Massive spontaneous hemothorax in patients with neurofibromatosis type 1.

Spontaneous hemothorax is an uncommon event that can occur in patients with a history of neurofibromatosis type 1 because of intrathoracic vascular malformations that predispose to aneurysms or bleeding from thoracic tumors. Only 53 cases of this rare association have been reported in the literature since 1975. We described 2 cases: one patient was a 73-year-old man with a right hemothorax secondary to an intercostal neurofibroma; the other was a 35-year-old woman with a left hemothorax secondary to a neurofibroma that compromised the internal mammary artery. Our review of the literature found that 61.8% of cases involved women with a mean age of 43.9 years. There was a certain tendency toward left-sided (56.4%) hemothorax, and the intercostal space was the most common site of bleeding. Treatment was most often surgical (58.2%) in reported cases, although selective artery embolization is also a valid choice. Exitus occurred during 30.9% of the reported episodes, and survival was higher in patients who were treated with surgery or arterial embolization than in those in whom only a thoracic drain was placed or who received no invasive treatment (P=.02).