Necrotizing enterocolitis following a single very low dose of octreotide in a patient with congenital hyperinsulinism: a case successfully managed with 18F-DOPA PET/CT-guided surgery

Octreotide is considered a second-line treatment for congenital hyperinsulinism unresponsive to diazoxide. Necrotizing enterocolitis (NEC) is a serious adverse effect of octreotide, typically occurring in a dose-dependent manner. Here, we report a case of necrotizing enterocolitis following a single administration of a very low dose of octreotide. A female infant was admitted on day 3 of life with severe hypoglycemia. Laboratory findings revealed hyperinsulinemia and hypoketotic hypoglycemia, confirming a diagnosis of congenital hyperinsulinism. Despite diazoxide therapy, adequate glycemic control was not achieved. As a second-line intervention, a single subcutaneous injection of octreotide (1.6 μg/kg) was administered. Two days post-administration, the patient developed abdominal distension and significant vomiting. NEC was diagnosed, necessitating bowel decompression surgery. Subsequent 18F-DOPA positron emission tomography/computed tomography revealed a focal lesion extending from the pancreatic head to the body. The lesion was successfully resected with preservation of nearly the entire normal pancreas. This case highlights that even a very low dose of octreotide may precipitate necrotizing enterocolitis, warranting close monitoring. Lesion localization using 18F-DOPA positron emission tomography/computed tomography is critical in guiding surgical management of congenital hyperinsulinism.