hours.
But a patient on long-term 5 mg of prednisone/day
should receive 25 mg of prednisone (or parenteral
equivalent) preoperatively and 25 mg of hydrocortisone
Table 6
Protocol for supplementation of patients on glucocorticoid therapy who are undergoing dental care
Protocol
Dental
procedure
Routine
procedures
No supplementation
needed
No supplementation
needed
No supplementation
needed
Aldosteronism
It is the hypersecretion of the mineralocorticoid aldosterone.
Treatment
Aldosterone antagonistsspironolactone25 to 100 mg TDS.
Secondary aldosteronism
Increased production of aldosterone in response to activation of the reninangiotensin system. It occurs in association with the accelerated phase of hypertension or on the
basis of underlying edema disorder due to overproduction
of renin.
It may present in many forms of edemacirrhosis,
nephrotic syndrome or cardiac failure and is characterized
by hypokalemic alkalosis, moderate to severe increase in
plasma renin activity and moderate to marked increase in
aldosterone.
Most important aspect is the precipitation of hypertensive crisis during surgery or trauma or any activity that
displaces abdominal contents. Orthostatic hypertension
and shock are expected. Any drug is not given unless a
thorough review is done.
Adverse reactions are seen with opioid, histamines,
adrenocorticotropin and glucagons that directly release
catecholamine from the tumor; drugs like tricyclic antidepressants that block neuronal uptake of catecholamines, and
indirectly acting sympathomimetic drugs like methyl dopa.
Sex Hormones
Androgens and estrogens are the essential hormones for
sexual development and fertility. The secretion of these
hormones is controlled by the HP axis, GnRH, LH and
FSH release.
The predominantly male sexual hormone, namely, the
androgens and testosterones regulate sexual differentiation, virilization and hormonal changes accompanying
puberty, development of testes, ultimately leading to spermatogenesis and puberty.
The female sex hormones, namely, the estrogen and
progesterone are essential for development of sexual characters, menstrual cycle and ovulation.
Pregnancy epulis
Recurrent aphthous ulcers
Herpes labialis lesions
Candida infections.
Idiopathic
Gonadal disease
Adrenal disease
Hypothalamic disease
(e.g. Gonadotropin independent/dependent precocious
puberty, McCuneAlbrights syndrome).
Increases vascular dilation, thus increases permeability (result in edema and accumulation of inflammatory cells)
Increases proliferation of newly formed capillaries in
gingival tissues (increased bleeding tendency)
Alters rate and pattern of collagen production.
550
PREGNANCY
Pregnancy is one condition where endocrine changes are
the most significant alterations due to production of
maternal and placental hormones.
Dental management guidelines during pregnancy
Prescription of drugs
It is always safe to avoid any drug during pregnancy especially during first trimester when the organs are in the formative stage. The drugs may cross the placental barrier
and may be toxic or teratogenic to the fetus since the liver
and kidneys are still immature.
In unavoidable circumstances the drugs that are
approved only may be given.
Anesthetics Local anesthetics with adrenaline are relatively safe with required amount eventhough they cross the
placental barrier. Lidocaine and prilocaine are safer than
bupivacaine, which can cause fetal bradycardia.
Analgesics The analgesic of choice is paracetamol. Ibuprofen is the next choice but is best avoided in the second
trimester. Other NSAIDs can constrict ductus arteriosus,
postpartum hemorrhage and delayed labor especially in
the third trimester. Prolonged high dose of opioids leads to
congenital abnormalities and respiratory depression.
Antibiotics Penicillins, erythromycin (except in etiolate
form) second and third generation cephalosporins, metroinidazole and clindamycin are considered safe. However,
an increased dose or more frequent administration may
be needed as the volume of distribution is increased in
pregnancy.
Tetracycline is contraindiacated as it chelates to
hydroxyapatite of developing teeth and causes enamel
hypoplasia.
Oral manifestations
2.
3.
70100% of patients. When chronic hypoparathyroidism develops during the neonatal period, it is important to differentiate this from genetic diseases such
as DiGeorges syndrome (caused by a 22q11 deletion)
KenneyCaffey disease (locus mapped to chromosome
1q42q43), or the Barakat syndrome (caused by
GATA3 haploinsufficiency).
Addisons disease (AD) and adrenal cortex autoimmunity: In the course of APS type 1, AD tends to be the
third disease to appear after chronic candidiasis and/
or hypoparathyroidism, and it develops usually before
15 years of age and affects 2293% of patients. In most
cases the disease is heralded by the presence of adrenocortical autoantibodies (ACA), frequently found at the
onset of the other main clinical manifestations of this
type of APS (candidiasis and/or hypoparathyroidism).
salivary testosterone levels may also be useful in behavioral studies of aggression, depression, abuse, violent and
antisocial behavior.
Estradiol can be detected in saliva in concentrations
that are only 12% of serum concentrations. These concentrations are similar to the serum concentrations of free
estradiol, which can diffuse into saliva. Salivary estradiol
levels followed the same trends as serum estradiol levels
during a menstrual cycle. Furthermore, salivary estriol
levels showed a very high correlation with serum levels of
free estriol in pregnant women, and salivary estriol levels
were suggested as a means for the assessment of feto-placental function.
Salivary progesterone levels showed good correlation
with serum levels during the menstrual cycle and reected
the free serum progesterone levels. Salivary progesterone
levels can be useful for the prediction of ovulation, with
serum progesterone levels, and salivary estradiol and progesterone levels can be used for the evaluation of ovarian
function. Decreased salivary estriol is suggested as a marker
of fetal growth retardation. Furthermore, an increased salivary estriol-to-progesterone ratio may be a predictor of
pre-term delivery.
In general, serum and salivary levels of protein hormones are not well correlated. These hormones are too
large to reach saliva by means of passive diffusion across
cells or by ultraltration, and the detection of these hormones in saliva is primarily due to contamination from
serum through GCF or oral wounds. Therefore, serum levels of protein hormones such as gonadotrophins, prolactin
and thyrotropin cannot be accurately monitored by means
of salivary analysis (Vining and McGinley, 1986, 1987).
Salivary monitoring of hormone levels has many advantages over the more conventional serum analysis. Hormone
evaluation often necessitates multiple sample collection in
a relatively short time interval, which makes the noninvasive collection of saliva ideal for this purpose. However,
it is important to consider the possible limitations of salivary analysis for hormone evaluation. Hormones enter
saliva by passive diffusion and ultraltration, and active
transport of hormones into saliva does not exist. Therefore,
mostly lipid-soluble and hormones with small molecular
weight can be detected in saliva. Most hormones are
protein-bound in serum, and thus salivary hormone levels
represent the free hormone levels which are available for
diffusion into saliva. This may provide more clinically useful information, since free serum hormone levels are the
biologically active fraction of hormone in serum. For accurate results, a constant and predictable correlation must
exist between salivary and serum hormone levels. However,
different hormones are bound to similar serum carrier proteins, and thus changes in levels of one hormone may
affect the free levels of others. For hormones that demonstrate a constant but low salivary-to-serum ratio, a sufciently large sample volume or a more sensitive analysis
553
History of Psychiatry
Tracing the timeline backwards, in 10,000 BC there was no
difference between medicine, magic and religion. Looking
at the history of medicine it may seem that Psychiatry as
a specialty is relatively new. However the history of Psychiatry dates back to more than 2,000 years when medicine itself had its birth as Science in ancient Greece. In
the ancient world, Psychiatric illness was believed to come
from the Gods and the curse of the devils. People have
always been fascinated and puzzled, not being able to
understand insanity. It is interesting to note the varying
554
Absence of health
Disease is what doctor treats
Biological disadvantage
Pathological process
Presence of suffering.
Axis II
Personality disorders
Axis III
Axis IV
Axis V
Criticisms of classifications
F10F19
F20F29
F30F39
F40F49
F50F59
F60F69
F70F79
Mental retardation
F80F89
F90F99
There is no doubt that classification is needed in psychiatry and it helps the clinicians to communicate with one
another about the diagnoses given to a patient and it helps
to understand the implications of the diagnoses, in terms
of their symptoms, prognosis, treatment and sometimes
etiology. However, such classifications, on many occasions
are criticized as being inappropriate or even harmful. It can
be argued that allocating patients to a diagnostic category
distracts form the understanding of their unique personal
difficulties. It is important to combine the two because these
qualities can modify prognosis and these personal difficulties should be taken into account for a holistic treatment
of the disorder. It is also worth noting that some conditions
could be sub-threshold and resemble the disorders in the
classification but do not meet full diagnostic criteria. It is
not infrequent to see such patients presenting to a variety
of services other than psychiatry. These sub-threshold conditions are however clinically significant and it is important to recognize these conditions to reduce the psychiatry
morbidity or comorbidity.
oral condition it becomes important to liaise with the mental health services to treat the dependence. We aim to equip
you to diagnose these dependence syndromes with the diagnostic criteria which are common for all substances.
Three or more of the following to be present during the previous
year
1. Strong desire or a sense of compulsion to take the substance
(craving)
2. Difficulty in controlling substance taking behavior (loss of control)
3. Physiological withdrawal symptoms when substance use is reduced,
e.g. tremousless, anxiety and lack of sleep with alcohol withdrawal
4. Need to increase the intake over a period of time to get the same
effect (tolerance)
5. Neglect of alternative pleasures or interests (salience)
6. Continuing to use despite knowing it to be harmful
World Health Organization, 1992.
lasts for more than 2 weeks duration. It has to be differentiated from normal sadness. Such patients have impaired
patterns of mood, thoughts and behavior which sometimes
lasts for a long period of time. It causes a lot of distress to
the person and impairs his/her quality of life. It is also
accompanied by a high rate of suicide which is at least 7%
in men and 1% in women (Blair-West et al, 1999; Weissman
et al, 1996). The 12-month prevalence of depression in the
community is between 2 and 5% and the lifetime rate lies
between 10 and 20% (Alonso et al, 2004). In 2000, the
WHO identified major depressive disorder as the fourth
ranked cause of disability and premature death in the
world (Murray and Lopez, 1997). WHO has projected that
by 2020, major depression will rise in disease burden to be
second only to ischemic heart disease. This disorder is very
common all over the world with a lifetime prevalence rate
of 17% and a recurrence rate of more than 50% (Kessler
et al, 1994; Weissman et al, 1996). People with chronic disease have a higher chance of developing depression. It is
40% for people with coronary artery disease and 25% for
patients with cancer (Musselman et al, 1998; Patten, 1999).
Neurological disorders associated with a higher frequency
of depression include multiple sclerosis, Parkinsons disease, head trauma and stroke (Patten et al, 2000; Poewe
and Luginger, 1999). Around one-third of patients with
depression develop alcohol or illicit substance misuse in
their lifetime (Baker and Dawe, 2005).
Feeling sad most of the time (but may feel a little better in the
evening)
Lose interest and enjoyment in life (anhedonia)
Feeling tired easily with reduced energy levels
Amphetamines
Cocaine
Speedball
Heroin
Cannabis, hashish
LSD
Ecstasy, ketamine
Inhalants
Steroids
Ether
GHB
Glue
Take 12 hours to get off to sleep, and then wake up earlier than
usual
THC, marijuana
Speed
Loss of self-confidence
Opium
Crack
Morphine, methadone
MDA, MDMA
Crystal meth
Grass
Freebase
Feel irritable
Feel worse at a particular time each day, usually in the morning
Depression
Depressive disorder also known as major depressive disorder or unipolar depression is a psychiatric illness which
556
557
a cliff is fear. Both fear and anxiety can be helpful, helping us to avoid dangerous situations, making us alert and
motivating us to deal with problems. However, if it becomes
too strong or goes on for too long it can interfere with our
daily activities and make our lives miserable.
Anxiety causes numerous physiological (in the body)
symptoms and cognitive symptoms (in the mind) shown in
two separate boxes below.
Physiological symptoms of anxiety
Irregular hear beats (palpitations)
Sweating
Muscle tension and pain
Shakes and tremors
Butterflies in the stomach
Tightness in the chest
Breathing heavily
Dizziness
Faintness
Indigestion
Diarrhea
take to avoid the fearful situation. This avoidance behavior keeps expanding to include more and more situations
and circumstances. The sufferer usually knows that there is
no actual danger, may feel silly about this fear but still is
unable to control it. Such a phobia can start after a distressing or a traumatic experience, many a times in early life.
Many of us worry before meeting new people and before
going to parties but once we are there we can cope and
enjoy the situation. Some people become very anxious about
such situations, cannot enjoy them and at worse totally
avoid such social situations. This condition is called social
phobia. Such people tend to worry about becoming the
center of attention wherever they nd themselves among
people. They worry that everybody is looking at them and
watching what they are doing. At their worst, these feelings of fear and bodily symptoms can end up in a panic
attack. A panic attack lasts for a few minutes during which
the person feels overwhelmingly anxious and is terried
of losing control, going mad or dying. These feelings reach
a peak and then pass off rapidly leaving the person feeling
weak and exhausted.
Panic disorder
Panic disorder is a type of mental illness with recurrent
and unexpected intense episodes of anxiety called as panic
attacks. A panic attack is characterized by intense apprehension and terror accompanied by physical symptoms
like palpitations, chest pain, dizziness, sweating and difficulty in breathing. These attacks have an abrupt onset
and peak in intensity within 10 minutes. These attacks are
not associated with any external event or situation and
come out of the blue. Such episodes often result in calls to
paramedics and visits Accident and Emergency Department.
Panic disorder could sometime be a lifelong illness that
remains only partially responsive to treatment. Women
appear to have more severe form of the disease than men
(Yonkers et al, 1998) and is much more commoner in women
(Barzega et al, 2001; Kessler et al, 1994).
Agoraphobia
The term agoraphobia means fear of open spaces. Such
people not only fear open spaces but also presence of a
crowd. They fear not having an immediate and easy escape
to a safe place (usually home). Such patients generally
have a fear of leaving home, fear of entering shops, crowds,
public places, or of traveling alone in trains, buses, or
planes. The severity of anxiety symptoms and the extent
of avoidance varies from patient to patient. However, for
some people it is the most incapacitating of all phobic
disorders and they become completely housebound. Some
people are terrified by the thought of collapsing and being
left helpless in the public. The lack of an immediately
available exit seems to be a key feature in these patients
with agoraphobia who tend to be women in early adult life.
Schizophreniadental implications
Dentists who are familiar with the signs and symptoms of
schizophrenia are likely to feel more secure while treating
patients with schizophrenia. It makes the dentist more confident while obtaining consultative advice from the patients
psychiatrist. Dentists can provide the full range of service
to such patients and constitute to the psychotherapeutic
aspect of management. In addition to being able to communicate effectively with such patients, the dental treatment
may need to be modified because of the patients impaired
ability to think logically. It is very important for all dentists
to be aware of the local and systemic effects of psychiatric
medications and the adverse interactions between these
drugs and medications used in dentistry.
Schizophrenia can be conceptualized as a group of disorders, with variable presentations, best described in three
different dimensions: positive symptoms, disorganized symptoms and negative symptoms. The positive symptoms are
the exaggeration or distortion of normal functions, most
commonly delusions and hallucinations which are described
earlier in the chapter. Disorganized symptoms are inferred
from patients speech and are also known as thought disorder. It is manifested as rapid shifts between topics that
either have a loose logical association or are completely
unrelated. They may also exhibit inappropriate affect manifested by childish silliness and unpredictable agitation.
(Andreasen et al, 1995; Marder, 1996). They also exhibit a
range of neurocognitive impairments in the areas of memory, attention and executive functions, which in turn impairs
their vocational and social adjustment (Green, 1996).
Negative symptoms are less dramatic but equally debilitating. They include reduced emotional responsiveness (at
affect); reduced speech output and poor thought content;
561
inability to plan, initiate and persist in goal directed activities (avolition) and poor self-care in terms of washing,
bathing and cooking. They also show social withdrawal
and loss of pleasure in previously enjoyed activities (anhedonia) (Kaplan, 1994 and Sadock, 1995).
Substance abuse frequently accompanies schizophrenia. The prevalence rate of alcohol abuse in schizophrenia
is as high as 63% in some studies (Dixon, 1999). Other
commonly abused drugs are cannabis and cocaine. Some
patients seem to self-medicate their positive symptoms
with these illicit drugs which in turn makes the psychosis
worse. Abuse of these substances strongly correlates with
medical and psychosocial problems like inadequate diet,
homelessness, poor compliance with medications and
multiple episodes of the disorder (Dixon et al, 1999). More
than three-fourths of patients with schizophrenia smoke
and this is frequently associated with emphysema, lung
cancer, cardiac disease and oral cancer (McEvoy and
Brown, 1999).
Patients with schizophrenia are often having a disinterest in performing appropriate preventive oral hygiene
techniques (Friedlander and Liberman, 1991; Thomas et al,
1996). This in turn leads to the development of advanced
oral disease. This is compounded by reduced access to
care, prolonged hospitalizations, impaired nances and
paucity of clinicians who are comfortable in caring such
people. Some antipsychotics, namely, chlorpromazine and
thioridazine cause profound hyposalivation through their
anticholinergic effects (Sreebny, 1989). This is worsened
by the co-administration of antiparkinsonian agents to
counteract the extrapyramidal side effects (EPSE) of the
conventional (dopamine agonist) antipsychotics. The resultant hyposalivation causes intensication of periodontal
disease and rapid caries progression (Gupta et al, 1993).
Therefore, a lot of people with schizophrenia have a higher
requirement for periodontal treatment, dental restorations
and dental extractions (Velasco et al, 1997). The conventional antipsychotics have adverse side effects, especially
the movement disorders that mimic neurological diseases.
These movement disorders frequently have an orofacial
component and are seen to develop in a time-dependent
fashion. These movement disorders are caused by the blockade of basal ganglia dopamine D2 receptors in the extrapyramidal system (Holloman and Marder, 1997).
Effects of antipsychotic drugs on orofacial movements
Acute diagnoses which generally manifest within the first 5 days of
starting treatment:
562
Patients with bulimia nervosa often present with bilateral and occasional unilateral parotid gland swelling. The
incidence of parotid gland swelling is 1015% in people
with bulimia (Brady, 1985). The submandibular salivary
gland is involved infrequently. The exact pathogenesis of
these glandular enlargements has not been determined. It
is generally accepted that multiple emetic episodes cause
an autonomic neuropathy (Ascoli et al, 1993). With sympathetic nerve impairment, individual acinar cells enlarge
and lead to clinically visible gland swelling (Ascoli et al,
1993). Such an asymptomatic bilateral parotid enlargement
often presents a diagnostic dilemma to the dentists. As these
patients with bulimia nervosa and parotid gland swelling
are usually secretive about their self induced vomiting (SIV)
(purging), the diagnosis will have to be made by conducting a thorough clinical examination and serum electrolyte
study. Early recognition and prompt diagnosis when such
patients present to the dentists can avoid the later serious
medical complications. The need for these patients to seek
psychiatric care and discontinue SIV is mandatory.
Eating disorders have various orodental adverse effects.
Holst and Lange in 1939 coined the term perimylolysis to
describe the distribution of erosion on the upper palatal
surfaces secondary to vomiting, reux and regurgitation
(Holst and Lange, 1939). Several research studies till date
have shown that SIV results in increased frequency of
erosion on palatal surfaces (Hellstrom, 1977). Whether the
caries experience in eating disorder individuals is greater
than in normal population remains unclear (Hurst et al,
1977). Salivary ow increases dramatically prior to vomiting because the medullary center that controls vomiting is
connected to salivary nuclei (Edgar, 1992). With respect to
SIV, the stimulated salivary ow should therefore be altered.
Research has discovered reduced bicarbonate in bulimics
along with increased salivary viscosity (Edgar, 1992). One
study has found increased frequency of periodontal disease in patients with eating disorders (Touyz et al, 1993).
Angular cheilitis, candidosis, glossitis and oral mucosal
ulceration are possible sequelae of nutritional deciency.
There has not been any report of malignant change associated with SIV (Brady, 1980).
The dental care demanded by individuals with eating
disorders is very challenging. Although the dentist might
suspect vomiting as the cause of erosion, these patients will
not readily admit to such behavior because they can be
highly secretive and embarrassed by it. The patients motivation to reduce the frequency of SIV will increase once
the dentist is able to openly relate the progress of dental
erosion with the vomiting. Toothbrushing after vomiting
is generally regarded as inadvisable because the softened,
demineralized surface is more susceptible to toothbrush
abrasion (Milosevic et al, 1997; Robb et al, 1995).
Patients whose teeth have been damaged as a consequence of an eating disorder are most likely to present rst
to the dentists. In many cases the dentist is in a position to
564
MANAGEMENT OF PSYCHIATRIC
DISORDERS
Broad Principles
Some patients who receive psychiatric treatment for mental health problems may be reluctant to admit it. This is
most often because of the perceived stigma associated with
mental illness. It is important and could be quite tricky for
a dentist to overcome such barriers and obtain the necessary information. It always helps to take a supportive and
non-judgmental attitude, and advise patients that such
information will be held confidential and also that it is
indispensable to provide a safe dental care.
Patients with mental health problems may be uncooperative and irritable during dental treatment. They may
appear unappreciative and may seem to have numerous
complaints that are inconsistent with the objective ndings
(Korszun and Ship, 1997). It is always benecial to liaise
with the patients psychiatrist before beginning any dental
treatment. Information requested should include at least
the latest mental state, risk assessment and the list of psychotropic medications. In addition, a history of alcohol
and illicit drug use is always useful. Such patients should
undergo a liver function test, full blood count and coagulation prole before commencing the dental treatment.
Medications
Antidepressants
Antidepressants are drugs that relieve symptoms of depression. They were first developed in 1950s and have been used
regularly since then. There are almost 30 different kinds of
antidepressants which belong to the four major categories:
tricyclics, mono amine oxidase inhibitors (MAOIs), SSRIs
and serotonin noradrenaline reuptake inhibitors (SNRIs).
Antidepressants work by increasing the activity of certain
neurotransmitters mainly serotonin and noradrenaline.
Antidepressants are used to treat moderate to severe depressive illness, severe anxiety and panic attacks, OCD, chronic
pain, eating disorders and PTSD.
The tricyclic antidepressants such as imipramine, amitriptyline, nortryptiline and dosulepin cause side effects
such as dry mouth, slight tremor, tachycardia, constipation,
sleepiness and weight gain. In addition to these anticholinergic side effects, men may experience delayed ejaculation
and difculty in getting or keeping an erection. Tricyclic
antidepressants are less commonly used as they are dangerous in overdose.
565
antipsychotics include risperidone, olanzapine, quetiapine, amisulpiride and clozapine. Risperidone is the only
atypical antipsychotic which is available in the form of
long acting injection.
Clozapine is an atypical antipsychotic medication and
the only one that has shown to be more effective for people who do not respond to other sorts of antipsychotics.
In addition to the side effects of the atypicals mentioned
above, clozapine also produces increased salivation. The
main drawback is that it can affect the bone marrow reducing the white cell count causing agranulocytosis which
can be fatal. For this reason, patients taking clozapine need
weekly full-blood count for the rst 6 months, 2 weekly for
the next 6 months, and 4 weekly thereafter.
Most patients with schizophrenia need to take antipsychotics for a long time. On stopping the treatment, symptoms
of schizophrenia usually come back, if not immediately,
often within 6 months. It is advisable to reduce the dose of
the medication gradually, only in discussion with a psychiatrist who will monitor for early signs of relapse.
Antipsychotics
Starting from the mid 1950s several medications were
discovered that reduce the symptoms of schizophrenia
and other psychotic disorders. They came to be known as
antipsychotic medications. These older drugs are called
typical or first generation antipsychotics. They work by
reducing the action of dopamine in the brain (dopamine
antagonist). Some typical antipsychotics are chlorpromazine, haloperidol, pimozide, trifluoperazine and sulpiride.
Some of these typical antipsychotics are available in long
acting depot injection form which can be administered
once in 2 to 4 weeks for people who do not comply with
oral medications. The typical antipsychotics have more
side effects than atypicals. The side effects include stiffness and shakiness as in Parkinsons disease along with
feeling sluggish and slow in their thinking. Other side
effects include uncomfortable restlessness (akathisia) and
sexual side effects. A long-term side effect is tardive
dyskinesiapersistent movements generally of the mouth
and tongue which is difficult to treat and very disabling.
Over the last 10 years, several newer medications have
been in use. They work on a different range of chemical
messengers in the brain including the serotonin system
(serotonin dopamine antagonists). These came to be known
as atypical or second generation antipsychotics. These
atypical antipsychotics are less likely to cause parkinsonian side effects although they may cause weight gain
and problems with sexual functions. They may also help
the negative symptoms on which the older drugs have
very little effect. They also seem to have much less propensity to cause tardive dyskinesia. The common side
effects of atypicals include weight gain, sexual side effects,
glucose intolerance and increased chance of developing
Type 2 diabetes and sedation. The commonly used atypical
566
Anti-manic agents
Medications used to treat mania include mood stabilizers
such as lithium and valproate. Other medications used to
treat mania include antipsychotics and benzodiazepines.
Medications used to prevent the relapse of manic episodes
in manic depression (bipolar disorder), include drugs such
as lithium, valproate, carbamazepine and lamotrigine.
Lithium has over the last 40 years been the most commonly used drug to prevent relapse. Lithium is a safe drug
when taken at the correct dose, however it has a narrow
therapeutic window (0.6 to 0.8 mmol/l) and becomes unsafe
above this level in the blood. The common side effects
include ne tremors, metallic taste in the mouth, tiredness,
weight gain and underactive thyroid gland. Long-term treatment with lithium can cause renal impairment. It is thus
important to periodically check for serum lithium level,
thyroid functions and renal functions including creatinine
clearance. Lithium is an ion and is excreted unchanged from
the kidneys. Drugs such as diuretics and NSAIDs can dangerously increase the serum lithium levels and so it is very
important to check for drug interactions before prescribing
for a patient on lithium.
Valproate and semi-sodium valproate are becoming
widely used treatment for mania and bipolar disorder. The
common side effects of valproate include sleepiness, dizziness, increased appetite and weight gain, skin rashes and
irregular periods. Very rare side effects include pancreatitis and liver failure. It is again very important to check for
drug interactions as valproate is a hepatic enzyme inhibitor and reduces the metabolism of other medications.
Carbamazepine is usually used as a second line treatment
for bipolar disorder. Unlike valproate, carbamazepine is
a hepatic enzyme inducer and increases the metabolism
of other medications and thus reduces their efcacy. Interestingly it reduces its own level and needs bigger doses with
longer treatment. Lamotrigine also helps to prevent mood
swings particularly of severe depressive episodes. It is however not used as a monotherapy for bipolar disorder.
Electroconvulsive therapy
Electroconvulsive therapy (ECT) is a treatment used in
psychiatry for severe mental illnesses. It was originally
developed in the 1930s and was used widely during the
1950s and 1960s for a variety of conditions. Since then its
use was declined. ECT remains a controversial treatment,
which some people have strong feelings about. There are
those who claim it can be a lifesaving procedure, while
others feel it should be banned. ECT is a way of causing
someone to have a seizure and it is this seizure that is
needed for the treatment to work. The seizure is made to
happen by passing an electric current across the persons
brain in a carefully controlled way from a specially developed ECT device. The current can be administered to the
whole brain when it is called bilateral ECT or just to the
non-dominant hemisphere called the right unilateral ECT.
The seizure itself is very similar to the seizures that occur
in people with generalized epilepsy, but it is caused on
purpose in very controlled circumstances using generalized anesthesia and muscle relaxant, just like for a surgical
operation. The aim of ECT is to cause a generalized cerebral seizure between 10 and 50 seconds long using the
right dose of electricity. The current recommendation is to
use ECT for treatment resistant severe depression, severe
mania and catatonia (NICE, 2003). ECT is used more as a
lifesaving treatment for quick resolution of very severe
symptoms and is always supplemented with a continuation of the most appropriate pharmacotherapy.
Psychological (talking therapies)
There are several types of talking treatments also known
as psychotherapy. These are different ways of helping people to overcome stress, emotional problems, relationship
problems and troublesome habits. The commonality in these
treatments is talking to another person and sometimes
567
CHAPTER
19
Fibro-Osseous Lesions
568
Bone Diseases
Osteogenesis Imperfecta
Osteopetrosis
Cherubism
Infantile Cortical Hyperostosis
Idiopathic Osteosclerosis
Gorhams Disease
Pagets Disease of Bone
FIBRO-OSSEOUS LESIONS
most bro-osseous jaw lesions can be assigned with reasonable certainty into one of the several categories:
I.
II.
Fibrous dysplasia
Reactive (dysplastic) lesions arising in the tooth-bearing
area
A. Periapical cemento-osseous dysplasia
B. Focal cemento-osseous dysplasia
C. Florid cemento-osseous dysplasia
III. Fibro-osseous neoplasms: Cementifying fibroma, ossifying fibroma or cemento-ossifying fibroma
Another classification which attracted many pathologists
was given by Fowler. He classified fibro-osseous lesions as
follows:
I.
II.
Osseous dysplasia
A. Non-hereditary
1. Periapical osseous dysplasia
2. Focal osseous dysplasia
3. Florid osseous dysplasia
B. Hereditary
1. Familial gigantiform cementoma
Fibro-osseous neoplasms
A. Conventional ossifying fibroma
B. Juvenile, active, or aggressive forms of ossifying fibroma
Gigantiform Cementoma
Gigantiform cementoma is a rare, benign fibro-cementoosseous disease of the jaws. It is characterized by formation
570
Etiology
Ossifying fibroma occurring in the jaw seems to arise from
the periodontal membrane, which contains pluripotential
cells capable of forming cementum, bone and fibrous tissue.
Clinical features
Ossifying fibroma shows a definite female predilection,
with the mandible (premolarmolar area) involved more
than maxilla in most of the cases. These occur mostly in
the 3rd and 4th decades of life. The lesions are restricted
571
Histopathogic features
Microscopic examination of psammomatoid juvenile ossifying fibroma shows well demarcated but unencapsulated
lesion composed of numerous small rounded mineralized
collagenous bodies (psammomatoid ossicles) uniformly
distributed within a cellular fibroblastic stroma. The cellularity of the fibroblastic stroma varies in different tumors
and at different sites within the same lesion. Occasional
shrunken cells, representing nuclei of osteocytes, are embedded within the ossicles, and there is usually a collagen outer
band to these mineralized bodies. Some of the ossicles
may show a basophilic center and eosinophilic fringe. In
the periphery of the lesions, some of the ossicles show a
transition into small bone trabeculae. The tumor infiltrates
and destroys the adjacent bone with focal induction of
reactive bone formation. Development of aneurysmal cyst
is followed by focal myxoid change in the stroma with hemorrhage and osteoclastic giant cells, with gradual expansion and formation of cysts with thin fibrous walls. The
differential diagnosis with other fibro-osseous lesions of
the jaw, such as cemento-ossifying fibroma, osteoid osteoma or bone dysplasia, should be made with a mandatory
pathological study, and is largely based on the nature of
the calcified products of the tumor. The mineralized tissue
of central ossifying fibroma is composed of a variable
combination of mature and immature bony trabeculae and
lobulated basophilic masses of cementum-like material.
There may be occasional concentrically laminated particles,
called cementicles, but these are not a prominent feature
of psammomatoid entity.
Microscopic features of trabecular variant of juvenile
ossifying broma shows an unencapsulated tumor mass
inltrating into the surrounding bone and reactive bone
formation at the periphery. The tumors show a characteristic loose structure with cell-rich stroma composed of
broblastic spindle cells that produce little collagen.
Anastomosing trabeculae of osteoid is seen in a pattern
that resembles paintbrush strokes. The osteoid areas
mature into woven bone trabeculae that are rimmed with
osteoblasts and show osteocytes embedded in it. Aggregates
of osteoclastic giant cells are commonly present and seen
in association with the bony trabeculae and in separate
foci in the brous stroma, usuallybut not alwaysat sites
of hemorrhage. Mitotic gures may be observed in the
stroma but are never numerous. Cystic degeneration and
aneurysmal bone cyst formation have been described in a
few cases.
Management and prognosis
Recurrence after surgical management is common and is
reported to range from 30 to 56% in psammomatoid juvenile ossifying fibroma. Recurrence may be attributed to
difficulty in proper resection caused by the location of
the lesion and the infiltrative nature of tumor borders.
Fibrous Dysplasia
Fibrous dysplasia of bone is an uncommon congenital skeletal disorder. It is characterized by the replacement of normal bone and marrow by fibrous tissue, within which
irregular trabeculae of woven bone are haphazardly distributed. The maturation of bone is arrested at the woven bone
stage. It may affect single (monostotic) or multiple bones
(polyostotic) and may be associated with endocrinopathies.
A paper by von Recklinghausen in 1891 was probably
the rst citation of the disease. Albright pointed out two
cases that were described by von Recklinghausen as
examples of osteitis brosa generalisata due to hyperparathyroidism were almost certainly examples of brous dysplasia. The term brous dysplasia was rst suggested by
Lichtenstein in 1938 as a designation for multiple bone
lesions that were described by Albright et al as osteitis
brosa generalisata. The lesions of brous dysplasia were
initially considered to be primarily polyostotic. Lichtenstein
and Jaffe later expanded this concept and noted that isolated (monostotic) form of disease also occurred and was
by far more common than the polyostotic. McCune and
Albright in 1936 and 1937 respectively showed an association of polyostotic brous dysplasia with abnormal skin
pigmentation, and precocious puberty because of which
this association has been termed as McCuneAlbrights
syndrome. JaffeLichtenstein described the association of
polyostotic brous dysplasia with abnormal skin pigmentation; thus it was named as JaffeLichtenstein syndrome.
Etiology
The molecular mechanism responsible for fibrous dysplasia
is a postzygotic activating mutation of the GNAS1 gene
that encodes for the Gs subunit of the heterotrimeric
G protein complex. The result is constitutive activation of
the adenylyl cyclase enzyme and overproduction of
3,5-cyclic adenosine monophosphate. The most common
GNAS1 gene mutations are a replacement of arginine by
either cysteine or histidine at codon 201 (R201C or R201H),
but other mutations have also been identified. The severity
of the disease phenotype is thought to depend on when the
mutation occurs during embryogenesis. If the mutation
occurs during the formation of the inner cell mass, all three
germ cell layers will be affected and the phenotype will be
573
McCuneAlbrights syndrome. If it occurs later in development, only one or two germ cell layers will be affected and
the phenotype is less severe. Fibrous dysplasia is considered a disease of cells of the mesenchymal stem cell/
osteoblastic lineage in which excess cyclic adenosine monophosphate impairs the ability of the stem cell to differentiate
into a mature functioning osteoblast.
Clinical features
Fibrous dysplasia is mainly diagnosed before the age of
30 years and is equally seen in both the sexes. It occurs
both in polyostotic and monostotic forms. Monostotic
form is at least six times more common than polyostotic
form.
In polyostotic form of disease two or more bones are
affected generally the long bones, ribs and skull, although
any bone may be affected. The lesions are often found
unilaterally. Painless expansion of the affected area is most
common complaint of the patient. Pathological fracture with
resultant pain and bone deformity are other symptoms.
Presence of abnormal skin pigmentation with polyostotic
brous dysplasia is termed as JaffeLichtenstein syndrome. The abnormal hyperpigmentation seen resembles
caf-au-lait spots which mean coffee with milk. The hyperpigmentation tends to be present on the same side and on
the skin overlying the lesions of brous dysplasia. The
hyperpigmented macules are well-dened and have irregular borders which sometimes distinguish them from the
lesions associated with neurobromatosis. The color can
be medium to dark brown.
Association of endocrine abnormalities with skin
hyperpigmentation and polyostotic brous dysplasia is
termed as McCuneAlbrights syndrome. A variety of
endocrine abnormalities such as accelerated skeletal growth,
acromegaly, gigantism, hyperprolactinemia, Cushings
syndrome, hyperthyroidism, hyperparathyroidism, diabetes mellitus, hypothalamic hypogonadism and hypophosphatemic rickets, gynecomastia, spermatogenesis in young
boys and sexual precocity in girls have been associated
with the disease. Sexual precocity is the most common
endocrine abnormality seen in the affected individual.
Male children exhibit enlarged genitalia and advanced
secondary sex characteristics. Female children manifest
estrogen excess.
Monostotic form of the disease is seen in 8085% of
cases. The jaws are the most common areas affected, with
the maxillary jaw being more common than the mandibular jaw. The maxillary lesions frequently involve a group
of contiguous bones separated by sutures (i.e. maxilla,
zygoma, sphenoid and occiput) and thus are not strictly
monostotic lesions. Such lesions are more appropriately
classied as craniofacial brous dysplasia. The craniofacial region is involved in up to 25% of the patients with
monostotic brous dysplasia, and in approximately 4060%
574
Figure 2
Histopathologic features
Laboratory investigations
Figure 4
575
BONE DISEASES
The bones of the facial skeleton particularly the maxilla
and mandible are affected by various diseases. These diseases present with a myriad of clinical features and characteristic oral manifestations. This section will attempt to
highlight common bone diseases affecting the maxillofacial
skeleton.
Osteogenesis Imperfecta
Osteogenesis imperfecta has also been referred to as
brittle bone disease, Vrolik syndrome and EkmanLobstein
syndrome.
Osteogenesis imperfecta is an inherited disorder of the
connective tissue having an autosomal dominant pattern
of inheritance. However, autosomal recessive and nonhereditary types have also been known to occur. The condition is characterized by fragile bones that tend to break
easily, often from the mildest of trauma.
Pathophysiology
The primary pathology in osteogenesis imperfecta is the
disturbance in the synthesis of type I collagen (predominant
protein of the extracellular matrix of most tissues). In bone,
this defect of extracellular matrix causes osteoporosis. The
affected bones become weak and fragile thereby making
them susceptible to fracture. Type I collagen is also a
major constituent of dentin, sclerae, ligaments, blood vessels and skin; therefore, abnormalities of these structures
576
Clinical features
Type I It is the most common and mildest form of osteogenesis imperfecta. These patients present with normal or
close to normal stature. They may have muscle weakness
and hypermobility of joints. Since the bones are fragile
they tend to fracture easily and most of the fractures occur
before puberty. Fragility of the walls of the blood capillaries may be seen. The patients may present with a triangular shaped face. Some authors describe the facies to be
similar to that of cleidocranial dysostosis. The skull may
be disproportionately large with a temporal bulge thereby
causing the ears to be pushed outward and forward. The
forehead is broad with frontal bossing giving rise to a
mushroom-shaped skull.
Hearing loss due to osteosclerosis, may be usually seen
in the 2nd and 3rd decades of life. These patients typically
present with blue, purple or gray tinted sclera (Figure 5).
The sclera in these individuals tends to be extremely thin,
therefore allowing the color of the underlying choroid to
be transmitted. Most of the patients have normal teeth
whereas some may present with opalescent brittle teeth.
Histologically collagen has normal structure but the quantity of the collagen present is usually less than normal.
Type II It is the most severe and lethal form of this condition. Most infants are still born or die shortly after birth.
The death may occur mostly due to respiratory distress or
intracerebral hemorrhage. It is estimated that almost 90%
of the infants do not live longer than a month. Severe bone
deformity and multiple fractures are evident. These individuals have a small stature with underdeveloped lungs.
Blue colored sclera is seen. The collagen that is formed is
insufficient in quantity and is of poor quality. Dentinogenesis imperfecta may be seen.
Based on the radiographic ndings of the long bones
and ribs, Type II osteogenesis imperfecta is subdivided into
groups A, B and C. Type IIA demonstrates broad and short
long bones with broad and beaded ribs. Type IIB shows
broad and short long bones with thin ribs that have little
or no beading. Type IIC group shows thin and longer long
bones with thin and beaded ribs.
Type III In Type III osteogenesis imperfecta the bones
are extremely fragile and hence have an increased tendency to fracture. Fractures are often present at birth.
These individuals are usually short statured. The sclera has
a bluish tint. It has been reported that the mortality rate is
generally higher at adolescence. Kyphoscoliosis causes
cardiopulmonary distress resulting in death. A barrelshaped rib cage is seen. The patients may have a triangular
face. Hearing loss can be seen; some patients may present
with brittle opalescent teeth. Histologically the collagen is
improperly formed.
Type IV Individuals suffering from Type IV osteogenesis
imperfecta may show mild to moderate bone fragility. It is
considered that the severity of this form of the condition
is in between the severity of Type I and Type III osteogenesis imperfecta. These individuals may present with a mildto-moderate bone deformity and a slightly shorter than
average stature. Bones are fragile and fractures are usually
present before puberty. The sclera is generally white (normal) in color. Some patients may present with a faint blue
tint. Triangular face and barrel-shaped rib cage is usually
seen. Teeth may be brittle and appear opalescent in some
patients. Hearing deficits may be encountered. Histologically there is improper formation of collagen.
Type V It has a dominant inheritance pattern. Individuals
of this form of the condition do not exhibit mutations in
type I collagen genes. It mimics the symptoms and signs of
Type IV osteogenesis imperfecta.
The site of fracture shows hypertrophic calluses. Radiographs of long bones reveal a radiodense band adjacent to
the growth plate. The sclera is white and teeth are normal.
The characteristic microscopic nding is the presence of
mesh-like pattern of bone.
Figure 5
Type VI This subtype of osteogenesis imperfecta mimics
the symptoms and signs of Type IV. It is believed to be
inherited as a recessive trait.
The characteristic sh scale appearance of bone under
a microscope is unique for this type of osteogenesis imperfecta. Blood investigations reveal minimally elevated levels of alkaline phosphatase.
Orofacial manifestations
Patients suffering from this condition can present with triangular facies as a result of the soft craniofacial bones
along with a large and thin calvarium.
Osteogenesis imperfecta can be associated with dentinogenesis imperfecta. The color of teeth affected by
dentinogenesis imperfecta may vary from blue to gray to
brown. The outer enamel may often chip off to reveal the
underlying soft dentin.
Other skeletal and dental disturbances noted are the frequent presence of a skeletal class III malocclusion (especially
in type III and type IV osteogenesis imperfecta) and anterior
and posterior crossbites.
Radiographic features
General radiographic findings The common feature in
all forms of osteogenesis imperfecta is osteoporosis. In the
milder forms of the disease, the bones exhibit thin cortices
and minimal fractures. However, in severe forms of the
disease the long bones are stunted and exhibit extensive
fractures. The skull is poorly mineralized and exhibits
wormian bones. Spinal deformities such as flattened spinal
bones (platyspondyly) and S-shaped scoliosis. Other characteristic features associated with osteogenesis imperfecta
include cod fish vertebrae, popcorn calcifications in the
metaphyseal-epiphyseal region of long bones, especially
of the knee and ankle caused due to repeated microfractures at the growth plate, basilar invagination or impression (projection of the tip of the odontoid process above
the McGregor line) and Tam OShanter skull (caused by
large and thin cranium with platybasia).
Radiographic findings specific to oral cavity Teeth may
have bulbous crowns constricting at the cervical portion
and stunted roots. The pulp chamber and radicular portion
of the pulp may be partially or totally obliterated.
Dental considerations
Since most patients especially in the younger age group
have high susceptibility for fractures, excessive force should
best be avoided during extraction of teeth. Tooth wear
may be managed esthetically with restorations or crowns.
Syndrome association
Datta and others (2005) reported a 34-week pre-term baby
suffering from Bruck syndrome, which is a combination of
arthrogryposis multiplex congenita (congenital contractures
578
Management
Osteopetrosis
Osteopetrosis is also known as marble bone disease
and AlbersSchnberg disease. It was first described by
AlbersSchnberg, a German radiologist in 1904.
Osteopetrosis is a rare disorder of bone that arises from
defective differentiation and function of osteoclasts and
reduced generation of superoxide by leukocytes. As the
osteoclasts fail to resorb bone, the bones become dense
and fragile. Bone fragility occurs because very few collagen bers connect osteons adequately and the remodeling
of woven bone to compact bone is defective. This, in turn,
will make bones vulnerable to fracture.
The actual incidence of osteopetrosis is unknown.
However, Beighton et al (1979) reported that the overall
incidence of osteopetrosis was approximately one case in
100,000500,000 population.
