WOFAPS 2025 8th World Congress of Pediatric Surgery

Fahar Sadaf

national Institute of Child Health

“Subthreshold HAEC Episodes in Hirschsprung’s Disease: Rethinking the Diagnostic Scoring System”

Purpose:

To evaluate the clinical presentation and symptom patterns of Hirschsprung associated enterocolitis (HAEC) in pediatric patients, and to assess the reliability of the traditional HAEC scoring system in identifying clinical episodes, particularly those presenting with high- frequency symptoms but lower cumulative scores.

Method:

A cross-sectional study was conducted from 2018 to 2023 at a tertiary care pediatric surgical setup. Pediatric patients with biopsy-proven Hirschsprung's disease presenting with symptoms of enterocolitis were included. Patients with syndromic conditions, incomplete records, or lack of parental consent were excluded. Each episode of enterocolitis was evaluated using the standardized HAEC (Hirschsprung associated enterocolitis) scoring system, and the frequency of presenting symptoms was analyzed.

Results:

A total of 45 HAEC episodes were identified in 20 children (14 males, 6 females) with a median age of 5 years (IQR: 2–9). The rectosigmoid region was involved in 70% of cases. Over 20 episodes scored below the traditional HAEC cutoff (<10–12). Foulsmelling stools, explosive loose stools, and fever were present in 100% of cases; lethargy (60%), abdominal distension (55%), prior HAEC history (68%), and neutrophilia (40%) were also common. Despite lower scores, high-frequency symptoms strongly correlated with clinical HAEC, highlighting the importance of individual symptom assessment.

Conclusion:

A significant proportion of HAEC episodes occurred below the currently accepted scoring threshold. These findings support reconsideration of the existing cutoff, highlighting the importance of individual symptom patterns. Lowering the threshold may improve early diagnosis and outcomes. Multicenter validation is recommended to refine and standardize HAEC diagnostic criteria.