Sturge-Weber Syndrome Guidelines

Updated: Sep 26, 2023
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Guidelines Summary

American Academy of Dermatology

In 2021, the American Academy of Dermatology (AAD) released a consensus statement for the management and treatment of port-wine birthmarks (PWBs) in Sturge-Weber syndrome (SWS). The AAD recommends treating PWBs at an early age to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. In the United States, 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. [115]

The Sturge Weber Foundation

A panel of 13 national peer-recognized experts in neurology, radiology, and ophthalmology with experience treating patients with SWS, supported by the Sturge Weber Foundation, developed clinical recommendations for the management and treatment of SWS. The panel found that children with a high-risk 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. [116]