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?

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2024年8月6日

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Ali Talebi,
Mohammadsadegh Talebi Kahdouei,
Elmira Hajiesmaeil Memar,
Bahar Ashjaei,
Hojatollah Raji,
Maryam Ghavami Adel

摘要

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.