Hyperinsulinisme congénital du nouveau-né : évolution après pancréatectomie subtotale

Congenital hyperinsulinism in newborns is a rare disease but it is a severe affection by its rebel hypoglycaemia that leads to important brain damage. We report the case of a full-term boy been born by caesarean for macrosomia with a born-weight of 4800 g. He presented from the first day of life severe and lasting hypoglycaemia in spite of a high glucose delivery rates (16 mg/kg per minute) and a treatment by diazoxide. The diagnosis of a congenital hyperinsulinism is then evoked and confirmed by several high insulinemia. Sandostatine was not accessible to this patient and a distal subtotal pancreatectomy was realized at 50 days of age. The anatomopathological examination of the pancreas does not find abnormalities. After a postoperative transient Diabetes mellitus, the glycaemia were normalized gradually. At five months of age, the glycemia was 4.8 mmol/L, the glycated haemoglobin (HbA1c) was 6.4% and the insulinemia was 1 μUI/mL. The transfontanelle ultrasonography and the electroencephalogram were normal. After two years of age, the child presents epilepsy mastered by the valproate of sodium and a severe psychomotor retardation.