Congenital hyperinsulinemia with grade 4 intraventricular hemorrhage: a case report with a 2-year follow-up.

Massive intraventricular hemorrhage associated with hypoglycemia has rarely been reported. We present a late preterm baby girl with severe hypoglycemia after birth. Despite high glucose infusion rates, her glucose levels remained in the 30s to 50s (mg/dL) during the first week of life with a brief period of normoglycemia. On day 2, her computed tomography scan of head showed extensive intraventricular hemorrhage with intraparenchymal extension, which was reported as unexpected for an infant born close to term. She was diagnosed with congenital hyperinsulinemia. Her glucose levels normalized on diazoxide therapy started on day 6. In the absence of any other clear cause, the massive brain hemorrhage was thought to be secondary to prolonged severe hypoglycemia the infant suffered. She remains in global developmental delay at her age at 26 months. This case highlights the importance of early referral of neonatal hypoglycemia for prompt diagnosis and management to avoid the consequences of prolonged low blood glucose.