title: Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure.
abstract: Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.
authors.affiliation: Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya 466-8560, Japan. wsumida@med.nagoya-u.ac.jp <wsumida@med.nagoya-u.ac.jp>
authors.forename: Wataru
authors.initials: W
authors.lastname: Sumida
authors.forename: Kenitiro
authors.initials: K
authors.lastname: Kaneko
authors.forename: Yukio
authors.initials: Y
authors.lastname: Ogura
authors.forename: Takahisa
authors.initials: T
authors.lastname: Tainaka
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Ono
authors.forename: Takahiko
authors.initials: T
authors.lastname: Seo
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Kiuchi
authors.forename: Hisami
authors.initials: H
authors.lastname: Ando
mesh_terms: D018376:Cardiovascular Abnormalities / Q000150:complications*; D004065:Digestive System Abnormalities / Q000150:complications; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors; D011169:Portal Vein / Q000002:abnormalities*