Will My Child Get Diabetes After Pancreatectomy for CHI?

If your child requires a near-total pancreatectomy for diffuse Congenital Hyperinsulinism (CHI), it is highly likely they will eventually develop insulin-dependent diabetes, though usually not immediately ^[1]. While only a small percentage develop diabetes right after surgery, nearly all children who undergo this procedure will require insulin within 10 to 11 years ^[1][2]. Additionally, about half will need daily digestive enzyme supplements to help their bodies break down food ^[1].

While this surgery is lifesaving when medications fail to control severe low blood sugars, it trades an acute, dangerous condition for manageable, long-term chronic conditions.

Why the Surgery Requires Removing So Much of the Pancreas

The long-term effects of surgery depend entirely on whether your child has focal or diffuse CHI.

• Focal CHI: The abnormal cells are clustered in one tiny area, so surgeons can simply remove that small section (a lesionectomy). This preserves most of the healthy pancreas, meaning the risk of diabetes or digestive issues is extremely low ^[1].
• Diffuse CHI: The overactive cells are spread throughout the entire pancreas. To protect your child’s brain from severe, persistent hypoglycemia (low blood sugar), surgeons cannot just remove a small piece; they must remove 95% to 98% of the pancreas. This is called a near-total pancreatectomy ^[3]. Because so much tissue is removed, your child’s long-term metabolic outcomes are completely different.

The Timeline for Developing Diabetes

Parents often fear their child will wake up from a near-total pancreatectomy with diabetes, but the reality is usually more gradual.

• Immediately after surgery: Only about 13% of children require insulin right away ^[1]. Paradoxically, about 60% of children still experience mild hypoglycemia for a period of time because the tiny 2-5% of the pancreas left behind is still overproducing insulin ^[1]. Do not panic if this happens. Your medical team expects this and will manage it using careful feeding schedules or lower doses of medications (like diazoxide) until the remaining cells settle down.
• Over the next decade: As your child grows, that small remnant of the pancreas “burns out” and can no longer produce enough insulin for their growing body.
• Long-term expectations: The risk of diabetes climbs steadily over time. By 7 years post-surgery, nearly 80% of children require insulin, and by 11 years, the rate reaches 96% ^[1]. On average, the median time from surgery to the onset of diabetes is roughly 10 years ^[2].

When diabetes does occur (often referred to as post-pancreatectomy diabetes or Type 3c diabetes), the first year can be uniquely challenging. These children have a higher risk of severe low blood sugars compared to typical Type 1 diabetes because they lack glucagon, the hormone the pancreas normally releases to raise blood sugar when it falls ^[2]. Because of this, endocrinologists typically use continuous glucose monitors (CGMs) to safely track blood sugar levels and catch lows before they become severe.

Managing Digestion (Exocrine Pancreatic Insufficiency)

The pancreas has two main jobs: producing hormones like insulin to regulate blood sugar, and creating juices to digest food. Removing most of the pancreas affects both systems.

After a near-total pancreatectomy, about 49% of children develop clinical symptoms of Exocrine Pancreatic Insufficiency (EPI) ^[1]. This means the pancreas does not produce enough enzymes to properly digest fats and proteins. Without treatment, this can lead to frequent loose, fatty stools (steatorrhea), gas, and poor growth. Doctors will monitor your child’s stool (often using a simple fecal elastase test) to catch this early.

Fortunately, EPI is highly treatable. If your child develops this, they will be prescribed pancreatic enzyme replacement therapy (PERT). This involves taking specialized enzyme granules or pills with the first bite of every meal and snack. For infants, the capsules are opened and the granules are mixed into a tiny spoonful of acidic food, like applesauce (they should never be mixed into a full bottle). With proper enzyme therapy, your child will be able to absorb their nutrients, gain weight normally, and follow standard growth curves.