Madelung's Deformity

Author: Anay Patel

Topic updated on 03/15/13 8:45pm

Introduction

A 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 dyplasia
2. Leri-Weil dyschondrosteosis
3. Pseudoachondroplasia
4. Ellis-van Creveld (EVC) syndrome
5. Achondroplasia

PREFERRED RESPONSE ▶

DISCUSSION: Leri Weill dyschondrosteosis is a skeletal dysplasia characterized by short stature and bilateral Madelung deformities of the wrist (Illustration A). 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.

Illustrations: A

REFERENCES:

1. Jones KL ed. Smith's Recognizable Patterns of Human Malformation, 6th ed. Philadelphia, PA: Elsevier Saunders; 2006:510-511.

2. Spranger JW, Brill PW, Poznanski AK, eds: Bone Dysplasias: An Atlas of Genetic Disorders of Skeletal Development, 2nd ed. Oxford, England: Oxford University Press; 2002:336-338.

Question Authors:

Michael Hughes MD,

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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

PREFERRED RESPONSE ▶

DISCUSSION: 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.

Illustrations: A

REFERENCES:

1. Waters PM. The Upper Limb. In Morrissy RT, Weinstein SL (EDS): Lovell and Winters Pediatric Orthopaedics ED, 6. Philadelphia, PA, Lipincott Williams & Wilkins, 2006. PP 921-986

2. James MA, Bednar M. Deformities of the Wrist and Forearm. In Green DP, Hotchkiss, RN, Pederson, WC (EDS): Greens Operative Hand Surgery, ED 5. New York, NY. Elsevier Churchill Livingstone, 2005. PP 1469-1505

Question Authors:

Patrick O'Donnell MD/PhD, John Badylak MD,

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Groups

ABOS II: Madelung's Deformity

General - Oral Boards Review

7/29/2011

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Evidence & References Show References

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Jones KL ed. Smith's Recognizable Patterns of Human Malformation, 6th ed. Philadelphia, PA: Elsevier Saunders; 2006:510-511.
Spranger JW, Brill PW, Poznanski AK, eds: Bone Dysplasias: An Atlas of Genetic Disorders of Skeletal Development, 2nd ed. Oxford, England: Oxford University Press; 2002:336-338.
Waters PM. The Upper Limb. In Morrissy RT, Weinstein SL (EDS): Lovell and Winters Pediatric Orthopaedics ED, 6. Philadelphia, PA, Lipincott Williams & Wilkins, 2006. PP 921-986
James MA, Bednar M. Deformities of the Wrist and Forearm. In Green DP, Hotchkiss, RN, Pederson, WC (EDS): Greens Operative Hand Surgery, ED 5. New York, NY. Elsevier Churchill Livingstone, 2005. PP 1469-1505

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Madelung's Deformity

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