Pancreas divisum is a birth defect in which parts of the pancreas do not join together. The pancreas is a long flat organ located between the stomach and spine. It helps in food digestion.
Pancreas divisum is the most common birth defect of the pancreas. In many cases, this defect goes undetected and causes no problems. The cause of the defect is unknown.
As a baby develops in the womb, two separate pieces of tissue join together to form the pancreas. Each part has a tube, called a duct. When the parts join together, a final duct, called the pancreatic duct, is formed. Fluid and digestive juices (enzymes) produced by the pancreas normally flow through this duct.
If the ducts do not join while the baby is developing in the womb, pancreas divisum results. Fluid from the two parts of the pancreas drains into separate areas of the upper portion of the small intestine (duodenum). This occurs in 5 to 15% of people.
If a pancreatic duct becomes blocked, swelling and tissue damage (pancreatitis) may develop.
It results from failure of fusion of dorsal and ventral pancreatic anlages. As a result, the dorsal pancreatic duct drains most of the pancreatic glandular parenchyma via the minor papilla. Although controversial, this variant is considered as a cause of pancreatitis.
Pancreatic divisum can result in a santorinicele, which is a cystic dilatation of the distal dorsal duct, immediately proximal to the minor papilla.
Most patients with a pancreas divisum are asymptomatic but is more frequently seen in patients with chronic abdominal pain and idiopathic pancreatitis than in the general population
Three subtypes are known:
- type 1 (classic): no connection at all; occurs in the majority of cases: 70%
- type 2 (absent ventral duct): minor papilla drain all of pancreas while major papilla drains bile duct; 20-25%
- type 3 (functional): filamentous or inadequate connection between dorsal and ventral ducts: 5-6%
- Abdominal pain, most often in the mid-abdomen, that may be felt in the back
- Abdominal swelling (distention)
- Nausea or vomiting
Note: Unless you have pancreatitis, you will not have symptoms.
Exams and Tests
- Abdominal ultrasound
- Abdominal CT scan
- Amylase and lipase blood test
- Endoscopic retrograde cholangiopancreatography (ERCP)
- Magnetic resonance cholangiopancreatography (MRCP)
- Endoscopic ultrasound (EUS)
This was the traditional method of diagnosis where a pancreas divisum was suspected when there was no contrast extending towards the pancreatic tail upon administration at the ampulla of Vater.
This is the standard method of evaluation in modern times.
The key imaging features are:
- the dorsal pancreatic duct being in direct continuity with the duct of Santorini, which drains into the minor ampulla
- ventral duct, which does not communicate with the dorsal duct but joins with the distal bile duct to enter the major ampulla
- Some authors suggest increased sensitivity of secretin MRCP (S-MRCP) in detection sensitivity of pancreas divisum 2
The following treatments may be needed if you have symptoms of the condition, or if pancreatitis keeps returning:
- ERCP with a cut to enlarge the opening where the pancreatic duct drains
- Placement of a stent to prevent the duct from getting blocked
- You may need surgery if these treatments do not work.
There are several management options in selected cases which include 6:
- minor papillectomy
- minor papilla stenting
- balloon dilatation of an any associated stricture
Most of the time, the outcome is good.
The main complication of pancreas divisum is pancreatitis.
When to Contact a Medical Professional
Call for an appointment with your health care provider if you develop symptoms of this disorder.
Because this condition is present at birth, there is no known way to prevent it.