It is imperative to prevent recurrent episodes of low blood glucose. Once the diagnosis is made these children should have intravenous glucose/enteral feeds to maintain normal blood glucose levels. If possible they should be referred to specialized centres for further management.

The medical therapy involves first trying oral medications which can stop/reduce the inappropriate insulin secretion. These drugs include diazoxide (usually in conjunction with chlorothiazide, as diazoxide can cause excess fluid to accumulate) and very rarely nifedipine.

If there is no response to diazoxide and nifedipine the second line therapy includes the use of hormones called glucagon and Octreotide. These hormones are used for stabilization and octreotide has been used in the long term management of some patients with Congenital Hyperinsulinism.

It is also now imperative to identify those children who may have the focal forms of hyperinsulinism. This is where only a tiny region of the pancreas is producing too much insulin rather than the whole of the pancreas (diffuse). The current methods of identifying these children include pancreatic venous sampling, intra-arterial calcium stimulation tests, and more recently performing a 18FDOPA-PET scan. The identification of these children is important since surgical removal of the focal area allows these children to be cured.

There are still a group of children who have very severe hyperinsulinism and fail to respond to medical therapy. These children may require surgical removal of part or whole of the pancreas. Those who have a large part of the pancreas removed are at an increased risk of developing diabetes mellitus and will need pancreatic enzymes as the pancreas also produces digestive enzymes.

In those children who require an operation to remove nearly the whole pancreas, it is possible to do this using keyhole surgery.