II. Signs
-
Port-Wine Stain in ophthalmic nerve distribution
- Occurs above palpebral fissure
- Bilateral involvement
- Lesions of both upper and lower Eyelids
III. Associated conditions
-
Seizures (80%)
- Onset before age 1 year
- Related to Vascular Malformation of leptomeninges
- Mental Retardation (60%)
- Hemiplegia (30%)
- Neonatal Glaucoma (30%)
IV. Diagnosis
- Pediatric ophthalmology consult
-
Skull XRay
- Double contoured parallel streaks of calcification
- Head CT or Head MRI