Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein.
Congenital absence of the portal vein (CAPV) with an extrahepatic portosystemic shunt is a rare malformation; the completely absent type, Abernethy malformation type I, is especially rare. Liver transplantation for CAPV type I has been recently recognized as the only curative operation, but few reports have been published so far; meanwhile, auxiliary partial orthotopic liver transplantation (APOLT) has been proposed to be a very effective option, especially for pediatric patients. Here we present an 18-year-old adult patient with CAPV, asplenia, and an iliac shunt vessel who was managed successfully with APOLT using a small-for-size graft. To the best of our knowledge, this is the first adult patient who has experienced success with APOLT for CAPV. This is a feasible procedure: it not only fulfills the metabolic demands of the liver for adult patients but also potentially cures CAPV.
Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. matsuura@pedsurg.med.kyushu-u.ac.jp
Toshiharu
T
Matsuura
Yuji
Y
Soejima
Tomoaki
T
Taguchi
D000293:Adolescent; D000013:Congenital Abnormalities / Q000601:surgery*; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D011169:Portal Vein / Q000002:abnormalities*; D016896:Treatment Outcome