CASE #1: Strawberry hemangioma

Two types of vascular birthmarks occur in infancy: (1) strawberry (infantile) hemangiomas (SHs), and (2) vascular malformation. SHs proliferate, involute, and disappear during infancy. Vascular malformations (e.g., stork-bite hemangiomas, port-wine stains [capillary vascular malformation], and masses of abnormal swollen veins [venous malformations]) persist into adulthood. SHs are the most common type of tumors to occur during infancy.1-3 Most SHs are small and pose no risk to the infant.

It was once believed that SHs were not present at birth and arose ex nihilo in the subsequent weeks. In fact, more than 50% of SHs are present at birth as telangiectasias surrounded by a halo of pallor, pale or erythematous patches (i.e., skin that blanches and ecchymotic red or crimson macules).

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These patches and papules rapidly grow over the course of weeks. This rapid growth, which is out of proportion to the size of the baby, is the hallmark of SH. As proliferation accelerates, the SH becomes elevated and may be domed, verrucous, lobulated, plaquelike, tumoral, bosselated or pebblelike. Most SH growth takes place in the first four to six months of life.

Once SHs reach their peak size, they can involute suddenly in a matter of weeks, or slowly over the course of years. About half of all SHs involute by age 5 years, 70% by age 7 years and 100% by age 10 years. SHs that involute can leave behind scar tissue, telangiectasia, or redundant or anetodermic skin. Approximately 40% of SHs that involute by age 6 years leave behind residual changes, whereas 80% of SHs that involute after age 6 years leave behind residual changes. Infantile hemangiomas that take longer to involute have a higher incidence of permanent cutaneous residua.

A SH can be divided by the depth of the abberent blood vessels. In this schema, the three types hemangiomas are superficial, deep and combined. Superficial hemangiomas and combined hemangiomas are the most common. Superficial hemanigomas possess erythematous, bright-red, vascular, lobular plaques that resemble the bumpy surface of a strawberry. Combined hemangiomas possess a configuration resembling that of a poached egg, with a well-circumscribed superficial portion overlying a less-well-defined deeper component.

Eighty percent of SHs are small, focal and solitary. Most SHs range in size from 0.5 to 5.0 cm in diameter; the smallest can be 0.1 to 2.0 cm and the largest 20 to 30 cm. Approximately 60% occur on the head and neck, 25% on the trunk and 15% on the arms and legs. There can be multiple hemangiomas, sometimes in segmental or localized patterns.

SHs occur in 10% to 12% of white non-Hispanic infants, 1.4% of black infants and 0.8% of Asian infants. These lesions are more common in girls than in boys with a ratio of 3-5 females:1 male. In cases in which the SH involves a syndrome, the ratio is 7-9 female:1 male.

A number of age and maternal factors appear to increase the likelihood that a child will have a hemangioma, including premature birth, multiple gestations, maternal use of chorionic villus sampling, maternal age, placenta previa and pre-eclampsia.

The incidence of infantile hemangiomas is approximately 25% to 30% for preterm infants weighing <1.0 kg at birth; 15 % for preterm infants weighing between 1.0 and 1.5 kg; and for infants weighing >1.5 kg, the incidence is the same as that for term infants.

The etiology and cell of origin of SH has yet to be determined. SHs do not seem to have a genetic basis, as they are sporadic rather than autosomal. Possible cells of origin include placental tissue, endothelial progenitor cells and mesenchymal stem cells.

Placental tissue and SH stain for certain tissue-specific markers, including Lewis Y, merosin, and Fc gamma RII, but most notably glucose transporter 1; it is thought that some of these cells colonize the fetus and give rise to the SH, but this theory is not certain. There are also mesenchymal cells present in SH. Studies have suggested the importance of estrogen signaling in hemangioma proliferation.

The differential diagnosis (including syndromes that involve hemangiomas) consists of: angiosarcoma, arteriovenous malformation, blue rubber bleb syndrome, cherry angiomas, capillary malformation, congenital hemangioma (noninvoluting and rapidly involuting), Cobb syndrome, Dabska tumor, dermatofibrosarcoma protuberans, infantile fibrosarcoma, infantile myofibromatosis, kaposiform hemangioendothelioma, Kaposi’s sarcoma, lipoma, lipoblastoma of infancy, lymphatic malformation, lymphangioma, pyogenic granuloma, teratoma, venous malformation, diffuse neonatal hemangiomatosis, Gorham syndrome, rhabdomyosarcoma, Riley-Smith syndrome, and tufted angioma.

A variety of medical and surgical treatments exist for SH. Until recently, the main medical treatment was corticosteroids either given as injections 3.0-5.0 mg/kg per treatment or orally at 2.0-3.0 mg/kg/day for two to three months.

Class I topical steroids and topical imiquimod (Aldara) have been used as well. Recombinant interferon alpha (2a or 2b) and vincristine (Oncovin) have also been used.

A promising new treatment is the nonselective blocker propranolol (Inderal) at a dose of 2.0-3.0 mg/kg/day in two to three divided doses for two to 10 months. Propranolol starts to work in 24 hours and can lead to 100% resolution of SH. The medication must be given in controlled settings, as it can lead to a drop in BP. The optimal dosing schedule for propranolol has yet to be determined. Pulsed dye laser (585 nm or 595 nm) can be used for flat lesions. Surgical excision is seldom advised.

Most SHs pose no risk to an infant. Those that that do can impinge on vital structures (usually the face and neck), ulcerate, bleed, cause high-output cardiac failure, cause platelet trapping (i.e., Kasabach-Merritt phenomenon), cause significant structural abnormalities or disfigurement (in particular on the lips or nose), and — in cases of multiple or segmental SH — be associated with one or more underlying congenital anomalies.

It is useful to review a few of the syndromes of which SH can be a part. PHACE syndrome consists of Posterior fossa (structural brain abnormalities present at birth); Hemangiomas of the face, head, and neck (segmental, >5.0 cm in diameter); Arterial lesions (especially carotid, cerebral, and vertebral); Cardiac anomalies (coarctation of the aorta in addition to many other structural anomalies); and Eye abnormalities.4

PELVIS syndrome consists of Perineal hemangioma with any of the following: External genital malformations, Lipomyelomeningocele, Vesicorenal abnormalities, Imperforate anus, and Skin tags.5

SH usually can be managed with reassurance to the parents, but if the lesions are large or in sensitive locations, referral to a specialist with experience in the management of SH is warranted.

After clinicians injected this patient’s lesion with 20 mg of corticosteroids every 14 days for six weeks, it shrank to the point that the parents were willing to wait for it to eventually involute.