Postoperative Hirschsprung's associated enterocolitis (HAEC): transition zone as putative histopathological...

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Pathology

Background:

• Hirschsprung's-associated enterocolitis (HAEC) is a severe complication of Hirschsprung disease (HD) with unknown pathogenesis.
• The transition zone (TZ) length in HD has been underexplored as a predictor for postoperative HAEC (post-HAEC).

Purpose of the Study:

• To identify predictive factors for post-HAEC.
• To investigate the role of histopathological findings, particularly TZ length, in predicting post-HAEC.

Main Methods:

• Retrospective analysis of Hirschsprung disease patients (2010-2022) with >6 months follow-up.
• Detailed histopathological examination of resected bowel, focusing on TZ length and inflammation in the ganglionic zone.
• Bivariate and multivariate regression analyses to identify predictive factors for post-HAEC.

Main Results:

• A TZ length < 2.25 cm was identified as an independent predictor of post-HAEC (p=0.0096).
• Preoperative HAEC history and TZ < 2.25 cm correlated with early post-HAEC development.
• Histological inflammation in the ganglionic zone and TZ < 2.25 cm were associated with a higher risk of post-HAEC (p=0.0074, p=0.001 respectively).
• Severe post-HAEC occurred more frequently in patients with preoperative HAEC, histological inflammation, and short TZ.

Conclusions:

• A transition zone (TZ) length < 2.25 cm is a significant predictor for postoperative Hirschsprung's-associated enterocolitis (post-HAEC).
• Preoperative clinical presentation and histopathological inflammation are associated with more severe post-HAEC.
• Histopathological findings, including TZ length, can aid in identifying high-risk patients for HAEC and implementing preventive measures.