Congenital Hyperinsulinism
A rare genetic disorder causing excessive insulin production in newborns and infants, often requiring surgical pancreatectomy.
Full Definition
Congenital hyperinsulinism (CHI) is a genetic disorder characterized by dysregulated insulin secretion from pancreatic beta cells, leading to severe hypoglycemia in neonates and infants. The condition may be focal or diffuse, with focal disease often amenable to limited pancreatic resection while diffuse disease may require near-total pancreatectomy. Surgical intervention is considered when medical management fails to control hypoglycemia. Preoperative imaging and intraoperative frozen section analysis are crucial for determining the extent of resection needed.
Usage
Usage note: Always specify focal versus diffuse type when describing surgical planning.
In Context
- "The patient with focal congenital hyperinsulinism underwent successful enucleation of the pancreatic lesion." — Surgical case report
- "PET-CT imaging revealed focal uptake consistent with congenital hyperinsulinism in the pancreatic head." — Radiology report