Sturge-Weber Syndrome Variant With Atypical Intracranial Findings: Case Report

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 comm...

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Bibliographic Details
Published in:Journal of child neurology 2006-02, Vol.21 (2), p.155-157
Main Authors: Yallapragada, Anil V., Cure, Joel K., Holden, Kenton R.
Format: Article
Language:English
Subjects:
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
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Description
Summary: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. ( J Child Neurol 2006;21:155—157; DOI 10.2310/7010.2006.00026).
ISSN:0883-0738
1708-8283
DOI:10.1177/08830738060210020801