title: Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver.
abstract: Congenital absence of the portal vein is an extremely rare anomaly, in which enteric blood bypasses the liver and drains into the inferior vena cava. A 16-year-old girl was referred to our hospital presenting with liver tumor. Although she had suffered from galactosemia soon after birth, the galactosemia had improved spontaneously 1 year later. Between the ages of 8 and 12 years, chronic hepatitis with a mild elevation of aspartate transaminase (AST) and alanine transaminase (ALT) was observed, but liver tumor had not been detected on computed tomography (CT) in regular medical examinations. However, at age 16, liver tumors, 10 cm in diameter, were found. Abdominal angiography indicated complete absence of the portal vein, suggesting that enteric blood was bypassing the liver and draining into the inferior vena cava. In biopsy specimens obtained under ultrasonographic guidance, liver tumors were confirmed histologically as hyperplastic nodules. In addition to this case report, the clinical features of 25 reported cases of congenital absence of the portal vein are reviewed.
authors.affiliation: Department of Gastroenterology, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Masaki
authors.initials: M
authors.lastname: Takayanagi
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Shiratori
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Imai
authors.forename: Shuntaro
authors.initials: S
authors.lastname: Obi
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Tateishi
authors.forename: Miho
authors.initials: M
authors.lastname: Kanda
authors.forename: Tomonori
authors.initials: T
authors.lastname: Fujishima
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Akamatsu
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Koike
authors.forename: Keisuke
authors.initials: K
authors.lastname: Hamamura
authors.forename: Takuma
authors.initials: T
authors.lastname: Teratani
authors.forename: Takashi
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Shuichiro
authors.initials: S
authors.lastname: Shiina
authors.forename: Masamichi
authors.initials: M
authors.lastname: Kojiro
authors.forename: Masao
authors.initials: M
authors.lastname: Omata
mesh_terms: D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000175:diagnosis*; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology; D011859:Radiography