title: Liver transplantation with preservation of the inferior vena cava in case of symptomatic adult polycystic disease.
abstract: Adult polycystic liver disease (APLD) is a rare disorder of the liver parenchyma, the treatment of which is still controversial. Conservative surgery may have a significant morbidity and is often ineffective in the long run. Liver replacement may be indicated in case of incapacitating hepatomegaly. Patients (one male, five females) undergoing liver transplantation for symptomatic APLD is presented in this study. The particular nature of this series is the fact that successful transplantation was performed in all cases with preservation of the recipient's inferior vena cava and without use of veno-venous bypass despite massive hepatomegaly and previous extensive liver surgery (in three cases). There was minimal morbidity and no mortality. All patients have excellent quality of life with a median follow-up of 41 months (range: 12-58) as testified by a median Karnofsky score of 90% (range: 80-100%).
authors.affiliation: Department of Abdominal transplantation, hepatobiliary and endocrine surgery, Liver Transplant Program, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium. lerut@chir.ucl.ac.be
authors.forename: Jan
authors.initials: J
authors.lastname: Lerut
authors.forename: Olga
authors.initials: O
authors.lastname: Ciccarelli
authors.forename: Matthieu
authors.initials: M
authors.lastname: Rutgers
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Orlando
authors.forename: Jules
authors.initials: J
authors.lastname: Mathijs
authors.forename: Etienne
authors.initials: E
authors.lastname: Danse
authors.forename: Eric
authors.initials: E
authors.lastname: Goffin
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Gigot
authors.forename: Pierre
authors.initials: P
authors.lastname: Goffette
mesh_terms: D000328:Adult; D003404:Creatinine / Q000097:blood; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D011788:Quality of Life; D014682:Vena Cava, Inferior / Q000601:surgery*
chemical_list: D003404:Creatinine