神经纤维瘤病 1 型颈椎脊柱侧凸、脊髓分叉和椎动脉至硬膜外静脉瘘的矫正。

IF 1.4 Q2 OTORHINOLARYNGOLOGY Journal of Craniovertebral Junction and Spine Pub Date : 2024-01-01 Epub Date: 2024-03-13 DOI:10.4103/jcvjs.jcvjs_138_23
Devon LeFever, Thomas Hanks, Rakesh Kumar, Philip Louie, Jean-Christophe Leveque
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引用次数: 0

摘要

神经纤维瘤病-1(NF1)具有多系统影响,包括脊柱侧凸、硬膜异位和动静脉瘘(AVF),因此给患者带来了复杂的挑战。我们介绍了一例 31 岁男性 NF1 患者的病例,该患者患有严重的颈椎脊柱侧弯、硬膜外翻、颈部脊髓分叉和动静脉瘘,突显了处理此类复杂病例的复杂性。患者的右臂和右腿迅速变弱,影像学检查显示其颈椎严重畸形,硬脊膜褶皱将脊髓一分为二。手术治疗解决了涉及右侧椎动脉和硬膜外静脉的高流量动静脉瘘,因此必须切除动脉。患者接受了后路融合术和椎板切除术,术后神经状况稳定,三个月后感觉缺失和乏力明显改善。该病例强调了量身定制的纯后路方法(包括硬脊膜褶松解)的重要性,该方法可使脊髓重新定位到不太紧张的位置,从而对同时伴有脊柱后凸畸形和椎动脉AVF的复杂NF1病例进行有效减压。
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Correction of cervical kyphoscoliosis, bisected spinal cord, and vertebral artery to epidural vein fistula in neurofibromatosis type 1.

Neurofibromatosis-1 (NF1) presents complex challenges due to its multisystemic effects, including kyphoscoliosis, dural ectasia, and arteriovenous fistulas (AVF). We present a case of a 31-year-old male with NF1 exhibiting severe cervical kyphoscoliosis, dural ectasia, a bisected cervical cord, and an arteriovenous fistula, highlighting the intricacies of managing such intricate cases. Rapid weakening in the patient's right arm and leg prompted imaging revealing severe cervical kyphotic deformity and a dural fold dividing the spinal cord. Surgical intervention addressed a high-flow arteriovenous fistula involving the right vertebral artery and an epidural vein, necessitating sacrifice of the artery. Posterior fusion and laminectomy were performed, resulting in stable neurological status postoperatively and significant improvement in sensory loss and weakness at three months. This case underscores the importance of a tailored posterior-only approach, involving dural fold release, to allow the spinal cord to relocate to a less tense position, thus demonstrating effective decompression in complex NF1 cases with concurrent kyphotic deformity and vertebral artery AVF.

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来源期刊
CiteScore
1.90
自引率
9.10%
发文量
57
审稿时长
12 weeks
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