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CASE PRESENTATION

HEMANGIOMA

Clinical Supervisor :
Dr. Herry Setya Yudha Utama, SpB, MHKes, FInaCS

Created by :
Harvien Bhayangkara
1102013124

CLINICAL CLERKSHIP OF SURGERY DEPARTEMENT


FACULTY OF MEDICINE YARSI UNIVERSITY
ARJAWINANGUN DISTRIC GENERAL HOSPITAL 2017
CHAPTER I
CASE PRESENTATION

I. IDENTITY
Date of hospital entry : December 5th 2017
Name : Mr. S
Age : 57 years old
Gender : Male
Occupation : Self employed
Addres : Kaliwedi
Religion : Muslim
Marital status : Married

II. ANAMNESIS
Main complaint
Patient complain of lump in left neck.

History of disease
Mr. S came to RSUD Arjawinangun with complain of a red lump in left neck since ±
1 months ago. The lump initially small, then getting bigger, and painless. Denied a history
of trauma.

History of past disease


Mr. S said he never had experienced the same symptoms before. The patient had no
history of surgery. Patient said he never had a history of hypertension or diabetes.

History of family disease


Mr. S said, there is no family members with the same disease as patient.

III. PHYSICAL EXAMINATION


a. Present Status
Genereal condition : Mild pain
Awareness : Composmentis
Blood pressure : 110/70 mmHg
Pulse : 80 x/minute
Breathing : 20 x/minute
Temperature : 36,7 oC

Head
Form : Normocephale, symmetrical
Hair : Black, no hair fall
Eye : Anemic conjungtivas (-/-), icteric schleras (-/-)

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Ear :Normal form, cerumen (-), intact thympany
membrane
Nose : Normal form, septum deviation (-), epitaxis(-/-)
Mouth : Normal
Neck
Enlargement of lymph nodes (-), trachea in the middle, lump found in left
neck
Thorax
Lungs – pulmonary
Inspection : The chest is symmetrical both left and right
Palpation : Fremitus vocale and tactile are symmetrical,
crepitation (-), tenderness (-), rebound
tenderness (-)
Percussion : Resonance sound in both lung fields
Auscultation : Vesicular and bronchial sound in the entire
lung field, ronchi (-/-), wheezing (-/-)
Abdomen
Inspection : Flat, symmetrical, mass (-)
Palpation : Tenderness (-), rebound tenderness (-)
Percussion : Tympani sound in four quadrants
Auscultation : Intestine sound (+)
Extremities
Upper
Muscle Tone : normal
Movement : active / active
Mass :-/-
Strenght :5/5
Oedema :-/-
Lower
Muscle tone : normal
Movement : active / active
Mass :-/-
Strenght :5/5
Oedema :-/-

b. Localized Status
Neck Region
Inspection : looks a red lump like strawberries.
Palpation : tenderness (+), uneven surface, mobile

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c. Laboratory Examination
Test Result Unit

Full Blood

Hemoglobin 13.0 gr/dl

Hematocrit 37.3 %

Leukocyte 6.82 10e3/μL

Trombocyte 241 10e3/μL

Erythrocyte 4.36 mm3

Erythrocyte Indexes

MCV 85.5 Fl

MCH 29.7 Pg

MCHC 34.8 g/dl

RDW 12.3 Fl

MPV 7.8 Fl

PDW 42.4 Fl

Counts (DIFF)

Eosinophil 14.6 %

Basophil 0.8 %

Segmen 57.4 %

lymphocytes 21.1 %

monocytes 4.3 %

Luc 1.9 %

Coagulation

Clotting time 4’ Minute

Bleeding time 2’ Minute

Immunology

HBsAg 0,04

Anti HIV non reactive

Chemical clinics

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Random blood 170 mg/dl
Glucose

IV. DIAGNOSIS
Hemangioma

V. DIFFERENTIAL DIAGNOSIS
Soft tissue tumor

VI. TREATMENT
Operative
Eksisi
Medicamentosa
Rl
Cefazolin
Ketorolac
Ranitidin

VII. PROGNOSIS
Ad vitam : dubia ad bonam
Ad sanationam : dubia ad bonam
Ad fungsionam : dubia ad bonam

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CHAPTER II
LITERATURE REVIEW

