HEMANGIOMA

 "Mbah Dukun, where are you, I need your help, my baby is cursed", someone came to Mbah Dukun Bagong, the Indonesian Modern Shaman. "hmmm, Your baby is not cursed, but he get Hemangioma" explain Mbah Dukun. So what is Hemangioma?

DEFINITION

Hemangioma is an abnormal proliferation of blood vessels that can occur in any tissue containing blood vessels. So, the hemangioma can occur in the cutis, subcutis, muscle, liver, gastrointestinal tract, brain, lung, or bone. Until now. it is still a debate, whether the hemangioma is a tumor, hamartoma, or vascular malformations.

EPIDEMIOLOGY

The prevalence of hemangioma ± 1-3% in neonates and ± 10% in infants up to age 1 year. Location common hemangioma of the head and neck (60%), and about 20% it is the multiple lesions. Babies born prematurely is a risk factor that has been identified, particularly neonates with birth weight below 1500 grams. Incidence rate of femalethan male 3 to 1.
Complications of hemangiomas are more common in babies of women than men, and more common in whites. Most hemangiomas arise de novo without a family history (sporadic), but there are several studies that reported that hemangiomas associated with autosomal-dominant gene.
Pathogenesis
Until now, the pathogenesis of hemangiomas is still unknown. Although growth factors, hormonal, and mechanical influences in expected to cause abnormal proliferation in tissue hemangioma, but the main cause that lead to defects in hemangiogenesis remains unclear. And has not been proven to date about the genetic influence.
Vascularization of the skin begins to form on the 35th day of gestation, which continued until several months after birth. Vascular system maturation occurs in the 4th month after birth.
Angiogenic factors likely play an important role in proliferation and involution phase of hemangiomas. Rapid growth in hemangioma endothelial having similarities with capillary proliferation in tumors. Endothelial proliferation is influenced by angiogenic agents. Angiogenic works in two ways:
1. Directly affect vascular endothelial mitosis,
2. Indirectly affect macrophages, mast cells, and helper T cells.
Heparin is released macrophages stimulate endothelial cell migration and capillary growth. In addition to heparin alone act as an agent of angiogenesis. Effects of angiogenesis is inhibited by the presence of protamin, cartilage, and some corticosteroids.
Corticosteroid inhibition of this concept is applied to therapy in several types of hemangioma involution phase. Angioplastin, one internal fragment of plasminogen is a potent and specific inhibitor for endothelial proliferation.
Macrophages produce angiogenesis stimulators or inhibitors. In the proliferative phase, hemangiomas in tissue infiltration by macrophages and mast cells, whereas the phase of involution there is infiltration of monocytes. It is estimated that macrophage infiltration is affected by monocyte chemoattractant protein-1 (MCP-1), a glycoprotein which acts as a chemotaxis mediators. This substance is produced by vascular smooth muscle cells in the proliferative phase, but not generated by the involution phase of hemangiomas or vascular malformations. The presence of MCP-1 can down regulated by dexamethasone and interferon alpha. Interferon alpha proved to inhibit migration of endothelial chemotaxis induced by the stimulus. This gives the additional effect of interferon alpha in reducing the number and activity of macrophages. The evidence above describe the effects of dexamethasone and interferon alpha in the proliferative phase of hemangioma.

CLASSIFICATION

In 1982, John Mulliken and Glowacki Julie makes the classification of vascular anomalies that occur in the skin of children is based on histological lesions and behavioral biology. The two main groups: vascular malformation and Hemangioma.
Vascular malformations usually appear at birth, and will grow in line with the growth and development of children. Radiological, in these lesions there is no tissue parenchyma, and composed mostly by blood vessels. In Histological, Looks endothelial cells that have a slow turnover, Endothelial mature, normal number of mast cells, and a thin basal membrane. Vascular malformations are classified into type 'high flow' and 'low flow'. Included in the high flow is arterial malformations and arteriovenous malformations. While that includes the low flow of venous malformations, capillary, and lymphatic
Hemangiomas are generally not visible or faint at birth. Then will experience rapid growth phase that began around the age of 6 weeks and will continue until the age between 6-20 months. After that involution of hemangiomas will experience until the age between 5-7 years. Radiological, looks a lot lobuler parenchymal tissue and demarcated. Histologically, there are fetal epithelial type that have a rapid turnover, increased number of mast cells, and membrane basalisnya multilaminer

Based on morphology, hemangiomas are divided into hemangiomas localized, segmental, and multiple

Localized Hemangioma in Eye

.Localized Hemangioma , which is the most common type, has a strict limit, growing from a single focus, not didjumpai type linear or geometric growth.

Segmetal Hemangioma

 Segmental hemangioma growing like plaque, which appears on the skin of a specific territory, growing linear or geometric. This type is more frequently ulcerated, impaired growth and development, and dap [at the same time with visceral hemangiomas, and have a worse prognosis.

