Predominantly adolescent females are affected by a ratio of 4:1. The deformity is bilateral most often.
- Initially asymptomatic, progressive clinical deformity
- Pain at wrist
- Loss of wrist extension
- Compromised forearm rotation
- “Bayonet” deformity- prominent distal ulna as a result of dorsal subluxation
- Girls are more often affected than boys
- Presents between the ages of 6 and 13 years
Premature growth plate arrest at the medial volar aspect of the distal radius causes Madelung deformity. Repetitive traumatic pressure may result in Madelung-like deformity. Mutation or absence of the short stature homeobox (SHOX) gene is thought to be the cause of congenital Madelung deformity.
Congenital Madelung deformity can occur as a part of Leri-Weill dyschondrosteosis (LWD) or Turner syndrome.
Key Features from X-Ray
- increased dorsal and radial convexity of the distal radius
- increased volar and ulnar tilt of the distal radial articular surface
- widened interosseous space
- relative dorsal position of the ulnar head
- pyramiding of the carpus
MRI is done on the patients who need the surgical release of Vickers’ ligament to prevent deformity progression.
- Turner syndrome
- Nail-patella syndrome
- Hereditary, multiple exostoses
- Ollier’s disease
- Multiple epiphyseal dysplasias
- Mucopolysaccharidoses (Hurler and Morquio syndrome)
Conservative management of Madelung’s deformity may be helpful for skeletally mature patients with the following measures:
- to reduce pain
- Cold whirlpool immersion
- Transcutaneous electrical nerve stimulation
- limited pronation and supination suggests the need of exercise to help maintaining and/or increasing the power of involved muscles, i.e.- pronators and supinators
- to reduce pain
- oral analgesics
- activity restriction
- volar splint
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