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 REVIEWIn 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 RELEVANCEThese 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.The goals of this consensus statement are to review the literature and provide an approach to risk stratification and evaluation of PWBs, offer guidance on diagnostic workup for patients with suspected or newly diagnosed SWS, and assess current treatment options for PWBs in light of the patient's age and condition severity. The treatment recommendations are currently applicable to all patients with a PWB.
MethodsTwelve national experts in dermatology were consulted to develop a consensus statement on the management and treatment of cutaneous manifestations of SWS as part of a larger consensus statement. The panel was created from a list of experts provided by the Sturge-Weber Foundation who had significant experience in treating patients with SWS and patients with PWBs who agreed to participate. Three key needs were identified: (1) risk stratification and evaluation of PWBs, (2) optimum treatment strategies for PWBs, and (3) specific recommendations regarding light-based therapies. The expert group was divided into 4 subgroups that formulated questions to address each topic. An extensive literature review was performed using PubMed for English-language articles published between December 1, 2008, and December 1, 2018, an arbitrarily selected date range, to explore articles within the past 10 years. Articles before 2008or after 2018were added by the expert panel based on importance. Search terms in...