A Case Report of Congenital Hyperinsulinism Associated With a Pancreatic Head Lesion Successfully Treated by Duodenum-Preserving Pancreatic Head and Body Resection

Abstract

A six-month-old boy was diagnosed as having congenital hyperinsulinism (CHI) due to recurrent hypoglycemia in the neonatal period. He was initially diagnosed as having diffuse-type CHI, as there were no gene mutations, such as the paternally inherited mutation of ABCC8 or KCNJ11. At five months of age, he underwent 18F-fluoro-L-DOPA positron emission tomography (18F-DOPA PET), which revealed a multifocal lesion in the pancreas head. His diagnosis was then changed to focal-type CHI, and he was transferred to our hospital for surgery. Duodenum-preserving pancreatic head resection, pancreas and jejunum anastomosis, and Roux-en-Y reconstruction were performed at six months of age. He was discharged 44 days after the operation. One year has passed since the operation was performed, and the patient has experienced no complications associated with his blood regulation. Although focal-type CHI without gene mutations is relatively rare in Japan, we recommend 18F-DOPA PET as a reliable examination method and our surgical management as the standard strategy for treating focal-type CHI associated with a pancreatic head lesion.