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马德隆畸形的诊断与治疗进展

Advances in diagnosis and treatment of Madelung's deformity.

作者信息

Dang Youting, Du Xiaolong, Ou Xuehai, Zheng Qianjin, Xie Fei

机构信息

Department of Pediatric Orthopedics, Honghui Hospital, Xi'an Jiaotong University Xi'an 710054, Shaanxi, The People's Republic of China.

Department of Hand Surgery, Honghui Hospital, Xi'an Jiaotong University Xi'an 710054, Shaanxi, The People's Republic of China.

出版信息

Am J Transl Res. 2023 Jul 15;15(7):4416-4424. eCollection 2023.

Abstract

In this paper, a review of the literature was performed to critically evaluate relevant published research on diagnosis and treatment of Madelung's deformity. Madelung's deformity is a metaphyseal dysplasia of the distal radius, where the distal radial joint tilts to the volar and ulnar sides, combined with distal ulnar subluxation and elbow dislocation. The main pathogenic factors of this disease include idiopathic, hereditary and acquired factors. So far, it is believed that Madelung's deformity is mainly associated with trauma, epiphyseal dysplasia, nutritional disorders, and gene deletion or mutation. It is more common in females, and is an autosomal dominant inheritance disease. Most patients suffer from this disease bilaterally. Madelung's deformity may occur as a complication of Leri-Weill dyschondrosteosis. Most patients usually have no clinical symptoms in the early stage, and some patients come to the hospital due to wrist pain, stiffness, deformity and a shorter forearm. X-ray film is the main diagnostic method for this disease. Magnetic resonance imaging can show local soft tissue and bone abnormalities in the early stage, so it is used for the early diagnosis of this disease. The ulnar angle can be classified into different types based on the size of the distal radius palmar angle. For severe deformity and symptoms, surgical treatment is often required, including soft tissue release, distal radius osteotomy, ulnar shortening osteotomy, distal ulnar resection, and distal radioulnar joint fusion. Some procedures have better clinical results in relieving pain and improving mobility.

摘要

本文对相关文献进行了综述,以批判性地评估有关马德隆畸形诊断和治疗的已发表研究。马德隆畸形是桡骨远端的干骺端发育异常,桡骨远端关节向掌侧和尺侧倾斜,伴有尺骨远端半脱位和肘关节脱位。该疾病的主要致病因素包括特发性、遗传性和后天性因素。迄今为止,人们认为马德隆畸形主要与创伤、骨骺发育异常、营养紊乱以及基因缺失或突变有关。其在女性中更为常见,是一种常染色体显性遗传病。大多数患者双侧患病。马德隆畸形可能作为勒里-韦伊软骨发育不全的并发症出现。大多数患者在早期通常没有临床症状,一些患者因腕部疼痛、僵硬、畸形和前臂缩短而前来就医。X线片是该疾病的主要诊断方法。磁共振成像在早期可显示局部软组织和骨骼异常,因此用于该疾病的早期诊断。根据桡骨远端掌侧角的大小,尺偏角可分为不同类型。对于严重畸形和症状,通常需要手术治疗,包括软组织松解、桡骨远端截骨术、尺骨缩短截骨术、尺骨远端切除术以及桡尺远侧关节融合术。一些手术在缓解疼痛和改善活动度方面具有较好的临床效果。

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