A Case of Unexpected Tracheal Stenosis in Marfan Syndrome after Surgical Repair for Acute Type A Aortic Dissection / 日本心臓血管外科学会雑誌

ABSTRACT

A 29-year-old woman with severe chest and back pain was referred to our hospital. She exhibited the following physical symptoms of Marfan syndrome arachnodactyly, wrist sign, thumb sign, pectus excavatum, pes planus, scoliosis, and myopia. Computed tomography revealed a Stanford type A aortic dissection with dilatation of the aortic root, therefore, emergency surgery was performed. Total arch replacement, including an elephant trunk procedure, was performed, followed by valve-sparing aortic root replacement using the reimplantation technique. Following an initially uneventful postoperative course, she was reintubated on the third postoperative day due to laryngeal edema and aspiration. On the sixth postoperative day, it was difficult to ventilate her due to severe tracheal stenosis. Although we managed to return her to spontaneous breathing under proper sedation, it was difficult to maintain stable ventilation. She developed a recurrent respiratory distress following physical irritation such as intratracheal aspiration or a postural change. We believe that during the perioperative management of patients with Marfan syndrome with thoracic deformities, such as pectus excavatum and scoliosis, the possibility of postoperative tracheal stenosis due to tracheal fragility, tracheal edema, and compression of the surrounding tissues must be considered.