[1]臧婧羽,袁小建,程思旸,等.单中心不同手术方式治疗全结肠型巨结肠疗效分析[J].临床小儿外科杂志,2018,17(02):106-111.
　Zang Jingyu,Yuan Xiaojian,Cheng Siyang,et al.The efficacy analysis of different operative methods in treating Total Colonic Aganglionosis (TCA) in a Single Center.[J].Journal of Clinical Pediatric Surgery,2018,17(02):106-111.
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单中心不同手术方式治疗全结肠型巨结肠疗效分析

《临床小儿外科杂志》[ISSN:1671-6353/CN:43-1380/R] 卷: 第17卷 期数: 2018年02期 页码: 106-111 栏目: 专题讨论 出版日期: 2018-02-28
Title:
The efficacy analysis of different operative methods in treating Total Colonic Aganglionosis (TCA) in a Single Center.
作者:
臧婧羽; 袁小建; 程思旸; 等
上海交通大学医学院附属新华医院小儿外科，国家临床重点专科
Author(s):
Zang Jingyu1; 2; Yuan Xiaojian2; Cheng Siyang1; Hong Zhihua2; Wang Jun1; 2; Chen Jie1; 2.
1.Department of Pediatric Surgery,Xinhua Hospital of Jiaotong University School of Medicine,Shanghai 200092 China.
关键词:
全结肠巨结肠; Ｈｉｒｓｃｈｓｐｒｕｎｇ病; 外科手术; 小肠结肠炎
Keywords:
Total Colonic Aganglionosis;  Hirschsprung Disease;  Surgical Procedures; Operative;  Enterocolitis
文献标志码:
A
摘要:
目的　分析我院２００１年至２０１６年收治的ＴＣＡ患儿的临床资料，比较ＴＣＡ患儿不同手术
方式的临床疗效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，均行二期根治手术，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ａｓｓｏｃｉａｔｅｄｅｎｔｅｒｏｃｏｌｉｔｉｓ，ＨＡＥＣ）的发生与术
后不同病变段的病理分级具有一定相关性。
Abstract:
ObjectiveThis 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.Methods53 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 which 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.ResultsAmong 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).ConclusionThe 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.

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