Published online Jun 28, 2014. doi: 10.4329/wjr.v6.i6.252
Revised: March 19, 2014
Accepted: May 16, 2014
Published online: June 28, 2014
Congenital hyperinsulinism (CHI) is a rare but complex heterogeneous disorder caused by unregulated secretion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift diagnosis and institution of appropriate management is crucial to prevent or minimise adverse neurodevelopmental outcome in children with CHI. Histologically there are two major subtypes of CHI, diffuse and focal disease and the management approach will significantly differ depending on the type of the lesion. Patients with medically unresponsive diffuse disease require a near total pancreatectomy, which then leads on to the development of iatrogenic diabetes mellitus and pancreatic exocrine insufficiency. However patients with focal disease only require a limited pancreatectomy to remove only the focal lesion thus providing complete cure to the patient. Hence the preoperative differentiation of the histological subtypes of CHI becomes paramount in the management of CHI. Fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography (18F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and localisation of the focal lesion. The aim of this review article is to give a clinical overview of CHI, then review the role of dopamine in β-cell physiology and finally discuss the role of 18F-DOPA-PET imaging in the management of CHI.
Core tip: This manuscript describes how the advent of fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography (18F-DOPA-PET) scanning has revolutionised the management of patients with a very complex condition called congenital hyperinsulinism. 18F-DOPA-PET scanning allows the accurate pre-operative localisation of the focal lesion in these patients which can then be surgically removed allowing complete cure from the hypoglycaemia.