Hemangiomas

Epidemiology

Hemangiomas are proliferative endothelial vascular lesions that are identified by their characteristic clinical appearance and course. Forty percent of hemangiomas are present at birth and 60% appear within the first few months of life. Hemangiomas are more common in females than in males (5:1). Common sites of occurrence include skin, face, orbits, larynx, nasal cavity, and deep neck spaces.

The classification proposed by Mulliken and Glowacki distinctly separates hemangiomas from vascular malformations based on biological and clinical characteristics. Previous descriptive terms such as strawberry, capillary, juvenile, or cellular are not currently used to describe hemangiomas. These older names are now encompassed by the term hemangioma. Most authors now feel that cavernous hemangiomas are venous malformations. Port-wine hemangiomas are now considered capillary malformations.

Clinical Findings

Superficial hemangiomas are bright red papular lesions. Subcutaneous hemangiomas often present as a bluish mass that may be difficult to differentiate from a venous malformation or an arteriovenous malformation.

Hemangiomas rapidly grow during the first 12 to 18 months of life (proliferative phase). This is followed by gradual regression (involuting phase) over the next 6 to 10 years. Approximately half of all lesions will completely involute, whereas the remainder will partially involute. Incomplete involution may result in residual telangiectasias, hypoplastic patches, or scarring.

Hemangiomas usually arise in the superficial layers of the skin and mucosa. The majority of hemangiomas have an uneventful course with spontaneous and complete involution. More advanced lesions, depending on their location, may cause severe facial disfigurement, impaired vision, or respiratory stridor.

In the past, hemangiomas have been associated with a variety of syndromes that include Dandy–Walker, Klippel–Trenaunay, Sturge–Weber, Beckwith–Wiedemann, Hippel–Lindau, and Rendu–Osler–Weber. There is also an association with Kasabach–Merritt syndrome; a consumptive coagulopathy complicated by platelet trapping, hemorrhage, or high-output congestive heart failure that may occur with large hemangiomas. These associations were identified prior to the Mulliken and Glowacki classification system. Further investigations will be needed to determine whether these associations are still valid using the currently accepted classification scheme.