DEVELOPMENTAL DISTURBANCES OF THE JAW Agnathia Micrognathia Macrognathia Dental Arch Anomalies: Malocclusion

DEVELOPMENTAL DISTURBANCES OF THE LIPS AND PALATE A. B. C. D. E. Labial Pits Cleft Lip and Palate Cheilitis Glandularis Cheilitis Granulomatosa Double Lip

DEVELOPMENTAL DISTURBANCES OF THE TONGUE

Microglossia and Macroglossia Ankyloglossia Scrotal Tongue (Fissured) Lingual Varicosities Lingual Tonsil

Psoriasiform Lesions: Benign Migratory Glossitis (Geographic Tongue, Erythema Migrans, Reiter's Syndrome)
Median Rhomboid Glossitis Fordyce Granules (Ectopic sebaceous glands) Ectodermal dysplasia

Agnathia:

A developmental defect, which is characterized by partial or complete absence of the maxilla or mandible. It is a suppressed gene expression with variation in clinical severity. It is not uncommon for the ear to be involved

Micrognathia:
A small mouth seen in association with: Pierre-Robin syndrome, Scleroderma, Infection or trauma to the temporomandibular joint during fetal development and congential heart disease Treatment: Reconstructive surgery.

Pierre Robin Sequence

Congenital- possibly genetic 10% to 20% if first degree relative affected Micrognathic Mandible Cleft Palate Posterior displacement of tongue Lack of support of tongue musculature Airway obstruction Treatment Plastic Surgery

Dental Arch Anomalies:

Malocclusion occurs as a result of discrepancy in jaw bone and tooth size, with offspring following familial hereditary patterns from parents. According to Angle's classification, 30% of the population will fit into a Class II or Class III type of malocclusion. Treatment: Orthodontic therapy/surgery.

Congenital lip pits are hereditary developmental defects which are found, clinically, as unilateral or bilateral depressions most commonly located on the lower lip vermilion border. Sometimes encountered with Von de Woude Syndrome which is associated with pits of lower lip, cleft lip/palate. Treatment: None.

Labial Pits:

Commissural Lip Pit

Paramedial Bilateral Lip Pits

Cleft Lip and Palate

Etiology and Pathogenesis: Cleft lip and palate account for approximately 50% of all cases; isolated cleft lip and isolated cleft palate each occur in about 25% of cases. The incidence is 1-in-700 to 1,000 births, with variable racial predilection. Cleft lip with or without cleft palate is more common in males. The multifactorial inheritance implies that many contributory genes interact with the environment and collectively determine whether a threshold of abnormality is breached, resulting in a defect in the

Cleft Lip

Cleft lip, generally, occurs at about the 6th to 7th week in utero, as a result of failure of the epithelial groove, between the medial and lateral nasal processes to be penetrated by mesodermal cells. Clinically, the Veau System of classification for cleft lip and palate is widely used. There are four (4) major categories with emphasis on degree of clefting. Cleft lip may vary from a pit or small notch in the vermilion border to complete cleft extending into the floor of the nose. Class I cleft lip is a unilateral notching of the vermilion border that does not extend into the lip. Class II cleft lip involves the body of the lip, but does not involve the floor of the nose. Class III cleft lip are unilateral extending through the lip into the floor of the nose. Any bilateral cleft lip is classified as Class IV.

Cleft Lip

Cleft Lip

Bilateral Cleft of Upper Lip

Cleft Palate

Cleft palate is a result of epithelial breakdown about the eight week of embryonic development, with ingrowth failure of mesodermal cells and lack of lateral palatal segment fusion. Clinically, the Veau System of classification for cleft lip and palate is widely used. There are four (4) major categories with emphasis on degree of clefting Clefting of the palate limited to the soft palate is Class I. Class II extends no further than the incisive foramen. Class III involve complete unilateral clefts extending from the uvula to the incisive canal and the alveolar process. Class IV clefts of the palate are bilateral complete clefts involving soft and hard palate on both sides of the premaxilla, leaving it free and mobile.

Cleft Palate

Cleft Palate communicating with the nose

Bifid Uvula

Submucosal Palate Cleft Bifid Uvula

Complications of Cleft Palate

Clefts of the palate are often associated with uvulopharyngeal incompetence or eustachian tube dysfunction. Recurrent otitis media and hearing deficits are common complications. Palatal pharyngeal incompetence results from failure of the soft palate and pharyngeal walls to make contact during swallowing and speech thus preventing the necessary muscular seal between the nasal and oral pharynx. Speech is characterized by emission from the nose and has a hypernasal sound. Abnormalities of tooth number, size, morphology, calcification, and eruption are seen. The lateral incisor is most often involved. There is a high incidence of congenitally missing teeth, especially the maxillary lateral incisor.

