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Consensus Statement
November 11, 2020

Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome

Author Affiliations
  • 1Department of Dermatology, University of California, Irvine School of Medicine, Irvine
  • 2The Sturge-Weber Foundation, Houston, Texas
  • 3Department of Dermatology, University of North Carolina, Chapel Hill
  • 4Department of Pediatric and Adolescent Dermatology, University of California, San Diego
  • 5Department of Pediatric and Adolescent Dermatology, Rady Children’s Hospital, San Diego, California
  • 6Division of Dermatology, Department of Pediatrics, Nationwide Children’s Hospital, Columbus, Ohio
  • 7Division of Dermatology, Department of Pediatrics, The Ohio State University College of Medicine, Columbus
  • 8Department of Dermatology, School of Medicine, University of California, San Francisco
  • 9Department of Dermatology, New York University School of Medicine, New York
  • 10Laser & Skin Surgery Center of New York, New York
  • 11Department of Pediatrics, Division of Dermatology, Seattle Children’s Hospital/University of Washington School of Medicine, Seattle
  • 12Department of Dermatology, St Luke’s University Health Network, Easton, Pennsylvania
  • 13Pediatric and Adolescent Dermatology, Dell Children’s Medical Center, Austin, Texas
  • 14Department of Pediatrics, Dell Medical School, University of Texas at Austin
  • 15Department of Medicine, Division of Dermatology, Dell Medical School, University of Texas at Austin
  • 16Department of Dermatology, Baylor College of Medicine, Houston, Texas
  • 17Department of Surgery, Beckman Laser Institute and Medical Clinic, University of California, Irvine
  • 18Department of Biomedical Engineering, Beckman Laser Institute and Medical Clinic, University of California, Irvine
  • 19Department of Dermatology and Pediatrics, Mayo Clinic, Rochester, Minnesota
JAMA Dermatol. Published online November 11, 2020. doi:10.1001/jamadermatol.2020.4226
Key Points

Question  What are clinical practice guidelines for treatment and management of port-wine birthmarks, including those associated with Sturge-Weber syndrome?

Findings  In this consensus statement, 10 key recommendations for treatment of port-wine birthmarks were formulated. These recommendations address risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies.

Meaning  The recommendations provided in this statement may help guide clinical decision-making for these patients.

Abstract

Importance  Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome involving the skin, brain, and eyes. Consensus recommendations for management are lacking.

Objective  To consolidate the current literature with expert opinion to make recommendations that will guide treatment and referral for patients with port-wine birthmarks (PWBs).

Evidence Review  In this consensus statement, 12 nationally peer-recognized experts in dermatology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies. A systematic PubMed search was performed of English-language articles published between December 1, 2008, and December 1, 2018, as well as other pertinent studies identified by the expert panel. Clinical practice guidelines were recommended.

Findings  Treatment of PWBs is indicated to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. Better outcomes may be attained if treatments are started at an earlier age. In the US, pulsed dye laser is the standard for all PWBs regardless of the lesion size, location, or color. When performed by experienced physicians, laser treatment can be safe for patients of all ages. The choice of using general anesthesia in young patients is a complex decision that must be considered on a case-by-case basis.

Conclusions and Relevance  These recommendations are intended to help guide clinical practice and decision-making for patients with SWS and those with isolated PWBs and may improve patient outcomes.

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