Systematic Review on Questionnaires Reporting Bowel Function and Health-related Quality of Life in Patients With Hirschsprung Disease

Aim: Impaired bowel function and reduced health-related quality of life (HR-QoL) are common after surgery for Hirschsprung disease (HD). There is yet no agreement on how to report these outcomes. This systematic review aims to describe which questionnaires, their properties, and how frequently they are used to report outcomes. Based on this, we recommend questionnaires to be used in future studies. Methods: Predetermined searches were conducted in Pubmed, Embase, Cochrane Library and Trials, Ovid Medline, and Cinahl with no lower time limit until March 2024. The Covidence software was used in data collection, eligibility criteria and PRISMA guidelines were applied, and the review was PROSPERO-registered. Results: Of 2925 identified studies, 357 (12 %) met inclusion criteria. Bowel function was reported in 350 studies with 232 different questionnaires. HR-QoL was assessed with 32 different questionnaires in 80 studies. Of the questionnaires, nine (4 %) reporting bowel function and 13 (41 %) reporting HR-QoL questionnaires were validated. The most commonly used bowel function questionnaires were non-validated, developed for patients with anorectal malformations, and without a control population. Most HR-QoL questionnaires were validated and developed for the general population. Conclusion: Questionnaires to record bowel function in HD patients are mostly non-validated, whereas use of validated questionnaires is the norm when reporting HR-QoL. We recommend either HAQL, BFS or PICS to report bowel function. PedsQL Generic Core Scale and PedsQL Gastrointestinal Symptom Scale or HAQL, and either SF-36 or QLI in combination with GIQLI or HAQL is recommended to report HR-QoL in HD children and adults, respectively. Level of Evidence: IV.