Bulging of the major duodenal papilla
Article information
Quiz
A 76-year-old woman presented with upper abdominal pain of sudden onset. She had a history of hepatitis B virus-related liver cirrhosis, had been taking tenofovir for two years, and had no fever. An abdominal examination revealed tenderness without rebound in the right upper quadrant. The results of blood tests were as follows: white cell count 10,000/mm3, hemoglobin 13.5 g/dL, aspartate aminotransferase 331 IU/L, alanine aminotransferase 106 IU/L, alkaline phosphatase 106 IU/L, gamma glutamyl peptidase 183 U/L, total bilirubin 1.4 mg/dL, and direct bilirubin 0.5 mg/dL. Serum amylase and lipase levels were within normal ranges. Abdominal computed tomography showed slight dilatation of the common bile duct and a 1.4 cm cystic mass at the ampullary level (Fig. 1A). The initial endoscopic retrograde cholangiopancreatography (ERCP) demonstrated smooth bulging of the major duodenal papilla (Fig. 1B, C). Deep bile duct cannulation failed, and a 5 Fr 3 cm pancreatic stent was placed (Geenen Pancreatic Stent; Cook Medical LLC) because of the repeated pancreatic duct cannulation. The second attempt at ERCP showed persistent bulging of the major duodenal papilla (Fig. 1D). Bile duct cannulation was successful after needle-knife infundibulotomy (Fig. 1E), and cholangiography revealed saccular dilatation and contrast retention at the ampullary level (Fig. 1F). Biliary stenting with a 7 Fr 5 cm stent (C-Flex Double Pigtail Biliary Stent; Boston Scientific Corporation) was performed. The patient was discharged without complications.
(A) Abdominal computed tomography showed a 1.4 cm cystic mass (asterisk) at the ampullary level. (B) Initial endoscopic retrograde cholangiopancreatography demonstrated smooth bulging of the major duodenal papilla. (C) Type III periampullary diverticulum was noted. Orifice was well seen. (D) Second endoscopic retrograde cholangiopancreatography showed persistent bulging of the major duodenal papilla. (E) Deep bile duct cannulation was successful after infundibulotomy. (F) Cholangiography showed saccular dilatation at the ampullary level.
What is the most likely diagnosis?
Answer
The diagnosis was choledochocele. After two weeks, the biliary and pancreatic stents were removed. The bulging of the major duodenal papilla had deflated. Since then for approximately three years, the patient has been asymptomatic and has been uneventfully undergoing follow-up for liver cirrhosis.
Choledochocele is an uncommon congenital malformation of the biliary tract and is classified as a Type III choledochal cyst according to the Todani system.1 The pathogenesis of choledochocele has not yet been elucidated.2 The clinical manifestations vary; upper abdominal pain with nausea and vomiting is the most common presenting symptom.2 Acute pancreatitis, stone formation within the cystic dilatation, jaundice, and cholangitis have also been described.2 Malignancy of the Ampulla of Vater and bile duct has been reported only in adults3,4 The estimated incidence of malignancy in choledochocele is approximately 2.5%,2 which is much lower than that of the other types of choledochal cysts. ERCP is the most widely used method for the diagnosis and concomitant treatment of choledochocele.2 During endoscopic examination, the choledochocele is seen as a smooth round protrusion of the major duodenal papilla. The overlying mucosal surface is soft on palpation. With contrast injection into the bile duct, enlargement of the choledochocele develops and the retention of the contrast inside the cystic dilatation is seen. Endoscopic therapy is considered the standard of care in most patients with symptomatic choledochocele given the low risk of malignancy in choledochocele.2 Endoscopic sphincterotomy is usually used, but endoscopic snare resection of the choledochocele has been reported.2 There are no established recommendations for surveillance after endoscopic therapy. However, the need for surveillance to detect biliary tract cancer should be discussed with the patient.
Notes
Conflicts of Interest
Jimin Han is currently serving on the KSGE Publication Committee; however, Jimin Han was not involved in the peer reviewer selection, evaluation, or decision process for this article. Han Taek Jeong has no potential conflicts of interest to declare.
Funding
None.