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

Autor: Sabeti S; Department of Dermatology, University of California, Irvine School of Medicine, Irvine, California., Ball KL; The Sturge-Weber Foundation, Houston, Texas., Bhattacharya SK; Department of Ophthalmology & Bascom Palmer Eye Institute, University of Miami, Miami, Florida., Bitrian E; Department of Ophthalmology & Bascom Palmer Eye Institute, University of Miami, Miami, Florida., Blieden LS; Department of Ophthalmology & Cullen Eye Institute, Baylor College of Medicine, Houston, Texas., Brandt JD; Department of Ophthalmology, University of California, Davis, Sacramento, California., Burkhart C; Department of Dermatology, University of North Carolina, Chapel Hill, North Carolina., Chugani HT; Department of Neurology, NYU School of Medicine, New York, New York., Falchek SJ; Department of Neurology, Nemours duPont Hospital for Children, Wilmington, Delaware., Jain BG; Department of Neurology, Nemours duPont Hospital for Children, Wilmington, Delaware., Juhasz C; Departments of Pediatrics and Neurology, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan., Loeb JA; Department of Neurology and Rehabilitation Medicine, University of Illinois at Chicago, Chicago, Illinois., Luat A; Departments of Pediatrics and Neurology, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan; Department of Pediatrics, Central Michigan University, College of Medicine, Mt. Pleasant, Michigan., Pinto A; Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts., Segal E; Hackensack University Medical Center, Hackensack Meridian School of Medicine and Northeast Regional Epilepsy Group, Hackensack, New Jersey., Salvin J; Previous affiliation Division of Pediatric Ophthalmology, Nemours duPont Hospital for Children, Wilmington, Delaware., Kelly KM; Department of Dermatology, University of California, Irvine School of Medicine, Irvine, California. Electronic address: kmkelly@uci.edu.
Jazyk: angličtina
Zdroj: Pediatric neurology [Pediatr Neurol] 2021 Aug; Vol. 121, pp. 59-66. Date of Electronic Publication: 2021 May 06.
DOI: 10.1016/j.pediatrneurol.2021.04.013
Abstrakt: Background: Sturge-Weber syndrome (SWS) is a sporadic, neurocutaneous syndrome involving the skin, brain, and eyes. Because of the variability of the clinical manifestations and the lack of prospective studies, consensus recommendations for management and treatment of SWS have not been published.
Objective: This article consolidates the current literature with expert opinion to make recommendations to guide the neuroimaging evaluation and the management of the neurological and ophthalmologic features of SWS.
Methods: Thirteen national peer-recognized experts in neurology, radiology, and ophthalmology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included (1) risk stratification, (2) indications for referral, and (3) optimum treatment strategies. An extensive PubMed search was performed of English language articles published in 2008 to 2018, as well as recent studies identified by the expert panel. The panel made clinical practice recommendations.
Conclusions: Children with a high-risk facial port-wine birthmark (PWB) should be referred to a pediatric neurologist and a pediatric ophthalmologist for baseline evaluation and periodic follow-up. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, routine screening for brain involvement is not recommended, but brain imaging can be performed in select cases. Routine follow-up neuroimaging is not recommended in children with SWS and stable neurocognitive symptoms. The treatment of ophthalmologic complications, such as glaucoma, differs based on the age and clinical presentation of the patient. These recommendations will help facilitate coordinated care for patients with SWS and may improve patient outcomes.
(Copyright © 2021 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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