Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein

✍ Scribed by Toshiharu Matsuura; Yuji Soejima; Tomoaki Taguchi

• Publisher: John Wiley and Sons
• Year: 2010
• Tongue: English
• Weight: 142 KB
• Volume: 16
• Category: Article
• ISSN: 1527-6465
• DOI: 10.1002/lt.22179

✦ Synopsis

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.