title: An unusual case of Klippel-Trénaunay-Weber syndrome presenting with portosystemic encephalopathy.
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.
authors.affiliation: Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto. mayazaki@hsp.md.shinshu-u.ac.jp
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.forename: Kazuma
authors.initials: K
authors.lastname: Kaneko
authors.forename: Kana
authors.initials: K
authors.lastname: Tojo
authors.forename: Daigo
authors.initials: D
authors.lastname: Miyazaki
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shimojima
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Ueda
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000792:Angiography; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D019339:Port-Wine Stain / Q000209:etiology; D014057:Tomography, X-Ray Computed