Document Detail


Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report.
MedLine Citation:
PMID:  12383373     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE AND IMPORTANCE: Spinal cord involvement in Klippel-Trenaunay-Weber (KTW) syndrome is rare. Cases of intradural spinal cord arteriovenous malformations (AVMs) have been associated with this syndrome. Likewise, cases of epidural hemangioma and angiomyolipoma have been reported to occur at the same segmental level as cutaneous hemangioma in KTW syndrome. This report details a rare case of an extradural thoracic AVM in a patient with KTW syndrome. CLINICAL PRESENTATION: A 30-year-old man presented with a 10-month history of progressive myelopathy, bilateral lower-extremity weakness, and numbness, with the right side affected more than the left. His symptoms had progressed to the point that he was unable to walk. The patient had the characteristic manifestations of KTW syndrome, including numerous cutaneous angiomas and cavernomas, limb hypertrophy and syndactyly, and limb venous malformations. A magnetic resonance imaging scan and subsequent angiogram demonstrated a large extradural AVM causing cord compression at the T3-T4 levels. INTERVENTION: The patient underwent two separate endovascular procedures, including embolization of upper thoracic and thyrocervical trunk feeders. Subsequently, he underwent T1-T4 laminectomy and microsurgical excision of the AVM. Clinically, the patient improved such that he could walk without assistance. CONCLUSION: KTW syndrome represents a spectrum of clinical presentations. Although involvement of the spinal cord is uncommon, the manifestations of this syndrome may include both intradural and extradural AVMs in addition to various tumors.
Authors:
Michael J Alexander; Peter M Grossi; Robert F Spetzler; Cameron G McDougall
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurosurgery     Volume:  51     ISSN:  0148-396X     ISO Abbreviation:  Neurosurgery     Publication Date:  2002 Nov 
Date Detail:
Created Date:  2002-10-17     Completed Date:  2002-12-04     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1275-8; discussion 1278-9     Citation Subset:  IM    
Affiliation:
Division of Neurosurgery and Interventional Neuroradiology, Duke University Medical Center, Duke Clinic, 4505 Busse Building, Durham, NC 27710, USA. michael.alexander@duke.edu
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MeSH Terms
Descriptor/Qualifier:
Adult
Arteriovenous Malformations / complications,  etiology*,  therapy
Embolization, Therapeutic
Humans
Klippel-Trenaunay-Weber Syndrome / complications*
Laminectomy
Male
Spinal Cord Compression / etiology
Thorax / blood supply*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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