Surgical long-term outcomes of Hirschsprung Disease in the Democratic Republic of Congo: a case series of 31 patients in a resource-limited setting

Background: Hirschsprung disease (HD) contributes significantly to the burden of disease related to conditions amenable to pediatric surgery within the Democratic Republic of Congo (DRC). However, it has received less clinical attention due to limited resources. We aimed at assessing the long-term follow-up outcomes following an innovate resection approach in South Kivu, eastern DRC. Methods: In this case series, the authors elucidate the admissions for HD spanning from 2016 to 2021 at a Pediatric Surgical Center in the DRC. All patients underwent surgical management. Due to the absence of an extemporaneous biopsy, the resection margin was defined by a formula developed by the authors: 5 cm for patients under 5 years, 5 cm plus 1 cm per age from 6–10 years, and 5 cm plus 0.5 cm per age above 10 years. Post-operative mortality and complication rates were equally computed. Descriptive statistics were calculated for continuous variables. Discharge-to-Follow-up data were visualized using time-to-event curves. Results: Thirty-one patients aged 195 (interquartile range: 18–669) days were included in this study. Thirteen (58.93%) were from mining areas. The patients were surgically managed and follow-up for a year. 6.45% died in the 30-day post-operative period and 19.35% were lost to follow-up. The complaints were recorded from the fourth month following the surgery with most of the patients complained about fecal soiling (58.06%). The overall complaint rate was 80% (95%CI:56.20%-90.69%). The overall rate of incontinence was different compared to those with fecal soiling (p = 0.04). Although lacking statistical significance, the length of stay (LoS) post-surgery predicting an 9% increased incidence of complaints during the follow-up period (adjusted Hazard Ratio: 1.09, 95% CI:0.97–1.23). Conclusion: HD cause avertable morbidity in South Kivu. These data suggest that the long-term outcome in HD can be mitigated through appropriate surgical management during the operative and intervention aimed at optimizing the LoS.