Spontaneous Haemothorax Due to an Intercostal Artery Aneurysm in a Patient with Neurofibromatosis Type I

Background Haemothorax is characterized by pleural fluid with a haematocrit exceeding 50% of whole blood. While mostly associated with thoracic trauma, spontaneous haemothorax (SH) can be associated with vascular abnormalities. Neurofibromatosis type I (NF1), a genetic disorder affecting connective tissue integrity, is a rare cause of SH. We report a case of SH secondary to an intercostal artery aneurysm in a patient with NF1.

Methods & Materials A 37-year-old male with Crohn’s disease and type I neurofibromatosis was admitted for intense left-sided chest pain following a milder episode two days prior. Initial assessment excluded myocardial infarction (negative troponin, normal ECG). Still, chest CT revealed left pleural effusion and a 13 mm aneurysm of the tenth intercostal artery at the para-aortic level, suggesting spontaneous haemothorax from aneurysmal rupture. No active bleeding was detected.

During transfer to our thoracic surgery department, hemoglobin levels declined from 12.5 to 11.3 g/dL but remained within normal limits, and the patient was hemodynamically stable, obviating transfusion. Given the high surgical risk of intraoperative bleeding, a multidisciplinary approach was chosen. Interventional radiology performed a transfemoral TEVAR in the descending thoracic aorta, followed by coil embolization of the intercostal artery.

Postprocedural CT showed an increased haemothorax volume, but the patient remained haemodynamically stable. The following day, VATS with biportal access was performed for haemothorax evacuation. Intraoperatively, blood accumulation was mainly extrathoracic, with parietal pleura detachment; partial pleurectomy was conducted to facilitate lung adhesion. No active bleeding was observed.

Recovery was uneventful, with stable hemoglobin and no complications. The patient was discharged after 12 days, with lifelong aspirin (100 mg/day) recommended for secondary prevention.

Results Arterial lesions, including aneurysms, affect 2.3–3.6% of neurofibromatosis (NF) patients, predisposing them to spontaneous hemothorax due to vascular abnormalities like intimal proliferation and arterial dysplasia. In stable patients, coil embolization is preferred over conservative management. Thoracotomy is reserved for active bleeding, with a 40% mortality rate. NF-related mortality (33 – 36%) remains high, necessitating preventive strategies.

Conclusion This case highlights the necessity of an interdisciplinary approach in managing intercostal artery aneurysms and hemothorax in neurofibromatosis type I.

Publication History

Article published online:
25 August 2025