Acute Fatal Chest Pain: Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type I

NF-1 is a genetic disorder with a rare incidence rate of approximately 1 in 3000. Arterial stenosis due to connective tissue dysplasia is a well-known vascular complication of NF-1, as well as aneurysms, which constitutes 3.6% of people diagnosed with this disease. Spontaneous hemothorax is one of the rare complications of NF-1. It is potentially fatal with a mortality rate as high as 36% and postoperative mortality of 33%. Massive and potentially fatal hemothorax caused by vascular complications is extremely uncommon. We present a rare case of massive hemothorax in a 62-year-old woman with NF-1 due to a spontaneous rupture of the right intercostal artery.