An Unusual Case of Klippel-Trénaunay-Weber Syndrome Presenting with Portosystemic Encephalopathy
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...
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Published in | Internal Medicine Vol. 47; no. 18; pp. 1621 - 1625 |
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Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
Published |
Japan
The Japanese Society of Internal Medicine
01.01.2008
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Subjects | |
Online Access | Get full text |
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Summary: | 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. |
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Bibliography: | ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Case Study-2 ObjectType-Feature-4 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0918-2918 1349-7235 1349-7235 |
DOI: | 10.2169/internalmedicine.47.1130 |