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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 (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) 
 

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Questions (2)

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

QID:3147
1

Cleidocranial dysplasia

13%

(186/1397)

2

Leri-Weil dyschondrosteosis

45%

(635/1397)

3

Pseudoachondroplasia

14%

(190/1397)

4

Ellis-van Creveld (EVC) syndrome

18%

(254/1397)

5

Achondroplasia

9%

(120/1397)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

Leri Weill dyschondrosteosis is a skeletal dysplasia characterized by short stature and bilateral Madelung deformities of the wrist. The SHOX gene is located on the X and Y chromosomes and a mutation on either of the sex chromosomes leads to the dysplasia (sex linked dominant).

Madelung deformity is a result of disruption of the volar ulnar physis of the distal radius (leading to radial inclination and a radiopalmar tilt). Symptoms from the wrist deformity include ulnocarpal impaction, restricted forearm rotation, and median nerve compression.

Cleidocranial dyplasia is an autosomal dominant defect in CBFA-1, a transcription factor that activates osteoblast differentiation. Pseudoachondroplasia is an autosomal dominant mutation in COMP on chromosome 19. Ellis-van Creveld (EVC) syndrome is an autosomal recessive mutation in the EVC gene causing acromesomelic shortening and postaxial polydactyly among other anomalies. Achondroplasia is an autosomal dominant mutation of FGFR-3.

Illustration A demonstrates a Madelung deformity of the wrist with characteristic increased radial inclination and increased volar tilt.

ILLUSTRATIONS:

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Question COMMENTS (2)

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

QID:476
1

Premature fusion of the distal radial ulnar joint

3%

(52/1678)

2

Physeal growth mismatch between the distal radius and ulna

16%

(264/1678)

3

Nutritional deficiency affecting the physeal zone of provisional calcification

1%

(18/1678)

4

Impaired growth of the volar and ulnar aspect of the distal radial physis

78%

(1311/1678)

5

Unrecognized trauma

2%

(26/1678)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

Madelung's deformity is that of excessive ulnar/palmar angulation of the distal radius caused by impaired growth of the volar and ulnar aspect of the distal radial physis. It may be caused by either a bony lesion in the palmar/ulnar corner of the distal radial physis or an abnormal radial-carpal ligament (Vicker's ligament). The other answers do not cause Madelung's deformity. Leri-Weill dyschondrosteosis is a rare genetic disorder caused by mutation in the SHOX gene that causes mesomelic dwarfism with associated Madelung's defomity of the forearm.

Illustration A is a radiographic example of Madelung's deformity.

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