Caesarean section in a parturient with respiratory failure caused by cystic fibrosis

We describe a 27-year-old primigravida suffering from cystic fibrosis. Her chest was colonised with Burkholderia cepacia and she was in respiratory failure for which she required constant nasal intermittent positive pressure ventilation. In view other rapid deterioration, Caesarean section was performed under epidural anaesthesia at 25 weeks gestation. A live 790-g boy was delivered. Post-operatively she made steady progress for 5 days although still requiring nasal ventilatory support. Thereafter she developed pneumonia and required tracheal intubation and ventilation on the eighth day. Her increasing hypoxaemia and pulmonary hypertension failed to respond to any therapy including inhaled nitric oxide and she died on the tenth postoperative day.