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Sturge-Weber Syndrome

Aka: 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

Family Practice Notebook

Patients should address specific medical concerns with their physicians.

Although access to this page is not restricted, the information found here is intended for use by medical providers.

This page was written by Scott Moses, MD, last revised on 10/21/2007 and last published on 5/9/2012.

This is one of 5737 pages in the Family Practice Notebook