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.
We describe the anaesthetic management of the surgical separation of a pair of thoracopagus-cardiopagus twins with a common right atrium and a myocardial tissue bridge containing vascular channels between their ventricles. One of them died during the procedure, the surviving twin is now two years old. The survival of one twin for two years without significant sequelae, after the surgical separation of twins with shared right atrium and fused ventricles, has not previously been reported. Careful preoperative assessment is essential to anticipate potential serious problems during the procedure.
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