Spontaneous Hemothorax Complicating von Recklinghausen Disease: Case Report and Treatment Algorithm

Neurofibromatosis type 1 (NF1) is generally a benign disease but has the potential for rare and fatal complications. Vascular signs and symptoms associated with NF1 are reported in only 1–3% of patients. Pulmonary complications have been rarely described in the literature and spontaneous rupture of a major thoracic artery is, however, extremely uncommon. We report the case of a patient with NF1 admitted to the emergency department for the management of a spontaneous life-threatening left hemothorax. The patient became hemodynamically unstable after thoracic drainage. Computed tomography angiography revealed extravasation at the level of the eighth intercostal artery. Digital angiography showed an intercostal aneurysm. Catheterization of the eighth intercostal artery was unsuccessful and an open surgery was finally performed. We highlight the importance of recognizing that patients with a benign disease like NF1 may present to the emergency department with a spontaneous life-threatening hemothorax. Immediate transfer for embolization is not always the best treatment management but thoracotomy is preferred in unstable patients.