REPLACEMENT OF THE DESCENDING THORACIC AORTA FOR MASSIVE ANEURYSM IN NEONATAL MARFAN-SYNDROME

We describe a patient with neonatal Marfan syndrome presenting with ma ssive ascending and descending thoracic aortic aneurysm. Because of ra pidly progressive respiratory distress due to tracheobronchial compres sion, emergency replacement of the descending thoracic aorta with a 12 -mm PTFE vascular prosthesis was undertaken at 1 month of age. The pos toperative course was complicated by bilateral tension pneumothorax co ntributing to irreversible respiratory failure. The unique clinico-pat hological features and the relevant surgical implications of the case are discussed.