Outcomes of patients with Hirschsprung’s disease post-pull-through procedure

Abstract

Background: In low- to middle-income countries, diagnosing and managing Hirschsprung’s disease (HD) presents challenges. This study aimed to determine the outcomes of patients with HD post-pull-through (PT) surgery in the Department of Paediatric Surgery at Chris Hani Baragwanath Academic Hospital (CHBAH) between 01 January 2016 and 30 June 2024.

Methods: In Section 1, demographics and complications (according to the Clavien-Dindo Classification System) were collected retrospectively. In Section 2, the functional bowel outcomes (using the Rintala Bowel Functional Score) were collected prospectively.

Results: There were 78 HD-diagnosed patients in the database; 43 (55%) were included in section 1, and 32 (74%) in section 2. Thirty-seven patients (86%) were male, and 21 (49%) had inside flushing toilets. Thirty-two (74%) patients had a transition zone at the rectosigmoid junction, and five (14%) had total colonic HD. The median (IQR) age at diagnosis was 3 (22) months, and only 13 (30%) were diagnosed before 1 month. The median (IQR) age at PT was 19 (28.8) months. Eighteen (42%) patients had no postoperative complications, and 21 (49%) needed surgery-PT. Nineteen patients (59%) had excellent continence, and 11 (34%) had good continence. There was no statistically significant difference in functional outcomes, age at first diagnosis (p = 0.924), available toilet facilities (p = 0.791), or the level of disease (p = 0.624).

Conclusion: The majority of HD patients at CHBAH had a delayed diagnosis and some postoperative complications following PT. However, most patients had excellent to good functional bowel outcomes.