Sturge-Weber syndrome variant with atypical intracranial findings: case report

J Child Neurol. 2006 Feb;21(2):155-7. doi: 10.1177/08830738060210020801.


Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical evidence of cerebral leptomeningeal angiomatosis. Considering that intracranial venous anomalies also are likely compatible with the embryologic explanation of Sturge-Weber syndrome, this child can serve as an unusual example of Sturge-Weber syndrome type II.

Publication types

  • Case Reports

MeSH terms

  • Cephalometry
  • Cerebral Angiography
  • Cerebral Veins / abnormalities*
  • Cerebral Veins / pathology
  • Child
  • Cranial Sinuses / abnormalities
  • Cranial Sinuses / surgery
  • Follow-Up Studies
  • Glaucoma / diagnosis*
  • Humans
  • Hydrocephalus / diagnosis*
  • Intracranial Arteriovenous Malformations / diagnosis*
  • Magnetic Resonance Angiography
  • Magnetic Resonance Imaging
  • Male
  • Orbit / blood supply
  • Sturge-Weber Syndrome / diagnosis*