I. DEFINITION
Hemangiomas are benign tumors of blood vessels that proliferate from cells
vascular endothelium is followed by continuous involution causing abnormalities
is the result of anomalous development of the vascular plexus. Hemangioma often
occurs in infants ie 1.1% to 2.6% and children of 10% to 12%. These lesions are
more common in women than men with a 3: 1 ratio. Hemangioma lesions are
absent at birth. They manifest in the first month of life, indicating a rapid
proliferation phase and gradually involve in the form of a perfect lesion

II. EPIDEMIOLOGY
Hemangioma is the most common tumor on babies and children. Actually
hemangioma rarely cause death or disruption functions that are fatal, but often
cause stress and lack of confidence. 4-10% hemangiomas occur in Caucasian
infants with a prevalence of 3-5 times higher on baby girl. The disease is rare in
infants with dark skin. The incidence also increased in preterm babies, baby birth
weight <1200 grams, and on pregnancy in old age; it is associated with the
possibility of hypoxia as cause. The most common hemangioma lesion area is in
the craniofacial area (60%) followed the torso area (25%) and the extremities
(15%). A total of 80% of hemangiomas occur only in one area of the lesion, the
remaining 20% of lesions multiple. Multiple hemangioma usually accompanied by
hemangioma in the organs of the body especially the liver.

III. PATHOPHYSIOLOGY
Hemangiomas are endothelial tumors with a unique biologic behavior—they grow
rapidly, regress slowly, and never recur. The three stages in the life cycle of a
hemangioma, each characterized by a unique assemblage of biologic markers and
processes, are :
1. the proliferating phase (0 – 1 year of age)
2. the involuting phase (1 – 5 years of age)
3. the involuted phase (>5 years of age).

These stages are typically clinically apparent and can be distinguished


microscopically and immunohistochemically. In the proliferating phase, the
hemangioma is composed of plump, rapidly dividing endothelial cells that form
tightly packed sinusoidal channels. Even at this early stage, the endothelial cells
express phenotypic markers of mature endothelium, in addition to markers of
activated endothelium. Urinary markers of angiogenesis, such as basic fibroblast
growth factor and high molecular weight (MW) matrix metalloproteinases
(MMPs) are usually high in infants with proliferating hemangiomas and diminish
to normal levels during regression.

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In the involuting phase, there is decreasing endothelial proliferation, increasing
apoptosis, and the beginning of fibrofatty replacement of the hemangioma. The
net result is loss of volume of the tumor and increasing softness of the overlying
skin.

During the involuted phase, after regression is complete, all that remains are a few
tiny capillary-like feeding vessels and draining veins (some of which can be
abnormally large) surrounded by islands of fibrofatty tissue admixed with dense
collagen and reticular fibers. The endothelium lining these vessels is flat and
mature. Multilaminated basement membranes persist around the residual tiny
capillary-sized vessels.

IV. CLASSIFICATION
In general, experts classify hemangiomas into three types: Capillary hemangioma,
consisting of a child's capillary hemangioma (nevus vasculosus, strawberry
nevus), pyogenic granuloma, and cherry-spot. Cavernosum hemangioma and
Mixed hemangiomas.
Clinical features of hemangioma vary according to the type. Capillary
hemangioma (strawberry nevus) appears as patches of bright red, tense and
lobular form, demarcated, which can occur at various places on the body. In
contrast to capillary hemangioma, the lesions in cavernosum hemangiomas are not
strictly defined, may be erythematous macules or red to purple nodes. When
pressed deflate and will quickly bulge again when released.

Hemangioma Kapiler Hemangioma kavernosum

Clinical features of mixed hemangiomas are a combination of capillary and


cavernous type. The lesion is a soft, bluish-red tumor that in its development can
give a picture of keratotik and verukosa. Most are found in the inferior extremity
and are usually unilateral.

