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Correction of cervical kyphoscoliosis, bisected spinal cord, and vertebral artery to epidural vein fistula in neurofibromatosis type 1

Authors :
Devon LeFever
Thomas Hanks
Rakesh Kumar
Philip Louie
Jean-Christophe Leveque
Source :
Journal of Craniovertebral Junction and Spine, Vol 15, Iss 1, Pp 123-126 (2024)
Publication Year :
2024
Publisher :
Wolters Kluwer Medknow Publications, 2024.

Abstract

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.

Details

Language :
English
ISSN :
09748237
Volume :
15
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Journal of Craniovertebral Junction and Spine
Publication Type :
Academic Journal
Accession number :
edsdoj.9a90a0b2620b4cb380ec330268f48998
Document Type :
article
Full Text :
https://doi.org/10.4103/jcvjs.jcvjs_138_23