- Case Report
- Open Access
Double major papilla of Vater - a rare endoscopic finding during endoscopic retrograde cholangiopancreatography: a case report
© Katsinelos et al; licensee BioMed Central Ltd. 2009
Received: 19 September 2009
Accepted: 21 October 2009
Published: 21 October 2009
A double major papilla of Vater is a rare congenital anomaly with only three documented cases described in the literature.
We report the case of a 19-year-old man, with chronic ulcerative pancolitis and congenital sphrerocytosis, who underwent endoscopic retrograde cholangiopancreatography because he had persistent elevation of liver enzymes and normal MRI cholangiography. During endoscopic retrograde cholangiopancreatography, a double papilla of Vater with separate drainage for the bile duct and the pancreatic duct was observed.
Endoscopic retrograde cholangiopancreatography showed normal pancreatogram and findings compatible with sclerosing cholangitis.
The Vaterian system includes the common bile duct and the main pancreatic duct (duct of Wirsurg), which, as they conjoin at the level of duodenum, they form the major papilla of Vater. 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 Wirsurg gives rise to a double papilla of Vater [1–3].
We report a young man with double papilla of Vater wherein cannulation of the common bile duct and pancreatic duct could be achieved through either orifice independently.
A 19-year-old Greek man was referred to our unit for endoscopic retrograde cholangiopancreatography (ERCP) with suspicion of sclerosing cholangitis, because of persistent elevated levels of asparate aminotransferase, alanine aminotransferase and alkaline phosphatase and normal MRI cholangiography.
Medical history revealed congenital spherocytosis and ulcerative pancolitis.
Interest in the anatomy of the descending duodenum and papilla began with advent of ERCP as diagnostic procedure in the early 1970s. The liver, gallbladder, biliary tree and pancreas originate in the hepatic diverticulum, which develops at the beginning of the 4th embryonic week . At this week, the developing extrahepatic biliary tree becomes organized. Establishment of the lumen of the extrahepatic biliary tree occurs by a process of recanalization . During the recanalization process, the common bile duct and the pancreatic duct coalesce within one duct, the ampulla, before they open into the duodenum on the major papilla [1–3]. If they fail to coalesce, they remain as independent ducts and open into the duodenum separately [1–3].
Silvis et al described variations in the normal duodenal papilla at ERCP . They commented that double papilla of Vater is a rare anatomic variant and is not usually apparent without close inspection. The existence of a double major papilla with two neighboring independent papillary structures which drain biliary and pancreatic ducts separately, is very unusual. Simon et al observed a double papilla of Vater in 0.18% of 1800 patients who underwent ERCP . A search in Medline revealed only three documented cases of double papilla of Vater [5–7]. 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.
Our case is intriguing because the patient had two independent papillary structures with two distinct, separate orifices and each orifice demonstrated prominent papillary fronds, typically seen at the ostium of the major papilla. Moreover there was no communication between the two orifices during cannulation with catheter, excluding the development of an acquired choledochoduodenal fistula.
Our case demonstrates that if the endoscopist observes two separate papillae in the duodenum, with the proximal one draining the bile duct and the distal one draining the pancreatic duct, without communication between them, this depicts a rare anatomic variation.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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