Tracheal compression in a patient with Marfan's syndrome-associated tracheomegaly treated by an XXL stent: the largest diameter airway stent ever placed in a previously undescribed airway condition.

摘要

A 43-year-old man was referred to our institution with severe extrinsic compression of the trachea at the level of the main carina secondary to an aortic aneurysm, causing respiratory distress and requiring mechanical ventilation. The patient had a past history of Marfan's syndrome and tracheomegaly (the estimated tracheal diameter bronchoscopically was 28 mm). Palliation of the compression was successfully achieved by a custom-made, self-expandable, fully covered metallic stent with a diameter of 28 mm and a length of 60 mm. The patient was weaned off ventilation. The stent stayed in place for 2 years without major complications. This case represents the first stent ever inserted in a very rare condition combining tracheomegaly and extrinsic compression in Marfan's syndrome. It is also the first report of successful placement of the largest tracheal stent which was manufactured exclusively for the airway.