Systematic appraisal of the guidelines for the diagnosis and treatment of Hirschsprung's disease

Pediatr Surg Int. 2022 Sep;38(9):1197-1208. doi: 10.1007/s00383-022-05151-w. Epub 2022 Jul 22.


Background: Some guidelines for management of Hirschsprung's disease (HSCR, HD) have been developed, but their quality is vague. This study will systematically assess the quality of guidelines and analyze the key recommendations and the best evidence for guidelines.

Methods: Applicable guidelines were retrieved using a systematic search of databases. The Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool was used to assess the quality of the guidelines. Then, the recommendations and evidence for the included guidelines were extracted and compared.

Results: A total of nine guidelines were included in this study, and only one had an overall standardized score of more than 60%, indicating that it is worthy of recommendation. The problems identified included ambiguous and low-quality evidence; obvious distributional heterogeneity among the recommendations; a lack of in-depth discussion on the interpretation of staging, diagnostic methods, conservative treatment, and surgical staging of disease.

Conclusion: The quality of guidelines varies widely, and there is a lack of high-quality professional opinions and supporting evidence for the main recommendations. At present, only comprehensive guidelines can be considered high-quality and there is still room for improvement.

Keywords: Appraisal; Diagnosis; Guidelines; Hirschsprung’s disease; Treatment.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Databases, Factual
  • Hirschsprung Disease* / diagnosis
  • Hirschsprung Disease* / surgery
  • Humans