ABSTRACT

Marfan syndrome is a heritable disorder of the connective tissue. Management of anesthesia for Marfan’s syndrome has specific precautions for valvular diseases, thoracic aortic aneurysm and skeletal joint laxity. Patients with Marfan’s syndrome must be carefully assessed preoperatively for specific complications. Perioperative hypertension and hypotension must be avoided. Patients may have significant pulmonary problems including restriction of lung function due to pectus excavatum or kyphoscoliosis, intrinsic pulmonary involvement with emphysema, bronchogenic cysts and "honeycomb lung" that lead to spontaneous pneumothorax.

The anesthetic technique chosen should not decrease myocardial contractility and should avoid sudden increases in contractility, in order to minimize the risk of aortic dissection or rupture. A 10-year-old child with Marfan’s syndrome was planned to undergo surgery for repair of bilateral subluxation of lenses. Following prophylaxis for infective endocarditis, thiopental and fentanyl was administered for anesthesia induction and vecuronium was used for endotracheal intubation. Laryngoscopy was made carefully as not to cause joint dislocation. Haemodynamic stability was assessed during anesthesia.

The aim of this report is to discuss anesthetic management in Marfan’s syndrome and specific complications that could be seen during and following the operation.