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[夏科-马里-图思病患者的治疗:骨科方面]

[Treatment for Patients with Charcot-Marie-Tooth Disease: Orthopaedic Aspects].

作者信息

Watanabe Kota

机构信息

Department of Physical Therapy, Sapporo Medical University School of Health Science.

出版信息

Brain Nerve. 2016 Jan;68(1):51-7. doi: 10.11477/mf.1416200345.

DOI:10.11477/mf.1416200345
PMID:26764299
Abstract

The orthopedic manifestations in patients with Charcot-Marie-Tooth disease include deformity and dysfunction of the extremities and spine. Conservative treatment is the first choice. Orthosis and rehabilitation can improve function, and are important for the prevention of joint contractures. Foot problems are most commonly observed and require surgical treatment. Foot deformities include pes cavus, cavovarus, claw toes, or drop foot. Single or combined surgeries selected for soft tissues are plantar release, tendon transfer, or Achilles tendon lengthening, and those for bones are osteotomies and joint fusions. The upper limb initially demonstrates loss of power of the intrinsic hand muscles followed by symmetrical atrophy of the forearm muscle groups. The typical hand deformity is claw hand. Tendon transfer, joint fusion, soft tissue release, or nerve decompression procedures are performed for correction of hand deformities. Acetabular dysplasia in the hip joints is sometimes observed and osteotomy is selected as surgical treatment in such cases. The associated spinal deformity is scoliosis with or without kyphosis. Similar to treatment of idiopathic scoliosis, posterior spinal fusion is performed in patients with progressive spinal deformities.

摘要

夏科-马里-图斯病患者的骨科表现包括四肢和脊柱的畸形与功能障碍。保守治疗是首选。矫形器和康复治疗可改善功能,对预防关节挛缩很重要。足部问题最为常见,需要手术治疗。足部畸形包括高弓足、爪形足、爪状趾或垂足。针对软组织选择的单一或联合手术有足底松解术、肌腱转移术或跟腱延长术,针对骨骼的手术有截骨术和关节融合术。上肢最初表现为手部固有肌肉力量丧失,随后是前臂肌群的对称性萎缩。典型的手部畸形是爪形手。为矫正手部畸形需进行肌腱转移术、关节融合术、软组织松解术或神经减压手术。髋关节有时会出现髋臼发育不良,这种情况下选择截骨术作为手术治疗方法。相关的脊柱畸形是伴有或不伴有脊柱后凸的脊柱侧凸。与特发性脊柱侧凸的治疗类似,对于进行性脊柱畸形患者需行后路脊柱融合术。

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