Hyperinsulinism Treatment & Management

Updated: Dec 16, 2015
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
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Treatment

Medical Care

Maintaining normoglycemia is essential to prevent neurologic sequelae. Infants with hyperinsulinism are at higher risk of neurologic sequelae than infants with hypoglycemia from other causes. Because insulin inhibits lipolysis and ketogenesis, hyperinsulinism results in the paucity of alternative fuel used by the brain.

The glucose output from the liver is 2-3 mg/kg/min in adults. Infants and children have a greater need for glucose and have a maximal output estimated at 5-7 mg/kg/min. Patients with hyperinsulinism may require very high glucose infusion rates (20-30 mg/kg/min) to maintain normoglycemia. Attempts should be made to keep blood glucose levels at 60 mg/dL or higher at all times.

Healthy neonates and infants can fast for 6 hours without experiencing hypoglycemia. This equates to skipping one feeding in the infant who is fed ad libitum.

Medications should be administered to suppress insulin secretion or stimulate glucose release.

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Surgical Care

Gastrostomy tube

Gastrostomy tube placement may be indicated in extreme cases to administer food if the infant is unable to handle the increased glucose requirements.

Partial or near-total pancreatectomy

Pancreatectomy is reserved for infants who fail to establish adequate control on medical therapy.

Although most surgeons initially remove 95% of the pancreas, a near-total (98%) pancreatectomy appears to be most effective in preventing hypoglycemia in the newborn period for those with diffuse potassium channel disease (SUR1 or Kir6.2 mutations). Remarkably, the elevated lifelong risk of diabetes mellitus is more closely related to the intrinsic error in regulated insulin release, rather than to the extent of pancreatectomy. [15] One recent study showed almost 94% of focal hyperinsulinism cases required no further treatment, versus 41% with diffuse hyperinsulinism that showed continued hypoglycemia postoperatively. [16]

Close monitoring of blood glucose levels is indicated to ensure glycemic control and to minimize hypoglycemia. If hypoglycemia persists, medical therapy should be reattempted. If medical therapy is unsuccessful, a second pancreatectomy may be indicated. The authors' experience indicates that clinically significant pancreatic regrowth can occur in infants after near-total pancreatectomy. A Whipple procedure is unwarranted because it cannot guarantee remission of diffuse disease.

Limited pancreatectomy is indicated for patients with focal disease.

Complications include pancreatic exocrine insufficiency, diabetes mellitus, and injury to the common bile duct.

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Consultations

See the list below:

  • Pediatric endocrinologist

  • Pediatric surgeon

  • Neonatologist

  • Geneticist (if family history is present or suspected)

  • Closest tertiary referral center (academic children's hospital) for possible enrollment in clinical research protocols

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Diet

Frequent feedings by gastrostomy help maintain euglycemia but do not prevent the need for intravenous dextrose administration before surgery.

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