動静脈瘻により四肢麻痺を来たしたneurofibromatosis

Symptomatic arteriovenous fistula in a patient with neurofibromatosis typeⅠ

I．はじめに

　neurofibromatosis typeⅠ（NFⅠ）は常染色体優性遺伝形式をとり，全身の神経線維腫の発生を特徴とする疾患である^7）．血管系の異常を多く認めるものはvascular neurofibromatosisともいわれ，血管閉塞，動脈瘤，動静脈瘻（AVF）などの報告が見られる^7）．われわれは最近，頭蓋外椎骨動脈のAVFによる四肢麻痺で発症し，諸検査にてAVFとそれに隣接するfusiform動脈瘤，さらにモヤモヤ血管を伴う中大脳動脈の閉塞を合併したNFⅠを経験した．この症例に対してinterventionalに瘻孔を閉塞し，神経症状の著しい改善を認めたので，若干の考察と共に報告する．

This paper reports the case of a 59-year-old woman who had a history of neurofibromatosis (NF Ⅰ) present-ing progressive quadriplegia and urinary incontinence due to a cervical arteriovenous fistula (AVF). MRI re-vealed a huge flow void mass in the cervical sub-cutaneous 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.