Scoliosis Correction in an Adolescent with a Rigid Spine Syndrome: Case Report
Scoliosis Correction in an Adolescent with a Rigid Spine Syndrome: Case Report
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患有脊柱强直综合症的青少年脊柱侧弯矫正：病例报告
DOI：
10.1097/01.brs.0000182313.40748.d3
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发表时间：
2005
期刊：
影响因子：
3
通讯作者：
J. Dormans
中科院分区：
文献类型：
--
作者：
A. Arkader;H. Hosalkar;J. Dormans
研究设计. 1例强直性脊柱综合征（RSS）患者脊柱侧凸的详细介绍和治疗方法。Objective.报告脊柱侧凸合并RSS的保守和手术治疗结果，并在此基础上提出评估和治疗方案。背景数据总结。先天性肌营养不良症（CMD）是一组以出生时肌张力减退和一般非进行性肌无力为特征的疾病。许多CMD患者存在脊柱强直。RSS被归类为CMD，其特征是脊柱早期僵硬、肢体痉挛和限制性呼吸功能障碍。RSS脊柱侧凸的治疗方法尚未建立。方法.详细的病史，诊断测试和治疗的青少年RSS与进行性脊柱侧凸和颈椎伸展挛缩。支具治疗的作用，Botox®（Allergan，Inc.，Irvine，CA），并定义了手术矫正的细节，包括背部肌肉的组织学。结果在这个对支具治疗和Botox®注射无反应的刚性脊柱侧凸的RSS病例中，成功地进行了前路胸腰椎融合术和内固定术以矫正脊柱侧凸，并通过后路入路和枕颈融合术解决了颈椎问题。我们概述了手术过程的细节和恢复脊柱平衡的矢状面和冠状面。结论. CMD的染色体基础和RSS的特征，包括诊断测试，进行了审查。对于保守治疗无效的进行性脊柱不平衡的RSS，采用脊柱畸形融合、矫正和内固定的手术干预是安全的，可以提供有希望的结果。
Study Design. Details of presentation and approach to the treatment of scoliosis in a case of a Rigid Spine Syndrome (RSS). Objective. To report on the results of conservative and operative treatment of scoliosis associated with RSS, and, based on this, to propose an assessment and treatment protocol for this condition. Summary of Background Data. Congenital muscular dystrophies (CMD) are a group of disorders marked by hypotonia at birth and a generally nonprogressive course of muscle weakness. Spinal rigidity is present in a number of patients with CMD. RSS is classified as a CMD, and is characterized by early rigidity of the spine, limb contractures, and restrictive respiratory dysfunction. An approach to the treatment of scoliosis in RSS has not been established. Methods. Details of history, diagnostic tests, and treatment of an adolescent with RSS associated with progressive scoliosis and cervical spine extension contracture is presented. The role of brace treatment, Botox® (Allergan, Inc., Irvine, CA), and details of operative correction, including histology of back muscles, is defined. Results. In this case of RSS with rigid scoliosis nonresponsive to brace therapy and Botox® injections, an anterior thoracolumbar spine fusion with instrumentation was successfully performed for correction of the scoliosis, and the cervical spine was addressed through a posterior approach and an occiput-cervical fusion. We outline the details of surgical procedure and restoration of spinal balance in both sagittal and coronal planes. Conclusions. The chromosomal bases of CMD and characteristic features of RSS, including diagnostic tests, have been reviewed. Surgical intervention with spinal deformity fusion, correction, and instrumentation is indicated in RSS with progressive spinal imbalance not responding to conservative therapy is safe and can give promising results.