A case of marfan syndrome successfully treated by elective surgery for persistent air leakage after the onset of hemopneumothorax

Abstract

An 18-year-old woman, who had been diagnosed as having Marfan syndrome, annuloaortic ectasia, aortic regurgitation, and mitral valve prolapse at 2 years of age, consulted a pediatrician complaining of left back pain. The patient was found to have a pneumothorax and a pleural effusion on chest X-ray. Tube thoracotomy was performed, and 800 ml of a bloody effusion was drained out. Although hemostasis was obtained, the air leakage continued. Thus, the patient was admitted to our department for surgical treatment. Bullae were detected at the apex of the left lung on computed tomography. After evaluation of the patient's cardiac function, the patient underwent thoracoscopic surgery. Fibrous adhesions with neovascuarzation and bullae were seen in the apex of the left lung. A thoracoscopic bullectomy was performed, and the patient's postoperative course was uneventful. The patient was discharged on the fifth postoperative day. It is important to avoid perioperative cardiovascular events in patients with Marfan syndrome who undergo surgery. Therefore, in patients with Marfan syndrome who require surgery for pneumothorax or hemopneumothorax, a cardiovascular evaluation using ultrasonography should be performed before surgery. The essential point of perioparative management in patients with Marfan syndrome is to avoid an elevation or a rapid change in the blood pressure.