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Madelung's Deformity

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Topic updated on 02/12/14 10:04pm
Introduction
  • congenital dyschondrosis of the distal radial physis that leads to
    • partial deficiency of growth of distal radial physis  
    • excessive radial inclination and volar tilt
    • ulnar carpal impaction
  • Epidemiology
    • occurs predominantly in adolescent females
      • common in gymnasts
  • Pathophysiology
    • caused by disruption of the ulnar volar physis of the distal radius
      • repetitive trauma or dysplastic arrest
    • one hypothesis is due to tethering by Vickers ligament
      • Vickers ligament is a fibrous band running from the distal radius to the lunate on the volar surface of the wrist
      • a pathologically thick radiolunotriquetral ligament
  • Genetics
    • X-linked dominant disorder
  • Associated conditions
    • Leri-Weill dyschondrosteosis
      • rare genetic disorder caused by mutation in the SHOX gene  
        • SHOX stands for short-statute homeobox-containing gene
        • anatomically at the tip of the sex chromosome
      • causes mesomelic dwarfism (short stature)
      • associated Madelung's defomity of the forearm
Presentation
  • Symptoms
    • most are asymptomatic until adolescence
    • symptoms include
      • symptoms of ulnar impaction
      • median nerve irritation
  • Physical exam
    • leads to radial and volar displacement of hand 
    • restricted forearm rotation
Imaging
  • Radiographs
    • can see proximal synostosis
    • characteristic undergrowth of the volar, ulnar corder of the radius
    • increased radial inclination
    • increased volar tilt
  • MRI
    • indications
      • concern for pathologic Vickers ligament
    • views
      • thickening ligament from the distal radius to the lunate 
Treatment
  • Nonoperative
    • observation only
      • indications
        •  if asymptomatic
    • physiolysis
      • indications
        • decreased range of motion or tightness at the wrist
      • increase wrist range of motion to physiologically loosen Vickers ligament
    • restricted activity
      • indications
        • athletes with repetitive wrist impaction
      • technique
        • cessation of weight-bearing activities until pain ceases
  • Operative
    • corrective osteomy +/- distal ulnar resections
      • indications
        • pain
        • cosmetic deformity a concern
        • functional limitations
Techniques
  • Corrective osteomy +/- distal ulnar resection 
    • goals
      • restore mechanics of distal radius
    • approach
      • volar approach to the distal radius
    • technique
      • severe deformities may benefit from a staged procedure with initial distration external fixation to avoid neurovascular stretching injury of a single procedure
      • dome osteotomy allows correction of coronal and sagittal deformity
  • Release of Vickers ligament
    • approach
      • volar approach to the distal radius
    • technique
      • release a pathologically thick ligament
      • ligament approx 0.5 to 1.0 cm in diameter

 

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Qbank (2 Questions)

TAG
(OBQ10.59) Which of the following skeletal dysplasias is caused by a sex linked mutation of the short stature homeobox (SHOX) gene? Topic Review Topic

1. Cleidocranial dysplasia
2. Leri-Weil dyschondrosteosis
3. Pseudoachondroplasia
4. Ellis-van Creveld (EVC) syndrome
5. Achondroplasia

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TAG
(OBQ08.90) Madelung's deformity of the distal radius is caused by which of the following? Topic Review Topic

1. Premature fusion of the distal radial ulnar joint
2. Physeal growth mismatch between the distal radius and ulna
3. Nutritional deficiency affecting the physeal zone of provisional calcification
4. Impaired growth of the volar and ulnar aspect of the distal radial physis
5. Unrecognized trauma

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