Beckwith-Wiedemann Syndrome And Prolonged Hypoglycemia

Macroglossia, prenatal or postnatal overgrowth, macrosomia, macroglossia, and abdominal wall defects (omphalocele, umbilical hernia, or diastasis recti) permit early recognition of BeckwithWiedemann syndrome. Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others. The frequency of hypoglycemia in this population is between 30% and 50%. The cause of hypoglycemia is unclear but supports a hyperinsulinemia as the major factor. The majority of infants with hypoglycemia will be asymptomatic and have resolution of the hypoglycemia within the first 3 days of life. Less than 5% will have hypoglycemia beyond the neonatal period. As conclusion, in patients with BWS levels of insulin and blood glucose must be monitorized beginning from first hours of life. Hypoglycemia may be prolonged in presence of hyperinsulinemia as like this present case.