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

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Abstract

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.

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Abbreviations

AGA:

American Gastroenterological Association

AGREE II:

Appraisal of Guidelines for Research and Evaluation II

ANOVA:

Analysis of Variance

ARM:

Anorectal manometry

BE:

Barium enema

ERNICA:

European Reference Network for rare Inherited and Congenital (digestive and gastrointestinal) Anomalies

ERPT:

Endorectal pull-through

FTRB:

Full-thickness rectal biopsy

GRADE:

Grading of Recommendations Assessment, Development and Evaluation

HAEC:

Hirschsprung-associated enterocolitis

HD:

HDSR, Hirschsprung’s disease

HE:

Hematoxylin–eosin

ICCs:

Intra-group correlation coefficients

NSCT:

Neural stem cell transplantation

LAPT:

Laparoscopic-assisted pull-through

OCEBM:

Oxford Centre for Evidence-Based Medicine

PRISMA-P:

Preferred Reporting for Systematic Reviews and Meta-analyses Protocols

RCT:

Randomized controlled trial

RSB:

Rectal suction biopsy

SPSS:

Statistical Product and Service Solutions

TCA:

Total colonic aganglionosis

TEPRT:

Transanal endorectal pull-through

UEG:

United European Gastroenterology

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Funding

This study was supported by Yunnan young academic and technical leaders reserve talent Project (No. 202105AC160049) to D.L.S.; Yunnan Province Joint Special Project of Science & Technology Department of Yunnan Province and Kunming Medical University (No. 2019FE001-167) to C.Y.Y. and Special program for famous doctors of Yunnan 10000 person plan (YNWR-MY-2020-050) to C.Y.Y.

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Author notes

  1. Jia-Jun Lv and Ting Yang contributed equally to this work.

Authors and Affiliations

  1. Department of Gastrointestinal Surgery, Second Affiliated Hospital of Kunming Medical University/Second Faculty of Clinical Medicine, Kunming Medical University, Kunming, 650101, China

    Yun-Ying Gong, Ting Yang, Xing-Zong Huang, Lin Zhang, Jin-Hong Wu, Yun-Yun Cen & Da-Li Sun

  2. Department of Gastroenterology, Second Affiliated Hospital of Kunming Medical University/Second Faculty of Clinical Medicine, Kunming Medical University, Kunming, 650101, China

    Jia-Jun Lv

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  1. Yun-Ying Gong
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Contributions

All the authors contributed in the preparation of this work. GYY, LJJ, YT and SDL were drafted and revised the article; GYY, LJJ, CYY and SDL were responsible for the theme, final editing, and preparation of the manuscript for submission; GYY, HXZ, ZL and WJH were analyzed the data and GYY interpreted the results of data; GYY and SDL critically revised the manuscript. All authors read and approved the final manuscript.

Corresponding authors

Correspondence to Yun-Yun Cen or Da-Li Sun.

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Gong, YY., Lv, JJ., Yang, T. et al. Systematic appraisal of the guidelines for the diagnosis and treatment of Hirschsprung’s disease. Pediatr Surg Int 38, 1197–1208 (2022). https://doi.org/10.1007/s00383-022-05151-w

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