Hand Surgery Source

MADELUNG'S DEFORMITY

Introduction

Madelung’s deformity was first reported by Malgaigne in 1855, but the disorder is named for Madelung’s because of his detailed description, published in 1878. Classic Madelung’s deformity is believed to be hereditary, but a Madelung-type deformity can be caused by trauma to the distal radius physis, infection, isolated osteochondroma, or multiple hereditary exostosis. This rare condition usually presents between the ages of 6 and 13 years; girls are affected more frequently than boys (3:1). The deformity may go unnoticed for years, but with growth and the onset of puberty, pain, decreased range of motion, and cosmetic issues may manifest.1,2,3

Pathophysiology

  • Madelung’s deformity is caused by abnormal growth arrest of the palmar-ulnar distal radius physis.3
  • An abnormality of the palmar ligament that tethers the lunate to the palmar-ulnar radius epiphysis and metaphysis (Vickers ligament) is contributory.
  • The disease process involves:
    • Abnormal growth of the distal radial epiphysis and in many cases bowing of the radius.
  • Arching of the radius
  • Premature fusion of the ulnopalmar distal radial epiphysis.
  • Delayed development of the ulnar and anterior parts of the distal radial epiphysis.
    • Inclined radial distal joint surface to the anterior and ulnar area
  • Anterior translation of the hand and the wrist
  • Dorsal dislocation of the ulna in its distal posterior part

Related Anatomy

  • Distal radius physis
  • Palmar ligament
  • Vicker’s ligament
  • Lunate
  • Ulna

Incidence and Related Conditions

  • Madelung’s deformity is a rare disorder that frequently occurs bilaterally.
  • The disease is linked with several skeletal disorders, including dyschondrosteosis, Turner syndrome, and Léri-Weill syndrome.
  • Approximately 10% of children with fractures of the distal radius physis may experience growth arrest of the physis and subsequent deformity. Isolated distal radius deformity without radial shaft bowing is more commonly associated with trauma and repetitive trauma such as gymnastics.  Madelung'sdeformity with a bowed forearm is more commonly associated with skeletal disorders like dyschondrosteosis.3
  • The family history is often positive.

Differential Diagnosis

  • Dyschondrosteosis
  • Infection
  • Inflammatory conditions (eg, rheumatoid arthritis)
  • Léri-Weill syndrome
  • Multiple hereditary exostosis
  • Osteochondroma
  • Rickets
  • Trauma
  • Turner syndrome
ICD-10 Codes

MADELUNG'S DEFORMITY

Diagnostic Guide Name

MADELUNG'S DEFORMITY

ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

DIAGNOSIS SINGLE CODE ONLY LEFT RIGHT BILATERAL (If Available)
MADELUNG'S DEFORMITY Q74.0      

ICD-10 Reference

Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Madelung's Deformity
  • Madelung’s deformity of right wrist in 14 year old female
    Madelung’s deformity of right wrist in 14 year old female
  • Madelung’s deformity of right wrist in 14 year old female lateral view
    Madelung’s deformity of right wrist in 14 year old female lateral view
  • Madelung’s deformity of left wrist in 14 year old female
    Madelung’s deformity of left wrist in 14 year old female
  • Madelung’s deformity of left wrist in 14 year old female lateral view in max dorsiflexion
    Madelung’s deformity of left wrist in 14 year old female lateral view in max dorsiflexion
  • Madelung’s deformity of left wrist in 14 year old female lateral view in max palmar flexion
    Madelung’s deformity of left wrist in 14 year old female lateral view in max palmar flexion
  • Madelung’s deformity of left wrist in 11 year old female
    Madelung’s deformity of left wrist in 11 year old female
Symptoms
Deformed wrist that may worsen with growth spurts
Ulnar wrist plan
Large lump on dorsal wrist
Nocturnal paresthesia
Typical History

The patient will usually be a short female between the ages of 6 and 13 years. The patient or parent will note minor disorder of the wrist that was barely noticeable in childhood. In the months or years just before presentation, pain will have begun and increased, along with an abnormal appearance of the wrist. The main presenting complaints are likely to be pain and aesthetic concerns. The patients often have a history of activities involving repetitive loading of the wrist or trauma to the hand and wrist (eg, high-level gymnastics).

