Two cases of congenital hyperinsulinism coexisting congenital portosystemic shunt

Abstract

 Congenital hyperinsulinism（CHI）is a disease characterized by persistent hypoglycemia due to excessive secretion of insulin. Congenital portosystemic shunt（CPSS）is a rare vascular malformation and induces a direct bypass of the liver connecting the portal vein to the body’s circulatory system. We experienced two cases of CHI with coexisting CPSS which closed spontaneously.

 Total bile acid and ammonia were monitored until the complete closure. Because some CPSS have symptoms and do not close depending on the site, we should distinguish coexisting CPSS when we diagnosed CHI with elevated total bile acid and ammonia.