Diagnosis and Localization of Focal Congenital Hyperinsulinism by ¹⁸F-Fluorodopa PET Scan

Objectives

To assess the accuracy of ¹⁸F-fluoro-l-dihydroxyphenylalanine ([¹⁸F]-DOPA) PET scans to diagnose focal versus diffuse disease and to localize focal lesions in infants with congenital hyperinsulinism.

Study design

Twenty-four infants with hyperinsulinism unresponsive to medical therapy were studied. Patients were injected intravenously with [¹⁸F]-DOPA, and PET scans were obtained for 1 hour. Images were coregistered with abdominal CT scans.

Results

The diagnosis of focal or diffuse hyperinsulinism was correct in 23 of the 24 cases (96%) and equivocal in 1 case. [¹⁸F]-DOPA PET identified focal areas of high uptake of radiopharmaceutical in 11 patients. Pathology results confirmed that all 11 had focal adenomatosis, and the locations of these lesions matched the areas of increased [¹⁸F]-DOPA uptake on the PET scans in all of the cases.

Conclusions

[¹⁸F]-DOPA PET scans were 96% accurate in diagnosing focal or diffuse disease and 100% accurate in localizing the focal lesion. These results suggest that [¹⁸F]-DOPA PET imaging should be considered in all infants with congenital hyperinsulinism who need to have pancreatectomy.

Abbreviations: ASVS, Selective pancreatic arterial calcium stimulation with hepatic vein insulin sampling, [¹⁸F]-DOPA, ¹⁸F-fluoro-l-dihydroxyphenylalanine, THPVS, Transhepatic portal venous insulin sampling