LIVER TRANSPLANTATION (LTx) FOR POLYCYSTIC LIVER DISEASE (PLD).

Abstract

362 Polycystic liver disease (PLD) may provoke massive hepatomegaly, resulting in severe physical-social handicap. Data on the role of liver transplantation (LTx) in the management of PLD are rare and conflictory. Conservative surgery (resection-fenestration) is indicated in large single cysts but its value in small diffuse cysts is questionable. In addition conservative surgery is not devoid of morbidity/mortality. LTx offers the prospect of a fully curative treatment but controversy remains because those patients usually have preserved liver function. Thus, we reviewed our experience with LTx for PLD. 13 adult female patients received a LTx for PLD (small diffuse cysts) between 1990 and 1998. Mean age was 44 yo (36-49). All 13 suffered from combined liver+kidney cystic disease but only I had renal failure and required a combined L and kidney Tx. 12 had preserved kidney function {mean creatinine 1.14 mg/dl (0.91-1.67)} and received a LTx only. Indications for Tx were massive hepatomegaly causing physical handicap (n=13, dyspnea, abdominal pain, vena cava compression, fatigue, dyspepsia), social handicap (n=13), malnutrition (n=2), cholestasis and/or portal hypertension (n=4), associated renal failure (n=1). No marginal liver donors were used. Cold ischemia time was 8.9 hrs (3-15). LTx caused no technical difficulty in 12/13 patients {surgery duration 6.6 hrs (5-8), blood transfusion 7.7 units (0-22)}. One patient who had repeated unsuccessful attempts at liver mass reduction preTx died of massive bleeding and pulmonary emboli. Native liver weight was 5 to 15 kg. PostTx immunosuppression consisted of triple drug therapy with CsA or FK506, imuran and steroid (stopped at 3 mths). All grafts displayed excellent immediate function. Morbidity included biliary stricture (2), revision for bleeding and hepatitis (1), pneumothorax and subphrenic collection (1), bleeding from a tracheostomy (1). There was no post-operative death or graft loss. Both patient and graft survival is 92% (follow-up: 5 mths-8 yrs). Of the 12 patients who received a LTx alone, 1 needed a KTx 4 years postLTx. Kidney function has remained stable in the others, despite the use of CsA or FK506 (last follow-up creatinine: 1.63 mg/dl (0.93-2.85). Despite long-term immunosuppression, LTx had a dramatic impact on daily quality of life enabling these patients to go back to a fully active life style. Conclusion: This is the largest series of LTx for PLD. LTx (or combined kidney + LTx) offers the chance of a definitive treatment in patients with extensive PLD (diffuse small cysts) that has evolved into severely handicapping hepatomegaly. Patient complaints and chances of definitive palliation offered by LTx must be balanced against the risk of Tx and long-term committment to immunosuppression.