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  • Title: Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein.
    Author: Matsuura T, Soejima Y, Taguchi T.
    Journal: Liver Transpl; 2010 Dec; 16(12):1437-9. PubMed ID: 21117255.
    Abstract:
    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.
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