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It has been nearly 140 years since Dr Harald Hirschsprung first described neonatal megacolon as a distinct congenital disease and 75 years since Dr Orvar Swenson’s landmark article in the New England Journal of Medicine described a successful surgical treatment.1 The surgical innovations that followed changed Hirschsprung disease (HSCR) from an often-fatal diagnosis to a chronic manageable condition.2 However, many questions remain about this challenging disease.