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PET scan in management of congenital hyperinsulinism

Congenital hyperinsulinism (CHI) is a cause of severe hypoglycemia in the neonates. The pancreatic involvement can be diffuse or focal. The preoperative differentiation of these two forms is very important because the surgical management is radically different. The focal form of the disease can be cured if the focal lesion can be localized accurately and resected completely. In recent years, considerable progress has been made in the biochemical, morphological and molecular genetic differentiation of CHI. Fluorine-18 L-3,4-dihydroxyphenylalanine positron emission tomography (18F-DOPA-PET) has been introduced for differentiation between focal and diffuse CHI. The ability to take up L-DOPA and convert it into dopamine is correlated with the activity of the aromatic amino acid decarboxylase and is increased in the hyper functional pancreatic area in comparison to normally functioning pancreas. The high sensitivity of this method allows the surgeon to perform appropriate resection of a focus without the risk of long-term diabetes. Patients with CHI should be managed within a strong network of diagnostic, treatment, and research institutions.