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Living domino liver transplantation in an adult with congenital absence of portal vein
Author(s) -
Takeichi Takayuki,
Okajima Hideaki,
Suda Hiroko,
Hayashida Shintarou,
Iwasaki Hironori,
Ramirez Manuel Zeledon,
Ueno Mikako,
Asonuma Katsuhiro,
Inomata Yukihiro
Publication year - 2005
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.20561
Subject(s) - medicine , liver transplantation , surgery , hepatic encephalopathy , transplantation , inferior vena cava , cuff , encephalopathy , vein , polysplenia , portal vein thrombosis , portal vein , cirrhosis , situs inversus
Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35‐year‐old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 μg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end‐to‐end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life. (Liver Transpl 2005;11:1285–1288.)