ANESTHESIA AND THE BECKWITH-WIEDEMANN SYNDROME

Infants with Beckwith-Wiedeman syndrome usually present different abno rmalities which may require surgical correction. Anaesthetic managemen t may be complicated by abnormal airway anatomy, congenital heart dise ase and severe hypoglycaemia. Careful preoperative evaluation, periope rative monitoring and suitable choice of anaesthetic technique are req uired for a successful outcome. We report the perioperative management of a patient with Beckwith-Wiedemann syndrome presenting for omphaloc oele surgery on his first day of life and for bilateral inguinal herni a repair four months later.