Elsevier

The Journal of Hand Surgery

Volume 32, Issue 9, November 2007, Pages 1393-1401
The Journal of Hand Surgery

Congenital
Madelung’s Deformity: A Spectrum of Presentation

https://doi.org/10.1016/j.jhsa.2007.08.012Get rights and content

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

Type of study/level of evidence

Diagnostic III.

Section snippets

Materials and Methods

We performed a comprehensive medical record search for the years 1960 to 2006 for subjects with a diagnosis of Madelung’s deformity. Institutional review board approval was obtained for this retrospective review. Inclusion criteria were a diagnosis of Madelung’s deformity based on the classic description of distal radius and ulna abnormalities7, 8, 9, 10, 11 and adequate preoperative radiographs of the wrist, forearm, and elbow. Subjects with idiopathic and familial Madelung’s deformity were

Distal Radius Versus Entire Radius Madelung’s Deformity

We classified 31 extremities in 18 subjects as having a distal radius Madelung’s deformity (Fig. 1) and 15 extremities in 8 subjects as having an entire radius deformity (Fig. 2). The 6 subjects with unilateral Madelung’s deformity included 5 subjects with a distal radius deformity and one subject with an entire radius deformity. There were no instances of bilateral involvement in which one extremity was classified as entire radius and one as distal radius.

Radial Bow

The radial bow on the AP radiograph

Discussion

Previous descriptions of Madelung’s deformity have concentrated on the bony abnormality at the distal radius and wrist. In their classic description of the 12 features of Madelung’s deformity, Dannenberg et al7 focused on the wrist but did mention that the entire diaphysis of the radius may be involved. The deformity at the radiocapitellar joint and the increased sagittal bow of the radius were not included among the 12 features. Subsequent reports on Madelung’s deformity17, 18, 19, 20 also

References (23)

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    Surg Gynecol Obstet

    (1909)
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