Spinal anaesthesia for caesarean section in a patient with Cockayne syndrome

Cockayne syndrome is a rare inherited disorder with photosensitivity, dysmorphism, short stature and neurological deficits. Sufferers with this condition rarely survive into reproductive life. We report the successful delivery of a woman in her second pregnancy with Cockayne syndrome, her first pregnancy having resulted premature labour, caesarean section under spinal anaesthesia with considerable hypotension, and neonatal death. On this occasion she was hospitalised at 19 weeks' gestation and delivered by elective caesarean section at 34 weeks. She had a Mallampatti score of 3, so spinal anaesthesia using 1.8 mL of hyperbaric bupivacaine was given with good effect and mother and baby did well. The aetiology and clinical symptoms of this rare congenital syndrome, together with the anaesthetic implications and management, are described and discussed.