Anaesthesia and the Beckwith-Wiedemann syndrome

Infants with Beckwith-Wiedemann syndrome usually present different abnormalities which may require surgical correction. Anaesthetic management may be complicated by abnormal airway anatomy, congenital heart disease and severe hypoglycaemia. Careful preoperative evaluation, perioperative monitoring and suitable choice of anaesthetic technique are required for a successful outcome. We report the perioperative management of a patient with Beckwith-Wiedemann syndrome presenting for omphalocoele surgery on his first day of life and for bilateral inguinal hernia repair four months later.