The Frequency and Characteristics of Inflammatory Bowel Disease in Pediatric Patients With Hirschsprung's Disease: A Case-Control Study Publisher



Dara N ; Ziba N ; Mohajerzadeh L ; Zarinfar Y ; Dara A ; Hosseini A ; Tajalli S ; Guimaraes NS ; Dooki ME ; Hajipour M ; Imanzade F
Source: Gastroenterology and Endoscopy Published:2025

Abstract

Background: Hirschsprung disease is associated with enterocolitis and inflammatory bowel disease (IBD) as it affects bowel innervation from birth. This study is about the evaluation of IBD in children with diagnosed Hirschsprung disease. Methods: A case-control study was designed between 2012 and 2021, including patients diagnosed with Hirschsprung disease and IBD (case group) and without IBD (control group). Patients were randomly allocated within each group in a ratio of 4 to 1. The 4:1 ratio of controls to cases was chosen to increase the statistical power of the analysis, given the relatively small number of IBD cases within our Hirschsprung's disease patient population at our center. A checklist was used in the collection of clinical and demographic data, and the data were analyzed using SPSS version 25. Results: This study consisted of 87 patients diagnosed with Hirschsprung's disease, which included 17 patients diagnosed with Hirschsprung's disease and IBD (case group) and 70 controls without IBD (control group). The frequency of IBD among diagnosed Hirschsprung patients is 19.5 ​%. The age of all patients at presentation averaged 20.2 ​± ​33.7 months, with more males noted (62.1 ​%). Typical symptoms preceding diagnosis were constipation (76.7 ​%) and abdominal swollen belly (15.1 ​%). In the IBD population, 17.3 ​% were diagnosed with ulcerative colitis and 2.3 ​% with Crohn's disease at a mean age of 47.9 ​± ​45.7 months. Vomiting (31.3 ​%) and constant watery stools (31.3 ​%) were symptoms of IBD. Mean Age, Failure to thrive, and Diarrhea were the only two variables that were statistically significant in females, p ​< ​0.05. Conclusion: The present study showed very significant differences in the clinical features of Hirschsprung disease patients with and without inflammatory bowel disease and highlighted the importance of timely IBD diagnosis and intervention in children to prevent the worsening of the condition. © 2025 Elsevier B.V., All rights reserved.