Hirschsprung's disease in low- and middle-income countries.

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.