Surgical treatment of organic hyperinsulinism in infancy. Surgical procedure in the absence of a demonstrable insulinoma and a peroperative diagnosis of nesidioblastosis.

Abstract

This study consists of a review of 5 children operated on for organic hyperinsulinism. The diagnosis could be established by demonstrating the repeated presence of elevated serum insulin levels during hypoglycaemia and/or a rapid glucose disappearance. From our experience with this series and by a review of the literature the following data are relevant to the management of these children. Because of the risk of brain damage there is no justification for prolonged diagnostic and therapeutic trials. Surgical exploration of the pancreas is indicated without delay. When during exploration no localised lesion can be found, the diagnosis "nesidioblastosis" can be established by frozen section evaluation. Whether a diagnosis of nesidioblastosis has been established or not, a 90-95% subtotal pancreatectomy has to be performed subsequently, because this operation will cure most of the children with organic hyperinsulinism, has negligible complications and preserves normal exocrine and endocrine pancreatic function. A near-total pancreatectomy has to be performed without delay in patients with recurrent hypoglycaemia caused by persisting organic hyperinsulinism after the first operation.