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

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 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...

Characteristics of mucocutaneous vascular malformations drawn from a decade of a multidisciplinary committee experience

“…15 Previous publications recommend early treatment initiation (before the age of 10, 18 6, 19 or 1 20 ); however, we found no supporting evidence for this. Recent studies report that CM located in the face and neck 21 or proximally in extremities 22 tend to have better outcomes, with fewer sessions needed. We also observed that CM located distally in extremities, especially if >5 cm 2 , typically receive more sessions with worse results.…”

Consensus Statement for the Management and Treatment of Sturge-Weber Syndrome: Neurology, Neuroimaging, and Ophthalmology Recommendations

“…Eight national experts in neurology and radiology, and five national experts in ophthalmology, were consulted to form a consensus statement on the management and treatment of SWS as part of a larger consensus statement. 1 Three key topics were established: (1) evaluation of port-wine birthmark (PWB) and risk stratification, (2) consultation and referral, and (3) optimum treatment strategies. Questions were formulated for each key topic, and an extensive literature review was performed using PubMed for English language articles published between 2008 and 2018.…”

“…In an earlier article, we discussed the dermatologic management and treatment. 1 In this article, we provide clinical practice guidelines to guide the care of patients with SWS, focusing on the neurological and ophthalmologic features of the disease. The goals of this consensus are to (1) review the literature to provide an approach to risk stratification and evaluation of patients with SWS, (2) offer guidance on the neurological, neuroimaging, and ophthalmologic evaluation for patients with suspected or newly diagnosed SWS and indications for referral to an ophthalmologist or neurologist, and (3) assess the current treatment options for brain and eye manifestations with considerations for age and severity of disease.…”

Basal cell carcinoma arising within port‐wine stain

“…The port-wine stain (PWS) is a capillary malformation affecting the superficial vascular plexus of the skin. 1 It is typically unilateral and involves the ophthalmic (V 1 ), maxillary (V 2 ), and/or mandibular (V 3 ) divisions of the trigeminal nerve. Sturge-Weber syndrome (SWS) is a rare, congenital, non-heritable neurocutaneous disorder characterized by a facial PWS in the distribution of the trigeminal nerve associated with abnormal venous vessels in the leptomeninges and eye.…”