Fatal right spontaneous haemothorax in Von Recklinghausen's disease.

Spontaneous massive haemothorax is uncommon and usually occurs as a result of pulmonary infarction, arteriovenous fistula, neoplasm, ruptured aortic aneurysm, rupture of pleural adhesions or pleural endometriosis. Massive haemothorax in Von Recklinghausen's disease occurs rarely but with potentially fatal results in spite of surgery. We present a case of a spontaneous massive exsanguinating haemothorax in a patient with neurofibromatosis type 1 caused by rupture of a branch of the right subclavian artery. Bleeding was probably due to neurofibromatous invasion of the arterial wall.