Madelung's deformity

CONGENITAL SUBLUXATION OF THE
WRIST (MADELUNG’S DEFORMITY)

Pathogenesis (Brailsford):
 Stunted development of inner third of the growth cartilage at the lower end of the radius, due to still unknown cause.
 Growth of the outer two-thirds continues and, as a result, the radial shaft is bowed backwards, the interosseous space is increased, there is overgrowth of lower end of ulna and is subluxated backwards.

Soft tissue changes:• Abnormal tethering of soft tissues from the distal radius to the carpus and ulna.
• These have included aberrant ligaments and pronator quadratus muscle insertions
• