Madelung Deformity
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Direct answer
Madelung deformity is a dysplasia of the distal radius in which growth of its volar and ulnar part is restricted, producing volar and ulnar tilt of the wrist and a prominent dorsal ulnar head. The restriction is attributed to a thickened volar ligament, the Vickers ligament, which tethers the carpus to the radius. It is typically seen in adolescent girls, is often bilateral, and is linked with Leri-Weill dyschondrosteosis.
What you must remember
- Deformity: volar and ulnar tilt of the distal radius with a prominent dorsal ulnar head, usually becoming evident during the pubertal growth spurt.
- Vickers ligament: a hypertrophied volar ligament from the ulnar side of the distal radius, which restrains growth of the volar-ulnar radius.
- Sex and age: typically girls in early adolescence, with the deformity often present on both sides.
- Leri-Weill dyschondrosteosis: a pseudoautosomal dominant disorder caused by SHOX gene abnormality, of which Madelung deformity forms part of the spectrum.
- Other associations: Turner syndrome and other SHOX-related states, so height and the family history should be recorded.
- Symptoms: pain, reduced wrist extension and a visible prominence on the ulnar side of the wrist.
- Treatment: observation or splinting for mild cases; Vickers ligament release, corrective osteotomy or ulnar procedures for symptomatic or progressive deformity.
A structured route from wrist prominence to surgery
A 13-year-old girl has a painful, prominent bump on the ulnar side of both wrists, with reduced extension and short stature on examination. The reasoning separates a growth disorder from a simple injury before any surgery is considered.
- Recognise the pattern: look for a prominent dorsal ulnar head and reduced wrist extension, usually in both wrists and with no history of significant trauma.
- Radiograph the wrist: measure the volar tilt and ulnar inclination of the distal radius, and look for a wedge-shaped distal radial physis.
- Look for an underlying syndrome: record height, the family history and features of Turner syndrome, since these suggest SHOX-related disease such as Leri-Weill dyschondrosteosis.
- Choose conservative care first: mild and asymptomatic cases are observed, while splinting and physiotherapy relieve pain and preserve function.
- Consider surgery for progression: a symptomatic or progressive deformity may be treated by Vickers ligament release, with a corrective osteotomy or ulnar shortening in selected cases.
- Monitor the growing wrist: the result is reviewed until skeletal maturity, and the opposite wrist is assessed at the same time.
Viva angles on the Vickers ligament
Examiners usually test whether you can distinguish this dysplasia from trauma and identify its genetic association.
- Trap: calling every ulnar wrist prominence a distal radius fracture. Correct: Madelung deformity is a bilateral, slowly developing growth-related dysplasia with no history of trauma, and the wedge-shaped physis is the pointer.
- Trap: treating the radius as the only abnormal bone. Correct: the dorsal ulnar head is displaced as part of the same process, so the wrist is assessed as one unit.
- Trap: operating on a mild, stable deformity in a child. Correct: most patients are managed conservatively, and surgery is kept for pain, loss of function or progressive deformity.
Frequently asked questions
What is the Vickers ligament in Madelung deformity?
The Vickers ligament is a thickened volar ligament that runs from the ulnar side of the distal radius and tethers the carpus. In Madelung deformity it is believed to restrain growth of the volar-ulnar part of the radius, which leads to the characteristic tilt.
Which syndrome is associated with Madelung deformity?
Leri-Weill dyschondrosteosis is the classic association, caused by abnormality of the SHOX gene in the pseudoautosomal region. Turner syndrome and other SHOX-related disorders can also produce a Madelung-like deformity of the wrist.
Which sex is more commonly affected by Madelung deformity?
Madelung deformity is seen more often in girls, typically during early adolescence. The deformity is frequently bilateral, which is an important clue when a child presents with a painful wrist.
What surgical options are used in Madelung deformity?
Surgery is considered for symptomatic or progressive deformity and may include Vickers ligament release, corrective osteotomy of the distal radius, or ulnar shortening in selected cases. Surgical choice depends on the pattern of deformity and the growth remaining in the child.
Which radiographic findings suggest Madelung deformity?
A wedge-shaped distal radial physis, increased volar tilt and ulnar inclination of the radius, and a prominent dorsal ulnar head are the main radiographic findings. Comparing the opposite wrist helps confirm that the deformity is developmental rather than traumatic.