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Congenital hyperinsulinism (CHI) is a cause of severe hypoglycaemia in the neonatal period.1 The histological differentiation of CHI into focal and diffuse disease has radically changed the surgical management of patients with the disease.2 Correct localisation and limited excision of the focal lesion results in complete cure of the patient. Recent advances in fluorine-18 l-3,4-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scanning are beginning to provide greater accuracy in preoperative differentiation of focal and diffuse disease and correct localisation of focal lesions.3–5 The principle of this test is that, pancreatic islets take up l-3, 4-dihydroxyphenylalanine (l-DOPA), and convert it to dopamine by DOPA decarboxylase, present in the islet cells. However, the role of dopamine in pancreatic β-cells remains unclear.
A 3-day-old neonate presented with severe hyperinsulinaemic hypoglycaemia. He failed to respond to all forms of treatment and required further investigations to differentiate diffuse from focal disease. He underwent an integrated [18F]DOPA PET/CT scan. Figure 1 shows more than twofold uptake of [18F]DOPA in the head of the pancreas compared with the body and tail of the pancreas, proving focal disease.4 A computed tomography (CT) scan combined with the PET scan colocalised the focal lesion in the head of the pancreas at the junction of the portal vein and superior vena cava (size 6.1 mm). At the time of surgery the focal lesion was found exactly where the PET/CT localisation suggested and was excised with complete resolution of the hyperinsulinism. [18F]DOPA PET/CT scanning is now the preferred method for differentiating diffuse and focal CHI.
This article has been adapted from Kapoor R R, Gilbert C, Mohnike K, Blankenstein O, Fuechtner F, Hussain K. Congenital hyperinsulinism: [ Archives of Disease in Childhood - Fetal and Neonatal Edition 2008;93:166
Competing interests: None.
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