目的 分析先天性消化道畸形儿童术后的综合发展情况,为临床长期随访预警体格发育及神经心理发育偏离并及时给予有效干预措施提供科学依据。方法 收集2019—2020年于首都儿科研究所附属儿童医院普通外科住院确诊为胆总管囊肿、先天性巨结肠及胆道闭锁的患儿病例资料,并在术后3个月采用《7岁以下儿童生长标准》、儿童神经心理行为检查量表、婴幼儿生活质量量表及儿童生存质量普适性核心量表分别进行体格、神经心理发育及生活质量评价。分析不同消化道畸形儿童体格发育落后的影响因素及神经心理发育和生活质量状况。结果 共纳入先天性消化道畸形患儿560例。胆总管囊肿为最常见的类型(60.9%),先天性巨结肠并发症发生率最高(37.6%)。与胆总管囊肿相比,先天性巨结肠及胆道闭锁儿童组生长迟缓、低体重检出率更高,差异有统计学意义(χ2=26.721、45.082,P<0.05)。对胆总管囊肿患儿体格发育影响因素进行Logistic回归分析,发现手术年龄<3岁是胆总管囊肿患儿生长迟缓(OR=2.316)的危险因素,并发症的发生(OR=2.410)、多次手术(OR=6.427)是胆总管囊肿患儿低体重的危险因素,母乳喂养是生长迟缓和低体重的保护因素(OR=0.321、0.162)。对先天性巨结肠体格发育影响因素进行Logistic回归分析,发现并发症的发生(OR=2.428、2.025)及早产(OR=5.129、10.319)是先天性巨结肠生长迟缓和低体重的危险因素,母乳喂养(OR=0.260、0.323)是先天性巨结肠体格生长缓慢和低体重的保护因素。胆道闭锁儿童中神经发育迟缓及生活质量受损的比例分别为30.0%(6/20)及41.7%(5/12)。胆道闭锁儿童大运动、适应能力及语言发育落后于胆总管囊肿和先天性巨结肠患儿更为显著,差异有统计学意义(P<0.05)。结论 先天性消化道畸形儿童在体格及神经心理发育方面均存在不同程度减损。因此在外科干预后,更应关注到术后儿童长期的综合发育,针对消化道畸形儿童可能存在的体格发育及神经心理发育问题,建立多学科综合诊治平台,联合保健科、普外科和营养科对患儿进行长期的多学科综合管理和前瞻性随访研究。
Abstract
Objective To analyze the comprehensive development of children with congenital gastrointestinal malformations in postoperative period, in order to provide a scientific basis for alerting growth and developmental deviations during clinical long-term follow-up and giving timely and effective interventions. Methods Case data of children with confirmed diagnosis of choledochal cyst, hirschsprung disease and biliary atresia who were hospitalized in the Department of General Surgery of the Children's Hospital affiliated to the Capital Institute of Pediatrics in 2019 to 2020 were collected. At 3 months postoperatively, physical, neurodevelopmental, and quality of life assessments were conducted among children using the Growth Standards for Chinese Children under 7 Years of Age, Children's Neuropsychological Behavioral Examination Scale(2016 version), the Pediatric Quality of Life Inventory Infant Scale or the Pediatric Quality of Life Inventory(version 4.0), respectively. The influencing factors of physical development delay and the status of neurodevelopment and quality of life among children with different gastrointestinal malformations were analyzed. Results A total of 560 children with congenital gastrointestinal malformations were included in this study. Choledochal cyst was the most common type, accounting for 341 cases (60.9%). Hirschsprung disease had the highest complication rate of 37.6%. The detection rates of growth retardation and low body weight were significantly higher in children with hirschsprung disease and biliary atresia compared to choledochal cyst (χ2=26.721、45.082,P<0.05). For children with choledochal cysts, age at surgery less than 3 years old was a risk factor for growth retardation (OR=2.316), while the occurrence of complications (OR=2.410), multiple surgeries (OR=6.427) were risk factors for low body weight; breastfeeding was a protective factor for growth retardation and low body weight(OR=0.321, 0.162). For children with hirschsprung disease, the occurrence of complications(OR=2.428, 2.025) and premature(OR=5.129, 10.319) were risk factors for growth retardation and low body weight, and breastfeeding(OR=0.260, 0.323) was a protective factor for growth retardation and low body weight. Neurodevelopmental delay and impaired quality of life in children with biliary atresia accounted for 30.0% (6/20) and 41.7% (5/12), respectively. The gross motor, adaptability and language development of children with biliary atresia significantly lagged behind those of children with choledochal cyst, hirschsprung disease (P<0.05). Conclusions Children with congenital gastrointestinal malformations exhibit varying degrees of impairment in both physical and neuropsychological development. Therefore, after surgical intervention, greater attention should be paid to the long-term comprehensive development of these children. In response to the potential growth and neuropsychological development issues in children with gastrointestinal malformations, a multidisciplinary integrated diagnosis and treatment platform should be established, collaborating with departments of health care, general surgery, and nutrition to conduct long-term multidisciplinary comprehensive management and prospective follow-up studies on these patients.
关键词
先天性消化道畸形 /
体格发育 /
神经心理发育 /
生活质量
Key words
congenital gastrointestinal malformations /
physical development /
neuropsychological development /
quality of life
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基金
北京市卫健委高层次公共卫生技术人才建设项目(AL-02-19);北京市医院管理中心第四批“登峰”人才计划(DFL20221103)
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