Pancreas divisum: Clinical manifestations and diagnosis
- Evan L Fogel, MD
Evan L Fogel, MD
- Professor of Medicine
- Indiana University School of Medicine
- Stuart Sherman, MD
Stuart Sherman, MD
- Professor of Medicine and Radiology
- Indiana University School of Medicine
- Glen Lehman Professor of Gastroenterology
- Director Of ERCP Services
- Clinical Director, Division of Gastroenterology and Hepatology
Congenital anomalies and variants of the pancreas are seen in approximately 10 percent of the general population. While many are found coincidentally, a portion of these anomalies and variants are clinically significant and cause symptoms in childhood or adulthood.
This topic will review the epidemiology, pathogenesis, clinical manifestations, and diagnosis of pancreas divisum. The management of pancreas divisum is discussed separately. (See "Treatment of pancreas divisum".)
Pancreas divisum is the most common congenital pancreatic anomaly, occurring in approximately 10 percent of individuals [1-3]. The frequency with which pancreas divisum is seen on endoscopic retrograde cholangiopancreatography (ERCP) depends upon the number of patients with pancreatitis in the study population and the completeness of pancreatography. In one retrospective study of 1825 successful consecutive ERCPs, pancreas divisum was found in 7.5 percent . The prevalence of pancreas divisum was significantly higher in patients presenting with idiopathic pancreatitis as compared with controls (50 versus 4 percent). In another retrospective study that included 809 patients who underwent ERCP, pancreas divisum was more frequent in patients with pancreatitis as compared with those with biliary diseases or obscure abdominal pain (9 versus 2 percent) .
Normal pancreatic duct development — The pancreas is formed from fusion of the dorsal and ventral anlages, which develop from the embryologic foregut (figure 1) . The ventral system also gives rise to the hepatobiliary system. At approximately the seventh intrauterine week of life, the ventral pancreas rotates posterior to the duodenum and comes to rest posterior and inferior to the head portion of the dorsal pancreas. The ventral bud forms the inferior part of the head of the pancreas and the uncinate process, whereas the dorsal bud becomes the tail and the body. The ventral pancreas represents 2 to 20 percent of the pancreatic parenchymal mass. Fusion of the ductular network of the two buds gives rise to the main pancreatic duct. The accessory pancreatic duct (of Santorini), which often persists, is derived from the dorsal pancreatic duct proximal to the site of fusion. Fusion of the ductal system occurs in over 90 percent of individuals, although variations in patency of the accessory duct occur.
Pancreas divisum — Failure of fusion of the ventral and dorsal duct system results in pancreas divisum.
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- Normal pancreatic duct development
- Pancreas divisum
- - Subtypes
- - Pathogenesis of pancreatitis in pancreas divisum
- ASSOCIATED PANCREATOBILIARY ABNORMALITIES
- CLINICAL MANIFESTATIONS
- Diagnostic evaluation
- - Asymptomatic
- - Symptomatic or evidence of pancreatitis/complications
- Magnetic resonance cholangiopancreatography
- Endoscopic retrograde cholangiopancreatogram
- ADDITIONAL TESTS FOR PATHOLOGIC PAPILLARY NARROWING
- Pancreatic juice collection
- Secretin ultrasound
- Diagnostic stenting
- Papillary manometry and patency assessment
- DIFFERENTIAL DIAGNOSIS