Fact-sheet: Madelung disease
Updated on 04/19/2018 at 8:03 AM
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Definition
Premature fusion of the distal half of the growth plate of the distal radial epiphysis: early spontaneous epiphysiodesis resulting in wrist deformity.
Also known as "spontaneous and progressive anterior subluxation of the wrist", congenital (often bilateral) or acquired (more often unilateral)
- Congenital: isolated or associated with other abnormalities (Leri-Weill syndrome, dyschondrosteosis)
- Acquired: post-traumatic (injury to the growth plate during the prepubertal period), infectious, Ollier disease, idiopathic
In idiopathic and congenital forms: several pathophysiological hypotheses:
- abnormal presence of the radiolunate ligament (Vickers ligament), normally absent: a hypertrophied structure thought to cause mechanical stress at its radial insertion near the growth plate
- abnormal migration of the lunate during embryonic development
- ischemic
Clinical features
Decreased range of motion: limitation of supination or even dorsiflexion.
Pronation and flexion generally normal.
Progressive wrist deformity
Detected around 10-12 years of age
Pain
Radiography
- short radius, laterally curved
- triangular distal end of the radius
- distal radioulnar diastasis
- posterior dislocation or subluxation of the distal ulna
- triangular appearance of the carpus: pyramidal arrangement of the proximal row
- triangular metaphyseal-epiphyseal lucent area along the distal ulnar border of the radius: distal radial groove, thought to result from an abnormal, hypertrophied radiolunate ligament
CT
Similar to radiography
MRI
Similar to radiography and CT
+ Visualization (if present) of the abnormal radiolunate ligament
+ Epiphysiodesis
+ Growth plate injury
+ Secondary radiocarpal chondropathy and arthropathy
Nuclear medicine
Not recommended
Management
Clinical and radiologic diagnosis
Splinting +/- surgery (osteotomy and excision of the Vickers ligament)
Differential diagnosis
None