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
  • Madelung’s AP X-ray right wrist
    Madelung’s AP X-ray right wrist
  • Madelung’s AP X-ray right wrist
    Madelung’s AP X-ray right wrist
  • Madelung’s AP and lateral X-ray left wrist
    Madelung’s AP and lateral X-ray left wrist
  • 3D r reconstruction from Distal Radius CT: i - epiphysiodesis; 2 - Dorsal displaced distal ulna
    3D r reconstruction from Distal Radius CT: i - epiphysiodesis; 2 - Dorsal displaced distal ulna
Treatment Options
Treatment Goals

Goals:

  • Correct the cosmetic deformity associated with forearm shortening, limited supination, and dorsal distal ulna displacement.
  • Decrease the progressive pain associated with the Madelung's deformity particularly as it  worsens with growth.
Conservative
  • Activity modification
  • NSAIDs
  • Resting splint
Operative
  • Patient age and the possibility of distal ulnar growth are important considerations when planning surgical treatment.
    • Before skeletal maturity: physiolysis combined with release of the abnormal Vickers ligament
    • Post skeletal maturity: osteotomy of the radius alone or in conjunction with an ulnar shortening osteotomy
Treatment Photos and Diagrams
Madelung 's Deformity Osteoplasties
  • Teenager female with bilateral painful Madelung's Deformities. Note X-rays of right wrist and forearm
    Teenager female with bilateral painful Madelung's Deformities. Note X-rays of right wrist and forearm.
  • Teenager female with bilateral Madelung's Deformities. Note X-rays of left wrist and forearm.
    Teenager female with bilateral Madelung's Deformities. Note X-rays of left wrist and forearm.
  • Osteoplasties of the right distal radius and right radial shaft. The radial shaft's dorsal apex angulation (bowing) will be corrected with a closing wedge osteoplasy (1). The Vicker's ligament when present must be released (2 circle). The distal radius deformity will be corrected by a biplantar osteoclasts (3). The osteotomy will be opened ulnarly and volarly.
    Osteoplasties of the right distal radius and right radial shaft. The radial shaft's dorsal apex angulation (bowing) will be corrected with a closing wedge osteoplasy (1). The Vicker's ligament when present must be released (2 circle). The distal radius deformity will be corrected by a biplantar osteoclasts (3). The osteotomy will be opened ulnarly and volarly.
  • Osteoplasty and joint location is outlined by K-wires and verified by fluoroscopy.
    Osteoplasty and joint location is outlined by K-wires and verified by fluoroscopy.
  • Right osteoplasties initially stabilized with K-wires, closing wedge bone used to graft opening distal wedge, and final stabilization done with T-plate and a stacked AO plate to increase plate length proximally.
    Right osteoplasties initially stabilized with K-wires, closing wedge bone used to graft opening distal wedge, and final stabilization done with T-plate and a stacked AO plate to increase plate length proximally.
  • Left osteoplasties initially stabilized with K-wires, closing wedge bone used to graft opening distal wedge, and final stabilization done with a extra long T-plate.
    Left osteoplasties initially stabilized with K-wires, closing wedge bone used to graft opening distal wedge, and final stabilization done with a extra long T-plate.
  • Female teenager 12 years after bilateral radial osteoplasties: 1- active pronation; 2 - active supination; 3 - active dorsiflexion; 4 - active palmar flexion.
    Female teenager 12 years after bilateral radial osteoplasties: 1- active pronation; 2 - active supination; 3 - active dorsiflexion; 4 - active palmar flexion.
  • Madelung’s deformity of left wrist in 11 year old female. Note epiphysiodesis at arrow.Used Mad 2
    Madelung’s deformity of left wrist in 11 year old female. Note epiphysiodesis at arrow.Used Mad 2
  • Madelung’s deformity of left wrist in 11 year old female. Note epiphysiodesis at arrow. Tracing paper used to define wedge size and location.
    Madelung’s deformity of left wrist in 11 year old female. Note epiphysiodesis at arrow. Tracing paper used to define wedge size and location.
  • Madelung’s deformity of left wrist in 11 year old female. Tracing paper used to define wedge size and location.
    Madelung’s deformity of left wrist in 11 year old female. Tracing paper used to define wedge size and location.
  • Madelung’s deformity of left wrist surgical exposure with closing distal wedge and opening radial shaft wedge.
    Madelung’s deformity of left wrist surgical exposure with closing distal wedge and opening radial shaft wedge.
  • Madelung’s deformity of left wrist post osteoplasty and internal fixation.
    Madelung’s deformity of left wrist post osteoplasty and internal fixation.
  • Madelung’s deformity of left wrist post osteoplasty three year follow up comparison views.
    Madelung’s deformity of left wrist post osteoplasty three year follow up comparison views.
  • Madelung’s deformity of left wrist post osteoplasty three year follow up
    Madelung’s deformity of left wrist post osteoplasty three year follow up
CPT Codes for Treatment Options

