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浙江大学学报(医学版)  2019, Vol. 48 Issue (5): 487-492    DOI: 10.3785/j.issn.1008-9292.2019.10.04
专题报道     
Ⅲ-b型先天性肠闭锁患儿的治疗
马东(),赖登明,赵晓霞,胡书奇,吕成杰,黄寿奖,秦琪,钭金法*()
浙江大学医学院附属儿童医院 国家儿童健康与疾病临床医学研究中心, 浙江 杭州 310052
Therapeutic experience of type Ⅲ-b congenital intestinal atresia
MA Dong(),LAI Dengming,ZHAO Xiaoxia,HU Shuqi,LYU Chengjie,HUANG Shoujiang,QIN Qi,TOU Jinfa*()
The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China
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摘要:

目的: 总结Ⅲ-b型先天性肠闭锁患儿的临床特点和治疗经验。方法: 回顾性分析浙江大学医学院附属儿童医院新生儿外科2015年1月至2017年12月收治的12例Ⅲ-b型先天性肠闭锁患儿的临床资料,总结其临床特点和治疗经验。结果: 术中明确诊断为Ⅲ-b型先天性肠闭锁患儿12例,其中2例患儿的家属术中放弃治疗。1例患儿初次手术时仅对闭锁近端小部分肠管进行了裁剪成形,术后出现肠梗阻,再次剖腹探查确诊为十二指肠扩张伴有肠动力异常,行全十二指肠裁剪成形术;1例患儿因远端正常肠管较长,予部分苹果皮样肠管切除,未行肠系膜成形术,剩余8例患儿均行全十二指肠裁剪成形术和肠系膜成形术。1例患儿家属术后早期放弃治疗,1例患儿术后因复杂性先天性心脏病死亡,5例患儿术后合并短肠综合征。8例存活的患儿术后均接受肠外营养支持治疗,其中5例患儿肠外营养支持时间超过42 d,出院后继续随访1~3年,近期治疗效果满意。结论: Ⅲ-b型先天性肠闭锁患儿术中应尽量保留远端苹果皮样肠管并行肠系膜成形;对近端扩张肠管进行裁剪成形,术后予营养支持,可促进残留肠管的代偿和肠适应。

关键词: 肠闭锁/外科学肠梗阻/先天性短肠综合征/并发症短肠综合征/外科学胃肠外营养回顾性研究    
Abstract:

Objective: To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA). Methods: The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively. Results: Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory. Conclusions: For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.

Key words: Intestinal atresia/surgery    Intestinal obstruction/congenital    Short bowel syndrome/complications    Short bowel syndrome/surgery    Parenteral nutrition    Retrospective studies
收稿日期: 2019-07-30 出版日期: 2020-01-04
:  R726  
通讯作者: 钭金法     E-mail: madongdr@zju.edu.cn;toujinfa@zju.edu.cn
作者简介: 马东(1988—), 男, 硕士, 住院医师, 主要从事新生儿肠闭锁研究; E-mail: madongdr@zju.edu.cn; https://orcid.org/0000-0001-6291-8448
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马东
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秦琪
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引用本文:

马东,赖登明,赵晓霞,胡书奇,吕成杰,黄寿奖,秦琪,钭金法. Ⅲ-b型先天性肠闭锁患儿的治疗[J]. 浙江大学学报(医学版), 2019, 48(5): 487-492.

MA Dong,LAI Dengming,ZHAO Xiaoxia,HU Shuqi,LYU Chengjie,HUANG Shoujiang,QIN Qi,TOU Jinfa. Therapeutic experience of type Ⅲ-b congenital intestinal atresia. J Zhejiang Univ (Med Sci), 2019, 48(5): 487-492.

链接本文:

http://www.zjujournals.com/med/CN/10.3785/j.issn.1008-9292.2019.10.04        http://www.zjujournals.com/med/CN/Y2019/V48/I5/487

图 1  Ⅲ-b型先天性肠闭锁术中及术后图片
例序肠闭锁部位近端非苹果皮样肠管长度(cm)保留肠管总长度(cm)肠管裁剪肠系膜成形并发症住院时长(d)转归出院后3个月体质量
“—”:无相关资料.
1十二指肠02术中放弃,死亡
2回肠7513520康复出院正常
3空肠35术中放弃,死亡
4十二指肠060肠梗阻、短肠综合征77康复出院正常
5十二指肠0402术后当天死亡
6空肠3409术后第8天家属放弃治疗,死亡
7空肠2035短肠综合征50康复出院偏低
8十二指肠06527康复出院正常
9十二指肠06521康复出院正常
10十二指肠050短肠综合征56康复出院正常
11十二指肠040胆汁淤积、短肠综合征70康复出院偏低
12十二指肠050胆汁淤积、短肠综合征50康复出院正常
表 1  12例Ⅲ-b型先天性肠闭锁患儿手术、转归及随访情况一览
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