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Review
. 2021 Jan 1;157(1):98-104.
doi: 10.1001/jamadermatol.2020.4226.

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

Sara Sabeti  1 , Karen L Ball  2 , Craig Burkhart  3 , Lawrence Eichenfield  4   5 , Esteban Fernandez Faith  6   7 , Ilona J Frieden  8 , Roy Geronemus  9   10 , Deepti Gupta  11 , Andrew C Krakowski  12 , Moise L Levy  13   14   15 , Denise Metry  16 , J Stuart Nelson  17   18 , Megha M Tollefson  19 , Kristen M Kelly  1
Affiliations
Review

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

Sara Sabeti et al. JAMA Dermatol. .

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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Figures

Fig 1.
Fig 1.
(a) and (b) Port-wine birthmarks with the highest risk of Sturge-Weber syndrome
See this image and copyright information in PMC

References

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