Madelung deformity

Madelung deformity is due to defective development of ulnar third of the epiphysis of the distal radius, which results in a radial shaft that is bowed with an increased interosseous space, and dorsal subluxation of the distal ulna.

It can be bilateral in 50-66% of patients. It often occurs as rare congenital deformity and does not usually manifest until 10-14 years. It may also be seen as an acquired consequence of trauma to the growth plate, e.g. Salter V fracture. The congenital form has an autosomal dominant inheritance with a variable penetrance. 

Epidemiology

The abnormality is more common in females.

Clinical presentation

Most non-traumatic cases of Madelung deformity present with progressive deformity during late childhood or early adolescence ³.  

Pathology

The common mechanism for all causes of Madelung deformity is due to partial closure, or failure of development of the ulnar side of the distal radial growth plate. There is an arrest of epiphyseal growth of the medial and volar (anterior) portions of the distal radius. This leads to shortening of the radius and relative overgrowth of the ulna. The underlying cause of this is unclear, with possibilities including ³:

• vascular insufficiency
• trauma
• infection (osteomyelitis)
• muscular disorders

Associations

• Leri-Weill syndrome: an autosomal dominant dyschondrosteosis (a form of mesometric dwarfism)
• Turner syndrome: affects wrists and hands with osteopenia, metacarpal bones shortening (especially 4th4^th) and decreased carpal angles, which resemble Madelung deformities (usually bilateral)
• Madelung dyschondrosteosis
• gonadal dysgenesis
• nail-patella syndrome ⁵
• diaphyseal aclasis (hereditary multiple exostosis)
• Hurler mucopolysaccharidosis ¹
• achondroplasia ¹
• Ollier disease ¹

Presentation is with deformity, decreased grip strength and often with pain in the wrist relating to ulnocarpal impaction ^1,3.

Radiographic features

The deformity is characterised by:

• dorsal and radial bowing of the radius
• exaggerated palmar (up to 35°)  and ulnar tilt (up to 60°) of the radiocarpal articulation ³
• failure of ossification of the ulnar side of the distal radial epiphysis
• exaggerated radial inclination
• decreased carpal angle below 118°; normal from 118° to 139°.
• carpal subluxation in a palmar and ulnar direction
• lunate is gradually forced to the apex of the V-shaped radioulnaocarpal joint
• “V-shaped” proximal carpal row = herniated proximal carpal row.
• dorsal subluxation of the distal ulna and positive ulnar variance
• wedging of the carpus between the radius and ulna

Treatment and prognosis

Many treatments have been proposed and tried, often with limited success. Conservative measures tend to be ineffective. Surgical options include ³:

• radial epiphysiodesis
• radial corrective osteotomy
• radial physiolysis
• ulnar epiphysiodesis
• excision of the distal ulnar
• ulnar shortening osteotomy

Diggerantial diagnosis

• trauma

  • distal radius and ulna trauma can, espaciily while the physis in open,can cause Madelung deformity when distal radius medial joint fuses with the ulna while the lateral joint space continues to grow.

• Infectioninfection
  • can cause abnormal fusion of the distal radius and ulna growth plates, The Madelung deformity occurs when distal radius medial joint fuses with the ulna while the lateral joint space continues to grow.
• Turner syndrome.

  • in females with a 45/XO.

  • wrists and hands osteopenia, metacarpal bones shortening (espacially 4th) and decreased carpal angles which resemble Madelung deformities,usually bilateral

• Skeletalskeletal dysplasia.

  • Ollier disease, hereditary multiple exostoses, mucopolysaccharidoses...

  • usually bilateral can look like Madelung deformity because of asymmetric growth along the distal radius and ulna growth plates

History and etymology

The Madelung deformity was first described in 1878 by Otto Wilhelm Madelung, German surgeon (1846-1926) ⁴.

Madelung deformity should not be confused with Madelung-Launois-Bensaude syndrome (or Brodie syndrome II) which is characterised by lipomatous accumulation in the subcutaneous regions of the neck and upper thorax and upper limbs ⁶.