Surgical management of medically-refractory hyperinsulinism

Congenital hyperinsulinism (CHI) and insulinomas are the most common causes of medically-refractory pediatric hyperinsulinism. Children with CHI or insulinoma treated from 1/1/2014-1/1/2019 at an academic center were retrospectively analyzed. Primary outcome was persistent intravenous dextrose requirement at discharge. Eleven patients were identified: six with diffuse-type CHI, three with focal-type CHI, two with insulinoma. Median age at diagnosis was 20 days (1 day-16 years). Preoperative functional imaging (18F-Fluoro-l-DOPA PET-CT scan) accurately localized 66% of focal-type CHI lesions. All patients with focal-type CHI and insulinoma were cured by local resection. All patients with diffuse-type CHI underwent near-total pancreatectomy (NTP): four patients were cured of hyperinsulinism, of which 2 developed insulin-dependent diabetes, while two patients were palliated to home enteral glucose infusion. Localized resection cures children with focal, insulin-secreting lesions. NTP may cure diffuse-type CHI; potential complications include diabetes, exocrine insufficiency, and persistent hypoglycemia from residual hypersecreting pancreatic tissue. Congenital hyperinsulinism (CHI) and insulinomas are the most common causes of medically-refractory pediatric hyperinsulinism, causing potential complications including permanent brain injury. 18F-Fluoro-l-DOPA PET-CT scan can be used to localize focal insulin-secretion lesions preoperatively. Focal-type CHI and insulinoma are cured by localized resection. Diffuse-type CHI requires near-total pancreatectomy for cure, but complications include diabetes, exocrine insufficiency, or persistent hypoglycemia from residual foci of hypersecreting pancreatic tissue. •Congenital hyperinsulinism and insulinoma cause refractory hypoglycemia in children.•Genetic testing is important for diagnosis, but should be confirmed by pathology.•18F-Fluoro-L-DOPA PET-CT scan can be used to accurately localize focal lesions.•Focal-type CHI and insulinoma are cured by localized resection.•Diffuse-type CHI requires near-total pancreatectomy without guarantee of cure.