Pathophysiology
The gene for adult osteopetrosis has been mapped to chromosome 1p21 (Van Hul, 1997).
Whyte (1999) described few probable reasons for the
functional failure of osteoclasts such as abnormalities in
the osteoclast stem cell or its microenvironment, osteoblast
precursor cells or the mature heterokaryon or in the bone
matrix.
Other factors that may predispose to altered bone
resorption that may inuence osteoclastic activity include
synthesis of abnormal parathyroid hormone and defective
production of interleukin-2 (IL-2). Hofbauer (1999) reported
osteopetrosis in cathepsin K (cysteine protease important
for osteoclast function) decient mice.
Types
Three distinct clinical forms of osteopetrosis are recognized:
1.
2.
3.
Cherubism
Cherubism is a non-neoplastic hereditary bone lesion that
is histologically similar to central giant cell granuloma.
It affects the jaws of children bilaterally and symmetrically, producing the characteristic cherubic appearance.
Cherubism has also been referred to as familial or hereditary fibrous dysplasia, bilateral giant cell tumor and familial
multilocular disease.
Cherubism was rst described by Jones in 1933. He
termed it familial multilocular disease of the jaws. However, as the cystic nature of the condition was invalidated
Jones and others were the rst to use the term cherubism.
The word cherub originally designated a member of the
second order within the Christian celestial chorus. These
were creatures with severe, staring eyes (including eyes on
the wings and the body) and a wheel below the feet.
Angels constituted another order within the celestial chorus, and angels with childish, full-cheeked faces, often
gazing upward, were widely depicted in baroque art. Thus,
the term cherubism, is actually inappropriate for the disease, because the typical clinical picture does not resemble
a classical cherub but a baroque angel.
According to the WHO classication, cherubism belongs
to a group of non-neoplastic bone lesions affecting only the
jaws. It is a rare, benign condition with autosomal dominant inheritance, and it is one of the very few genetically
determined osteoclastic lesions in the human body.
580
Genetic basis
The locus for the cherubism gene is 4p16. The most reasonable conclusion from the linkage data obtained in a study
of four families by Tiziani and others is that the locus for
cherubism is located on the telomeric side of D4S1582.
Ueki et al detected point mutations causing amino acid
substitutions in the SH3-binding protein SH3BP2.
Clinical features
Cherubism appears to have 100% penetrance in males and
only 5070% penetrance in females. Although the condition is known to be hereditary, in some cases there has
been no detectable family history, and although it usually
occurs bilaterally, there have also been cases of unilateral
involvement, perhaps because of incomplete penetrance or
new mutations.
Typically, the jaw lesions of cherubism remit spontaneously when affected children reach puberty, but the reason
for this remission is unknown. The reduction in osteoclast
formation caused by sex steroids and the increase in plasma
concentrations of estradiol and testosterone at puberty
both suggest that the genetic defect responsible for the
localized increase in osteoclasts in cherubism is overridden
and normalized by the increased synthesis of sex steroids.
Affected children are normal at birth and are without
clinically or radiographically evident disease until 14 months
to 3 years of age. At that time, symmetric enlargement of
the jaws begins. Typically, earlier the lesion appears, the
more rapidly it progresses. The self-limited bone growth
usually begins to slow down when the patient reaches
5 years of age, and stops by the age of 1215 years. At
puberty the lesions begin to regress. Jaw remodeling continues through the 3rd decade of life, at the end of which
the clinical abnormality may be subtle.
The signs and symptoms depend on the severity of
the condition and range from clinically or radiographically
undetectable features to grotesquely deforming mandibular and maxillary overgrowth with respiratory obstruction
and impairment of vision and hearing. Cherubism was
reportedly fatal in one case, where aspiration occurred
because of the grotesque facial deformity.
The jaw lesions are usually painless and symmetric
swellings, having orid maxillary involvement (Figures 6
and 7). The lesions, which are rm to palpation and nontender, most commonly involve the molar to coronoid
regions, the condyles usually being spared, and are often
associated with cervical lymphadenopathy. Enlargement of
the cervical lymph nodes contributes to the patients fullfaced appearance and is said to be caused by reticuloendothelial hyperplasia with brosis. The lymph nodes become
enlarged before the patient reaches 6 years of age, decrease
in size after the age of 8 years, and are rarely enlarged
after the age of 12 years. Intraoral swelling of the alveolar
ridges may occur. When the maxillary ridge is involved,
Figure 6
Figure 7
the palate assumes a V shape. A rim of sclera may be visible beneath the iris, giving the classic eye to heaven
appearance.
Numerous dental abnormalities have been reported, such
as agenesis of the second and third molars of the mandible,
displacement of the teeth, premature exfoliation of the
primary teeth, delayed eruption of the permanent teeth,
and transpositions and rotation of the teeth. In severe
cases, tooth resorption occurs.
Although cherubism was initially described as a familial
disease affecting the jaws, cases without any apparent hereditary origin have been reported. In a few cases cherubism has
been described as being associated with other diseases and
conditions such as Noonans syndrome, gingival bromatosis, psychomotor retardation and obstructed sleep apnea.
In order to overcome the limitations of Arnotts classication, Kalantar Motamedi developed a different classication system, which addresses both the involvement and
aggressive behavior of the disease.
Radiographic staging
Arnott suggested the following grading system for the
lesions of cherubism:
Grade I (divided into five classes): Lesions of the mandible without signs of root resorption
Grade II (divided into three classes): Lesions of the
mandible and maxilla without signs of root resorption
Grade III (divided into five classes): Aggressive lesions
of the mandible with signs of root resorption
Grade IV (divided into three classes): Lesions involving the mandible and the maxilla and showing signs
of root resorption
Grade V: Massively growing, aggressive and extensively deforming juvenile cases involving the maxilla
and the mandible and which may include the coronoid
process and condyles.
Radiographic features
581
Figure 8
Orthopantomograph showing bilateral multilocular radiolucencies involving the mandible and displacement of teeth in
cherubism in a 7-year-old patient. Reproduced with permission from editor, JCDA. Ongole R, Pillai RS, Pai KM.
Cherubism in siblings: a case report. J Can Dent Assoc 2003;69(3):15054
Figure 9
Orthopantomograph showing bilateral multilocular radiolucencies involving the mandible and displacement of teeth in
cherubism in a 14-year-old patient. Reproduced with permission from editor, JCDA. Ongole R, Pillai RS, Pai KM.
Cherubism in siblings: a case report. J Can Dent Assoc 2003;69(3):15054
and tartrate-resistant acid phosphatase, which is characteristic of osteoclasts. The collagenous stroma, which contains a large number of spindle-shaped fibroblasts, is
considered unique because of its water-logged, granular
nature.
The capillaries exhibit large endothelial cells and perivascular capillary cufng. The eosinophilic cufng is considered to be a characteristic feature of cherubism. However,
these deposits are not present in many cases, and their
absence does not exclude the diagnosis of cherubism.
Resolving lesions of cherubism show an increase in brous
tissue, a decrease in the number of giant cells and formation of new bone.
Figure 10
Orthopantomograph showing bilateral multilocular radiolucencies affecting the body and ramus of the mandible in cherubism.
The radiograph also shows the lower right second molar appearing to be floating. Courtesy: Department of Oral Medicine and
Radiology, MCODS, Mangalore
Differential diagnosis
Treatment
Giant cell granuloma of the jaws, osteoclastoma, aneurysmal bone cyst, fibrous dysplasia and hyperparathyroidism
are considered in the differential diagnosis of cherubism.
Giant cell granuloma and osteoclastoma are histologically similar to cherubism. However, giant cell granuloma
is usually unilateral and usually affects patients between
20 and 40 years of age, whereas cherubism is a symmetric
lesion affecting children.
Osteoclastoma rarely occurs in the jaws, unlike cherubism.
Aneurysmal bone cyst may also exhibit giant cells, but
its main feature is a cavity lined with tissue other than
endothelium.
Both brous dysplasia and hyperparathyroidism contain
large numbers of osteoclasts. However, histologic examination of the classic form of brous dysplasia reveals trabeculae of immature bone resembling Chinese characters
within the proliferating stroma. These trabeculae are not
rimmed by osteoblasts. Furthermore, polyostotic brous
dysplasia rst presents in the 2nd or 3rd decade of life.
Hyperparathyroidism rarely affects the jaw in an isolated manner. Its histologic features differ from those of
cherubism in which it does not contain the mononuclear
stromal cell population that is characteristic of the latter.
Finally, peritrabecular brosis is a feature of hyperparathyroidism but not cherubism. Serum concentrations of
parathyroid hormone and calcium also help to distinguish
these lesions. Levels of serum alkaline phosphatase are
generally elevated in cases of brous dysplasia. In cases of
hyperparathyroidism, levels of serum calcium are elevated,
levels of serum phosphorus are decreased and levels of
serum alkaline phosphatase are generally within normal
levels.
Idiopathic Osteosclerosis
Idiopathic osteosclerosis has also been termed dense bone
island and enostosis.
It is believed that enostosis is a counterpart of exostoses
that occur in the inner surface of the cortical plates within
the cancellous bone. It represents a focus of mature compact
(cortical) bone within the cancellous bone (spongiosa).
The etiology for their occurrence is unknown. These are
asymptomatic, benign and usually found on routine radiographic investigations. Greenspan (1995) in a review of
enostosis described that enostosis is probably congenital or
developmental in origin and represents failure of resorption
during endochondral ossication.
Characteristic features
Idiopathic osteosclerosis have known to occur anywhere
in the skeleton. However, the sites that are commonly
involved are the pelvis, femur, other long bones, spine and
the mandible.
McDonnell (1993) in a review of 107 patients reported
that the average age at which idiopathic osteosclerosis
was discovered was 36 years and women exhibited enostosis twice as much as men. Mandible was most commonly
affected. However, he reported the presence of enostosis
in the maxilla in a few individuals. The common site of
occurrence is the mandibular rst molar region. Almost
10% of the enostosis occurring in the mandibular molar
region tend to resorb the roots of the mandibular rst molar.
The typical radiographic appearance is usually a solitary
radiopacity with relatively ill-dened or diffuse border.
However, occasionally the internal structure of the sclerotic
area may show variable radiopacity (Figure 11).
Kawai et al (1996) reported a gigantic dense bone island
of the jaws. The dimensions of these large enostoses
Figure 11
Gorhams Disease
Gorhams disease is also called BreschetGorham syndrome, Gorhams osteolysis, Gorhams syndrome, Gorham
Stout syndrome, massive osteolysis, vanishing bone
disease and phantom bone disease.
Gilbert Breschet, Lemuel Whittington Gorham and
Arthur Purdy Stout were the rst few people who described
this condition. However, JBS Jackson in 1838 published
the rst case report. Subsequently Gorham and coworkers
(1954) and Gorham and Stout (1955) reported patients suffering from massive osteolysis.
Gorhams disease is a rare condition characterized by
proliferation of vascular channels that result in destruction
and resorption of the osseous matrix.
Etiopathogenesis
Figure 12
Orthopantomograph revealing enostoses in relation to the mandibular right second molar, second premolar and mandibular left
first premolar. The radiopaque foci are homogeneously dense and blend into the normal trabecular bone pattern producing a
brush like border. Courtesy: Department of Oral Medicine and Radiology, MCODS, Mangalore
585
Many other investigators however demonstrated osteoclasts in the lytic front of the lesion. The role of osteoclasts is
supported by the fact that the use of calcitonin and bisphosphonates (inhibitors of osteoclastic activity) to treat patients
suffering from Gorhams disease arrests bone resorption.
Devlin and others (1996) proposed that interleukin-6
(IL-6) can be a potential mediator of the massive osteolysis.
They found that the levels of cytokine IL-6 increases almost
by seven times when compared to the levels in a normal
patient.
They believe that there are multiple sources of interleukins such as the blood vessels or dilatation of the vascular
marrow or the osteoclasts or cells in the bone marrow
microenvironment, including monocytes, broblasts, and
lymphocytes are potential sources of IL-6.
Clinical features
Gorhams disease is usually discovered in the first 4 decades
of life. However it may affect any age group and exhibits
no sex predilection.
The disease commonly affects the pelvis and shoulder.
Other sites that are affected are the scapula, clavicle, maxillofacial skeleton, ribs, spine, skull and the bones of the
extremities.
Severe complications include paraplegia, when the spine
is involved and respiratory difculty when the thorax
involved.
It is believed that the maxillofacial skeleton in involved
in about 25 to 30% of the patients. Deformed mandible or
maxilla causes malocclusion, mobility of teeth and occasionally results in pathological fracture.
Classification of idiopathic osteolysis
(Hardegger et al, 1985)
Type 1 Hereditary multicentric osteolysis with dominant
transmission. Usually seen between the age of 2 and 7 years.
Associated with spontaneous pain and swelling beginning
in the hands and feet. Carpotarsal osteolysis occurs over
the period of a few years. The progression ceases normally
in adolescence.
Type 2 Hereditary multicentric osteolysis with recessive
transmission similar to type 1, but may be associated with
severe generalized osteoporosis.
Type 3 Non-hereditary multicentric osteolysis with
nephropathy. It appears in childhood. There is a gradual
disappearance of the carpus with the tarsal bones involved,
but to a less degree. Proteinuria is seen. Death occurs usually due to renal failure and malignant hypertension.
Type 4 Gorhams massive osteolysis (GorhamStout syndrome). Monocentric occurrence in any part of the skeleton may start at any age. Normally hemangiomatous tissue
is found in the osteolytic region. It has neither a hereditary
586
Oral manifestations
The maxilla and mandible can be affected. It is estimated
that the maxilla is almost twice as commonly affected than
the mandible. The involved jaw will be enlarged. Enlarged
maxilla will cause widening of the alveolar ridge and flattening of the palatal vault. Spacing between teeth may be
a secondary complication.
In advanced cases, the lips fail to cover the enlarging
jaws resulting in an open mouth state.
Laboratory investigations
Patients with Pagets disease may demonstrate very high
levels of serum alkaline phosphatase (helps to estimate
bone formation), especially when multiple bones are affected.
It is believed that the total alkaline phosphatase levels
have only 78% sensitivity of detecting Pagets disease.
Therefore, bone specific alkaline phosphatase levels are
more accurate.
However, serum calcium and serum phosphorus levels are
normal. Other investigations that can help in the identication of the extent and severity of bone turnover include
estimating levels of deoxypyridinoline and N-telopeptide
of type I collagen (help in estimating amount of bone
resorption). Urinary excretion of deoxypyridinoline and
N-telopeptide are elevated. Alpha-alpha type I C-telopeptide
fragments are sensitive markers of bone resorption for assessing disease activity and monitoring treatment outcome.
Radiographic features
The radiographic findings in Pagets disease depend on the
phase of the disease. Radiographs may exhibit areas of
osteolysis and areas of bone deposition or an intermediate
phase.
There are very unique radiographic features of Pagets
disease such as osteoporosis circumscripta, blade of
grass lesion, brim sign, framed vertebrae and cotton-wool
appearance.
Osteolytic zones in the skull represented by large circumscribed radiolucent areas are referred to as osteoporosis circumscripta (usually seen in the frontal and occipital
bones). In the intermediate phase, as bone deposition occurs,
areas of patchy sclerosis are seen in the skull and the jaws,
giving rise to the cotton-wool appearance. Hypercementosis
and loss of lamina dura around the roots of teeth is usually seen in Pagets disease. Rarely, root resorption may
be seen.
Long bones of the skeleton reveal a V-shaped pattern
that divides the healthy bone from its pagetic counterpart.
This unique nding is termed blade of grass lesion. Thickening of the iliopectineal line in the pelvic bone is termed
brim sign. Enlargement of the vertebral bodies with thickened cortices and vertical striations gives rise to the appearance of framed vertebrae.
588
acid was associated with a signicantly higher therapeutic response rate and a more rapid reduction in bone
turnover than that achieved with 60 days of oral risedronic acid. Moreover, biochemical remission was sustained after 24 months of follow-up in zoledronic acid
recipients.
Cytotoxic drug such as mithramycin (plicamycin) has
also been tried where the disease is refractory to bisphosphonates. However, it is seldom used these days.
589
CHAPTER
20
Autoimmune Disorders
Balaji Rao B, Sumanth KN
Sjgrens Syndrome
Mikuliczs Disease (Benign Lymphoepithelial Lesion)
Aphthous Stomatitis (Aphthous Ulcers, Canker Sores,
Recurrent Aphthous Stomatitis)
Giant Cell Arteritis
Periodontal Disease
Rheumatoid Arthritis
Pemphigus
Pemphigus Vulgaris
Pemphigus Vegetans
590
Pemphigus Foliaceous
Brazilian Pemphigus
Pemphigus Erythematosus
Paraneoplastic Pemphigus
Bullous Pemphigoid
Cicatricial Pemphigoid
Autoimmune Polyendocrinopathy
Candidiasis-Ectodermal Dystrophy
Systemic Sclerosis
Myasthenia Gravis
Autoimmunity
Failure of the immune system to tolerate (antigen-specific
immunological unresponsiveness) self-tissues. It is a condition in which structural or functional damage is caused by
the action of immunologically competent cells or antibodies
against normal components of the body.
When the concept of autoimmunity came to be accepted
as a pathogenic mechanism, a large number of diseases
were suggested to have an autoimmune etiology, based
on the nding of autoantibodies in the patients. However,
autoantibodies have also been found in the serum or tissues of otherwise normal and healthy elderly individuals.
Autoantibodies are also formed following tissue injury and
may serve a role in the removal of the products of tissue
1.
2.
3.
4.
5.
a.
2.
2.
Sjgrens Syndrome
(GougerotSjgren Syndrome)
Described in Chapter 11 (Diseases of Salivary Glands) on
page 265.
Mikuliczs Disease
(Benign Lymphoepithelial Lesion)
Described in Chapter 11 (Diseases of Salivary Glands) on
page 265.
Aphthous Stomatitis
(Aphthous Ulcers, Canker Sores, Recurrent
Aphthous Stomatitis)
Described in Chapter 7 (Vesiculobullous Disorders) on
page 174.
591
Figure 1
Muki proposed an autoimmune basis for giant cell arteritis because it occurred more frequently in patients with
other autoimmune disorders such as thyroid disease or
rheumatoid arthritis.
Clinical features
Periodontal Disease
For almost two decades the concept of autoimmune pathogenesis for periodontal disease was considered.
Alphonse et al (1981) have detected rheumatoid factor
by latex slide agglutination in subgingival plaque,
592
2.
3.
4.
5.
6.
Rheumatoid Arthritis
Rheumatoid arthritis (RA) is a chronic systemic inflammatory disorder that may affect many tissues and organs
skin, blood vessels, heart, lungs and musclesbut principally
attacks joints, producing a non-suppurative proliferative
synovitis that often progresses to destruction of the articular cartilage and ankylosis of joints. Although the cause
for RA remains unknown, there is convincing evidence
that autoimmunity plays a pivotal role in its chronicity
and progression, likening this condition to other so-called
connective tissue diseases. RA can affect the TMJ.
Pathogenesis
It is currently believed that RA is triggered by the exposure of an immunogenetically susceptible host to an arthritogenic microbial antigen.
The involved mechanisms in the causation could be:
1.
2.
3.
4.
2.
3.
Clinical features
2.
3.
1.
2.
Joint manifestations
Katz classified the joint manifestations as:
3.
4.
593
Figure 2
Erosions of the cortical outline of the condyle and reduced joint space. Courtesy: Department of
Oral Medicine and Radiology, MCODS, Mangalore
Fever
Weight loss
Fatigue
Increased susceptibility to infections
1.
2.
3.
4.
Pleural effusions
Fibrosing alveolitis
Bronchiolitis
Caplans syndrome
G. Cardiac
1.
2.
3.
4.
5.
Pericarditis
Myocarditis
Endocarditis
Coronary vasculitis
Granulomatous aortitis
B. Musculoskeletal manifestations
H. Ocular
1.
2.
3.
1.
2.
3.
4.
Osteoporosis
Bursitis
Muscle wasting
C. Hematological manifestations
1.
2.
3.
Anemia
Thrombocytosis
Eosinophilia
Laboratory findings
1.
2.
3.
Visceral arteritis
Pyoderma gangrenosum
Digital arteritis
1.
2.
3.
4.
Episcleritis
Scleritis
Scleromalacia
Keratoconjunctivitis sicca
I. Amyloidosis.
D. Vasculitis
E. Neurological
594
F. Pulmonary
Diagnosis
The diagnosis of RA is based on a carefully obtained history and a diligent physical examination to unravel subtle
signs of inflammation. One should strive to detect the condition as early as possible to prevent crippling deformities.
The American Rheumatism Association diagnostic criteria aid in clinical diagnosis. These criteria were designed
principally for research and literary purposes.
A. Classical rheumatoid arthritis This category requires
seven of the following criteria to match. In criteria 1 through
5, the joint signs or symptoms must be continuous for at
least 6 weeks.
1.
2.
Morning stiffness.
Pain on motion or tenderness in at least one joint
(observed by the physician).
3. Swelling (soft tissue thickening or fluid, not bony
overgrowth alone) in at least one joint (observed by a
physician).
4. Swelling (observed by a physician) of at least one
additional joint (symptom free period between the two
joint involvements may not be more than 3 months).
5. Symmetrical joint swelling (observed by a physician)
with simultaneous involvement of the same joint on
both sides of the body (bilateral involvement of proximal interphalangeal, metacarpophalangeal or metatarsophalangeal joints is acceptable without absolute
symmetry). Involvement of the terminal phalangeal
joint will also satisfy this criterion.
6. Subcutaneous nodules (observed by a physician) over
bony prominences, on extensor surfaces or in juxtaarticular regions.
7. Radiographic changes typical of RA (which must
include at least bony decalcification localized to or
most marked adjacent to the involved joints and not
just degenerative changes). Degenerative changes do
not exclude patients from any group classified as RA.
8. Positive agglutination testdemonstration of the
rheumatoid factor by any method which, in two
laboratories, has been positive in not over 5% of normal controlsor positive streptococcal agglutination
test (the latter is now obsolete).
9. Poor mucin precipitates from synovial fluid (with shreds
and cloudy solution).
10. Characteristic histologic changes in synovium with three
or more of the following marked villous hypertrophy,
proliferation of superficial synovial cells, often with palisading, marked infiltration of chronic inflammatory
cells (lymphocytes or plasma cells predominating)
with tendency to form lymphoid nodules, deposition
of compact fibrin either on surface or interstitially,
foci of necrosis.
11. Characteristic histologic changes in nodules show
granulomatous foci with central zones of necrosis,
Morning stiffness
Tenderness or pain on motion (observed by a physician)
with history of recurrence or persistence for 3 weeks
History or observation of joint swelling
Subcutaneous nodules (observed by a physician)
Elevated erythrocyte sedimentation rate or C-reactive
protein
Iritis (of dubious value as a criterion except in the
case of juvenile RA).
1.
2.
Pemphigus Vulgaris
Pemphigus Vegetans
Described in Chapter 7 (Vesiculobullous Disorders) on
page 174.
Pemphigus Foliaceous
Described in Chapter 7 (Vesiculobullous Disorders) on
page 174.
Pemphigus Erythematosus
(SenearUsher Syndrome)
Paraneoplastic Pemphigus
Pemphigus vulgaris
Pemphigus vegetans
Pemphigus foliaceous
Pemphigus erythematosus (SenearUsher syndrome)
Brazilian pemphigus (Fogo selvagem)
Benign familial chronic pemphigus (HaileyHailey
disease)
Paraneoplastic pemphigus.
Pemphigus vegetans is a variant of pemphigus vulgaris
and pemphigus erythematosus is a variant of pemphigus
596
Bullous Pemphigoid
Described in Chapter 7 (Vesiculobullous Disorders) on
page 174.
Cicatricial Pemphigoid
Described in Chapter 7 (Vesiculobullous Disorders) on
page 174.
Figure 3
5.
1.
2.
3.
4.
Clinical features
Epidermolyis bullosa acquisita may have at least three different clinical presentations: the classical presentation,
bullous pemphigoid like presentation and a cicatricial
pemphigoid like presentation.
The classical presentation is a non-inammatory, bullous disease with an acral distribution that heals with
scarring and milia formation. It is a mechanobullous disease marked by skin fragility. These patients have erosions, blisters and scars over trauma prone surfaces (back
of the hands, knuckles, elbows, knees, sacral area and toes)
(Figure 3).
A scarring alopecia and some degree of nail dystrophy
may be seen. The lesions heal with scarring and frequently
with the formation of pearl like, milia cysts within the
scarred areas.
The bullous pemphigoid like presentation is a widespread inammatory vesiculobullous eruption involving
the trunk, central body, skin folds and the extremities. The
bullous lesions are surrounded by inamed or urticarial
skin. The distribution of lesions like bullous pemphigoid is
Histologic features
Routine histologic examination of lesion on skin obtained
from EBA patients show a subepidermal blister and a clean
separation between the dermis and the epidermis with
inflammatory infiltrate.
Immunologic parameters
Patients with EBA have IgG deposits within dermal
epidermal junction of their skin. This is detected by direct
immunofluorescence. IgG is the predominant immunoglobulin class, but deposits of complement IgA, IgM, factor B
and properdin may also be detected.
The localization of immune deposits within the dermal
epidermal junction of the skin of epidermal junction of the
skin of the patients is the gold standard for the diagnosis.
SLE
Discoid lupus erythematosus
Drug-induced lupus erythematosus
Lupus nephritis
597
5.
6.
Figure 4
2.
598
3.
4.
Cutaneous lesions Patients present with a typical erythematous rash over the malar region, which is popularly referred to as the butterfly rash. It is evident as
an erythematous and slightly edematous lesion located
on the cheeks, extending across the bridge of the nose
(Figure 4). It is generally the first manifestation of the
disease. It frequently occurs after exposure to sun. The
rash heals well without scarring or pigmentation.
Patients may also present with a pruritic maculopapular rash, which may resemble lesions of drug
eruption.
Joint manifestations About 95% of the patients show
joint involvement. Arthritis with pain on movement,
tenderness, or effusion is present in about 80% and
arthralgia is present in about 15% of the patients with
SLE. The proximal interphalangeal, knee, wrist and
metacarpophalangeal joints are most often involved.
Swan neck deformities occur in 15% of the individuals but unlike rheumatoid arthritis, no bony erosion
is seen.
Renal disease Renal involvement in SLE ranges from 29
to 53%. The WHO grading of type of renal lesions are
a. Type I: normal
b. Type II: mesangial hypercellularity and immune
deposits confined to mesangium. These patients
have mild proteinuria
c. Type III (focal proliferative lupus nephritis):
proteinuria, hematuria and occasionally nephrotic
syndrome
Figure 5
2.
3.
4.
Autoimmune polyendocrinopathycandidiasisectodermal
dystrophy (APECED) is a rare condition which requires at
least two of the following conditions to make the diagnosis: hyperparathyroidism, Addisons disease and chronic
mucocutaneous candidiasis.
Porter et al (1995) and Perniola et al (1998) described
developmental defects of teeth and oral candidiasis as the
characteristic oral ndings in these individuals.
Clinical features
Management
1.
2.
2.
3.
4.
Prognosis
Literature review reveals a 5-year survival rate in almost
95% of the patients of SLE. However, these patients need
to be constantly medicated with the lowest possible doses
of corticosteroids.
600
AUTOIMMUNE POLYENDOCRINOPATHY
CANDIDIASISECTODERMAL DYSTROPHY
3.
4.
Management
APECED management requires antifungal therapy and the
management of endocrinopathy.
Dental considerations
Median rhomboid glossitis is considered as a specific
pathognomonic oral manifestation associated with diabetes.
However, various oral conditions are worsened in a diabetic individual such as gingivitis and periodontal diseases, oral candidiasis, delayed socket healing after extraction and burning tongue.
Specific considerations pertaining to dental treatment
1.
2.
3.
Figure 6
However, all types of routine dental treatment may be performed safely in the dental setting in individuals whose
blood glucose levels are under control.
SYSTEMIC SCLEROSIS
Systemic sclerosis can be described as chronic, progressive
dermatosis characterized by board-like hardening and
immobility of the affected skin, with visceral involvement,
especially of lungs, esophagus, kidneys and heart. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of
collagen. Curzio in 1973, published the first detailed description of a scleroderma-like disease. A variant of systemic
sclerosis is termed acrosclerosis, which is a combination of
Raynauds phenomenon and scleroderma affecting the
extremities.
Epidemiology
Figure 7
b.
601
Orofacial manifestations
Figure 8
Radiographic features
Figure 9
Generalized widening of periodontal ligament space in systemic sclerosis. Courtesy: Dr Ajit Auluck
602
MYASTHENIA GRAVIS
The term myasthenia gravis (MG) was coined by combining two Greek words for muscle and weakness to form the
word myasthenia and adding the Latin word gravis, which
means severe. The autoimmune etiology for MG was proposed for the first time in 19591960 by two independent
workers, Simpson and Nastuck.
Myasthenia gravis is an autoimmune disorder of the
neuromuscular junction, in which autoantibodies are
directed toward acetylcholine receptors (Ac in the motor
end-plate units of skeletal muscle, resulting in a progressive fatigability of the skeletal muscle. In the initial stages,
extraocular muscles are affected. As the disease progresses
2.
3.
4.
5.
6.
7.
8.
9.
604
CHAPTER
Granulomatous Diseases
Adel Kauzman, Iona Leong
21
Tuberculosis
Wegeners Granulomatosis
Syphilis
Sarcoidosis
Orofacial Granulomatosis
Blastomycosis
Histoplasmosis
Aspergillosis
Zygomycosis (Mucormycosis, Phycomycosis)
Granulomatous diseases are a heterogeneous group of disorders that are characterized by a specific pattern of chronic
inflammation called granulomatous inflammation, in which
granulomas are formed. Granulomas usually develop as a
result of a cell-mediated hypersensitivity reaction to a
non-degradable antigen. The clinical significance of a histological finding of granulomatous inflammation is that this
type of inflammation is associated with a relatively limited
number of conditions.
A granuloma is a focal aggregate of inammatory cells,
composed predominantly of epithelioid macrophages,
which may be surrounded by a rim of lymphocytes and/or
plasma cells. Epithelioid macrophages resemble epithelial
cells, having abundant pale pink cytoplasm, vesicular and
oval nuclei and indistinct cell borders (Figure 1). Some
activated macrophages may fuse to form multinucleated
giant cells which may be located within the center or
periphery of the granuloma (Figure 2). In Langhans type
giant cells the nuclei are arranged in the periphery, often in
the shape of a horseshoe. The nuclei of foreign body type
giant cells are arranged haphazardly in the cytoplasm.
Some granulomas may show central areas of necrosis.
Caseous necrosis, in which the dead tissue is soft and dry
and resembles cheese, is classically associated with tuberculosis. Long-standing granulomas may be surrounded by
a rim of brous connective tissue and broblasts.
Granulomas can be classied as immune or foreign body
type. Foreign body type granulomas develop in response to
inert endogenous or exogenous foreign material (Table 1).
Figure 1
TUBERCULOSIS
Figure 2
Table 1
Infections
Bacterial
Tuberculosis
Leprosy
Syphilis
Others
Foreign body
Diseases of unknown
etiology
Silica
Beryllium
Talc
Suture material
Starch
Wegeners granulomatosis
Sarcoidosis
Orofacial granulomatosis
Crohns disease
Fungal
Blastomycosis
Histoplasmosis
Aspergillosis
Zygomycosis
Others
Parasitic
Leishmaniasis
Schistosomiasis
Figure 3
Figure 4
Clinical features
Tuberculosis is classified as pulmonary or extrapulmonary.
Pulmonary tuberculosis can be primary or secondary.
Primary pulmonary disease results from an initial infection with M. tuberculosis in previously unexposed individuals and is usually asymptomatic. The lesion is usually
peripheral and localized to the middle and lower lung
zones and is accompanied by hilar or paratracheal lymphadenopathy. In most cases, the lesion heals spontaneously
and may later be evident as a small calcified nodule (Ghon
lesion) (Figure 3). Approximately 510% of patients progress directly from initial infection to active disease, usually
because of an existing state of immunosuppression. This is
especially seen in children and in persons with impaired
immunity, such as those with malnutrition or HIV infection. The initial lesion enlarges, cavitates, invades and
destroys bronchial walls and blood vessels. Large numbers
of bacilli spread into the airways and the environment
through expectorated sputum. Patients may develop pleural effusion and progressive primary tuberculosis.
Hematogeneous dissemination may result in fatal miliary
tuberculosis (Figure 4) or tuberculous meningitis.
Secondary pulmonary tuberculosis, also known as postprimary disease, is usually due to endogenous reactivation
of latent infection. Triggers for reactivation include immunosuppression, especially AIDS, malnutrition and vitamin D
deciency. The disease usually occurs in the apical and
posterior segments of the upper lung lobes, where the high
Figure 5
Panoramic radiograph of a patient with a history of tuberculosis showing calcified cervical and submandibular lymph nodes
remission or chronic disease with a progressively debilitating course (consumption). Individuals with chronic disease
continue to discharge tubercle bacilli into the environment.
Symptoms and signs are often non-specic and insidious
early in the course of secondary pulmonary tuberculosis,
consisting mainly of fever, night sweats, weight loss,
anorexia, general malaise and weakness. Cough eventually develops in most individuals, often initially non-productive and subsequently accompanied by the production
of purulent sputum. The sputum may be blood-streaked
due to blood vessel involvement.
Virtually any organ system may be affected by extrapulmonary tuberculosis, but the most commonly involved
sites are the lymph nodes, pleura, genitourinary tract, bones
and joints, meninges, peritoneum, pericardium, and the
head and neck region. In the head and neck, tuberculosis can
involve the larynx, middle ear, nasal cavity, nasopharynx,
oral cavity, parotid gland, esophagus and spine. As a result
of hematogeneous dissemination in HIV-infected individuals, extrapulmonary tuberculosis is seen more commonly
nowadays than in the past.
Lymph node tuberculosis (tuberculous lymphadenitis)
is the most common form of extrapulmonary tuberculosis
and is especially common in HIV-infected persons. It usually
presents as painless swelling of the lymph nodes, most
commonly at cervical and supraclavicular sites (scrofula).
Lymph nodes are usually discrete in early disease but may
develop caseous necrosis and form stulas through the overlying skin draining caseous material. Involved nodes may
radiographically appear calcied (Figure 5). Pulmonary
tuberculosis is unusual in patients with scrofula.
Oral manifestations
The most common manifestation of tuberculosis in the oral
cavity is a chronic painless ulcer. Less frequently tuberculous lesions present as nodular, granular or rarely firm
608
Figure 6
Figure 7
Due to inconsistent results and high rates of falsenegative and false-positive results, serological tests for the
diagnosis of active tuberculosis are not recommended.
Screening for latent M. tuberculosis infection can be
performed with skin testing with puried protein derivative (PPD). However, the test is of limited value in the
diagnosis of active disease because of its low sensitivity
and specicity. False-negative reactions are common in
immunosuppressed patients and in those with overwhelming tuberculosis. Positive reactions are elicited from individuals who have been infected with M. tuberculosis but
do not have active disease and from persons sensitized by
non-tuberculous mycobacteria or Bacille CalmetteGurin
(BCG) vaccination.
The gold standard for identifying M. tuberculosis
infection is an interferon- release assay which measures
interferon- titers released by T cells in response to stimulation with certain TB-speic antigens, such as ESAT-10
and CFP-10.
Management protocols and prognosis
Because of drug resistance, cure of tuberculosis requires
prolonged concomitant administration of at least two agents
to which the organism is susceptible. Four major drugs are
considered as first-line treatment agents: isoniazid, rifampin
(rifampicin), pyrazinamide and ethambutol. Second-line
drugs include streptomycin, kanamycin, amikacin, capreomycin, ethionamide, cycloserine, para-aminosalicylic acid
and fluoroquinolone antibiotics such as ofloxacin. Secondline agents are used in patients resistant to first-line therapy, because of their lower efficacy and higher toxicity.
Infectious cases should be diagnosed rapidly and appropriately treated until cure. The development of drug-resistant tuberculosis is primarily the result of monotherapy or
failure of the healthcare provider to prescribe at least two
drugs to which the tubercle bacilli are susceptible. Resistance
occurs also if the patient fails to take properly prescribed
therapy. Multidrug-resistant tuberculosis (MDR-TB) is a
form of tuberculosis that does not respond to the standard
drug treatment, i.e. the two main rst-line drugs: isoniazid
and rifampicin. The term extensive (extreme) drug resistant
tuberculosis (XDR-TB) has been used to describe a form of
MDR-TB resistant to any uoroquinolone and one of three
injectable aminoglycosides (capreomycin, kanamycin and
amikacin).
Strategies for prevention and disease control include
BCG vaccination and treatment of persons with latent tuberculosis infection who are at high risk of developing active
disease, such as those infected with HIV, close contacts of
persons with known or suspected active tuberculosis, persons
with medical risk factors associated with reactivation of
tuberculosis, medically underserved and low-income populations, alcoholics, injection drug users, persons with abnormal chest radiographs compatible with past tuberculosis,
609
SYPHILIS
Syphilis, also known as lues, is a chronic sexually transmitted disease caused by Treponema pallidum. The disease
has diverse clinical presentations and is characterized by
periods of active disease and latency.
The causative agent of syphilis, T. pallidum subspecies
pallidum, hereafter referred to as T. pallidum, is a microaerophilic spirochaete which cannot be grown in vitro.
The only known natural host for T. pallidum is the human.
The genome has been fully sequenced and is small, rendering T. pallidum an obligate parasite with limited metabolic capabilities. Other pathogenic treponemes also can
cause disease in humans, such as yaws and pinta.
Syphilis occurs worldwide and usually is transmitted by
sexual contact or from mother to infant. T. pallidum can
be transmitted as a blood-borne infection, but this is infrequent. Syphilis is most common among the poor, those who
lack access to healthcare and in those with many sexual
partners. According to WHO estimates, approximately
12 million new cases of venereal syphilis occurred in 1999,
most of them in developing countries. Congenital syphilis
is a leading cause of perinatal and neonatal death in many
of these countries. In North America and western Europe,
syphilis is less common and disproportionately affects minority populations, men who have sex with men, and persons
using cocaine and other drugs. The individuals at highest
risk for syphilis also are at increased risk for HIV infection,
and the two frequently coexist, as syphilis facilitates the
transmission of HIV.
Pathogenesis
T. pallidum rapidly penetrates intact mucous membranes
or microscopic abrasions in skin. It then enters the lymphatics and blood to produce systemic infection. Despite
induction of a strong humoral and cell-mediated immune
response, T. pallidum is able to survive in the untreated
human host for decades and may continue to be transmitted or cause end-organ damage. T. pallidum can be transmitted from a syphilitic woman to her fetus across the
placenta at any stage of pregnancy. The manifestations of
congenital syphilis are thought to be due to the immune
response of the host, rather than a direct toxic effect of the
pathogen.
612
Clinical features
Oral manifestations
Untreated syphilis is a chronic illness which is traditionally divided into primary, secondary and tertiary stages. In
the primary stage a chancre appears at the site of inoculation about 21 days post-infection. The chancre begins as a
painless papule that becomes ulcerated. Primary lesions
most commonly occur on the genitalia and are frequently
accompanied by regional lymphadenopathy. The primary
lesion usually resolves spontaneously within 46 weeks,
In secondary syphilis, maculopapular and nodular mucosal lesions are common. Supercial mucosal erosions, called
mucous patches, may be seen on the lips, oral mucosa,
tongue, palate and pharynx. The mucous patches are typically painless, oval to crescentic erosions, surrounded by a
red periphery. Snail track ulcers are serpiginous lesions
that may arise de novo, or form by coalescence of a number of mucous patches. Condylomata lata are broad-based
verrucous plaques which can be seen in secondary syphilis
of the oral cavity.
The oral manifestations of tertiary syphilis are mostly due
to gumma formation. Gummas are caused by endarteritis
obliterans and tend to involve the hard palate, tongue or
lower alveolus. The gummas form swellings which may
coalesce to form serpiginous lesions and eventually ulcerate,
resulting in bone destruction, palatal perforation and oronasal stula. Intraosseous gummas appear as ill-dened
radiolucent lesions. Interstitial glossitis is rare and is the
result of contracture of the tongue musculature after healing
of a gumma. An association between interstitial glossitis
and oral squamous cell carcinoma of the tongue has been
suggested, but this may be due to the carcinogenic agents
formerly used to treat syphilis (arsenicals and heavy metals)
rather than the infectious agent. Tertiary syphilis can also
give rise to both unilateral and bilateral trigeminal neuropathy and facial nerve palsy.
Oral manifestations of congenital syphilis include
Hutchinson teeth which are screwdriver-shaped incisors that
may show notching of the incisal edge. Mulberry molars
have multiple poorly developed cusps. The facies of patients
with congenital syphilis show frontal bossing, saddle nose,
and poorly developed maxillae. Rhagades are linear scars at
the angles of the mouth caused by secondary bacterial infection of a facial rash occurring in early congenital syphilis.
Histopathologic features
Primary and secondary syphilis are characterized by dense
plasma cell infiltrates, especially in a perivascular distribution, and by capillary endothelial proliferation and obliteration of small blood vessels. However, the microscopic
features are not specific for syphilis. Plasma cells are common in oral biopsies, especially those taken from the gingiva.
The WarthinStarry method is a silver impregnation technique which can be used to demonstrate T. pallidum, but
the stain is not specific and may label other spirochaetes
which inhabit the oral cavity. Gummas are composed of
aggregates of epithelioid and giant cells, forming granulomas,
usually with a prominent plasma cell infiltrate. T. pallidum
is rarely demonstrated in gummas.
Diagnosis
The diagnosis of syphilis is usually based on clinical signs
and symptoms and serologic tests. T. pallidum cannot be
detected by culture. Dark-field microscopic examination of
exudate is of limited value in oral lesions because other commensal Treponema species found in the human mouth can
be confused with T. pallidum. There are two types of serological tests for syphilis: treponemal and non-treponemal.
Commonly used non-treponemal tests include the rapid
plasma reagin (RPR) and venereal disease research laboratory (VDRL) tests. The non-treponemal tests measure IgG
and IgM directed against the cardiolipinlecithincholesterol antigen complex and are used for screening or for
quantification of serum antibody. Treponemal tests are used
for confirmation of reactive non-treponemal results and
include fluorescent treponemal antibody-absorbed (FTAABS) test and T. pallidum particle agglutination assay
(TPPA). The treponemal tests are qualitative tests and are
not used in assessing treatment responses.
Treatment
Parenteral long-acting penicillin G is the drug of choice
for all stages of syphilis. Resistance to penicillin has not
been described. Other antibiotics effective against syphilis
include the macrolides such as erythromycin and azithromycin, as well as tetracycline antibiotics, such as tetracycline and doxycycline. Unfortunately, with increased use
of azithromycin for many infections, there has been an
increased prevalence of macrolide-resistant T. pallidum.
Vaccines are not available for this disease. Testing for HIV
status in affected patients is also recommended.
Blastomycosis
Blastomycosis is caused by Blastomyces dermatitidis, a
dimorphic fungus that grows in soil and decaying wood.
The organism is endemic in some parts of the United States
and Canada, but infections are also seen in Mexico, MiddleEast, Africa and India. Infection occurs through inhalation
of the conidiae (asexual spores). In the lungs at body temperature, the conidiae are transformed into yeasts that
multiply through budding. Yeasts represent the pathogenic
forms of the organism. The infection usually is contained
in the lungs and most patients are either asymptomatic or
develop mild non-specific flu-like symptoms. In some
patients, hematogeneous spread can occur.
613
Figure 8
Histoplasmosis
Histoplasmosis is caused by Histoplasma capsulatum, a
dimorphic fungus found in soil contaminated with bird or
614
Figure 9
Figure 10
Aspergillosis
Aspergillosis is a fungal infection caused by members of
the Aspergillus species which are ubiquitous saprophytic
molds growing on organic matter. Sporulation of the mold
produces large numbers of conidia that can be inhaled by
humans without adverse consequences in the majority of
cases. In some patients, the inhaled organisms can cause
allergic fungal sinusitis, allergic pulmonary aspergillosis
or asthma. A mass of fungal hyphae, called aspergilloma,
can form in the sinuses or the lungs of patients suffering
from cavitary tuberculosis, bronchiectasis or lung abscesses.
