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:
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.
Clinical analysis for associated congenital anomalies in congenital complex anorectal malformation
摘要
Abstract
Objective
2
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
2
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
2
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 (
P
<
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 (
P
>
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 (
P
>
0.05).
Conclusion
2
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
references
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