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Madelung's deformity: a spectrum of presentation
Lukas P Zebala1, Paul R Manske, Charles A Goldfarb
1Department of Orthopaedic Surgery, Washington University School of Medicine at Barnes Jewish Hospital, St. Louis, MO, USA.
Most Madelung's deformity patients have dyschondrosteosis. This condition can affect the entire radius or just the distal radius, with entire radius involvement leading to more severe outcomes.
Area of Science:
- Orthopedics
- Genetics
- Pediatric Orthopedics
Background:
- Madelung's deformity is a rare congenital condition affecting the wrist.
- Its association with dyschondrosteosis and the extent of radial involvement are not fully understood.
Purpose of the Study:
- To investigate the hypothesis that all Madelung's deformity subjects have dyschondrosteosis.
- To determine if Madelung's deformity affects the entire radius or is limited to the distal aspect.
Main Methods:
- Radiographic and medical record review of 26 subjects with Madelung's deformity.
- Assessment of radius/ulna length, radial bow, deformity severity, and joint space.
- Classification based on whether the deformity involved the entire radius or only the distal radius.
Main Results:
- 18 subjects (31 extremities) had distal radius Madelung's deformity; 8 subjects (15 extremities) had entire radius involvement.
- Both groups showed decreased height and limb length; entire radius group had more severe deformities.
- Dyschondrosteosis was present in all entire radius subjects and most distal radius subjects.
Conclusions:
- Madelung's deformity exists on a spectrum, affecting either the entire or distal radius.
- Entire radius involvement correlates with more severe skeletal abnormalities and shorter limb lengths.
- Dyschondrosteosis is a common comorbidity in patients with Madelung's deformity.
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