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

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.

Key words: congenital gastrointestinal malformations, physical development, neuropsychological development, quality of life