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Liver Transplantation for Polycystic Liver Disease. The University Hospital Essen experience

Thursday, 25 November, 2010 - 09:10
Group 3 - 1

Εισαγωγή

Objective: Polycystic liver disease (PLD), commonly associated with polycystic kidney disease, can result in massive hepatomegaly leading to abdominal distension, pain and debilitating symptoms. Hepatic failure is uncommon. Surgical interventions have been associated with significant morbidity and inconsistent long-term palliation. Liver transplantation (LTX) offers curative treatment, but its relevance as treatment modality is controversy, considering the absence of liver failure, organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD.

Material and Methods: Nineteen adults (16 females and 3 males) underwent LTX for diffuse PLD between 1992 and 2009. Mean age was 45 years. All of the patients had combined liver and kidney disease, but only eight required combined liver and kidney transplantation. Indications for transplantation were massive hepatomegaly, malnutrition, cholestasis and portal-hypertension. Eight patients underwent livermass reduction pre-LTX. Native liver weight was 6-15 Kg. Two patients received a right split liver graft.

Results: One patient who received a split liver graft died intraoperatively of bleeding due to massive portal-hypertension. Three patients died between 4th and 30th postoperative day due to sepsis and multiple-organ-failure. At present eleven patients are alive, relieved of symptoms and with good graft function (follow-up 6 months to 12 years).

Conclusions: Our experience demonstrates that PLD-patients with extensive hepatic involvement treated by LTX have good long-term prognosis and excellent symptoms relief. LTX might be considered in severe PLD-cases und must be balanced against the risks of LTX and lifelong commitment to immunosuppression, where conventional surgery is not a curative option.

Full Text

Objective: Polycystic liver disease (PLD), commonly associated with polycystic kidney disease, can result in massive hepatomegaly leading to abdominal distension, pain and debilitating symptoms. Hepatic failure is uncommon. Surgical interventions have been associated with significant morbidity and inconsistent long-term palliation. Liver transplantation (LTX) offers curative treatment, but its relevance as treatment modality is controversy, considering the absence of liver failure, organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD.

Material and Methods: Nineteen adults (16 females and 3 males) underwent LTX for diffuse PLD between 1992 and 2009. Mean age was 45 years. All of the patients had combined liver and kidney disease, but only eight required combined liver and kidney transplantation. Indications for transplantation were massive hepatomegaly, malnutrition, cholestasis and portal-hypertension. Eight patients underwent livermass reduction pre-LTX. Native liver weight was 6-15 Kg. Two patients received a right split liver graft.

Results: One patient who received a split liver graft died intraoperatively of bleeding due to massive portal-hypertension. Three patients died between 4th and 30th postoperative day due to sepsis and multiple-organ-failure. At present eleven patients are alive, relieved of symptoms and with good graft function (follow-up 6 months to 12 years).

Conclusions: Our experience demonstrates that PLD-patients with extensive hepatic involvement treated by LTX have good long-term prognosis and excellent symptoms relief. LTX might be considered in severe PLD-cases und must be balanced against the risks of LTX and lifelong commitment to immunosuppression, where conventional surgery is not a curative option.

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