title: Living domino liver transplantation in an adult with congenital absence of portal vein.
abstract: 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 microg/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.
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Science, Kumamoto University, 1-1-1 Honjyo, Kumamoto, Japan. ttake@wg7.so-net.ne.jp
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takeichi
authors.forename: Hideaki
authors.initials: H
authors.lastname: Okajima
authors.forename: Hiroko
authors.initials: H
authors.lastname: Suda
authors.forename: Shintarou
authors.initials: S
authors.lastname: Hayashida
authors.forename: Hironori
authors.initials: H
authors.lastname: Iwasaki
authors.forename: Manuel Zeledon
authors.initials: MZ
authors.lastname: Ramirez
authors.forename: Mikako
authors.initials: M
authors.lastname: Ueno
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Asonuma
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Inomata
mesh_terms: D000328:Adult; D000641:Ammonia / Q000097:blood; D017772:Amyloid Neuropathies / Q000601:surgery; D000714:Anastomosis, Surgical; D004322:Drainage; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery; D013152:Splanchnic Circulation; D020858:Tissue and Organ Harvesting / Q000379:methods; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000601:surgery
chemical_list: D000641:Ammonia