Document Detail


An unusual case of Klippel-Trénaunay-Weber syndrome presenting with portosystemic encephalopathy.
MedLine Citation:
PMID:  18797123     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report a unique male patient presenting with portosystemic encephalopathy (PSE) due to intrahepatic portohepatic venous (PHV) shunts. He was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS) based on the findings of a hemitruncal port-wine stain with subcutaneous arteriovenous fistulae and varicose veins in the legs. However, limb-hypertrophy, which is one of the most cardinal manifestations of KTWS, was absent, and in KTWS, PSE is quite a rare clinical manifestation. Hence, the clinical picture of this patient was unusual. Our clinical observation suggests that KTWS can be one of the underlying disorders causing PSE.
Authors:
Masahide Yazaki; Kazuma Kaneko; Kana Tojo; Daigo Miyazaki; Yasuhiro Shimojima; Kazuhiko Ueda; Shu-ichi Ikeda
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-09-16
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  47     ISSN:  1349-7235     ISO Abbreviation:  Intern. Med.     Publication Date:  2008  
Date Detail:
Created Date:  2008-09-17     Completed Date:  2009-01-07     Revised Date:  2009-04-16    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  1621-5     Citation Subset:  IM    
Affiliation:
Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto. mayazaki@hsp.md.shinshu-u.ac.jp
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MeSH Terms
Descriptor/Qualifier:
Angiography
Hepatic Encephalopathy / etiology,  pathology*
Humans
Klippel-Trenaunay-Weber Syndrome / complications,  diagnosis*,  pathology*
Male
Middle Aged
Port-Wine Stain / etiology
Tomography, X-Ray Computed
Comments/Corrections
Comment In:
Intern Med. 2009;48(6):493-4; author reply 495   [PMID:  19293555 ]

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


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