Investigating the use of nerve fiber diameter for a more accurate diagnosis of the length of the aganglionic segment in patients with Hirschsprung's disease compared to intraoperative biopsy

Abstract

Objective: The aim of this study is to investigate the accuracy of utilizing nerve fiber 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.