Immunocompromised patients, such as individuals with
AIDS, bone marrow or organ transplant recipients, and
patients with hematological malignancies, can develop
invasive disease, where primary disease of the lungs is followed by widespread hematogeneous dissemination and
multiple organ involvement.
Aspergillus fumigatus and A. avus are the most common species to cause human aspergillosis. A. fumigatus is
Figure 11
Figure 12
Clinical features
The clinical manifestations of foreign body reactions are
usually non-specific and vary from a localized mass to
superficial erosions and ulcerations. In the oral cavity, the
gingiva seems to be the most common site of involvement
and the condition has been termed granulomatous or foreign body gingivitis. Dental materials are the most common causative factors in these cases, but other substances
including hair and nails have been reported. The condition
presents clinically as a localized change in gingival color,
ulceration or diffuse gingival erythema. Lesions start at
the interdental papilla and extend laterally. An important
diagnostic clue is that the condition does not regress with
improvement of oral hygiene measures.
Histopathologic features
Biopsy material from the gingiva can show granulomas and
foreign body giant cells in the absence of microorganisms.
The foreign substance can be identified in hematoxylin
and eosin stained sections (Figure 13) or by polarized light
microscopy. Energy-dispersive radiographic microanalysis
can help confirm the presence of foreign material in tissue
sections and identify its nature. In the absence of identifiable foreign material, other causes of granulomatous
inflammation have to be investigated. In some cases, the
inflammatory response is lichenoid in appearance and the
foreign body is too small to be identified microscopically.
A diagnosis of lichen planus can therefore be rendered,
but the lesions will not respond to conventional treatment
of lichen planus.
Management protocols and prognosis
The treatment of foreign body reactions includes the elimination of the offending agent and excision of involved
tissues. This results in adequate clinical response in the
Figure 13
WEGENERS GRANULOMATOSIS
Wegeners granulomatosis (WG) is a systemic autoimmune
disease that is invariably fatal if left untreated. The disease
may occur in generalized and limited forms, and consists
of the classical triad of (i) necrotizing granulomatous
inflammation involving the upper respiratory tract, the
lower respiratory tract, or both, (ii) necrotizing glomerulonephritis, and (iii) systemic vasculitis involving small to
medium-sized vessels (capillaries, venules, arterioles, or
arteries).
WG is an uncommon disease, with an estimated prevalence of 3.0 per 100,000 persons in the United States. It is
more common in whites than in blacks and affects both
sexes equally. The mean age at onset is approximately
40 years, but the disease can occur at any age, with 15%
of cases occurring in patients younger than 19 years.
Etiology and pathogenesis
The etiology of WG has not been fully elucidated. Genetic
factors, exposure to environmental antigens such as silica
and infection (in particular with Staphylococcus aureus)
seem to play an important role in disease pathogenesis.
frequently involved. The process originates in the interdental papillae and extends laterally to involve the rest of
the gingiva. Periodontal bone loss has been described and
can result in tooth mobility and extraction. Non-healing
of extraction sockets is possible. Oral ulcers affecting any
mucosal surface can be seen in WG, but are non-specific
and seem to develop at an advanced stage of the disease,
usually after renal involvement occurs. Necrosis and ulceration of the palate can be seen.
Histopathologic features
Wegeners granulomatosis is characterized microscopically by granulomatous inflammation, geographic necrosis and vasculitis. The granulomas are ill-defined, surround
areas of necrosis, and are not as compact and as defined
as those seen in tuberculosis and sarcoidosis. In lung biopsies, areas of necrosis are surrounded by a shell of fibroblastic, inflammatory and giant cells, producing palisading
granulomas. The inflammatory infiltrate can be dense,
mixed, and non-specific and overshadow the underlying
pathology. Special stains do not show microorganisms and
foreign body cannot be identified by polarizing light
microscopy. Affected vessels show a transmural inflammatory reaction, with focal or diffuse polymorphonuclear
infiltrates causing vessel wall destruction and fibrin deposition within the walls and the lumen. The necrosis seen
seems to be secondary to the vasculitis.
Oral mucosal biopsies show an inammatory inltrate
composed mainly of neutrophils and eosinophils that occasionally form microabcesses. Giant cells are common and
ill-dened granulomas can be seen. Vasculitis is less prominent in mucosal biopsies from the head and neck region,
including the mouth, mainly due to the small size of these
biopsies and lack of larger vessels in mucosa. Strawberry
gingivitis shows pseudoepitheliomatous hyperplasia and a
prominent vascular component associated with red blood
cell extravasation.
Diagnosis and laboratory findings
The diagnosis of WG is based on history, clinical presentation, laboratory test results and microscopic findings of
necrotizing granulomatous vasculitis in a biopsy specimen.
Laboratory tests show an elevated erythrocyte sedimentation rate (ESR), high C-reactive protein, leukocytosis and
thrombocytosis. Renal involvement can result in urinary
sediment, erythrocyturia, proteinuria and high serum creatinine levels. Chest radiographs and computerized tomography can confirm the presence of pulmonary infiltrates or
nodules. Detection of c-ANCA by indirect immunofluorescence and confirmation of the specificity of ANCA against
proteinase-3 (PR3-ANCA) by ELISA yields a specificity of
99% and a sensitivity of 73% in the diagnosis of WG.
Identification of c-ANCA is an adjunctive test, as false positive results can be seen in some infectious and neoplastic
618
SARCOIDOSIS
Sarcoidosis is a relatively common multisystem immunemediated disease primarily affecting the lungs and lymphatic systems. It is characterized by the presence of
non-caseating granulomas in affected organs. The etiology of sarcoidosis is unknown, but familial, spatial (e.g.
among people in the same household), seasonal and occupational clustering of the disease have been reported, suggesting multiple underlying factors, including genetic
predisposition, infectious agents and environmental exposures. Possible microbial triggers include mycobacterial
and propionibacterial organisms, with the mycobacterial
catalase-peroxidase (mKatG) protein as a potential candidate antigen. Sarcoidosis likely develops from an exaggerated cellular immune response (acquired, inherited or both),
to a limited class of persistent antigens or self-antigens.
Space-occupying granulomas form from the accumulation
of mononuclear inflammatory cells, mostly CD4 Th1 lymphocytes and mononuclear phagocytes in affected organs.
Organ dysfunction and tissue injury result from physical
distortion of the tissue by the granulomas.
Sarcoidosis is found throughout the world, but is most
prevalent in United States and Scandinavian populations.
In the United States there is a three-fold to ve-fold increased
incidence in blacks compared to whites. Geographic, ethnic and genetic factors are linked to the specic clinical
characteristics of patients with sarcoidosis. Blacks are more
Figure 14
Clinical features
Sarcoidosis is more common in young and middle-aged
adults, with approximately 75% of the cases in individuals
younger than 40 years. Females appear to be slightly more
susceptible than males. Sarcoidosis has a variable clinical
presentation and course, and can affect many organs and
tissues, but the lungs are most commonly affected. Unlike
many lung diseases, sarcoidosis favors non-smokers. Other
organ systems that may be affected by sarcoidosis include
the heart, liver, spleen, bones, skin, eyes, lymph nodes,
parotid glands, and uncommonly, the oral cavity. The
majority of patients with sarcoidosis are asymptomatic,
with the disease being discovered on routine chest radiographs. Symptomatic sarcoidosis may develop abruptly
over a period of a few weeks. Less frequently sarcoidosis
arises insidiously over months or years without significant
symptoms. Symptomatic patients usually present with
respiratory and skin manifestations or with constitutional
and non-specific symptoms such as fever, night sweats,
fatigue and malaise. Dry cough, dyspnea and chest pain
are frequent respiratory complaints. Cutaneous manifestations occur in approximately 25% of patients and include
erythema nodosum and lupus pernio. Erythema nodosum
are scattered tender erythematous nodules occurring frequently on the lower legs, while lupus pernio represents
chronic, violaceous lesions involving the face, limbs, back
and buttocks. Ocular symptoms may be due to anterior
uveitis or lacrimal gland involvement resulting in keratoconjunctivitis sicca. Two distinctive clinical syndromes
are associated with acute sarcoidosis. Lfgren syndrome, a
form of acute sarcoidosis usually found in white females,
consists of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia. Heerfordt syndrome (uveoparotid
fever) is characterized by parotid enlargement, anterior
uveitis, facial paralysis and fever.
Oral manifestations
Excluding salivary gland and lymph node involvement,
oral manifestations of sarcoidosis are uncommon, and the
disease is typically diagnosed before oral symptoms appear.
Sarcoidosis may involve any oral mucosal site, most commonly buccal mucosa, followed by gingiva, lips, floor of
mouth, tongue and palate. The lesions most commonly present as submucosal masses, which may vary in color from
normal to brownish-red or violaceous or they may be hyperkeratotic. Sarcoidosis of the major and minor salivary
glands may result in xerostomia. Sarcoidosis can cause
bilateral enlargement of the major salivary glands which,
in conjunction with xerostomia and keratoconjunctivitis
Figure 15
The overall prognosis of sarcoidosis is good. Spontaneous resolution is common and in most patients symptoms
resolve spontaneously within 2 years without treatment.
Poor prognostic indicators include chronic disease, older age
at onset, black race, lupus pernio, neurosarcoidosis, cardiac
involvement and advanced pulmonary disease. Approximately 410% of patients die of progressive respiratory,
central nervous system or cardiac involvement.
OROFACIAL GRANULOMATOSIS
Orofacial granulomatosis (OFG) is a clinical and pathologic term introduced by Wiesenfeld in 1985 to describe
a group of conditions affecting the oral and maxillofacial region, and characterized microscopically by noncaseating granulomatous inflammation. The spectrum of
OFG includes cheilitis granulomatosa (CG) of Miescher,
MelkerssonRosenthal syndrome (MRS), Crohns disease,
sarcoidosis and other granulomatous inflammatory conditions that could affect this region. Some cases of OFG can
develop secondary to a chronic dental infection or to a
contact hypersensitivity reaction while others appear to be
idiopathic. A preliminary diagnosis of OFG should be followed by a thorough clinical and laboratory investigation
to identify and treat any possible underlying local or systemic disease and thus idiopathic OFG is a diagnosis
acquired by exclusion.
The etiology of OFG is unknown and the disease is
thought to represent an abnormal immune reaction. Infection, allergy and genetic predisposition have been suggested. Monoclonal lymphocytic expansion, which may be
secondary to chronic antigenic stimulation, has been identied in OFG. Cytokine production by the monoclonal lymphocytic proliferation could stimulate granuloma formation.
The spectrum of OFG encompasses cheilitis granulomatosa (CG) of Miescher and MelkerssonRosenthal syndrome (MRS). Systemic conditions such as tuberculosis,
Crohns disease, sarcoidosis and other granulomatous
inammatory conditions could present with granuloma
formation in the oral and maxillofacial region.
Clinical features
The most consistent finding in OFG is a painless, persistent
diffuse swelling involving one or both lips (macrochelia).
The swelling can be unilateral or involve the whole lip
(Figure 16). In the early phases of the disease, the swelling
is usually soft, intermittent and recurrent. Later, the swelling becomes permanent and fibrotic. Generalized edema,
erythema and non-specific erosions and ulcerations may
be seen in the mouth. Gingival swelling can be seen in some
cases. Some patients develop swelling elsewhere in the face,
with or without lip involvement, making the diagnosis
more difficult. Other reported manifestations of OFG include
Figure 16
Figure 17
exclusion of possible causes of granulomatous inflammation should be followed by a systematic work-up, including
clinical, laboratory and radiographic investigations, to
rule out underlying local or systemic disease.
All oral foci of infection should be identied and
treated. Elimination diet and patch testing against potential allergens such as food additives, cosmetics, fragrances
and dental hygiene products should be performed if contact hypersensitivity reaction is suspected. Chest radiographs and serum levels of angiotensin-converting enzyme
can be obtained to screen for evidence of sarcoidosis.
Complete blood count, ESR, and serum levels of folic acid,
vitamin B12, and iron are useful in patients with unusual
gastrointestinal manifestations. These patients also should
undergo specialized gastrointestinal examination to assess
for Crohns disease. If this investigation is negative, it can
be repeated later, especially if clinically indicated and in
younger patients with OFG as these patients are more
likely to have concomitant intestinal disease. Tuberculin
test and chest radiographs should be performed even if
acid-fast stains were negative on histology to exclude
tuberculosis.
Management protocols and prognosis
Figure 18
Figure 20
Figure 19
Figure 21
mucosa and deep linear ulcers involving the buccal vestibule (Figure 18). Aphthous ulcers can be seen, but their
significance in Crohns disease is uncertain, as they occur
as frequently in the general population and in the same
age group affected by Crohns disease. Other reported
oral manifestations include fibroepithelial hyperplasia,
granulomatous gingivitis (Figure 19), angular cheilitis,
persistent submandibular and superficial cervical lymphadenopathy, and metallic dysguesia. Less than 1% of
patients with Crohns disease may develop diffuse stomatitis, with some cases caused by Staphylococcus aureus,
and others being non-specific. A rare condition called
pyostomatitis vegetans may be associated with Crohns disease. Pyostomatitis vegetans is usually seen on the buccal,
labial mucosa, soft palate, ventral tongue and facial gingiva
and is characterized by the development of multiple yellowish, serpiginous pustules on erythematous oral mucosa
(Figure 20). The pustules often rupture, leading to erosions
and fissuring.
Histopathologic features
In the bowel, Crohns disease is characterized by transmural non-necrotizing granulomatous inflammation. Oral
lesions show non-necrotizing granulomas in the submucosa
similar to those seen in OFG (Figure 21). The severity of the
granulomatous inflammation may vary tremendously from
623
624
CHAPTER
Sexually Transmitted
Diseases
Praveen BN, Nagaraj A, Ravikiran Ongole
22
Fellatio Syndrome
Condyloma Acuminatum
Oropharyngeal Gonorrhea
Syphilis or Lues
FELLATIO SYNDROME
It is described as submucosal hemorrhage secondary to
repetitive negative pressure and/or blunt trauma associated with fellatio (Terezhalmy et al, 2000).
These oral lesions are typically found in sexually active
adults. However, presence of such lesions in children may
be associated with sexual abuse.
Clinical features
The submucosal hemorrhages are characteristically seen at
the junction of the soft and hard palate without involvement of the uvula, pharyngeal wall or other oropharyngeal
structures.
These lesions may appear bilaterally as solitary lesions,
occasionally connected hemorrhagic bridge. They may also
appear as a well-dened band of ecchymosis stretching
across the soft palate.
These non-ulcerated hemorrhagic areas are painless and
do not blanch on palpation.
Diagnosis and differential diagnosis
A good personal history will help in the diagnosis. However,
other conditions that may mimic such lesions are frequent
use of drinking straws, habit of sucking on candies (produces negative pressure), petechiae secondary to forceful
sneezing, vomiting or coughing, upper respiratory tract
infections, blood dyscrasias, infectious mononucleosis, nasopharyngeal tumors and anticoagulant or antithrombotic
medications.
625
Management
Patient should be made aware of the nature and cause of
the lesion. Hemorrhagic diathesis should be considered when
the lesions do not subside in about 10 days time following
cessation of the habit.
SYPHILIS OR LUES
Syphilis is a chronic venereal infection caused by fragile
spirochete Treponema pallidum.
The term syphilis was coined by the Italian physician and
poet Girolamo Fracastoro (1530). Other names which have
been used in literature are French disease, Italian disease,
Christian disease, British disease, lues venerea and Cupids
disease. In the 16th century syphilis was recognized as
Great Pox in Europe.
Diseases caused by other species of Treponema include
Yaws (caused by T. pertenue), Pinta (caused by T. carateum)
and bejel (endemic syphilis, caused by T. endemicum).
The major source of transmission of syphilis is by sexual
contact (acquired). The other modes of transmission include
transplacental spread (congenital/neonatal) or accidental
inoculation of the causative organism (non-sexual modes
of transmission).
T. pallidum is a delicate fastidious spirochete whose
only natural hosts are humans. It is of 1015 m in length
and 0.2 m in thickness, tapering ends and possesses
1015 spirals. It has a graceful to and fro and angulating
626
movement under dark-eld microscopy in a wet preparation. Organisms can be demonstrated in tissue/tissue uids
by silver staining and immunouorescence. Pathogenic
T. pallidum (Nicholas strain) has not yet been able to culture but organisms can be maintained in rabbits testicular
tissue with retaining its pathogenicity. In refrigerated blood
the organisms die within 5 days and can be easily killed
with soap and water.
Pathogenesis
The organisms gain entry through mucous membrane or
abraded skin during sexual contact. It is estimated that only
about 50% of people who come in contact develop syphilis.
It is believed to be due to the presence of local factors and
immobilins in the blood which immobilize T. pallidum.
Incubation period varies from 9 to 90 days.
Natural immunity to syphilis does not occur in humans
and vascular changes appear to be more signicant, characterized by endarteritis and periarteritis. Fibroblastic proliferation leads to brosis and scar formation.
Clinical features
Primary syphilis Primary syphilis is typically acquired
via direct sexual contact with the infectious lesions of a
person with syphilis. Approximately 1090 days after the
initial exposure (average 21 days), a skin lesion appears at
the point of contact, which is usually the penis, vagina or
rectum, but can occur anywhere on the body.
It is estimated that only about 2% of the primary lesions
are evident on extragenital sites such as the rectum, ngers,
lips, tongue, palate, tonsils, nipple and chin.
Lee et al (2006) reported a case of syphilitic chancre
presenting as a solitary nodule of the nipple. Primary lesions
on the ngers usually result from contact with genital lesions
during sexual foreplay or as an occupational exposure
in physicians and nurses as a result of direct contact with
infectious ulcers in their patients. Little (2005) suggested
that syphilis can be spread by direct contact with mucosal
lesions of primary and secondary syphilis or blood and
saliva from infected patients.
This lesion, called a chancre, is a rm, painless ulceration localized at the point of initial exposure to the spirochete. The chancre begins as a papule that subsequently
ulcerates. The chancre may persist for 38 weeks and usually heals spontaneously. Localized lymphadenopathy may
be evident.
The oral primary syphilitic lesion (chancre) like elsewhere
in the body, is generally seen after about 3 weeks after the
exposure at the site of inoculation of the virus. Initially a
papule is formed which subsequently ruptures to form a
painless ulcer. The ulcer is generally punched out and may
be indurated. Regional lymphadenopathy is usually a characteristic feature. The chancre resolves in about 4 weeks
leaving a scar.
Neurosyphilis
Tertiary syphilis can cause unilateral and bilateral trigeminal neuropathy and facial nerve palsy. It is also believed
that syphilitic osteomyelitis may give rise to trigeminal
neuropathy.
Congenital Syphilis
Congenital syphilis or prenatal syphilis is caused when
T. pallidum is transmitted to the offspring by an infected
mother. T. pallidum crosses the placenta only after the
16th week of intrauterine life.
Untreated mothers can drastically affect the status of the
offspring. Most of the pregnancies result in spontaneous
abortion, stillbirth, premature delivery or perinatal death.
Prematurity and low-birth weight have also been reported.
Kassowitzs law is an empirical observation used in
context of syphilis. It states that greater the duration between
infection of mother and pregnancy, better is the outcome
for the infant. Better outcome includes lesser chances of
stillbirth and of developing congenital syphilis.
The rate of vertical transmission in untreated women is
70100% for primary syphilis, 40% for early latent syphilis
and 10% for late latent disease.
These statistics show very clearly that the longer the
interval between infection and pregnancy, the better is the
prognosis for the newborn.
Based on the time of presentation of the signs and
symptoms, the clinical manifestations can be categorized
as early manifestations (occurring in the rst 2 years of
life) and late manifestations occur after 2 years of age.
628
Early manifestations The earliest clinical finding is persistent rhinitis. Other associated findings include hepatomegaly, splenomegaly, glomerulonephritis and nephrotic
syndrome.
Palms and soles of these neonates show erythematous
maculopapular rashes or vesiculobullous lesions. Generalized lymphadenopathy is evident. Bone lesions such as
osteochondritis and osteomyelitis.
Late manifestations Hutchinsons triad (interstitial keratitis, peg-shaped upper incisors, and eighth cranial nerve
deafness), saber shins, irregular thickening of the sternoclavicular portion of the clavicle (Higoumenakis sign), flaring scapulas, mental retardation and hydrocephalus are the
late manifestations of syphilis.
Bilateral hydrarthrosis (Cluttons joints) and neurosyphilis are other common late manifestations of syphilis.
Orofacial manifestations in congenital syphilis Localized periostitis of the frontal and parietal bones is manifested as frontal bossae of parrot. Appearance of the
Olympian row is seen when the supraorbital region is
involved.
Inammation of the nasal mucosa may destroy the
underlying bone and cartilage, perforate the nasal septum
and manifests as saddle nose.
A short maxilla along with the saddle nose may present
as concave or shallow-dish appearance of the middle third
of the face. A relative mandibular prognathism results in a
bulldog jaw.
Other oral changes include high palatal arch. The dental
anomalies of congenital syphilis only arise in teeth in which
calcication occurs during the rst year of life, namely,
the permanent incisors and rst molars.
Hutchinsons teeth are short, barrel-shaped or peg-shaped
widely spaced central incisors. Other ndings include
notching of incisal edges. The incisors have a screwdrivershaped morphology (convergence of the lateral margins
toward the incisal edge). Mulberry molars, hypocalcication of enamel and presence of rhagades extending from
the angle of the mouth are other prominent features. Occasionally, atrophic glossitis and facial neuropathies may
be seen.
Diagnosis
Along with the typical history and clinical picture of
syphilis, serological tests are necessary for an accurate
diagnosis.
Dark-eld microscopy has been used extensively for
the diagnosis of primary syphilis. However, it cannot differentiate T. pallidum from the other treponemal species.
Serological tests can be used effectively to diagnose
secondary, latent and tertiary syphilis. The serologic tests
include non-treponemal tests (Venereal Disease Research
Laboratory [VDRL] test and the Rapid Plasma Reagin [RPR]
5.
Management
6.
3.
4.
7.
CONDYLOMA ACUMINATUM
Condyloma acuminatum commonly termed genital or venereal wart occurs commonly on external genitalia; however,
oral lesions can occur either through orogenital sex or by
auto-inoculation of genital lesions. Condyloma acuminatum is caused by human papilloma virus types 6, 11 and
16. Condylomata acuminata are now thought to be one of
the four most common STDs.
Clinical features
Typical lesions are evident as solitary or multiple papular
eruptions. These eruptions are usually flesh colored and
appear pearly, filiform, fungating, cauliflower or plaque like.
The most commonly affected areas are the penis, vulva,
vagina, cervix, perineum and perianal area. Rarely, mucosal lesions in the oropharynx, larynx and trachea have
been reported.
Oral manifestations
The typical oral lesions of condyloma acuminatum are minute, multiple pink colored nodules which tend to coalesce
630
OROPHARYNGEAL GONORRHEA
Gonorrhea is a caused by Neisseria gonorrheae. The disease is characterized by purulent inflammation of mucous
membrane surfaces.
Clinical features
The bacterial infection spreads through sexual contact.
Gonococcal infections are 1.5 times more common in men
than in women. It is usually seen in sexually active adolescents and young adults. Like most STDs, the presence of
gonorrhea in a child can be considered as an indicator for
sexual abuse of the child.
Men may present with urethral discomfort, dysuria and
purulent discharge. Other notable feature is epididymitis.
Women may complain of thin, purulent and mildly
odorous discharge from the vagina. Another important
complication of gonorrhea in women is pelvic inammatory disease (PID). PID indicates that the offending organism has ascended to involve the endometrium, fallopian
tubes, ovaries and peritoneum. PID is characterized by midline mild or severe pain and cramps.
Right upper quadrant pain from perihepatitis (Fitz
HughCurtis syndrome) may occur following the spread of
organisms upward along peritoneal planes.
Occasionally rectal discharge, bloody stools and pruritus
may be evident.
Oral manifestations
Oral manifestations
Diagnosis
In the initial stages of the infection, patients may complain of burning sensation, drying of the mouth and foul
breath.
This phase may be followed by the evidence of painful
ulcers usually seen on the lips, gingival, tongue, palate
(hard and soft) and the tonsillar regions.
The ulcers are typically punched out in the interdental
gingiva. The tongue may exhibit papillary atrophy. The
ulcers are usually covered by grayish-white or yellowishwhite pseudomembrane. Pharyngitis is a characteristic
feature.
Regional lymphadenopathy may be evident. In some
patients, the TMJ may be affected (gonococcal arthritis of
TMJ).
Diagnosis
The evidence of gram-negative diplococci in a Gram stained
smear of exudates from the gonococcal lesions is suggestive of N. gonorrheae infection.
Management
The drugs of choice are doxycycline or azithromycin with
ofloxacin.
OROPHARYNGEAL TRICHOMONAL
INFECTION
Trichomonas vaginalis is the causative agent of trichomoniasis. It is a parasitic protozoan with humans as the only natural host. It infects the squamous epithelium of the genital
tract. Incubation time is generally between 4 and 28 days.
It is believed that T. vaginalis infections are a marker for
high risk sexual behavior as they are generally associated
with other STDs, especially gonorrhea.
Management
Oropharyngeal gonorrhea is best managed with intramuscular injection of ceftriaxone. Other drugs that have been
used effectively include cefixime and ciprofloxacin.
OROPHARYNGEAL CHLAMYDIAL
INFECTION
Chlamydial infection is caused by Chlamydia trachomatis,
an obligate intracellular bacterium that infects the urethra,
epididymis, uterus and cervix. The bacterium is usually
spread through sexual activity.
However, it can also spread vertically to cause pneumonia and conjunctivitis in neonates.
Chlamydial infection is usually seen in the 2nd and 3rd
decades of life.
Clinical features
Women may present with dysuria, vaginal bleeding, vaginal discharge and lower abdominal pain. Men may exhibit
dysuria, rectal and/or urethral discharge and proctitis.
Clinical features
Women complain of foul smelling, frothy vaginal discharge. However, the pathognomonic sign is the presence
of strawberry cervix or colpitis macularis on colposcopy.
Patients may also complain of lower abdominal pain and
dysuria. However, some patients may remain asymptomatic.
Male patients may remain asymptomatic or present
with urethritis.
Oral manifestations
The characteristic feature is strawberry-like inflammation
of the oral mucosa secondary to vasodilation. The mucosal
surface may show presence of exudate.
Diagnosis
The gold standard for diagnosing trichomonads is by culture. The Diamonds medium is the ideal medium for this
procedure.
Few simple diagnostic tests that may be used are the
immediate examination of a wet slide and whiff test.
The easiest method to visualize motile trichomonads
is by placing a small amount of vaginal discharge on a
631
632
CHAPTER
Carbohydrates
Proteins
Lipids
Vitamins
Vitamin A
Vitamin D
Vitamin K
Vitamin C
Vitamin B Complex
Nutriology as per Dorlands Medical Dictionary is the science of nutrition. It is the science of how the body utilizes
food to meet requirements for development, growth, repair,
and maintenance. Nutrients are biochemical substances that
can be supplied only in adequate amounts from an outside
source, usually from food.
The relationship between nutrition and oral health is
multifaceted. Nutrition has both local and systemic
impacts on the oral cavity. While diet and eating patterns
have a local effect on the teeth, saliva and soft tissues, the
systemic impact of nutrition also has considerable implications and it too merits assessment as a component of
comprehensive care. The systemic effect is the impact of
the nutrients consumed as they assume their biological
functions in relation to the development and maintenance
of the extra- and intraoral structures and secretions. The
oral cavity is often one of the rst sites where nutrient
deciencies can be clinically noted.
Clinical manifestations of nutrient deciencies can
have a signicant impact on the function of the oral cavity. Functional properties of the oral cavity include taste,
salivation, mastication and swallowing food. Any alterations in the structure and function of the oral cavity may
compromise intake and contribute to the development of a
nutrient-deciency state. When the associated oral structures are affected, these alterations may be compounded
23
Metabolic Disorders
Lysosomal Storage Diseases
Lipoid Proteinosis
HandSchullerChristian Disease or Multifocal
Eosinophilic Granuloma
LettererSiwe Disease
Lipid Reticuloendothelioses
Gaucher Disease
NiemannPick Disease
CARBOHYDRATES
Carbohydrates have been the major sources of energy
since the dawn of history and furnish up to 90% of energy
needs. Carbohydrates provide about 4 kcal/g. The average
adult stores about 300 g of carbohydrate in the liver and
muscle tissue as glycogen.
Mainly provides fuel for the body, especially the central nervous system.
Adequate consumption has a protein sparing function,
i.e. proteins consumed are used for anabolic function
rather than as an energy source.
In the absence of carbohydrates, fat metabolism is
incomplete and leads to formation of ketone bodies.
Carbohydrates are required for the formation of structural
components such as cartilage, nervous tissue and bone.
Sex
Sources
Grains (wheat, corn, rice, oats, rye, barley, buckwheat and
millet) provide complex carbohydrates and starches. Vegetables, especially root and seed varieties (potatoes, sweet
potatoes, beet and peas) contain considerable amounts of
starch. Milk is a good source of lactose. Dietary fiber can be
obtained from whole grain breads and cereals and legumes.
Dental considerations
For decades, dietary carbohydrates have one of the major
constituents incriminated in the causation of caries. The
occurrence of caries depends upon the frequency of consumption, chemical constitution, route of administration,
and physical properties of carbohydrates.
Sugar substitutes have been claimed to be non-cariogenic
due to their inability to act as substrates for the enzyme
glucosyltransferase. Xylitol is one such commonly used
sugar substitute. It has been found to be non-cariogenic.
Some studies also show that chewing xylitol gum has
Recommended dietary allowance of infants and
children
Age of infant
Energy (kcal)
Weight (kg)
36 months*
700
69 months*
810
8.5
912 months*
950
9.5
6 months
583
5.4
612 months
844
8.6
Actual body
weight (kg)
Man
60.0
Woman
50.0
Energy RDA
Activity
category
For actual
body weight
Percentage
difference
52.0
Sedentary
Moderate
Heavy
2,425
2,875
3,800
2,115
2,492
3,293
13
13
13
44.0
Sedentary
Moderate
Heavy
1,875
2,225
2,925
1,740
1,958
2,594
12
12
11
634
Table 2
Functions
Table 1
PROTEINS
Second to water, proteins are the most abundant substances in the body. The structural units of proteins are
amino acids. Of the 20 amino acids present in nature, nine
are considered essential, i.e. these are to be supplied by
the diet.
Functions
Sources
Meat and milk food groups provide most of the dietary
proteins. Soy is also a good source of proteins.
Dental considerations
During tooth development, mild-to-moderate protein deficiency results in smaller molars, chemical alterations of
the exposed enamel surface, significantly delayed eruption
and retardation of mandibular development. Smaller salivary glands develop resulting in decreased salivary flow.
This saliva has a different protein, amylase and aminopeptidase activity, thereby compromising its immune function.
Delayed eruption and decreased salivary flow lead to
increased incidence of dental caries. Epithelium, connective
tissue and bone may be poorly developed. Insufficient
intake of protein results in negative nitrogen balance,
decreased levels of secretory IgA. This leads to a lowered
resistance to infections, reduced ability to withstand the
stresses of injury or surgery, and prolonged recovery time.
PEM may be a major reason for the occurrence of necrotizing ulcerative gingivitis (NUG) and noma.
LIPIDS
Lipids provide more energy per gram than either carbohydrates or proteins and are an essential component of tooth
enamel and dentin.
Chemistry and sources
Deficiency
Protein energy malnutrition This term covers the spectrum of clinical conditions seen in undernutrition. The two
clinical forms of protein energy malnutrition (PEM)
include kwashiorkor and marasmus.
Kwashiorkor occurs when a child is fed on a diet with
very low protein content relative to energy. This results in
a high level of plasma insulin and low levels of plasma
cortisol. This hormonal pattern leads to an uptake of
amino acids in muscle, diverting these from liver, leading
to decreased albumin synthesis and therefore edema.
Thus a child with kwashiorkor shows apathy, lethargy
and severe anorexia. There is generalized edema, muscle
wasting in shoulders and upper arms. The child may have
a moon face. Potbelly due to weakness of abdominal
muscles occurs. Skin changes in the form of thickening,
cracking and areas of denudation occur. Hair changes
color and becomes sparse.
Marasmus occurs when there is inadequate food intake
resulting in energy deciency. This leads to low insulin
and high plasma cortisol levels. This results in amino acids
being released from muscles making them available for
protein synthesis.
A child with marasmus presents with no subcutaneous fat
and wasted muscles. The body weight is severely reduced.
There is no edema, and skin and hair changes are mild or
absent.
Studies have shown that the incidence of PEM among
Indian children could be as high as 51.670%.
Some fatty acids, such as oleic acid act as growth factors for lactobacilli, others, such as lauric acids inhibit
the growth of streptococci.
635
2.
Vitamin A
Dietary sources
3.
4.
5.
6.
VITAMINS
Vitamins are catalysts for all metabolic reactions using
proteins, fat and carbohydrates for energy, growth and cell
maintenance. Vitamins are vital to life, but are required in
minute amounts.
Vitamins are classied as fat solublevitamins A, D, E, K
and water solublevitamins B, C.
Functions
Vitamin A functions at two levels in the body: the first is
in the visual cycle in the retina of the eye; and the second
is in all body tissues where it systemically maintains the
growth and soundness of cells.
In the visual system, carrier bound retinol is transported
to the retina. Rhodopsin, the visual pigment critical to
dim-light vision, is formed in rod cells after conversion of
all-trans-retinol to retinaldehyde, isomerization to the
11-cis-form, and binding to opsin. Alteration of rhodopsin
through a cascade of photochemical reactions results in
the ability to see objects in dim light. The speed at which
rhodopsin is regenerated is related to the availability of
retinol. A decient intake of vitamin A thus leads to night
blindness.
636
Table 3
Group
Mean requirement
(mg RE/day)
Recommended safe
intake (mg RE/day)
180
190
200
200
250
375
400
400
450
500
330400
600
270
300
500
600
300
300
600
600
Pregnant women
370
800
Lactating women
450
850
Adolescents
1018 years
Adults
Females
1965 years
65 years
Males
1965 years
65 years
Dental considerations
Vitamin A is necessary for growth of both soft tissue and
bone. It is required for resorption of old bone and synthesis of new bone, formation of ameloblasts, odontoblasts,
and maintenance of the integrity of epithelial tissues.
Severe deciency of vitamin A may result in enamel
hypoplasia and defective dentin formation in developing teeth. Odontoblasts lose the ability to arrange themselves in normal parallel linear formation leading to
altered dentin deposition. This further leads to degeneration and atrophy of ameloblasts. This results in enamel
hypoplasia characterized by defects in enamel matrix and
incomplete calcication.
Clinical applications
Carotenoids possess antioxidant properties and are very efficient in scavenging singlet oxygen and peroxyl radicals.
These free radicals are known to damage the structure and
function of cell membranes. Thus a diet rich in antioxidants
is associated with a lower risk of cancer and heart disease.
Stich and colleagues gave large quantities of -carotene
and sometimes vitamin A to chewers of betel quids in Kerala,
India, and to Canadian Inuits with pre-malignant lesions
of the oral tract and witnessed reductions in leukoplakia
and micronuclei from the buccal mucosa.
However, the amount of supplements used in these studies have been large and hence, not advisable other than
increasing consumption of fruits and vegetables.
Vitamin D
Functions
Vitamin D is required to maintain normal blood levels of
calcium and phosphate, which are in turn needed for the
normal mineralization of bone, muscle contraction, nerve
conduction and general cellular function in all cells of
the body. Vitamin D achieves this after its conversion to
the active form 1,25-dihydroxy vitamin D (1,25-(OH)2D),
or calcitriol. This active form regulates the transcription
of a number of vitamin D-dependent genes that code for
calcium-transporting proteins and bone matrix proteins.
Vitamin D also modulates the transcription of cell cycle
proteins, which decrease cell proliferation and increase
cell differentiation of a number of specialized cells of the
body (e.g. osteoclastic precursors, enterocytes, keratinocytes). This property may explain the actions of vitamin D
in bone resorption, intestinal calcium transport and skin.
Sources
The most physiologically relevant and efficient way of
acquiring vitamin D is to synthesize it endogenously in the
skin from 7-dehydrocholesterol by sunlight (UV) exposure.
In most situations, approximately 30 minutes of skin exposure (without sunscreen) of the arms and face to sunlight can
provide all the daily vitamin D needs of the body (Table 4).
637
Table 4
Group
RNI (mg/day)
Dental considerations
5
5
5
5
5
Adolescents
1018 years
Adults
1950 years
5165 years
65 years
5
10
15
Pregnant women
Lactating women
It is recommended that individuals not synthesizing vitamin D should correct their vitamin D status by consuming
the amounts of vitamin D appropriate for their age group.
Other food sources include cod liver oil, catsh, salmon,
turnip greens, tuna, milk, egg yolk and butter.
Deficiency
Rickets is a clinical syndrome that occurs when there is a
deficiency of vitamin D in the growing skeleton. Infants with
rickets exhibit delayed development and muscle hypotonia, craniotabes (small non-calcified areas in skull bones),
bossing of frontal and parietal bones, swelling of the rib
costochondral junctions (rickety rosary). Severe rickets may
be associated with hypocalcemic tetany, giving rise to laryngeal stridor when the vocal cords are affected.
Vitamin D deciency in the adults is termed osteomalacia. Osteomalacia is characterized by bone pain, pathologic
638
Vitamin K
Vitamin K is an essential fat-soluble micronutrient. Thus far,
the only unequivocal role of vitamin K in health is in the
maintenance of normal coagulation. The vitamin K-dependent
coagulation proteins are synthesized in the liver and comprise factors II, VII, IX and X, which have a hemostatic role
(i.e. they are procoagulants that arrest and prevent bleeding), and proteins C and S, which have an anticoagulant
role (i.e. they inhibit the clotting process). Despite this
duality of function, the overriding effect of nutritional
vitamin K deficiency is a bleeding tendency caused by the
relative inactivity of the procoagulant proteins.
Chemistry and function
Vitamin K is the family name for a series of fat-soluble
compounds which have a common 2-methyl-1,4-naphthoquinone nucleus but differ in the structures of a side chain
at the 3-position. These are synthesized by plants and bacteria. In plants, the only important molecular form is phylloquinone (vitamin K1). Bacteria synthesize a family of
compounds called menaquinones (vitamin K2).
The biological role of vitamin K is to act as a cofactor
for a specic carboxylation reaction that transforms selective glutamate (Glu) residues to -carboxyglutamate (Gla)
residues. The reaction is catalyzed by a microsomal enzyme,
-glutamyl, or vitamin K-dependent carboxylase.
Dietary sources
Phylloquinone is distributed ubiquitously throughout the
diet, and the range of concentrations in different food categories are very wide. In general, the relative values in
vegetables confirm the known association of phylloquinone with photosynthetic tissues, with the highest values
being found in green leafy vegetables. The next best sources
are certain vegetable oils (e.g. soybean, rapeseed and
olive); other vegetable oils, such as peanut, corn, sunflower and safflower. Menaquinones seem to have a more
restricted distribution in the diet than does phylloquinone.
Menaquinone-rich foods are those with a bacterial fermentation stage.
Intestinal microora synthesize large amounts of
menaquinones, which are potentially available as a source
Table 5
Group
RNIa (mg/day)
5b
10
15
20
25
Adolescents
Females, 1018 years
Males, 1018 years
3555
3555
Adults
Females
1965 years
65 years
Males
1965 years
65 years
55
55
65
65
Pregnant women
55
Lactating women
55
The RNI for each group is based on a daily intake of approximately 1 mg/kg
Dental considerations
Oral manifestations of vitamin K deficiency include gingival bleeding, petechiae and ecchymoses. Bleeding on brushing occurs when prothrombin levels fall below 35% and
spontaneously when the levels fall below 20%.
Characteristics of water-soluble vitamins: These are
water-soluble organic substances. The B vitamins also
contain nitrogen. These vitamins are readily absorbed in
the jejunum. The body stores very small quantities of each
of these vitamins and hence, daily intake is important.
Vitamin C
Vitamin C (chemical names: ascorbic acid and ascorbate)
is a six-carbon lactone which is synthesized from glucose
by many animals. Vitamin C is synthesized in the liver in
some mammals and in the kidney in birds and reptiles.
However, several species including humans are unable to
synthesize vitamin C. When there is insufficient vitamin C
in the diet, humans suffer from the potentially lethal deficiency disease scurvy.
Functions
Vitamin C is an electron donor (reducing agent or antioxidant), and probably all of its biochemical and molecular roles can be accounted for by this function.
Dietary sources
Ascorbate is found in many fruits and vegetables. Citrus
fruits and juices are particularly rich sources of vitamin C
but other fruits including cantaloupe and honeydew melons, cherries, kiwi fruits, mangoes, papaya, strawberries,
tangelo, tomatoes and water melon also contain variable
amounts of vitamin C. Vegetables such as cabbage, broccoli, Brussels sprouts, bean sprouts, cauliflower, mustard,
greens, red and green peppers, peas and potatoes are also
important sources of vitamin C (Table 6).
Deficiency
Deficiency of vitamin C leads to scurvy, which can manifest
in as little as 20 days. From the 15th century, scurvy was
dreaded by seamen and explorers forced to subsist for months
on diets of dried beef and biscuits. Scurvy was described by
the Crusaders during the sieges of numerous European cities,
and was also a result of the famine in 19th century Ireland.
Three important manifestations of scurvy, namely, gingival changes, pain in the extremities, and hemorrhagic
manifestations precede edema, ulcerations, and ultimately
death. Skeletal and vascular lesions related to scurvy probably arise from a failure of osteoid formation. In infantile
scurvy, the changes are mainly at the sites of most active
bone growth; characteristic signs are a pseudoparalysis
639
Vitamin B Complex
Group
Vitamin B1Thiamine
RNI (mg/day)
25
30
30
30
35
Adolescents
1018 years
40
Adults
1965 years
65 years
45
45
Pregnant women
55
Lactating women
70
Sources
RNI (mg/day)
Dental considerations
0.2
0.3
0.5
0.6
0.9
Adolescents
Females, 1018 years
Males, 1018 years
1.1
1.2
Adults
Females, 19 years
Males, 19 years
1.1
1.2
Pregnant women
1.4
Lactating women
1.5
Table 8
Group
RNI (mg/day)
0.3
0.4
0.5
0.6
0.9
Adolescents
Females, 1018 years
Males, 1018 years
1.0
1.3
Adults
Females, 19 years
Males, 19 years
1.1
1.3
Pregnant women
1.4
Lactating women
1.6
Oral symptoms are a prominent feature of riboflavin deficiency. These are characterized by angular cheilitis and
glossitis.
In the initial stages, there is a reddish appearance of the
tip and lateral margins of the tongue. The tongue appears
to be coarsely granular due to atrophy of liform papillae
and swelling of fungiform papillae. Severe cases lead to
atrophy of all papillae causing the tongue to appear smooth
and glazed. Riboavin deciency also leads to increased
vascularization causing the tongue to appear magenta in
color.
Angular cheilitis initially presents as pallor of the lips
at the angles of the mouth. This is followed a few days
later by reddening of lips due to the desquamation of the
epithelium and ssuring at the angles of the mouth. There
may be only one or multiple ssures. These ssures develop
a yellow crust, which can be removed without bleeding.
If the deciency is not treated, the ssures become deep
and may bleed and become painful. Secondary infection
with oral and/or skin microorganisms may occur. The
incidence of the orolingual and dermal lesions in India is
high about 510%, particularly in pregnant women and in
school-going children.
Angular stomatitis, however, may be associated with
iron deciency anemia. Angular cheilitis, however, is
often associated with fungal infections, lip-sucking and
dehydration.
Patients with riboavin deciency also reveal a scaly,
greasy dermatitis of the alae of nose and nasolabial folds.
Corneal vascularization, supercial and interstitial keratitis have also been reported in ariboavinosis. Impairment
of psychomotor performance tests in riboavin deciency
has been reported to occur since physical work increases
riboavin requirement. Riboavin deciency has also been
implicated in the etiology of cataract.