Basically hemangioma is divided into two namely capillary hemangioma and


hemangioma cavernosum. Capillary hemangioma (superficial hemangioma)
occurs in the upper skin, whereas cavernosum hemangioma occurs in deeper skin,
usually in the dermis and subcutis. In some cases these two types of hemangiomas
can occur together or are called mixed hemangiomas.
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1. Capillary hemangioma
a. Strawberry hemangioma (simplex hemangioma)
Capillary hemangioma is present at birth or a few days after birth. It is more
common in premature infants and will disappear within days or weeks (Hall,
2005). Looks as red spots are getting bigger and bigger. The color turns red, taut
and lobular, firmly bound, and hard on palpability. Spontaneous involution is
characterized by the colorfulness of color in the central region, the lesion
becoming less tense and more flat.
b. Pyogenic granuloma
This lesion results from frequent capillary proliferation following trauma, not by
inflammation, although often with secondary infection. Lesions are usually
solitary, can occur at any age, especially in children and most commonly in the
distal parts of the body that are often traumatized. First shaped erythematous
papules with rapid enlargement. Some lesions can reach a size of 1 cm and can be
stemmed, easily bleeding.

2. Hemangioma cavernosum
These lesions are not strictly defined, may be erythematous macules or red to
purple nodes. When pressed it will deflate and quickly swell again when released.
The lesion consists of a mature vascular element. The cavernous form seldom
involves spontaneous involution
Hemangioma cavernosum is sometimes present in deep tissue layers, in muscles
or internal organs.

3. Hemangioma Mixture
This type consists of a mixture of capillary type and cavernous type. The clinical
picture also consists of a description of both types. Mostly found in the inferior
extremity, usually unilateral, solitary, may occur from birth or childhood. The
lesion is a soft, bluish-red tumor which later on in its development can give a
picture of keratotik and verukosa.
The location of a mixed hemangioma in the superficial and deep skin layers, or
internal organs.

V. CLINICAL MANIFESTATION
Clinically the diagnosis of hemangioma is not difficult, especially in typical
lesions. The general clinical picture is the presence of red spots arising from birth
or some time after birth, growth relatively quickly within weeks or months.
Although the pathogenesis of hemangiomas is still a theory that needs to be
proved more clearly, the clinical symptoms of hemangiomas that are consistent
with their growth in each phase are as follows.
a. Phase Proliferation
In the early stages of infantile hemangioma it looks like a pale area of the
skin, macula erythema, telangiectasia or ecchymosis spots. Hemangioma
grows rapidly during the first 6 - 8 months of infancy. If the tumor has

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penetrated the superficial dermis, the skin becomes elevated, protruding,
and bright red. If the proliferating tumor is lower and subcutaneous, the
skin becomes slightly elevated and bluish in color.
b. Involution Phase
Hemangioma peaks before the age of one year, and after that growth is
proportional to the growth of children. The initial sign of the involution
phase is the loss of a bright red color that turns purple and is not bright, the
skin gradually blanches, an imperfect gray lining / coat is formed and the
tumor feels less tension. This phase continues until the child is 5-10 years
old. Usually the last color mark disappears at the age of 5-7 years.
c. Involuted Phase
50% regression occurs when the child is 5 years old and 70% at the age of 7
years, and continues until the child is 10-12 years old. Approximately 50%
of children will be cured and the former hemangioma resembles normal
skin, leaving the remaining cutaneous blemish, telangiectasis, crepelike
laxity, yellowish hypoelastic patches, scars (if present at the time of
proliferation phase), or fribrofatty residues. Even a fairly large cutaneous
hemangioma can experience total regression. In contrast, a flat superficial
dermal hemangioma can permanently change the skin texture.

Signs of Hemangioma
A. Capillary hemangioma
The signs of capillary hemangioma are:
• It occurs at birth and arises a few days after birth.
• Seemed as a red, glowing, lobular-shaped, tightly firm and firmly on the
palpation and getting bigger and bigger.
• The size and dlm vary greatly, some are subcutaneous in bluish color.
• Spontaneous involutions are characterized by the colorlessness of the central
region, the lesions becoming less tense and flatter.

