目的 分析我院2001年到2016年收治的TCA患儿的临床资料,比较TCA患儿不同手术方式的临床效果并总结相关诊治经验。方法 回顾性分析2001年至2016年本院收治的TCA患儿53例,男37例,女16例,均行Ⅱ期根治手术,平均(12.9±3.6)个月。根据手术演变过程及手术方式分为J-pouch、Duhamel、Soave三组,对其术前临床表现、术后并发症以及术后病理、长期随访资料等进行回顾性分析。结果 J-pouch组(2001～2005)13例,男9例,女4例; Duhamel组(2006～2010)12例,男10例,女2例; Soave组(2011～2016)28例,男18例,女10例。Soave手术时间(2.8±0.9)h较J-pouch组(4.1±1.2)h及Duhamel组(3.7±1.1)h明显缩短(F=7.695,P<0.001); Soave组患儿住院时间(8.9±2.3)d较J-pouch组(15.9±2.8)d及Duhamel组(13.9±2.1)d明显缩短(F=41.820,P<0.001)。从并发症来看,术后小肠结肠炎、肛门狭窄的发生率无统计学意义(P>0.05)。肛周糜烂的发生率Soave术明显高于其他两组,差异有统计学意义(χ2=14.770,P<0.001)。长期随访各组部分患儿存在肠功能异常的情况,差异无统计学差异(χ2=0.663,P>0.05)。结论 经腹会阴Soave根治术手术时间及患儿住院时间较短,是一种安全有效的手术方式。长期随访患儿均获得较好的排便功能,远期营养发育基本正常。巨结肠相关性小肠结肠炎(Hirschsprung-associated enterocolitis,HAEC)的发生与术后不同病变段的病理分级具有一定相关性。
Objective This study retrospectively reviewed the clinical data of TCA patients in our hospital from 2001 to 2016 and compared the outcomes of each surgical procedure of TCA. Methods 53 patients(37 males with average age of 12.9±3.6 months)were diagnosed with TCA between 2001 and 2016.All patients were treated with secondary radical operation.According to the methods of operation,the patients were divided into three groups in which the groups were J-pouch,Duhamel and Soave procedures.The relevant data of preoperative clinical manifestations,surgical methods,postoperative complications and long-term outcomes were collected and reviewed. Results Among the 53 cases,13 children(9 males)performed J-pouch procedure from 2001 to 2005 while 12 children(10 males)performed Duhamel procedure from 2006 to 2010 and 28 children(18 males)underwent Soave procedure from 2011 to 2016.The operation time of the Soave group(2.8±0.9 h)was significantly shorter than the J-pouch group(4.1±1.2)h and the Duhamel group(3.7± 1.1)h(F=7.695,P<0.001).The hospitalization time of the Soave group(8.9±2.3)d was significantly shorter than the J-pouch group(15.9±2.8)d and the Duhamel group(13.9±2.1)d(F=41.820,P<0.001).The incidence of perianal erosion in the Soave group was significantly higher than that in the other two groups(χ2=14.770,P<0.001).There was no significant difference in intestinal function among the three groups in the long term(χ2=0.663,P>0.05). Conclusion The trans-perineum Soave radical operation were a relatively safe and effective surgical method with shorter operation time and hospitalization time.Children had better defecation function and their developmental condition was basically normal in a long-term follow-up.A pathological Hirschsprung-associated enterocolitis(HAEC)score for the remaining small intestine segments can probably predict the occurrence of postoperative HAEC.
