What are clinical practice guidelines for the treatment and management of port-wine birthmarks, including those associated with Sturge-Weber syndrome?
Clinical Applications
Dr. Hsu is a member of the DermWorld Editorial Advisory Workgroup.
By Sylvia Hsu, MD, FAAD, March 1, 2022
In this month’s Clinical Applications column, DermWorld Editorial Advisory Workgroup Member Sylvia Hsu, MD, FAAD, talks with Kristen M. Kelly, MD, FAAD, and Sara Sabeti, MS4, about their JAMA Dermatology paper, "Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome."
DermWorld: What is the difference between a nevus simplex on the nape of the neck and a port-wine stain? Aren’t they both capillary malformations?
DermWorld: At what age would you start treating a child with a port-wine birthmark on the face?
Dr. Kelly and Ms. Sabeti: Treating PWB at an earlier age, particularly in the first months of life, is safe and many experts agree that early treatment can result in better outcomes — as early as the patient is seen. Early treatment can minimize psychosocial distress later in life and can also decrease development of nodules. Young infants also have an advantage over toddlers in that pulsed dye laser treatment can be performed without general anesthesia more easily in the younger age group. Of course, each patient’s circumstance is unique, so a thorough discussion with family should take place prior to treatment initiation.
DermWorld: What is the first-line treatment for a port-wine birthmark on the face?
Dr. Kelly and Ms. Sabeti: The first-line treatment for a PWB on the face is pulsed dye laser. This device has a long history of efficacy and safety for the treatment of PWB and can be used in all patients. There are a variety of other devices (long pulsed 532 nm and intense pulsed light) that may be used for PWB for those without access to a pulsed dye laser or in treatment-resistant lesions.
DermWorld: What is the definition of Sturge-Weber syndrome?
DermWorld: When do I need to worry about glaucoma in a patient with Sturge-Weber syndrome? Does the incidence of glaucoma depend upon distribution (V1 versus V2) or laterality (unilateral versus bilateral) of the port-wine birthmark?
Dr. Kelly and Ms. Sabeti: The incidence of glaucoma is greater in patients whose PWB is on the forehead or overlies the eyelids or eye. Studies in recent years have shown that a PWB defined as being in a high-risk distribution does not necessarily follow a trigeminal nerve distribution but is defined as one that involves the forehead from the midline to an imaginary line between the outer canthus of the eye and the top of the ear including the upper eyelids. Bilateral PWB do pose a higher risk than unilateral ones, but involvement of the forehead and eyelids is a stronger risk factor. For this reason, any child with a high-risk facial PWB should be referred to an ophthalmologist for a baseline eye examination as well as periodic follow-up and neurology consultation should also be considered.
Kristen M. Kelly, MD, FAAD, is professor and chair of the Department of Dermatology at the University of California, Irvine. Equipment for this research was provided by Solta; Syneron/Candela; Thermi RF; Michaelson Diagnositics. Dr. Kelly serves as a consultant for: Shanghai Fudan-Zhangjiang Bio-pharmaceutical Co., Ltd, Syneron-Candela, Sciton. Research was supported by Michaelson Diagnostics, Biophotas, Orlucent, ASLMS; NIH; Sturge-Weber Foundation, and UC Irvine ICTS.
Sara Sabeti, BS, is a fourth-year medical student at the University of California, Irvine School of Medicine. The manuscript was supported by The Sturge-Weber Foundation. Sabeti was provided a grant to attend The Sturge-Weber Foundation conference.
Their paper appeared in JAMA Dermatology.
Disclaimer: The views and opinions expressed in this article do not necessarily reflect those of DermWorld.
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