Department of Orthopaedics and Sports Medicine, Harborview Medical Center, University of Washington, Seattle, WA, USA.
J Am Acad Orthop Surg. 2013 Jun;21(6):372-82. doi: 10.5435/JAAOS-21-06-372.
Madelung deformity is a rare congenital anomaly of the wrist caused by asymmetric growth at the distal radial physis secondary to a partial ulnar-sided arrest. The deformity is characterized by ulnar and palmar curvature of the distal radius, positive ulnar variance, and proximal subsidence of the lunate. It more commonly occurs in females than males and typically affects both wrists. The deformity can occur in isolation or as part of a genetic syndrome. The pattern of inheritance varies, with some cases following a pseudoautosomal pattern and many others lacking a clear family history. Nonsurgical management is typically advocated in asymptomatic patients. Few studies exist on the natural history of the condition; however, extensor tendon ruptures have been reported in severe and chronic cases. Stiffness, pain, and patient concerns regarding wrist cosmesis have been cited as indications for surgery. Various techniques for surgical management of Madelung deformity have been described, but clear evidence to support the use of any single approach is lacking.
马德隆畸形是一种罕见的腕关节先天性畸形，由桡骨远端骺板的不对称生长引起，这是由于尺侧部分生长阻滞。畸形的特征是桡骨远端的尺侧和掌侧弯曲、阳性尺侧偏斜和月骨近端下沉。它在女性中比男性更常见，通常影响两个手腕。这种畸形可以单独发生，也可以作为遗传综合征的一部分。遗传模式不同，有些病例遵循假常染色体遗传模式，而许多其他病例则没有明确的家族史。无症状患者通常采用非手术治疗。关于这种情况的自然病史的研究很少；然而，在严重和慢性病例中已经报道了伸肌腱断裂。僵硬、疼痛以及患者对腕部美容的关注已被认为是手术的指征。已经描述了多种手术治疗马德隆畸形的技术，但缺乏支持任何单一方法使用的明确证据。
