A Madelung deformity is a condition affecting the wrist, characterized by a developmental or acquired deficiency in the volar-ulnar (palm-side and pinky-finger side) aspect of the distal radius (the wider end of the forearm bone closest to the wrist). This deficiency can lead to a cosmetic or functional deformity of the wrist.
Here's a more detailed breakdown:
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Definition: Madelung deformity is a skeletal dysplasia, meaning it's an abnormality in bone development, specifically affecting the distal radius.
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What happens? Due to abnormal growth at the distal radial physis (growth plate), the radius bows and shortens, causing the wrist to sublux (partially dislocate) in a palmar and ulnar direction. The ulna (other forearm bone) may also become prominent.
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Causes:
- Genetic Predisposition: Most cases are thought to be inherited, often related to a mutation in the SHOX gene (Short Stature Homeobox-containing gene) located on the sex chromosomes.
- Acquired: Though less common, Madelung deformity can also result from trauma, infection, or tumors affecting the growth plate of the distal radius.
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Symptoms:
- Wrist pain
- Limited range of motion, especially in wrist extension and supination (rotating the palm upward)
- Visible deformity of the wrist, with a dorsal prominence of the ulna
- Weak grip strength
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Diagnosis: Diagnosis typically involves a physical examination, review of medical history, and imaging studies such as X-rays. X-rays will reveal the characteristic bowing and shortening of the radius and subluxation of the wrist.
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Treatment: Treatment depends on the severity of symptoms and the patient's age. Options include:
- Conservative Management: This may include bracing, activity modification, and pain management.
- Surgical Intervention: Surgery may be necessary to correct the deformity and improve wrist function. Surgical options include:
- Resection of the Vickers ligament (a thickened ligament that contributes to the deformity)
- Distal radius osteotomy (cutting and realigning the radius)
- Ulnar shortening osteotomy (shortening the ulna to improve alignment)
- Epiphysiodesis (fusing the growth plate) to prevent further deformity in growing children.
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Prevalence: While not extremely common, Madelung deformity is more frequently seen in females, often manifesting during adolescence.