METABOLIC DISORDERS
Lysosomal Storage Diseases
Lysosomes are subcellular organelles containing specific
hydrolases that allow targeted processing or degradation
of proteins, nucleic acids, carbohydrates and lipids. There
are more than 40 different types of lysosomal storage
diseases.
Metabolic diseases can be categorized as:
1.
2.
3.
Cutaneous porphyria is divided into congenital erythropoietic porphyria (Gunthers disease), porphyria cutanea
tarda and erythropoietic protoporphyria.
Clinical features
Acute intermittent porphyria It is an autosomal dominant disorder caused by the deficiency of uroporphyrinogen I synthetase. Acute attacks in this disease are triggered
by alcohol consumption, tobacco use, stress and hormonal
changes such as in luteal phase of menstrual cycle and
pregnancy. Men and women are equally affected. The
symptoms of the disorder are frequently manifested after
puberty.
In acute episodes the urine turns purple-red following
exposure to sunlight. Acute attacks are characterized by
nausea, vomiting, malaise, myalgia and chest pain. Some
individuals may present with depression, confusion, hallucinations and seizures. Severe forms of the condition
may result in respiratory paralysis.
Downey (1992) reported a patient who complained of
severe abdominal pain, seizures, diarrhea, drooling of saliva
associated with a metallic taste immediately after placing
a xed partial denture containing pallidum (76%), copper
(10%) and gold (2%). The symptoms subsided on removing
the denture. This patient was diagnosed as suffering from
acute intermittent porphyria.
Acute intermittent porphyria can be diagnosed by the
qualitative assessment of urine for porphobilinogen.
Hereditary corpoporphyria It is the rarest form of acute
porphyria characterized by the deficiency of enzyme corpooxidase. It is an autosomal dominant condition which is
usually more common in women. The trigger factors are the
same as that of the acute intermittent porphyria. However,
acute attacks are less common and less severe.
The clinical symptoms are the same as that of the acute
intermittent type. These patients may exhibit cutaneous
photosensitivity.
Elevated levels of coproporphyrins in stool analysis is
diagnostic of hereditary corpoporphyria.
Variegate porphyria The term variegate' is used to
describe the variety of clinical manifestations that this form
of porphyria exhibits. It can manifest as an acute form, cutaneous form, both or occasionally appear clinically latent.
Protocoproporphyria hereditaria or South African porphyria (widely prevalent in the white population of South
Africa) are other names by which variegate porphyria is
known.
Vareigate porphyria is diagnosed based on the marked
increase in the levels of protoporphyrin in stool.
ALA dehydratase-deficient porphyria It is an acute
form of porphyria whose pattern of inheritance is still not
known. The clinical presentation mimics that of acute
intermittent porphyria.
Congenital erythropoietic porphyria Congenital erythropoietic porphyria or Gunthers disease is a rare autosomal
recessive disorder that usually presents with marked skin
photosensitivity, hypertrichosis, blistering, scarring, milia
formation and dyspigmentation of the photo-exposed areas.
The enzyme URO-3-synthetase is decient. It typically
begins in infants or young children.
Sun-exposed regions of the body such as the hands, neck
and face may exhibit vesicular or bullous lesions. These vesicles subsequently rupture and heal with scar formation.
Owing to its physical afnity to calcium phosphate, porphyrin binds to teeth, nails and bones. Both the deciduous
and permanent dentition appear red or brown in color
(erythrodontia).
Porphyria cutanea tarda It is an autosomal dominant
disorder and the most common of all the porphyrias. It is
caused by the deficiency of URO-decarboxylase.
Alcohol consumption, hepatitis C and medications such
as iron supplements and hormonal replacements can predispose to porphyria cutanea tarda.
Cutaneous photosensitivity is the most striking feature.
Exposure to sun leads to the formation of vesicles and bullae which subsequently heal forming crusts. These individuals may exhibit hyperpigmentation and hirsutism.
Erythropoietic protoporphyria It is an autosomal dominant disorder caused by the deficiency of ferrochelatase.
The striking feature of this type of porphyria is the development of cutaneous manifestations of pain, redness, itching and burning sensation within a few minutes of sun
exposure. Thickening of the skin and nail deformities are
seen in chronic cases. Unlike other forms of porphyria
hyperpigmentation and hirsutisms are rare.
Orofacial manifestations and dental
considerations
Individuals suffering from cutaneous porphyria may present with long eyelashes, bushy eyebrows and facial hair
(facial hirsutism).
Oral mucosa is very vulnerable even to the mildest trauma
due to the accumulation of porphyrin precursors. Intraoral
erosions and bullae may be seen.
Patients suffering from congenital erythropoietic porphyria
(CEP) may exhibit pallor of the oral mucosa. Occasionally,
the alveolar mucosa may appear bluish purple owing to
the discolored underlying alveolar bone.
Deciduous dentition of CEP patients may appear deep
red-brown in color. Permanent teeth may not be severely
discolored. The cervical regions of teeth are more intensely
discolored compared to the occlusal region. These discolored teeth tend to uoresce with Woods light (near UV
light of 365 nm wavelength).
It is believed that the concentration of porphyrin is higher
in the dentin compared to the enamel.
643
644
Safe drugs
(commonly used)
Contraindicated drugs
(commonly used)
Acetaminophen
Alcohol
Clindamycin
Acyclovir
Diclofenac
Amoxicillin
Erythromycin
Amphotericin B
Lidocaine
Bupivacaine
Mepivacaine
Codeine
Miconazole
Dexamethasone
Prilocaine
Gentamicin
Pyrazolones
Ibuprofen
Carbamazepine
Iron
Phenytoin sodium
Nitrous oxide
Dapsone
Penicillins
Sulfonamides
Streptomycin
Lipoid Proteinosis
Lipoid proteinosis also known as hyalinosis cutis et mucosae or UrbachWiethe disease. It is transmitted as an autosomal recessive disorder. Lipoid proteinsosis is characterized
by generalized thickening and scarring of the skin, mucosae and occasionally some viscera secondary to diffuse
deposition of a hyaline-like substance.
Recent studies have shown that lipoid proteinosis is
caused by the mutations in a glycoprotein (extracellular
matrix protein 1) that is expressed in various tissues. The
mutation was mapped to a locus on chromosome 1q21.
Clinical features
Deposits in the vocal cords may manifest as inability of
babies to cry at birth and hoarseness of voice from early
infancy or childhood.
The skin characteristically shows whitish, moniliform
papules on the eyelids (blepharosis moniliformis, eyelid beading), and yellowish or waxy papules or nodules on the lips,
knuckles, hands, knees, elbows, axillae and the perineal
regions.
Occasionally, the face may exhibit vesiculobullous eruptions which later form scars. Patchy alopecia has also been
reported.
Other features include dyspnea, dysphagia, corneal opacities and glaucoma. Calcications of intracerebral parasellar or hippocampal gyri may be associated with epilepsy,
646
LIPID RETICULOENDOTHELIOSES
Gaucher Disease
Gaucher disease is a rare autosomal recessive disorder
characterized by defective function of the catabolic
enzyme -glucocerebrosidase, leading to an accumulation
of its substrate, glucocerebroside, in the mononuclear
phagocyte system, lymph nodes, bone marrow; Kupffer
cells in the liver; osteoclasts in bone; microglia in the central nervous system; alveolar macrophages in the lungs;
and histiocytes in the gastrointestinal tracts, genitourinary
tracts, and the peritoneum. Cells with this deposition are
termed Gaucher cells.
Classification
LettererSiwe Disease
It is named after Erich Letterer and Sture August Siwe.
Unlike the other forms of Langerhans cell histiocytosis,
LettererSiwe disease is an acute disease and almost
always seen in infants. It usually runs a short course and
ends fatally.
Clinical features
In LettererSiwe disease, recurrent pyoderma-like lesions
with crusting and scaling, vesicopustular and purpuric
eruption occurs in crops over the face, scalp and trunk,
resembling seborrheic dermatitis.
Oral manifestations
In most of the cases, oral manifestations may not be
appreciated owing to the rapid course of the disease.
However, mucosal ulceration associated with hemorrhage,
gingival inflammation, extensive loss of the maxilla and
mandible and spontaneous exfoliation of teeth may be
seen.
Diagnosis
Tzanck smear of the vesicopustules shows pale histiocytes
which can be used as a rapid screening test.
Skin biopsy shows a proliferative reaction with extensive upper dermal inltration and epidermal invasion
with histiocytes, some of which are atypical, along with
erythrocytes.
Typically foam cells and brosis are appreciated
histologically.
NiemannPick Disease
It is characterized by the deficiency of the enzyme, sphingomyelinase, and accumulation of sphingomyelin in tissues.
Classification
It can be classified into two categories based on biochemical and molecular criteria. The first category includes types
A and B, the second, type C.
Clinical features
The organs most commonly involved are spleen, liver,
bone marrow, lymph nodes and lungs. The entire central
nervous system also becomes involved. The patient presents with massive visceromegaly and severe neurologic
deterioration.
Diagnosis is achieved by the estimation of sphigomyelinase activity in leukocytes or cultured broblasts.
Prognosis is poor and affected infants die before the
age of 3 years. Antenatal diagnosis is possible.
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SECTION
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CHAPTER
Incinerated Teeth
Postmortem Pink Teeth
Tongue Hemorrhage
Social Profiling
Craniofacial Identification
Cranial Suture Pattern
Frontal Sinus Configuration
Skull-Photograph Superimposition
Challenges in Superimposition
Three-Dimensional Digital Superimposition
Facial Approximation
Clay Modeling
Computer-assisted Approximation
Dental Identification
What is Identification?
Importance of Teeth in Postmortem Identification
Principle of Dental Identification
Dental Identification Procedure
24
DENTAL IDENTIFICATION
What is Identification?
Identity refers to the characteristics by which a person
may be recognized (Acharya and Taylor, 2003) and identification is the establishment of a persons individuality.
Accurate identification of the dead is required both for
legal and humanitarian reasons (Brown, 1984). It enables
the settlement of insurance and property, permits remarriage of the surviving spouse and facilitate last rites of the
body in accordance with appropriate religious customs.
Haglund and Morton (1994) believe that a majority of
individual identifications in forensic settings are nonproblematic since most individuals die in the company of
family and friends. These deaths may occur at home or in
a hospital, allowing visual identification. However, in cases
where the body is burned, traumatized or decomposed,
visual identification can prove unreliable. Hence, Sopher
(1972) believes that visual recognition and use of personal
effects are the least dependable methods of identification
in such circumstances. Therefore, the safest option is for
Figure 1
including intact and missing teeth, caries and other pathology, restorations, attrited and rotated teeth should be carefully charted on the postmortem form. This will be the
basis for later comparison and verification. Brown (1984)
suggests that postmortem examination should be undertaken by dentists working in pairsone examining and
the other recording. The roles are then reversed and the
procedure repeated as an added check. Any dental
evidence missing should be reported to the forensic
pathologist or accompanying law enforcement personnel.
Subsequent dental evidence recovered must be labeled
properly.
656
The dental examination must be complemented with postmortem dental radiography as this will reveal what cannot
be observed clinically. Radiographic lms may be stabilized
in the mouth using readily available materials such as gauze
or cotton rolls. Whenever possible, postmortem radiographs
should be taken to replicate the type and angle of the antemortem radiographs (Goldstein et al, 1998). According to
these authors, if the difference in horizontal angulation
between the ante- and postmortem radiographs is more than
10, comparison will not facilitate identication. However,
changes in the vertical angulation or the focal point-lm
distance do not affect the outcome of comparison. Pretty and
Dental records contain information on treatment and dental status of a person during life. These antemortem records
can be obtained from the local dentist, specialist or hospital records. Whenever possible, the original records should
be examined. Records include hand-written dental charts,
radiographs, study casts and photographs. An individual
may have visited more than one dentist for therapy and
multiple dental records may exist. Relevant information
from the dental records should be transcribed onto the
modified Interpol antemortem odontograph (the yellow
form) (Figure 2).
Contribution of dental records in successful identication
depends on the availability of original records, their completeness and quality (Acharya and Taylor, 2003). By maintaining good dental records, dentists reect their awareness
and responsibility to forensic identication (note: quality
dental records are also important as a counter to malpractice lawsuits). It is also important that these records are
relatively recentrecords made for a 6-year-old child may
not be useful 20 years later in adulthood.
Figure 2
It is necessary for the forensic odontologist to be absolutely certain for an identication to be positive. One also
needs to remember that any attempt at establishing identity
is addressed to the legal authorities. Hence, the report and
conclusion should be as denitive and clear as possible.
Digital radiography in dental identification Traditional
methods of radiography involve the use of film. The advent
of digital radiography over the past decade, which replaces
the radiographic film with an image capturing sensor,
658
CHALLENGES IN POSTMORTEM
EXAMINATION
Examining dental remains may be challenging in cases of
rigor, incineration and other circumstances. Described
below are some techniques to overcome difficulties, as well
as precautions to be taken while using them.
Rigor Mortis
Limited opening of the mouth due to severe postmortem
rigor will affect dental examination since access to the
oral cavity is limited and viewing the dentition is impaired.
In such cases, where the muscles are extremely rigid, the
mouth will have to be forced open. The use of trismus
screws (made from acrylic) for obtaining initial opening in
the anterior dentition followed by Fergusson or Heister
mouth gags to increase jaw separation at the posterior
region has proven useful in the authors experience.
However, one must be careful not to fracture the teeth.
Nakayama and coworkers (2001) have advocated an intraoral approach for myotomy of the temporalis muscle. They
contend that mouth opening demands relaxation of the
temporalis and, hence, its myotomy will facilitate easy
Incineration
Due to the fragility of dental tissues consequent to fire, it
is essential to maintain their integrity during examination
of charred human remains. According to Delattre (2000),
charred dental remains are by nature rather delicate and
can crumble if not handled cautiously. The color of the teeth,
apparently, gives an indication of its relative fragility
blackened teeth are less fragile than those that are ashen
gray (the appearance of incinerated teeth is described later
in this chapter). The lips and cheek may contract in prolonged or intense fire and withdraw exposing the anterior
teeth. As a result, these teeth are the most exposed to fire
and the enamel and dentin on their anterior surface can
simply crack off when touched. To prevent the loss of tooth
structure, Mincer and associates (1990) have listed a variety of materials that can stabilize incinerated teeth. They
endorse clear acrylic spray paint and cyanoacrylate glue
as the best agents since these are readily available, economical, portable, permeate the teeth well and set fast.
However, in their study, not all specialists surveyed considered reinforcing incinerated teeth essential. Many felt
problems could be circumvented by very careful handling
of the teeth or photographing the teeth prior to handling.
Intraoral radiography without jaw opening can be accomplished by removing the tongue through the floor of the
mouth and inserting the films through the opening. This
has been referred to as the tunneling technique by
Griffiths and Bellamy (1993), who have also suggested the
use of material such as bubble plastic or a sheet of foam to
protect the fragile skeletal and dental remains during
transportation.
Jaw Resection
Depending on the circumstances of death and condition of
the body, the forensic dentist can decide to cut open the soft
tissue of the cheek to reach the teeth, or remove the jaws
entirely. Resected jaws are easier to examine and radiograph.
However, such invasive procedures can pose obstacles,
especially when the body needs to be viewed by relatives.
Furthermore ethical and legal hurdles may be faced.
Therefore, one must consider jaw resection as a last option
and obtain prior permission from the forensic pathologist.
659
Figure 3
A
(A, B) Intraoral approach for myotomy: when the temporalis is attached to the top of the coronoid process, myotomy is
accomplished with scissors only; (C, D) in case the temporalis is attached both to the coronoid and mandibular notch,
a channel retractor is inserted to draw forward the deep end of the muscle to facilitate dissection. (Reprinted from Nakayama Y,
Aoki Y, Niitsu H, et al (2001), Forced oral opening for cadavers with rigor mortis: two approaches for the myotomy on
the temporal muscles, Forensic Science International, Vol. 118, pp. 3742, with permission from Elsevier)
Incinerated Teeth
Crown
Muller and associates (1998) have undertaken one of the
few detailed works on changes that teeth undergo when
exposed to high temperatures. They observed that enamel
shows crazing (fine surface cracks) at 150C but retained
its normal color although at 200C, enamel loses its gloss.
Further increase in temperature causes more crazing
initially over the neck, then more coronallyfollowed by
a few frank cracks.
Initial separation of enamel from dentin occurs at 400C
and the enamel shell separates totally at 450C. At 500C,
Harsnyi (1975) has observed deep longitudinal furrows on
the crown which almost divide it into several pieces. At this
temperature, the pulp chamber and root canal are preserved
and not narrowed. Above 500C, the crown undergoes
fragmentation and consistently becomes smaller with further rise in temperature. The narrowed cavities of the pulp
chamber and root canal are recognizable even at 1,100C.
The coronal dentin is exposed at 450C (following total
separation of the enamel shell). Cracks appear at 600C with
disintegration from 800C onward. While enamel is converted to powder, dentin is not. This has been attributed to
hypermineralized pretubular zone of the dentin. Myers
and colleagues (1999) believe that the mineral content of
dentin, located within its organic matrix composed of type I
collagen, helps stabilize the collagen against thermal denaturation and shrinkage. The color changes in the crown
and root at different temperatures has been demonstrated
by Muller and associates (1998) (Table 1). However, these
authors have cautioned that in real-life scenarios, it may be
much more difcult to observe the color changes since teeth
found at the site of re need to be carefully cleaned. They
added that the presence of a supercial metallic layer,
Root
200
Crown
300
Crown
Root
Black
Crown
Dark brown/gray
Root
Shiny black
Crown
Root
Gray/brown
Crown
Root
Crown
Root
Crown
Root
Crown
White dentin
Root
Crown
White dentin
Root
Crown
Chalky white
Root
Chalky white
Crown
Chalky white
Root
Chalky white
400
500
600
700
800
900
1,000
1,100
1,150
Appearance
Crown
Tongue Hemorrhage
Hemorrhage of the tongue can be observed associated with
strangulation deaths. Bockholdt and Maxeiner (2002) consider tongue hemorrhage to be the result of either cranial
congestion, compression by the dental arches or the hyoid
bone pressing onto the base of the tongue following neck
compression. These authors have reported tongue hemorrhage in 71% of homicidal strangulations, 19% of which were
the result of biting caused by compression of the tongue
between the teeth. However, tongue injury was observed
in only 5% of suicidal strangulations. A protruded tongue,
compressed between the jaws, is observed in a significant
number of suicidal hangings although the number of grossly
visible tongue bites with hemorrhages is small; a similar
small number of internal hemorrhages restricted to the tip
or anterior part of the tongue (development during a protrusion of the tongue in the course of the hanging agony)
are evident. However, extensive internal hemorrhages in
suicidal strangulations should be treated with some degree of
cautionBockholdt and Maxeiner (2002) have cited reports
of internal tongue hemorrhage in victims who were first
strangulated and then hung by the killer (simulated suicide), i.e. extensive internal tongue hemorrhage could indicate a presumed sucide as being a homicide. But they may
also be the result of highly atypical suicidal strangulation.
SOCIAL PROFILING
According to van Wyk (1976), information about habits and
occupational antecedents can be obtained from the dentition.
This can provide valuable information on the persons social
background and prove useful in identification. Based on
personal observation and epidemiological studies, van Wyk
(1976) categorized such features as: (i) lesions of the teeth
only, (ii) lesions of the teeth and soft tissues, and (iii) lesions
of the soft tissues only. This section, however, will emphasize on the lesions of the teeth, since they are more likely
to survive postmortem vagaries than soft tissues.
According to Gustafson (1966), grooving of the upper
anterior teeth may be encountered in cobblers, carpenters
and electricians. Such a groove commonly results from the
habit of opening hairpins with an upper central incisor
the constant abrasion of the metal pin against the tooth
edge eventually leaves a shallow groove. Among pipe
662
can be a crucial piece of evidence in identifying the edentulous as they remain intact or only slightly damaged following
traumatic accidents. Dentures may have been fabricated to
replicate an individuals dentition. For example, Taylor and
coworkers (2002) were able to positively identify an individual based on, among other evidences, distinct features of a
maxillary denture which contained a midline diastema and
gold inlays on two of the teeth (Figure 4). In addition, the type,
size and shape of teeth used in the denture can be used for
identification. This, however, warrants detailed dental records.
Therefore, use of denture-marking systems may be more practical; comparison of palatal rugae may also hold some use.
Figure 4
Figure 5
A
Denture marking microchip with inscription (A) and the microchip incorporated in a maxillary denture (B).
(Reprinted from Rajan M, Julian R (2002), A new method of marking dentures using microchips, Journal of Forensic
Odonto-Stomatology, Vol. 20, No. 1, pp. 15, with permission from authors)
663
denture of the deceased can be compared to previous dentures recovered from the decedents home, or plaster models that may be available with the dentist or laboratory
technician. Palatal rugae are well protected by the orofacial tissues in fires and traumatic accidents. In a recent
pilot study, Muthu, Subramanian and colleagues (2005)
have found that rugae were unaltered in 93% of victims of
third-degree burns; they also discovered that the rugae
withstood decomposition and were recognizable in more
than 80% of the cases. Furthermore, rugae patterns are
unique to an individual and can be used in identification
Limson and Julians study (2004), which obtained highpercentage accuracy in identifying individuals in a simulated ante- and postmortem comparison of the palatal
rugae, bears reasonable testimony to this premise. These
authors employed a softwareRUG FP-ID Matchfor the
comparison. First, rugae outlines on the casts were demarcated using a graphite pencil and photographed. The
image was transferred to a computer where the medial and
lateral ends of the rugae were marked manually (Figure 6).
Based on the principle commonly employed in fingerprint
analysis, the software compared the marked points
between ante- and postmortem cast images to ascertain a
match.
CRANIOFACIAL IDENTIFICATION
In cases where comparative dental identification is not
possible due to reasons such as unavailability of dental
Figure 6
664
Figure 7
A
Cranial suture pattern of different skulls. (Reprinted from Sekharan PC (1985), Identification of skulls from its suture pattern,
Forensic Science International, Vol. 27, No. 3, pp. 20514, with permission from Elsevier)
Skull-Photograph Superimposition
Superimposing the facial photograph (of the suspected
deceased) and skull was first successfully put to use in a
criminal case by Glaister and Brash (1937). They superimposed antemortem photographic negatives of the heads of
two homicide victims to the respective postmortem skull
radiographs and obtained positive identification. Since the
anterior dentition may be visible on many personal photographs, the use of tooth size, shape, angulations may be of
particular relevance in superimposition. However, adjustments need to be made to orient the ante- and postmortem
images. It is advisable to use superimposition as a corroborative tool, rather than the sole method, for identification.
Actually, it may have more value in exclusion rather than
identification per se. Nevertheless, it gains prominence in
the Indian context since research and case work have been
reported on a regular basis. The infrequent availability of
quality dental records has probably encouraged its use in
forensic identification. It includes photographic superimposition, video superimposition and newer digital techniques.
Photographic superimposition
The conventional superimposition method, photographic
superimposition utilizes antemortem photographs, radiographs or portraits (Nickerson et al, 1991) for comparison
with the skull specimen at hand. The skull is photographed
and its tracing laid over that of the antemortem photograph.
The superimposed images are carefully studied to determine
how closely the skull and face fit each other. The general size
and shape of the skull and bony landmarks such as the orbits,
nasal cavity, dentition, etc. are used to indicate the proximity
of the fit. The facial soft tissue thickness should also be considered during superimposition. Recently, Bilge and coworkers
(2003) have reported a case where identification was established by superimposing a photograph of an unidentified skull
with the antemortem photograph (Figure 8) using commercially available software such as Adobe Photoshop. They
state that the photo-to-photo superimposition technique
is quite a supportive system for positive identification, especially when the questioned person displays uncommon features, and good quality photographs are achieved.
As an aid to enhance the success of photographic
superimposition, Jayaprakash and colleagues (2001) have
suggested that the skull surface details be studied carefully
and correlated to that of the face photograph before drawing a conclusion. The similarity between the dry skulls and
photographs with respect to, for example, the frontal eminence or nasal bone morphology (Figures 9 and 10) can
be correlated adding due weight to the superimposition.
Nevertheless, a number of practical problems may undermine the outcome of photographic superimposition:
666
Figure 9
A
Figure 10
A
Challenges in Superimposition
Video superimposition
The advent of videography led to improvements in conventional still photography superimposition. Video superimposition usually makes use of two video cameras, one
focussing on the skull and another on the antemortem
photograph. The resulting images are mixed into one
composite image (Nickerson et al, 1991) using image mixing devices. The advantage of video superimposition over
photographic superimposition is that the skull can be
placed on a flexible mounting device and its angular position and distance from the camera adjusted according to
the position of face on the photograph. In addition, image
mixers enable added advantage of fade-in and fade-out,
and vertical and horizontal sweep mechanisms. This
allows different images to be progressively superimposed
upon each other.
FACIAL APPROXIMATION
According to Phillips and coworkers (1996), facial approximation is a combination of art and science in which a 3D
representation of the facial features is produced using the
skull as foundation. These authors have described the following steps to manually approximate a face: first, one
needs to make an assessment of the ethnicity, sex and age
using the skull bones and teeth. This information can add
value to, and assist in, the approximation. The mandible is
articulated with the skull and jaws and the soft tissue profile is drawn around the underlying bone using average
facial tissue thickness; the nasal profile can be constructed
The use of three-dimensional (3D) superimposition imaging techniques has been suggested by Nickerson and
coworkers (1991). The antemortem photograph is digitized
to produce a two-dimensional (2D) image whereas the
skull is laser-scanned to produce a 3D image. The 2D digitized facial image is mapped onto a polygona process
known as texture mapping. The texture-mapped image
can be manipulated as easily as the 3D skull model. This
texture-mapped image is then projected onto the 3D skull
Clay Modeling
667
Computer-assisted Approximation
Vanezis and associates (2000) have developed a computerassisted 3D facial approximation technique. To begin with,
the defects and natural orifices of the skull are covered
with cotton and the skull placed on a turn-table. The table
is rotated 360 and scanned under a low-intensity laser
beam. This results in the production of 200250 skull profiles which are fed to a computer system to enable viewing
of a 3D skull image (viewing is achieved by means of a
Facial Reconstruction software). The same software is then
used to mark landmarks on the skull image at which the
facial depth has been previously determined (Figure 11).
From a database of facial templates, a face is chosen
which has average features and matches the skull anthropologically; landmarks corresponding in location to those
of the skull are placed on the face (Figure 12). The facial
template is then carefully placed on the skull image so that
Figure 11
A
Views of digitized image of the skull with landmarks in position. (Reprinted from Vanezis P, Vanezis M,
McCombe G, et al (2000), Facial reconstruction using 3D computer graphics, Forensic Science International,
Vol. 108, pp. 8195, with permission from Elsevier)
668
Figure 12
A
Anthropologically matching facial template with landmarks in position. (Reprinted from Vanezis P, Vanezis M,
McCombe G, et al (2000), Facial reconstruction using 3D computer graphics, Forensic Science International,
Vol. 108, pp. 8195, with permission from Elsevier)
Figure 13
A
The facial template has been warped on the skull-image to produce an approximated face. (Reprinted from Vanezis P,
Vanezis M, McCombe G, et al (2000), Facial reconstruction using 3D computer graphics, Forensic Science International,
Vol. 108, pp. 8195, with permission from Elsevier)
Estimating age by observing the teeth clinically is a convenient and economical method. In clinical set-ups, the
age of children is routinely estimated by examining the
type of tooth that has emerged into the oral cavity
although, this method falls short of scientific credibility. A
more objective clinical count of the number of erupted
teeth has also been developed for assessing age in young
669
Table 2
Stage
Coded symbol
Ci
Coalescence of cusps
Cco
Coc
Cr
Cr
Crown complete
Crc
Ri
Cli
Rc
Ac
Demirjians method
Figure 14
T
P
Figure 15
A
Two distinct bite marks (A, B) on the back of a victim made by the same dentition. In both photographs, mandibular arch mark is
further from the scale. (Reprinted from Sheasby DR, MacDonald DG (2001), A forensic classification of distortion in human
bitemarks, Forensic Science International, Vol. 122, pp. 758, with permission from Elsevier)
Type of Injury
A bite mark may appear as an indentation, contusion, abrasion, laceration or avulsion. Initially, compression of the skin
Site of Injury
Bite marks may be found on any part of the body (Pretty
and Sweet, 2000). However, females are bitten most often
on the breast followed by the arms, legs and thighs while
males are bitten on the arm, hands/fingers and the back.
Interestingly, many of the male victims received bites during the course of a crime committed by them, i.e. they were
bitten by their victims, probably in self-defense.
673
Figure 16
1. Very mild bruising, no individual tooth marks present,
diffuse arches visible, may be caused by something other
than teethlow forensic significance
Increasing severity
2. Obvious bruising with individual, discrete areas associated with teeth, skin remains intactmoderate forensic
significance
3. Very obvious bruising with small lacerations associated
with teeth on the most severe aspects of the injury, likely
to be assessed as definite bite markhigh significance
4. Numerous areas of laceration, with some bruising,
some areas of the wound may be incised. Unlikely to
be confused with any other injury mechanisma high
forensic significance
High
forensic
significance
5. Partial avulsion of tissue, some lacerations present indicating teeth as the probable cause of the injurymoderate
forensic significance
6. Complete avulsion of tissue, possibly some scalloping
of the injury margins suggested that teeth may have
been responsible for the injury may not be an obvious
bite injurylow forensic significance
The bite mark severity and significance scale. (Reprinted from Pretty IA (2006), The barriers to achieving an evidence base for
bitemark analysis, Forensic Science International, Vol. 159S, pp. S11020, with permission from Elsevier)
674
Figure 17
A
Visual index of the bite mark severity and significance scale. (Reprinted from Pretty IA (2006), The barriers to achieving an
evidence base for bitemark analysis, Forensic Science International, Vol. 159S, pp. S11020, with permission from Elsevier)
Photographs
High-quality photographs provide a permanent record of
the bite mark appearance. No time should be lost in
obtaining photographs as the injury rapidly changes
appearance due to healing in living victims or postmortem change in the deceased. Color and black-and-white
photographs may be taken using off-angle light source.
Orientation and close-up photographs (Figure 18) should
be taken: orientation photographs depict the location of
the bite mark on the body; close-up photographs of the
bite mark should be made with a rigid reference scalea
ruler, such as the ABFO No. 2 scale, should be placed on
the same plane as the bite mark. The entire scale and bite
mark must be visible on the photograph. A second closeup photograph depicting the bite mark without the scale
can be made to indicate that no portion of the mark has
been concealed by the scale. The camera should be positioned directly over the injury site with the long axis of the
lens perpendicular to the surface of the bitten skinthis
decreases perspective distortion of the image due to offangle camera position (Sweet and Pretty, 2001). If the bite
is on a curved surface and the upper and lower arch marks
are widely spaced, separate photographs of each mark
Saliva Swab
It is reasonable to assume that a bite cannot be inflicted
without leaving saliva behind. Saliva deposited in the bitten
area may be a source of cellular DNA, enabling a direct link
to the suspect. Care should be taken not to wash the bite
area before saliva swabbing. According to Clift and Lamont
(1974), the amount of saliva deposited in a bite mark is
about 0.3 ml, distributed over an area of 20 cm2. However,
by the time of evidence collection, the bite area may have
dried up. Therefore, a cotton swab moistened with distilled
or clean tap-water should be used for swabbing. This
rehydrates the dry cells in the bite area. The swab is held
vertical along its long axis making circular motion with
moderate pressure over the bitten surface. A second swab,
which is dry, is used to collect the moisture left on the skin
by the first swab and cells that remain. This constitutes the
double swab technique recommended by Sweet and associates (1997) and is supposed to yield higher amounts of
salivary DNA. Both swabs are then air-dried at room temperature for about 30 minutes. Following this, the swabs
should be placed in paper envelopes to facilitate continued
air circulation around the swab tips (Sweet and Pretty,
2001). These are labeled and stored under refrigeration.
The latter prevents degradation of salivary DNA and bacterial growth in case of delay in lab processing. A third
control swab may be taken from an anatomically similar
Figure 18
A
(A) Anatomical location of bite mark on victims left shoulder; (B) close-up photograph of the bite mark.
(Note: The use of flexible scales is not recommended for bite mark evidence collection and ABFO No. 2 scale (Figure 17)
is preferred). (Reprinted from Pretty IA (2006), The barriers to achieving an evidence base for bitemark analysis,
Forensic Science International, Vol. 159S, pp. S11020, with permission from Elsevier)
676
testimony that proves that the evidence has not been altered
or tampered with in any way since it was obtained. This is
necessary both to assure its admissibility in courts of law
and its probative value in preceding investigations.
All evidence obtained must be labeled and stored appropriatelythe case number, date, time, place, as well as any
witnesses involved must be recorded at every step of evidence collection to maintain the chain of custody. In practical terms, a chain of custody is the documentation and
677
Figure 19
process is yet to be achieved and conclusion of the analysis is usually based on the experts level of personal experience and judgment (Rothwell, 1995).
Vertical grooves*
Branched grooves*
Bifurcated grooves*
Intersected grooves
Reticular grooves
Other grooves (comma, ellipse, triangle, horizontal,
etc.)
Figure 20
A
(A) Invisible lip print prior to and (B) after developing with lysochrome powder. (Reprinted from Navarro E,
Castell A, Lpez JL, et al (2006), Criminalisitic, effectiveness of lysochromes on the developing of invisible
lipstick-contaminated lipmarks on human skina preliminary study, Forensic Science International,
Vol. 158, pp. 913, with permission from Elsevier)
However, further studies on whether the lip prints developed can be accurately compared to those of a suspect are
warranted. According to Tsuchihashi (1974), lip grooves
are on the zone of transition of the lips which, being
extremely mobile, are affected by the pressure applied by
the lips and their direction. Consequently, there is the possibility that lip prints of one individual may be mistaken
for anothers. However, the classication provided and
quadrant-based recording of the lip grooves will probably
render a successful comparison. The suggested method for
comparison is to examine lip prints closest to the mid-line
and then work laterally (Tsuchihashi, 1974). Whether lip
print evidence is admissible in court and can stand the
rigors of legal scrutiny is a matter of debate. According to
Ball (2002), there is very little science and research to support a methodology for collecting and comparing lip print
evidence which has gained acceptance within the forensic
community. Further research to verify the uniqueness of
lip prints, develop protocols for lip print investigation and
establish its accuracy in comparative analysis is essential,
without which it would fail to meet any scientic standards of reliability (Ball, 2002).
679
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Oral Radiology
II
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SECTION
IX
Basics of
Radiology
685
697
683
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CHAPTER
25
2.
3.
Franklin W McCormack
Edmund C Kells (18561928)
Edmund Kells was born in New Orleans, Louisiana, to a
dentist Charles E Kells. He became a dentist, researcher,
and inventor. His experiments caused the loss of most of
his left arm. In 1899, he set up the first X-ray laboratory
(The New Orleans X-Ray Laboratory) for radiographs and
fluoroscopic examinations. He demonstrated the radiograph of chest and hip, a bullet in the head and the measurement of a root canal. He hired the first acknowledged
dental assistantMalvina Cuera, before which, his wife
686
Franklin McCormack, an American medical X-ray technician, employed paralleling technique principles in intraoral radiography. He wrapped the film with a black paper
and used a flat metal plate to make the film packet rigid.
He was also known for using bite blocks and hemostat as
film holders to stabilize the film in the mouth.
Readers are encouraged to visit the website of the
roentgen museum for more information on the chronology of events in origin of radiology (http://www.roentgenmuseum.de).
Particulate Radiation
Particulate radiation consists of a stream of atomic or subatomic particles that transmit kinetic energy by means of
their small masses moving at very high velocities. They may
carry a positive charge (alpha particles), negative charge
(beta particles) or no charge (neutrons). Examples of particulate radiation are alpha rays, beta rays and cathode rays.
Electromagnetic Radiation
There are two concepts to understand electromagnetic
radiation, namely, classical theory and modern quantum
theory. According to the classical theory, the flow of
energy at the universal speed of light through free space or
through a material medium is in the form of the electric
and magnetic fields that make up electromagnetic waves
such as radio waves, visible light, and gamma rays. In
such a wave, time-varying electric and magnetic fields are
mutually linked with each other at right angles and perpendicular to the direction of motion (Figure 1).
In terms of the modern quantum theory, electromagnetic radiation is the ow of photons (also called light
quanta) through space. Photons are packets of energy (h)
that always move with the universal speed of light. The
symbol h is Plancks constant, while the value of is the
same as that of the frequency of the electromagnetic wave
of classical theory. The spectrum of frequencies of electromagnetic radiation extends from very low values over the
range of radio waves, television waves, and microwaves to
visible light and beyond to the substantially higher values
of ultraviolet light, X-rays, and gamma rays.
Figure 1
Propagation vector
Electric field
Magnetic field
Electromagnetic spectrum
687
Production of X-rays
Figure 2
1
10, 000
1
1000
1
100
1
10
1
X-ray radiography
10
100
Ultraviolet ray
Sun lamp
1000
10,000
100,000
1,000,000
Measured in meters
X-ray therapy
1
1000
1
100
1
10
1
Light ray
photography
Infrared ray
toaster
Microwave
oven
Radar
10
100
1000
10,000
Television
100,000
1,000,000
Radio
10,000,000
100,000,000
Non-ionizing radiation
Ionizing radiation.
Figure 3
X-ray photon
Near miss
Direct hit
X-ray photon
High speed electron
Target
Bremsstrahlung radiation
Figure 4
A
Characteristic radiation. (A) Interaction with inner shell electron. (B) interaction with outer shell electron.
Courtesy: Dr Jaideep Shekhar
Properties of X-rays
1.
2.
12.
13.
14.
15.
16.
17.
18.
Figure 5
Figure 6
Figure 7
Figure 9
A
(A) X-ray tube head with a long cone. (B) X-ray tube with a short cone
691
Figure 10
4
5
10
11
12
7
9
13
14
15
1. Swivel arms
2. Yoke
3. External housing
4. Step down transformer
5. Step up transformer
6. Borosilicate glass housing
7. Cathode
8. Molybdenum focusing cup
9. Tungsten filament
17
16
Figure 11
Low vapor pressureit helps in maintaining the vacuum in the tube at high temperatures during the operation of the machine.
692
Figure 12
Figure 13
2.
3.
4.
5.
Figure 14
Rectangular collimators
Diaphragm collimators (Figure 15)
Tubular collimators.
A rectangular collimator (Figure 16) reduces the skin surface exposure by almost 60% compared to that of a diaphragm collimator.
Figure 15
Figure 17
Coherent scatter
Compton scatter
Photoelectric
absorption
Figure 18
Figure 16
Scattering
Coherent scatter
Rectangular collimator
Absorption
Photoelectric effect.
Compton scatter accounts for about 62% of the interactions of X-rays with matter. It occurs when a X-ray photon interacts with an outer electron. The incident photon
collides with the electron, which receives kinetic energy
and recoils from the point of impact. The incident photon
is deflected by its interaction and scattered from the site of
collision (Figure 19).
695
Figure 19
Absorption
Photoelectric effect
Photoelectric effect accounts for about 30% of all the
interactions of X-rays with matter. It is characterized
696
Figure 20
CHAPTER
26
Radiation Biology
Shubhasini AR, Bhanushree R, Praveen BN
Sources of Radiation
Natural Radiation
Man-made Radiation
697
structure remains unaffected. Exposure of enzymes, however, has a cascading effect, since altered enzymes may not
perform their physiological actions, resulting in intracellular molecular alterations. These changes occur at radiation
doses much higher than that which causes cell death.
The nucleus is more often affected due to radiation, but
changes can occur in mitochondria on exposure to higher
doses of radiation. Mitochondria become swollen with disorganization of internal cisternae.
Osteoradionecrosis
It refers to the secondary infection of irradiated bone.
Radiation exposure to bone affects the vascularity of bone,
destroys osteoblasts and some osteoclasts. The normal
vascular bone marrow is converted to fatty and fibrous
marrow. Thus, the marrow tissue becomes hypoxic, hypovascular and hypocellular.
If secondary infection is then superimposed on irradiated bone, the bone readily undergoes necrosis. Infection
may occur from sequelae of caries, periodontitis, denturesore or a tooth extraction. Osteonecrosis is more common
in mandible than the maxilla, presumably because of the
rich vascular supply of maxilla, and also since the mandible is irradiated more frequently.
Thus, before the initiation of radiotherapy, the dental
status of a patient should be assessed. Any pre-existing
disease such as caries, periodontal disease, third molar
pathology, defective restoration, etc. should be treated.
X-ray induced case of severe dermatitis was published in July 1896. The first dose limit of 10 rad
per day (or 3,000 rad per year) was recommended in
1902 based on fogging observed on a photographic
plate.
The destructive effect of X-rays on living tissues,
such as skin, blood forming-organs and reproductive organs was first evident by the animals studies
performed in 1903.
In 1928, the first formal radiation unitthe roentgen,
was adopted. In 1953 and 1954, the bone marrow
dose limit of 300 millirem (mrem) per week and skin
dose limit of 600 mrem per week was adopted by
ICRP and NCRP (National Council on Radiation
Protection and Measurements), respectively.
An annual occupational dose limit of 5 rem per year
was recommended by ICRP in 1957. A lifetime occupational dose limit of 235 rem for individuals in the
age group between 18 to 65 years and an annual
dose limit of 500 mrem per year to public was recommended by NCRP in 1958. The three parts of
dose controljustification, optimization and limitation was put forth by ICRP, in 1961 (Table 3).
The ALARA (As Low As Reasonably Achievable)
guidelines for radiologic protection for radiation
personnel, the maximum annual radiation dose limit
of 5 rem per year was recommended.
In 1980, ICRP limited the radiation exposure to 10 rem
over any 5-year period and 5 rem in any 1 year. The
public limiting dose as 100 mrem per year averaged
over any 5-year period.
Table 1
Source
Dose (Sv)
Natural
Cosmic
0.4
Terrestrial
External
Radon
Other
0.5
1.2
0.3
2.4
Total
Man-made
Medical (estimated)
Diagnostic X-ray
Nuclear medicine
2
0.5
0.08
0.01
0.01
0.01
0.01
2.5
Total
Cosmic sources
It is estimated that cosmic radiation accounts for about
8% of the annual total body exposure.
In different parts of the world the cosmic radiation varies
in dosage due to difference in altitude (radiation doubles for
every 6,000 feet) and magnetic eld. The sources of cosmic
radiation are protons, electrons, X-rays and gamma rays.
Terrestrial sources
Terrestrial sources originate from both external and internal
sources; external sources being soil and internal sources,
including radon and other nuclides that are inhaled or
ingested. Radon contributes 1.2 mSv to natural radiation
and the average whole body radiation from it is 228 mrem
per year. Potassium-40 and carbon-14 are two of the main
enlisted internal sources of radiation. The entrapment of
radioactive materials by the bodys own tissues through
the soil, water and air constitute about 11% of our total
annual exposure.
Man-made Radiation
Figure 1
Nuclear
medicine
4%
Consumer
products
3%
Other
1%
Cosmic rays
8%
Terrestrial
8%
Internal
sources
11%
Sources of radiation
exposure radon
54%
Medical X-rays
11%
Natural Radiation
The natural background radiations are from three sources
cosmic radiation, terrestrial radiation and internal radiation.
The annual effective dose of radiation from cosmic and
terrestrial sources is about 2.4 millisieverts (mSv) worldwide. The average whole body radiation to an individual
from background radiation is estimated to be around
620 mrem.
702
Table 2
Unit of measure
Conversion equivalent
1 curie
1 becquerel
1 disintegration/second
1 millicurie (mCi)
37 megabecquerel (MBq)
1 rad
1 rem
1 roentgen (R)
1 megabecquerel (MBq)
1 gray (Gy)
100 rad
1 joule/kilogram
1 sievert (Sv)
100 rem
1 Gy Wr
1 coulomb/kilogram (C/kg)
3,880 roentgen
Dose limits
The regulatory bodies at the international and national
levels, namely, ICRP, its counterpart the NCRP in the United
States, and the Atomic Energy Regulatory Board (AERB) in
India have laid down recommendations for radiation safety
and protection to humans without limiting their beneficial
application. In spite of low dosage in dental radiography,
radiation should be minimized wherever practicable. The
efficacy of radiology department lies in wise choice of
investigative procedures which aims at reduced radiation
exposure and diagnostic accuracy.
