Anaesthesia and Marfan's syndrome: case report

Abstract

An 18-year-old male with Marfan's syndrome underwent surgery for repair of an ascending aortic dissection. The clinical features of this congenital syndrome and the acute management of its complications are described. As elective surgery is frequently required for ocular, musculo-skeletal or cardiovascular problems in people with Marfan's syndrome, patients must be carefully assessed preoperatively for specific complications. Significant pulmonary problems include restriction of lung function due to pectus excavatum or kyphoscoliosis and intrinsic pulmonary involvement with emphysema, bronchogenic cysts and "honeycomb lung." This leads to a significant incidence of spontaneous pneumothorax and the danger of tension pneumothorax with positive pressure ventilation. The commonest causes of sudden death are cardiovascular complications, in particular rupture and dissection of the ascending aorta. Preoperative assessment should include echocardiography to determine the size of the aortic root. The anaesthetic technique chosen should both decrease myocardial contractility and avoid sudden increases in contractility, in order to minimise the risk of aortic dissection or rupture.