An unusual case of Klippel-Trénaunay-Weber syndrome presenting with portosystemic encephalopathy.

  title={An unusual case of Klippel-Tr{\'e}naunay-Weber syndrome presenting with portosystemic encephalopathy.},
  author={Masahide Yazaki and Kazuma Kaneko and Kana Tojo and Daigo Miyazaki and Yasuhiro Shimojima and Kazuhiko Ueda and Shu-ichi Ikeda},
  journal={Internal medicine},
  volume={47 18},
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… CONTINUE READING