Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report

Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of large intrathoracic vessels, and dysplastic alterations of small vessels in highly vascularized mesenchymal tumors. Most commonly, tumor-related hemothorax is due to neurofibroma, and the most frequently involved artery is the intercostal artery.

A 48-year-old male referred to our hospital with a chief complaint of dyspnea from a week ago. His symptoms began with pleuritic chest pain followed by dyspnea. The physical examination showed many neurofibromatosis lesions and café-au-lait macules on the skin. Thoracotomy was performed during the hemothorax examination. Moreover, inflammation of the pleura and fibrothorax were reported without any site of bleeding.

Vascular complications should be regarded in NF-1. Any patient presents with hemodynamic disorder or pleural effusion should be examined and treated promptly