Ectopic pancreas associated with choledochal cyst and multiseptate gallbladder

Congenital choledochal cyst is a rarely seen malformation of childhood, particularly when associated with multiseptate gallbladder or ectopic pancreas. The current case represents a 15-day-old boy with jaundice. Ultrasonography suggested a cystic lesion, probably of the common bile duct. The patient underwent a total excision of type I choledochal cyst and gallbladder with Roux-en-Y anastomosis, and a wedge biopsy from the liver. Gross examination revealed multiple septa dividing the gallbladder into multiple compartments. The outer and inner surfaces of the choledochal cyst were unremarkable. Microscopically, the cyst wall was composed of dense fibrous tissue with a single layer of cubic to columnar cells constituting the overlying epithelium. Serial sections incidentally revealed ectopic pancreatic tissue lying along the cyst wall characterized by acini, islets, and ductal structures with endocrine cells reactive for chromogranin A. Septa dividing the gallbladder were composed of fibrotic stalks containing smooth muscle fibers. Areas of papillary hyperplasia and intestinal metaplasia of gallbladder epithelium were also noted. The liver biopsy specimen demonstrated the presence of intrahepatic bile ducts, subsequently confirmed by cytokeratin 7. To our knowledge, this case represents the 1st one associated with these 3 entities and only the 2nd choledochal cyst with ectopic pancreatic tissue in its wall.