Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases

Abstract

Background: Annular pancreas (AP) concurrent with pancreaticobiliary maljunction (PBMJ), an unusual coexisted congenital anomaly, often presented symptoms and subjected surgical treatment at the early age of life. We reported the first adult case of concurrent AP with PBMJ presented with symptoms until his twenties, and performed a literature review to analyze the clinicopathological features of such cases comparing with its pediatric counterpart.

Case presentation: The main clinical features of this case were abdominal pain and increased levels of plasma amylase as well as liver function test. A complete type of annular pancreas with duodenal stenosis was found, and dilated common bile duct with high confluence of pancreaticobiliary ducts was also observed. Meanwhile, extremely high levels of bile amylase were detected both in common bile duct and gallbladder. The patient received duodenojejunostomy (side-to-side anastomosis) as well as choledochojejunostomy (Roux-en-Y anastomosis), and was discharged in a good condition.

Conclusion: AP concurrent with PBMJ usually presents as duodenal obstruction in infancy, while manifests as pancreatitis in adulthood. Careful long-term follow-up is required for children with AP considering its association with PBMJ which would induce various intractable pathologic conditions in the biliary tract and pancreas.

Figure 1

CT image and MRCP appearance. Computerized tomography scan showed the dilated descending part of duodenum (arrow) with stenosis at the distal end of dilated duodenum (A). Magnetic resonance cholangiopancreatography revealed fusiform dilatation of the common bile duct with high confluence of pancreaticobiliary ducts (arrow) with a common channel measured 24 mm in length (B).

Figure 2

Endoscopic appearance. Endoscopy showed an enlarged pylorus and excessively ectatic duodenal cavity. Endoscopic retrograde cholangiopancreatography (ERCP) was not successful in confirming the pancreaticobiliary maljunction due to duodenal stenosis (arrow).

Figure 3

Intraoperative appearance. During laparotomy, a complete ring of pancreatic tissue (arrows) surrounded the descending duodenum (A). Gas charging demonstrated duodenal stenosis with dilatation of the stomach and duodenal bulb (triangle) (B).