A previously healthy 23-year-old woman presented to hospital with 2 months of intermittent dyspepsia following meals and acute-onset epigastric pain, nausea and vomiting. Based on the results of laboratory investigations (lipase > 1900 [normal < 286] U/L), acute pancreatitis was diagnosed. It was treated conservatively with analgesics and intravenous hydration. The results of other laboratory tests, including those for amylase, leukocytes and creatinine, were within normal limits. No obvious reversible causes of pancreatitis were initially identified. The patient reported consuming alcohol rarely. Ultrasonography of the abdomen ruled out gallstones. A computed tomography (CT) scan of her abdomen and pelvis showed that her pancreas had a relatively normal appearance; however, assessment was limited because of minimal intra-abdominal fat in her upper abdomen in the region of the pancreatic head. After 2 days, the patient's pain had improved but her lipase level remained above 500 U/L. The patient's abdominal pain and lipase levels (829 U/L) increased after she resumed eating solid foods.Ultrasonography performed 21 days after admission showed gallbladder "sludge" with no evidence of stones.Laparoscopic cholecystectomy was performed. The results of tests for autoimmune pancreatitis (antinuclear antibodies and IgG subtypes) were negative. However, the patient had persistent abdominal pain and an elevated lipase level above 1000 U/L. Twenty-eight days after admission, she underwent magnetic resonance cholangiopancreatography, which showed pancreas divisum. Following endoscopic retrograde cholangiopancreatography (Figure 1), a sphincterotomy on the major papilla was performed with no noteworthy clinical improvement. Six weeks later, endoscopic retrograde cholangiopancreatography was repeated, with sphincterotomy of the minor papilla and placement of a stent. The patient's pain resolved, and her lipase levels returned to normal within 14 days.
Discussion
Anatomy and epidemiologyIn over 90% of healthy people, the dorsal and ventral endodermal pancreatic buds fuse to form the adult pancreas. However, in about 10% of the population, fusion does not occur, resulting in pancreas divisum (Figure 2), which is the most common congenital ductal anomaly of the pancreas. 1 The classic anatomy of pancreas divisum consists of a small ventral duct, which drains through the larger major papilla, and the larger dorsal duct, which drains through the smaller minor papilla.
2,3In patients with a large dorsal duct, the majority of the pancreatic secretions (up to 2000 mL daily) pass through this duct.2 Pancreas divisum through the minor papilla (instead of the major papilla) can result in inadequate drainage and pain
Key points• Pancreas divisum is a congenital anomaly that occurs in about 10% of the population and is a potentially treatable cause of acute pancreatitis.• The lifetime risk of pancreatitis for patients with pancreas divisum (5%-10%) is similar to risk in the general population.• Pancreas divisum should be considered in pa...