The FP recognized that the baby had a large infantile hemangioma on her face that needed immediate treatment to prevent amblyopia in the left eye. There was also concern for PHACE (posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome. Any large segmental hemangioma on the face could be part of PHACE syndrome and therefore would require evaluation of the eyes, central nervous system, and heart.
Hemangiomas consist of an abnormally dense group of dilated blood vessels that are thought to occur sporadically. They are characterized by an initial phase of rapid proliferation followed by spontaneous and slow involution. Often, they lead to complete regression. Most childhood hemangiomas are small and innocuous, but some can grow to threaten a particular function or even an infant’s life.
Rapid growth during the first month of life is the historical hallmark of hemangiomas because rapidly dividing endothelial cells are responsible for the enlargement of these lesions. The hemangiomas become elevated and may take on numerous morphologies (dome-shaped, lobulated, plaque-like, and/or tumoral).
The proliferation phase occurs during the first year, and most growth takes place during the first 6 months of life. Proliferation then slows, and the hemangioma begins to involute. Typically, 50% of childhood hemangiomas will involute by age 5, 70% by age 7, and the remainder will take an additional 3 to 5 years to complete the process of involution.
Large periocular hemangiomas demand prompt treatment to prevent debilitating consequences such as amblyopia. Propranolol is the first-line treatment for function-impairing and rapidly proliferating infantile hemangiomas. It has been successfully used to treat periorbital infantile hemangiomas and other problematic infantile hemangiomas.
The FP administered all the appropriate vaccines to the infant and told her parents to bring her to a pediatric dermatologist and an ophthalmologist.
Photos and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Usatine R, Madhukar M. Childhood hemangiomas and vascular malformations. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas of Family Medicine. 2nd ed. New York, NY: McGraw-Hill; 2013:636-641.
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