Anaesthesia and Marfan’s syndrome: case report

Abstract

The incidence of Marfan's syndrome in the general population is thought to be between 1:10,000 and 1:50,000. t Hence it is possible that at some stage during their practising life many anaesthetists will be called upon to treat such a patient. We recently anaesthetized a patient for a cardiac procedure who demonstrated most of the features of this generalised disorder of connective tissue.Case report An 18-year-old male presented to hospital four days after the acute onset of left sided chest and neck pain. The pain was associated with severe dyspnoea, nausea and faintness and lasted about 15 seconds. It was followed by constant, heavy retrosternal chest pain. Similar episodes of diffuse, heavy chest pain were described on several occasions over the previous two years.The patient gave a history of multiple recurrent dislocations of both shoulders and also of a dislocation of his left ankle. He had undergone surgery at the age of 12 for the correction of a severe pectus excavatum deformity, at 15 years of age for osteo-chondritis dissecans of the left knee and at 17 years, for torsion of the fight testis.Examination of this tall (185 cm), thin (62 kg) patient revealed the typical features of Marfan's syndrome; an arm span that was greater than his height, a cathedral palate, winging of the scapulae, ulnar deviation of fire mctacarpophalangeal joints and residual pectus excavatum. He had also an abberant left radial artery, situated on the dorsum of the forearm.There were significant abnormalities on examination of the cardiovascular system. The blood pressure in the left arm was 110160 mmHg and in the right arm 120f70 mmHg. His apex beat was widely displaced to the 7th left intercostal space, mid axillary line. There was an ejection systolic murmur heard to the right of the sternum with a mid-systolic click at the left sternal edge.A chest x-ray showed a prominent and slightly tortu ous aorta with cardiomegaly and a marked thoracic scoliosis. There were no interstitial pulmonary changes. His ECG showed right atrial hypertrophy and large left ventricular complexes. The following day an aortogram was performed, outlining a large false aortic sac with a possible entry site proximal to the innominate artery. The aortic root was not outlined. He was treated with oral CAN J ANAESTH 1987 ! 34:3 / pp311-4

Anesthetic management of a child with both Marfan syndrome and Turner syndrome

“…Prognathism and a high palata may pose difficulties during tracheal intubation. Positive pressure ventilation may cause pneumothorax [12,13]. The anesthetic approach employed in the present case took into account the association of Marfan sydrome with Turner syndrome.…”

Marfan syndrome

“…Hyperextensibility of joints with habitual dislocations is common. [18] Kyphosis or scoliosis may be present. In eyes, bilateral ectopia lentis caused by weakening or rupture of suspensory ligaments are common.…”

“…[1] The prevalence has been estimated as 1 in 5–10,000 individuals. The main target organs of this disorder are cardiovascular system, eyes, and skeleton.…”

Tension pneumothorax accompanied by type A aortic dissection

“…Marfan's syndrome was the suspected cause of tension pneumothorax and type A aortic dissection 1 2. However, there was no family history of Marfan's syndrome or ectopia lentis, and skeletal findings were not confirmed.…”