Surgical management of congenital hyperinsulinism of infancy

Abstract

Congenital hyperinsulinism of infancy (CHI) is characterized by inappropriate insulin secretion resulting in persistent hypoglycemia, which can lead to irreversible severe neurological damage in the infant. Many patients with CHI will respond to medical therapy, but surgery is necessary in those that do not. There are 2 main histologic subtypes, diffuse and focal, both of which may require different surgical strategies. Near-total pancreatectomy is the procedure of choice for diffuse CHI, whereas a localized resection is curative in focal CHI. Open surgery is the traditional approach to pancreatic resection. However, laparoscopy is increasingly used, particularly in localized resection for focal disease. We describe both methods of pancreatectomy.