Intracranial hypertension in Sturge–Weber/Klippel–Trenaunay–Weber overlap syndrome due to impairment of cerebral venous outflow

Klippel–Trenaunay–Weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. Sturge–Weber Syndrome (SWS), also a m...

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Bibliographic Details
Published in:Journal of clinical neuroscience 2002-05, Vol.9 (3), p.330-333
Main Authors: Kiley, Michelle A., Oxbury, John M, Coley, Stuart C.
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Cerebral Angiography
Cerebral Veins - diagnostic imaging
Cerebral Veins - physiopathology
Cerebrospinal fluid. Meninges. Spinal cord
Female
Humans
Intracranial Hypertension - diagnosis
Intracranial Hypertension - etiology
Klippel-Trenaunay-Weber Syndrome - complications
Klippel-Trenaunay-Weber Syndrome - physiopathology
Klippel–Trenaunay–Weber, Sturge–Weber, idiopathic intracranial hypertension, venous obstruction
Magnetic Resonance Imaging
Medical sciences
Nervous system (semeiology, syndromes)
Neurology
Regional Blood Flow
Sturge-Weber Syndrome - complications
Sturge-Weber Syndrome - physiopathology
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Summary:Klippel–Trenaunay–Weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. Sturge–Weber Syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. Cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.
ISSN:0967-5868
1532-2653
DOI:10.1054/jocn.2001.1041