title: Cadaveric liver transplantation in biliary atresia splenic malformation syndrome with the absence of retrohepatic inferior vena cava, preduodenal portal vein, and intestinal malrotation: a case report.
abstract: A 9-month-old female infant with biliary atresia underwent cadaveric liver transplantation due to progressive cholestatic hepatitis following a Kasai operation. She had biliary atresia splenic malformation syndrome (BASM) composed of an absent retrohepatic inferior vena cava with an azygous connection, preduodenal portal vein, polysplenia, and intestinal malrotation. A portal vein thrombosis developed on the 4th postoperative day requiring immediate treatment by thrombectomy. The patient is well with normal liver function at 3 months follow-up. Although BASM may render the transplantation more difficult, the presence of BASM is no longer a contraindication to liver transplantation.
authors.affiliation: Department of General Surgery and Transplantation, Memorial Hospital, Okmeydani, Istanbul, Turkey. ebru.senoran@memorial.com.tr
authors.forename: E
authors.initials: E
authors.lastname: Sen-Oran
authors.forename: Y
authors.initials: Y
authors.lastname: Yankol
authors.forename: B
authors.initials: B
authors.lastname: Tuzun
authors.forename: B
authors.initials: B
authors.lastname: Kocak
authors.forename: T
authors.initials: T
authors.lastname: Kanmaz
authors.forename: K
authors.initials: K
authors.lastname: Acarli
authors.forename: M
authors.initials: M
authors.lastname: Kalayoglu
mesh_terms: D000015:Abnormalities, Multiple; D001656:Biliary Atresia / Q000601:surgery*; D002102:Cadaver; D004386:Duodenum; D005260:Female; D006801:Humans; D007223:Infant; D045822:Intestinal Volvulus; D007422:Intestines / Q000002:abnormalities; D016031:Liver Transplantation; D011169:Portal Vein / Q000002:abnormalities; D013154:Spleen / Q000002:abnormalities; D014019:Tissue Donors; D014682:Vena Cava, Inferior / Q000002:abnormalities