Treatment

Treatment and prognosis depends on severity of clefting. Aesthetic considerations and hearing and speech deficits often result in developmental problems. Treatment is chronologically segmental and requires a multidisciplinary team including medical dental and surgical specialists. Cleft lip is repaired in early infancy, when the child weighs at least 10 pounds and has a hemoglobin of 10 mg/dl. Cheiloplasty is often required later in life. Closure of soft palate defects with sliding or pharyngeal flaps is usually done by age one (1).

Treatment

Palatal obturators may assist in feeding. Early audiologic and speech evaluation is recommended. Chronic otitis media is related to improper orientation of the eustachian tubes. Preventive dental services are very important to allow for future orthodontic therapy. Autogenous bone graft from the iliac crest can be used to re-establish maxillary arch continuity.

Double lip:

Characterized by a fold of excessive tissue on the inner mucosal aspect of lip. When lip is tensed, the double lip resembles "cupid's bow." Double lip cannot be seen when lips are at rest. Treatment: Re-constructive surgery. Asher Syndrome: double lip, blepharochalasis and nontoxic thyroid enlargement.

Double lip

Asher Syndrome
Asher Syndrome: double lip, blepharochalasis (recurring edema of the upper eyelids) and nontoxic thyroid enlargement (50%). Autosomal dominant inheritance

Fordyce Granules

Fordyce Granules (Fordyce's Spots): Etiology: Ectopic sebaceous glands. Clinical: Located classically on buccal mucosa, appearing as numerous ovoid discrete yellow spots. Also, present on tongue, palate, lip and gingiva. Histology: Sebaceous gland lobules which are not associated with hair follicles. It explains occurrence of sebaceous neoplasms orally. Treatment: None.

Fordyce Granules

Fordyce Granules

Microglossia and Macroglossia

Microglossia and Macroglossia are relatively an underdeveloped small tongue or an excessive large tongue. Macroglossia may be congenital or secondary to systemic conditions (i.e. Acromegaly, Hypothyroidism, Tumors, Amyloidosis, Sarcoidosis). Beckwith's Hypoglycemia Syndrome: Macroglossia, neonatal hypoglycemia, microcephaly, umbilical hernia, visceromegaly and giantism. Treatment: Re-constructive surgery.

Microglossia

Microglossia

Causes of Microglossia

Macroglossia

 Ankyloglossia:

Congenital defect with result from fusion of the tongue to the floor of the mouth, by a deficient lingual frenum. Consonants and diphthongs mostly affected. Treatment: Surgical excision.

Ankyloglossia

Lingual thyroid nodule:

Lingual thyroid nodule: Posterior to foramen caecum. May be only viable thyroid tissue. Associated with tightness in throat, may effect voice, breathing, and swallowing. Most arise in females during puberty, adolescence, pregnancy or menopause -- R/O adenoma or carcinoma. Treatment: Excision after confirmation of existence of thyroid tissue in neck via radioactive iodine uptake.

Lingual Thyroid

Lingual Thyroid

Scrotal Tongue (fissured):

Occurs as deep furrows and crevices on the dorsum. This condition is usually painless, unless inflammatory reaction to food. This is a component of the Melkersson-Rosenthal Syndrome. Treatment: None,. Brush daily with soft toothbrush.

Fissured (Scrotal Tongue)

Fissured Tongue

Lingual Varicosities:
Clinical presentation of engorged prominent blood vessels (veins) on the ventral tongue surface. It is considered a part of the aging process and cardiovascular disorders (Hypertension). Treatment: None. Refer for medical examination.

Lingual Varicosities

Lingual Tonsils

Lingual Tonsils are lymphoid aggregates located on the posterior lateral aspects of the tongue, which undergo inflammation and enlargement. Other lymphoid aggregates in the oral and para oral tissues undergo similar reactive hyperplasia; not to be mistaken for SCCa which will not regress with time. Treatment: Antibiotic if needed.

Hairy tongue

Hairy tongue: Hypertrophy of filiform papillae, with lack of normal desquamation. Etiology is unknown. Treatment: None, cleans daily with soft tooth-brush.

Hairy Tongue

Hairy Tongue

Hairy Tongue

Ectodermal dysplasia:

Ectodermal dysplasia: A X-linked recessive disorder, showing oligodontia (fewer teeth than normal), defective development of hair, nails, sweat glands. These patients, therefore, exhibit low tolerance to heat. Prognosis: Good. Treatment: None.

Ectodermal Dysplasia

Ectodermal Dysplasia