2019 Volume 33 Issue 7 Pages 736-743
Patients with neurofibromatosis type 1 may develop massive hemothorax due to blood vessel fragility. A 43-year-old woman with neurofibromatosis type 1 was emergently transferred to our hospital because of left back pain. On dynamic computed tomography and thoracic puncture, a massive left hemothorax was noted. Since the circulation of the patient was stable, transcatheteric arterial angiography was performed. An intercostal arterial aneurysm was pointed out and transcatheteric arterial embolization was performed for it. However, the intrathoracic bleeding was not controlled, and the patient entered a hemorrhagic shock state. Emergency thoracotomy was performed, and a wide range of intercostal arteries were ligated based on the findings of angiography. However, it was difficult to achieve hemostasis because the fragility of the patient's blood vessels. The coils embolized in the intercostal artery were easily exposed in the surgical field. Finally, bleeding was controlled by pressure hemostasis using various surgical dressings. In past reports, seventy-seven patients with neurofibromatosis type 1 developed massive hemothorax. A total of eighty-three episodes of hemothorax occurred, and the life-saving rate was 74.4%. If the circulation of a patient is stable, transcatheteric arterial embolization may be the first-choice treatment. However, patients with neurofibromatosis type 1 have fragile blood vessels, and there are some cases whereby it is difficult to stop bleeding with transcatheteric arterial embolization.
