Hemangiomas are the most common tumors of infancy. The incidence at birth is reported to be 1% to 3% rising to 10% to 12% by one year of age in Caucasian children. Although hemangiomas are most often not present at birth, approximately 80% are noted in the first month of life. Affected girls out number boys by a 3:1 ratio. There is also a reported increased incidence of hemangiomas in premature infants weighing less than 1000 grams. The head and neck regions make up less than 14% of the total body surface, yet approximately 60% of all hemangiomas occur there.
Hemangiomas are true neoplasms that grow by endothelial proliferation. Mast cells are abundant, up to 40 times above their normal level. In vitro studies suggest that mast cells influence the migration, but not the proliferation, of capillary endothelial cells. Details of the role mast cells play in the development and resolution of hemangiomas is currently the subject of intensive research.It is rare for hemangiomas to be fully mature at birth. The first sign is either an erythematous macular patch or a blanched area also known as "herald spot."
There are four phases in the life cycle of a hemangioma:
Proliverative Phase: The hemangioma grows very rapidly in both volume and size. The surface can become bright red and elevated. This phase can last up to one year.
Stationary Phase: The hemangioma remains bright red but becomes soft and compressible. This phase can last from one to two years.
Involution Phase: The hemangioma begins to diminish in size and its color begins to fade from bright red to dull red or pink. As this phase continues, gray-white areas begin to appear. This phase can last a variable period of time.
Involuted Phase: Involution is complete; however, the hemangioma usually does not completely disappear. Some fibro-fatty swelling often remains at the site of the hemangioma. There is a variable degree of skin atrophy, tiny wrinkles and few telangictatic vessels (star-burst of capillaries). This phase is usually completed by the time the child is nine years of age.
Most hemangiomas require no treatment. Total involution commonly occurs in 50% to 65% of hemangiomas by age five, 70% by seven years, and more than 90% by nine years. However, large or strategically located hemangiomas which are likely to cause functional impairment may need to be managed aggressively to avert or relieve associated problems. Among the tumors that should be considered for therapeutic intervention are hemangiomas obstructing the visual axis, airway, oropharynx, or auditory canals; large ulcerative tumors with secondary hemorrhage or infection; hemangiomas associated with Kasabach-Merrit syndrome and hemangiomas involving the head and neck, which may be a source of psychosocial trauma to affected children and are prone to long-term cosmetic deformities. Large facial and multiple cutaneous hemangiomas are frequently associated with visceral hemangiomas. These patients should be carefully monitored for symptoms related to the visceral lesions and considered for ultrasonography with Doppler studies.
In the absence of life-threatening complications dictating immediate intervention, and when treatment of a hemangioma is justified, systemic corticosteroids can be used to slow the growth of a hemangioma by interfering with their ability to stimulate new blood vessel growth. A small hemangioma may respond to an injection of the steroid but larger hemangiomas will likely require the use of oral steroids for their management. Slowing in the growth of the hemangioma along with softening of the lesion and fading of the color is often seen within the first one to two weeks following the intiation of steroid treatmeant.
Propanolol is a non selective beta-blockers that has been found to be benefical in managing large hemangiomas. It is a cardiac medication, and in 2008, it was incidentially discovered by a group of French physicans that when Propanolol was administered to children for treatment of a heart condition who also happened to have a large hemangioma, the hemangioma rapidly regressed, often within the first 24 hours following the initation of the drug. Currently there is not a general concensus amoung physicians regarding a treatment protocol using Propranolol to manage infantile hemangiomas. Several clinical trials are currently underway. I will keep you updated as the results are published.
An absolute indication for surgical treatment of hemangiomas include functional eyelid obstruction caused by a periocular hemangioma or an airway compromised by a subglottic hemangioma that has failed steroid therapy. Early debulking may also prevent continued derangement of tissues while awaiting involution of large, peduculated tumors which may distort facial landmarks or those prone to infection, ulceration or bleeding. Especially troublesome are the unique and fortunately rare cases of massive, rapidly enlarging hemangiomas that hinder important functions and endanger life. In such cases, combination therapy to include steroids, interferon alpha 2a and surgical intervention is often required.
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