Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver.
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.
Tanaka
Yasuo
Y
Department of Gastroenterology, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Takayanagi
Masaki
M
Shiratori
Yasushi
Y
Imai
Yasuo
Y
Obi
Shuntaro
S
Tateishi
Ryosuke
R
Kanda
Miho
M
Fujishima
Tomonori
T
Akamatsu
Masatoshi
M
Koike
Yukihiro
Y
Hamamura
Keisuke
K
Teratani
Takuma
T
Ishikawa
Takashi
T
Shiina
Shuichiro
S
Kojiro
Masamichi
M
Omata
Masao
M
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