18-F-L 3,4-Dihydroxyphenylalanine PET/Computed Tomography in the Management of Congenital Hyperinsulinism

Congenital hyperinsulinism (HI) is the most common cause of persistent hypoglycemia in neonates and infants. Several genetic mutations have been identified and are associated with 2 distinct histopathologic forms of disease: diffuse and focal. Targeted clinical evaluation to distinguish medically treatable disease from disease requiring surgical management can prevent life-threatening complications. Detection and localization of a surgically curable focal lesion using PET imaging with 18-F-L 3,4-dihydroxyphenylalanine ([18F]-FDOPA) has become standard of care. This article provides guidelines for the selection of patients who can benefit from [18F]-FDOPA-PET/computed tomography and protocols and tips used to diagnose a focal lesion of HI.