What is a pancreatectomy?

The majority of children diagnosed with congenital hyperinsulinism (HI) can be managed with medication. Children with the focal form of HI or those with hypoglycemia that cannot be controlled through medical therapy may need part or nearly all of their pancreas removed. Surgery to remove the pancreas is called a pancreatectomy.

Pancreatectomy for children with hyperinsulinism

A baby with hyperinsulinism who needs surgery will have either a partial pancreatectomy or near-total pancreatectomy (removing approximately 98% of the pancreas), depending on the type of HI they have.

A child with diffuse HI, in which all the cells in the pancreas are overproducing insulin, will usually need a near-total pancreatectomy.

A child with focal disease, in which the cells overproducing insulin are clustered in one spot (a focal lesion) will only need the portion containing the lesion removed in a partial pancreatectomy. The exact percentage of the pancreas that needs to be removed depends on the size of the lesion and its location. 

Pancreatectomies at CHOP

The team at the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia has the most experience of any center in the world in performing this delicate surgery — with more than 550 pancreatectomies performed since 1998.

Our cure rate for children with focal HI is excellent; 97% of children with the focal form of HI are cured through a partial pancreatectomy.

A pancreatectomy for children with diffuse HI does not cure the HI but significantly reduces the severity of the hyperinsulinism and allows for effective medical treatment.

What to expect during surgery

The surgeon will make a cut across the baby’s abdomen, just above the belly button, to reveal the pancreas, which is about 3 inches long. The surgeon examines the pancreas with a magnifying glass and gently feels it. Tissue samples from different areas of the pancreas are sent to Pathology to be examined to confirm if the HI is focal or diffuse.

For children with diffuse HI, the surgeon leaves only a very small amount of the pancreas. He will also place a gastrostomy tube to help with management of the HI after surgery.

For children who have suspected focal HI undergo a specialized PET scan prior to surgery. This scan helps the surgeon find the focal lesion in the pancreas. Focal lesions can be as small as 1/10 of an inch (2 or 3 millimeters) in size, so they can be difficult to find.

A focal lesion in the tail or the body of the pancreas is straight forward to remove. When removing the lesion, the surgeon will avoid disturbing the main pancreatic duct, which carries pancreatic secretions.

If a lesion is in the head of the pancreas, the surgery may be more complex because the head of the pancreas contains important blood vessels and ducts. In order to remove the lesion while saving the body and tail of the pancreas, the surgeon may need to perform a Roux-en-y loop, a procedure that allows the pancreatic secretions to continue to drain into the intestine.

To make sure all of a lesion is removed, our surgeon works closely with CHOP pathologists and endocrinologists during the surgery. Frozen tissue samples from the pancreas are examined under a microscope to look for any sign of HI cells.

What to expect after a pancreatectomy

Post-pancreatectomy, children will recover in the Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit. Their blood sugar levels are closely monitored. They are not allowed to eat by mouth for 5 to 7 days after surgery, and they received nutrition through an IV line.

After recovery from surgery, most children with diffuse disease may still require medication and/or dextrose to maintain safe glucose levels. Parents will be trained on how to check glucose levels at home, and how to give medications and dextrose, if needed, so they are prepared to continue care after discharge.

Children with focal HI will undergo a fasting test to confirm they have been cured.

Families can expect to stay at CHOP anywhere between four and eight weeks. Children who have undergone a near-total pancreatectomy tend to need a longer recovery time.

To ensure continued care for children with diffuse disease, the HI Center also offers the HI Multidisciplinary Clinic for follow-up appointments, where families can see the specialists they need — endocrinologists, gastroenterologists, nutritionists, geneticists, genetic counselors and psychologists — in one location all in the same day.

Experience counts

The combination of expertise in diagnosing the type of HI, state-of-the-art testing and a high volume of pancreatectomies positions the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia (CHOP) as the world’s premier hospital for this delicate, complex surgery.

Children needing surgery and their families have access to a team that includes endocrinologists, surgeons, pathologists, radiologists, anesthesiologists, neonatologists, gastroenterologists, geneticists, genetic counselors, nutritionists, nurse practitioners and social workers — all with extensive experience in HI. The team works closely together to give children with HI the best possible outcomes.