Spontaneous Hemothorax in Neurofibromatosis Type 1: A Surgical Case Report

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder associated with vascular abnormalities, including spontaneous hemothorax and arterial aneurysms. We present a rare case of spontaneous hemothorax in which an apparently hemostatic sub-pleural hematoma began to bleed again after the patient was repositioned. A 47-year-old man with NF-1 presented with the sudden onset of left-sided chest pain. Contrast-enhanced chest computed tomography revealed a massive left-sided hemothorax with a tortuous intercostal artery and an adjacent hematoma. The patient underwent emergency video-assisted thoracoscopic surgery (VATS), which revealed extensive intrathoracic clots and a subpleural hematoma near the aortic arch. Although no active bleeding was observed intraoperatively, hemostatic agents were applied to the suspected site. However, massive rebleeding occurred following patient repositioning, prompting an urgent re-thoracotomy. Active bleeding from the intercostal artery was identified and successfully controlled. The patient recovered without recurrence of hemothorax. Spontaneous hemothorax in patients with NF-1 warrants early recognition and surgical intervention. Vascular reconstruction is often challenging due to arterial fragility, underscoring the importance of careful intraoperative hemostasis and treatment selection. Furthermore, surgeons should be aware that temporary hemostasis may be achieved due to tamponade by a subpleural hematoma, potentially masking an underlying vascular injury. Careful intraoperative assessment and readiness for reintervention are essential in managing such cases.