B. Hemangioma cavernosum
• The lesion is not firmly defined, it can be an erythematous macula or red to
purple nodes
• When pressed deflate and will quickly swell again when released
• The lesion consists of a mature vascular element.
• Kevernosum forms are rare involutions

C. Hemangioma Mixture.
• Campuarn between capillary and cavernous species.
• Signs and symptoms consist of two types of hemangiomas.
• Most are found in inferior extremities, usually unilateral, solitudinal.
• May occur from birth / childhood
• Lasi is a soft, bluish-red tumor, which may in its development give a picture of
keratotik and verakosa

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Clinical symptoms
- Depending on the type:
a. Capillary hemangioma, "Port wine stain" no skin bumps.
b. "Strawberry mark", protruding like a mulberry fruit.
c. Hemangioma cavernosum, palpable warm and "compressible".

VI. SUPPORTING EXAMINATION


In almost all cases, the diagnosis can be exclusively enforced based on physical
examination and history of the disease. However, some types of hemangiomas
may be misinterpreted as vascular malformations or other types of tumors, so the
following investigations are required:
1. Ultrasound
Ultrasonography is useful for distinguishing hemangiomas from deep or
subcutaneous dermic structures, such as cysts or lymph nodes. Ultrasound
generally has limitations to evaluate the size and spread of hemangiomas. It is also
said that doppler ultrasound (2 kHz) can be used for high blood vessel density
(more than 5 blood vessels / m2) and changes in arterial peaks. The examination
using this tool is a sensitive and specific examination to recognize an infantile
hemangioma and differentiate it from other soft tissue masses.
2. MRI
MRI is the imaging modality of choice because it is able to know the location and
spread of both cutaneous hemangioma and extractutan. MRI may also help
distinguish hemangiomas that are proliferating from other high-vascular lesions
(eg arteriovenous malformations). Hemangioma in the involution phase provides
an overview as in low-vascular lesions (eg venous malformations.
3. CT Scan
In centers that do not have MRI facilities, can use CT scan although this method is
less able to describe the characteristics or blood flow. The use of contrast can help
distinguish hemangiomas from malignant diseases or other masses that resemble
hemangiomas.
4. Plain Photos
Examination of plain photos such as X-rays, can still be used to see if
hemangiomas interfere with the airway.
5. Skin biopsy
Biopsy is necessary if there is any doubt of diagnosis or to rule out a caposiform
haemangioendothelioma or malignant disease. Immunohistochemical examination
can help make the diagnosis. The complications that can occur in a biopsy are
bleeding.

VII. HISTOLOGICAL FINDING


The three stages of histologic appearance of hemangioma correlate with its
clinical course.

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During the proliferating phase, growth is rapid and frequent observation is needed
to document the growth pattern. Few indicators predict the eventual volume of a
particular hemangioma or forecast the timing and outcome of involution.
Typically, hemangiomas begin to plateau in growth by 10 to 12 months, although
some demonstrate growth stabilization earlier or later.
During the involuting phase, after 1 year of age, the growth of the hemangioma
slows, and, for a time, is commensurate to that of the child. The telltale signs of
regression appear. The skin begins to pale, typically beginning at the center of the
lesion and a patchy grayish discoloration becomes discernable. The hemangioma
is softer on palpation.
The involuting phase extends from 1 year until 5 to 7 years of age. The rate of
regression is unrelated to the appearance, depth, gender, site, or size of the
hemangioma. Typically, the final traces of color disappear by 5 to 7 years of age

