Total colonic aganglionosis in a case of anorectal malformation: A case report

Background: Hirschsprung’s disease (HD) rarely co-occurs with anorectal malformation (ARM). If it occurs, the classical variety of HD is mostly associated. Total Colonic Aganglionosis (TCA) in a case with ARM is exceedingly rare.
Case Presentation: A 3-day-old female neonate presented with neonatal intestinal obstruction. of such a rare association. Examination revealed a perineal fistula. A colostomy was formed for persistent abdominal distension, but it did not work properly. Re-exploration revealed TCA with a transition zone at the level of the terminal ileum.
Conclusion: Though ARM is associated with several anomalies of various body systems, its association with TCA is exceedingly rare.