Double major papilla: a very rare endoscopic picture

Variations in the bile duct and pancreatic duct opening are related to the process of rotation and recanalization during embryologic development. Complete non-union of distal common bile duct and duct of Wirsung gives rise to a double papilla of Vater [1]. A 48-year-old woman presented to the emergency room complaining of severe upper abdominal pain which later shifted to the right upper quadrant. Physical examination was remarkable for right upper quadrant and epigastric tenderness. Laboratory data showed abnormal liver chemistry and white blood count of 13000/mm3. Abdominal ultrasound demonstrated a dilated common bile duct (diameter 11 mm) and MRI cholangiography revealed choledocholithiasis and a small stone embedded in the ampulla. Endoscopic retrograde cholangiopancreatography (ERCP) was performed showing a small biliary stone in the orifice of upper major papilla and the presence of a second major papilla located about 1 cm inferior to the upper one (Fig. 1). Cannulation of upper and lower papilla showed a dilated common bile duct with stones and a normal pancreatogram, respectively (Fig. 2). Endoscopic sphincterotomy of upper papilla was performed with extraction of biliary stones. A search in PubMed yielded only four documented cases of double papilla of Vater [2-5]. The existence of this rare anatomic anomaly does not predispose to any pancreatobiliary disease, but is an opportunity for another learning experience contributing to expert competency in ERCP and improved quality of care for patients.