Reassessing Pancreatectomy in Diffuse Congenital Hyperinsulinism: A Tale of 2 Brothers With Homozygous KCNJ11...

Congenital hyperinsulinism (CHI) is a rare but serious disorder characterized by a dysregulated increase in insulin secretion, leading to hypoglycemia. Existing literature on CHI highlights the importance of early recognition and maintenance of blood glucose levels, due to the risk of neurological damage posed by uncorrected hypoglycemia. The cases presented highlight the treatment of 2 brothers who developed neonatal hypoglycemia due to diffuse CHI resulting from homozygous KCNJ11 variants. These cases demonstrate the challenges in maintaining normoglycemia in cases of CHI through medical and surgical therapies. The older sibling, Brother 1, underwent pharmacological treatment and a near-total pancreatectomy at 2.5 months. The outcomes of his treatment highlight the limitations of pancreatectomy in the management of diffuse CHI, as he experienced challenges such as continued hypoglycemic episodes and eventual development of diabetes. Brother 2 was managed with pharmacological therapies and a long-term feeding regimen via gastrostomy. At 6 years he was able to maintain normoglycemia with weaning of octreotide therapy. This paper contributes to our understanding of how to best manage diffuse CHI by emphasizing the limitations and adverse long-term outcomes of pancreatectomy-namely ongoing hypoglycemia and development of diabetes and pancreatic exocrine insufficiency.