AKA Encephalotrigeminal angiomatosis

NOTE: Not a genetic condition, mutation is somatic and occurs before birth.

Port wine stain on forehead, scalp or around eyes due to over abundance of capillaries near surface of skin.

Clinical:

• Triad:
  • Facial port wine nevus
  • Leptomeningeal angiomatosis
  • Glaucoma
• Contralateral hemiplegia
• Gingival overgrowth
• Multiple impactions
• Seizure disorder, mental retardation