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桡骨纵列发育不全的当前治疗理念

Current concepts in the management Radial Longitudinal Deficiency.

作者信息

Bhat Anil K, Acharya A M

机构信息

Unit of Hand and Microsurgery, Department of Orthopaedics, Kasturba Medical College Hospital, Manipal University, India.

出版信息

J Clin Orthop Trauma. 2020 Jul-Aug;11(4):597-605. doi: 10.1016/j.jcot.2020.05.027. Epub 2020 Jun 25.

Abstract

Radial longitudinal deficiency represents a spectrum of musculoskeletal hypoplasia and dysplasia affecting the upper limb involving mainly the radial aspect of the forearm, wrist, and hand and not infrequently the proximal arm. 2/3rd of the patients with this condition suffer from an associated medical or musculoskeletal disorder and 1/3rd of them show features of a well-known syndrome with systemic manifestations. Hence it is mandatory to do a detailed clinical, radiological and laboratory evaluation which should also include genetic counselling and assessment. Its management has evolved greatly since the beginning of last century. The vast variations developed for the treatment only reflects on the persisting controversy on the ideal treatment which still eludes the medical fraternity. Current treatment options for wrist deformities include radialization or centralization with or without distraction which unfortunately has often shown poor outcomes with high rates of recurrence and poor growth of ulna leading some workers to suggest alternative techniques, which include microsurgical reconstruction using the proximal fibula and the second toe. The management of the associated hypoplastic thumb has been encouraging with recent improvements in classifications and increased options for milder deformities. The article reviews the management options available for this common condition with respect to the recent developments in literature.

摘要

桡骨纵列发育不全表现为一系列影响上肢的肌肉骨骼发育不全和发育异常,主要累及前臂、腕部和手部的桡侧,上臂近端也常受累。2/3患有这种疾病的患者伴有相关的内科或肌肉骨骼疾病,其中1/3表现出具有全身表现的知名综合征的特征。因此,必须进行详细的临床、放射学和实验室评估,这也应包括遗传咨询和评估。自上世纪初以来,其治疗方法有了很大的发展。治疗方法的巨大差异仅反映出对于理想治疗方法仍存在持续的争议,这仍然困扰着医学界。目前治疗腕部畸形的选择包括桡骨化或中心化,伴有或不伴有牵张,但不幸的是,这些方法常常显示出不良结果,复发率高,尺骨生长不良,这使得一些研究人员建议采用替代技术,包括使用近端腓骨和第二趾进行显微外科重建。随着分类方法的最新改进以及轻度畸形治疗选择的增加,相关发育不全拇指的治疗一直令人鼓舞。本文结合文献中的最新进展,综述了针对这种常见疾病的治疗选择。

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Current concepts in the management Radial Longitudinal Deficiency.桡骨纵列发育不全的当前治疗理念
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Surgical techniques for reconstruction of the hypoplastic thumb.发育不全拇指重建的手术技术。
J Hand Surg Eur Vol. 2019 Jan;44(1):15-24. doi: 10.1177/1753193418793579. Epub 2018 Aug 22.
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Outcomes of radialization for radial longitudinal deficiency: 20 limbs with minimum 5-year follow-up.
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Prenatal Detection of Upper Limb Differences With Obstetric Ultrasound.产前超声检查对上肢体差异的检测
J Hand Surg Am. 2015 Jul;40(7):1310-1317.e3. doi: 10.1016/j.jhsa.2015.04.013. Epub 2015 May 28.
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Soft tissue release and bilobed flap for severe radial longitudinal deficiency.
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