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HEMANGIOMAS

Definition:
Infantile hemangiomas are benign vascular tumors that are characterized by a rapid growth phase followed by a spontaneous involution phase. Most infantile hemangiomas are found in the skin, and may also occur in the liver, spleen, gastrointestinal tract, airway and central nervous system.

Complications
As many as 24% of infants with hemangiomas will have complications and can even be life threatening if not treated timely and aggressively.

Eyes: Periorbital hemangiomas pose considerable risk to vision and should be carefully monitored with early involvement by an ophthalmologist to prevent permanent damage including blindness. Ears: Hemangiomas involving the ear may obstruct the external auditory canal, resulting in otitis externa or a temporary conductive hearing loss, which ultimately may cause speech delay. Airway: Airway hemangiomas can be life-threatening. Hemangiomas in the areas of the chin, lower lip, mandibular region and anterior neck pose the greatest risk. Subglottic hemangiomas can cause hoarseness and stridor (noisy breathing) with progression to respiratory failure, often occurring at 6 to 12 weeks of age. Permanent disfigurement: The location, rate of growth and depth within the skin of the hemangioma, the age of the child and presence of complications govern the likelihood that hemangiomas will cause permanent damage. Hemangiomas involving the lips, nose, ears and forehead, and those with deep ulceration, are at greatest risk for scarring. In these locations large or rapidly growing lesions will leave residual fibrofatty tissue, even after the hemangioma has involuted. Ulceration: This is the most frequent complication and typically occurs in deep, rapidly enlarging hemangiomas or in those located in trauma- or pressure-prone areas of the body. These can be excruciatingly painful and carry the risk of infection, hemorrhage and scarring. When hemorrhage occurs, blood loss usually is minimal and can be controlled with direct pressure. Hemangiomas of the anogenital region are particularly at risk for ulceration and infection and are accompanied by severe pain upon urination or defecation. Superinfection may lead to cellulitis, osteomyelitis, septicemia and, in some cases, has been lethal.

Treatment
Recently it was recognized that Propranolol is very effective for the treatment of hemangiomas. Propranolol is a medication that has been used for many years to treat high blood pressure.

Who is at risk?
A recent study showed 1/3 of the patients had a first-degree relative with a vascular anomaly and 12% had a firstdegree relative with a hemangioma. The mechanisms for the development of infantile hemangiomas are not completely understood and their pathogenesis is just now being elucidated. The current theory is that endothelial precursor cells in circulation respond to hypoxic stimuli and undergo vasculogenesis (the formation of primitive blood vessels from angioblasts), leading to the formation of infantile hemangiomas. Several risk factors for the development of infantile hemangiomas exist, including female sex, Caucasian race and preterm birth. Increased maternal age, multiple gestation, pre-eclampsia, placenta previa, maternal history of infertility, chorionic villus sampling, assisted reproductive technologies and ovulation promotion also have been suggested as additional risk factors.