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.
The abnormality is more common in females.
Most non-traumatic cases of Madelung deformity present with progressive deformity during late childhood or early adolescence 3.
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
- infection (osteomyelitis)
- muscular disorders
- Leri-Weill syndrome: an autosomal dominant dyschondrosteosis (a form of mesometric dwarfism)
- Turner syndrome
- 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.
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
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.
- 1. Lovell WW, Winter RB, Morrissy RT et-al. Lovell and Winter's pediatric orthopaedics. Lippincott Williams & Wilkins. (2006) ISBN:0781753589. Read it at Google Books - Find it at Amazon
- 2. Henry A, Thorburn MJ. Madelung's deformity. A clinical and cytogenetic study. J Bone Joint Surg Br. 1967;49 (1): 66-73. J Bone Joint Surg Br (abstract) - Pubmed citation
- 3. Yang JH, Sohn YH, Ko SY et-al. Anthropological analysis of Koreans using HLA class II diversity among East Asians. 2010;doi:10.1111/j.1399-0039.2010.01511.x - Pubmed citation
- 4. O. W. Madelung "Die spontane Subluxation der Hand nach Vorne." Verhandlungen der deutschen Gesellschaft für Chirurgie, Berlin, 1878, 7: 259-276.
- 5. Golding JS, Blackburne JS. Madelung's disease of the wrist and dyschondrosteosis. J Bone Joint Surg Br. 1976;58 (3): 350-2. J Bone Joint Surg Br (abstract) - Pubmed citation
- 6. Murphey MD, Carroll JF, Flemming DJ et-al. From the archives of the AFIP: benign musculoskeletal lipomatous lesions. Radiographics. 24 (5): 1433-66. doi:10.1148/rg.245045120 - Pubmed citation