Ruptured Occipital Artery Aneurysm Following Craniotomy in a Patient with Neurofibromatosis Type 1: Case Report.

Objective: Neurofibromatosis type 1 (NF1) is associated with vascular fragility, which results in aneurysms, arteriovenous fistulas, and dissections. Here, we describe a case of endovascular treatment of a ruptured occipital artery aneurysm that occurred after a craniotomy in a patient with NF1.

Case presentation: A 46-year-old man with a history of NF1 underwent a right lateral suboccipital craniotomy to remove a cavernous hemangioma in the right middle cerebellar peduncle. Severe bleeding occurred in the occipital artery during the craniotomy. Due to vessel fragility, coagulation and ligation were not possible, and pressure hemostasis was achieved using cellulose oxide and fibrin glue. On postoperative day 12, the patient developed a sudden swelling on the right side of the neck as well as tracheal compression. Contrast-enhanced CT revealed a ruptured aneurysm in the right occipital artery. Transarterial embolization was performed under general anesthesia the same day. Right external carotid angiography showed an 18-mm-diameter fusiform aneurysm in the occipital artery. The aneurysm ruptured inferiorly to form a large pseudoaneurysm with significant jet flow. An arteriovenous fistula was also observed in a nearby vein. A microcatheter was inserted into the fusiform aneurysm under proximal blood flow control, and embolization was performed using coils and N-butyl-2-cyanoacrylate.

Conclusion: Compared to surgical repair of ruptured occipital artery aneurysms, endovascular treatment appears to be safe, effective, minimally invasive, and rapid. Ruptured occipital artery aneurysms in NF1 patients can cause neck swelling and airway compression and should be recognized as a potentially lethal condition.