Congenital hyperinsulinism: localization of a focal lesion with 18F-FDOPA positron emission tomography

Hyperinsulinemic hypoglycemia of infancy, also known as congenital hyperinsulinism, is a group of disorders characterized by dysregulated insulin release. Neonates with severe, persistent hyperinsulinemic hypoglycemia who are unresponsive to medical therapy require pancreatectomy to prevent brain da...

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Bibliographic Details
Published in:Pediatric radiology 2022-04, Vol.52 (4), p.693-701
Main Authors: States, Lisa J., Becker, Susan A., De León, Diva D.
Format: Article
Language:English
Subjects:
Brain damage
Brain injury
Children
Damage prevention
Dihydroxyphenylalanine
Emission analysis
Fluorine isotopes
Hypoglycemia
Imaging
Insulin
Lesions
Levodopa
Localization
Medical imaging
Medicine
Medicine & Public Health
Mutation
Neonatal Imaging
Neonates
Neuroimaging
Neuroradiology
Newborn babies
Nuclear Medicine
Oncology
Pediatrics
Positron emission
Positron emission tomography
Radiology
Tomography
Ultrasound
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Summary:Hyperinsulinemic hypoglycemia of infancy, also known as congenital hyperinsulinism, is a group of disorders characterized by dysregulated insulin release. Neonates with severe, persistent hyperinsulinemic hypoglycemia who are unresponsive to medical therapy require pancreatectomy to prevent brain damage from hypoglycemia. To date, multiple genetic mutations and syndromes and several unique histopathological entities have been identified in children with hyperinsulinism. Histopathology is characterized as diffuse, focal or atypical. Surgical resection of a focal lesion results in a cure in up to 97% of these children. Imaging with 6-fluoro-( 18 F)-L-3,4-dihydroxyphenylalanine ( 18 F-FDOPA) positron emission tomography (PET) is the test of choice for identifying and localizing a focal lesion and has proved to be an invaluable guide for surgical resection. Genetic evaluation is essential for determining who will benefit from PET imaging. This article provides an approach to determine who should be imaged, how to set up a protocol and how to interpret the imaging findings. The diagnosis and management of this disorder require a multidisciplinary approach to prevent brain damage from hypoglycemia.
ISSN:0301-0449
1432-1998
DOI:10.1007/s00247-021-05206-5