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

Hyperinsulinism (HI) is the most frequent cause of persistent hypoglycemia in neonates and infants. Untreated, this condition can cause permanent neurological impairment. Medical therapies such as diazoxide and octreotide are effective in some cases, but patients with severe KATP-adenosine triphosphate sensitive potassium channel mutations are often unresponsive, requiring surgery. This study evaluates surgical outcomes in HI patients at our institution and compares them with those reported in the contemporary literature.

A retrospective review of 14 patients who underwent pancreatectomy for HI between 2008 and 2023 was performed. Collected data included demographics, genetic findings, imaging, surgical approach, operative details, complications, and long-term outcomes.

Eleven patients (78.6%) had congenital and three (21.4%) noncongenital HI. ABCC8 or KCNJ11 mutations were identified in 50% of cases. The functional imaging localized focal disease in three noncongenital HI patients. Near-total pancreatectomy was performed in cases of diffuse disease, while subtotal resection was performed in cases of focal lesions. One patient died from sepsis. At 10-y mean follow-up, 38.4% developed diabetes mellitus, 30.8% had recurrent hypoglycemia, and 30.8% remained euglycemic. Neurodevelopmental delay occurred in 30.8%.

Pancreatectomy (near total and subtotal) remains an essential treatment for refractory (diazoxide unresponsive) HI. Our results, aligned with international experience, emphasize the value of early genetic testing, functional and advanced imaging, and multidisciplinary long-term care.