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[杜氏肌营养不良症中脊柱侧弯的多节段融合]

[Multi-segmental fusion of scoliosis in Duchenne's muscular dystrophy].

作者信息

Hopf C, Forst R, Forst J, Eysel P, Reitter B

机构信息

Orthopädische Klinik der Universität Mainz.

出版信息

Z Orthop Ihre Grenzgeb. 1994 Sep-Oct;132(5):377-82. doi: 10.1055/s-2008-1039840.

DOI:10.1055/s-2008-1039840
PMID:7985396
Abstract

Operations in scoliosis in patients suffering from an advanced stage of Duchenne muscular dystrophy are associated with a higher risk due to the extent of the curves, the respiratory insufficiency and frequent cardiomyopathia. Progressive scolioses in 20 wheelchair patients with an age between 10.5-18.3 years (mean 14.6 years) were treated by CDI. The mean preoperative angle in this group was 70.6 degrees, the postoperative angle 31.2 degrees (mean correction 39.4 degrees or 55.8%). The preoperative lordosis of the lumbar spine (mean angle 4.1 degrees) was corrected to 17.8 degrees postoperatively. The average intraoperative blood loss (2300 ccm) was evident more compared with idiopathic scoliosis. One neurologic complication (postoperative disturbance of bladder function) was observed. Nowadays early surgical correction and stabilization (Cobb angle > 20 degrees) has to be recommended as the treatment of choice for scolioses in Duchenne muscular dystrophy using multisegmental instrumentation methods to enable rapid mobilisation and a postoperative care without brace or cast. This conception allows a prophylactic operation including the following targets: prolongation of life expectancy, improvement of sitting position and prevention of rapid deterioration of lung function including assisted mechanical ventilation in late stages.

摘要

患有晚期杜氏肌营养不良症的脊柱侧弯患者进行手术时,由于侧弯程度、呼吸功能不全和频繁的心肌病,风险较高。对20名年龄在10.5至18.3岁(平均14.6岁)的轮椅患者的进行性脊柱侧弯采用CDI进行治疗。该组术前平均角度为70.6度,术后角度为31.2度(平均矫正39.4度或55.8%)。术前腰椎前凸(平均角度4.1度)术后矫正至17.8度。与特发性脊柱侧弯相比,术中平均失血量(2300立方厘米)明显更多。观察到1例神经并发症(术后膀胱功能障碍)。如今,对于杜氏肌营养不良症的脊柱侧弯,建议早期手术矫正和稳定(Cobb角>20度)作为首选治疗方法,采用多节段器械固定方法,以便能够快速活动,术后无需使用支具或石膏。这一理念允许进行预防性手术,包括以下目标:延长预期寿命、改善坐姿以及预防肺功能迅速恶化,包括在晚期进行辅助机械通气。

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1
[Multi-segmental fusion of scoliosis in Duchenne's muscular dystrophy].[杜氏肌营养不良症中脊柱侧弯的多节段融合]
Z Orthop Ihre Grenzgeb. 1994 Sep-Oct;132(5):377-82. doi: 10.1055/s-2008-1039840.
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Two-year results for scoliosis secondary to Duchenne muscular dystrophy fused to lumbar 5 with segmental pedicle screw instrumentation.杜氏肌营养不良继发脊柱侧弯采用节段性椎弓根螺钉内固定融合至腰5的两年随访结果
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引用本文的文献

1
Surgery for scoliosis in Duchenne muscular dystrophy.杜氏肌营养不良症脊柱侧弯的手术治疗
Cochrane Database Syst Rev. 2015 Oct 1;2015(10):CD005375. doi: 10.1002/14651858.CD005375.pub4.
2
Scoliosis in Duchenne's muscular dystrophy: a changing trend in surgical management : a historical surgical outcome study comparing sublaminar, hybrid and pedicle screw instrumentation systems.杜氏肌营养不良症中的脊柱侧凸:手术治疗方式的变化趋势:对比皮下、杂交和椎弓根螺钉内固定系统的历史手术结果研究。
Eur Spine J. 2010 Mar;19(3):376-83. doi: 10.1007/s00586-009-1163-x. Epub 2009 Sep 17.
3
A Dutch guideline for the treatment of scoliosis in neuromuscular disorders.
荷兰神经肌肉疾病脊柱侧弯治疗指南。
Scoliosis. 2008 Sep 26;3:14. doi: 10.1186/1748-7161-3-14.
4
Surgical treatment of spinal deformities in Duchenne muscular dystrophy: a long term follow-up study.杜氏肌营养不良症脊柱畸形的外科治疗:一项长期随访研究
Eur Spine J. 2004 Aug;13(5):441-8. doi: 10.1007/s00586-002-0515-6. Epub 2004 Apr 24.