胆道闭锁Kasai手术后自体肝存活20年以上研究

20-year transplant-free survival of biliary atresia after Kasai operation

目的通过分析胆道闭锁患儿Kasai术后自体肝存活超过20年患者的病例特点，总结影响其远期预后的相关因素。方法对自1989年3月至1993年3月在本医疗中心诊断为胆道闭锁并行手术治疗的患儿37例进行回顾性研究，其中男22例，女15例，手术日龄在60d以内、61～80d、81～100d、100d以上的患儿分别为6例、14例、14例和3例。所有患儿均为Ⅲ型胆道闭锁。胆道闭锁患儿术后自体肝存活超过20年的患儿共有11例，其中男7例，女4例。分析并比较其远期自体肝存活率、性别、手术年龄、术后早期胆管炎的发作、Kasai术后肝门部胆管梗阻再手术等因素。结果手术Et龄在60d以内、61～80d、81～100d、100d以上手术患儿长期生存分别为3例、4例、3例和1例，按手术年龄相比较，远期自体肝存活率的差异无统计学意义（x^2值=1．669，P=0．644）。26例在术后1个月内发生早期胆管炎，其中长期生存5例；11例患儿术后1个月内无早期胆管炎，长期生存6例，按术后早期胆管炎的发作相比较，远期自体肝存活率的差异有统计学意义（x^2值=4．614，P=0．032）。肝门部胆管梗阻再次肝门空肠吻合手术7例，长期生存2例。结论Kasai手术是治疗胆道闭锁的一种有效手段，术后部分患儿可获得长期生存。手术年龄对远期预后有影响，但在一定年龄范围内（〈100d），手术年龄并不是决定预后的最重要因素。术后早期胆管炎是影响胆道闭锁患儿Kasai手术，长期自体肝存活的主要因素之一，并可能引起肝门部胆管梗阻，可显著降低患儿远期生存率。胆道闭锁Kasai术后出现肝门部胆管梗阻的患儿，行再次手术仍有远期存活的可能。

Objective To review the clinical features of BA （biliary atresia） patients surviving over 20 years after Kasai operation without liver transplantation and summarize their prognostic factors. Methods A total of 37 BA patients diagnosed and surgically treated at our center from March 1989 to March 1993 were studied retrospectively. There were 22 males and 15 females. Their operative ages were 〈60 （n = 6）,61-80 （n = 14）,81-100 （n = 14） and 〉100 （n = 3） days respectively. All of them were classified as type III BA. Among them, 7 males and 4 females survived over 20 years without transplantation. Their long-term transplant-flee survival rate, gender, operative age, occurrence of early postoperative cholangitis and hilar bile duct obstruction after Kasai operation were analyzed. Results The operativeagewas 〈60 （n=3）,61-80 （n=4）,81-100 （n=3） and ≥100 （n= 1） days. The operative age had no statistic significance toward long-term transplant-flee survival（x^2 = 1. 669, P = 0. 644）. In 26 patients, cholangitis occurred within 1 month, 5 patients with early cholangitis achieved long-term survival. The other 11 patients did not experience early cholangitis, and 6 of these patients obtained long-term survival. There was statistical significance between the patients with or without early cholangitis （X^2 = 4. 614, P = 0. 032）. Seven patients underwent reoperation with portojejunal anastomosis for hilar bile duct obstruction, in which 2 patients obtained long-term survival. Conclusions The Kasai procedure is an effective method for treatment of BA, some patients treated with Kasai operation can obtain long-term survival. The operative age influenced prognosis within a certain age （〈100 days） and it was not the most significant prognostic factor. Early cholangitis after Kasai operation is one of major prognostic factors of determining long-term survival. Early cholangitis can also cause hilar bile duct obstruction so as to significantly reduce long-term survival rate. However, reoperation for hilar bile duct obstruction may still lead to long-term survival.