Spontaneous Hemothorax in a Patient With von Recklinghausen's Disease

Type I neurofibromatosis (NF-1) is a rare autosomal dominant disease. It can affect any organ system including vascular tissues. A 53 years old man, with a past medical history of NF-1, retinitis pigmentosa and hypertension attended to the emergency department for chest pain and palpitations and was discharged 2 days after acute coronary syndrome was ruled out. During this admission an echocardiogram was performed which showed a left ventricular hypertrophy with normal ejection fraction and a chest X-ray which revealed no pathologic images. No invasive procedures were preformed. Three days after discharge, he returned to our hospital for sudden onset of oppressive chest pain in the right arm, irradiated to the ipsilateral shoulder, chest and back. After several tests, a diagnosis of hemothorax was made. Hemoglobin levels declined during the first 2 days of admission from 12.1 to 9.6 g/dL, although the patient remained hemodynamic stable. An arteriography was performed, which showed the presence of bleeding from a branch of the right subclavian artery, which was selectively catheterized and embolized with coils. Afterwards, a video-assisted thoracoscopy was made, in order to drain the hemothorax and to carry out a visual review of the pleural cavity. The patient had a good clinical and radiologic progression and was discharged after few days. After a year of follow-up, the patient has remained clinically asymptomatic with no further episodes of active bleeding.