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脊髓空洞症的骨科表现:综述

Orthopedic Manifestations of Syringomyelia: A Comprehensive Review.

作者信息

Fadila Mohamad, Sarrabia Geva, Shapira Shay, Yaacobi Eyal, Baruch Yuval, Engel Itzhak, Ohana Nissim

机构信息

Orthopedic Department, Meir Medical Center, Kfar Saba, Affiliated to Faculty of Health Sciences and Medicine, Tel-Aviv University, Tel Aviv 6997801, Israel.

Spine Surgery Unit, Meir Medical Center, Affiliated to Faculty of Health Sciences and Medicine, Tel-Aviv University, Tel Aviv 6997801, Israel.

出版信息

J Clin Med. 2025 May 1;14(9):3145. doi: 10.3390/jcm14093145.

Abstract

: Syringomyelia is a complex neurological disorder characterized by a fluid-filled cavity (syrinx) within the spinal cord, frequently resulting from altered cerebrospinal fluid (CSF) dynamics. While its clinical manifestations are diverse, orthopedic complications such as scoliosis, pes cavus, and Charcot arthropathy may represent early diagnostic clues yet are often under-recognized. : This comprehensive review synthesizes the current literature on the pathophysiology, clinical presentation, diagnostic strategies, and management approaches of syringomyelia, with a specific emphasis on its orthopedic manifestations. Additionally, we present a detailed case of neuropathic shoulder arthropathy associated with advanced syringomyelia. : Orthopedic involvement in syringomyelia includes progressive spinal deformities and neurogenic joint destruction, particularly affecting the shoulder and elbow. Scoliosis is frequently observed, especially in association with Chiari malformations, and may precede neurologic diagnosis. Charcot joints result from impaired proprioception and protective sensation. The case presented illustrates the diagnostic challenges and therapeutic dilemmas in managing advanced neuro-orthopedic complications in syringomyelia. : Syringomyelia should be considered in the differential diagnosis of atypical musculoskeletal presentations. Early recognition and multidisciplinary management are essential to prevent irreversible orthopedic sequelae. Conservative treatment remains the mainstay in stable cases, while surgery is reserved for progressive disease. Orthopedic assessment plays a pivotal role in the diagnostic pathway and long-term care of affected patients.

摘要

脊髓空洞症是一种复杂的神经系统疾病,其特征是脊髓内有一个充满液体的腔隙(空洞),通常由脑脊液(CSF)动力学改变引起。虽然其临床表现多种多样,但诸如脊柱侧弯、高弓足和夏科关节病等骨科并发症可能是早期诊断线索,但往往未得到充分认识。

本综述综合了目前关于脊髓空洞症的病理生理学、临床表现、诊断策略和管理方法的文献,特别强调了其骨科表现。此外,我们还介绍了一例与晚期脊髓空洞症相关的神经性肩关节病的详细病例。

脊髓空洞症的骨科表现包括进行性脊柱畸形和神经源性关节破坏,尤其影响肩部和肘部。脊柱侧弯很常见,特别是与 Chiari 畸形相关时,可能在神经学诊断之前出现。夏科关节是由本体感觉和保护性感觉受损引起的。所呈现的病例说明了在处理脊髓空洞症晚期神经骨科并发症时的诊断挑战和治疗困境。

在非典型肌肉骨骼表现的鉴别诊断中应考虑脊髓空洞症。早期识别和多学科管理对于预防不可逆的骨科后遗症至关重要。在病情稳定的情况下,保守治疗仍然是主要方法,而手术则用于治疗进行性疾病。骨科评估在受影响患者的诊断途径和长期护理中起着关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0c91/12072671/66ec4f576430/jcm-14-03145-g001.jpg

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