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

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