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

Judith Lindert; Anders Telle Hoel; Eberhard Schmiedeke; Joe I Curry; Stavros Loukogeorgakis; Eva Amerstorfer

doi:10.3390/children11111284

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

Children (Basel). 2024 Oct 24;11(11):1284. doi: 10.3390/children11111284.

Authors

Judith Lindert^{1

2}, Anders Telle Hoel³, Eberhard Schmiedeke⁴, Joe I Curry², Stavros Loukogeorgakis^{2

5}, Eva Amerstorfer⁶

Affiliations

¹ Department of Paediatric Surgery, University Hospital Rostock, 18057 Rostock, Germany.
² Specialist Colorectal Service, Department of Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital, London WC1N 3BH, UK.
³ Department of Pediatric Surgery, Oslo University Hospital, 0372 Oslo, Norway.
⁴ Clinic for Paediatric Surgery and Paediatric Urology, Klinikum Bremen Mitte, 28205 Bremen, Germany.
⁵ UCL-GOSH Institute of Child Health, University College London, London WC1E 6BT, UK.
⁶ Department for Pediatric and Adolescent Surgery, Medical University of Graz, 8036 Graz, Austria.

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; bowel function; continence score; incontinence; patient-reported outcome measurements (PROM); scoring postoperative results.

Grants and funding

The work itself did not receive any funding. This research was prepared for the OASIS-Holistic Care in Hirschsprung Disease Symposium, funded by the Deutsche Forschungsgemeinschaft (DFG), project number 525829860, and “European Joint Programme Rare Disease” Network Funding, project number 463001064.