Surgical Management of Medically-Refractory Hyperinsulinism

Abstract Background Congenital hyperinsulinism (CHI) and insulinomas are the most common causes of medically-refractory pediatric hyperinsulinism. Methods 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. Results 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. Conclusions 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 retained hypersecreting pancreatic tissue. Summary 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 retained foci of hypersecreting pancreatic tissue.