Do Histologic Features of the Proximal Margin of Resected Specimens Predict Clinical Outcomes in Hirschsprung Disease?

Objective Patients with Hirschsprung disease (HSCR) can experience obstructive symptoms despite adequate resection. We sought to determine if submucosal nerve thickness or length of ganglionated bowel in the resected specimen correlated with functional outcomes. Methods A retrospective study of patients who underwent surgery between 2015-2019 was performed. The resected specimen was scanned to measure areas of the thickest submucosal nerves and the length of the ganglionated segment. Functional outcomes were collected via chart review. Results Thirty patients were included. The median age at pull-through was 4.5 months (interquartile range {IQR} 0.5 - 6.7 months); 70% were male, and 57% had a Swenson pull-through. The median size of the thickest nerves was 28 micrometers (IQR 24, 32). Three specimens had a nerve thickness of >40 micrometers. The median length of the resected ganglionated segment was 4.4 cm (IQR 2.2, 7.2). Out of the total, 53% of patients experienced post-operative enterocolitis; 13% required further surgery. At a median of 25.3 months (IQR 17.6, 42.2 months) from pull-through, 33% did not require any bowel regimen therapy. Utilizing logistic regression, neither submucosal nerve thickness nor length of the resected ganglionic segment correlated with outcomes. Conclusion While continued bowel management therapy was common, no correlation was found between histologic findings and functional outcomes.