Total colonic aganglionosis – long-segment Hirschsprung disease management successfully performed in Papua New Guinea

Abstract

Neonatal bowel obstruction is a common surgical pathology and is associated with neonatal sepsis. Medical management resolves most cases, however, a few cases cause challenges for the surgical division whereby resources are diverted by surgically treatable cases such as bowel atresia, Hirschsprung’s disease (HD), malrotation and even anorectal anomalies with imperforated anus. The diagnosis of HD is challenging but it can be diagnosed in the neonatal period. In low and middle-income countries (LMICs) the diagnosis is often missed, and the resulting late presentation is associated with significant co-morbidities. The following case report describes a neonate who was delivered in a village before being transferred to the hospital. A major surgical pathology was then corrected successfully using the limited resources that were available.