Bowel Function Score in Long-Term Follow-Up for Children with Hirschsprung Disease: OASIS-Holistic Care in Hirschsprung Disease Network Position Paper

Abstract

Introduction: The assessment of bowel function in patients with Hirschsprung disease (HD) remains controversial, as several different bowel function scores are used in the literature and are therefore not suitable for reliable comparison. Conference Section: The OASIS-Holistic Care in Hirschsprung Disease Network Group addressed this issue and evaluated the most commonly used scores for their utility in HD patients. Scoping Review and Expert Group Consensus were performed. It was agreed that the ideal score for patients with Hirschsprung disease should be a comprehensive, validated score that allows reliable assessment of bowel function and continence, depicts changes according to management and natural history, and quantifies baseline parameters. This score should also enable comparisons of bowel function in patients born with HD worldwide. Concluding Remarks: The OASIS-Holistic Care in Hirschsprung Disease Network Group agreed that this ideal score does not yet exist and is therefore a future goal of the group. Meanwhile, clinicians should use a score for comparable, standardized, objective assessment, and the Rintala Score is suggested. Future developments may also lead to easily accessible patient-reported outcome measures and semi-automated dashboards that allow automated data extraction from electronic health records.

Keywords

• Hirschsprung disease
• continence score
• scoring postoperative results
• incontinence
• bowel function
• patient-reported outcome measurements (PROM)