Neurofibromatosis type 1 (NF1)-associated vasculopathy is uncommon but may result in catastrophic arterial rupture. We report a life-threatening presentation successfully managed with emergency endovascular exclusion. A 44-year-old woman with known NF1 presented with acute chest pain, dyspnea, and dysphonia. Computed tomography angiography demonstrated a 23mm ruptured aneurysm arising from the ostium of the left vertebral artery associated with hemomediastinum, airway displacement, and massive hemothorax. Emergency endovascular repair using covered stent-grafts achieved complete aneurysm exclusion while preserving vertebral artery patency. Thoracic drainage was required. The postoperative course was uneventful without neurologic deficit.
Michiels et al. (2026) studied this question.