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Updated: Apr 30 2023

Madelung's Deformity

Images - lateral - colorado_moved.jpg - colorado_moved.jpg deformity.jpg deformity.jpg deformity.jpg
  • summary
    • Madelung's Deformity is a congenital dyschondrosis of the distal radial physis that leads to partial deficiency of growth of the distal radial physis.
    • Diagnosis is made radiographically with disruption of the ulnar volar physis of the distal radius, excessive radial inclination, excessive volar tilt, and ulnar carpal impaction.  
    • Treatment is observation in patients who are asymptomatic. Operative management is indicated for patients with wrist pain, decreased range of motion, and/or functional limitations.
  • Epidemiology
    • occurs predominantly in adolescent females
      • common in gymnasts
  • Etiology
    • Pathophysiology
      • caused by disruption of the ulnar volar physis of the distal radius
        • repetitive trauma or dysplastic arrest
        • leads 
          • excessive radial inclination and volar tilt
          • ulnar carpal impaction
      • 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 (radio-lunate ligament)
        • may be accompanied by anomalous palmar radiotriquetral ligament
    • Genetics
      • autosomal dominant
    • 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 deformity of the forearm
  • Presentation
    • Symptoms
      • most are asymptomatic until adolescence
      • symptoms include
        • symptoms of ulnar impaction
        • median nerve irritation
    • Physical exam
      • leads to ulnar and volar displacement of hand
      • restricted forearm rotation
  • Imaging
    • Radiographs
      • can see proximal synostosis
      • characteristic undergrowth of the volar, ulnar corner 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
        • indications
          • if asymptomatic
      • restricted activity
        • indications
          • activities with repetitive wrist impaction
          • recommend cessation of weight-bearing activities until pain decreases
    • Operative
      • physiolysis with release of Vickers ligament
        • indications
          • wrist pain or decreased range of motion
          • efficacy of prophylactic release of Vickers ligament in mild deformity in skeletally immature patients unknown
      • radial corrective osteomy+/- distal ulnar shortening osteotomy
        • indications
          • wrist pain or decreased range of motion
          • cosmetic deformity
          • functional limitations
      • DRUJ arthroplasty
        • indications
          • highly controversial
          • painful DRUJ instability and limited supination/pronation
          • significant deformity may require staged procedures
  • Techniques
    • Physiolysis and release of Vickers ligament
      • approach
        • volar approach to the distal radius
      • technique
        • release a pathologically thick ligament
        • ligament approximately 0.5 to 1.0 cm in diameter
        • bar resection and fat grafting in the physis
    • Corrective radial osteotomy +/- distal ulnar shortening osteotomy
      • goals
        • restore mechanics of distal radius
      • approach
        • volar approach to the distal radius
      • technique
        • severe deformities may benefit from a staged procedure with initial distraction external fixation to avoid neurovascular stretching injury of a single procedure
        • codome osteotomy allows correction of coronal and sagittal deformity
  • Complications
    • Incomplete physiolysis or premature growth arrest
    • Violation of radiocarpal or ulnocarpal joint
    • Incomplete deformity correction
    • Recurrent deformity
    • Nonunion of the osteotomy site
    • Continued ulnar impaction (if radial osteotomy done alone)
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