Multiple Hemangioma

Multiple hemangiomas, for example of this type of Neonatal hemangiomatosis, a multiple hemangioma of the skin with ketch-size small (2 mm cm -2). Often accompanied by hemangioma in the gastrointestinal, liver, brain, and lungs

Another classification divides the depth-based hemangiomas of the skin surface. Superficial or cutaneous hemangioma, which is 50-60% of all hemangiomas will be colored like a strawberry when mature. The deep subcutaneous hemangiomas or if its location deep enough it will look like flesh-colored growth. And if it is further to the superficial then it will look like bluish nodules and sometimes found telangaktesi or dilated veins in the surrounding skin. Included in this group of intramuscular hemangioma and skeletal. If there is a superficial hemangiomas (red) and induration found beneath it, then type this into the mixture or compound Hemangioma. Visceral hemangioma, hemangioma is located in internal organs such as liver, intestine, lung, brain

Mulliken at (1988) divided into 3 types of hemangiomas, the type of capillary, cavernous, and mixed. Capillary hemangiomas are the most common type, with a 1-1.5% incidence rate in infants. This type has a prominent clinical appearance of rounded, sometimes lobulated, and red. Histologic form of capillary vessels with thin walls, which are limited by a single layer of flattened endothelial or convex, and periendotel layer and reticular tissue. In general, a type of Low flow.
Cavernous hemangioma is a type of high flow and are histologically composed of channel-channel vascular dermis is irregular and its location in the deep. Cavernous vessels and sinusoids are separated by connective tissue tangled stroma.Penampilan is customarily clinical lesions with purplish-red hues on the surrounding skin.
Mixed type hemangioma composed of capillary and cavernous components. This type is more frequently found in the cavernous type of appea.


CLINICAL FEATURES

Clinical picture is the most important factor in upholding the diagnosis of hemangioma. In general, hemangiomas are not immediately apparent at birth but the first few weeks after birth. Several types of hemangioma can appear at birth as a vague lesion on the skin, which vary from a nevus until pale red macules that resemble bruises. Very rarely hemangiomas are already fully formed at birth

In the proliferative phase, Hemangiomas grow rapidly during the 6-8 first weeks after birth. Hemangioma located on the surface of the skin, the skin will stand out and light pink. However, when these lesions grow in the deeper layers of the dermis, subcutis, or muscle, the skin covering it can be colored blue, and few stand out, also happens dilated veins.
In the involution phase, hemangiomas reach peak proliferation at the end of the first year. After that, a hemangioma grows proportionally to the growth of infants.
The color of the light gradually turns into a faint. The skin began to pale, and the consistency of a soft tumor. This phase generally lasts until the child is aged 5-10 years. Hemangioma regression speed is not related to gender, location, size, and morphology. Involution period will expire at 5-year olds (50%), and at the age of 7 years (70%). Expiration of involution occurs at age 10-12 Years

EXAMINATION SUPPORT

Most hemangiomas easy at diagnosis without the need for investigation. However, deep hemangiomas or superficial lesions of dubious require imaging examination for confirmation of diagnosis and evaluation of the extension.

1. Radiography
Plain radiographs have limited utility in the diagnosis of hemangioma. The picture shown to be a shadow period isodens with muscle, when near the bone to give you a periosteal reaction
2. Ultrasonography (USG)
Ultrasonography (USG) is the nonivasif commonly used as a support for the diagnosis of hemangioma is deep and viscera.Ultrasound image of hemangiomas varies and is not specific for example on hepatic hemangioma, which gives an overview ekogenic.
3. CT-Scan
On CT scan without contrast, hemangiomas appear as hypodense lesions (low-density mass) and the presence of normal tissue surrounding encouragement
4. MRI
MRI with contrast has a high specificity and can distinguish hemangioma with other diseases
5. Angiography
Angiography is the standard to determine whether vascular disease included in the high flow or low flow

COMPLICATIONS
Bleeding is the most frequent complications compared with other complication. The cause is trauma from the outside or spontaneous rupture of blood vessel walls due to the thinness of the skin over the surface of the hemangioma, while the blood vessels beneath it continues to grow.
Ulcers can cause pain and increase the risk of infection, bleeding and cicatrix. Ulcer is the result of necrosis. Ulcers can also occur due to rupture. Large cavernosum hemangioma which can be followed by ulceration and secondary infection
Thrombocytopenia is a rare complication, usually on a large hemangioma. Formerly thought that thrombocytopenia is caused by a hyperactive spleen. It turned out later that in tissue hemangioma are experiencing sequestering platelet collection.
Hemangioma on the periorbital region greatly increases the risk of visual impairment and should be monitored more frequently. Amblyopia can be the result of blockage of the axis of vision (visual axis). Most complications that occur are hidden Astigmatism caused by pressure or pressure in the eyeball into the retrobulbar tumors. Hemangioma of the eyelids can interfere with normal visual development and should be treated in the first few months of life.
Very small percentage of hemangiomas can cause airway obstruction, heart failure.