先天性复杂肛门直肠畸形合并脊髓发育异常患儿排便功能的临床研究

赵焓滨; 侯金平; 侯金凤; 迭小红; 孙静; 张敏; 王佚

doi:10.16016/j.1000-5404.201812104

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先天性复杂肛门直肠畸形合并脊髓发育异常患儿排便功能的临床研究

临床医学 | 更新时间：2026-01-09

- 先天性复杂肛门直肠畸形合并脊髓发育异常患儿排便功能的临床研究
- Defecatory function in children with congenital complex anorectal malformations complicated by spinal cord dysplasia
- 第三军医大学学报 2019年41卷第12期页码：1167-1173
- 作者机构：
  
  400014 重庆，重庆医科大学附属儿童医院胃肠新生儿外科, 儿童发育疾病研究教育部重点实验室，儿童发育重大疾病国家国际科技合作基地，儿科学重庆市重点实验室
- 作者简介：
  
  [王佚，E-mail:wy757311@hotmail.com WANG Yi, E-mail: wy757311@hotmail.com
- 基金信息：
- DOI：10.16016/j.1000-5404.201812104
  中图分类号： R334;R726.571;R744
- 收稿：2018-12-12，
  
  修回：2019-02-13，
  
  纸质出版：2019-06-30
- 稿件说明：
移动端阅览
赵焓滨, 侯金平, 侯金凤, 等. 先天性复杂肛门直肠畸形合并脊髓发育异常患儿排便功能的临床研究[J]. 第三军医大学学报, 2019,41(12):1167-1173.

Hanbin ZHAO, Jinping HOU, Jinfeng HOU, et al. Defecatory function in children with congenital complex anorectal malformations complicated by spinal cord dysplasia[J]. Journal of Third Military Medical University, 2019, 41(12): 1167-1173.
赵焓滨, 侯金平, 侯金凤, 等. 先天性复杂肛门直肠畸形合并脊髓发育异常患儿排便功能的临床研究[J]. 第三军医大学学报, 2019,41(12):1167-1173. DOI： 10.16016/j.1000-5404.201812104.

Hanbin ZHAO, Jinping HOU, Jinfeng HOU, et al. Defecatory function in children with congenital complex anorectal malformations complicated by spinal cord dysplasia[J]. Journal of Third Military Medical University, 2019, 41(12): 1167-1173. DOI： 10.16016/j.1000-5404.201812104.

摘要

目的

探讨脊髓发育异常与先天性复杂肛门直肠畸形(anorectal malformations

ARMs)的关系及其对排便功能的影响。

方法

回顾性分析2012年10月至2017年2月我科148例经分期手术治疗的ARMs患儿的临床资料，按腰骶部MRI检查结果分为脊髓发育正常组和脊髓发育异常组，分析脊髓发育异常在各类型ARMs中分布的差异及对肛门直肠周围肌肉发育情况、肛门直肠测压和术后排便功能的影响。

结果

① 本组148例ARMs患儿中34例(23.0%)检查结果提示脊髓发育异常，包括终丝脂肪变21例(61.8%)、椎管内脂肪瘤7例(20.6%)、脊髓空洞1例(2.9%)、椎管内囊性占位3例(8.8%)、混合型2例(5.9%)；②按照Krickenbeck国际分类法对患儿的肛门直肠畸形进行分型，合并有脊髓发育异常的病例包括肛管直肠狭窄1例(占20.0%)、ARMs不伴瘘管5例(25.0%)、ARMs伴复杂直肠会阴瘘1例(20.0%)、ARMs伴直肠尿道球部瘘8例(22.2%)、ARMs伴直肠尿道前列腺部瘘6例(18.2%)、ARMs伴直肠膀胱颈部瘘1例(25.0%)、ARMs伴直肠舟状窝瘘8例(24.2%)、ARMs伴直肠阴道瘘2例(66.7%)、一穴肛2例(22.2%)，各病理类型组间脊髓发育异常比例差异无统计学意义(

=0.874)；③145例患儿在术中进行了肛门直肠周围肌肉发育情况评估，其中有26例肛门直肠周围肌肉发育好、105例发育良、14例发育差，脊髓正常组和异常组间肛门直肠周围肌肉发育差异有统计学意义(

=0.005)；④44例患儿关瘘术6个月后行肛门直肠测压，脊髓发育正常组和异常组肛管静息压分别为(35.0±14.8)、(27.9±14.3)mmHg(

=0.268)，脊髓发育正常组直肠肛管抑制反射(rectoanal inhibitory reflex

RAIR)引出率低于脊髓发育异常组(45.9%

71.4%)，但差异无统计学意义(

=0.412)；⑤按修正的Rintal评分量表随访患儿的排便功能，脊髓发育正常组和异常组间排便功能构成比差异无统计学意义(

=0.416)。

结论

脊髓发育异常与ARMs肛周肌肉发育情况有关，提示ARMs患儿均应常规行腰骶部MRI检查以明确是否合并先天性脊髓发育异常。

Abstract

Objective

To explore the relationship between spinal cord dysplasia and congenital complex anorectal malformations (ARMs) and how they affect the defecatory function in children.

Methods

We retrospectively analyzed the clinical data of 148 children undergoing staged surgeries for complex ARMs between October

2012 and February

2017. According to the findings by lumbosacral magnetic resonance imaging (MRI)

the children were divided into normal spinal cord group and abnormal spinal cord development group to analyze the distribution of spinal cord dysplasia in different types of ARMs and the impact of anomalies in spinal cord development on the development of the perianal muscles

anorectal manometry

and the defecatory function.

Results

Of the 148 children

34 (23.0%)

showed anomalies in spinal cord development

including steatosis of the terminal filament in 21 (61.8%) cases

intraspinal lipoma in 7 (20.6%) cases

syringomyelia in 1 (2.9%) case

intraspinal cystic lesion in 3 (8.8%) cases

and mixed spinal cord anomaly in 2 (5.9%) cases. We classified AMRs into different types according to Krickenbeck international classification

and abnormal spinal cord development was found in 20.0% (1/5) of the patients with rectal stenosis

25.0% (5/20) of the patients with rectal atresia

20.0% (1/5) of the patients with perineal fistula

22.2% (8/36) of the patients with rectobulbar fistula

18.2% (6/33) of the patients with rectoprostatic fistula

25.0% (1/4) of the patients with rectovesical fistula

24.2% (8/33) of the patients with vestibular fistula

66.7%(2/3) of the patients with rectovaginal fistula

and 22.2% (2/9) of the patients with cloaca; No significant difference was found in the incidences of abnormal spinal cord development among the 9 groups (

=0.874). A total of 145 patients were evaluated for perianal muscle development during the surgery

and 26 patients had excellent

105 had good

and 14 had poor development; the development of perianal muscles differed significantly between the children with normal and those with abnormal spinal cord development (

=0.005). Forty-four patients received anorectal manometry

which Showing no significant difference in resting anal pressure between the children with normal and abnormal spinal cord development (35.0±14.8

27.9±14.3 mmHg

=0.268); the positivity rate of rectoanal inhibitory reflex (RAIR) was lower in normal spinal cord group than in spinal cord dysplasia group (45.9%

71.4%)

but the difference was not statistically significant (

=0.412). Follow-up of the patients for defecatory function assessment using the modified Rintal scale showed no significant difference in the constituent ratios of defecatory function bet

ween the 2 groups (

=0.416).

Conclusion

Spinal cord dysplasia is associated with perianal muscle development in children with complex ARMs

for whom routine lumbosacral MRI should be performed to detect the presence of spinal cord dysplasia.

关键词

Keywords

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