BACKGROUND: Von Recklinghausen’s disease, also known as Type 1 neurofibromatosis (NF1), is a genetic disorder characterized by skin tumors, neurofibromas of multiple organs and vascular abnormalities. Spontaneous thoracic hemorrhage is a rare but potentially fatal consequence of this disorder. After a review of the literature over the last 10 years and on the basis of a case study, the aim of this study was to report the challenges of management of this pathology.

CASE REPORT: We report a rare case of a 45-years-old male with a medical history of neurofibromatosis who complained of a 3-day history of progressive dyspnea. At his admission to the Emergency Department, the patient was hemodynamically stable. A chest computed tomography (CT) scan showed a large left hemothorax with mediastinal shift to the right without active bleeding. A chest tube was introduced, and conservative treatment was followed. Another CT scan performed 2 days later revealed a middle lobar pulmonary embolism on the opposite side. A full treatment of anticoagulation was administered, and the patient was released after 8 days of hospitalization. Three weeks later, a new chest CT scan indicated the absence of vascular aneurysm or source for hemothorax.

CONCLUSIONS: Our systematic literature review found 15 articles which were described as early as 2005. To our knowledge, endovascular treatment produces the best immediate successful result (100%) and may be used in adjunction with video-assisted thorax surgery (VATS) or thoracic drainage to optimize outcomes. In the present case, conservative treatment showed a good result despite anticoagulation for pulmonary embolism. The endovascular approach seems to be the most promising, but treatment needs to be tailored to each individual patient.

Keywords: Chest Tubes, Hemothorax, Neurofibromatosis 1, Pulmonary Embolism, Dyspnea, Tomography, X-Ray Computed