| Intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow. | |
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MedLine Citation:
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PMID: 12093150 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Michelle A Kiley; John M Oxbury; Stuart C Coley |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia Volume: 9 ISSN: 0967-5868 ISO Abbreviation: J Clin Neurosci Publication Date: 2002 May |
Date Detail:
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Created Date: 2002-07-02 Completed Date: 2002-10-11 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 9433352 Medline TA: J Clin Neurosci Country: Scotland |
Other Details:
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Languages: eng Pagination: 330-3 Citation Subset: IM |
Copyright Information:
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Copyright 2002 Published by Elsevier Science Ltd. |
Affiliation:
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Department of Clinical Neurology, Radcliffe Infirmary, Oxford, OX2 6HE, UK. michelle.kiley@fmc.sa.gov.au |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Cerebral Angiography Cerebral Veins / physiopathology*, radiography Female Humans Intracranial Hypertension / diagnosis, etiology* Klippel-Trenaunay-Weber Syndrome / complications*, physiopathology* Magnetic Resonance Imaging Regional Blood Flow Sturge-Weber Syndrome / complications*, physiopathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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