title: Successful hepatic transplantation in congenital absence of recipient portal vein.
abstract: The first case of hepatic transplantation in a patient with congenital absence of the portal vein (CAPV) is reported. A 10-year-old girl with biliary atresia and CAPV underwent successful hepatic transplantation and has normal liver function 9 months after transplantation. This case is only the seventh reported case of CAPV. Patients with CAPV commonly have additional liver anomalies, cardiac and inferior vena cava anomalies, and polysplenia. Surprisingly, hepatic encephalopathy is not a prominent feature in patients with CAPV despite systemic drainage of mesenteric venous blood. CAPV should not be considered a contraindication to hepatic transplantation.
authors.affiliation: Department of Surgery, University of Chicago Pritzker School of Medicine, Ill.
authors.forename: E S
authors.initials: ES
authors.lastname: Woodle
authors.forename: J R
authors.initials: JR
authors.lastname: Thistlethwaite
authors.forename: J C
authors.initials: JC
authors.lastname: Emond
authors.forename: P F
authors.initials: PF
authors.lastname: Whitington
authors.forename: P
authors.initials: P
authors.lastname: Vogelbach
authors.forename: D K
authors.initials: DK
authors.lastname: Yousefzadeh
authors.forename: C E
authors.initials: CE
authors.lastname: Broelsch
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D001656:Biliary Atresia / Q000150:complications; D002648:Child; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications; D016031:Liver Transplantation; D011169:Portal Vein / Q000002:abnormalities*; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed