questions
2

Madelung's Deformity

Author:
Topic updated on 03/05/16 11:09am
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 (short 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 radial 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) 

 

Please Rate Educational Value!
4.0
Average 4.0 of 23 Ratings

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

PREFERRED RESPONSE ▶
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

PREFERRED RESPONSE ▶



Cases

http://upload.orthobullets.com/cases/2514/ebb1e6b3-4cc3-4a0b-ab21-bc973d37e7da_wp_20160225_001.jpg http://upload.orthobullets.com/cases/2514/13cf619e-2725-4ac4-ad5c-2f8a57ead9fd_wp_20160225_002.jpg
HPI - History of injury to left wrist 06 months back while playing handball. Had mild...
poll
2/25/2016
174 responses
See More Cases

Groups


Evidence



Topic Comments