Anaesthesia for Rett syndrome.

Rett syndrome is a devastatingly disabling neurological disease that is only observed in girls. Scoliosis occurs in roughly half the girls and surgery may be required. Anaesthesia is described in three patients. Sudden death may be a feature of the disease which occurred four weeks postoperatively in one case. Although a long QTc interval may be seen, it did not occur in any of our cases.