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  • Title: 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, Ikeda S.
    Journal: Intern Med; 2008; 47(18):1621-5. PubMed ID: 18797123.
    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.
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