Choledochal Cyst With Intestinal Metaplasia Presenting as an Unusual Case of Recurrent Cholangitis

Abstract

This is the case of a 64 year old African-American female with a history of recurrent cholangitis and choledolithiasis requiring repeated Endoscopic retrograde cholangiopancreatography (ERCP) procedures. She presented to our institution with severe abdominal pain. She underwent multiple prior ERCPs at outside institution which showed fusiform dilation of biliary tree with recurrent choledocholithiasis. During ERCP at our institution she was noted to have fusiform dilation of biliary tree with very low cystic duct take off marked by very thick cystic duct insertion wall. Single operator cholangioscopy confirmed thick cystic duct insertion wall and spy bite biopsies of thick septum at cystic duct insertion showed inflammatin with intestinal metaplasia. She underwent hepaticojejunostomy with excision of the biliary tree was performed without post-surgical complications. Final pathology revealed cholechocal cyst with focal intestinal metaplasia within biliary mucosa. Choledochal cysts are rare congenital anomalies involving intra- or extrahepatic ducts1-3 with an incidence of 1 in 100,000 - 150,000 in western countries but a remarkably high incidence of 1 in 1,000 in Asia8. Etiology of choledocal cysts remains unclear; however the most popular theory proposed by Babbitt's4 which attributes the cyst formation to abnormal pancreaticobiliary duct junction (APBDJ). This allows pancreatic fluids to flow into the bile duct causing inflammation and weakness of the duct wall leading to cyst formation. cyst formation. In our present case the cyst represents the category type 1A. There is a strongIn our present case the cyst represents the category type 1A. There is a strong association of malignancy with type I and type IV cysts9, however all types are resected despite histo-pathology findings to prevent malignant transformation. Amongst the various laparotomy vs laparoscopic surgical approaches, Roux-en-Y, Hepaticojejunostomy, and complete cyst resection are currently the procedure of choice. This case highlights potential stepwise progression to cholangiocarcinoma with intestinal metaplasia as intermediate step.