先天性复杂肛门直肠畸形合并畸形的临床分析

张敏; 孙静; 王至立; 谷成超; 李晓庆; 洪思琦; 王佚

doi:10.16016/j.1000-5404.201802036

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先天性复杂肛门直肠畸形合并畸形的临床分析

临床医学 | 更新时间：2026-01-09

- 先天性复杂肛门直肠畸形合并畸形的临床分析
- Clinical analysis for associated congenital anomalies in congenital complex anorectal malformation
- 第三军医大学学报 2018年40卷第17期页码：1590-1594
- 作者机构：
- 作者简介：
- 基金信息：
- DOI：10.16016/j.1000-5404.201802036
  中图分类号：
- 收稿：2018-02-05，
  
  纸质出版：2018-09-15
- 稿件说明：
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张敏, 孙静, 王至立, 等. 先天性复杂肛门直肠畸形合并畸形的临床分析[J]. 第三军医大学学报, 2018,40(17):1590-1594.

Min ZHANG, Jing SUN, Zhili WANG, et al. Clinical analysis for associated congenital anomalies in congenital complex anorectal malformation[J]. Journal of Third Military Medical University, 2018, 40(17): 1590-1594.
张敏, 孙静, 王至立, 等. 先天性复杂肛门直肠畸形合并畸形的临床分析[J]. 第三军医大学学报, 2018,40(17):1590-1594. DOI： 10.16016/j.1000-5404.201802036.

Min ZHANG, Jing SUN, Zhili WANG, et al. Clinical analysis for associated congenital anomalies in congenital complex anorectal malformation[J]. Journal of Third Military Medical University, 2018, 40(17): 1590-1594. DOI： 10.16016/j.1000-5404.201802036.

摘要

Abstract

Objective

To investigate and summarize the congenital anomalies associated with congenital complex anorectal malformation (ARM) so as to provide the reference for the prognosis evaluation and treatment of the congenital complex ARM children.

Methods

A retrospective study was carried out on 105 children with the congenital complex ARM undergoing staged surgical treatment in our department from January 2012 to December 2015. They were 76 males and 29 females

and freshly diagnosed in 1 to 345 d (median age 2 d) after birth. The pathological types of ARM

associated congenital anomalies and clinical outcomes were collected and analyzed.

Results

① Among the 105 enrolled subjects

103 cases had at least one associated malformation (98.10%)

including 95.35% of cardiac structural deformity (82/86 with freshly echocardiographic diagnosis)

56.19% of genitourinary system malformations (59/105)

29.3% of spinal cord deformities (22/75) and 16.19% of skeletal deformities (17/105). ② For the 86 children with echocardiographic diagnosis for the first time

there were 82 identified

including 44 cases of patent ductus arteriosus (PDA

53.66%)

12 cases of ventricular septal defect (VSD

14.63%) and 45 cases of atrial septal defect (ASD

54.88%). There were still 24 cases with malformations in 6 to 9 months after first-stage surgery. The self-healing rate for PDA

VSD and ASD was 95.45%

41.67% and 66.67%

respectively

with statistical differences in the rate (

0.05). There was no statistical difference in the size of defect in cardiac structural deformity between those with total self-healing of cardiac structural deformity and those without (

0.05). ③ Physical examination and urological ultrasound imaging showed that there were 59 cases with malformations in urinary system among the 105 patients

including 7 cases of hydronephrosis (

10 mm)

7 cases of unilateral renal agenesis

3 cases of fused kidney

6 cases of unilateral renal dysplasia

(10.17%)

and 9 cases of hypospadias (15.25%). And 4 cases of hydronephrosis were recovered in 6 to 9 months after first-stage surgery. ④ There were no significant differences in cardiac structural malformations and combined system malformations between those belonging to high (supralevator) and those to intermediate (partially translevator) and low (translevator) ARMs groups according to Wingspread classification and Wingspread classification (

0.05).

Conclusion

Congenital ARM children are prone to accompany with multiple system malformations

with heart structural malformations most common. Some simple associated malformations have a tendency of self-healing

and they should be closely followed up. For some complex cardiac malformations

such as severe hydronephrosis

tethered cord and other serious complications

close follow-up and timely intervention therapy should be given in clinical practice.

关键词

Keywords

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