Understanding hemangiomas of infancy: Problematic hemangiomas require attention

August 1, 2008

Recognizing pediatric hemangiomas that require close follow-up and aggressive treatment is critical to patient care, an expert says.

Key Points

New York - Although most hemangiomas of infancy (HOI) don't require treatment, dermatologists must know how to identify potentially problematic ones for closer follow-up and perhaps early treatment, an expert says.

As dermatologists' understanding of HOI has grown, classification systems for these benign tumors have grown more precise, says Amy J. Theos, M.D., assistant professor of dermatology and director of pediatric dermatology at the University of Alabama, Birmingham.

"In the past," she says, "terms such as 'strawberry,' 'capillary' and 'cavernous' hemangioma were applied incorrectly to a variety of vascular lesions."

"The term 'hemangioma' should be reserved for vascular lesions that show evidence of cellular proliferation," Dr. Theos tells Dermatology Times.

Researchers have divided congenital hemangiomas into rapidly involuting congenital hemangiomas (RICH) and noninvoluting congenital hemangiomas (NICH), Dr. Theos says.

Experts also have theorized that although HOI and congenital hemangiomas share some clinical, histologic and radiographic similarities, they may represent different entities (North PE, et al. Arch Dermatol. 2001;137:1607-1620).

"In the last few years," she says, "we've recognized that there are two main patterns of growth - focal, which are discrete lesions, and segmental, which are widespread and plaque-like hemangiomas."

Focal vs. segmental

Segmental hemangiomas are associated with a higher incidence of complications, such as ulceration and airway involvement, and systemic anomalies.

One concern is PHACES syndrome, which occurs in approximately 20 percent of infants with segmental facial hemangiomas. Its characteristics include posterior fossa abnormalities, hemangiomas (segmental facial), arterial anomalies, coarctation of the aorta/cardiac defects and eye abnormalities.

Other associations with PHACES syndrome include arterial occlusion with cerebral infarction, as well as visceral hemangiomas and migraines, Dr. Theos says.

Likewise, she says segmental HOI in a "beard" distribution can signal subglottic involvement. Subglottic hemangiomas present with hoarse crying, biphasic stridor or croup-like cough, and can be life-threatening.

Lumbosacral HOI, on the other hand, are associated with anogenital abnormalities, spinal dysraphism, renal and urologic abnormalities and skin tags, Dr. Theos says.

Segmental hemangiomas or multiple HOI can signal visceral involvement that can lead to problems such as high output heart failure or gastrointestinal hemorrhage, she says.

"The diagnosis of HOI is usually straightforward, and a thorough history and examination are all that are needed," she says.

GLUT1, a relatively recently discovered immunohistochemical marker, can be a useful marker when the diagnosis is still in question, she says.

This glucose transporter is expressed in vascular endothelia of the placenta, retina and brain and is strongly expressed in all stages of infantile hemangiomas (North PE, et al. Arch Dermatol. 2001;137:559-570).

It's also positive in extracutaneous HOI (Drut RM, Drut R. J Clin Pathol. 2004;57:1197-1200), and negative in malformations and other vascular tumors, such as RICH and NICH.

As for individual treatments, Dr. Theos advises caution when using a pulsed-dye laser (PDL) to treat HOI.

"Compared to using it for a port wine stain," she says, "there's a higher risk of ulceration. So, we tend to use lower energy levels."

Similarly, she says, "Systemic corticosteroids are still the treatment of choice for proliferating high-risk lesions. We tend to prescribe high-dose, longer courses of systemic steroids in these children. One risk is adrenal suppression."