1.
Dose limits
Application
Dose limits
Occupational
Effective dose
15 mSv
50 mSv
Panoramic radiograph
2.724.3
Rem
Sievert
Cephalometric radiograph
Whole body
0.05
Maxillomandibular MSCT
2801,410
50
0.5
15
0.15
Skin
50
0.5
Extremity
50
0.5
Examination
Skull radiograph
PA chest radiograph
Health risk
704
Public
Intraoral radiograph
6 years
Overweight (15%)
2 years
Alcohol ingestion
1 year
1 year
207 days
Home accidents
74 days
Drowning
24 days
7 days
Medical radiation
6 days
Occupational exposure
0.3 rem/yr from age 18 to 65
1 rem/yr from age 18 to 65
15 days
51 days
Effective dose
(mSv)
Equivalent period of
natural background
radiation
0.18
2.6 weeks
0.029
3.4 days
1 year
88
4 years
Barium meal
2.5 years
PA: posteroanterior.
Lumbar spine
35
Abdomen
25
Effects resulting from acute whole body external exposure of radiation to men
025 r
25100 r
100200 r
200300 r
300600 r
600 or more
No detectable
clinical effects
Slight transient
change in
lymphocytes and
neutrophils
Nausea and
fatigue with
possible vomiting
above 125 r
Latent period up to
2 weeks or perhaps
longer
Delayed effects
may occur
Disabling sickness
not common,
exposed individuals
should be able to
proceed with usual
duties
Reduction in
lymphocytes and
neutrophils with
delayed recovery
Diarrhea, hemorrhage,
purpura, inflammation
of mouth and throat,
fever toward the end of
first week
Delayed effects
possible, but
serious effects on
average individual
very improbable
Delayed effect
may shorten life
expectancy in the
order of 1%
Recovery in likely
about three months,
unless complicated by
poor previous health,
superimposed injuries
or infections
r: Rads.
NCRP
ICRP
Relative to stochastic
effects
Relative to deterministic
effects
150 mSv equivalent dose limit to lens of eye and 500 mSv
annual equivalent dose limit to skin and extremities
Relative to deterministic
effects
Embryo/fetus
None established
2.
3.
4.
5.
6.
7.
3.
4.
Figure 2
600
Examination
Effective
dose (mSv)
Equivalent period of
natural background
radiation
0.0023
8.8 hours
0.0043
17.5 hours
0.0083.7
1.5 days
0.0163.5
3 days
0.0074
1.3 days
0.0144
2.6 days
550
500
450
mA of 103 Gy
400
D
350
300
250
200
F
150
100
50
0
50
60
70
80
Kilovoltage
90
100
Digital Radiography
Numerical formatting with networked computer system in
obtaining high quality image constitutes digital radiography.
This adopted software technology in imageology has the
additional advantage of reduced exposure to both patient
and operator, speedy recovery of the image, digital storage
of the image with its electronic transmission, discontinuation of chemical processing and its associated hazardous
wastage, elimination of lead foil, saving darkroom equipment and adjustable diagnostic quality of the image.
Intensifying Screen
The rare earth phosphors in modern intensifying screens
emit green light on interaction with X-rays. The rare earth
intensifying screens decrease patient exposure up to 55%
in panoramic and cephalometric radiography.
A further reduction in patient exposure during extraoral radiography may be achieved with the use of T-grain
lm. Introduced as T-Mat by the Eastman Kodak Company
in 1983, this lm contains silver halide grains that are
tabular or at in shape. With its at surface and greater
cross-section oriented toward the X-ray source, it can
gather more light from intensifying screens.
T-grain lm used with rare earth screens is twice as fast
as calcium tungstate screen-lm combinations and thrice
as fast as conventional rare earth screen-lm combinations
with retained image quality. The T-grain technology was
incorporated into intraoral lm by Kodak to achieve the
faster speeds of E and F lms.
Extraoral lms exposed by intensifying screens achieve
a level of image resolution that is about half of the direct
exposure intraoral lm. One reason for image degradation
in extraoral imaging systems is crossover; which refers to
the loss of image sharpness and resolution resulting from
light emitted by one screen passing through the X-ray lm
to expose the emulsion on the opposite side of the double
emulsion lm. The Ultra-Vision (DuPont) and Ektavision
(Kodak) screen lm systems were designed to minimize
crossover by using phosphors that emit ultraviolet light,
which is less able to pass through the lm base to expose
the opposite emulsion.
707
Results have shown that images produced by these systems have higher resolution than corresponding rare earth
screen-lm systems. This allows for the use of a screen
one speed class higher and a 50% reduction in patient
exposure.
Unlike digital intraoral imaging, there is no signicant
dose reduction to be gained by replacing extraoral screenlm systems with digital imaging. Image resolution with
digital systems appears to approach that obtained with rare
earth regular-speed screens matches with T-Mat lm.
Grids
Grids are the devices used in extraoral radiography to
improve the image quality by reducing the scattered radiation. Though the grids require slightly higher exposure, the
advantage of obtaining high quality radiographs, transcend
the minor risk of increased radiation dose to the patient.
This in turn reduces the repeated chance of exposure.
Figure 3
16" distance
8" distance
Image
Film
Figure 4
708
Figure 5
300 mremfilm
90 mremdigital radiography
140 mremcomputed
radiography
20 mrem
300 mrem
350 mrem
30 mrem
100 mrem
175 mrad
lter and for above 70 kVp, 2.5 mm aluminum lter are the
recommendations.
Figure 6
Cylinder tip
Cylinder
Lead
diaphragm
Aluminum filter
Aluminum filter
Milliampere-seconds
Exposure time is the most vital factor in influencing
diagnostic quality. Both overexposed and underexposed
radiographs result in repeated exposures, thereby leading
to needless additional patient exposure. Milliampere-second
(mAs) is the combination of milli amperage and exposure
time affecting the quantity of X-rays produced and sequentially the image density.
Patient exposure is directly related to mAs. Typically a
radiograph of correct density will demonstrate very faint
soft tissue outlines. Enamel and dentin will have an optical
density of about 1.0. Optimal image density can be obtained
by using values listed, after considering the age and physical stature of the patient. For example, 3.5 mAs is suggested
for an average adult when F-speed lm and an operating
kilovoltage of 70 are used. This value may be arrived at
by using milliamperage of 10 and an exposure time of
0.35 second. If the kilovoltage is increased to reduce image
contrast, the mAs must be decreased or the radiograph will
be overexposed.
Phototiming technique uses a phototimer to measure
the quantity of radiation reaching the lm and automatically terminates the exposure when enough radiation has
reached the lm to provide the required density. A form of
this technology is currently available with some panoramic
machine.
Protecting Personnel
Radiation and humans are inseparable. Minimal usage
in daily activities, diagnostic and therapeutic modalities is
710
2.
3.
2.
3.
4.
Figure 7
Figure 8
Lead gloves
2.
Figure 9
2.
Shielding
Radiation shielding is a mass of radiation absorbing material placed around the radiation source to reduce the radiation to a safe level for humans.
Different types of shielding in diagnostic radiology are:
1.
2.
3.
4.
5.
Personnel shielding
Appropriate personnel shielding can be achieved by having
(i) a lead protective barrier of 1.5 mm between operator and
X-ray tube; (ii) lead apron and gloves of 0.25 mm thickness (Figures 8 and 9); (iii) lead gonadal shield of 0.5 mm
thickness. For any specialized radiological investigation,
Patient shielding
The susceptible organs, namely, thyroid, breasts and gonads
should be shielded in children and young adults with
0.25 mm of lead thickness for lead apron, collar and gloves
and gonadal shield of 0.5 mm of lead thickness.
711
A few of the mandatory decisive factors about X-ray equipment room are:
1.
1.
2.
3.
4.
5.
2.
3.
Ionization
Photographic effect
Luminescence
Scintillation.
Ionization
The process of converting an atom or molecule into an ion
by adding or removing charged particles such as electrons
or ions is termed as ionization.
Principle
Figure 10
Radiation
source
+
A
Ionization
chamber
Ionization chamber
Figure 11
Photographic effect
Principle The ability of radiation to blacken the photographic film.
Application
Luminescence
Principle The property of certain materials that emit
light when stimulated by a physiological process, a chemical or electrical action, or by heat.
Functioning mode When radiation strikes luminescence
materials, the electrons are raised to higher orbital levels.
When they fall back to their original orbital level, light is
emitted. The amount of light emitted is proportional to the
radiation intensity.
Example: Lithium fluoride will emit light when stimulated
by heat.
Radiation measuring device on luminescence property
is thermoluminescence dosimetry (TLD), a method used to
measure exposure to patients and personnel.
Scintillation
Personnel dosimeter
Personnel Dosimetry
1.
2.
3.
4.
Figure 12
1.
2.
During fluoroscopy
The protective apron should always be worn during fluoroscopy. Preferably two dosimeters should be worn by
radiation personnel.
One at the collar level outside the lead apron indicates
an accurate estimate of the radiation dose to the unprotected regions of head and neck; and the other at the trunk
level underneath the lead apron illustrates an accurate
estimate of the radiation to the protected organs. If only
one dosimeter is worn it must be worn at the collar outside
the lead apron, because, the neck receives 1020 times
more radiation than the trunk which is protected by lead.
TLD badge
714
Darkroom
For manual processing of films, well-equipped darkroom
under recommended guidelines is obligatory to get good
quality radiographs. This will indirectly reduce the exposure to the patient.
1.
2.
3.
Operational check
Daily
Monthly
Check and analyze problems with the film, developing process, the X-ray unit or the user
Six monthly
4.
5.
6.
Quality assurance
Continuing education
715
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SECTION
Radiographic
Methodology
719
724
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Chapter-27.indd 717
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CHAPTER
27
Intraoral Films
INTRAORAL FILMS
These films are used for periapical, bitewing and occlusal
radiography. These are also referred to as direct action films
(primary sensitive to X-rays) and non-screen films (not used
in combination with intensifying screens).
Extraoral Films
Cassettes
Intensifying Screens
Grids
The outer jacket (on the tube side/white surface) is incorporated with a raised dot, which relates with a similar area on
the dental lm inside the packet. This dot aids in orientation
of the lm during the exposure (always places such that the
dot lies closer to the incisal or occlusal aspect of the teeth).
Once the lm is processed the orientation of the raised dot
is used to identify the left and right sides of the patient.
Lead foil Once the outer jacket is opened a thin sheet of
lead foil is seen. This lead foil has parallel indentations or
markings along the surface in one corner of the foil. If the
film is exposed placing the wrong side toward the tube
head, these markings are seen on the resultant radiograph.
If the wrong side of the film is exposed, though the radiograph will reveal an image, the image will appear light.
The markings on the film will help to identify the cause for
Figure 1
Black-colored
paper wrapper
Lead foil
Outer protective
plastic jacket
719
1.
2.
Base
The base of the intraoral film is made up of polyester polyethylene terephthalate and is about 0.22 mm in thickness.
It is believed that a blue tinted base will improve the viewing characteristics of the radiograph.
Requirement of an ideal base:
1.
2.
3.
4.
Figure 2
Overcoat
B
A
S
E
Emulsion consisting of
silver halide grains
720
EXTRAORAL FILMS
These films are called screen films or indirect films as the
radiographic film is placed between two intensifying screens
within a cassette. Screen films are employed in extraoral
radiography such as orthopantomograph, lateral cephalograph, skull views, TMJ views, and lateral oblique views of
the mandible. Extraoral films are usually available in three
sizes: (i) 6 12 inches (orthopantomography), (ii) 8 10
inches (all other skull radiographs: PNS, PA view, lateral
cephalogram, etc.) and (iii) 6 8 inches (TMJ views, lateral
oblique).
Cassettes
Occlusal film
IOPAR film
size 2
IOPAR
pedodontic
film size 0
Various film sizes, namely, the occlusal, size 2 adult film and
size 0 child or pedo film
Cassettes are light tight containers, which help in maintaining a uniform and intimate contact between the film and
the intensifying screens. Cassettes are available in various
sizes and shapes conforming to the various sizes of radiographic film (6 8 inches, 8 10 inches and 6 12 inches).
These are usually made of plastic or thin metal. Basically
extraoral lm cassettes are available in two specications,
namely, rigid and exible cassettes.
1. Rigid cassettes These are made of thin metal framework.
The surface on the exposure side is made up of plastic and
the non-exposure surface has metal clamps, which help in
Figure 4
A
(A) Intensifying screens within an extraoral cassette. (B) Extraoral film cassette of the size 8 10 inches.
(C) Film cassette for orthopantomography of the size 6 12 inches
721
Figure 5
Figure 6
X-ray beam
Phosphor layer
Film
Reflecting layer
Base
Intensifying Screens
Intensifying screens are used in pairs and are positioned
on either side of a double emulsion film.
Composition of intensifying screen Intensifying screens
are made of four principal components: the base, reflecting layer, phosphor layer and the coat (Figure 5).
Base
The base of the intensifying screen is made up of the same
material that is used in an intraoral film, i.e. polyethylene
terephthalate. The base is usually 0.25 mm in thickness.
The base acts as a supportive material.
Reflecting layer
It is made up of titanium dioxide. It is placed between the
base and the phosphor layer. It helps in reflecting back
on to the phosphor layer any light that is emitted by it.
However, the reflecting layer may add to the unsharpness
of the resultant image.
Grid
are some of the salts that are used. The presence of these
salts has led to the naming of intensifying screens as salt
screens.
However, rare earth materials are used these days such
as terbium activated gadolinium oxysulde and thulium
activated lanthanum oxybromide.
Coat
The coat is made up of plastic and has a thickness of 8 m.
It provides protection to the underlying phosphor layer.
As the coat is made up of plastic it can be easily cleaned.
It should be ensured that the coat is free from scratches
and dirt.
Grids (Figure 6)
During a radiographic exposure, almost 30% of the scattered photons (formed as a result of Compton scattering)
leave the site imaged and exit the body. These scattered
photons will result in the formation of dark areas on the
radiograph, which interfere with the diagnostic quality of
the radiographic image. However, the use of grids increases
the patient exposure by two-fold.
Therefore, grids should be used only when contrast on
a radiograph is really necessary. As the grid contains radiopaque absorbing materials, the exposure time has to be
doubled when using a grid. If the exposure time is not
changed, the resultant radiograph might show multiple
thin white lines, which minimize the density of the radiograph. These lines that are seen on the radiographic image
are called grid lines.
Phosphor layer
Radiosensitive phosphor salts are incorporated into this
layer of the intensifying screen. Inorganic salts such as
calcium tungstate, zinc sulfide, and zinc cadmium sulfate
722
Composition
A grid is made up of alternating strips of lead (radiopaque
material) and plastic (radiolucent material) spacers (Figure 7).
Figure 7
Patient
Types of grid
t
Grid
Receptor
Gridline diagram
1.
2.
3.
4.
5.
Linear grid
Focused grid
Pseudo focused grid
Crossed grid
Moving grid or PotterBucky diaphragm.
Functions
Grids are placed between the object to be imaged and the
image receptor system (film) to preferentially minimize the
amount of scattered radiation reaching the film. Grids
reduce film fog from scattered radiation, thereby increasing the contrast of the resultant image.
723
CHAPTER
28
Radiographic Techniques
Gail F Williamson, Kvan Kamburoglu*,
Suman Jai Sanghar, Jai Sanghar N,
Rinky Nacchyon, Apeksha Mainali,
Ravikiran Ongole, Praveen BN
CONVENTIONAL IMAGING
Extraoral Radiography
TMJ Radiography
Transcranial View
Transpharyngeal View
(Parma Projection, Macqueen-Dell Technique)
Transorbital View
Technique
Landmarks for Positioning in Skull
Radiography
Posteroanterior Projection
Occipitofrontal Projection
PA Mandible
PA Cephalometric
Rotated PA View
30 Occipitomental
Parietoacanthial View
(Open Mouth Waters View)
Acanthoparietal Projection
(Reverse Waters Method)
Lateral View
Lateral Cephalometry
Intraoral Radiography
Panoramic Radiology
Origin of Panoramic Radiology
Concepts of Panoramic Imaging
Characteristics of Ghost Images
Indications of Panoramic Radiograph
Disadvantages and Limitations
SPECIALIZED IMAGING
Computed Tomography (CT)
Working Principle
Computed Tomographic Scanner Assembly
Advantages of CT
Disadvantages of CT
Uses of CT in Dentistry
*Dr Kvan Kamburog lu is grateful to Tomoloji Dentomaxillofacial Radiology Center and Gulhane Military Medical Academy, Dentomaxillofacial Radiology Center, Ankara for their support.
724
Fundamentals of CBCT
Advantages and Disadvantages/Limitations of
CBCT
CBCT Clinical Applications
Nuclear Medicine
Radiopharmaceuticals
Bone Scanning
Salivary Gland Scans
Sialography
Indications of Sialography
Procedure
Injection of the Contrast Medium
Phases of Sialography
Contraindications of Sialography
Arthrography
Uses of Arthrography
Types of Arthrography
Procedure
Limitations
Complications
Ultrasonography
Principle
Different Types of Scans
Different Types of Scanners Used
Instrumentation
Doppler Ultrasound
CONVENTIONAL IMAGING
INTRAORAL RADIOGRAPHY
Gail F Williamson
Intraoral radiography is an important tool for proper diagnosis and treatment. Intraoral radiographs allow the clinician to view the teeth and supporting structures revealing
conditions that may not be apparent clinically. Intraoral
radiography is accomplished by placement of a small
image receptor (IR) inside the mouth behind the teeth and
structures of interest with the X-ray beam aligned externally
over the corresponding area. Available intraoral receptors
include radiographic film, rigid digital sensors and photostimulable phosphor plates. Intraoral radiography involves
the acquisition of periapical and bitewing images that can
be combined to form surveys. Periapical radiographs record
the entire tooth or several teeth and the surrounding structures including the apical regions, the trabecular bone, the
periodontal ligament space and the lamina dura. By contrast, bitewings record the crowns, proximal contacts of the
teeth and the alveolar bone crests. Typically, a complete
indicated when three-dimensional (3D) imaging is necessary to evaluate and plan treatment in situations such as
implant placement, failed endodontic treatment, pathological conditions, difcult surgical procedures and evaluation
of craniofacial anomalies.
Figure 1
A
settings (Figure 3A, B). Kilovoltage controls the penetrating power of the X-ray beam and image contrast or differences in darkness. Milliamperage and time control the
number of X-rays and image density or the overall darkness of a radiographic image. Most intraoral X-ray
machines have fixed kilovoltage and milliamperage settings which permit adjustment of the exposure time only.
The length of the exposure time is dependent on a number
of factors including the kilovoltage and milliamperage
settings, collimation of the X-ray beam, patient size, area
of interest and the type of receptor.
Collimation restricts the size of the X-ray beam, reduces
patient exposure and improves image quality by reduction
of scatter radiation and minimizing penumbra production.
Collimation is accomplished with open-ended position indicating devices (PIDs) that are either circular or rectangular
in shape and vary in length from 20 to 40 cm (Figure 4).
Long rectangular collimation restricts the X-ray beam and
Figure 2
B
Figure 3
A
(A) Intraoral dental X-ray machine control panel. (B) X-ray head and PID;
HeliodentPlus Sirona Dental Systems, Inc., Long Island City, New York
726
Figure 4
(Figure 3A). Exposure time increases as the survey progresses from the anterior to the posterior regions of the
mouth. Digital receptor exposures follow these same general principles but the time settings are lower for each area
when compared to lm. The speed of the receptor has an
inuence on the exposure time as well. Speed indicates the
sensitivity of a receptor to X-rays; the faster the receptor,
the less the radiation required to produce a diagnostic
image. Currently, F-speed is the fastest lm available for
intraoral radiography, providing signicant dose reduction
and comparable performance compared to D-speed lm.
Digital receptors, both rigid sensors and phosphor plates,
provide equal or greater dose savings than F-speed lm
with equivalent diagnostic utility. Intraoral X-ray machines
manufactured today are compatible with lm, rigid sensors
and phosphor plates.
Figure 5
A
Vertical angulation. (A) Positive vertical angulation is used for maxillary periapicals as demonstrated by this premolar placement.
(B) Negative vertical angulation is used for mandibular periapicals as demonstrated by this incisor periapical placement
727
Figure 6
Figure 7
Outer canthus
Pupil of eye
Ala of nose
Tip of nose
728
Table 1
Patient type
Adolescent dentition
Adult dentate/artially
Adult edentulous
New patient
Bitewings/panoramic
Bitewings/panoramic
FMS when indicated
Bitewings/panoramic
FMS when indicated
Individualized
Based on exam
Bitewings at 612
month intervals
Bitewings at 612
month intervals
Bitewings at 618
month intervals
Not applicable
Bitewings at 1224
month intervals
Bitewings at 1836
month intervals
Bitewings at 2436
month intervals
Not applicable
Selected
PAS/BWS as needed
Selected
PAS/BWS as needed
Selected
PAS/BWS as needed
Selected
PAS/BWS as needed
Not applicable
Growth and
development
Not applicable
Not applicable
Other
Preliminary Procedures
Before radiographs are prescribed, a complete medical
and dental history must be taken, reviewed and the chief
complaint identified. The dentist should examine the dentition to determine if radiographs are indicated. If radiographs
are necessary, the type and number of projections should
be identified. The American Dental Association (ADA) and
the FDA have established guidelines for the selection of
dental radiographic examinations in the United States.
Dental radiographs should be prescribed according to
729
Figure 8
Figure 9
Figure 10
observation of restorative treatments and/or missing that
match from periapical to periapical and/or periapical to
bitewing will ensure accuracy of arrangement.
Paralleling technique
The paralleling technique requires placement of the receptor parallel to the tooth or teeth of interest both in the
vertical and horizontal planes. The X-ray beam is directed
so that it strikes both the tooth and the receptor at a right
angle (Figure 9). Typically, the paralleling technique is
accomplished with ringed instruments that aid the clinician
in establishing the correct object to receptor relationship,
730
Figure 11
Figure 12
Table 2
Figure 13
Periapical view
Maxillary
Mandibular
Incisor
40 to 50
5 to 15
Lateral-canine
40 to 50
5 to 15
Premolar
Molar
25 to 35
20 to 30
10 to 15
5 to 5
731
Figure 14
A
Rigid wire dental sensors. (A) Size 2 and 1 rigid digital sensors. Schick CDR Elite,
Schick Technologies, Inc., Long Island City, New York. (B) Digital molar periapical image
Figure 15
A
Phosphor plate receptors. (A) Size 0, 1, 2 and 4 phosphor plates. The opposite side or emulsion side is
directed toward the X-ray source. ScanX Phosphor Storage Plates, Air Techniques, Inc., Melville, New York.
(B) Phosphor plate premolar periapical
Photostimulable phosphor plates Photostimulable phosphor plates (PSPs) or storage phosphor plates (SPPs) are
wireless digital receptors that handle similar to film (Figure
15A, B). A separate plate is needed for each exposure and
a processing step is required before the images can be
viewed on the computer monitor. Phosphor plate receptors
have a single emulsion coated on the exposure side of the
plate composed of europium-activated barium fluorohalide.
Rather than an identification dot, plate receptors have a
letter or number to identify the exposure side of the receptor. Care must be taken to prevent abrasion, crimping or
creasing of the emulsion to avoid permanent image artifacts and frequent plate replacement. Once the plate is
exposed, the latent image is stored in the emulsion until
the plate is scanned by a helium laser beam (Figure 16).
When the phosphor plate is scanned, it emits light in proportion to the exposure received. The light detected by the
photomultiplier is converted from analog-to-digital data
and the visible image is displayed on the monitor for viewing. The scanning takes several seconds causing a slight
delay between image capture and display. Before reuse, the
plate must be erased by white light to remove any remnant
image. Typically, the plate is erased prior to exit from the
scanning unit. The primary advantages of the phosphor
plate are its thin, wireless construction while the disadvantages are susceptibility to emulsion scratches producing
artifacts and delayed image viewing.
Figure 16
Table 3
Head position: midsagittal plane perpendicular and occlusal plane parallel to floor
Projection
Teeth recorded
Horizontal angle
Vertical angle
Central ray
Maxillary incisor
periapical
Proximal between
central incisors
Tip of nose
Maxillary lateral-canine
periapical
Proximal between
lateral & canine
Ala of nose
Maxillary premolar
periapical
Proximal between
premolar teeth
Maxillary molar
periapical
Outer canthus to
mid-cheek
Premolar bitewing
Proximals between
premolar teeth
5
Pupil of eye to
occlusal plane
Molar bitewing
Proximals between
1st & 2nd molar
teeth
5
Outer canthus to
occlusal plane
Head position: midsagittal plane perpendicular and mandibular arch parallel to floor
734
Mandibular incisor
periapical
Proximal between
central incisors
Center of chin
Proximal between
lateral & canine
Mandibular premolar
periapical
Proximal between
premolar teeth
Mandibular molar
periapical
Outer canthus to
mid-mandible
Diagram
Figure 17
Bitewing radiography
Bitewing radiographs are most frequently taken in the
posterior regions of the mouth of the premolar and molar
teeth for the detection of proximal caries and alveolar
bone loss (Figure 19A, B). For adult patients, the size 2
receptor is preferred. Bitewings can be oriented in either
the horizontal or vertical plane with the receptor positioned
parallel to the teeth crowns. Horizontal bitewings are the
most common but vertical bitewings are taken particularly
when moderate or greater alveolar bone loss is present.
Anterior bitewings can be taken to view bone levels in the
anterior segments of the mouth as well (Figure 20). A size 1
receptor is used to capture incisor and lateral-canine bitewing views. The clinician can select traditional tabs or
instrument holders to accomplish the bitewing survey.
Figure 18
Figure 19
A
Bitewings can be taken with a ringed instrument or with a tab. (A) Bitewing taken with the tab bitewing technique.
(B) Tab bitewing survey
735
Figure 20
Head position
Placement
Maxillary anterior
Horizontal or vertical in
anterior region
60
Maxillary posterior
55
2 cm below pupil of
the eye
Mandibular anterior
Horizontal or vertical in
anterior region
15
Angulation
Central ray
Figure 21
A
Topographical maxillary anterior occlusal. (A) Clinical photograph. (B) Occlusal image
Figure 22
A
Topographical maxillary posterior occlusal. (A) Clinical photograph. (B) Occlusal image
occlusal plane is at a 45 angle above horizon. The clinician can align the X-ray head at a 45 angle to use as a
comparison to check for proper head position. The occlusal plane should be parallel to the open end of the PID. The
midsagittal plane is positioned perpendicular to the floor.
The receptor can be positioned horizontally or vertically as
needed. With the mouth opened slightly, place the receptor
against the occlusal surfaces of the mandibular teeth with
the dot convexity toward the tongue and positioned labially. The patient bites together lightly to secure the receptor. The approximate bisecting angle for this projection is
15. The horizontal angle is directed through the proximal contacts of the mandibular incisor teeth and the central ray is directed through the center of the mentum. The
exposure time is the same time as the mandibular incisor
periapical for selected receptor (Figure 23A, B).
737
Figure 23
A
Topographical mandibular anterior occlusal. (A) Clinical photograph. (B) Occlusal image
Table 5
Projection
Head position
Placement
Angulation
Central ray
Maxillary
Horizontal or vertical
in anterior sectant
1 cm posterior to bregma
Mandibular
anterior
Mandibular
posterior
Horizontal or vertical
in anterior sextant
Figure 24
A
Figure 25
A
Cross-sectional mandibular anterior occlusal. (A) Clinical photograph. (B) Occlusal image
eventually learn how to avoid them altogether. A diagnostic periapical depicts the area of interest including the
entire length of the teeth from the crown to the root apices
with at least 34 mm of bone surrounding the apices
and open proximal contacts. The image should have adequate contrast and density and be free of technical errors.
A diagnostic bitewing displays the area of interest, the
proximal surfaces of the maxillary and mandibular teeth
crowns and the alveolar bone crests. Like periapicals, adequate contrast and density and absence of technical errors
are necessary components of a quality image. When evaluating a full-mouth survey, every apex and every proximal
surface should be visible somewhere within the composite
of images.
A number of technical errors can be produced when the
parameters of intraoral technique are not properly followed.
Common errors encountered in intraoral radiography include
739
Figure 26
A
Cross-sectional mandibular posterior occlusal. (A) Clinical photograph. (B) Occlusal image
Table 6
Error
Description
Correction
Foreshortening
Elongation
Horizontal overlap
Cone cut
Central ray of the X-ray beam is not aligned with the middle of
receptor producing a partial image or cut
Improper placement
Underexposure
Evaluate the patient size, set the correct time for each view
and hold down the exposure button until the exposure is
complete
Overexposure
Evaluate the patient size, set the correct time for each view
are dot reversal and a light image from lead foil absorption of the X-rays. Backward plate placement results in
reversal of the letter or number identifier.
Vertical angulation
Receptor placement
The most common technical error regardless of the type of
receptor is placement. Each periapical and bitewing placement requires inclusion of specific structures on each view.
When the expected structures are not recorded, retakes may
be necessary to gain the missing information. In addition
to incorrect location, portions of the teeth such as the
crowns or apices can be cut-off and necessitate a retake
(Figure 27). Backward placement of film and plate receptors
is possible as well. Hallmarks of backward film placement
740
Figure 27
Figure 28
Horizontal overlap
Proximal overlap is the primary error that occurs when the
horizontal angulation is misdirected (Figure 30). Diagonal
rather than perpendicular entry of the X-ray beam to the
horizontal planes of the teeth causes superimposition of
the proximal surfaces from one tooth onto the next. The
proximal surfaces of the teeth and alveolar bone margins
are obscured from view and the structures are distorted in
width as well. This error is especially egregious on bitewings as it limits or prevents assessment of the teeth for
proximal caries and alveolar bone loss. The open end of
the PID should be horizontally parallel to the buccal plane
of the teeth of interest to direct the X-rays through the
teeth contacts (Figure 31).
Figure 29
Figure 30
Cone cuts
A cone or PID cut is the direct result of misalignment of the
central ray (Figure 32). When the central ray is not directed
to the middle of the receptor, a portion of the receptor is
not exposed and no image is produced in that region. Cone
cuts can occur on any aspect of a radiographic image. The
use of the classic receptor placement for each view and
central ray alignment with external entry points will help
eliminate cone cuts. Ringed instruments with beam guides
eliminate this error when properly assembled.
Exposure
A variety of exposure errors can occur that diminish
the diagnostic quality of a radiographic image. Improper
741
Figure 31
Figure 33
Figure 34
Figure 32
Intraoral radiography is indispensable for proper diagnosis and treatment of dental patients. A variety of intraoral projections can be taken to visualize the teeth and
surrounding structures including periapicals, bitewings and
occlusals. Intraoral radiographs should be prescribed by the
dentist according to selection criteria guidelines to ensure
that the benet of the examination outweighs the risk. The
clinician must have the requisite knowledge and skill to
effectively manage the patient and competently conduct all
aspects of the imaging procedure. Adherence to the tenants of radiographic technique will maximize the diagnostic result while at the same time minimize patient radiation
exposure through retake prevention.
EXTRAORAL RADIOGRAPHY
Technique
Extraoral radiography, in contrast to the intraoral radiography, is made with indirect exposure films, where the film
is placed inside a tight cassette between intensifying screen
on either side and then the exposure is made after proper
positioning of the patient with reference to the long axis
of the cassette.
Image receptors
Image receptors used here are otherwise known as screen
films or indirect exposure films, as they are used along
with intensifying screens. These screens emit visible light
on exposure to X-rays. The emitted visible light will in
turn expose the films. Due to the variation in the properties of intensifying screens, proper screen-film combination must be used as recommended by the screen and film
manufacturer so that the emission characteristics of the
screen match the absorption characteristics of the film.
Dimensions of the lms used in skull radiography are
8 10 inches and for lateral oblique views, it is 5 7 inches.
Intensifying screens
Due to density of the skull, intensifying screens must be
used to minimize the amount of radiation to the patient,
which also helps in reducing the scattered radiation. Intensifying screens permit a good radiograph to be produced
with the patient receiving a much lower dose of radiation.
ine
al l
eat
l line
lom
eata
m
o
Orbit
Infraorbitomeatal line
Acan
thiom
eatal
M
en
line
to
me
at
al
lin
e
Nasion
el
lab
External
acoustic meatus
Angle of mandible
(gonion)
Acanthion
Mental point
743
Radiation protection
Routine radiation protection measures should be applied.
The most effective method of dose reduction is a careful
technique to avoid the need for repeat radiograph.
Radiographic techniques
1.
Positioning errors
1.
2.
3.
4.
Lateral view
Modifications
Lateral cephalometric
Patient preparation
Figure 36
744
Standard occipitomental
Modifications
30 occipitomental
Parietoacanthial projection (Waters view, PNS view)
Open mouth Waters view
Modified parietoacanthial projection (modified
Waters)
5.
6.
7.
8.
Lateral oblique
Ramus of the mandible
Body of the mandible
9.
TMJ projections
Transcranial
Transpharyngeal
Transorbital.
POSTEROANTERIOR PROJECTION
Figure 37
Occipitofrontal Projection
Occipitofrontal projections can be taken with different
degrees of beam angulation. The choice of the angulation
depends on the anatomy, which needs to be demonstrated.
10
CR
Indications
Figure 38
CR
15
Film placement
Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device.
Patient positioning
Caldwell method.
Skull position for 15 PA Caldwell view showing the central ray
directed at a caudal angle of 15 to the canthomeatal line
PA Mandible
Indication
The indication of this technique is to demonstrate the
transverse or oblique fracture of the body and rami of the
mandible, not evident in other projections.
Cassette and patient positioning is the same as that for
PA projection except that the central ray is directed perpendicular to the cassette and centered in the midline through
the cervical spine at the level of the angles of the mandible.
Limitation of this technique is that the central body of
the rami is not well shown because of the superimposed
spine.
PA Cephalometric
Cephalometric radiography ensures standardization and
reproducibility of the images.
Indications
Film positioning
Patient positioning
Patient positioning
Central ray
30 OCCIPITOMENTAL
Indications
Rotated PA View
Indications
Technique
Indications
Central ray
PARIETOACANTHIAL VIEW
(Open Mouth Waters View)
Indications
This technique is useful for the evaluation of maxillary
sinuses and it also demonstrates frontal sinuses, ethmoidal
sinuses, orbit, zygomaticofrontal suture and nasal cavity.
In Waters technique the neck is hyperextended enough
to place the dense petrosae immediately below the maxillary sinus oor.
Film placement
Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device.
Patient positioning
The patients neck is hyperextended and centered over the
cassette perpendicular to the acanthion.
The patients chin is placed resting over the cassette
and adjusted so that MSP is perpendicular to the plane of
the cassette.
The head is adjusted so that the OML forms an angle of
37 from the plane of cassette (Figure 39).
Central ray
Perpendicular to the cassette exiting at the acanthion.
Figure 39
MODIFIED PARIETOACANTHIAL
PROJECTION (Modified Waters View)
Indication
The modified Waters method is a good projection to demonstrate blow-out fractures.
Although the parietoacanthial (Waters) projection is
widely used, this technique is modied by making the patient
to extend his/her neck to a lesser degree. This method is
referred to as shallow Waters .
In this method, OML is adjusted to form an approximately
55 angle with the plane of the cassette (Figure 40A, B).
The resulting radiograph demonstrates the facial bone
with less axial angulation than with the Waters method.
ACANTHOPARIETAL PROJECTION
(Reverse Waters Method)
Indication
Reverse Waters method is used to demonstrate the facial bones
when the patient cannot be placed in a prone position.
37
Patient positioning
CR
Figure 40
A
55
CR
CR
35
Illustrations showing the skull position and entry of central ray for the modified Waters view
Patient positioning
Figure 41
37
Structures seen
Reverse Waters demonstrates superior facial bone. The
image is similar to that obtained with Waters method, but
facial structures are considerably magnified.
SUBMENTOVERTEX VIEW
(Base or Full Axial Projection, Schuller Method)
Indications
748
LATERAL VIEW
Film placement
Indications
Figure 42
Figure 43
CR
CR
Film placement
Film placement
Patient positioning
Patient positioning
Central ray
It is directed horizontally to a point 2.5 cm inferior to the
outer canthus of the eye (Figure 43).
Central ray
Horizontal beam is centered on the EAM.
To visualize the relationship of the soft and hard tissues of the face.
To assess the position of the teeth in relation to each
other and to the hard tissues and adjacent structures.
Indications
Film placement
Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device.
749
Figure 44
CR
30
CR
37
Central ray
The central ray is aimed upward from below the occiput
so that the central ray forms an angle of 30 to the horizontal, and is centered through the condyles.
Patient positioning
Central ray
Directed through the foramen magnum at a caudal angle of
30 to the OML or 37 to IOML. Central ray enters approximately 2 inches above the glabella and through the
level of EAM.
Indications
Film placement
Cassette with the film is placed perpendicular to the floor,
with long axis vertically in a cassette holding device.
Patient positioning
Central ray
Indications
Patient positioning
Central ray
Position of patient and cassette
Central ray
Bimolar Projection
This technique is used in orthodontic practice. It shows
both left and right oblique lateral views on one film. The
technique incorporates a hinged lead shield to prevent
exposure of the other side of the film.
TRANSPHARYNGEAL VIEW
(Parma Projection, Macqueen-Dell Technique)
This technique provides a sagittal view of the medial pole
of the condyle.
Indications
Film placement
A 5 7-inch cassette is used. Film markers should be
used to indicate the side.
The cassette with the film is placed on the side of
interest, with the TMJ being centered over the cassette.
The cassette in this position is stabilized by the patient.
Patient positioning
TMJ RADIOGRAPHY
TRANSCRANIAL VIEW
Central ray
TRANSORBITAL VIEW
It provides an anterior view of the TMJ, perpendicular to
the transcranial and transpharyngeal projections.
Indications
Film placement
Patient positioning
The patients head is tilted downward 10, so that the canthomeatal line is horizontal.
Central ray
Central ray is oriented downward approximately by 10
and laterally approximately 30 through the ipsilateral
orbit, centered over the TMJ of interest.
If the condylar motion is limited, then only the neck is
visible due to superimposition of the temporal component
on the condylar head.
This view is rarely being used now, due to unwanted
radiation exposure to the eyes.
In addition to the above-mentioned techniques for
viewing the TMJ, reverse Townes view and submentovertex projections can be used to assess the TMJ.
752
Central ray
The central ray is directed through the foramen magnum
at a caudal angle of 30 to OML. It enters approximately
2 inches (6.3 cm) above the glabella and passes through
the level of EAM (Figure 45C).
Structures shown are:
May Method
When a patient with suspected fracture of zygomatic
arch comes to the department, radiographs that are usually advised for these patients are submentovertex view,
Schullers method and sometimes Waters view as well.
But there are always chances of superimpositions of other
structures on the zygomatic arch when these methods are
used. Thus, when a radiographic view of zygomatic arch is
required, the alternative method would be May method,
also known as tangential projection.
This method demonstrates:
Zygomatic arch
Temporal process of zygomatic arch
Temporal bone
Useful in patients who have depressed fractures or flat
cheek bones.
Positioning of patient
The patient is asked to stand in an upright position. Arms
are placed in comfortable position, shoulders are made to lie
in the same horizontal plane. The midsagittal plane of the
body is centered to center of the image receptor (Figure 46A).
Figure 45
A
30
CR
(A) Position of patient in Townes method. (B) Upright radiograph. (C) Upright radiograph with central ray
directed 30 degrees to OML
Figure 46
B
15
CR
(A) Position of the patient in May method. (B) Upright radiograph. (C) Central ray in May method
Mandibular condyle
Mastoid cells
Lateral portion of the petrous pyramid
Superimposed internal and external acoustic meatuses
Mastoid emissary veins.
753
Figure 47
B
15
CR
(A) Position of the patient and direction of central ray in axiolateral oblique projection/
original law method. (B) Upright radiograph
Central ray
Mandibular condyle
Lateral portion of the petrous pyramid
Superimposed internal acoustic meatus
Mastoid emissary veins when present
Mastoid cells.
754
Central ray
The central ray is directed perpendicular to exit the acanthion
(Figure 48C).
The structures shown are:
Petrous ridges
Zygomatic bone
Inferior orbital margin
Nasal septum
Mandible.
Figure 48
B
55
CR
35
(A) Position of patient in parietoacanthial projection. (B) Upright radiograph. (C) Central ray
Figure 49
B
CR
37
(A) Patient positioning in acanthioparietal positioning. (B) Upright radiograph. (C) Central ray
Orbit
Zygomatic bone
Maxillary sinus
Petrous ridge.
Figure 50
PANORAMIC RADIOLOGY
Panoramic radiology is considered a curvilinear variant of
conventional tomography, in which a single tomographic
image of facial structures includes both maxillary and
mandibular dental arches and their supporting structures.
Panoramic radiography is derived from the word panorama which refers to an unobstructed or complete view
of a region in every direction. The term orthopantomography was coined by Paatero and Sairenji which is derived
from orthostraight or correct, pan (abbreviation for panorama), tomotomography is the imaging of depth of tissue layers without the interference of tissue above and
below that plane of tissue.
756
2.
3.
4.
5.
Figure 51
Figure 53
Figure 54
Figure 52
6.
7.
8.
Working principle
In rotational panoramic radiography, the patient is stationary and the X-ray source (tube head) and the cassette
carriage assembly (connected to each other via a C-arm),
rotate around the patients head (Figure 56).
As the C-arm rotates, the X-ray tube head and the cassette carriage assembly (Figure 57) rotate in the same direction, whereas the lm along with the cassette moves in a
direction opposite to movement of the tube head. The rate
of the lm movement is adjusted such that it matches with
the speed of the slit shaped beam imaging the patient.
A narrow beam of X-rays (facilitated by a slit collimator
within the X-ray tube head) sweeps across in a horizontal
757
Figure 55
Figure 57
Cassette carriage
assembly
Figure 58
Figure 56
Center of
rotation
C-arm
Cassette
carriage
assembly
X-ray
source
X-ray tube
head
Film within
film-cassette
carriage
758
Figure 59
Image characteristics
A smile-line is created
Patient is positioned with the
chin lifted up
2.
3.
4.
5.
759
6.
2.
3.
4.
5.
SPECIALIZED IMAGING
COMPUTED TOMOGRAPHY (CT)
It is also referred to as computed axial tomography, computed tomographic scanning, axial tomography and computerized transaxial tomography.
Godfrey Hounseld and Allan Cormack were instrumental in the development of CT and were awarded the
Nobel Prize in Medicine in 1979 for their efforts. This
radiographic technique blends the principles of thin layer
radiography (tomography) with the use of a computer to
synthesize the image (computed).
760
Table 7
Tissue
CT number
Air
1000
Lung
200
Fat
100
Water
CSF
15
Working Principle
Blood
20
Gray matter
40
White matter
45
Muscle
50
Medullary bone
300
Cortical bone
1000
Though CT scanners are available in different system configurations, they all have the same basic components:
1.
2.
3.
4.
Gantry
Patient support couch
Computer
Control console.
Gantry
The gantry is made up of the detector array, patient support couch, and the X-ray tube or source. The gantry can
be tilted up to 300. The facility to tilt helps in excluding
structures from the scan that may degrade the final image
(e.g., metallic dental restorations).
Detector array The detector is made up of multiple discrete cells or detectors. Each of these detectors acquires
specific information for each slice of the scan and transmits it to the computer. The present fourth generation CT
scanners have up to 2,400 detectors.
The detectors contain either a crystal scintillation
detector or a gas-lled detector. Commonly used scintillation detectors are manufactured with cesium iodide (CsI),
bismuth germanate or cadmium tungstate (CdWO4). These
solid-state detectors are coupled optically to a photodiode.
Solid-state detectors exhibit detection efciency that
approaches 100% but cannot be packed closely together.
