A systematic review of school functioning in pediatric patients with Hirschsprung disease

To better target future educational outcomes of pediatric patients with Hirschsprung disease (HSCR), we conducted a systematic review without meta-analysis on the impact of HSCR on school functioning (SF). PubMed, Embase, Scopus, Web of Science, Cochrane, PsycINFO, and ERIC were searched from inception to December 2023, following the systematic reviews of PRISMA guidelines. Original studies where SF was measured as one of the outcomes were identified by searching relevant databases and manual searching. Data describing the impact of HSCR on SF were extracted. Screening, data extraction, and quality assessment were performed by at least two authors. 2042 articles were identified, 1300 records were screened after removal of duplicates. Forty records were assessed for eligibility and twelve studies which evaluated the SF in children between the ages of 6 and 17 with HSCR were analyzed. Among the twelve studies, five were retrospective cohort studies, one prospective cohort study, one longitudinal cohort study, and five case-control studies. Five studies measured general SF including attention, memory, schoolwork, and missing school. Among these five studies, three showed domain scores for SF lower than matched healthy controls or the reference normative population. Additionally, peer relationship problems/peer rejection/bullying as a specific area of SF were reported in another five studies using three different assessment instruments. Other specific aspects of SF such as school absences, school environment, school attitude, school performance, interpersonal relationships, and education services were assessed by different generic or disease-specific scales in the included studies. The lack of data measuring individual aspects made it hard to conclude the effects of HSCR in these aspects. HSCR negatively impacted general SF, especially in peer relationships. Due to the lack of studies utilizing standardized measures of SF on HSCR children, a multicenter larger sample size and longitudinal study is urgently needed, to provide robust evidence and to help inform targeted strategies to optimize educational outcomes for students with HSCR.