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CPT Code References

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Complications
  • Compression or irritation of nerves
  • Persistent or recurrent deformity
  • Wrist arthritis later in life
Outcomes
  • Conservative measures are generally unsuccessful.
  • Before skeletal maturity: pain reduced within 6 months post surgery; slight improvement in deformity
  • Post skeletal maturity: improved radiographic parameters, increased range of motion, and decreased pain 
Video
12 year follow-up after bilateral osteotomies for Modeling deformities
Key Educational Points
  • If Madelung’s deformity is untreated, then carpal tunnel syndrome may result.
  • Because most patients are closer to skeletal maturity at presentation, a physiolysis alone is not sufficient.
  • Although the results require confirmation, one study concluded that non-acquired Madelung’s deformity requires molecular screening for SHOX or XO mutation, which definitively diagnoses Léri-Weill dyschondrosteosis or Turner syndrome. 
  • Madelung's disorder's is an autosomal-dominant trait with incomplete penetrance.
References

New Articles

  1. Goldfarb CA, Wall LB, Bohn DC, et al. Epidemiology of congenital upper limb anomalies in a Midwest United Stated population: An assessment using the Oberg, Manske, and Tonkin Classification. J Hand Surg Am 2015;40(1):127-32. PMID: 25534840
  2. Farr S, Bae DS. Inter- and intrarater reliability of ulna variance versus lunate subsidence measurements in Madelung’s deformity. J Hand Surg Am 2014;40(1):62-6. PMID: 25300989

Reviews

  1. Kozin SH, Zlotolow DA. Madelung’s deformity. J Hand Surg Am 2015;40(10):2090-8. PMID: 26341718
  2. Knutsen EJ, Goldfarb CA. Madelung’s deformity. Hand (NY) 2014;9(3):289-91. PMID: 25191158
  3. Zebala LP, Manske PR, Goldfarb CA.  Madelung's deformity: a spectrum of presentation.  J Hand Surg Am; 2007; 32A:1393-1401

Classics

  1. Lambrinudi CC. Two cases of Madelung’s deformity. Proc R Soc Med 1927;20(7):1045-6. PMID: 19985804
  2. Anton JI, Reitz GB, Spiegel MB. Madelung’s deformity. Ann Surg 1938;108(3):411-39. PMID: 17857243
  3. Murphy MS, Linscheid RC, Dobyns JH, Peterson HA.  Radial opening wedge osteotomy in Madelung's deformity.  J Hand Surg Am; 1996; 21A:1035-1044.