VIII. Management
Management of hemangioma in general there are 2 ways :
a. Conservative way
On the natural course of hemangioma lesions will experience enlargement in the
first months, then reach maximum enlargement and after that spontaneous
regression occurs around the age of 12 months, the lesion continues to regress
until the age of 5 years. Superficial hemangiomas or capillary hemangiomas are
often not treated because of this type of hemangioma if left alone will disappear
by itself and skin looks normal.
Conservative ways take advantage of the natural process of the hemangioma.
Observation was performed to see hemangioma enlarged in the first months, then
reach maximum and regression until age 5 tahun.Hemangioma strawberry fruit
should be allowed to experience spontaneous regression. So although large,
conspicuous, and seemingly frightening, this type requires no action other than the
installation of elastic bandages with little emphasis continuously. This action
helps speed up the regression process.
b. Active way
Management is actively performed by surgery, corticosteroid therapy, or radiation.
Treatment with several surgical actions are excision, cryo and laser surgery.
Surgery is usually indicated in hemangiomas that have not been spontaneously
regressed for more than nine years, there are signs of overgrowth, for example,
within a few weeks the lesion becomes 3-4 times larger and in giant hemangiomas
with thrombocytopenia.
Hemangiomas that require active therapy, among others, are hemangiomas that
grow on vital organs, such as the eyes, ears, and throat; bleeding hemangiomas;
ulcerated hemangiomas; infected hemangiomas; hemangiomas that grow rapidly
and cause tissue deformity (abnormalities).
Excisional actions are rare because hemangiomas tend to have severe bleeding. To
reduce bleeding, excision is done in combination with sclerotherapy. Another
technique is by cryo surgery. The working principle of crypto surgery is to cause

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necrosis of the cells caused by freezing and softening of the cells. This method
was introduced in the 1940s by using liquid nitrogen applied to cotton.
Then in 1961, Copper introduced a closed system by spraying nitrogen liquid. The
use of lasers can also be used as a hemangioma therapy, but maintenance costs are
relatively expensive.

Management of hemangioma actively, among others:


1. Surgery
The indication of excision surgery in hemangiomas is incomplete involution.
The effect of cosmetics on the face, Hemangioma located in the region
periorbita, nose, mouth, upper respiratory tract, ear canal, and hemangioma
are life-threatening child. Clearly localized hemangiomas or pedunkular
hemangiomas, especially those with ulcerated and recurrent bleeding, may be
considered for excision in infancy. Bleeding during excision can usually be
controlled by cauterization. Debulking hemangioma in the upper eyelid is
required when the hemangioma causes astigmat and does not respond to drug
therapy. Excision with a CO2 laser can open airway obstructive because of
subglottis hemangioma.
Indication:
• There are signs of hemangioma growth that is too fast
• Hemangioma giant with thrombocytopenia.
• There is no spontaneous-spontaneous regression, eg no hemangioma
shrinkage after 6-7 years.
Lesions located on the face, neck, hands or vulva that grow quickly, may
require local excision to control it.
2. Corticosteroids
Criteria for treatment with corticosteroids are:
1. When it involves one of the vital structures.
2. Grow rapidly and hold cosmetic destruction.
3. Mechanically obstruct one of the orifices.
4. There is a lot of bleeding with or without thrombocytopenia.
5. Causes cardiovascular decompensation.
Corticosteroids used include prednisone, which results in hemangioma
regression, ie for strawberry, cavernosum, and mixed forms. The dose is
orally 20-30 mg daily for 2-3 weeks and slowly lowered, treatment duration
up to 3 months. Treatment with large doses of corticosteroids will sometimes
lead to regression in rapidly growing lesions.
Cavernous hemangiomas that grow on the eyelids and disturb the vision are
generally treated with an injection steroid that decreases the size of the lesion
rapidly, so that vision development can be normal. Cavernous hemangioma
or mixed hemangiomas may be treated when steroids are administered orally
and direct injection to the hemangioma.
Prolonged use of peroral corticosteroids can increase systemic infections,
blood pressure, diabetes, gastric irritation, and stunted growth.

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3. Radiation
Treatment with radiation nowadays has been abandoned because it is less
good in bone, also cause complications of malignancy that occurs in the long
term and can cause fibrosis in healthy skin.
The flameus type is tackled with excision, if necessary with a skin graft. Can
also be done pengajakan (tattoo) to disguise the color. The charge with
Argon lasers is generally quite satisfactory
For cavernosum hemangioma, the only therapeutic way is extripation. On a
wide range can be assisted with an angiography guide. Embolization helps to
reduce the tumor to facilitate surgical action. Sometimes infiltration infiltrate
deeply so that extensive surgery is required. This disorder can recur from the
residual hemangiomas that are difficult to achieve in surgery
In the neck area, hemagioma is usually cavernous type which is a soft bump
that deflates when pressed and swelled when released again. This tumor is
treated with ektripasi. When large, the need for arteriography or flebografi
perks.

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