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Review

Sturge–Weber syndrome: advances in diagnostic testing and treatment

, &
Pages 329-338 | Published online: 10 Jan 2014
 

Abstract

Sturge–Weber syndrome (SWS) is a sporadic, congenital disorder that impairs the development of the skin, eye and brain. Owing to its multisystem clinical manifestations, a multidisciplinary approach from dermatologists, neurologists and ophthalmologists is required. This article reviews data on pathogenesis, diagnosis and treatment of SWS with a focus on neurologic and dermatologic abnormalities. In terms of diagnosis, MRI, PET and electroencephalogram are discussed. Treatment of seizures includes prophylactic anti-epileptic medications, focal resection and hemispherectomy. Various treatment options for port-wine stains are also discussed, including pulsed-dye laser and new techniques, such as photodynamic therapy and the concurrent use of diagnostic imaging with pulsed-dye laser. Data supporting different theories of pathogenesis are also reviewed.

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

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