Health Library

Anomalous Pancreaticobiliary Junction

The pancreas is a small organ in the abdomen (belly) that helps with the digestion of food. The bile ducts are the tubes that carry bile (a substance that helps with digestion) between organs of the digestive system.

APJ can occur with or without biliary cysts. A biliary cyst is a collection of fluid within the bile duct. The presence of cysts affects treatment.

APJ occurs in 1.5 to 3.2% of babies worldwide. The condition is more common in females and in people of Asian descent.

What Causes an Anomalous Pancreaticobiliary Junction?

APJ is a congenital condition, meaning that it is present at birth. APJ occurs when the ducts don’t move to the correct place when the fetus is developing during pregnancy.

What are the Symptoms of an Anomalous Pancreaticobiliary Junction?

One of the main symptoms of APJ is abdominal pain. Children with APJ also frequently develop infections, such as cholangitis (infection in the bile ducts) or recurrent episodes of pancreatitis (infection and swelling of the pancreas).

If your child has cholangitis, they may have one or more of the following symptoms:

• Fever and chills
• Pain in the upper right area of the belly
• Nausea and vomiting
• Jaundice (yellowing of the eyes and skin)
• Clay-colored stool (poop)
• Dark colored urine (pee)

If your child has pancreatitis, they may have one or more of the following symptoms:

• Upper abdominal or back pain
• Nausea and vomiting
• Feeling less hungry than normal

How is an Anomalous Pancreaticobiliary Junction Diagnosed?

To diagnose this condition, your child’s doctor will complete a physical exam. They will ask questions about your child’s symptoms and health history and order imaging tests to confirm the diagnosis. The doctor uses the images to find and analyze the unusual connection between the pancreas and bile duct.

The most common type of imaging is magnetic resonance cholangiopancreatography (MRCP), a specialized MRI of the pancreas and surrounding areas. Your child’s doctor may also use an endoscopic ultrasound or endoscopic retrograde cholangiopancreatography (ERCP) to confirm the diagnosis of APJ.

What are the Treatment Options for an Anomalous Pancreaticobiliary Junction?

There is a lifetime risk of cancer in the pancreas and bile ducts if APJ is not treated. The treatment for APJ is surgery. The type of surgery depends on whether or not your child has a biliary cyst:

• If they have a biliary cyst, their doctor will likely recommend surgery to remove the cyst as much as possible. During this procedure, the surgeon will close the unusual opening between the bile duct and the pancreas. They will make a new drainage tract for the bile duct.
• If they don’t have a biliary cyst, they will need to have surgery to close the unusual opening between the bile duct and the pancreas. The surgeon will make a new drainage tract for the bile duct.

Surgery is typically laparoscopic, which is minimally invasive and reduces risk. Patients are asleep under anesthesia, and surgery typically takes several hours, depending on the complexity of the condition. They may stay in the hospital for a few days or more after surgery.

Prognosis for an Anomalous Pancreaticobiliary Junction

After having surgery to correct APJ, the child is cured of the condition and their risk for future cancer is much lower. Your child’s specialist will let you know if they need to continue to see your child for ongoing care and monitoring.