Varela-Fascinetto G, Castaldo P, Fox I J, Sudan D, Heffron T G, Shaw B W, Langnas A N
Department of Surgery, University of Nebraska Medical Center, Omaha 68198-3280, USA.
Ann Surg. 1998 Apr;227(4):583-9. doi: 10.1097/00000658-199804000-00022.
To review a single center's 10-year experience with liver transplantation (LTx) for the biliary atresia-polysplenia syndrome (BA-PS) and to define surgical and clinical guidelines for its management.
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 optimal management of BA-PS patients undergoing LTx has yet to be determined.
From July 1985 to September 1995, 166 liver transplants were performed in 130 patients with BA and were included in the study. The malformations most commonly associated with BA-PS, surgical techniques used to overcome these anomalies, and surgical pitfalls that could have contributed to the outcome were characterized. Actuarial 10-year patient and graft survival for patients undergoing LTx for BA-PS were calculated and compared to those with isolated BA.
Ten patients (7.8%) with BA had associated PS. An additional patient with PS without BA was included in the study. The diagnosis of PS was unknown before the transplantation in 72% of cases. Thirteen liver transplants were performed in these 11 patients. Modifications of the usual surgical technique were used to overcome the complex anatomy encountered. There 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).
The association of BA with various anomalies should be considered a spectrum that may vary widely from patient to patient. The finding of two or more of these malformations in a patient awaiting transplantation should lead the surgeon to look systematically for other associated anomalies. With some special surgical considerations, the outcome in BA-PS patients should not differ from those with isolated BA.
回顾单中心10年里对胆道闭锁-多脾综合征（BA-PS）进行肝移植（LTx）的经验，并确定其治疗的外科和临床指南。
BA是小儿肝移植（LTx）最常见的适应证，12%的病例与PS相关。关于BA-PS的LTx仅有少数研究报道，且接受LTx的BA-PS患者的最佳治疗方案尚未确定。
1985年7月至1995年9月，对130例BA患者进行了166例肝移植，并纳入本研究。对最常与BA-PS相关的畸形、用于克服这些异常的手术技术以及可能影响预后的手术陷阱进行了描述。计算接受BA-PS的LTx患者的10年精算生存率和移植物生存率，并与孤立性BA患者进行比较。
10例（7.8%）BA患者合并PS。另有1例无BA的PS患者纳入研究。72%的病例在移植前PS诊断不明。这11例患者共进行了13例肝移植。采用常规手术技术的改良方法来克服遇到的复杂解剖结构。异常类型与预后之间无相关性，患者生存率（72%对73.5%，p = 0.79）或移植物生存率（56.4%对54.6%，p = 0.54）也无显著差异。
BA与各种异常的关联应被视为一个范围，患者之间可能差异很大。在等待移植的患者中发现两种或更多这些畸形应促使外科医生系统地寻找其他相关异常。经过一些特殊的外科考虑，BA-PS患者的预后应与孤立性BA患者无异。
