BILIARY ATRESIA-POLYSPLENIA SYNDROME - SURGICAL AND CLINICAL RELEVANCE IN LIVER-TRANSPLANTATION

Objective To review a single center's 10-year experience with liver tr ansplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-P S) and to define surgical and clinical guidelines for its management. Summary Background Data BA is the most common indication for pediatric liver transplantation (LTx) and is associated with PS in 12% of cases . Only a few studies of LTx for BA-PS have been reported, and the opti mal management of BA-PS patients undergoing LTx has yet to be determin ed. Methods From July 1985 to September 1995, 166 liver transplants we re performed in 130 patients with BA and were included in the study. T he malformations most commonly associated with BA-PS, surgical techniq ues used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial IO-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA. Results Ten patien ts (7.8%) with BA had associated PS. An additional patient with PS wit hout BA was included in the study. The diagnosis of PS was unknown bef ore the transplantation in 72% of cases. Thirteen liver transplants we re performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. Ther e was no association between the type of anomaly and the outcome, nor were there any significant differences in patient survival (72% vs. 73 .5%, p = 0.79) or graft survival (56.4% vs. 54.6%, p = 0.54). Conclusi ons The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding o f two or more of these malformations in a patient awaiting transplanta tion should lead the surgeon to look systematically for other associat ed anomalies. With some special surgical considerations, the outcome i n BA-PS patients should not differ from those with isolated BA.