Atlantoaxial Instability in Neurofibromatosis Type 1: A Case Report.

Sankalp Lal, Pankaj Kumar Damor, Mahendra Singh Tak, Mukesh Kumar Saini, Mahesh Bhati
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Abstract

Introduction: Neurofibromatosis (NF) is a cluster of conditions in which tumors grow in the nervous system. Even though the spine is the most common site of skeletal involvement in NF type 1 (NF-1), cervical spinal anomalies are infrequent.

Case report: The atlantoaxial joint is a critical transitional zone in the cervical spine and is prone to instability. We report the case of a 49-year-old gentleman with atlantoaxial instability due to NF-1 who presented with quadriparesis and was managed by reduction with skull traction followed by C1-C2 Goel-Harms fixation.

Conclusion: In the present case, there was substantial bleeding and significant vertebral dysplasia, which makes the case distinctly unique. Skull traction and C1-C2 Goel-Harms fixation with fusion should be considered for patients with NF-1 complicated by atlantoaxial instability.

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简介神经纤维瘤病(NF)是一种在神经系统中生长肿瘤的疾病。尽管脊柱是神经纤维瘤病 1 型(NF-1)最常见的骨骼受累部位,但颈椎异常并不常见:病例报告:寰枢关节是颈椎的关键过渡区,容易发生不稳定。我们报告了一例因 NF-1 导致寰枢椎不稳定的 49 岁男性病例,该患者出现四肢瘫痪,通过颅骨牵引复位后进行了 C1-C2 Goel-Harms 固定术:结论:在本病例中,存在大量出血和明显的椎体发育不良,这使得该病例具有明显的特殊性。对于并发寰枢椎不稳定的 NF-1 患者,应考虑采用颅骨牵引和 C1-C2 Goel-Harms 固定融合术。
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审稿时长
30 weeks
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