Comprehensive Review Sheds Light on Birthmarks

It happens to many new parents. Delighted with a perfect baby, they barely notice the pale pink or blue skin patch that is an hemangioma or "strawberry" birthmark. As the lesion rapidly grows, reddens and rises in the next months, they are alarmed. And, if it's on baby's face, they'll need a doctor's support and reassurance.

This finding, and the fact that some skin hemangiomas can indicate the presence of life-threatening conditions, are among the insights found in a comprehensive review of advances in research and technology related to hemangiomas in children.

The paper, by Medical College of Wisconsin researchers Beth A. Drolet, MD, and Nancy B. Esterly, MD, appeared in the New England Journal of Medicine and cites 69 research studies as evidence. Ilona J. Frieden, MD, a University of California-San Francisco Pediatric Dermatologist, is co-author. Drs. Drolet and Esterly both practice at Children's Hospital of Wisconsin, a major affiliate of MCW.

"Despite their frequency, the origin and development of hemangiomas is not completely understood, and their management remains a subject of considerable controversy," says Dr. Drolet, an Associate Professor of Dermatology. "Our goal is to update primary care physicians on many aspects of hemangiomas, in light of advances in classification, angiogenesis research, imaging, recently-appreciated associated complications or conditions, and new treatments such as interferon and lasers."

"We hope to alert physicians to new treatment options and to the growing number of exceptions to the long-standing rule that most hemangiomas are best left untreated," says Dr. Esterly, MD, "We are advising physicians to observe infants with hemangiomas carefully and to tailor their approach to the specific characteristics of each case." Dr. Esterly, who won the Society of Pediatric Dermatology's first Lifetime Achievement Award last year, is a Professor of Dermatology.

Hemangiomas are actually tumors composed of blood vessels. They are also the most common soft-tissue tumor of infancy, affecting five to ten percent of all one-year-olds. Hemangiomas are distinguished from other vascular birthmarks by exhibiting a rapid growth phase and an unpredictable, spontaneous resolution phase, during which they shrivel up and fade.

With the exception of those near the eye, ear or anogenital area, hemangiomas of the skin are generally harmless. Most grow to their full size and resolve without treatment within the first few years of life. Research, however, links the size or number of lesions in specific locations to vascular and spinal anomalies, or to the presence of internal hemangiomas, in the airway, viscera or liver, which pose lethal threats and require very close monitoring.

For example, the research cited in the study found that parents of children with obvious facial hemangiomas commonly experience fear, disbelief and mourning as intense as that experienced when children had more permanent malformations. It also found these parents were barraged with comments from strangers, including accusations of child abuse, and that more than half were dissatisfied with aspects of their medical care, particularly if no treatment was underway. The authors suggest that physicians reassure these parents with before-and-after photos of other children with hemangiomas that have involuted, or with photos of their own child's lesion in progressive stages of involution. They also suggest that any lesion that has not resolved by the age of four and has the potential for scarring be re-evaluated for surgery.

HEMANGIOMA FACTS

Other findings cited in the review by MCW researchers:

Suspected Causes:

• Children of women undergoing chorionic villus sampling, a pre-natal genetic testing technique, at eight to ten weeks, are ten times more likely to have hemangiomas.

• Their link with this procedure, which is known on rare occasion to disrupt vascular structures, suggests that errors in vasculogenesis, or blood vessel development, may provide a "fertile field" for hemangioma development.

• Malformed arteries in some patients with extensive facial hemangiomas have been linked to developmental defects occurring in the 8th to 10th week of gestation. This condition occurs most frequently in female infants.

• Recent research in angiogenesis, or development of new blood vessels from existing vessels, reveals that this process appears to play a role in hemangioma development.

Manifestations:

• Appearance varies, by stage of development, depth and location. They can be both deep and superficial and are usually solitary, but 20 percent are multiple lesions.

• They are three times more likely to occur in females, and 55 percent are present at birth, with the others developing shortly afterward. Rarely, they are fully grown at birth. These "congenital" lesions resolve rapidly, leaving noticeable skin defects.

• It's difficult to predict the length of growth phases for individual lesions. But generally, superficial lesions reach their maximum size by six to eight months, and deep hemangiomas may grow for 12 to 14 months, rarely, up to two years. Predicting involution is more difficult, but it usually begins with a change from bright red to a purple or gray color. About 20-40 percent will leave residual skin changes, and very large superficial facial lesions often leave disfiguring scars.

• Ultrasound, CT scans and Magnetic resonance imaging studies can make the diagnosis of hemangioma easier in difficult cases involving large congenital lesions, distinguishing them from vascular malformations and aggressive tumors.

Complications & Treatment:

• Visceral lesions, particularly those of the liver, carry a high risk of death and require meticulous monitoring. Multiple skin hemangiomas and large facial hemangiomas are associated with visceral lesions. These children should be monitored carefully and abdominal ultrasound exams should be considered.

• Airway hemangiomas may also be life-threatening, with respiratory failure often occurring at six to 12 weeks of life. About 50 percent of these infants have associated skin hemangiomas, particularly in the chin, lip, jaw or neck regions. Consequently, infants with skin hemangiomas and "noisy breathing' should have direct visualization exams of the airway.

• Ulceration is the most frequent complication of skin lesions and can be very painful, carrying the risk of infection, hemorrhage and scarring. It can be present at birth, causing irritability, sleeplessness and poor appetite.

• Hemorrhage is rare but alarming, usually involving minimal blood loss. In skin lesions, it can be controlled by direct pressure.

• Lesions of the anogenital region are most prone to ulceration and infection, causing severe pain on defecation or urination. Superinfection can be lethal. Topical or oral antibiotics, dressings, compresses, pulsed dye lasers, oral corticosteroids and pain relievers, or combinations thereof are used, and occasionally, surgery is warranted.

• Lesions of the eyelid can cause astigmatism or amblyopia and should be evaluated by an ophthalmologist.

• Lesions of the ear can obstruct hearing and cause speech delays.

Links to Other Disorders:

• Lumbosacral surface hemangiomas that span the midline may indicate spinal, anorectal and urogenital deformities and call for spinal imaging in these infants.

New Therapies:

• Lasers, corticosteroids, alpha interferon, and the prospect of newly-developed inhibitors of angiogenesis have led many researchers to question the universal hands-off approach to treatment, particularly in life-threatening airway and hepatic lesions, where treatment may vary but the decision to treat is straightforward.

• Treatment of large facial hemangiomas posing great risk of disfigurement remains a more difficult decision. The most recent consensus of researchers calls for closer, more frequent monitoring of infants with these lesions to better predict the ultimate size, period of growth, and rate of resolution of these tumors. Then, they caution, surgical treatment must be weighed carefully, since the resulting scar may be worse than the result of spontaneous regression.

MCW Health News presents up-to-date information on patient care and medical research by the physicians of the Medical College of Wisconsin.