Rare Presentation of the Type I Choledochal Cyst in a Young Adult

Abstract

Introduction: Incidence of malignancy in choledochal cyst is 7.5%. Most Common malignancy is cholangiocarcinoma (70%), Gall bladder adenocarcinoma (20%), rest others. It is more common in females, and as the age increases the incidence of malignancy increases, the mean age of presentation is 32 years. Although Type I choledochal cysts are more common, the incidence of malignancy is higher in type IV choledochal cysts. Japanese and Koreans have seen High Incidence of Gall bladder cancer in Pancreatico biliary maljuction(PBM). Case report: We present a rare primary presentation of choledochal cyst in 34yrs/male who presented with complaints of vague right upper quadrant Pain for 4 months. On further evaluation with imaging studies he was diagnosed to have Type I Choledochal cyst with Gall bladder malignancy along with pancreatico biliary maljunction. Patient underwent Radical cholecystectomy with cyst excision and hepaticojejunostomy. Conclusion: Adult Choledochal cyst presentation is usually in form of complication - Cystolithiasis, Infection, Pancreatitis, portal HTN, biliary cirrhosis, and malignancy. Mere Primary presentation of GB cancer along with Choledochal Cyst is quite unusual. We present this case as it is quite rare presentation.