Pancreatic Malformations

In about 10% of the population, the pancreas develops abnormally in utero. Most often, these abnormalities are not significant, and the pancreas still functions normally. The pancreas has two main jobs in the body: it produces digestive enzymes to break down fats, proteins, and sugars, and it produces hormones to regulate blood sugar. In some cases of an abnormal pancreas or absent pancreas, these hormones and enzymes may need to be replaced.

There are several types of pancreatic malformations. The most common include:

Pancreas divisum

Pancreas divisum malformation occurs in about 8% of the population, and describes non-union of the two duct systems in the pancreas. While the condition alone does not usually cause problems, sometimes it can cause pancreatitis if other risk factors are present.

Annular pancreas

An annular pancreas is shaped like a ring around the small intestine. It occurs in 5-15 per 100,000 people. The small ring of pancreatic tissue causes the intestine to be obstructed, and children may have repeated episodes of vomiting and feeding intolerance. Ultimately, surgery is required to bypass the blocked area. Some patients don’t have a very tight ring, and may not develop symptoms until they are older. In these cases, pancreatitis might be the first sign.

Pancreatic hypoplasia

This describes a pancreas that doesn’t fully develop. This usually occurs as part of another disorder. Children with pancreatic hypoplasia may not have enough pancreatic tissue to produce the digestive enzymes and hormones, and will require replacement.

Pancreatic aplasia

Exceedingly rarely, sometimes the pancreas does not develop at all. Children with pancreatic aplasia have symptoms shortly after birth because they don’t have digestive enzymes and lack the ability to regulate their blood sugar. Replacement of digestive enzymes and insulin to regulate blood sugar are necessary for these children to thrive.

At Children's Hospital of Philadelphia (CHOP), children with pancreatic malformations are evaluated and treated by doctors in the Pancreatic Disorders Program in the Division of Gastroenterology, Hepatology and Nutrition.