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Is the appendix a good organ to diagnose total colonic aganglionosis?

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Abstract

Purpose

The use of the appendix for diagnosis of Total Colonic Aganglionosis (TCA) remains controversial. This study aimed to categorize the presence of ganglion cells in the appendix and determine its reliability as a histological specimen for the diagnosis of TCA.

Methods

This was a combined retrospective and prospective study. Permanent histological specimens of normal appendices removed during appendectomy for malrotation or falsely presumed appendicitis, and from patients with short segment Hirschsprung’s disease (HD) or TCA were included. Permanent specimens of the appendix tip from Malone procedures were prospectively collected. All specimens were independently reviewed by two experienced pathologists in a standardized manner to assess for the presence of ganglion cells.

Results

A total of 112 appendices were evaluated. Nine came from patients with a diagnosis of TCA, and five from patients with HD. Ganglion cells were present in all specimens from patients with diagnoses other than TCA and were absent in all specimens from patients with TCA.

Conclusion

In the correct clinical setting, the absence of ganglion cells in the appendix can allow for a reliable diagnosis of TCA.

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Acknowledgements

The authors would like to thank Dr. Ubaid Ullah Ismail Khan, who was visiting our Center during initial discussions of this research, for his suggestions to use the tip of the appendix during Malone procedures as an additional study group.

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Authors and Affiliations

  1. International Center for Colorectal and Urogenital Care, Division of Pediatric Surgery, Children’s Hospital Colorado, 13213 E 16th Ave, Anschutz Medical Campus, Box 323, Aurora, CO, 80045, USA

    Marina L. Reppucci, Jill Ketzer, Alberto Pena, Luis de la Torre & Andrea Bischoff

  2. Department of Pathology and Laboratory Medicine, Children’s Hospital Colorado, Aurora, CO, USA

    Michael A. Arnold & Mark Lovell

  3. Department of Pathology, University of Colorado, Aurora, CO, USA

    Michael A. Arnold & Mark Lovell

  4. Department of Pediatric Surgery, Instituto Nacional de Pediatria, Mexico City, Mexico

    Karla Santos-Jasso

Authors
  1. Marina L. Reppucci
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  2. Michael A. Arnold
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  3. Mark Lovell
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  4. Karla Santos-Jasso
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  5. Jill Ketzer
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  6. Alberto Pena
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  7. Luis de la Torre
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  8. Andrea Bischoff
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Corresponding author

Correspondence to Andrea Bischoff.

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Reppucci, M.L., Arnold, M.A., Lovell, M. et al. Is the appendix a good organ to diagnose total colonic aganglionosis?. Pediatr Surg Int 38, 25–30 (2022). https://doi.org/10.1007/s00383-021-05023-9

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  • DOI: https://doi.org/10.1007/s00383-021-05023-9

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