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Published on: April 26, 2019
Hirschsprung's disease in low- and middle-income countries
Stephen Trinidad1, Peter Kayima2, Vihar Kotecha3
1Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 2023, Cincinnati, OH 45229, United States.
Insights
Hirschsprung
Area of Science:
- Pediatric surgery
- Gastroenterology
- Global health
Background:
- Hirschsprung's disease (HD) is a common cause of pediatric bowel obstruction in low- and middle-income countries (LMICs).
- Patients in LMICs often experience delayed diagnosis and management, leading to increased morbidity and mortality.
- Multifactorial barriers, including socioeconomic and cultural factors, limit timely diagnosis and access to specialized care.
Purpose of the Study:
- To describe the unique challenges and outcomes of Hirschsprung's disease management in LMICs.
- To identify opportunities for improving the diagnosis, treatment, and post-operative care of HD patients in resource-limited settings.
Main Methods:
- Review of clinical presentation, diagnostic methods, surgical management, and post-operative outcomes in LMICs.
- Analysis of factors contributing to delayed diagnosis and treatment.
- Discussion of resource limitations and their impact on patient care.
Main Results:
- Delayed presentation is common in LMICs, resulting in higher rates of obstruction, malnutrition, perforation, and mortality.
- Diagnosis often relies on clinical and radiographic findings due to limited pathology services.
- Multi-stage surgical procedures, including initial diversion, are more prevalent than single-stage pull-through.
- Post-operative complication and mortality rates are higher in LMICs.
Conclusions:
- Improving care for Hirschsprung's disease in LMICs requires addressing diagnostic delays, socioeconomic barriers, and resource limitations.
- Enhancing workforce training and infrastructure is crucial for better surgical outcomes.
- Despite challenges, recent advances show potential for improved patient care in these regions.
Abstract:
Hirschsprung's disease (HD) is one of the most common causes of pediatric bowel obstruction in low- and middle-income countries (LMICs). This paper describes the unique aspects of presentation, diagnosis, management and post-operative care and outcomes of HD in LMICs. In LMICs, patients with HD are much more likely to present in a delayed fashion with subsequent increased morbidity and mortality including higher rates of chronic obstruction, malnutrition with failure to thrive, complete obstruction and perforation. There are multifactorial causes for delay, with opportunities to improve initial timely diagnosis and referral, support families to address socioeconomic and cultural barriers, and improve workforce and infrastructure resources to provide definitive care. In LMICs, the diagnosis is often made based on clinical presentation and radiographic findings as pathological services may be limited. Initial diversion with multi-stage procedure, instead of a single-stage pull-through, predominates. This is also a result of multifactorial causes, including initial presentation to general surgeons at first-level hospitals instead of pediatric surgeons, delayed presentation with sick, malnourished children with significantly distended bowel, and a lack of fresh-frozen pathological services to guide the extent of resection. Post-operatively, HD patients in LMICs experience higher complication and mortality rates - likely stemming from sicker baseline presentations and more limited resources. Significant recent advances in care have occurred for patients with HD in LMICs, while opportunities to continue to improve care remain.
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