Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type 1 with Successful Transarterial Embolization
A 46-year-old Thai female presented with acute dyspnea and pleuritic chest pain which she had felt for one day. On general physical examination, she had tachypnea and subcostal retraction. Additionally, she had multiple café-au-lait spots on her trunk, plexiform neurifibroma on her left leg, severe kyphoscoliosis and pectus carinatum. Her blood pressure was 150/90 mm Hg, heart rate 34 beats/minute, and respiratory rate 122 breaths/minute. Oxygen saturation at room air was 85% and increased to 99% after she was given an oxygen mask with a bag 10 liters/minute. A chest examination showed decreased breathing sounds as well as dullness, on percussion, at the left lung; the trachea had shifted to the right. Her hematocrit value and hemoglobin levels were 30.6% and 9.4 mg/dL, respectively. Platelet count and coagulogram were within the normal limits.\nThe patien's chest film revealed haziness in her left lung, mediastinum shift to the right, and severe kyphoscoliosis (). She was referred to our department for an emergency computed tomography (CT) angiography of the chest in order to rule out a pulmonary embolism. The chest CT angiography did not reveal a pulmonary embolism. There was a massive left hemothorax with a total collapse of the left lung. An 8.0-mm saccular aneurysm was detected in the proximal left 5th intercostal artery, with adjacent sentinel hematoma (). A ruptured aneurysm with massive left hemothorax was diagnosed. An incidental intrathecal cyst from T4-T8 levels, possibly thoracic meningocele, was also present (not shown). The patient was scheduled for an emergency embolization.\nAt the interventional unit, a chest tube was inserted, in order to relieve dyspnea, using a 10 Fr Pigtal catheter (Flexima, Boston-Scientific, Cork, Ireland). A large amount of flesh blood was obtained. Then, a digital subtraction aortogram was performed, using a 5 Fr Pigtail catheter (Boston-Scientific, Cork, Ireland) from the right common femoral arterial approach. Findings showed a small saccular aneurysm at the proximal left 5th intercostal artery, with adjacent distal arteriovenous fistula between the left 5th intercostal artery and vein, which drained into the hemiazygose vein (). No contrast extravasation was detected. Selection of the left 5th intercostal artery was performed using a 5 Fr Mik catheter (Boston-Scientific, Cork, Ireland). Then, 5 mL of non-ionic contrast was administrated to clearly define the lesion and identify the proximal neck (). A 2.7 Fr microcatheter (Progreat, Terumo, Leuven, Belgium) was advanced through the 5 Fr Mik catheter into the artery directly proximal to aneurysm. Following this, the embolization was performed via a microcatheter, using a single fiber platinum microcoil (4 mm-2 cm, Diamond-18, Boston-Scientific, Cork, Ireland).\nThe post-embolization angiogram showed successful occlusion of the aneurysm and arteriovenous fistula (). Completion aortography confirmed complete occlusion of the lesion and an absence of other potential bleeding (). After embolization, the patient's clinical signs improved. The chest tube was removed on the seventh day after embolization. Follow-up chest radiograph revealed near-complete resolution of left pleural effusion, and norecurrent bleeding occurred. The patient was discharged on the twelfth day after embolization.\nAt her oneyear follow-up, the patient's general condition was good, and CT angiography of the chest revealed complete occlusion of the prior aneurysm. However, there was recurrence of a small arteriovenous fistula (). The re-embolization was recommended, but the patient refused the procedure because of no symptoms related to the fistula.