Gas-lled detectors contain either xenon or a xenon
krypton mixture. These gases have a high atomic number,
are inert, and display minimal afterglow. The gas is contained under high pressure in the detector array. The individual gas-lled detectors can be placed closely together;
the gas, however, in the detector only produces approximately 50% detector efciency. Regardless of the material
used to capture information, the spacing of each discrete
detector coupled with their detection efciency determines
the efciency of the scanner and the ultimate spatial
resolution.
X-ray source The X-ray source for the currently available scanners consists of an X-ray generator and an X-ray
tube. The X-ray generator is designed to produce a high
milliampere (up to 400 mA) beam at a nearly continuous
rate. The large amount of heat generated through continuous
beam production necessitates the use of a large rotating
anode and fairly large focal spot.
The X-ray beam is collimated before it traverses through
the patient (pre-patient collimation) and at the detector
array (post-patient collimation). The pre-patient collimation
decreases the radiation dose to the patient. The post-patient
collimation reduces the amount of scattered radiation that
Computer
The rapidity of capturing the image, acquiring data, and
larger matrix size (512 512) necessitates the use of highspeed computers.
Modern CT scans require computers that can solve up
to 30,000 equations simultaneously. The time it takes the
computer to generate a visible image after data acquisition
is termed reconstruction time. Reconstruction time for a
single slice is usually about 1 second. These computers can
constitute up to one-third the cost of a CT scanner.
Control console
The control console allows the operator to select the parameters of the CT scan, view the image as they are being
generated. Many consoles have two monitors so that the
technician and the radiologist can manipulate the image
as the data is acquired. Image data is stored into the computer so that it can be formatted later into a number of
ways. Data is stored either on magnetic tapes or disks. Most
CT images are viewed on a film. The electronic data from
each view is transferred onto a film using laser cameras.
The most common film format is 14 17 inches and may
contain 415 images.
Advantages of CT
Disadvantages of CT
Expensive.
Patients exposure to radiationCT is considered a high
radiation dose technique (depends upon region imaged,
number of slices, thickness of slice and kVp). For a
head scan the effective dose has been calculated as
24 mSv and 515 mSv for a body scan.
A mid skull dose for a PA view (PA skull) or Townes
view is about 35 mGy, whereas a mid skull dose of
about 3455 mGy is seen with a CT.
Production of artifacts, especially when metallic restorations are located in the plane of tissue being scanned.
These streak type artifacts may obscure radiographic
findings in the CT scan and render it useless for
diagnosis.
Uses of CT in Dentistry
Evaluation of the presence and extent of clinically suspected pathology in the head and neck region including cysts, tumors and infections.
Detection of the extension of disease process into the
paranasal sinuses, base of skull and orbit.
Determination of the location, extent and displacement
of maxillofacial skeletal fractures, including detection
of subdural and epidural hematomas.
Salivary gland imaging.
Evaluation of potential implant sites using 3D image
reconstruction.
Evaluation of the components of the TMJ.
CT-guided fine needle aspiration biopsies.
Virtual surgeries.
DENTOMAXILLOFACIAL CONE-BEAM
COMPUTED TOMOGRAPHY (CBCT)
Radiology has always been a tremendous asset in clinical
dentistry. Intraoral imaging, whether digital or film, continues to provide the best spatial resolution of any imaging
method currently available. However, because spatial information is lost when it is collapsed into a two-dimensional
(2D) image, in many cases, two or three intraoral radiographs
taken from different angles are recommended. The clinical
diagnostic capacity of intraoral radiography is influenced
by a number of variables, including beam angulation,
exposure time, receptor sensitivity, processing, viewing conditions, superimposition of anatomic structures and lesion
762
Historical Background
Computed tomography (tomography is derived from the
Greek words tomos, meaning slice or section, and graphia,
meaning describing) was introduced in 1972 by the engineer Godfrey Hounsfield and the physicist Allan Cormack.
In 1979, Cormack and Hounsfield, who had worked independently to develop CT, were jointly awarded the Nobel
Prize in Physiology or Medicine for their work on the
tomographic principles used in computing the spatial distribution of a physical quantity of an object examined
from different directions and transferring this data into
easily readable images. CT represented the first practical
medical application of the tomographic principle in the
field of medicine, providing high-contrast images without
superimposition of adjacent anatomic structures. The first
clinical CT scanners, installed between 1974 and 1976,
were dedicated to head-imaging only, and whole body
systems became available in 1976. Several hours were
needed just to acquire the raw data necessary to produce a
single image slice, and several days were required to
reconstruct an image from this raw data. Great improvements have since been achieved in speed, patient comfort
and resolution. Today, more anatomy can be scanned in
less time, which helps to eliminate artifacts from patient
motion and provides excellent diagnostic image quality at
the lowest possible effective doses.
Figure 60
A
X-ray source
X-ray source
Translation
Translation
Rotation
Rotation
Object
Object
Detector
Detector
X-ray source
X-ray source
E
Object
Detector
(A) First generation. (B) Second generation. (C) Third generation. (D) Fourth generation. (E) Helical CT.
Evolution of CT scanners from the original design
763
Fundamentals of CBCT
Whereas conventional CT scanners emit a fan-shaped X-ray
beam and require a stack of multiple slices to obtain a complete image, CBCT systems operate by focusing a coneshaped beam on a 2D detector that performs one pass or
less around the patients head to produce a series of 2D
images. A cone-beam algorithm is then applied to this image
dataset, allowing the operator to extract planar and curved
reconstructions of varying thicknesses in any orientation
and to generate accurate 3D images of bone and soft tissue
764
Table 8
Specications of some contemporary CBCT systems, including product, manufacturer and software name; detector type; patient positioning options; FOV and voxel
size options; effective doses according to FOV (for adults); and additional features
Company
Model
CBCT image
detector
Patient
positioning
Field of view
diameter/height
Voxel size
options
Estimated effective
doses for adult (ICRP
2007)
Software
Extra features
Quantitative
Radiology,
Verona, Italy
NewTomVGi
CBCT
Amorphous
silicon flat
panel
Standing
Seated
Wheelchair
From 0.075 to
0.30 mm
NNT viewer
Safe beam*
Pulse system**
FS: 0.3 mm
Quantitative
Radiology,
Verona, Italy
NewTom 5G
CBCT
Amorphous
silicon flat
panel, 20
25 cm
Supine
Ultra high:
0.075 mm;
High: 0.15 mm;
Low: 0.30 mm
NNT viewer
Safe beam*
Pulse system**
FS: 0.3 mm in standard
mode
FS: 0.15 mm in zoom
mode
Ergonomic, two stage
patient chair
J. Morita,
Kyoto, Japan
Veraviewepocs
3D and
Veraviewepocs
3De
CMOS flat
panel detector
Standing
Veraviewepocs 3D
4 4 cm;
4 8 cm;
8 8 cm
Veraviewepocs 3De
4 4 cm;
4 8 cm
0.125 mm
73 Sv (with 8 8 cm
FOV)
(Pauwels et al, 2012)
i-Dixel 2.0/One
data viewer/
One volume
viewer
CBCT Pano/
Ceph options
(Hybrid model)
3D Accuitomo
170
CBCT
CMOS flat
panel detector
Seated
From 0.08 to
0.250 mm
i-Dixel 2.0/One
data viewer/
One volume
viewer
Imaging
Sciences,
Hatfield,
PA, USA
I-CAT Classic
CBCT
Amorphous
silicon flat
panel, 20
25 cm
Seated
16 1322 cm
Xoran Cat
iCATvision
3DVR
Quick launch
InVivoDental and
Dolphin software
Imaging
Sciences,
Hatfield,
PA, USA
I-CAT Next
Generation
CBCT
Amorphous
silicon flat
panel, 20
25 cm
Seated
Standard: 16
6, 8, 10, 13 cm
Extended:
17 23 cm
From 0.125 to
0.4 mm
iCATvision
InVivoDental
3DVR
Vatech,
E-WOO
Technology,
South Korea
PaX-Duo 3D
Pano CBCT
Amorphous
flat panel
Standing
Wheelchair
accessible
From 5 5 to
15 13.5 cm
0.080.3 mm
FOV (5 5 cm)
42.52 Sv
FOV (12 8.5 cm)
120.69 Sv
Provided from company.
Unpublished
EasyDent/Ez3D
Auto-switching system
between sensors
Pulsed scan**
(Contd...)
J. Morita,
Kyoto, Japan
765
766
Table 8
Continued
Model
CBCT image
detector
Patient
positioning
Field of view
diameter/height
Voxel size
options
Estimated effective
doses for adult (ICRP
2007)
Software
Extra features
Vatech,
E-WOO
Technology,
South Korea
PaX-Reve 3D
CBCT/Pano/
Ceph
Amorphous
flat panel
Standing
Wheelchair
accessible
From 5 5 cm to
15 19 cm
From 0.08 to
0.25 mm
EasyDent/Ez3D
Kodak Dental
Systems,
Carestream,
Rochester,
NY, USA
Kodak 9000
3D & 9000c 3D
CBCT/Pano/
Ceph
CMOS sensor
with optical
fiber
Seated
Standing
Wheelchair
5 3.8 cm single
9 7 3.75 cm
stitched
0.0760.2 mm
5 3.8 cm
1940 Sv
(Pauwels et al, 2012)
Kodak Imaging
Software
Kodak Dental
Systems,
Carestream,
Rochester,
NY, USA
Kodak 9500
FOV CBCT
Amorphous
silicon flat
panel
Seated
Standing
Wheelchair
Medium:
15 9 cm
Large:
18.4 20.6 cm
0.20.3 mm
Kodak Imaging
Software
Planmeca
Oy, Helsinki,
Finland
Promax 3D
CBCT/Pano/
Ceph/
Flat panel
sensor
Seated
Standing
Wheelchair
8 8 cm, 8 5 cm,
4 8 cm, 4 5 cm
30306 Sv
(Qu et al, 2010)
Romexis
Planmeca
Oy, Helsinki,
Finland
Promax 3D
Max CBCT
Flat panel
sensor
Seated
Standing
Wheelchair
5.5 5 cm to
23 16 cm;
full skull: 26 23 cm
Romexis
Artifact removal
Full skull scan
Soredex,
Tuusulu,
Finland
Scanora 3D
CBCT Pano
CMOS flat
panel
Seated
From 6 6 cm to
14.5 13 cm
0.130.35 mm
On Demand
Soredex,
Tuusulu,
Finland
Scanora 3Dx
CBCT Pano
CMOS flat
panel
Seated
From 5 5 cm to
24 17 cm
0.10.5 mm
Not available
On Demand
MyRay,
Cefla Dental
Group, Imola,
Italy
Skyview CBCT
Image
Intensifier
CCD sensor
Supine
FOV diameter:
11, 15, 17 cm
Myray skyVIEW
Pulsed emission**
Sirona Dental
Systems,
Bensheim,
Germany
Galileos
Comfort CBCT
Cephalometric
Proprietary
Siemens
Technology
Standing
Seated
15 15 15 cm
0.150.3 mm
84 Sv
(Pauwels et al, 2012)
Galaxis, Sidexis,
Galelios Implant
*Safe Beam technology reduces radiation level according to patient size. **Pulse system that activates the X-ray source only when needed. Generally CBCT systems have stationary focal spot (FS) size of
0.50.8 mm. NewTom 5G allows the patient to be seated initially and then moved comfortably into a supine position. #Separate scans can be combined to obtain the whole arc.
Data is collected from different company sites and articles. Most features are optional and effective dose measurements vary between devices according to settings used.
Company
Figure 61
A
(A) Large FOV CBCT unit (iCAT Next Generation, Imaging Sciences, Hatfield, PA, USA) with a seated patient.
(B) Small FOV CBCT unit (Kodak 9000, Carestream, Rochester, NY, USA) with a standing patient
Figure 62
Cone-beam
Fan-beam
X-ray source
X-ray source
Detector
Secondary
reconstructions
Detector
Basis projections
Primary
reconstruction
Schematic diagram of cone-beam and fan-beam imaging geometries and secondary reconstruction processes
is much lower than CT and which reduces not only generator power, but also heat production. Finally, most CBCT
systems generate a pulsed X-ray beam that coincides with
detector activation, which markedly reduces total scan time
as well as heat production, which translates into lower
radiation doses to patients.
Detectors
All CBCT systems utilize an area detector to capture and
record images. Initially, CBCT detectors were configured
from an image intensifier combined with a charge-coupleddevice (II/CCD) detector, making them large and bulky,
and they tended to produce circular basis image areas
(spherical volumes) rather than rectangular ones (cylindrical
volumes). Nowadays, most CBCT units use flat panel detectors (FPD) comprising a large-area pixel array of hydrogenated amorphous silicon thin-film transistors or in some
more recent cases, large, complementary metal oxide semiconductor technology (CMOS) arrays. X-rays are detected
indirectly by a scintillator such as thallium-doped cesium
iodide or terbium-activated gadolinium oxysulfide, which
767
Figure 63
Sagittal
Axial
Coronal
Axial
Coronal
Sagittal
Schematic diagram showing axial, coronal and sagittal planes and corresponding CBCT images
Figure 64
Anisotropic
x=yz
Isotropic
x=y=z
768
Figure 65
B
4 4 cm
6 6 cm
10 10 cm
8 8 cm
14 10 cm
17 12 cm
CBCT images of a dry skull obtained using different FOVs of Accuitomo 170 (J Morita, Kyoto, Japan).
(A) 4 4 cm; (B) 6 6 cm; (C) 8 8 cm; (D) 10 10 cm; (E) 14 10 cm; (F) 17 12 cm
769
Table 9
Advantages
Lower radiation doses than conventional CT and beam collimation
Liability
In clinical practice, CBCT possesses a number of advantages over medical CT, such as lower effective radiation
doses, lower costs, fewer space requirements, easier image
acquisition, higher image accuracy and interactive display
modes such as mutiplanar reconstruction that are applicable to maxillofacial imaging. However, the disadvantages
of CBCT include higher doses than 2D imaging; the inability to accurately represent the internal structure of soft
tissues and soft tissue lesions; a limited correlation with
HUs for standardized quantification of bone density; and
the presence of various types of image artifacts, mainly those
produced by metal restorations, that can interfere with the
diagnostic process by masking underlying structures. In
addition, liability issues related to CBCT remain unresolved.
Table 9 shows advantages and disadvantages/limitations
of CBCT in routine clinical practice.
Advantages
Lower radiation doses than conventional CT CBCT
scanners provide adequate image quality for dentomaxillofacial examinations while delivering considerably smaller
effective doses than medical CT. Although not available
on all CBCT systems, beam collimation, which limits radiation to the area of interest, is a highly desirable function
that reduces doses by adjusting the irradiated field according to the FOV. In general, doses range from 13 to 82 Sv for
small and medium FOV CBCT, compared to 4741160 Sv
for medical CT. To ensure that optimal doses are achieved,
diagnostic and image quality requirements should be evaluated on a case-by-case basis and appropriate exposure
parameters and FOVs selected accordingly.
Lower time, space and cost requirements than conventional CT Dental CBCTs are compact (4 m2), easy to
operate, reasonably affordable and require relatively low
maintenance, making them a suitable choice not only for
hospitals and medical centers, but also for many dentists
in private practice. Scan time is comparable to that of
panoramic radiography, varying anywhere from approximately 120 minutes, depending upon FOV, number of
770
Limitations/disadvantages
Figure 66
A
CBCT image taken with (iCAT Next Generation, Imaging Sciences, Hatfield, PA, USA): (A) Axial view; (B) Simulated panoramic;
(C) Volumetric 3D representation of hard tissue; (D) Serial cross-sectional images; (E) Right generated cephalometric (MIP);
(F) Left generated cephalometric (MIP)
Figure 67
A
Comparison of CBCT ray-sum generated simulated lateral cephalometric image (A) and lateral view of volumetric rendering with
superimposition of airway space of the same patient (B). Images created using Invivo software (Anatomage, San Jose, CA)
Figure 68
Implant placement procedure by combining optical digital impression CEREC and Galileos CBCT images
(Sirona Dental Systems, Bernsheim, Germany)
manufacturing). Eventually, greater simplification and automation can be expected to lead to more precise dental
restorations that can be custom-fit to an individual patient.
Figure 68 shows an implant placement procedure by combining optical digital impression CEREC and Galileos CBCT
images (Sirona Dental Systems, Bernsheim, Germany).
Several CBCT units are also now capable of integrated facial
scanning, whereby CBCT acquisition is accompanied by a
concomitant 3D laser scan of facial soft tissue that is then
overlaid on the bony skull image; the availability of fully
integrated, accurate 3D information of both face and bone
772
Figure 69
A
(A) Axial CBCT and (B) MDCT images. Arrows show definitely higher soft tissue contrast in
MDCT image compared to CBCT
Figure 70
A
(A and B) Axial and (C) coronal CBCT images. Streak artifacts appear as linear hyperdensities that radiate from a metallic
object are shown with black arrows. Beam-hardening artifacts, which appear as dark bands adjacent to high-density
structures are shown with blue arrows
774
Oral implantology
The International Congress of Oral Implantologists,
Consensus Report cites four different areas in which CBCT
can be of use in oral implantology:
1. Diagnostics CBCT can be used in the identification
and evaluation of pathology, foreign bodies and defects.
2. Implant planning CBCT images not only have been
proven successful when used for linear measurement, but
also have been shown to provide reliable 3D information
for the assessment of relative bone quality and quantity, 3D
evaluation of ridge topography and pre-implantation
identification of vital anatomical structures such as the inferior alveolar nerve, mental foramen, incisive canal, maxillary sinus, ostium and nasal cavity floor. This information
can be used in the treatment planning process to identify
suitable implant sites and to determine whether or not
there is a need for surgical procedures, such as sinus lifting
and bone augmentation. CBCT is also recommended in
sinus grafting operations as a mean of better predicting
complications, thereby achieving better surgical outcomes.
Figure 71 shows curved and cross-sectional views used to
assess implant sites and nerve canal in the mandible. CBCT
bone images can also be fused with soft tissue images
acquired with digital impression techniques to enhance
planning efficiency (Figure 68).
3. Surgical guidance CBCT images have yielded promising results when used for surgical guidance. Commercially
Figure 71
Curved and cross-sectional views used to assess implant sites and nerve canal in the mandible
775
Figure 72
Stereolithographic guidance systems constructed for implant placement using implant software StentCad
(Ay Tasarim Ltd., Ankara, Turkey)
1. Eruption problems CBCT images may help in identifying and localizing impacted or displaced permanent,
supernumerary or supplementary teeth, such as third
molars and canine supernumeraries. In particular, CBCT
images can be used in the pre-surgical assessment of the
relationship between third molar apices and the mandibular canal. Figure 74 shows CBCT images taken from a
patient prior to surgical extraction of impacted third molar
tooth.
2. Oral and maxillofacial pathologies CBCT enables the
surgeon to visualize oral and maxillofacial pathologic
entities such as benign jaw bone tumors and cysts in three
dimensions, thereby assisting in diagnosis as well as in
planning appropriate treatment. Figure 75 shows CBCT
images of well-defined cystic pathologic lesions.
3. TMJ-related problems CBCT provides information
essential to the diagnosis of a variety of TMJ disorders,
including osteoarthritis, inflammatory arthritis, trauma
and developmental disorders. The ability to provide highresolution multiplanar images with a lower dose than CT
is rapidly making CBCT the imaging modality of choice
Figure 73
A
Cross-sectional CBCT images of patients with pain and discomfort in the implant region that reveal
implants mistakenly placed in the maxillary sinus (A), mandibular canal (B) and out of cortical plate (C).
Please see arrows (Courtesy: Dr Ilker Cebeci, Tomoloji Dentomaxillofacial Radiology Center, Ankara, Turkey)
Figure 74
A
CBCT images taken from a patient before surgical extraction of impacted right third molar tooth. Conventional panoramic
radiograph showing a possible relation between right mandibular molar tooth and mandibular canal (A). CBCT generated
panoramic (B) and cross-sectional views show a relationship with third molar apex and mandibular canal (C)
Figure 75
Patient 1
Patient 2
CBCT images of well-defined cystic pathologic lesions of two different patients
778
4. Craniofacial fractures CBCT has been used to document craniofacial fractures, including mandibular head
Figure 76
CBCT images of a patient with bone changes in TMJ. Images were reformatted in TMJ mode
Endodontics
The use of CBCT in endodontics is increasing rapidly. Units
with small FOVs offering high-resolution images of teeth
and related structures have been specifically recommended
in cases where 2D systems have failed to provide sufficient
information regarding the following:
1. Root-canal morphology CBCT images can be used to
precisely determine root curvature and to definitively
identify accessory canals and other anomalies when conventional imaging has suggested the presence of complex
tooth morphology.
2. Periapical pathosis CBCT can assist in the diagnosis of
dental periapical pathosis in patients who present contradictory or non-specific clinical signs and symptoms; who have
poorly localized symptoms associated with an untreated or
previously endodontically treated tooth, with no evidence of
pathosis identified by conventional imaging; and in cases
where anatomic superimposition of roots or areas of the
maxillofacial skeleton are required to perform task-specific
procedures. CBCT can also support a diagnosis of pathosis
that is non-endodontic in origin, determines the extent of the
lesion and identifies its effect on surrounding structures.
Figure 77
A
(A) CBCT axial image showing a root fracture line and (B) CBCT sagittal image showing a periapical lesion with
bony reaction and maxillary sinusitis. Please see arrows
Figure 78
unable to provide sufficient reliable information for periodontal assessment and treatment. CBCT may play a role
in the assessment of marginal bone contours and 3D defects,
especially furcations and infrabony defects, in which defect
780
Orthodontics
The use of CBCT in orthodontics has been gaining substantial popularity, although it is primarily recommended in
cases where conventional radiography cannot supply satisfactory diagnostic information. This can include assessments
of cleft-palate patients, unerupted and supernumerary tooth
localization, identification of root resorption caused by
unerupted teeth, evaluation of boundary conditions and
orthognathic surgery planning. CBCT imaging may also be
performed in other cases where it is likely to provide valuable diagnostic information. Figure 78 shows CBCT images
of a cleft palate patient with eruption problems.
Head and neck
Depending on the FOV used, CBCT images may show part
or all of the nasal cavity, paranasal sinuses, airway, cervical
vertebrae and temporal bone. In fact, specific ear, nose and
throat imaging programs have been increasingly included
in CBCT systems, suggesting that CBCT may at some point
entirely replace medical CT imaging in certain otolaryngology-related applications. For example, an innovative
C-arm CBCT system has been used in image-guided surgery of the frontal recess; the technology is portable and
provides near-real-time imaging to confirm and guide surgical treatment, thereby reducing the risk of disorientation
and iatrogenic injury. CBCT has also been found to provide reliable and accurate 3D analysis of the upper airway
that can be of help in assessing the presence and severity
of obstructive sleep apnea (Figure 67). Imaging of the temporal bone represents another promising area for CBCT,
whose high-resolution and nearly artifact-free multi-planar
reconstruction images make it possible to precisely assess
the intra-cochlear position of the electrode, including
visualization of each individual contact. It is this capacity
for precision that makes CBCT a perfect candidate for the
post-operative assessment and follow-up of cochlear implantation electrodes.
Historical Perspective
The phenomenon of nuclear induction later to be termed
as nuclear magnetic resonance (NMR) was described independently but almost simultaneously by Bloch and Purcell
and their colleagues in 1946 and for this they were jointly
awarded Nobel Prize in 1952. Much later, the term nuclear
was dropped, it is now commonly referred to as magnetic
resonance (MR).
In 1971, Damadian noted that in vitro animal tumors had
elevated MR relaxation times when compared to normal
control tissue and he formed an apparatus and method for
detecting cancer in tissue.
In 1973, Lauterbur published a paper and showed how
MR could be applied to imaging by applying a linearly
varying magnetic eld across a liquid. Paul C Lauterbur is
known as the Father of MRI.
In 1977, human Invivo images were demonstrated by
Manseld and Maudsley.
In 1980, multiplanar imaging ability were rst demonstrated by Hawkes.
In 1992, functional MRI (fMRI) of the brain was introduced by Ogawa.
Figure 79
Gradient coils
Main magnet
RF coil
Couch
z
x
RF receiver/
transmitter
switch
Main magnet
power supply
X-gradient
power supply
RF pulse
generator
Y-gradient
power supply
Z-gradient
power supply
RF pulse
amplifier
Image
processor
Central pulse
sequence controller
MR scanner
Figure 80
B0
Axis of precession
Axis of spin
proton
Proton magnetization
Proton exposed to a steady external magnetic field creates
a force that will act on its magnetic dipole moment and
orient it parallel with the external field, but due to the
spin, it does not swing in as a compass needle would do.
Instead, it performs a maintained circular movement called
precession which has its own axis of spin and rotates at an
angle around another axis that is parallel with the external field like toy spinning top in the gravitational field
(Figure 80). Magnetic dipole moment of the precessing proton have magnitude and a direction expressed by a vector
which is resolved in one component aligned with the axis
of precession known as the longitudinal component.
The second component which is oriented perpendicular
to the external eld and rotating with the frequency of
precession is called the transverse component. Within a
magnetic eld, all protons precess at the same frequency
782
w
External field
Figure 81
Figure 82
Low energy state
z
Longitudinal net
magnetization vector
B0
External field
xy transversal vectors
cancel because out of
phase
ii.
Table 10
Table 12
T1
T2
T1 longer than T2
Table 11
Pure liquids
Solids
Mobile molecules Molecules fixed
Intrinsic and local Local intrinsic field
field variations are
inhomogeneities
rapidly fluctuating
more permanent
and tend to
causing protons to
average out
systematically
dephase
Longer (seconds) Short (milliseconds)
Fat
Bone marrow
T1 time (ms)
T2 time (ms)
240250
6080
550
50
780
90
920
100
Muscle
CSF (water)
Table 13
T1 weighted image
T2 weighted image
Emphasizes T1 value of
tissues
To demonstrate anatomy
To identify inflammatory/pathological
changes
Other techniques allow the signal
from fat or water to be enhanced/
supressed
Fat saturationnulls the signal
from fat
Tissue type
860900
50
2,2002,400
5001,400
Figure 83
Figure 84
z
B0
z
90 RF pulse
y
MZ
Mxymax
w
Figure 85
z
180 RF pulse
Mxymax
y
Mz
x
x
Mz
Illustrations showing fanning out of the transverse component after the end of a 90 pulse and the application of
a 180 pulse reversing the longitudinal vector
Figure 86
z
y
Mxy
Mz
x
MR-contrast Agents
The relaxation times (expressed by T1 and T2) is shortened
when paramagnetic substance is targeted to tissue, disturbing admixture of strong magnetic dipoles due to the unpaired
electrons in their atoms.
Gadolinium (Gd) chelated to DTPAit is a rare earth
element which does not cross the normal bloodbrain barrier. This is used to detect defects in this barrier excreted in
the urine. It is utilized in urological MR-imaging. Using
additional ligands, Gd-chelates may be directed to biliary
excretion. Agents based on manganese (Mn) having paramagnetic properties similar to gadolinium have been
developed.
Gd and Mn based compounds are positive contrast agents
because of their inuence on the relaxation constants, notably T1, is utilized to produce images (T1 weighted) where
the MR signal strength is proportional to the concentration
of the agent.
Iron oxide particles effectively produce local eld in
homogeneities. These are negative contrast agents which
produce signal voids by strongly shortening T2. After IV
administration, it shortens the relaxation time in liver, spleen
and bone marrow, but not in tumors lodged in these tissues. This is used for gastrointestinal imaging.
Figure 87
y
z
Table 14
2.
CT
MRI
Ionizing radiation
Radiofrequency
Less expensive
Expensive
Short time
Long time
Can be used
5.
6.
3.
4.
7.
Advantages
Best resolution of tissues with low inherent contrast.
Uses non-ionizing radiationnon-hazardous.
Accurate and rapid localization of intracranial pathologies.
No streaking artifacts.
High-contrast images achievable.
Disadvantages
Image interpretation
Tissue
T1 image
T2 image
Water
Dark
Very bright
Tumors
Intermediate
Bright
Old blood
Bright
Bright
Fibrous tissue
Dark
Dark
Air
Dark
Dark
Bone calcium
Dark
Dark
Fresh blood
Dark
Dark
Organs
Intermediate
Dark
Fat
Very bright
Bright
1.
2.
3.
4.
5.
CI: Contraindicated
Table 15
Congenital disorders: T1 weighted sequences with coronal and axial images demonstrate abnormalities like
cleft lip and palate.
Infections: AIDS-generalized cervical lymphadenopathy with cystic lesions in the parotid.
Sinusitis: When complicated by serious condition like
a tumor, venous sinus thrombosis or an intracranial
extension of the infection.
Benign tumors: Hemangiomas, lymphangiomas, neurofibromas and schwannomas.
Malignant tumors: Diagnosis, staging and monitoring
of malignant tumors affecting the head and neck region.
TMJ: Articular disk perforations and disk displacements.
1.
2.
3.
4.
5.
6.
NUCLEAR MEDICINE
Nuclear medicine is a diagnostic radiation science utilizing radioactive compounds or tracers having affinities for
particular tissues or organs in the body (iodine to the thyroid gland); these are termed as target tissues.
The radioactive agents are administered to the patient
either orally, intravenous or intrathecally. These agents
concentrate in target tissues and emit radiation. These are
detected and imaged by a variety of external detectors and
imaging systems. This helps in studying the target tissue
under static and dynamic conditions. Such studies are called
scintigraphic scans, scintiscans or radionuclide scans.
When these are utilized for studying bone they referred
to as bone scans and when performed for salivary gland they
are termed as salivary gland scans. Other scans include
heart scans (to identify normal or abnormal blood ow to
the heart muscle, measure heart function or determine the
existence or extent of damage to the heart tissues after a
heart attack); thyroid iodine scans (to analyze the thyroid
function and show the structure of the gland. Larger doses
of radioactive iodine are used to destroy thyroid nodules
in the case of Graves syndrome); gallbladder or hepatobiliary scans (to evaluate both liver as well as gallbladder
function such as presence of gallstones); lung scans (to
evaluate the ow of blood and movements into and out of
the lungs, as well as the determination of the presence of
blood clots); Gallium scans (to evaluate infection and tumor),
brain scans and gastrointestinal scans.
Nuclear medicine tests are extremely sensitive to abnormalities in body organs structure and function. Tests using
nuclear medicine techniques are more sensitive and specic
for disease detection than most tests because they identify
abnormalities very early in the progression of a disease,
long before the medical problem would be apparent with
other diagnostic tests.
In 1958, Hal Angler used scintillation camera for imaging the entire system. In 1971, Subramanian and McFee used
99m-Tc (Technetium) labeled MDP (methylene diphosphonate) in bone scanning.
Radiopharmaceuticals
These are radioactive agents used in nuclear medicine procedures for imaging. They are produced using nuclear reactors and cyclotrons. Radiopharmaceuticals circulate and
concentrate to varying degrees in different organs throughout the body. The localized tracer uptake is dependent on
changes in regional blood flow and areas of altered bone
physiology and pathology which show an invariable alteration in the osteoblastic and osteoclastic activity.
The organs that receive the greatest exposure are referred
to as critical organs. For example, in bone scanning the bladder, skeleton and bone marrow receive the highest exposure.
788
Types of radiopharmaceuticals
1.
2.
Bone Scanning
Procedure
Ten to fifteen millicuries of 99m-Tc labeled compound is
injected IV. A waiting period of 23 hours permits the compound to accumulate in the skeleton and to be removed
from vascular and soft tissues via the urinary system. The
patient is then positioned under the gamma camera and
the desired bones are imaged. Whole body radiation dose
in bone scan is approximately 0.10.5 rad.
Working principle
Following administration of the radioisotope, they reach the
desired organs and emit gamma radiation, which is picked
up by the detectors placed outside the body within the
gamma cameras.
Gamma cameras are devices used to image the radioisotope distribution in the body, within the eld of view.
It consists of a sodium iodide detector of about 40 cm in
diameter. The detector assembly has a collimator which is
similar to a grid in radiography. The gamma radiation emitted by the patient passes through the holes in the collimator and reaches the detector, where they are converted into
light scintillation.
The photomultiplier tubes convert the light into electric
pulse which is then passed onto the computer. The computer constructs the distribution of the radioisotopes and
displays it on the monitor.
Interpretation of bone scans
Abnormalities in bone are manifested in a scan mostly as
areas of increased tracer concentration or as areas of very
2. Viability of bone grafts: An unsuccessful graft (nonvascularized) appears as a cold spot. On the other hand,
osteoblastic activity associated with a vascularized bone
graft is seen as a hot spot.
ULTRASONOGRAPHY
Principle
Images with ultrasound are accomplished with pulse-echo
technique with the help of transducer. Ultrasound transducers convert electrical energy into ultrasound energy
and vice versa. In ultrasonography, sound waves (pulse)
generated by the transducer emitted inside the patient will
be reflected back (echo) at organ boundaries and within
tissues depending on the composition of the matter. These
echoes then return to the transducer, where they are converted into electrical energy and then presented on the
display of sonographic instruments. The ultrasound instruments processes the echoes and present them as visible
dots. The location of each dot corresponds to the anatomic
location of the echo-generating structure. Gray-scale
image is achieved by different echo-strength and the echo
arrival time is used to determine the depth of the structure
that produced the echo. The ultrasound instruments processes the echoes and present them as visible dots, which
form the anatomic image on the display. Not all the ultrasound pulses are reflected back from any interface. Rather
789
Table 16
Acoustic
impedance
Reflection
Refraction
Attenuation
Anechoic
Hyperechoic
Hypoechoic
Isoechoic
790
2.
3.
Instrumentation
1.
2.
3.
Transmitter provides high amplitude voltage and controls the rate of pulses emitted by the transducer.
Transducers operate according to the principle of piezoelectricity. This principle states that some materials
(ceramics, quartz) produce a voltage when deformed
by an applied pressure. Piezoelectric crystal used in
ultrasonography is lead zirconate titanate. When the
transducer receives the electric impulses, the dipoles
inside the crystals are realigned to the electric field
causing the changes in the thickness of the crystal.
This leads to the formation of sound which is transmitted inside the patient. The echo reflected back to
the transducers which change in the shape of crystal
that results in the production of small oscillating voltages that can be measured or recorded.
Receiver and displayafter amplification the echo
voltages are digitalized, i.e. they pass through analogto-digital converters (ADC). Electric information from
image processor drives the display, which produces a
visual image.
Coupling agents
Even a very thin layer of air between the transducer and
the skin surface reflects all the sound, preventing any penetration into the tissue, thus an aqueous gel is applied over
the skin before application of the transducer which eliminates the air layer and facilitates sound passage in and out
of the tissue. Coupling agent consists of:
1.
2.
3.
4.
5.
Carbomer10 g
EDTA0.25 g
Propylene glycol75 g
Trolamine12.5 g
Distilled water up to 500 g.
Doppler Ultrasound
Echoes produced by moving objects have different frequencies than the pulses sent into the body. This is called
the Doppler effect, which is put to use in detecting and
measuring tissue motion and blood flow. Color depends
on the flow of blood toward or away from the transducer.
Colors are always opposite for artery and vein, so if artery
is red, vein will be blue or vice versa. This is used to see
tissue motion, obstruction and thrombosis. In addition, it can
also detect direction, speed and character of blood flow.
Color Doppler ultrasonography minimizes the need for
biopsy or ne needle aspiration cytology (FNAC). The
presence of blood ow signals in the center of node (this
indirectly denotes the existence of the converging sinuses)
suggests that the node is benign. The presence of peripheral ow suggests a malignant nature (tumors larger than
a few millimeters in diameter stimulate the growth of new
vessels).
It has also been applied to automatic door openers and
to burglar alarms.
Sialadenosis It reveals homogenous, echogenic parenchyma extending enlargement of the glands secondary to
tumor.
Pleomorphic adenoma It reveals homogenous ultrasonic
pattern with decreased echogenicity and smooth borders.
But occasionally, tumor is hyperechoic with cystic areas of
calcification.
Cystadenolymphoma It reveals hypoechogenic with
smooth border. Hypoechogenicity is homogenous/inhomogenous with cystic areas with multiple septae.
Disadvantages
Artifacts
1.
2.
Section thickness: It results from beam width perpendicular to the scan plane. Echoes are received that
originate not only from the center of beam but also
from off center. Third dimension volume is visible.
Reverberation (multiple reflection): It can occur between
the transducer and a strong reflector. Multiple echoes
may be sufficiently strong to be detected and cause
3.
4.
5.
6.
7.
SIALOGRAPHY
Sialography is the retrograde injection of contrast agent
(usually iodine based) into the ductal system of a salivary
gland. In 1925, Barsony introduced the technique of injecting a radiopaque medium (20% potassium iodide) into the
ductal system of salivary glands.
Indications of Sialography
1.
2.
3.
4.
5.
6.
7.
Procedure
Armamentarium
1.
2.
3.
4.
5.
6.
7.
Preimaging assessment
Patient should be asked about the following:
1.
2.
3.
4.
5.
4.
Technique
1.
2.
3.
4.
5.
6.
to suck on freshly cut lemon will produce saliva aiding in locating the duct orifice.
Contrast medium is injected into the salivary duct.
Phases of Sialography
1.
2.
3.
Ductal phase
Acinar phase
Evacuation phase
Ductal phase
The ductal phase begins with the injection of the contrast
medium and terminates once the parenchyma becomes
794
The evacuation phase evaluated under intermittent fluoroscopy for about one minute, while checking for spontaneous clearing of the contrast agent from the gland. A
normally functioning, unobstructed gland should be able
to clear nearly the entire contrast agent.
The second subphase is an evaluation of the glandular
response to stimulation using a sialogogue or 2% citric
acid drops placed on the tongue and intermittently monitoring the clearing of the contrast from the gland. The second subphase is performed if a signicant amount of
contrast is still present in the ductal system after the rst
subphase. The nonclearing or partial clearing of the gland
may be due to a stricture, a sialolith or both or an underlying physiologic abnormality.
Post-stimulation views of the gland may demonstrate
adequate clearing of the ductal system and intraparenchymal collections of contrast. Small 13 mm uniformly distributed collections of contrast agent may be seen as a
Contraindications of Sialography
One of the more commonly used approaches involves injection of contrast material into the lower joint spaces, referred
to as lower joint space or single contrast arthrography.
Perforations of the disk or posterior attachment are
demonstrated by contrast material simultaneously owing
into the upper joint space as the lower space is injected.
Another variation of the technique involves injecting contrast material into both the spaces and viewing the more
central portions of the joint with tomography. Because
contrast material is in both joint spaces, the outline of the
disk is proled, showing its conguration and position.
The outline of the disk can often be enhanced by using double contrast arthrography. This technique involves injecting a small amount of air along with a small amount of
contrast material into both joint spaces, producing a thin
coat around the periphery of both joint spaces that highlights the disk and the joint spaces.
ARTHROGRAPHY
Arthrography involves injection of a radiopaque contrast
material into the joint spaces. The space occupied by the
disk can then be visualized lying between the layers of
contrast material.
Dr Fleming Norgaard, in 1947 was the rst to successfully use contrast arthrography. But it was in the 1970s that
Wilkes and others introduced and popularaized the technique in the United States.
Procedure
1.
2.
Uses of Arthrography
3.
4.
5.
6.
7.
Types of Arthrography
1.
2.
8.
Limitations
1.
2.
Complications
1.
2.
3.
4.
5.
6.
7.
8.
THERMOGRAPHY
The rare serious complications associated with arthrography include joint sepsis, allergic reaction to the
iodinated contrast medium and hemarthrosis.
Pain during and after the procedure, extravasation of the
contrast medium, disk perforation and transient facial
paralysis are less serious complications of arthrography.
The radiation exposure to the patient can be significant, depending on the duration of fluoroscopy and
the number of tomographic exposures made.
The most frequent complication of the technique is the
extravasation of contrast medium into the capsule and
soft tissues around the joint, causing pain. Nonionic
contrast media will be the agents of choice to minimize
this discomfort.
Parotitis has been reported following arthrography with
large needles and cannulas.
Some patients experience a vagal reaction, as a result
of increased anxiety during the procedure, this can be
managed by administering 0.6 mg of atropine intravenously.
Intravasation of contrast material infrequently occurs.
Epinephrine in a dose of 0.03 ml (1:1000) per 3 ml of
contrast material is recommended because there is a
risk of an acute hypotensive episode with intravasation of higher doses.
Transient facial paralysis may result from a rapid infiltration of lidocaine. Some patients experience a moderate degree of pain as the needle is placed on the
Figure 88
37.0
36.2
35.4
34.6
33.8
33.0
32.2
31.4
30.6
29.8
29.0
C
Two examples of infrared images of the lateral aspect of the face. Courtesy: Prof Francis Ring, Head of Group,
Thermography, University of Glamorgan, UK
796
Figure 89
38.5 C
38
36
34
32
30
28
26
25.5 C
Infrared and visual image of a subject with an elevated body temperature. The color alarm clearly shows
the parts of the head with a temperature higher than 38C
Patient Preparation
Procedural Requirements
The room should be of adequate size to maintain a uniform temperature. A room approximately 8 10 feet size
is adequate to meet these requirements. During the examination, the patient should be positioned relatively equidistant and adequately spaced from each wall. The room
should be carpeted. Curtains may be used to prevent outside infrared radiation from entering the room. The room
Indications
Merla et al (2004) assessed the use of functional infrared
imaging in the diagnosis of the myofascial pain. They
concluded that functional infrared imaging seemed to distinguish healthy subjects from the patients suffering myofascial pain.
Gratt and Anbar (1998) summarized the following clinical applications for thermography in dentistry:
1.
2.
3.
4.
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Prelims-Ongole.indd ii
10/15/2012 2:06:46 PM
SECTION
XI
Processing of
Radiographs and
Radiographic
Interpretation
29 Latent Image Formation
30 Processing of Radiographic Films
31 Radiographic Faults
801
803
812
799
Chapter-29.indd 799
10/3/2012 10:59:18 AM
Prelims-Ongole.indd ii
10/15/2012 2:06:46 PM
CHAPTER
29
2.
1.
3.
Figure 1
Silver ion
Bromide ion
Sensitivity site
X-ray photon
Silver bromide
ee-
X-ray film
Interstitial
silver ions
e- e- e e-
e-
eee- e
e-
801
802
3.
4.
5.
6.
7.
As a result of Compton and photoelectric effects, electrons are displaced from the bromide ions producing
high-speed electrons (recoil electron) and scattered
X-ray photons.
These recoil electrons travel within the silver halide
crystal creating additional bromine atoms, scattered
photons and secondary recoil electrons until their
energy is dissipated.
These recoil electrons get trapped in the latent image
site and impart a negative charge to these sites.
The free interstitial silver ions which are positively
charged are drawn toward the negatively charged
latent image sites.
Within these sites the silver ion is neutralized resulting in an atom of sliver that gets deposited.
CHAPTER
Processing of Radiographic
Films
Ravikiran Ongole
Daylight Processor
Self-developing Film
30
Automatic Processing
Working Mechanism
Composition of Processing Solutions
Ideal requirements
1.
2.
2.
Manual processing
Timetemperature method (darkroom technique)
Visual method (darkroom technique)
Daylight processing (darkroom not required)
Self-developing films (darkroom not required)
Automatic processing.
Figure 1
Darkroom
Radiographic films are sensitive to X-rays and visible
light. In order to prevent exposure of the films to visible
light it is necessary to process the exposed radiograph in a
room specially designed to keep away visible light.
803
Figure 2
A
Conch shell
design
Working
area
(A) Illustration showing the doorless maze pattern of a darkroom. (B) Conch shell design for the darkroom.
Courtesy: Dr Jaideep Shekhar
Figure 3
Figure 4
3.
804
Figure 5
A
(A) Master tank with water and containing the developer and fixer solution tanks.
(B) Master tank covered with a lid when not in use
Figure 6
A
(A) Photograph showing the visible light mounted on the ceiling. (B) 15 W safe light in the orange-red spectrum of light
Figure 7
A
(A) Floating thermometer. (B) Photograph showing the floating thermometer in the master tank containing water
Figure 8
Dryer cabinet
Wet films following the processing can be dried effectively
using cabinet driers, which help in circulating warm air
(produced by heaters) around the films thereby hastening
the drying process (Figure 9).
