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Problems and outcome of Hirschsprung's disease presenting after 1 year of age in a developing country
S O Ekenze1, C Ngaikedi, A A Obasi
1Sub-Department of Paediatric Surgery, University of Nigeria Teaching Hospital, Enugu, Nigeria. sebekenze@gmail.com
Insights
Delayed diagnosis of Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Public Health
Background:
- Hirschsprung's disease (HD) diagnosis is often delayed in resource-limited settings.
- Late presentation of HD leads to significant complications and increased morbidity.
Purpose of the Study:
- To evaluate problems, treatment outcomes, and factors contributing to delayed Hirschsprung's disease presentation after one year of age.
- To identify challenges in managing Hirschsprung's disease in a resource-limited environment.
Main Methods:
- Retrospective study of 41 children over 1 year old with Hirschsprung's disease.
- Data collected from January 2000 to June 2009 at University of Nigeria Teaching Hospital, Enugu.
Main Results:
- 92.7% of children presented with complications.
- Late presentation attributed to delayed referral (65.9%), parental ignorance (26.8%), and poverty (7.3%).
- High colostomy rates (85.4%) and postoperative complications (48.8%) were observed. Good outcomes achieved in 75.6%.
Conclusions:
- Hirschsprung's disease presenting late is linked to high colostomy rates and morbidity.
- Improved diagnosis requires enhanced medical practitioner education and public awareness campaigns.
Background:
The purpose of the present study was to evaluate the problems, treatment outcome, and contributory factors to delayed presentation in Hirschsprung's disease (HD) after 1 year of age in a resource-limited setting.
Methods:
This retrospective study included 41 children aged >1 year with HD managed at the University of Nigeria Teaching Hospital, Enugu, in south eastern Nigeria, between January 2000 and June 2009.
Results:
Complications of HD were evident at presentation in 38 (92.7%) of the 41 children. Late presentation was due to delayed referral in 27 cases (65.9%), parental ignorance in 11 (26.8%), and poverty in 3 (7.3%). The HD was rectosigmoid in 33 patients (80.5%) and was of ultra-short length variety in 8 patients (19.5%). Thirty-five patients (85.4%) required colostomy for decompression, and colostomy-related complications occurred in 24 of them (68.6%). The definitive surgical procedure was a Swenson pullthrough in 34 cases (82.9%) and posterior myectomy in 7 others (17.1%). Twenty patients (48.8%) experienced at least one postoperative complication. After follow-up of 7-64 months (mean: 31 months), 31 (75.6%) patients had a good outcome, 6 (14.6%) had persistent constipation, 3 had (7.3%) incontinence, and one child (2.4%) died from overwhelming infection.
Conclusions:
Hirschsprung's disease presenting after 1 year of age may be associated with high colostomy rates and increased morbidity. Continued dissemination of updated information on HD to medical practitioners and a public awareness campaign may improve time to diagnosis.
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