Surgical management of medically-refractory hyperinsulinism
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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 residual 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 residual foci of hypersecreting pancreatic tissue.
DOI: 10.1016/j.amjsurg.2019.09.003
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