title: Liver transplantation for massive hepatomegaly due to polycystic liver disease: an extreme case.
abstract: BACKGROUND
  Polycystic liver disease (PLD) is due to a genetic disorder and frequently coexists with polycystic kidney disease (PKD). If the cysts produce symptomatology owing to their number and size, many palliative treatments are available. When none of the liver parenchyma is spared, or kidney insufficiency is marked, the only potentially curable treatment is liver transplantation (LT).
  
  
  METHODS
  A 49-year old woman, diagnosed with PLD and PKD, was listed in January 2008 for combined LT and kidney transplantation (KT). A compatible organ became available 8 months later. Despite preserved liver function, the patient's clinical condition was poor; she experienced dyspnea, advanced anorexia, abdominal pain, and severe ascites. At LT, which took 9 hours and was performed using the classic technique, the liver was hard, massive in size (15.5 kg), and not dissociable from the vena cava. The postoperative course was complicated by many septic episodes, the last one being fatal for the patient at 4 months after transplantation.
  
  
  CONCLUSIONS
  LT for PLD in many series shows a high mortality rate. The Model for End-Stage Liver Disease (MELD) score does not stage patients properly, because liver function is usually preserved. The liver can achieve a massive size causing many symptoms, especially malnutrition and ascites; in this setting LT is the only possible treatment. Patients with a low MELD score undergo LT with severe malnutrition that predisposes them to greater susceptibility to sepsis. To identify predictor factors, beyond MELD criteria that relate to the increased liver volume before development of late symptoms is essential to expeditiously treat patients with the poorest prognosis to improve their outcomes.
authors.affiliation: Hepatobiliary and Liver Transplant Unit, Department of Surgical and Gastroenterologic Sciences, University of Padova, Padova, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Gringeri
authors.forename: F E
authors.initials: FE
authors.lastname: D'Amico
authors.forename: D
authors.initials: D
authors.lastname: Bassi
authors.forename: C
authors.initials: C
authors.lastname: Mescoli
authors.forename: P
authors.initials: P
authors.lastname: Bonsignore
authors.forename: R
authors.initials: R
authors.lastname: Boetto
authors.forename: E
authors.initials: E
authors.lastname: Lodo
authors.forename: G
authors.initials: G
authors.lastname: Noaro
authors.forename: M
authors.initials: M
authors.lastname: Polacco
authors.forename: F
authors.initials: F
authors.lastname: D'Amico
authors.forename: P
authors.initials: P
authors.lastname: Boccagni
authors.forename: G
authors.initials: G
authors.lastname: Zanus
authors.forename: A
authors.initials: A
authors.lastname: Brolese
authors.forename: U
authors.initials: U
authors.lastname: Cillo
mesh_terms: D003560:Cysts / Q000150:complications*; D005260:Female; D006529:Hepatomegaly / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008107:Liver Diseases / Q000150:complications*; D016031:Liver Transplantation; D008875:Middle Aged; D009929:Organ Size; D011184:Postoperative Period