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Sheila Fallon Friedlander, M.D. offers tips for therapeutic decision-making when managing children with vascular lesions. She references a classification tool to improve diagnostic accuracy and discusses uses for and cautions with PDL therapy.
Therapeutic decision-making for vascular lesions in children presents several challenges. An accurate diagnosis is fundamental to the process, but then there are other issues to consider, according to Sheila Fallon Friedlander, M.D. who spoke to colleagues this past week at the MauiDerm 2016 meeting.
Discussing the diagnosis and management of pediatric vascular lesions, Dr. Friedlander told colleagues that a red spot in early infancy is likely to be an infantile hemangioma. The risk of these lesions is about 5% overall, but can be much higher in certain groups of infants.
“The risk of infantile hemangioma is higher in girls than in boys and it goes up in twins, children born to mothers with placental anomalies, and in premature babies. For a very low birthweight baby, the incidence has been reported as high as 16% to 17%,” she said. Dr. Friedlander is professor of clinical dermatology and pediatrics, Rady Children's Hospital and UCSD School of Medicine, San Diego, Calif.
A useful tool for assessing vascular lesions in children is available from the International Society for the Study of Vascular Anomalies (ISSVA). By entering “ISSVA Classification” into an Internet search, clinicians easily find this interactive site that provides a broad overview along with definitions and information on genetic mutations and associated syndromes.
“This is an incredible classification system that will help dermatologists in evaluating a baby with vascular lesions and determining what they are dealing with,” Dr. Friedlander said.
In terms of associations, new evidence suggests that in the case of a port wine stain, it is forehead involvement rather than distribution over the V1 area that presents a major risk factor for Sturge-Weber syndrome, she added.
“For the time being, however, I worry about kids who have a port wine stain with V1 or large, extensive involvement, as well as those whose lesion is on the forehead area. It is important to think about risk if the forehead is involved, even if it is not in the V1 distribution area,” Dr. Friedlander said.
Treatment with pulsed dye laser (PDL) continues to be a mainstay for treating port wine stains, and studies indicate that starting treatment early in life may afford the best outcomes. However, recent data suggesting that there is an increased risk of neurodevelopmental damage in children who are put under general anesthesia during the first two to three years of life is complicating the decision about when to begin PDL therapy.
“Now we face a real conundrum about whether to try to provide the best results for the patient or if we should worry more about a theoretical neurological risk from early intervention,” Dr. Friedlander said.
Based on the available evidence, she noted that when reasonable, she will try to delay treatment until the child is older or preferably use local anesthesia rather than general.
“The OR nurses, however, don’t like me so much when they have to deal with an awake and crying child,” Dr. Friedlander commented.
Clinicians using the PDL to treat port wine stains should be aware that findings of a recent survey raise concern about the risk of long-term alopecia when treatment is delivered to hair-bearing sites in very young children. Of the respondents who had used the PDL to treat port wine stains of the eyebrow and/or scalp, about one-fourth reported having at least one patient develop long-term alopecia (no sign of hair regrowth after several years of nontreatment). The overall incidence in the surveyed population was 1.5% to 2.6%.
“It is thought the alopecia can occur even if the laser settings are correct because the relatively thin skin in early infancy may leave the hair bulbs more vulnerable,” Dr. Friedlander added.
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For treatment of recalcitrant or recurrent port wine stains, there is evidence to show that use of lasers with a longer wavelength, either a 755 nm alexandrite or 1064 nm Nd:YAG, can be helpful. There are also data showing that using adjunctive topical imiquimod or rapamycin can improve the response to PDL treatment.
At the same time, however, it is important to be sure that the treatment failure is not due to misdiagnosis. In that regard, Dr. Friedlander presented one case of a child with an early segmental hemangioma and others with morphea that were initially misdiagnosed as a port wine stain and treated with the PDL.
“The child with hemangioma did not get proper systemic treatment early on, and for a child with morphea, treatment requires a systemic corticosteroid and perhaps methotrexate,” she said. “It is crucial to be certain of your diagnosis before embarking on laser therapy.”