Background: Pancreatic divisum, the most common congenital variant of the pancreatic anatomy, occurs when the ductal systems of the ventral and dorsal pancreatic ducts fail to fuse. As a result of nonunion of the ducts, the major portion of the pancreatic exocrine secretions enters the duodenum via the dorsal duct and minor papilla. Generally, it has been accepted that a relative obstruction to pancreatic exocrine secretory flow through the minor duct and minor papilla can result in pancreatitis in a small number of patients with pancreas divisum (with stenotic minor papilla). Incomplete or partial divisum is defined as the communication of the dorsal and ventral ducts via a tiny branch.
Pathophysiology: Most persons with an anomalous pancreatic ductal system are asymptomatic and completely unaffected by the condition; however, a significant number of patients present with recurrent attacks of acute pancreatitis. A relative obstruction to the flow of pancreatic juice at the level of an inadequately patent or stenosed minor papilla has been hypothesized to result in an increase in intraductal pressure with consequent pancreatitis.
In patients with pancreas divisum, pancreatic history has demonstrated changes of chronic pancreatitis in the dorsal duct distribution and normal parenchyma in the ventral duct distribution. The etiology of stenosis of the accessory papilla is not identical to that of the clinical entity of stenosis or papillitis of the ampulla of Vater. Relative stenosis of the minor sphincter is believed to occur because, as an outlet, it is too small to accept the exocrine drainage secretions from most of the adult pancreas. The slowly developing resistance to increased ductal pressure as the pancreas grows ultimately cannot be tolerated and pancreatitis ensues.
Frequency:
* Internationally: Pancreas divisum is observed in 6% of normal subjects at autopsy. The frequency with which pancreas divisum has been diagnosed during patient lifetime has increased with newer diagnostic modalities.
Mortality/Morbidity: An incidence of pancreas divisum as high as 10-20% has been reported in some series in patients with acute recurrent pancreatitis. The actual incidence of divisum and minor papilla stenosis is not known but certainly is significantly less than that of pancreas divisum alone.
Race: No racial predominance exists, although the anomaly has been noted more frequently in Caucasians.
Sex: Male-to-female ratio is 1:1.
Age: Age ranges from 7 months to 98 years, with a median age at diagnosis of 57 years.
Anatomy: The pancreas is formed during the embryologic stage from distinct ventral and dorsal buds, which arise from the duodenal diverticulum. The ventral duct undergoes rotation and fuses with the dorsal duct by the seventh week of intrauterine life.
In 1642, Wirsung demonstrated the main pancreatic duct, and in 1775, Santorini accurately described the ductal anatomy and demonstrated the accessory pancreatic duct.
Typically, the main pancreatic duct is derived from both embryologic parts of the pancreas; the dorsal pancreas provides the main duct in the tail and body of the gland and the ventral pancreas provides the main duct in the head of the gland. The accessory duct (of Santorini) is the remaining portion of the duct in the dorsal pancreas and may drain through an accessory papilla more proximally in the duodenal loop.
In pancreas divisum, the ventral and dorsal pancreatic ducts fail to fuse in utero, resulting in drainage of the bulk of pancreatic juice (80-95%) via the duct of Santorini through the relatively small minor papilla.
Clinical Details: The symptom complex of patients with pancreas divisum and minor papilla stenosis includes abdominal pain of varying intensity, with epigastric pain radiating to the back and pain brought on by alcohol intake and worsened by eating fatty foods. Patients usually present with nausea, vomiting, weight loss, diarrhea, and jaundice (which can be both obstructive and nonobstructive). Many patients are treated for gastritis, irritable bowel syndrome, or other conditions for a period prior to a more definitive investigation of pancreatic abnormality.
Patients usually present with increased serum amylase, lipase, bilirubin, white blood cell count, or elevated urinary amylase-to-urinary creatinine ratio.
Preferred Examination: Take a methodical approach to the patient with recurrent abdominal pain and pancreas divisum. The presence of clear-cut pancreatitis in association with this anomaly on presentation makes it easier to determine that the pancreas may be the site of origin of the abdominal pain. In patients with divisum who do not demonstrate clinical pancreatitis, determine the existence of accessory papilla stenosis. Some patients may demonstrate focal dilatation of the pancreatic duct at the minor ampulla (termed Santorinicele).
Endoscopic retrograde cholangiopancreatography (ERCP) is the test of choice for making a diagnosis of pancreas divisum. Occasionally, this anomaly can be depicted with CT but with a low sensitivity and requiring thin slices. However, magnetic resonance pancreatography (with or without secretin) may replace this invasive test for diagnostic purposes in the future.
Limitations of Techniques: ERCP is expensive and invasive, with a reported complication rate of 5%.
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