Type 1 Choledochal Cyst with Ectopic Pancreas and Septate Gallbladder.

Choledochal cysts (CCs), congenital cystic dilatation of the biliary tract, are more commonly identified in females and have been associated with a myriad of other developmental abnormalities. We present a male infant who was diagnosed with type I CC prenatally. He subsequently underwent cyst and gallbladder resection with hepaticoduodenostomy reconstruction at the age of 6 months. Pathologic examination confirmed type I CC with co-existing septate gallbladder and ectopic pancreas (Heinrich type 1). Although the clinical significance is unclear, this second case of CC with septate gallbladder and ectopic pancreas highlights the embryologic association of these abnormalities.