1. An unusual case of Klippel-Trénaunay-Weber syndrome presenting with portosystemic encephalopathy.
- Author
-
Yazaki M, Kaneko K, Tojo K, Miyazaki D, Shimojima Y, Ueda K, and Ikeda S
- Subjects
- Angiography, Hepatic Encephalopathy etiology, Humans, Klippel-Trenaunay-Weber Syndrome complications, Male, Middle Aged, Port-Wine Stain etiology, Tomography, X-Ray Computed, Hepatic Encephalopathy pathology, Klippel-Trenaunay-Weber Syndrome diagnosis, Klippel-Trenaunay-Weber Syndrome pathology
- Abstract
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.
- Published
- 2008
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