Surgery for congenital hyperinsulinism

Abstract

Transient hypoglycemia in the newborn period is common and generally associated either with immaturity of the glucose regulatory pathways (which responds to frequent feeds and resolves spontaneously within hours), or with stress-associated hyperinsulinism (which responds well to hyperglycemic drugs and resolves spontaneously within the first few weeks or months of life). Congenital Hyperinsulinism (HI) is the most frequent cause of persistent, long-term hypoglycemia in newborns and infants, and can lead to severe and irreversible brain damage and developmental delay. It is a rare congenital disorder of the glucose metabolism that has an estimated incidence of 1-1.4 cases per 50,000 live births, leading to about 80-120 new cases in the United States each year. An incidence as high as 1 in 2500 live births has been reported in populations with high consanguinity like Arabians and Ashkenazi Jews. Inappropriate oversecretion of insulin is the hallmark of HI, and the genetic background is quite variable. Depending on the genetic mutation, babies with HI may be treated medically or may require surgery either as a palliative treatment or as a definitive cure.