Surgery of persistent hyperinsulinaemic hypoglycaemia

Hyperinsulinism (HI) is the commonest cause of persistent or recurrent hypoglycaemia in childhood. HI is genetically and phenotypically diverse. Key management issues involve early diagnosis by insuring that appropriate investigations are undertaken at the point of hypoglycaemia, prevention of recurrent hypoglycaemia and clinical, biochemical and genetic characterisation of the HI syndrome. Children with persistent diazoxide resistant HI require investigation at specialist centres to differentiate those with a generalised disorder of the pancreas (diffuse HI; di-HI) from those with localised abnormalities within the pancreas (focal HI; fo-HI). Fo-HI may be managed by selective pancreatic resection of the focal abnormality. Di-HI is only managed by surgery if combination drug therapies are unable to prevent hypoglycaemia. Pancreatic β-cell dysfunction persists following subtotal pancreatectomy of di-HI.