Endocrine Abstracts

Background: Congenital Hyperinsulinism is a common cause of medically refractory hyperinsulinism in infants. Hence, surgical intervention is considered the gold standard treatment (1). We present a case of congenital hyperinsulinism in which the patient remained hypoglycaemic despite total pancreatectomy and remained independent of exogenous insulin subsequently for eleven years.

Case report: We present the case of a 24-year-old male who remained insulin-free 11 years after undergoing a total pancreatectomy for congenital hyperinsulinism. He presented with hypoglycaemia and hyperinsulinemia shortly after birth. He was treated initially with octreotide, diazoxide, and hydrocortisone, but these were ineffective. At one month of age, he underwent a 95% pancreatectomy; however, hypoglycaemia persisted. He was subsequently trialled on further medical treatment, which also failed. He continued on glucose/glucagon infusion and had a second pancreatectomy one month later, during which 98% of pancreas was removed but, hypoglycaemia continued. A week later, the remaining part of the pancreas was removed, and the entire duodenum was meticulously scraped. Histology revealed abnormal beta cells invading the wall of the duodenum. His hypoglycaemia persisted. A PET CT scan showed focal FDG uptake around the bed of the pancreatic head, suggesting that some abnormal pancreatic cells had adhered to the duodenal wall. Duodenectomy was not considered due to the high mortality risk. A PEG tube was inserted, and he was started on subcutaneous octreotide QDS and glucagon infusion. After two months, the glucagon infusion was stopped, oral feeding resumed and octreotide was continued. The patient experienced no further episodes of hypoglycaemia, and octreotide was continued until he was 10-year-old. A year later, HbA1c rose to 7.1%, and exogenous insulin was initiated. Initially, only rapid-acting insulin was used due to concerns about night-time hypoglycaemia. After four years, a multiple-dose insulin regimen was started.

References: 1. Zobel et al., Am J Surg., 2020