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一种治疗Klippel-Feil综合征患者基底凹陷的新技术:一个临床实例及文献简要综述

A Novel Technique for Basilar Invagination Treatment in a Patient with Klippel-Feil Syndrome: A Clinical Example and Brief Literature Review.

作者信息

Tanaka Masato, Askar Abd El Kader Al, Kumawat Chetan, Arataki Shinya, Komatsubara Tadashi, Taoka Takuya, Uotani Koji, Oda Yoshiaki

机构信息

Department of Orthopaedic Surgery, Okayama Rosai Hospital, Okayama 702-8055, Japan.

Department of Orthopaedic Surgery, Okayama University Hospital, Okayama 700-8558, Japan.

出版信息

Medicina (Kaunas). 2024 Apr 10;60(4):616. doi: 10.3390/medicina60040616.

Abstract

: To present a novel technique of treatment for a patient with basilar invagination. Basilar invagination (BI) is a congenital condition that can compress the cervicomedullary junction, leading to neurological deficits. Severe cases require surgical intervention, but there is debate over the choice of approach. The anterior approach allows direct decompression but carries high complication rates, while the posterior approach provides indirect decompression and offers good stability with fewer complications. : A 15-year-old boy with severe myelopathy presented to our hospital with neck pain, bilateral upper limb muscle weakness, and hand numbness persisting for 4 years. Additionally, he experienced increased numbness and gait disturbance three months before his visit. On examination, he exhibited hyperreflexia in both upper and lower limbs, muscle weakness in the bilateral upper limbs (MMT 4), bilateral hypoesthesia below the elbow and in both legs, mild urinary and bowel incontinence, and a spastic gait. Radiographs revealed severe basilar invagination (BI). Preoperative images showed severe BI and that the spinal cord was severely compressed with odontoid process. : The patient underwent posterior surgery with the C-arm free technique. All screws including occipital screws were inserted into the adequate position under navigation guidance. Reduction was achieved with skull rotation and distraction. A follow-up at one year showed the following results: Manual muscle testing results and sensory function tests showed almost full recovery, with bilateral arm recovery (MMT 5) and smooth walking. The cervical Japanese Orthopedic Association score of the patient improved from 9/17 to 16/17. Postoperative images showed excellent spinal cord decompression, and no major or severe complications had occurred. : Basilar invagination alongside Klippel-Feil syndrome represents a relatively uncommon condition. Utilizing a posterior approach for treating reducible BI with a C-arm-free technique proved to be a safe method in addressing severe myelopathy. This novel navigation technique yields excellent outcomes for patients with BI.

摘要

介绍一种治疗基底凹陷患者的新技术。基底凹陷(BI)是一种先天性疾病,可压迫颈髓交界处,导致神经功能缺损。严重病例需要手术干预,但对于手术方式的选择存在争议。前路手术可直接减压,但并发症发生率高,而后路手术提供间接减压,并发症较少且稳定性良好。

一名15岁患有严重脊髓病的男孩因颈部疼痛、双侧上肢肌肉无力和手部麻木持续4年就诊于我院。此外,在就诊前三个月,他出现麻木加重和步态障碍。检查发现,他双上肢和双下肢均有反射亢进,双侧上肢肌肉无力(MMT 4级),双侧肘部以下和双腿感觉减退,轻度大小便失禁,步态痉挛。X线片显示严重的基底凹陷(BI)。术前影像显示严重的BI,脊髓被齿状突严重压迫。

该患者采用无C形臂技术进行了后路手术。所有螺钉,包括枕骨螺钉,均在导航引导下插入合适位置。通过颅骨旋转和牵引实现复位。一年后的随访结果如下:徒手肌力测试结果和感觉功能测试显示几乎完全恢复,双侧手臂恢复(MMT 5级),行走平稳。患者的颈椎日本矫形外科学会评分为从9/17提高到16/17。术后影像显示脊髓减压良好,未发生重大或严重并发症。

基底凹陷合并Klippel-Feil综合征是一种相对罕见的疾病。采用无C形臂技术的后路手术治疗可复位的BI被证明是治疗严重脊髓病的一种安全方法。这种新颖的导航技术为基底凹陷患者带来了极佳的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ee/11051808/5be43783d8b0/medicina-60-00616-g001.jpg

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