ABSTRACT
Congenital hyperinsulinism (CHI), previously termed persistent hyperinsulinemic hyperglycemia of infancy (PHHI), is a spectrum of conditions characterized by profound hypoglycemia in the presence of inappropriately high insulin secretion. CHI usually presents within the first few hours or days of life in both premature and term babies but can occasionally present later in infancy or childhood. Babies can present with nonspecific symptoms of hypoglycemia such as poor feeding, irritability, or lethargy, or more severe symptoms such as seizures or coma. CHI is characterized by hyperinsulinemic, hypoketotic, hypofattyacidemic hypoglycemia. Surgical treatment is indicated when an infant remains dependent on intravenous glucose administration despite maximum medical treatment. The anterior surface of the pancreas is exposed by entering the lesser peritoneal sac via the gastrocolic omentum, ligating and dividing vessels in the greater omentum along the greater curvature of the stomach. The neurological outcome of patients with CHI depends on the age of presentation.
