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 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.
Yazaki
Masahide
M
Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto. mayazaki@hsp.md.shinshu-u.ac.jp
Kaneko
Kazuma
K
Tojo
Kana
K
Miyazaki
Daigo
D
Shimojima
Yasuhiro
Y
Ueda
Kazuhiko
K
Ikeda
Shu-ichi
S
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