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先天性无痛觉患者的夏科氏脊柱关节病:两例报告并文献复习

Charcot spinal arthropathy in patients with congenital insensitivity to pain: a report of two cases and review of the literature.

作者信息

Staudt Michael D, Bailey Christopher S, Siddiqi Fawaz

机构信息

Department of Clinical Neurological Sciences, London Health Sciences Centre, Western University, 339 Windermere Road, London, ON, N6A 5A5, Canada.

Division of Orthopaedic Surgery, London Health Sciences Centre, Western University, London, ON, Canada.

出版信息

Neurosurg Rev. 2018 Oct;41(4):899-908. doi: 10.1007/s10143-017-0814-3. Epub 2017 Jan 26.

Abstract

Charcot spine, or neuropathic spinal arthropathy, involves the progressive destruction of the spinal joint due to the lack of normal protective sensations and proprioception. A rare cause of Charcot spine is congenital insensitivity to pain, which is an absent or abnormal response to painful stimuli. There are few case reports describing this condition, and long-term follow-up data are limited. The presentation and treatment of two patients with Charcot spine secondary to congenital insensitivity to pain are described. Both cases were characterized by lumbar involvement and were treated with circumferential decompression and an extended lumbo-pelvic fusion construct. The cases described here demonstrate stable neurological status at 1.5 and 5 years follow-up. Patient characteristics, pre- and post-operative imaging, operative approach, and outcomes are described. The literature regarding this rare condition is also reviewed, with an emphasis on operative management and outcomes. Surgical management is traditionally complicated by a high rate of hardware failure and adjacent segment degeneration. The current review highlights the importance of prompt and aggressive management following diagnosis of Charcot spine, as well as extended follow-up.

摘要

夏科氏脊柱,即神经性脊柱关节病,是由于缺乏正常的保护性感觉和本体感觉而导致脊柱关节的渐进性破坏。夏科氏脊柱的一个罕见病因是先天性无痛觉,即对疼痛刺激无反应或反应异常。描述这种情况的病例报告很少,长期随访数据也有限。本文描述了两名因先天性无痛觉继发夏科氏脊柱患者的临床表现及治疗情况。两例均以腰椎受累为特征,均接受了环形减压及扩大的腰骶骨盆融合固定治疗。本文所述病例在1.5年和5年随访时神经功能状态稳定。描述了患者特征、术前和术后影像学表现、手术方式及结果。还对有关这种罕见疾病的文献进行了综述,重点是手术治疗及结果。传统上,手术治疗因内固定失败率高和相邻节段退变而复杂化。当前的综述强调了夏科氏脊柱诊断后及时积极治疗以及延长随访的重要性。

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