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Comparative Study
. 2024 Aug 6;17(1):220.
doi: 10.1186/s13104-024-06873-x.

Can the diameter of nerve fibers be effectively utilized to enhance the accuracy of determining the length of the aganglionic segment compared to intraoperative biopsy in patients with Hirschsprung's disease?

Ali Talebi  1 , Mohammadsadegh Talebi Kahdouei  2 , Elmira Hajiesmaeil Memar  3 , Bahar Ashjaei  4 , Hojatollah Raji  5 , Maryam Ghavami Adel  4
Affiliations
Comparative Study

Can the diameter of nerve fibers be effectively utilized to enhance the accuracy of determining the length of the aganglionic segment compared to intraoperative biopsy in patients with Hirschsprung's disease?

Ali Talebi et al. BMC Res Notes. .

Abstract

Objective: The aim of this study is to investigate the accuracy of utilizing neural fiber trunk diameter in accurately diagnosing the length of the aganglionic segment in patients definitively diagnosed with Hirschsprung's disease.

Results: In this study, 40 patients (19 males, 21 females; mean age 2.5 ± 2.2646 years) were assessed for Hirschsprung's disease. Constipation was the main symptom (75%), followed by abdominal issues. All underwent contrast enema and rectal suction biopsy for diagnosis, followed by surgery (predominantly Soave and Swensen techniques). Majority (85%) had rectosigmoid involvement. Neural fiber diameter was measured, with 52.5% ≤40 μm and 47.5% >40 μm. Statistical analysis showed 40% sensitivity(CI:95%) and 47% specificity(CI:95%) with a cutoff of 40.5 μm. Cohen's kappa index for aganglionic segment size was 0.7.

Keywords: Aganglionic segment; Biopsy; Hirschsprung; Neural fiber trunk diameter.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
ROC curve of neural fiber trunk diameter (talebi, et al., 2024)
See this image and copyright information in PMC

References

    1. Montalva L, et al. Hirschsprung disease. Nat Rev Dis Primers. 2023;9(1):54. 10.1038/s41572-023-00465-y - DOI - PubMed
    1. Matsukuma K, Gui D, Saadai P. Hirschsprung Disease for the practicing Surgical pathologist. Am J Clin Pathol. 2023;159(3):228–41. 10.1093/ajcp/aqac141 - DOI - PubMed
    1. Beltman L, et al. Determining the correct resection level in patients with Hirschsprung Disease Using Contrast Enema and full thickness biopsies: can the diagnostic accuracy be improved by examining Submucosal neural fiber trunk thickness? J Pediatr Surg. 2023;58(8):1463–70. 10.1016/j.jpedsurg.2022.08.019 - DOI - PubMed
    1. Muller CO, et al. Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung’s disease? Pediatr Surg Int. 2012;28(6):597–601. 10.1007/s00383-012-3094-6 - DOI - PubMed
    1. Smith C, Ambartsumyan L, Kapur RP. Surgery, Surgical Pathology, and postoperative management of patients with Hirschsprung Disease. Pediatr Dev Pathol. 2020;23(1):23–39. 10.1177/1093526619889436 - DOI - PubMed

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