Peds

Sturge-Weber Syndrome

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Sturge-Weber Syndrome

  • Signs
  1. Port-Wine Stain in ophthalmic nerve distribution
    1. Occurs above palpebral fissure
  2. Bilateral involvement
  3. Lesions of both upper and lower Eyelids
  • Associated conditions
  1. Seizures (80%)
    1. Onset before age 1 year
    2. Related to Vascular Malformation of leptomeninges
  2. Mental Retardation (60%)
  3. Hemiplegia (30%)
  4. Neonatal Glaucoma (30%)
  • Diagnosis
  1. Pediatric ophthalmology consult
  2. Skull XRay
    1. Double contoured parallel streaks of calcification
  3. Head CT or Head MRI
  • References
  1. Kramer (2000) Dev Med Child Neurol 42: 756-9 [PubMed]