[Symptomatic arteriovenous fistula in a patient with neurofibromatosis type I]

This paper reports the case of a 54-year-old woman who had a history of neurofibromatosis (NF I) presenting progressive quadriplegia and urinary incontinence due to a cervical arteriovenous fistula (AVF). MRI revealed a huge flow void mass in the cervical subcutaneous tissue as well as within the spinal canal. These flow voids originated in the left vertebral artery. A high intensity lesion was observed in the spinal cord adjacent to the flow void. Angiography revealed that the cervical AVF was fed by the third segment of the left vertebral artery with a rich communication with the intraspinal veins. Furthermore, an aneurysmal dilatation of the proximal vertebral artery and occlusion of the right middle cerebral artery with moyamoya vessels were found. Endovascular treatment using a Goldvalve detachable balloon successfully obliterated the AVF. Postoperative MRI and angiography showed evidence of the disappearance of AVF and postoperatively, the patient's neurological signs improved gradually. Sixteen reported AVFs accompanied with NF were reviewed.