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.
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.
Sen-Oran
E
E
Department of General Surgery and Transplantation, Memorial Hospital, Okmeydani, Istanbul, Turkey. ebru.senoran@memorial.com.tr
Yankol
Y
Y
Tuzun
B
B
Kocak
B
B
Kanmaz
T
T
Acarli
K
K
Kalayoglu
M
M
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