Fluorine-18 DOPA-PET and PET/CT Imaging in Congenital Hyperinsulinism

Congenital hyperinsulinism is the principle cause of hypoglycemia during infancy but successful treatment is difficult and persistent hypoglycemia carries the risk of neurologic damage. Focal and diffuse abnormalities are the common forms of hyperinsulinism. Identification and localization of focal hyperinsulinism can be cured by partial pancreatectomy. It has been shown that affected pancreatic areas utilize LDOPA in a higher rate than normal pancreatic tissue and, thus, labeling L-DOPA with fluorine-18 (FDOPA) allows functional mapping of hyperinsulinism using PET. This article presents a fundamental overview of the genetics background, pathology, management, and the role of FDOPA-PET imaging in hyperinsulinism.