[Massive mediastinal bleeding due to spontaneous rupture of the vertebral artery in von Recklinghausen disease].

In Von Recklinghausen disease, patients rarely demonstrate vascular abnormalities, but several reports have noted massive bleeding from vascular rupture. A 28-year-old man with Von Recklinghausen disease was admitted to our hospital because of swelling on the left neck accompanied by dyspnea. Neck and thoracic CT revealed a high density shadow on the mediastinum and left upper thorax which was suspected to indicate hemomediastinum. Under suspicion of bleeding from the intrathoracic artery, the patient underwent on surgery. Massive hematoma disrupted visual recognition of bleeding point, but under anatomical consideration, we concluded that the bleeding was from the ruptured left vertebral artery (VA) which branched independently from the aorta. The left VA was ligated distally and proximally. Left subclavian artery, injured unexpectedly, was not reconstructed but sutured carefully because of its fragility. Thus massive bleeding was controlled. Histology of the neighboring subclavian artery revealed involvement with neurofibrinomatosis. Although the patient's left arm demonstrated a slightly low blood pressure, he was discharged without complaint on the 56th postoperative day.