A Rare Case of Dorsal Agenesis of Pancreas, Choledochal Cyst, and Hirschsprung Disease in a Young Patient

Dorsal pancreatic agenesis is a rare congenital pancreatic malformation. There is just 1 reported case associating it with choledochal cyst. However, no cases have reported yet with both coexisting with Hirschsprung disease. We report a case of a 23-year-old man, presenting with on and off epigastric pain, sometimes radiating to the back. His medical records showed he had Hirschsprung disease as a neonate, for which he underwent Duhamel procedure. Ultrasound imaging revealed a choledochal cyst and a nonvisualized distal portion of the pancreas. Further cross-sectional imaging confirmed the findings—a type 1 choledochal cyst and a dorsal agenesis of the pancreas in a patient with Hirschsprung disease.