Zebala Lukas P, Manske Paul R, Goldfarb Charles A
Department of Orthopaedic Surgery, Washington University School of Medicine at Barnes Jewish Hospital, St. Louis, MO, USA.
J Hand Surg Am. 2007 Nov;32(9):1393-401. doi: 10.1016/j.jhsa.2007.08.012.
To evaluate the hypotheses that all Madelung's deformity subjects have dyschondrosteosis (defined as short stature and mesomelia, in addition to Madelung's deformity) and to evaluate the concept that Madelung's deformity may affect the entire radius.
A radiographic and medical records review was performed for 26 subjects (46 extremities) with Madelung's deformity. The radiographs were assessed for radius and ulna length, sagittal radial bow, severity of the Madelung deformity, and radiocapitellar joint space. The sagittal radial bow and the radiocapitellar joint space were used to classify subjects according to whether the Madelung deformity was limited to the distal radius or involved the entire radius.
Thirty-one extremities in 18 subjects were classified as having a distal radius Madelung deformity and 15 extremities in 8 subjects were classified as having an entire radius Madelung deformity. The radius and ulna length and subject height were significantly decreased compared with age- and height-matched normal values in both groups; the entire radius group was more severely affected. In addition, the entire radius group had more severe deformities with respect to lunate subsidence and ulnar tilt. All of the entire radius subjects and 9 of 14 of the distal radius subjects had dyschondrosteosis.
Madelung's deformity presents as a spectrum. It may affect the entire radius or it may affect only the distal radius. Extremities with involvement of the entire radius have a shorter radius and ulna, decreased height, and a more severe deformity than extremities with involvement of only the distal radius. Additionally, most subjects with Madelung's deformity have dyschondrosteosis.
评估所有马德隆畸形患者均患有软骨发育不全（定义为除马德隆畸形外，身材矮小和中肢短小）这一假说，并评估马德隆畸形可能影响整个桡骨的概念。
对26例（46个肢体）患有马德隆畸形的患者进行了影像学和病历回顾。对X线片评估桡骨和尺骨长度、桡骨矢状弓、马德隆畸形的严重程度以及桡骨头关节间隙。根据马德隆畸形是局限于桡骨远端还是累及整个桡骨，使用桡骨矢状弓和桡骨头关节间隙对患者进行分类。
18例患者的31个肢体被分类为桡骨远端马德隆畸形，8例患者的15个肢体被分类为整个桡骨马德隆畸形。与年龄和身高匹配的正常值相比，两组的桡骨和尺骨长度以及患者身高均显著降低；整个桡骨组受影响更严重。此外，整个桡骨组在月骨下沉和尺骨倾斜方面有更严重的畸形。所有整个桡骨受累的患者以及14例桡骨远端受累患者中的9例患有软骨发育不全。
马德隆畸形表现为一个谱系。它可能影响整个桡骨，也可能仅影响桡骨远端。与仅累及桡骨远端的肢体相比，累及整个桡骨 的肢体桡骨和尺骨更短，身高降低，畸形更严重。此外，大多数马德隆畸形患者患有软骨发育不全。