However, lms should not be placed close to the heat
source in the cabinet drier as they can get distorted. Alternatively, wall mounted fans over the drying racks can be
used. Cabinet driers may also be placed outside the darkroom.
Processing technique
Film hangers shown drying after use
806
Table 1
Figure 9
Visual Method
In this method, there is no predetermined time for which
the film is placed in the developer solution. The exposed
film is placed in the developer solution and taken out at
regular intervals and examined under safe light for the most
calcified/radiopaque structures to be evident. Appearance
of radiopaque structures (like the enamel cap, restorations,
artificial crowns) indicates the end point of developing
time. At this juncture, films are taken out of the developer
solution, rinsed in water and placed in the fixer solution.
Disadvantage Quality of images is not reproducible as
the technique is very subjective.
Development time
(minute)
68
70
4.5
72
76
80
2.5
9.
10.
11.
12.
Daylight Processor
It is essentially a plastic housing with a 15 W bulb. The
housing is just large enough to cover three plastic bowls
that contain developer, water and fixer solutions. It is like
a make-shift arrangement for a darkroom especially for
dental clinics and mobile dental clinics.
The operator can slide his/her hands via two sleeves provided at the sides of the plastic housing and then unwrap
the lm. A plastic, tinted see-through lid at the top of the
housing enables the operator to see within the plastic case
without causing unnecessary light exposure (Figure 10A, B).
Under safe light conditions, provided by the 15 W
orange-red bulb, the lm can be unwrapped, helped with
Self-developing Film
These have also been referred to as rapid autodeveloping
films. It is essentially a one-piece soft plastic (polyvinyl
chloride) pouch designed such that the film is located at
one end and a monobath of developer and fixer is located
at the other end. Both the ends are connected via a narrow
corridor that helps in carrying the processing solutions to
the film (Figure 11).
During the exposure, the lm end is positioned in the
mouth with the other end hanging outside the mouth. Once
the exposure is made, the pouch is rst washed to remove
the patients saliva. Then the lm is developed by holding the
monobath end of the pouch upright and rolling it down
until the processing chamber ruptures and empties into the
corridor. Continuing to roll the pouch ensures that all of
the monobath solution travels toward the lm end.
The user continues to hold the pouch upright and massages the monobath around in the lm end of the pouch
Figure 11
Self-developing film
Figure 10
A
(A) Daylight processing unit. (B) Small cups filled with processing solutions within the daylight processor
808
Figure 13
AUTOMATIC PROCESSING
Automatic film processing refers to the processing of
radiographs using machines specifically designed for that
purpose. Most automatic processors are not universal
(there are separate machines to process extraoral and
intraoral films). These processors have inherent limitations
with regard to the need for regular maintenance and cost
(Figure 12).
Working Mechanism
The automatic processor is made up of five basic systems
the transport system, the circulation and filtration system, the
replenishment system, the tempering system, and the dryer
system.
Figure 14
Figure 12
Figure 15
809
Replenishment system
As each film passes through the automatic processor, the
chemicals are changed slightly. To offset the resulting
deficiencies, new developer and fixer in measured amounts
are pumped into the solutions via the replenishment tanks
attached to the processor. These tanks should be checked
weekly and refilled periodically.
Tempering system
To maintain the desired temperature of the developer and
fixer, a heating device and automatic thermostat are used.
Water is passed through a mixing valve, so that it is 4F or
5F below the desired temperature, and then heated to the
desired temperature by the heating element in the machine.
The wash water temperature is controlled by a mixing
valve, which mixes the hot and cold water. A thermometer
gauge is located between the mixing valve and the wash
tank near the mixing valve.
Air-dryer system
To dry the film, there is a heater to heat the air and a
blower to direct the air. An efficient exhaust system ensures
that the warm, moist air is removed and only hot, dry air
is directed over the films as they move through the rollertransport system.
Developer
Activator
Preservative
Restrainer.
Developer
Function It converts the exposed silver halide grains to
black metallic silver. It is made up of phenidone and
hydroquinone. Phenidone acts as an electron donor and
810
Activator
Function Activators help in maintaining the alkaline pH
of the developer solution and cause the gelatin of the film
to swell thereby helping the diffusion of the developing
agents into the emulsion and reach the silver halide crystals. Developer solutions are active at an alkaline pH
(approximately 10).
This alkaline pH is achieved and maintained by the
addition of alkaline agents such as sodium or potassium
hydrozide and buffers like sodium carbonate, sodium
hydroxide and sodium metaborate or tetraborate.
Preservative
Function It prevents the oxidation of the developer solution by atmospheric oxygen. It also combines with the
oxidized developer solution (appears brown) and forms a
colorless soluble compound. The preservative is an antioxidant and is usually sodium sulfite.
Restrainer
Function It acts as an antifog agent. It minimizes/
restrains the development of unexposed silver halide grains.
Potassium bromide or sodium bromide is used as the
restrainer in the developer solution.
Fixing solution
Functions Fixing solution helps in removal of the
undeveloped silver halide grains from the emulsion. If the
unexposed silver halide grains remain on the film the radiographic image will appear black and will result in a nondiagnostic image.
Composition The fixer solution contains four constituents, which are dissolved in water. They are:
Clearing agent
Acidifier
Preservative
Hardener.
Clearing agent
Function Dissolves and removes the unexposed silver
halide crystals from the emulsion of the film. The removal
of the unexposed crystals takes place at a controlled slow
pace, however fixing for a prolonged time can lead to a
gradual loss of film density (silver grains dissolve in acetic
acid of the fixing solution).
Automatic processing
Darkroom is required
Technique sensitive
Negligible maintenanceneed to
clean the tanks and replace fresh
solutions every 23 weeks
Regular maintenanceneed to
clean rollers and servicing gear
mechanism
Expensive
Penny Test
Penny test or coin test is used to assess the safe light condition in the darkroom. It is a known fact that radiographic
film is sensitive to visible light and X-rays. Excessive exposure to safe light or an improper safe light condition can
lead to exposure of the film resulting in film fog.
Procedure
1.
2.
3.
In the darkroom, an exposed film is removed from the film packet and placed on the working bench in an area
where films are usually unwrapped.
A coin is placed on the film and left in place for a few minutes (usually the time it takes for a film to be unwrapped
and clipped onto a film holder for processing.
Process the film as usual. If the image of the coin is seen on the processed film then the darkroom does not have
optimum safe lighting.
811
CHAPTER
31
Radiographic Faults
Ravikiran Ongole, Praveen BN
Film Fog
Emulsion Peel and Scratched Film
Dark Spots or Lines
Static Electricity Artifact
Nail Marks or Kink Marks
Artifacts
Film Fog
Radiographic faults can occur at any stage in the radiographic process, right from handling and storing of films
to the processing. The causes for radiographic faults can
be categorized as:
812
Blank Radiograph
Dark Radiograph
Light Radiographs
Film Fog
Insufficient Contrast
Yellow or Brown Stains
Partial Image
Blisters on the Film
White Spots
Dark Spots on the Radiograph
Light Spots on the Radiograph
Emulsion Wash Away
Reticulation of Emulsion
Hyporetention
Dyschroic Fog
No Image/Blank Radiograph
Causes
Outdated films
Films exposed to extraneous radiation.
Figure 1
Figure 2
A
813
Figure 3
Figure 4
Figure 5
A
Cause
Causes
Cause
Cone Cut
Figure 6
Causes
Figure 7
A
(A) Partial image due to improper film placement resulting in the apices of premolars cut-off. (B) Partial image caused due to
opening of the patients mouth during exposure
815
Figure 8
Figure 9
Figure 10
Cause
Improper placement of film (Figure 8).
Overlapped Image
For all practical purposes, the central beam of X-rays should
be directed perpendicular to the film and tooth (in paralleling technique) and to the imaginary bisector bisecting
the long axis of tooth and long axis of film (in bisecting
angle technique) thereby passing through the interproximal
contacts between teeth.
Causes
Figure 11
Fore-shortened Image
The radiograph exhibits unusually short images (Figure 11).
Causes
Fore-shortened images
Causes
Blurred Image
Blurred images can either be total or partial. Partial blurring occurs when the film gets bent excessively when
Figure 13
Figure 12
Elongated images
Figure 14
A
817
Figure 15
Figure 17
Light radiograph
Figure 16
b.
Processing errors
Developer temperature too high
Film developed for a longer time
Concentration of the developer too high
Accidental exposure to light
Improper safe lighting.
Exposure errors
Insufficient mA, insufficient kVp, insufficient
exposure time
Film packet placed with the wrong side facing the
X-ray source
Increased film source distance
Processing errors
Placed in the developer solution for a short duration of time
Temperature of the developer solution too low
Depleted developer solution
Diluted or contaminated developer solution
Prolonged fixation.
Dark radiograph
b.
Blank Radiograph
Causes
Exposure errors
Excessive milliampere (mA), excessive kilovolt peak
(kVp), excessive exposure time
Insufficient filmX-ray source distance
818
Film Fog
Causes
Figure 18
Figure 19
Yellow/brown stains
Insufficient Contrast
Causes
Figure 20
Underexposed to radiation
Insufficient developing time.
Partial Image
White spots
Cause
Part of the film not immersed into the developer solution.
Causes
Causes
Figure 21
Figure 23
Figure 22
Causes
Inadequate washing
Remaining thiosulfate from fixer solution.
Dyschroic Fog
Fogging of the radiograph, characterized by the appearance of a pink surface when the film is viewed by transmitted light and a green surface when the film is seen by
reflected light.
Cause
Reticulation of the emulsion
Reticulation of Emulsion
Cause
Reticulation of emulsion results when a film is subjected
to a sudden temperature change between the developer
solution and the water bath (Figure 22).
Hyporetention
It appears as a yellowish stain on the radiograph that is
processed (Figure 23).
820
No Image/Blank Radiograph
Since none of the X-rays reach the film to form the latent
image, all the unexposed silver halide grains are removed
by the clearing agent resulting in a blank radiograph
(Figure 24).
Causes
ARTIFACTS
An artifact is a structure or radiographic appearance that
is normally not present in the radiograph and is produced
Figure 24
Blank radiograph
Figure 25
B
(A) Nose stud artifact. (B) Hair clip, nose stud and earrings artifacts. (C) Intraoral appliance.
(D) Cast partial denture. (E) Phalangioma
821
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SECTION
XII
Radiographic
Landmarks
825
837
842
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CHAPTER
Intraoral Radiographic
Anatomical Landmarks
32
Ravikiran Ongole
Mandibular Radiographs
Teeth
Periodontal Ligament Space
Alveolar Bone
During the process of interpreting a radiograph the radiologist is expected to identify normal radiographic anatomy, thereby distinguishing normal features from
pathology. These normal radiographical anatomical landmarks are unique to each area of the maxilla or mandible
and appear either radiopaque or radiolucent. They may be
used to identify a specific area of the jaw. For example, the
intermaxillary suture is unique to the radiographs of the
maxillary central incisors, mental foramen is typically
associated with the radiographs of the mandibular premolars etc. On occasions, these landmarks may exhibit some
minor variations (such as a bifid mandibular canal) or may
not be very evident on a faulty radiograph.
The landmarks discussed here are those that are seen in
intraoral periapical radiographs.
Teeth
Enamel cap, dentin, cementum, pulp.
Enamel appears almost snow white and is seen extending from the neck of the tooth, i.e. the cementoenamel
junction from one side to the other covering the tooth like
a cap (enamel cap). It is the most mineralized structure on
the radiograph (90% mineralized) and appears more radiopaque than other structures (Figure 1).
Dentin is seen as homogenous radiopacity (less radiopaque than enamel as it is 75% mineralized) and radiographically has the density of bone.
Figure 1
825
Figure 2
Figure 3
Figure 4
Alveolar Bone
Lamina dura, alveolar crestal bone, cancellous trabecular
bone.
Lamina dura
Lamina dura is a radiographic term used to describe alveolar bone proper (cortical bone), which forms the sockets of
teeth. It is also considered to be a specialized continuation of the cortical plate. Radiographically, lamina dura is
826
Figure 5
Figure 6
Figure 7
A
(A) Trabeculae are fine and dense in the maxillary anterior region. (B) Trabeculae are oriented irregularly and
bone marrow spaces are relatively
Alveolar crest
Crest of the alveolar bone is a radiopaque structure. It is
seen as a continuation of the lamina dura (cortical bone).
The junction between the alveolar crest and lamina dura is
seen as a sharp well-defined angle. Generally the crest of
the alveolar ridge is 1.5 mm apical to the cementoenamel
junction. In the anterior teeth, the alveolar crest terminates as a pointed projection and in the posterior teeth it
appears flat and parallel to the cementoenamel junction
(Figures 5 and 6).
Cancellous bone
It is present between the buccal and lingual cortical plates
in the maxilla and the mandible and accounts for most of
the bulk of the alveolar bone. The cancellous bone is made
Intermaxillary suture
Anterior nasal spine
827
Figure 8
A
(A) Radiograph of the mandibular anterior region showing less number of trabeculae that are oriented horizontally.
(B) Radiograph of the mandibular posterior region showing large bone marrow spaces
828
Figure 9
fossa. When an IOPAR is taken for the upper central incisors it is located between and roughly 2 cm beyond the
periapices of the central incisors.
Radiographic appearance (Figure 8B) It is a V-shaped
radiopaque structure.
Nasal fossae and floor of the nasal fossa
Figure 10
Figure 11
Figure 12
Nasal septum
Anatomical location/projection It is seen on periapical
radiographs taken in relation to maxillary central incisors.
It is a midline structure and seen extending superiorly
from the anterior nasal spine.
Radiographic appearance (Figure 10) The nasal septum
is a sharply defined linear radiopacity, which may generally appear to be deviated from the midline. However, on
occasions the image of septal cartilage and the vomer bone
may be superimposed over the nasal septum.
Incisive foramen
Anatomical location/projection Incisive foramen is
seen in relation to the middle and apical one-third of the
roots of the maxillary central incisors. It is seen on periapical radiographs taken in relation to maxillary central
incisors.
Radiographic appearance (Figure 11) It is radiolucent and
may have various appearances ranging from a smoothly
symmetric outline to irregular or ill-defined border and usually smaller than 1 cm in diameter.
Tip of the nose
Anatomical location/projection The image of the tip
of the nose is superimposed over the apical one-third of
the roots of the maxillary central and lateral incisors. It is
usually seen in individuals with a prominent and bulbous
nose tip. The shadow of the tip of the nose is seen when a
radiograph is taken for the maxillary central incisors.
Radiographic appearance (Figure 12) It appears as a
homogenous cup-shaped radiopacity with a definite outline.
Lateral fossa/incisive fossa
Anatomical location/projection It is seen in relation to
the apical region of the maxillary lateral incisor. Radiographs
of the lateral incisors will reveal this anatomic landmark.
Radiographic appearance (Figure 13) It appears as a
diffuse radiolucency.
829
Figure 13
Figure 14
Figure 15
Nasopalatine canal
Anatomical location/projection The nasopalatine canal
is rarely seen on periapical radiographs. However, they may
be evident on radiographs taken in relation to the maxillary central incisors.
Radiographic appearance (Figure 14) The lateral walls
of the nasopalatine canal appear radiopaque and extend
from the incisive foramen to the floor of the nasal fossa.
Figure 16
Figure 17
Figure 18
Figure 19
Maxillary sinus
Radiographic appearance In relation to the periapex of
the canine, the floor of the maxillary sinus and the floor of
the nasal fossa cross one another forming an inverted Y,
which is referred to as Y line of Innes (Figure 18).
Canine fossa
Anatomical location/projection Seen at the periapical
regions of the maxillary canine (Figure 19).
Radiographic appearance Seen as a diffuse radiolucency.
Figure 20
Figure 22
Figure 21
the maxillary sinus in relation to the apices of the molar
teeth. It is seen as a U-shaped or V-shaped radiopaque line.
The zygomatic bone is seen as a homogenous radiopacity
over the apices of the second and third molars, generally
obscuring the periapical region.
Pterygoid plates and hamular process
Anatomical location/projection The pterygoid plates and
hamular process are seen distal to the maxillary tuberosity.
They are generally seen on intraoral periapical radiographs
taken in relation to the third molars. However, in most
cases they may not be evident.
Figure 23
Figure 25
Figure 24
Symphysis
Anatomical location/projection Seen only in the first year
of life. It is seen in the region corresponding to the midline
of the mandible. It is seen on the radiographs of the deciduous mandibular central incisors.
Radiographic appearance Symphysis appears as a linear
radiolucency between the deciduous mandibular central
incisors and extending inferiorly to involve the lower border of the mandible (Figure 24).
Genial tubercles (mental spine) and lingual
foramen
Radiograph showing the pterygoid plates as a radiopaque
shadow distal to the maxillary tuberosity and the hamular
process as a radiopaque linear structure extending from the
inferior aspect of the medial pterygoid plate
Symphysis
Genial tubercles
Mental ridge
Mental fossa
Mental foramen
Mandibular canal
Mylohyoid ridge
Submandibular gland fossa
External oblique ridge
Inferior border of mandible.
Anatomical location/projection They are generally evident on the periapical radiographs of mandibular central
incisors and mandibular occlusal radiographs.
Radiographic appearance Genial tubercles are seen on
mandibular occlusal radiographs as discrete radiopaque
structures measuring about 4 mm in diameter. They are
present beneath the apices of the mandibular central incisors in the midline.
In periapical radiographs, they are seen as a well-dened
radiopaque mass enclosing a circular radiolucent area which
is referred to as the lingual foramen (incisal branches of
mental nerve exit out of the lingual foramen to innervate
the incisor teeth) (Figure 25).
Mental ridge
Anatomical location/projection Mental ridge is seen
superimposed over the apical one-third of the roots of the
mandibular central and lateral incisors. It appears to extend
833
Figure 26
Figure 27
Figure 28
Radiographic appearance Seen as two well-defined radiopaque lines extending bilaterally from the premolar region
toward the midline.
Mental fossa
Anatomical location/projection The mental fossa is an
anatomical depression present on the labial aspect of the
mandible extending bilaterally from the midline to the lateral incisor and occasionally up to the canine.
Radiographic appearance It is seen as a diffuse radiolucent area, which is superimposed over the roots of the
mandibular anterior teeth. The mental fossa is bounded
superiorly by the alveolar ridge and inferiorly by the mental ridge (Figure 26).
Mental foramen
Anatomical location/projection The mental foramen is
usually seen at the periapical regions of mandibular premolars. It is usually present equidistant from the both the
lower border of the mandible and the alveolar ridge.
Radiographic appearance It appears radiolucent and has a
round, ovoid or an elongated shape. It may or may not have
a well-defined radiopaque corticated border (Figure 27).
Mandibular canal
Anatomical location/projection The mandibular canal is
usually seen on periapical projection of mandibular molars.
The apices of the molar teeth may some times lie close to
the canal.
834
Figure 29
Figure 31
Figure 30
Figure 32
the external oblique ridge that runs along the apical thirds
of these teeth.
External oblique ridge
Anatomical location/projection It is seen on IOPARs of
mandibular posterior teeth. The external oblique ridge is
seen above and parallel to the mylohyoid ridge.
Radiographic appearance (Figure 30) The external
oblique ridge appears as a linear radiopacity which merges
with the alveolar bone as it traverses toward the premolar
region and superiorly it continues as the ascending ramus
of the mandible.
Figure 33
Figure 34
Maxilla
Mandible
Genial tubercles
Mental ridge
Mylohyoid ridge
External oblique ridge
Inferior border of the mandible
836
Maxilla
Mandible
Intermaxillary suture
Nasal fossa
Incisive foramen
Superior foramina of nasopalatine
canal
Lateral fossa/incisive fossa
Canine fossa
Nasolacrimal canal
Maxillary sinus
Symphysis
Lingual foramen
Mental foramen
Mandibular canal
Submandibular gland fossa
CHAPTER
Extraoral Radiographic
Landmarks
33
Praveen BN
Figure 1
23
20
22
12
21
10
24
16
8
17
1
19
11
13
18
15
6
7
4
14
3
2
Lateral cephalogram
837
Figure 2
1
2
8
9
4
10
5
6
11
7
12
13
14
1. Supraorbital canal
2. Nasal septum
3. Infraorbital margin
4. Ala of nose
5. Zygomatic bone
6. Maxillary sinus
7. Lateral wall of maxillary sinus
8. Frontal sinus
9. Zygomaticofrontal suture
10. Innominate line
11. Inferior orbital fissure
12. Sphenoid sinus
13. Foramen rotundum
14. Mid-palatal suture
Figure 3
1
2
3
4
3
5
7
14
15
8
16
9
10
13
1. Nasal septum
2. Mid-palatal suture
3. Sphenoid sinuses
4. Coronoid process of mandible
5. Ramus
6. Condyle of mandible
7. Clivus
8. Posterior pharyngeal
9. Anterior arch of atlas
10. Dens
11. Occipital condyle
12. Ossicles
13. External auditory meatus
14. Foramen spinosum
15. Foramen ovale
16. Hyoid bone
11
12
838
Figure 4
2
2
Open mouth
Closed mouth
Figure 5
19
18
17
2
3
16
5
15
6
8
9
14
11
13
10
12
7
839
Figure 6
1
1. Condyle head
2. Articular fossa
3. Articular eminence
2
3
Open mouth
Closed mouth
Figure 7
13
2
3
12
4
5
11
10
7
6
9
8
840
1. Foramen magnum
2. Posterior arch of atlas
3. Dorsum sellae
4. Frontal process of zygoma
5. Posterior wall of maxillary sinus
6. Inferior orbital fissure
7. Zygoma
8. Inferior rim of orbit
9. Nasal septum
10. Coronoid process of mandible
11. Condyle of mandible
12. Internal auditory meatus
13. Articular eminence
Figure 8
15
16
13
10
18
12
14
5
3
11
17
7
4
19
8
20
1. Mandibular canal
2. Inferior border of zygomatic arch
3. Posterior wall of maxillary sinus
4. Floor of maxillary sinus
5. Anterior wall of maxillary sinus
6. Inferior orbital canal
7. Nasal septum
8. Mental foramen
9. Soft tissue of inferior turbinate
10. Pterygomaxillary fissure
11. Coronoid process
12. Condyle
13. Inferior orbital rim
14. External auditory meatus
Orthopantomogram
Figure 9
27
28
22
25
21
22
21
21
21
21
21
24
26
29
23
30
Orthopantomogram
841
CHAPTER
34
Historical Perspectives
Indications for Dental Implants
Patient Management with Dental Implants
Upper lip
Implant
fixture
Crown
prosthesis
842
Historical Perspectives
The treatment options that are available for replacement
of single or multiple teeth in the oral cavity are removable
partial denture (RPD), fixed partial denture (FPD) and
implant prosthesis (IP). The RPDs which were not helpful
in the maintenance of existing bone, compromise the
esthetic result, need more bulk for cross-arch stabilization,
easily trap food debris and plaque, and interfere with the
speech and function. Similarly, the FPDs especially promote caries of the abutment teeth but if not well fabricated, lead to plaque retention in the pontic increasing
periodontal risk, involve damage to adjacent teeth, fracturing of the porcelain, and have esthetic concerns for the
anterior regions. The FPDs are contraindicated when there
is poor abutment support, inadequate hard and soft tissues
in the esthetic regions and in young patients with large
pulp horns. The dental implants would be an ideal alternative for replacement of teeth.
Historically, there were three distinct forms of dental
implants, namely, the subperiosteal (epiosteal), the transosseous (staple bone or transmandibular) and the endosteal
(blade or plate, ramusframe and root form) types. Dental
practitioners should be familiar with the radiographic
appearances of older dental implant systems although many
Figure 2
Posts will remain
outside the gingiva
as anchors for the
new prosthesis
Transosseous implants
Subperiosteal metal
framework
Mandible
Gingiva covering the
implant framework
of them are rarely used after the advent of the dental root
form implants.
Subperiosteal implants
Subperiosteal implants are metallic meshes that are custombuilt to fit over the alveolar processes and are located
underneath the periosteum (Mupparapu and Beideman,
2000). Direct bone impressions via surgery would lead
to the fabrication of mesh in a laboratory that fits under
the mucoperiosteum (Figure 2). The framework normally
rests on the mandible with no penetration into the bone.
Two surgeries were involved in the fabrication of these
implants. First surgery is for the impression and the second surgery is for the placement of the mesh. The first
subperiosteal dental implant in the world was placed by
George Dahl in Sweden. The first American subperiosteal
implants were developed in 1947. Gershkoff and Goldberg
placed the first subperiosteal complete denture implant
manufactured with Vitallium (Moore and Hansen, 2004).
Dr Leonard Linkow of New York made significant changes
to the original design by incorporating fenestrations into
the buccal peripheral struts which made it possible for the
mucoperiosteum to reattach the bone in between these
fenestrations. He also holds 35 patents on various designs
of subperiosteal and oral implants. Multiple metallic posts
extend from the mesh into the oral cavity crossing the
mucoperiosteal barrier to support the prosthesis. Mandibular subperiosteal implants have been shown to be successful in many clinical studies and some of the implants
reviewed were present in the mouth for more than 10 years.
The success rate for these implants varied in many studies
with higher success rate for 10-year periods and lower
success rates for longer periods (Moore and Hansen, 2004).
Computed tomography (CT) scans were also used to allow
Endosteal implants
There are three forms of endosteal implants: the root form,
the ramus frame and the plate or blade form implants.
Blade implants are rectangular in shape similar to a razor
blade. These are used over horizontal column of bone. One
or more posts usually extend into the oral cavity to permit
fixation of the prosthesis. The only blade implants that are
seen clinically at present are the previously placed implants
that either failed or fractured and the patient reported for
treatment. One such example where the blade implant failure led to the osteomyelitis in the mandible is shown in
Figure 3. The root form implants can be either cylindertype, screw root type or a combination of both. These
implants use the vertical column of bone unlike the blade
implants.
Types of dental implants available
(according to the material used)
The following are the commonly used implants according
to the type of material used for fabrication:
1. Metallic implants
a. Titanium
b. Cobaltchromiummolybdenum alloy with titanium,
aluminum, vanadium
c. Cobaltchromiummolybdenum
d. Stainless steel
e. Zirconium
f. Tantalum
g. Gold
h. Platinum
2. Non-metallic implants
a. Ceramics
b. Carbon.
The most commonly used implants are:
1. Commercially pure (CP) titanium This is light weight,
biocompatible and corrosion resistant. The material is many
times stronger than compact bone and the modulus of
elasticity is five times greater than that of compact bone.
This equals the mechanical stress transfer of compact bone.
843
Figure 3
Failing blade implants in the mandibular molar region as seen from the panoramic reformatted image from a
Denta-Scan CT study. On the right is a picture of a premolar blade implant manufactured by Oraltronics, Russia
Figure 4
Facial profile changes after loss of teeth including the collapse of the perioral soft tissues and loss of vertical dimension
Since the success of implant therapy is tied to the osseointegration, this is a crucial step in the overall implant therapy. Several medical conditions that would jeopardize the
success of an implant should be considered during the
screening process. The list (Hwang and Wang, 2006) includes
the following absolute contraindications and relative contraindications:
Absolute contraindications
1. Recent myocardial infarction or cerebrovascular accident
Due to the increased risk of complications following a
myocardial infarction (MI) or a cerebrovascular accident
(CVA), the dental practitioner must wait until preliminary
stabilization. It is recommended that elective dental care is
deferred for at least 6 months status post MI or CVA and
the patient is medically stable.
2. Valvular prosthesis placement It is essential that the
dental practitioner defers placement of implants in these
patients for 6 months to 1 year after the cardiac surgery.
Anticoagulants would be used depending on whether the
valve is bioprosthetic or mechanical in which case, caution
should be exercised.
3. Prior history of bleeding Since uncontrolled bleeding
stems from a multitude of systemic conditions, patients who
are on oral anticoagulant therapy should be carefully evaluated and the bleeding time, international normalized ratio
(INR) must be assessed. An INR of 2.2 or lower is recommended for surgical procedures by Fazio and Fang (2003).
4. Immunosuppression If the WBC count falls below
1,5003,000 cells/mm3 the patient is not considered a good
candidate to receive implants as the ability to combat infections, repair or regeneration would be compromised. With
a near normal WBC count and a grossly abnormal neutrophil
count (less than 2,000 cells/mm3), the normal range being
3,5007,000 cells/mm3, a medical consultation is necessary
before planning dental implants. It is generally considered
that when the patients CD4 T-cell count measures below
500 cells/mm3, he/she is considered immunosuppressed and
appropriate care should be exercised.
5. Active radiation and/or chemotherapy Both ionizing
radiation and chemotherapy disrupt hematopoiesis, and
implantation should be deferred in such situations as wound
healing is delayed. In addition, if the salivary glands are
involved in the line of fire, there would be substantial
xerostomia which in turn, would contribute to poor oral
hygiene and increase in dental caries secondary to xerostomia. Studies have shown that in about 335% of patients,
spontaneous or traumatic osteoradionecrosis would be a
complication (Marx and Johnson, 1987). When the patient
is on cytotoxic anticancer drugs, granulocytopenia followed by thrombocytopenia are expected which might
lead to infection, hemorrhage, mucositis and pain. Implant
845
therapy should be deferred until after the patient is completely off the cytotoxic medication.
6. Psychiatric disorders If a patient is unable to comprehend and anticipate dental treatment logically, then it is
advised not to place implants. Several conditions including psychotic disorders like schizophrenia, severe character
associated conditions like hysteria, borderline personality
disorders, dysmorphophobia, cerebral lesions, presenile
dementia as well as alcohol and drug abuse. Although
there are no biological reasons for patients with most of
the above disorders to lose implants, there are reported
cases of removal of osseointegrated fixtures based on psychiatric factors (Hwang and Wang, 2006).
7. Intravenous bisphosphonate treatment There is enough
evidence to date linking bisphosphonate use to either spontaneous or traumatic induction of osteonecrosis of the jaw.
Bisphosphonates inhibit bone resorption and hence are
used for treatment of osteoporosis, hypercalcemia of malignancy and Pagets disease. Bisphosphonates may inhibit
osteoclast precursors and cholesterol synthesis as well as
promote osteoclast apoptosis and osteoblast proliferation.
In a large study, among all the different forms of bisphosphonates that were used in cancer patients, pamidronate
(Aredia, Novartis, Basel, Switzerland) and zoledronic acid
(Zometa, Novartis, Basel, Switzerland) stood out as the
agents that were associated with most cases of osteonecrosis, majority of them being mandibular necrosis with recent
dentoalveolar procedures performed on them (Ruggiero et al,
2004). So far, there are no published studies on the risk
stratification of osteonecrosis of the jaws after drug discontinuation. Hence, the risk is omnipresent once the bisphosphonates have been used and even if they are discontinued.
Professional organizations like the American Dental Association, the American Association of Oral and Maxillofacial
Surgeons and the American Academy of Oral Medicine have
issued guidelines regarding the identification and management of bisphosphonate-related osteonecrosis of the jaw.
8. Acute infection The presence of acute infection is an
absolute contraindication for oral implant therapy. The
soft tissues and bone should be completely devoid of any
infection before the implant therapy can be instituted.
9. Morphology of the edentulous bone crest and vestibule
Intraoral inspection and palpation may reveal information
about the type of ridge, a suspected thin ridge or a flabby
ridge, shape of the ridge, muscle insertions, mandibular tori
and the floor of the mouth. A radiographic evaluation of
the crestal bone is mandatory before treatment planning.
A knife-edged ridge often needs correction before inserting implants. A bony plateau must be created that is wide
enough to insert implants with an adequate width. This
could mean reducing the existing crest by a few millimeters.
The depth of vestibule is an important factor to consider.
Bone resorption often leads to shallow vestibule. If that is
846
Figure 5
D1 bone (1,250 Hounsfield unit [HU]) bone is composed of almost all cortical bone mass located primarily in the anterior mandible.
D2 bone (8501,250 HU) bone is composed of a thick
crestal layer of cortical bone and coarse trabecular bone
underneath the cortical bone. This type of bone can
mostly be found in the anterior maxilla and mandible
and in the posterior mandible.
D3 bone (350850 HU) is composed of a porous crestal
layer of cortical bone and fine trabecular bone underneath the cortical bone. This type of bone can mostly
be found in the anterior and posterior maxilla but also
in the posterior mandible. It is also seen after osteoplasty of D2 bone.
D4 bone (150350 HU) bone is composed of primarily
fine trabecular bone and often the absence of cortical
bone. This type of bone can mostly be found in the
posterior maxilla and poses the greatest challenge in
implant placement (Figure 5). Implants usually placed
in D1 or D2 bone stand a very good chance of undergoing osseointegration, while the implants placed in
D3 or D4 bone either undergo fibrointegration or fail
to integrate at all. There are many factors that affect
the long-term success or failure of an implant.
Figure 6
Figure 7
Figure 9
Figure 8
Dental computed tomography
Multidetector computed tomography (MDCT) is an imaging technique that uses reconstruction mathematics to
create images based on a series of radiographs. The initial
CT concepts were developed by Alan Cormack and later
brought to fruition by a British engineer Godfrey Hounsfield
in 1972. The technique was first used to produce crosssectional images of the brain. The radiographic densities
of the imaged structures were compared along a scale with
the standard densities of water, air and the bone. The units
came to be known as Hounsfield units (HU) or CT numbers. The HU has been used for measurement of radiodensity of structures being examined. The numerical scale
ranges from air (1,000) to water (0) and to bone (1,000)
and upward of 1,000 for even denser structures. The application of HU measurement can decrease the observer bias
inherent in the interpretation of plain films. Overall, the
HUs are important in an objective determination of bone
density. Dental CT and the concepts of curved plane
tomography were first published by Schwarz and coworkers
in 1987 (Schwarz et al, 1987), which were utilized in the
dental CT studies for reformatting the multiplanar and
cross-sectional views. In addition, the dental CT programs
like the DentaScan were able to produce the panoramic
reformation from the conventional CT datasets (Figure 10).
This gained acceptance by the dental practitioners. Even
though the CT radiation dose was higher compared to all
the previous radiographic modalities, the benefit of accurate placement of implants outweighed any potential
risks.
849
Figure 10
Figure 11
Figure 13
Figure 12
Large volume
Height: >16 cm
Width: >18 cm
Figure 14
Nobel Biocare
Replace Select
Straight RP
[4.3 11.5 mm]
Nobel Biocare
Nobel Speedy
Groovy RP
[4.0 13 mm]
Nobel Biocare
Nobel Speedy
Shorty WP
[6.0 7 mm]
Nobel Biocare
Nobel Perfect
(View A)
[4.3 10 mm]
Nobel Biocare
Nobel Perfect
(View B)
[4.3 10 mm]
Nobel Biocare
Immediate
Provisional Implant
Nobel Biocare
(Branemark)
Self-tapping
[3.75 mm]
Nobel Biocare
(Branemark)
Conical Self-tapping
[3.75 10 mm]
Nobel Biocare
(Branemark)
Branemark MK I
[3.75 10 mm]
Nobel Biocare
(Branemark)
Branemark MK I
[3.75 13 mm]
Figure 15
Figure 16
Figure 17
Figure 18
CBCT showing the anterior extension of the mental foramen (arrows) in the edentulous mandible. The CBCT-based
cross-sections near the midline demonstrate the lingual foramen which would be a blind sac that derives the
neurovascular bundle from the lingual nerve and artery
853
The surgical template so formed will have the prefabricated implant guides. A typical surgical guide that has
been fabricated using an existing denture is shown in the
Figure 17.
Figure 19
CBCT cross-sectional views (parasagittal views) of the left mandible showing the dumbbell-shaped anatomy and
the clear delineation of the mandibular canal and the mental foramen (arrow)
Figure 20
Another instance of preimplant CBCT scan showing the reformatted panoramic view and cross-sectional views of the
left molar area. Note the measurements that are directly digitally obtained from the sections
854
Figure 21
CBCT reformatted panoramic view and maxillary anterior cross-sections showing knife-edge shaped ridge
Figure 22
CBCT reformatted panoramic view and maxillary posterior cross-sections showing severely resorbed ridges bilaterally
855
Figure 23
Bone grafting completed in the maxilla bilaterally via sinus lift procedure as shown in the
panoramic reconstruction of CBCT and left cross-sectional views of the maxilla
Figure 24
A SimPlant study of a patient showing the disastrous outcome of the implant fixtures. Poor planning and
lack of preimplant imaging usually leads to such results. Note the displacement of an implant
into the left maxillary sinus. Courtesy: Barry E Zweig, DDS
bone healing around the fixture. Three-dimensional imaging is recommended only when complications arise from
the placement. A good example would be to evaluate the
nerve canal proximity if the patient is symptomatic for
nerve injury. In such situations, a limited volume CBCT
would be ideal to evaluate the area. Dental implantology
and implant imaging are rapidly expanding areas of dentistry. The information presented in this chapter should
provide some basic concepts regarding implant selection,
imaging and placement strategies for both doctoral and
graduate level dental students. However, the reader is
encouraged to follow the literature on the subject for a
more up-to-date knowledge.
857
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SECTION
Appendices
XIII
Appendix 1
Appendix 2
Appendix 3
Appendix 4
Appendix 5
Terminologies
Summary of Radiographic Pathology
Characteristics of Ideal Radiograph
Indications for Intraoral Radiography
Patient Position for Extraoral Radiography
861
867
871
872
873
859
Appendices.indd 859
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APPENDIX
Terminologies
Ravikiran Ongole
Abscess
Agenesis
organ.
Ageusia
Anosmia
862
Disorder, congenital It is the disorder which occurs during the development process (intrauterine life). The manifestation of which is seen either at birth or later in life.
Disorder, developmental It is the abnormality that occurs
during the formative stage of an organ or tissue.
Disorder, genetic It is a disorder caused by abnormality
in the gene or an abnormality in mutation.
Disorder, inherited It is the disorder, which is transmitted
through genes to the offspring of the next generation or to
subsequent generations.
Dose It is the amount of energy absorbed per unit of mass
at a site of interest. Expressed in units of rad or gray.
Dose equivalent (DE) Absorbed dose multiplied by certain modifying factors. The principle factor is the quality
factor (QF), which corrects absorbed dose for relative biological damage for quality factors for:
Beta, gamma and X-rays 1
Alpha 20
1 rad 1 rem for beta, gamma and X-ray.
Various types of radiation, expressed in units of rem or
sievert.
a.
b.
c.
Diagnosis, differential Identification of a particular disease by differentiating it from all other disease processes
that may have similar symptoms and signs.
Drug, first pass effect All drugs that are absorbed from
the intestine enter the hepatic portal vein and pass through
the liver before they are distributed systemically.
Appendix 1 Terminologies
Drug, half-life The period of time required for the concentration or amount of drug in the body to be reduced to
exactly one-half of a given concentration or amount.
Dysgeusia
Dysosmia
Dysphagia
lowing.
or
E WT X HT
Epistaxis
Glossodynia
Glossopyrosis
Granuloma Tumor-like mass of inflammatory tissue consisting of a central collection of macrophages, often with
multinucleated giant cells, surrounded by lymphocytes.
Granulomatous Pertaining to a well-defined area that
has developed as a reaction to the presence of living
organisms or a foreign body. The tissue consists primarily
of histiocytes.
Gray (Gy) The international unit of absorbed dose.
1 Gy 100 rad; 1 Gy 1 joule/kilogram (J/kg).
Hamartoma Abnormal proliferation of tissues of structures native to the part.
Hematoma Tumor-like mass produced by coagulation of
extravasated blood into tissues from ruptured blood vessels.
Hemiparesis
Hemiplegia
Kerma is a non-stochastic quantity and dEtr is the expectation value of the sum of the kinetic energies. The unit for
kerma is joule per kilogram (J/kg) and its special name is
gray (Gy).
Paraplegia
L dE/dl
Paresis
Paroxysmal
Appendix 1 Terminologies
It accounts for the different degrees of damage produced by equal doses of different radiations.
Example: Radiation weighing factor (Wr): for X-rays,
gamma rays, beta particles 1
Neutrons (range) 220; alpha particles 20.
Sarcoma It is a malignant tumor arising from connective
tissue.
Septicemia
Quadriplegic
Sievert (Sv) The special name for the SI unit of equivalent dose, effective dose, and operational dose quantities.
The unit is joule per kilogram (J kg1).
1 Sv 100 rem; 1 Sv 1 Gy Wr.
Sign Any change in the body or its function, which is perceptible to a trained observer and may indicate a disease.
Sinus It is a blind tract that connects a cavity lined by
granulation tissue to the epithelial surface. This lining
may subsequently become epithelialized.
Stochastic effect It is the sub-lethal changes in the DNA
of an individual cell and the heritable effects for which the
probability of an effect occurs, but not its severity. It is
regarded as a function of dose without threshold.
Swelling It is a non-specific term used to describe any
enlargement or protuberance in the body.
Symptom Any change in the body or its function, which
is perceptible to the patient and may indicate a disease.
Teratoma A true neoplasm made up of a number of different types of tissues, none of which is native to the area
in which it occurs.
Therapy, curative Treatment directed toward eradication
of one or more of the agencies etiologic to the patients
condition, e.g. use of antibiotics such as penicillin.
Therapy, palliative or symptomatic Treatment directed
only toward relief of the patients symptoms. The natural
course of the disease is unaffected.
Therapy, restorative Therapy directed at rapid restoration
of health, usually regardless of the nature of the original
disease; restorative therapy is most frequently given during
convalescence, e.g. vitamin supplements.
Therapy, substitutive or replacement Treatment directed
toward supplying a material normally present in the body,
but absent in a specific patient because of disease, injury,
congenital deficiencies, etc., e.g. use of hormones.
Therapy, supportive Treatment directed toward maintaining the patients physiological or functional integrity
until more definitive treatment can be carried out, or until
the patients recuperative powers function to obviate the
need for further treatment.
865
866
Uncontrolled area It is an area where access is neither controlled nor restricted. In an uncontrolled area the shielding
should reduce the exposure rate to 2.6 mC/kg/week.
Vesicle Elevated blisters containing clear fluid and less
than 1 cm in diameter, e.g. herpes simplex infection,
herpangina, herpes zoster.
Viremia Presence of viruses in blood.
White lesion Non-specific term used to describe any
area of the oral mucosa that on clinical examination
appears whiter than the surrounding tissues and is usually
raised, roughened or otherwise of a different texture than
the adjacent normal tissue.
APPENDIX
Summary of Radiographic
Pathology
Medha Babshet
Table 1
Periapical
Others
Periapical granuloma
Periapical cyst
Dentoalveolar abscess
Periapical scar
Periapical cemento-osseous
dysplasia (lytic stage)
Primordial cyst
Stafnes bone cyst
Neurilemmoma
Traumatic neuroma
Post-surgical maxillary
cyst
Multilocular
Associated with teeth
Ameloblastoma
Keratocystic odontogenic tumor (OKC)
Botryoid odontogenic cyst
Odontogenic myxoma
Glandular odontogenic cyst
Central odontogenic fibroma
867
Table 2
Generalized
Condensing osteitis
Idiopathic osteosclerosis
Garreys osteomyelitis
Hypercementosis
Periapical cemento-osseous dysplasia
Focal cemento-osseous dysplasia
Cemento-ossifying fibroma
Cementoblastoma
Complex odontoma
Table 3
Tori
Exostosis
Osteomas
Foreign bodies
Mucosal cysts of maxillary sinus
Ectopic calcification
Sialolith
Rhinolith
Calcified lymph node
Phlebolith
Arterial calcification
868
Periapical
Pericoronal
Chronic osteomyelitis
Osteoradionecrosis
Focal cemento-osseous dysplasia
Florid cemento-osseous dysplasia
Fibrous dysplasia
Pagets disease (intermediate stage)
Cemento-ossifying fibroma
Osteogenic sarcoma
Desmoplastic ameloblastoma
Osteoblatic metastatic carcinoma
Chondroma
Chondrosarcoma
Table 4
Jaw lesions
Cotton wool
Ground glass
Hyperparathyroidism
Fibrous dysplasia
Orange peel
Fibrous dysplasia
Honeycomb appearance
Ameloblastoma
Central hemangioma of bone
Odontogenic myxoma
Moth eaten
Driven snow
Codmans triangle
Cumulus cloud formation
Osteosarcoma
Garres osteomyelitis
Calcifying subperiosteal hematoma
Ewings sarcoma
Osteogenic sarcoma
Leukemia
Syphilis
Hypervitaminoses A
Caffeys disease
Metastatic neuroblastoma
Fracture callus
Osteosarcoma
Ewings sarcoma
Hemangioma (central)
Chondrosarcoma
Odontoma (rarely)
Burkitts lymphoma
Hair-on-end
Stepladder appearance
Thalassemia
Sickle cell anemia
Thalassemia
Pagets disease
Diffuse sclerosing osteomyelitis
Florid osseous dysplasia
Sclerotic cemental masses
Hemangioma
Aneurysmal bone cyst
Cherubism
Ameloblastoma
Giant cell lesion of hyperparathyroidism
Central giant cell granuloma
Odontogenic cysts like odontogenic keratocyst, follicular and residual
Arteriovenous malformation
Burkitts lymphoma
Chronic suppurative osteomyelitis
Lytic variety of osteosarcoma
Osteoradionecrosis
Osteosarcoma (lytic type)
(Contd.)
