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杜氏肌营养不良症早期骨科治疗的临床结果

Clinical results of early orthopaedic management in Duchenne muscular dystrophy.

作者信息

Goertzen M, Baltzer A, Voit T

机构信息

Department of Orthopaedic Surgery, Heinrich-Heine-University, Düsseldorf, Germany.

出版信息

Neuropediatrics. 1995 Oct;26(5):257-9. doi: 10.1055/s-2007-979767.

Abstract

Although the primary defect of Duchenne muscular dystrophy has been found, there is no causal treatment to alter the natural course of this disease. Based on the recommendations by Glorion and Rideau with early treatment of contractures of the hips and the lower limbs we performed a modified release of the spina muscles, resection of tensor fasciae latae muscle and a lengthening of the tendo calcaneus in 32 patients. The mean age of DMD patients at time of operation was 6.1 years. The mean follow-up was 3.4 years. All children underwent mobilisation the day after surgery. Complete correction of all contractures was immediately achieved after operation and kept in all but two cases up to the follow-up examination after 3.4 years. No loss of ambulation was observed. Our results demonstrate that early selective surgery in DMD patients just at or better before the onset of contractures without performing an additional aponeurectomy of the iliotibial band and percutaneous tenotomy of the hamstrings according to the original Glorion-Rideau technique safely prevents severe contractures and thereby delays the progression of scoliosis.

摘要

尽管杜氏肌营养不良症的主要缺陷已被发现,但尚无改变该病自然病程的病因治疗方法。根据Glorion和Rideau关于早期治疗髋部和下肢挛缩的建议,我们对32例患者进行了改良的棘肌松解术、阔筋膜张肌切除术和跟腱延长术。杜氏肌营养不良症患者手术时的平均年龄为6.1岁。平均随访时间为3.4年。所有患儿术后第二天即开始活动。术后所有挛缩均立即得到完全矫正,除两例外,直至3.4年后的随访检查时仍保持矫正状态。未观察到行走能力丧失。我们的结果表明,在杜氏肌营养不良症患者挛缩刚出现时或更好地在挛缩出现之前进行早期选择性手术,而不按照原始的Glorion-Rideau技术对髂胫束进行额外的腱膜切除术和对腘绳肌进行经皮肌腱切断术,可安全地预防严重挛缩,从而延缓脊柱侧弯的进展。

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