Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease

Bowel dysfunction has been proven to be the most typical complication of long-segment Hirschsprung disease (LSHD). Additionally, bowel dysfunction in preschool and early childhood should be properly assessed, because it may result in persistent bowel dysfunction, social issues, and depression in adolescence and adulthood. This study evaluated bowel function during preschool and early childhood in patients with LSHD. A cross-sectional bowel function score (BFS) questionnaire survey was conducted on 270 infants with short-segment Hirschsprung disease (SSHD) and LSHD who underwent pull-through (PT) between January 2014 and December 2019 at the Children's Hospital of Nanjing Medical University. One hundred ninety patients who consented to the survey were asked to complete the questionnaire and were divided into two groups: the LSHD group (n = 42) and the SSHD group (n = 148). Bowel function outcomes were assessed by using a questionnaire. The total BFS score in the LSHD group was significantly lower than that in the SSHD group (15.0 [14.0, 17.0] vs. 18.0 [16.0, 19.0], p < 0.05) and did not improve with age (p > 0.05). Independent BFS items with lower scores were discovered in LSHD than in SSHD, including the capacity to hold back defecation, feeling/reporting the urge to defecate, frequency, soiling, and accidents (all p < 0.05). After subgroup analysis of follow-up age (3-5 years, 5 ~ 7 years, and > 7 years), there was no difference between the score of ability to hold back defecation over 7 years old and feeling/reporting the urge to defecate over 5 years old (all p > 0.05). The frequency and soiling scores were lower in the LSHD group than in the SSHD group over 5 years of age (all p < 0.05). Fortunately, there was no difference in constipation scores and social problems between the LSHD and SSHD groups in every sub-follow-up group (all p > 0.05).  Conclusion: Overall, bowel function at preschool and early childhood age was poorer in LSHD than in SSHD and did not improve with age. However, the ability to hold back defecation and feeling/reporting the urge to defecate with age in LSHD may be similar to those in SSHD. Simultaneously, the frequency, soiling, and number of accidents were consistently worse in the LSHD group.  Trial registration: This study was retrospectively registered in the ClinicalTrials database.gov (NCT05461924) in August 2022. What is Known: • Bowel function in SSHD is satisfactory or near-normal, and the general consensus is that the longer the aganglionic segment, the worse the bowel function after surgery. • Bowel functional outcomes of LSHD were not defined, and the limited reports of bowel functional outcomes compared with SSHD were extensively varied. Bowel dysfunction at preschool and early childhood may lead to persistent bowel dysfunction, social problems, and depression in adolescence or adulthood. What is New: • Bowel function in preschool and early childhood in patients with LSHD has not been defined and should be thoroughly assessed in a larger group of patients with precise definitions of incontinence or soiling. • The present study was primarily designed to evaluate bowel function at preschool and early childhood age in LSHD compared with SSHD in a relatively large number of HD cases using BFS.