869
Table 4
Continued
Jaw lesions
Beaten silver
Punched out
Multiple myeloma
Histiocytosis X
Eosinophilic granuloma
Metastatic lesions
Hyperparathyroidism
Fibrous dysplasia
Ballooning expansion
Follicular cyst
Aneurysmal bone cyst
Osteopetrosis
Hyperparathyroidism
Pyknodysostosis
Multilocular cyst
Ameloblastoma
Heart-shaped radiolucency
Nasopalatine cyst
Pear-shaped radiolucency
Globulomaxillary cyst
Histiocytosis X
Squamous cell carcinoma of gingiva
PapillonLefevre syndrome
Advanced periodontitis
Cherubism
870
Dilaceration of roots
Shell teeth
Dentinogenesis imperfecta
Ghost-like teeth
Regional odontodysplasia
Joint mice
APPENDIX
Characteristics of
Ideal Radiograph
Sharpness
Sharpness is also referred to as detail, resolution or definition. It refers to the capability of X-rays to reproduce distinct outlines of an object or to reproduce the smallest
details of an object.
Intraoral periapical radiographs have better resolution
than panoramic radiograph and computed tomographic
scans. Resolution is described as line pairs per mm.
Density
Magnification
Contrast
Contrast is difference in the degree of blackness (densities)
between adjacent areas on a dental radiograph.
A high contrast radiograph is one with very dark and
very light areas. Such a radiograph is considered to exhibit
Distortion
Distortion is the variation in the true size and shape of the
object being recorded. It results from unequal magnification
of different parts of the object.
Characteristic Curve
Characteristic curve is a graphic plot of relationship
between film density and exposure. It is also called H
and D curve after Hunter and Driffield.
871
APPENDIX
872
Indications for
Intraoral Radiography
APPENDIX
Figure 1
Figure 2
Posteroanterior view
Figure 3
Figure 5
Posteroanterior mandible
Figure 4
Figure 6
874
30 occipitomental view
Waters view
Figure 7
Figure 9
Figure 8
Figure 10
875
Figure 11
Figure 13
Figure 12
876
Figure 14
Figure 15
Figure 16
877
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Index
A
ABCDE warning signs, 369
Abdominal ultrasonography, 397
Abfraction, 458, 464
Abrasion, 452, 460
Acanthomatous ameloblastoma, 335
Acanthosis nigricans, 227
Accessory cusps, 44
Acid-enzyme theory, 407
Acinic cell adenocarcinoma, 297
Ackermans tumor, 366
Acquired immune deficiency syndrome
(AIDS), 99
Acquired immunity, 590
Acquired melanocytic nevus, 348
Acrodermatitis enteropathica, 228
Acrodynia, 63
Acromegaly, 536
Acrosclerosis, 601
Actinomycosis, 86
Activated partial thromboplastin time, 498
Acute adrenal insufficiency, 547
Acute dental caries, 413
Acute intermittent porphyria, 642, 643
Acute leukemia, 492
Acute lymphocytic leukemia, 386, 492
Acute lymphonodular pharyngitis, 90
Acute monocytic leukemia, 492
Acute necrotizing ulcerative gingivitis
(ANUG), 204, 452
Acute pain, 115
Acute pseudomembranous candidiasis,
135, 154
Acute radiation syndrome, 699
Acute sinusitis, 105
Acute suppurative osteomyelitis, 433
Added filtration, 694
Addisons disease, 66, 546
Adenocarcinoma, 295, 297
Adenoid cystic carcinoma, 298
Adenomatoid odontogenic tumor, 338
Adherence and adhesions, 247
Adult periodontitis, 442
Aerodontalgia, 118
African Kaposis sarcoma, 377
Agammaglobulinemia, 456
Age estimation methods, 669
Agenesis, 266
Aggressive periodontitis, 442
Aglossia, 22
Agnathia, 30
Agranulocytic angina, 491
Agranulocytosis, 455
Ankyloglossia, 23
classification, 23
ALARA principle, 704, 728
AlbersSchnberg disease, 578
Alcohol, 139, 384
Alkaptonuria, 76
Allergic rhinitis, 506
Allergic salute, 507
Allergic shiners, 507
Allodynia, 111
Allowable occupational dose, 704
Amalgam tattoo, 63
Ameloblastic
carcinoma, 363
fibrodentinoma, 342
fibroma, 342
fibro-odontoma, 342
fibrosarcoma, 366
sarcoma, 366
Ameloblastoma, 33
Amelogenesis imperfecta, 53, 54, 77
Amine odor test, 632
Amino acids, 635
Amlodipine, 446
Amorphous calcium phosphate, 464
Amputation caries, 401
Amyloid, 642
Amyloidosis, 642
Anaphylaxis, 481
Anatomical variations, 842
Androgens, 445
Andy Gump facies, 255
Anemia, 456, 485
Aneurysmal bone cyst, 322
Angina pectoris, 478
Angiogenesis, 385, 401
Angiolipoma, 355
Angiosarcoma, 377
Angles classification of malocclusion, 255
Angular cheilitis (perleche), 155, 156, 487
Anhidrotic ectodermal dysplasia, 222
Ankyloglossia, 23
Ankylosis, 253
Ann Arbor classification, 495
Annual dose limits, 710
Anodontia, 45
Anorexia nervosa, 524
Anorexia, 287
Antemortem radiographs, 657
Anterior median lingual cyst, 323
Antidiuretic hormone, 538
Antihypertensive therapy, 477
Anti-mongoloid eyelids, 646
Antinuclear antibody (ANA), 599
Anti-Saccharomyces cerevisiae antibodies, 624
Antiseptic mouthrinses, 508
Antistreptolysin O titers, 480
Antoni type B cells, 356
Antral polyps, 318
Antrocystectomy, 330
AP axial projection, 752
APECED, 600
Aphthous stomatitis, 211, 591
Aphthous ulcers, 104, 210, 211, 520, 591
Apical periodontal cyst, see Periapical cyst
Aplastic anemia, 448
Apnea, 512
Apple-green birefringence, 642
Areca nut, 383, 463
Argyll Robertson pupil, 627
Argyria, 64
Aromatic hydrocarbons, 383
Arrested caries, 412
Arsenic, 63
Arteriovenous malformations, 359
Arthrography, 795796
Articular capsule, 239
Articular disk, 239, 240, 246
Articular eminence, 239
Artifacts, 820
Aryl hydrocarbon hydroxylase, 383
Aschers syndrome, 27
Aschoffs nodules, 480
Ascorbate, 639
Ascorbic acid, 499, 639
Ash leaf macules, 233
AshboeHansen sign, 212
Aspergillosis, 109, 207, 615
Aspiration biopsy, 393
Aspirin, 483, 500
Asteroid bodies, 290, 619
Asthma, 508, 510
Atherosclerosis, 478
Atrophic glossitis, 487
Atrophic lichen planus, 148, 150
Attached gingiva, 210, 312, 346
Attrition, 459, 670
885
Index
B
Bacille CalmetteGurin (BCG) vaccination,
610
Baclofen, 122
Bacterial pharyngitis, 508
Bacterial sialadenitis, 282
Baelzs disease, 283
Barodontalgia, 118
Barosinusitis, 119
Barrel-shaped rib cage, 577
Bartholins duct, 267
Basal cell adenoma, 293
Basal cell carcinoma, 367
Basal cell nevus syndrome, 342
Battles sign, 263
Bay cyst, 320
B-cell immunity, 590
Beam limiting devices, 706
Bean-shaped cyst, 347
Bean-shaped radiopacities, 646
Bearded infant appearance, 584
BeckwithWiedemann syndrome, 27
Beedis, 381, 382, 383
Behets disease or syndrome, 31, 216
Bejel, 626
Bells palsy, 529
Bence Jones proteins, 497
Benign chondroblastoma, 351
Benign lymphoepithelial lesion, 277, 591
Benign migratory glossitis, 24
Benign mucosal cysts, 317
Benign mucous membrane pemphigoid, 188,
212
Benign osteoblastoma, 351
Benzopyrene, 383
Benzydamine hydrochloride, 25, 401
Beriberi, 640
BernardSoulier syndrome, 500
Betel quid chewers, 463
Betel quid lichenoid lesion, 153
Bifid condyles, 33
Bifid tongue, 24
Bilaminar zone, 240
Bilirubin, 61
Biliverdin, 62
Biological indicators, 671
Bird face deformity, 255
Bismuth grip, 63
886
Bisphosphonates, 588
Bite mark procedures, 672
Black beard sign, 588
Black box, 522
Black hairy tongue, 18
Black tarry stools, 523
Blade of grass lesion, 588
Blank radiograph, 820
Blastomycosis, 107, 613
Bleeding time, 498
Bleomycin, 402
Blepharosis moniliformis, 646
BlochSulzberger syndrome, 231
Blood dyscrasias, 440
Blood pressure, 475
Blood studies, 397
Bloody crusted appearance, 191
Blue bloaters, 510
Blue colored sclera, 577
Blue dye, 394
Blue, purple or gray tinted sclera,
577
Blurred image, 817
Bone scanning, 788
Bohns nodules, 316
Bollingers granules, 88
Botryoid odontogenic cyst, 311
Botryomycosis, 88
Bouins solution, 219
Boundary lubrication, 247
Brachytherapy, 400
Bradykinesia, 531
Brazilian pemphigus, 596
Breathlessness, 475, 505
Bremsstrahlung radiation, 688, 689
BreschetGorham syndrome, 585
Brim sign, 588
Brittle bone disease, 576
Broad saddle nose, 644
Bronchodilators, 510
Bronchogenic carcinomas, 511
Bronze diabetes, 67
Brown tumors, 516, 543
Bruck syndrome, 578
Brueghels syndrome, 532
Brush biopsy, 395, 398
Bruxism, 459
Buccal space, 427, 429
Bulimia, 287
Bulimia-related sialadenosis, see Anorexia
Bulimia nervosa, 524
Bull neck appearance, 431
Bulla spreading sign, see AshboeHansen
sign
Bulldog jaw, 628
Bulls eye lesion, 190
Bullous impetigo, 84, 193
Bullous pemphigoid, 185, 212, 596
Burkitts lymphoma, 496
Burning feet, 514
Burning mouth sensation, 487
Burning mouth syndrome, 127
Burning tongue, 272
Burtonian line, 64
BusseBuschke disease, 109
Butterfly rash, 84, 598
C
Caf-au-lait macules, 536
pigmentations, 66
spots, 234, 576
Caffeys disease, 583
CaffeySilverman syndrome, 583
Calcifying epithelial odontogenic tumor, 337
Calcifying odontogenic cyst, 312, 339
Calcium, 477
channel blockers, 446
hydroxide, 463
oxide, 463
Calculus, 451
Cameron ulcers, 523
Canalicular adenoma, 293
Cancrum oris, see Noma
Candida leukoplakia, see Chronic hyperplastic
candidiasis
Candidiasis, 101, 140, 153
classification, 154
Canine space, 429
Canker sores, 591
Capillary fragility test, 498
Capillary malformations, 359
Caplans syndrome, 594
Capsulitis, 249
Carbamazepine, 122
Carbohydrates, 634
Carbon monoxide, 383, 531
Carbonic acid, 463
Carboxymethylcellulose, 401
Carcinogens, 384
Carcinoma ex ameloblastoma, 364
Carious lesions, 410, 425
Carotenoids, 637
Carotid sheaths, 393
Carpal tunnel syndrome, 537, 540
Cascading waterfall appearance, 58
Casein phosphopeptide, 464
Caseous necrosis, 608, 609
Cat-scratch disease, 89
Causalgia, 311
Caviar lesions, 16
Celiac disease, 53, 214
Cell mediated immunity, 157, 590
Cell rests of Serres, 305
Cemental tears, 452
Cementicles, 470
Cementifying fibroma, 571
Cementoblastoma, 343
Cemento-ossifying fibroma, 571
Cephalometric radiography, 725
Cervical burn out, 461
Cervical enamel extensions, 43
Cervical esophagus, 393
Cervical lymphadenopathy, 508
Cetuximab, 402
Chalky, ground glass, granular and salt and
pepper appearance of bone, 516
Chancre, 206, 612, 626
Characteristic radiation, 688, 689
Charcots joints, 627
ChediakHigashi syndrome, 453, 491
Cheilitis glandularis, 283
Cheilitis glandularis apostematosa, 283
Cheilitis granulomatosa, 289, 620
Index
Chelation, 406
Chemical burn, 135
Chemical indicator, 706
Chemo-osteonecrosis, 588
Chemotherapeutic agents, 401
Chemotherapy, 104, 401, 845
Cherubism, 580
Chest pain, 475, 505
Chicken pox, 178, 201
Chicken-wire appearance, 490
Chicken-wire pattern, 351
Chinese script writing, 575
Chlamydial infections, 631
Chloasma, 64
Chloasma gravidarum, 551
Chloromas, 493
Chloroquine, 64, 65
Cholesterol clefts, 320
Cholesterol crystals, 318, 326
Chondroma, 350
Chondrosarcomas, 261, 372
Christmas disease, 503
Christmas tree pattern, 226
ChristSiemensTouraine syndrome, see
Hypohidrolic ectodermal dysplasia
Chromogenic bacteria, 73
Chronic atrophic candidiasis, 155
Chronic bronchitis, 505, 509
Chronic dental caries, 413
Chronic diffuse sclerosing osteomyelitis, 437
Chronic fatigue syndrome, 90
Chronic focal sclerosing osteomyelitis, 436
Chronic myelocytic leukemia, 493
Chronic hyperplastic candidiasis, 154
Chronic irreversible pulpitis, 414, 418
Chronic leukemia, 493
Chronic lymphocytic leukemia, 493
Chronic lymphoid leukemia, 493
Chronic mucocutaneous candidiasis
(CMC), 156
Chronic obstructive airway disease, 509
Chronic obstructive pulmonary disease, 509
Chronic osteomyelitis with proliferative
periostitis, see Periostitis ossificans
Chronic pain, 115
Chronic periapical abscess, 415
Chronic renal failure, 513
Chronic sclerosing sialadenitis, 285
Chronic sinusitis, 105
Chronic suppurative osteomyelitis, 434
Chronological hypoplasias, 52
Chvostek sign, 544
Chylothorax, 586
Cicatricial pemphigoid, 186, 213, 596
Cigarettes, 139, 381, 383
Cigars, 381
Ciprofloxacin, 79
Circumferential caries, 401
Circumscribed morphea, 601
Circumvallate papillae, 16
Cisplatin, 402
Civatte, hyaline, cytoid bodies, 150
Classification and staging system for oral
leukoplakia, 138
Classification of ankyloglossia, 23
Classification system for Kaposis sarcoma, 377
D
Dane particle, 526
Dapsone, 611
Dark-field microscopic examination, 613
Darkroom infrastructure, 804
Dark spots, 819
Daylight processor, 808
De Mussets sign, 627
De novo ameloblastic carcinoma, 364
Dead tracts, 468
Debulking, 399
Decortication, 436
Deep cervical nodes, 12
Deep fascia, 426
Deep fungal infections, 613
Deep hemangiomas, 207
Degenerative joint diseases 358
Demirjians method, 250
DennieMorgan lines, 670
Dens evaginatus, 41
Dens in dente, 40
Dens invaginatus, 40, 41
Dental attrition, 670
Dental caries, 405
classification, 410
Dental computed tomography, 849
Dental floss, 417, 460
Dental fluorosis, 55, 79
Dental identification, 654
procedure, 655
Dental implants, 842845
forms, 842
historical perspectives, 842
indications, 844
patient management, 845
post-operative evaluation, 857
887
Index
888
E
Eagles syndrome, 122
Eating disorders, 524
Echelon lymph nodes, 393
Ectodermal dysplasia, 222
Edrophonium test, 604
EDTA, 463
EhlersDanlos syndrome, 223, 453, 499
EkmanLobstein syndrome, 576
Electrical insulating oil, 693
Electric pulp tester, 423
Electromagnetic spectrum, 687, 688
ELISA test, 102
Elongated image, 816
Ely cyst, 251
Emphysema, 509
Enamel caries, 414
Enamel hypoplasia, 511, 516
Enamel pearls, 42
Enameloma, 42
Enchondromas, 350
Endarteritis obliterans, 612
Endemic Kaposis sarcoma, see African
Kaposis sarcoma
Endobone, 579
Endocrine glands, 532
Endocrinopathic pigmentation, 546
Energy requirement, 633
Enostosis, 584
Entropion, 195
Enucleation, 330
Environmental enamel hypoplasia, 51
Enzymatic theory, 407
Eosinophilic granuloma, 647
Epidermal growth factor receptor, 402
Epidermoid cysts, 327
Epidermolysis bullosa (EB), 193
types, 193
Epidermolysis bullosa acquisita, 596
Epilepsy, 530
Epiloia, see Tuberous sclerosis complex
Epiphora, 390
Epistaxis, 499
Epithelial dysplasias, 143
Epithelial rests of Malassez, 318
Epithelioid macrophages, 605
Epithelioma contagiosum, see
Molluscum contagiosum
Epithelioma cuniculatum, 367
Epsilon-aminocaproic acid (EACA), 502
Epstein pearls, 316
EpsteinBarr virus (EBV), 385, 450
Epulis fissurata, 354
Erbs palsy, 584
Erosion, 461
Erosive lichen planus, 147
Eruption sequestrum, 47
Erysipelas, 84
Erythema multiforme, 177, 189
F
Faces pain scale, 116, 117
Facet, 459
Facial approximation, 667
Facial hirsutism, 643
Facial neuropathies, 628
Facies leprosa, 611
Factitial injury, 452
Falling snowflakes, 328
Familial fibrous dysplasia, see Cherubism
Fanconis anemia, 488
Fascia, 428, 429
Fascial spaces, 426
Fat soluble vitamin, 636
Index
G
GABHS, 507, 508
Gag reflex, 844
Galacturonic acid, 463
Galvanizing, 464
Gamma rays, 687
Gangrenous, see Noma
GAPO, 48
Gardners syndrome, 341, 349
Gargoylism, 644
Garrs osteomyelitis, 437
Gastroesophageal reflux disease (GERD), 519
Gastrointestinal syndrome, 700
Gate control theory, 114
Gaucher cells, 648
Gaucher disease, 648
Gemination, 36
Generalized seizures, 530
Geniculate neuralgia, 124
Genital wart, see Condyloma acuminatum
Geographic tongue, 24
German measles, 91
Gestant odontome, 40
Ghost cells, 365
Ghost images, 759
Ghost-like appearance, 60
Giant cell arteritis, 591
Giant cell fibroma, 360
Giant cell granuloma, 352
Giant cell reparative granuloma, 352
Giant cell tumors, 542
Giant osteoid osteoma, 352
Gigantiform cementoma, 570
Gigantism, 656
Gilchrist disease, 107, 207
Gingival cyst of infants, 305
Gingival diseases modified by
medications, 440
Gingival hyperplasia, 483, 644
Gingival recession, 425
Ginglymodiarthroidal joints, 239
Glanders like disease, 85
H
HaileyHailey disease, 229
Hair-on-end radiographic appearance,
489, 490
Hairy leukoplakia, 102, 516
Hairy tongue, 18
Half value layer, 694
Halitosis, 561
Hallopeau type pemphigus vegetans, 596
Halo-shadow, 438
Hand, foot and mouth (HFM) disease,
179, 204
HandSchullerChristian disease, 647
Hangar-Rose skin test, 89
Hanging teeth, 390
Hansens disease, 610
Heart failure, 482
Heartburn, 519
Heat test, 423
Hebra nose, 88
889
Index
890
I
Ice pack test, 604
Icterus, see Jaundice
Idiopathic hypertension, see Hypertension
Idiopathic osteolysis, 586
Idiopathic osteosclerosis, 584
Idiopathic thrombocytopenic
purpura, 500
IgA, 195, 409
IgG, 409
Image guided radiotherapy (IGRT), 400
Index
J
JadassohnLewandowsky syndrome, 225
JaffeLichtenstein syndrome, 574
Jaundice, 68, 525
Jaw resection, 659
Jewels sign, 195
Jigsaw puzzle pattern, 588
JodBasedow phenomenon, 540
Joint mice, 251
Jugulo-digastric lymph nodes, 393
Junctional epidermolysis bullosa, 194
Junctional nevus, 348
Juvenile idiopathic arthritis, 251
Juvenile ossifying fibroma, 348, 572
Juxta articular chondromas, 350
K
Kaposis sarcoma, 69, 376
classification system, 377
Kassowitzs law, 628
Kawasaki disease, 232
Keratin pearls, 399
Keratoacanthoma, 347
Keratoconjunctivitis sicca, 270
Keratocystic odontogenic tumor, 304,
307, 339
Khaini, 381
Kidney shaped cyst, 316
Kilovoltage, 709
Kissing disease, see Infectious mononucleosis
Klestadts cyst, 315
Klinefelter syndrome, 326
KlippelTrenaunay syndrome, 358
Knurled effect, 814
Koebners phenomenon, 147
Koenens tumors, 233
Kohlschutter-Tonz syndrome, 56
Koiloncychia, 486
Kopliks spots, 91
Kuttner tumor, see Chronic sclerosing sialadenitis
Kvaals radiographic method, 672
Kveim test, 620
Kwashiorkor, 635
L
Labial salivary gland biopsy, 275
Lacrimal sac, 390
Lacrimo-auriculo-dento-digital (LADD)
syndrome, 266
Lagophthalmos, 610
Lamina dura, 825
Langhans type giant cells, 605
Latent bone cyst, 323
Latent image formation, 802
steps, 802
Latent syphilis, 627
Lateral oblique view, 750
Lateral periodontal cyst, 311
Lateral pharyngeal space, 430
Lateral pterygoid muscle, 242
Lateral radicular cyst, 319
Lazy leukocyte syndrome, 453, 456
Lead aprons, 709, 711, 715
Left shift, 492
Legionnaires disease (legionellosis), 504
Leiomyoma, 357
Leiomyosarcoma, 377
Lemierres syndrome, 90
Leongs premolar, see Dens evaginatus
Leontiasis ossea, 31
Lepra cells, 611
Lepromatous leprosy, 610, 611
Lepromin test, 611
Leprosy, 610
LettererSiwe disease, 648
Leucovorin, 402
Leukemia, 447, 455
Leukemia associated gingivitis, 440, 447
Leukemoid reaction, 492
Leukocyte disorders, 455
Leukocytosis, 492
Leukoedema, 13
Leukopenia, 491
Leukoplakia, 137, 225
classification and staging system, 138
clinical features, 140
medical management, 144
Leutic glossitis, 628
Levines ionic see-saw theory, 407
Levines sign, 478
LevyHollister syndrome, 266
Lichen planopilaris, 147
Lichen planus, 172, 189, 218
Lichenoid drug reaction, 152
Lichenoid mucosal reactions, 521
Lichenoid stomatitis, 483
Light spots, 819
Lincolns highway, 430
Lincolns sign, 588
Linea alba buccalis, 134
Linear en coup de sabre, 601
Linear accelerators, 400
Linear enamel caries, 411
Linear gingival erythema (LGE), 448
Linear IgA bullous dermatosis, 195
Linear IgA disease, 195
Lines of Zahn, 16
Lingual frenum, 626
Lingual pits 26
Lingual thyroid, 25
Lingual tonsils, 12
Lingual varices, 15, 69
Lip print, 678
Lipids, 635
Lipoid proteinosis, 646
Lipoma, 355
Liposarcoma, 373
Lipschtz bodies, 240
Lipstick sign, 268
Liquid crystal thermography (LCT), 796
Liver disease, 68, 503, 525
functions, 525
Liver metastasis, 397
Localized microdontia, 35
Lockjaw, 85
Lfgren syndrome, 619
Log wheel rigidity, 531
Loose bodies, see Joint mice
Loss of taste, 401
Lowenstein tumor, 367
Lower respiratory tract infections, 504, 508
LT ratio, 396
Ludwigs angina, 428
Lues, 626
Lues maligna, 207, 627
Luminescence, 713
Lumpy jaw, 86
Lupus erythematosus (LE), 170, 600
Luxation, 779
Lyells syndrome, 189
Lymph nodes, 495
Lymphatic malformations, 359
Lymphatics, 392
Lymphoid nodules, 595
Lymphomas, 495
Lymphoproliferative syndrome, 491
Lyonization effect/Lyon hypothesis, 54
Lysosomal storage diseases, 641
Lysosomes, 641
M
Macqueen-dell technique, 751
Macrodontia, 35
Macroglossia, 22
Macrognathia, 30
Macrophages, 605
Maculopapular lesions, 627
Maculopapular rash, 612
MAGIC syndrome, 215
Magnetic resonance imaging (MRI), 781
advantages, 787
common artifacts, 787
due to motion and use of
denture, 787
flow effect and movement
artifacts, 787
contrast agents, 786
disadvantages, 787
historical perspective, 781
image interpretation, 787
methods for obtaining spatial
resolution, 786
principles, 781
MR scanner, 781
nuclear magnetic dipole moment, 781
proton magnetization, 782
relaxation, 783
resonance, 783
spin-echo phenomenon, 784
uses, 787
Maillard pigments, 79
891
Index
892
N
Nanocomplex, 464
Nasal obstruction, 390
Nasopalatine duct cyst, 314
Nasopharyngeal cysts, 323
Natal and neonatal teeth, 46
Natal teeth, 46
Neck dissection, 399
Necrotic pulp, 466
Necrotizing fasciitis, 428
Necrotizing sialometaplasia, 215, 284
Necrotizing stomatitis, see Noma
Neisseria gonorrhoeae, 254
Neonatal line, 52
Neonatal lupus erythematosus, 598
Neonatal teeth, 46
Neonatal tetanus, 86
Neospinothalamic tract, 113
Nervus intermedius neuralgia, 124
Neuralgia, 120
Neurofibroma, 356
Neurofibrosarcoma, 378, 379
Neurogenic sarcoma, see Neurofibrosarcoma
Neurohypophysis, 538
Neuromelanin, 18
Neurosyphilis, 628
Neurovascular syndrome, 700
Neutropenia, 455, 491
Nevoid basal cell carcinoma syndrome
(NBCCS), 304
Nevus cells, 348
Niacin deficiency, 455
Nicotinamide adenine dinucleotide phosphate
(NADPH) oxidase, 384
Nicotine, 382
Nicotine stomatitis, 136
NiemannPick disease, 649
Nifedipine, 447, 483
Nikolskys sign, 182, 235
Nitrosamine, see Nicotine
Nitrosodiethanolamine, 382
Nitrosoproline, 382
Nociception, 111
Nociceptors, 11, 112
Noma, 87
Noma neonatorum, 87
Noma pudendi, 87
Non-Hodgkins lymphoma (NHL), 495
Non-homogeneous leukoplakia, 141
Non-lipid reticuloendothelioses, 647
Index
O
Obstructive sleep apnea syndrome, 512
Occipital neuralgia, 125
Occlusal enamel pearl, see Dens evaginatus
Occlusal radiographs, 395, 721
Occupational exposure, 703
Occupational hazards, 390
Odontecrexis, 119
Odontoameloblastoma, 336
Odontogenic carcinomas, 363
Odontogenic carcinosarcoma, 366
Odontogenic fibroma, 344
Odontogenic keratocyst, 307
Odontogenic myxomas, 344
Odontogenic sarcomas, 365
Odontogenic tumors, 332, 344
Olfactory reference syndrome, 561
Oligodontia, 45
Olympian row, 628
Omohyoid muscle, 399
Oncocytoma, 295
Oncogenes, 386
Opalescent brittle teeth, 577
Open apices method, 671
Open lock, see Dislocation
Open mouth Waters view, see Skull
radiography
OPG, 306, 320
Oral contraceptive, 447
Oral hairy leukoplakia, 100, 516
Oral melanoacanthoma, 347
Oral submucous fibrosis, 159
Oral warts, 346
Orofacial digital syndrome, 24
Orofacial granulomatosis, 289, 620
Orofacial pain, 115
Oromandibular dystonia, 532
Oromandibular limb hypogenesis syndrome, 22
Oropharyngeal candidiasis, 509
Oropharyngeal trichomonal infection, 631
Orthokeratinized odontogenic cyst, 307
Orthopnea, 475
Orthostatic hypotension, 563
OSMF, 159, 160, 161
Ossifying fibroma, 348, 571
Osteitis fibrosa generalisata, 543
Osteoarthritis, 251
Osteoarthrosis, 250
Osteoclast, 543, 578
Osteodentin, 640
Osteodystrophia deformans, 587
Osteogenesis imperfecta, 576
P
Paan, 381, 382
Pacemakers, 484
Pachyonychia congenita, 225
Pagets disease, 586
Palatal cancer, 590
Palatal rugae, 15, 663
Palatal torus, 19, 20
Paleospinothalamic tract, 113
Pancreatin, 511
Pancytopenia, 226, 488
Panhypopituitarism, 536
Pannus, 252
Panoramic radiograph, 759
disadvantages and limitations, 760
indications, 759
Panoramic radiology, 756
concepts, 756
technique and imaging principles, 756
working principle, 757
origin, 756
Pansinusitis, 105
Papillary cystadenoma lymphomatosum, 294
PapillonLefevre syndrome, 453
Paracoccidioidomycosis, 208
Parade ground fracture, 262
Paradental cyst, 320
Paramedian lip pits, 26
Paramolar, 46, 47
Paraneoplastic pemphigus, 184, 185, 596
Parapharyngeal space, 427
Parasitic cysts, 327
Parathyroid glands, 542
Paratracheal lymphadenopathy, 607
Parinaud oculoglandular syndrome, 89
Parkinsons disease, 278, 531
Parma projection, 751
Parotid enlargement, 290
Parotid papillae, 17
Paroxysmal nocturnal dyspnea, 475
Partial seizures, 530
Pastias lines, 82, 508
Patch test, 153
PatersonKelly syndrome, 487
Pathological fracture, 395, 396
Pathological wear, 464
Patient shielding, 711
Paul-Bunnell test, 90
Pediatric medicines, 462
Peg lateral, 35
PelEbstein fever, 495
Pellagra, see Niacin deficiency
Pelvic inflammatory disease, 630
Pemphigoid, 212, 596, 597
Pemphigus, 212, 596
Pemphigus erythematosus, 596
Pemphigus foliaceous, 181, 596
Pemphigus vegetans, 181, 596
Pemphigus vulgaris, 181, 596
Penny test, 811
Peptic ulcer disease, 523
Percussion test, 466
Perforations, 795
Periadenitis mucosa necrotica recurrents,
see Major aphthous ulcers
Periapical cemento-osseous dysplasia, 569
Periapical cyst, 318, 416
Periapical granuloma, 416, 419
Periapical pocket cyst, 320
Periodontal disease, 101, 440, 450, 592
Periodontal pain, 118
Periostitis ossificans, 437
Peripheral edema, 475
Peripheral giant cell granuloma, 352
Peripheral ossifying fibroma, 348
Pernicious anemia, 488
Personal protective equipment (PPE), 728
Personnel dosimeter, 713
Personnel shielding, 711
Petechiae, 69
Petit mal seizures, 530
Peutz-Jeghers syndrome, 66
PFAPA syndrome, 215
Phantom bone disease, 585
Pharyngitis, 507
Pharyngotonsillitis, 508
Phenytoin-induced gingival enlargement, 446
Pheochromocytoma, 549
Pheomelanin, 18
Phleboliths, 16
Phoenix abscess, 415
Phosphate, 488
Phosphopeptide, 464
Photoelectric effect, 696
Photographic effect, 713
Photopheresis, 184
Photostimulable phosphor plates, 733
Phycomycosis, 615
Pilocarpine, 288
Pink disease, 63
Pink form, 655
Pink panters, 510
Pink puffers, 510
Pink tooth, 466, 467
Pink tooth of Mummery, 80
Pinta, 626
Pintos ligament, 241
Pit and fissure caries, 410
Pituitary dwarfs, 536
Pityriasis rosea, 226
Plancks constant, 687
Plasmapheresis, 600
Platelet count, 500
Platelet disorders, 499
Platelets, 497
893
Index
Platyspondyly, 578
Pleomorphic adenoma, 291, 792
Pleomorphism, 398
Plumbism, 64
PlummerVinson syndrome, 487
Pocket dosimeter, 713
Podophyllin, 103, 630
Polonium, 382
Polyarthritides, 252
Polycyclic aromatic hydrocarbon (tars), 382
Polycythemia, 485
Polycythemia rubra vera, 485
Polyunsaturated fatty acids, 635
Pontiac fever, 512
Poorly differentiated squamous cell
carcinoma, 399
Popcorn calcifications, 578
Porphyria, 642
Porphyria cutanea tarda, 643
Portable handheld X-ray unit, 691
Position indicating device (PID), 694, 706, 709
Positive identification, 657, 678
Positron emission tomography, 294
Possible identification, 657, 678
Posterior auricular nodes, 91
Posterior cervical muscles, 245
Postmortem dental examination, 655
Postmortem identification, 655
Postmortem pink teeth, 661
Postmortem radiographs, 657
Postnasal drip, 105
Pregnancy, 445, 462, 550
Pregnancy gingivitis, 551
Pregnancy tumor, 445
Pregnancy-associated gingivitis, 445
Prenatal diagnosis of clefts, 28
Prevotella intermedia, 454
Prickle cells, 398, 399
Primary herpetic gingivostomatitis, 176, 200
Primary HPT, 542
Primary intraosseous squamous cell
carcinoma, 364
Primary hypertension, see Hypertension
Prinzmetals angina, 478
Probable identification, 657
Probe, 394, 395
Processing solutions, 810, 811
composition, 810
developer solution, 810
fixing solution, 810
test to determine the quality, 811
Progesterone, 550
Proliferative verrucous leukoplakia, 142
Proptosis, 390
Prostaglandins, 112
Protective co-contraction, 256
Protein energy malnutrition (PEM), 635
Proteins, 635
Proteolysis-chelation theory, 406
Proteolytic theory, 406
Prothrombin time, 498
Proton pump inhibitors, 269, 523
Protostylid, 44, 45
Protruded tongue, 662
Proximal surface caries, 417
Psammomatoid ossicles, 573
894
Pseudo macroglossia, 22
Pseudomicrodontia, 35
Psoriasis, 165, 221
Psychosomatic factors, 455
Pterygomandibular space, 427, 430
Ptyalism, 63, 278
Puberty-associated gingivitis, 445
Pulmonary tuberculosis, 608
Pulp calcifications, 468
Pulp canals, 42, 459
Pulp hyperemia, see Focal reversible pulpitis
Pulpal pain, 116
Pulse oximetry, 424
Pulse rate, 476
Pulse rhythm, 476
Punch biopsy, 398
Punched-out radiolucencies, 497
Purified protein derivative, 609
Pyoderma gangrenosum, 520
Pyogenic granuloma, 353
Pyostomatitis vegetans, 623
Q
Qualitative disorders, 491
Qualitative neutrophil defects, 488
Quantitative defects, 485
R
Rabbit fever, 83
Racial pigmentation, 17
Radiation, 701
sources, 701
cosmic sources, 702
natural radiation, 702
terrestrial sources, 702
Radiation biology, 690, 697
deterministic effects, 699
of total body radiation, 699
on tissues and organs, 699
effects on living systems, 697
bystander effect, 699
cell cycle effects, 698
direct effect, 697
effects in the developing embryo
and fetus, 700
on oral tissues, 700
film exposure and processing, 714
stochastic effects, 699
Radiation caries, 401, 412, 426, 701
types, 701
Radiation dose, 399
Radiation hazards, 685
Radiation-induced cancer, 700
Radiation-induced heritable diseases, 700
Radiation monitoring devices, 713
Radiation physics, 687
fundamentals, 687
electromagnetic radiation, 687
ionizing and non-ionizing
radiation, 688
particulate radiation, 687
production of X-rays, 688
radiation, 687
Radiation protection survey, 711
Radiation safety and protection, 701, 728
historical events in, 701
Index
S
Sabourauds agar, 108, 109, 157
Saddle nose, 617, 628
Safe light, 805
Saliva, 267
Saliva swab, 676
Salivary calculi, 280
Salivary dysfunction, 75, 400
Salivary flow rate, 408
Salivary gland scans, 789
clinical applications, 789
procedure, 789
Sandpaper rash, 82
Sandpaper-like exanthema, 508
Sanfilippo syndrome, 645
Sarcoidosis, 289, 618
Saucerization, 436
Scarlet fever, 82
Schaumann bodies, 290, 619
Scheie syndrome, 644
Schirmers test, 271, 272
Schuller method, 748
Schwannoma, 356
Scintigraphy, 271, 496, 543
Scintillation, 713
Sclerotic cemental masses, 570
SCOFF questionnaire, 525
Scooped dentin, 464
Scratch test, 81
Screw-driver appearance, 52
Screwdriver edge-shaped central incisors, 628
Screwdriver-shaped incisors, 613
Screw-type implants, 851
radiographic appearance, 851
Scrofula, 608
Scrotal tongue, 13
Scurvy, 499
Secondary aldosteronism, 549
Secondary dentin, 468
Secondary hypertension, 476
Secondary pulmonary tuberculosis, 607, 608
Selenium, 144, 385
Self-developing film, 808
Self-healing carcinoma, 347
SenearUsher syndrome, 596
Sentinel node, 394
Septic arthritis, 252
Sequelae of pulpitis, 413, 416
Sequestrated bone, 401
Sequestrectomy, 436
Serous non-secretory cyst, 317
Serum alkaline phosphatase levels, 575, 584
Serum bilirubin, 525, 526, 528
Sex hormones, 454
Sexually transmitted diseases, 625
Shagreen patch, 233
Shell teeth, 57
Shepherds crook deformity, 575
Shingles, 178, 201
Shovel-shaped incisors, 41
Sialadenitis, 281
Sialadenosis, 287
Sialography, 793, 795
contraindications, 795
indications, 793
injection of the contrast medium, 794
phases, 794
procedure, 793
armamentarium, 793
preimaging assessment, 793
preimaging instructions to the
patient, 793
technique, 793
Sialolithiasis, 280
Sialometry, 274, 290
Sialorrhea, 278
Sialosis, 287
Sicca complex, 277
Sicca syndrome, 271
Sickle cell anemia, 489
Sickle cell trait, 489
Siga, 409, 462
895
Index
896
T
Tabes dorsalis, 612, 627
Talon cusp, 39, 40
Tam OShanter skull, 578
Tanakas ligament, 241
Target or double halo appearance, 522
Target lesion, 190
Tars, 382383
Taurodontism, 42, 43
T-cell immunity, 590
Technetium scan, 394
Teletherapy, 400
Telomeres, 225, 385
Temporal space, 429
Temporalis muscle, 241
Temporomandibular joint (TMJ), 239,
241, 243, 593, 840
arterial supply, 243
examination, 243
radiography, 751
sensory innervation, 243
transcranial view, 751
transorbital view, 752
transpharyngeal view, 751
venous drainage, 243
Temporomandibular joint imaging, 849
Teratoid cysts, 327
Tertiary syphilis, 627, 628
Test cavity, 422
Tetanolysin, 85
Tetanospasmin, 85, 86
Tetanus, 85
Tetracycline, 78, 444
Thalassemia, 490
Thermal burns, 135
Thermography, 796
indications, 797
patient preparation, 797
procedural requirements, 797
techniques, 796
Thermoluminescent dosimetry (TLD)
badges, 714
Thiamin deficiency, 455
Thinning of articular disk, 246
Thiocyanate, 383
Third molars in age estimation, 671
Thistle-tube-shaped pulp chamber, 59
Thorny radiation, 585
Thrombocytopenia, 488, 500
Thrombocytopenic purpura, 456, 500
Thrombotic thrombocytopenic purpura, 500
Thyroid collars, 709, 711, 715
Thyroid gland, 538, 539, 542
Thyroid storm, 541
Thyrotoxic crisis, 541
Thyrotoxicosis, 540, 591
Thyroxin, 538, 540
Tic douloureux, see Trigeminal neuralgia
Tissue biopsy, 395, 614
TNM staging, 391
Tobacco, 138, 381
Toluidine blue staining, 142, 397
U
Ugly duckling sign, 369
Ulcerative colitis, 214, 520
Ulceronodular disease, 207, 627
Ultrasonography, 789792
Unicystic ameloblastoma, 336
Index
B1-thiamine, 640
B2-riboflavin, 640
C, 455, 639
D, 455, 637
E, 145, 455
K, 502, 638
Vitamin K deficiency, 503
Volatile sulfur compounds, 444
Von Ebners glands, 17
Von Recklinghausen disease of bone, 542
Von Willebrand factor, 497
Von Willebrands disease, 503
Vrolik syndrome, 576
W
V
Vanishing bone disease, 585
Varicella zoster infection, 178, 201
Variegate porphyria, 643
Vascular disorders, 474, 476, 498, 499
Vascular malformations, 68, 357, 358
Velscope and Vizilite plus, 395
Venereal disease research laboratory (VDRL)
tests, 613, 628
Venereal diseases, 625
Venereal wart, see Condyloma acuminatum
Verapamil, 446
Verocay bodies, 356
Verruca vulgaris, 346
Verrucous carcinoma, 366367
Verrucous hyperplasia, 346
Verrucous leukoplakia, 138, 141
Vertical angulation, 740
Vietnamese time-bomb, 85
Viral pharyngitis, 507
Visual analog scale (VAS), 116
Vital signs, 482, 550
Vital staining, 395, 397
Vitality tests, 468, 522
Vitamin(s), 636641
A (retinol), 636
B complex, 455, 640
Waddling, 587
Wallerian degeneration, 529
Wall mounted intraoral radiographic unit,
690
Warfarin, 484, 504
WarthinStarry method, 89, 613
Warthins tumor, 294, 295
Water brash, 278, 519
Water-clear cells, 310
Water lily sign, 328
Water soluble vitamins, 636, 639
Waters view, see Skull radiography
WBC disorders, 210
Weber and Fechners law, 111
Webers glands, 267
Weeping lubrication, 247
Wegeners granulomatosis, 617, 618
Well-differentiated squamous cell
carcinoma, 398
Wet beriberi, 640
Whartons duct, 267, 280
Wheezing, 505
Whiff test, 631
White blood cells, 490
White coat hypertension, 477
White lesion, 133
White sponge nevus, 166
X
Xeroderma pigmentosum, 226
Xerophthalmia, 271, 636
Xerosis, 636
Xerostomia, 267270, 700
X-rays, 685, 687, 689
properties, 689
publications, 685
X-ray beam angulation and
alignment, 727
X-ray equipment, 706, 710, 712
X-ray photons, 695
X-ray tube, 692
schematic diagram, 692
Xylitol, 276, 634
Y
Yaws, 612, 626
Yellow form, 657
YunisVaron syndrome, 48
Z
Zahn lines, 16
ZiehlNeelsen stain, 606, 609
Zinc sulfate, 229, 401
ZinsserEngman-Cole syndrome, 225
ZollingerEllison syndrome, 523
Zoster ophthalmicus, 179, 235
Zoster sine herpete, 124, 179
Zygomatic bone, 832
Zygomatic process, 832
Zygomycosis, 615
897