Case of the Week: June 29-July 6, 2012

Deformity of the forearm with limited mobility

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Case Details

There is a V-shaped carpal row, shortening of the radius with ulnar facing of the articular surface of the radius.

Answer

Diagnosis: Madelung’s Deformity

This is a deformity of the wrist in which there is disturbance of the medial and anterior growth plate of the radius, resulting in shortening of the radius, ulnar and ventral facing of the articular surface of the radius, V-shaped carpal row and wrist deformity.

Some patients develop dorsal subluxation of the ulna.

Usually there is progressive growth of the ulna. 

     Severity is variable, depending upon the degree of deformity, ranging from a perceptibly long ulna to radial deviation of the hand.   

  Etiology is variable, usually genetic.

It occurs most commonly in adolescent females with a mutation of the X chromosome, but can occur in other syndromes such as Turner’s, Leri-Weill dyschondrosteosis, achondroplasia, muchopolysaccharidosis, and multiple epiphyseal dysplasia.

It can occur due to trauma.    

Treatment is quite variable depending upon the degree of severity.

Splinting and reduction in activity is sufficient for some patients.

 Surgery varies form shortening osteotomies to fusion of radius and ulna to the carpus.

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    VCU Department of Pediatric Radiology Virginia Commonwealth University VCU Medical Center