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Pancreatectomy Outcomes in Pediatric Hyperinsulinism: A Retrospective Single-Center Experience

Seyithan Ozaydin1, Nihal Coskun2, Ipek Yildiz Ozaydin3

  • 1Department of Pediatric Surgery, Istinye University, Liv Hospital Bahcesehir, Istanbul, Turkey.

The Journal of Surgical Research
|March 15, 2026
PubMed

Insights

Surgery for hyperinsulinism (HI) is crucial for diazoxide-unresponsive cases. Pancreatectomy offers essential treatment, but long-term monitoring for diabetes and hypoglycemia is vital for infant outcomes.

Area of Science:

  • Pediatric Surgery
  • Endocrinology
  • Medical Genetics

Background:

  • Hyperinsulinism (HI) is a leading cause of persistent hypoglycemia in infants, potentially causing neurological damage.
  • Medical treatments like diazoxide and octreotide are not always effective, especially for severe KATP-channel mutations.
  • Surgery is often necessary for patients unresponsive to medical management.

Purpose of the Study:

  • To evaluate surgical outcomes in patients with hyperinsulinism at our institution.
  • To compare these outcomes with existing literature on surgical management of HI.
  • To highlight the importance of a multidisciplinary approach in managing HI.

Main Methods:

  • Retrospective review of 14 patients undergoing pancreatectomy for HI (2008-2023).
  • Data collected included demographics, genetic findings, imaging, surgical details, complications, and long-term follow-up.
  • Analysis of surgical approaches (near-total vs. subtotal pancreatectomy) based on disease presentation (diffuse vs. focal).

Main Results:

  • 50% of patients had ABCC8 or KCNJ11 mutations; 78.6% had congenital HI.
  • Functional imaging identified focal disease in 3 noncongenital HI patients.
  • At 10-year follow-up: 38.4% developed diabetes, 30.8% experienced recurrent hypoglycemia, 30.8% remained euglycemic, and 30.8% had neurodevelopmental delay.

Conclusions:

  • Pancreatectomy (near-total and subtotal) is a critical treatment for refractory hyperinsulinism.
  • Early genetic testing, advanced imaging, and comprehensive long-term care are essential for optimal patient outcomes.
  • Surgical results align with international findings, reinforcing its role in managing severe HI.
Abstract

Keywords:
Congenital hyperinsulinismHyperinsulinismNear-total pancreatectomyNesidioblastosisSubtotal pancreatectomy

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