title: Congenital absence of portal venous system associated with a large inferior mesenteric-caval shunt: a case report.
abstract: We encountered a 30-year-old man with congenital absence of the portal venous system associated with a large inferior mesenteric-caval shunt via the internal iliac veins, which was revealed by angiography and liver biopsy. He had experienced refractory hemorrhoidal bleeding and occasional dyspneic attacks, but no episode of portal encephalopathy. The serum ammonia level in peripheral blood was within normal limits, which suggests the presence of a homeostatic control mechanism. The clinical profile of this extremely rare case, including liver function, is described.
authors.affiliation: Department of Surgery, Tohkai Hospital, Nagoya, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Komatsu
authors.forename: M
authors.initials: M
authors.lastname: Nagino
authors.forename: N
authors.initials: N
authors.lastname: Hayakawa
authors.forename: H
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Y
authors.initials: Y
authors.lastname: Nimura
mesh_terms: D000328:Adult; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D007084:Iliac Vein / Q000002:abnormalities*; D008099:Liver / Q000098:blood supply; D008297:Male; D008642:Mesenteric Veins / Q000002:abnormalities*; D011169:Portal Vein / Q000002:abnormalities*; D011859:Radiography; D014682:Vena Cava, Inferior / Q000002:abnormalities*