Dermatology Book

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Sturge-Weber Syndrome

Aka: Sturge-Weber Syndrome
  1. 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
  2. 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%)
  3. Diagnosis
    1. Pediatric ophthalmology consult
    2. Skull XRay
      1. Double contoured parallel streaks of calcification
    3. Head CT or Head MRI
  4. References
    1. Kramer (2000) Dev Med Child Neurol 42: 756-9 [PubMed]

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