Preventive treatment with oral sirolimus and aspirin in a newborn with severe Sturge-Weber syndrome

Pediatr Dermatol. 2019 Jul;36(4):524-527. doi: 10.1111/pde.13841. Epub 2019 May 28.

Abstract

Sturge-Weber syndrome (SWS) is characterized by facial capillary malformation, leptomeningeal capillary malformations, and choroidal and episcleral vascular malformations. These malformations produce neurologic and ophthalmological symptoms including seizures and glaucoma. A premature male newborn without prenatal diagnosis presented with severe bilateral SWS and was started on systemic sirolimus and aspirin. The patient has remained seizure-free for 23 months and demonstrated an excellent response to pulsed dye laser treatment.

Keywords: laser; neonatal; therapy-systemic; vascular malformation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Administration, Oral
  • Aspirin / therapeutic use*
  • Drug Therapy, Combination
  • Electroencephalography / methods
  • Humans
  • Infant, Newborn
  • Infant, Premature*
  • Lasers, Dye / therapeutic use
  • Magnetic Resonance Imaging / methods
  • Male
  • Port-Wine Stain / diagnosis
  • Port-Wine Stain / surgery
  • Primary Prevention / methods
  • Prognosis
  • Risk Assessment
  • Seizures / prevention & control*
  • Severity of Illness Index
  • Sirolimus / therapeutic use*
  • Sturge-Weber Syndrome / diagnosis*
  • Sturge-Weber Syndrome / diagnostic imaging
  • Sturge-Weber Syndrome / drug therapy*
  • Treatment Outcome

Substances

  • Aspirin
  • Sirolimus