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. 2025 Mar 12;25(1):96.
doi: 10.1186/s12893-024-02684-w.

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

Fabrice Gulimwentuga Cikomola #  1   2   3 , Arsene Daniel Nyalundja #  4   5 , Alliance Wani Bisimwa  1   2 , Bijoux Safi Matabaro  1 , Franck Masumbuko Mukamba  1 , Costa Musilimu Sudi  1 , Paul Budema Munguakonkwa  1   2 , Leon-Emmanuel Mukengeshayi Mubenga  1   2 , Kinja Nyamugabo  2 , Désiré Munyali Alumeti  6 , Théophile Kabesha  6 , Marc Miserez  7 , Dieudonné Mushengezi Sengeyi  2   8
Affiliations

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

Fabrice Gulimwentuga Cikomola et al. BMC Surg. .

Abstract

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.

Keywords: Digestive Tract Congenital Anomalies; Long-term follow-up; Pediatric Surgery; Pull-through; South Kivu.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Ethical clearance was secured from the Institutional Review Board (IRB) at the Catholic University of Bukavu (U.C.B) (Ref. N* UCB/CIES/NC/020/2021). The requirement for the informed consent was waived by the IRB at the UCB due the retrospective design of the study. The gathered data was treated with confidentiality, and access was limited to the research team. Additionally, any details allowing for the identification of participant were removed, and the authors were barred from accessing such information. Consent for publication: Not applicable. The requirement for the consent for publication was waived by the IRB at the UCB due the retrospective design of the study. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Plain abdominal radiography in a newborn with HD. The x-ray reveals distended bowel loops, indicative of colonic dilation, along with air-fluid levels suggesting obstruction of fecal matter and gas
Fig. 2
Fig. 2
Intraoperative view of a Swenson technique. A classic abdomino-perineal pull through procedure: (A) abdominal and (B) perineal steps
Fig. 3
Fig. 3
A post-operative view of colostomy
Fig. 4
Fig. 4
Intraoperative view of distended bowel loops in an adult with HD. The black circle indicates the transition zone where the dilated colon abruptly narrows to the normal segment
Fig. 5
Fig. 5
Time-to-Event Curve of Surgically Managed Patients with HD at HPGRB. This curve displays the probability of occurrence of complaints over a one-year follow-up period: (A) for the overall cohort and (B) stratified by complaint types
See this image and copyright information in PMC

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