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Clinical Summary

Hemangiomas are benign vascular tumors characterized by a rapid proliferative phase followed by a spontaneous involutional phase. They are the most common soft-tissue tumors of infancy. The appearance of hemangiomas is defined by the lesion’s depth, location, and stage of evolution. A superficial hemangioma lies in the upper dermis and often originates as an erythematous macular patch, a pale macule, or a localized telangiectasia with a pale halo. The lesion grows and becomes vascularized during the first 2 months of life. The classic presentation is a bright red, slightly raised, noncompressible plaque. It commonly regresses by 2 to 3 years of age but may persist throughout life. Hemangiomas can also affect the airway, eyes, and liver or cause high-output cardiac failure if sufficiently large. The most important local complication is ulceration, which can be exquisitely painful. The differential diagnosis includes vascular malformations, malignant vascular neoplasms, pyogenic granulomas, and giant melanocytic birthmarks.

FIGURE 14.70

Strawberry Hemangioma. Raised umbilicated vascular lesion on the left upper chest consistent with strawberry hemangioma. (Photo contributors: Kara Shah, MD, PhD, and Katharine Hanlon.)

Management and Disposition

Most cases require serial observation as superficial hemangiomas usually regress without residual problems. Treatment of hemangiomas is indicated when there is an obstruction of a vital orifice (ie, airway, mouth, or nares), lesion in the periorbital region, or lesion in the GI tract or airway, or if hematologic or cardiovascular complications are present. Education and parental reassurance are important for small hemangiomas because there is great pressure to treat for cosmetic reasons. There is growing evidence supporting the use of topical β-blockers for superficial, uncomplicated hemangiomas to expedite resolution. For more complicated hemangiomas, first-line therapy is propranolol as it inhibits the growth and induces regression. Infants should undergo a pretreatment consultation with a pediatric dermatologist or vascular tumor specialist to determine eligibility and risk of starting β-blocker therapy. Due to the risk of hypoglycemia and hemodynamic changes, many higher risk infants are admitted for close monitoring when initiating propranolol therapy. Topical and systemic corticosteroids were the mainstay of therapy prior to the use of β-blockers and are still beneficial in children in whom β-blockers are contraindicated.

Pearls

  1. Kasabach-Merritt phenomena (hemolytic anemia, thrombocytopenia, and coagulopathy) is associated with Kaposiform hemangioendothelioma or tufted angiomas and not with common hemangiomas as was previously thought.

  2. As many as 20% of affected infants have multiple lesions.

  3. In contrast to hemangiomas, vascular malformations, such as port-wine stains, do not proliferate or involute. They persist throughout life and grow in proportion with the child.

  4. Children with a segmental distribution hemangioma of the scalp, trunk, upper extremity, or most commonly the face are at risk for PHACE syndrome (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies) and require further screening through ...

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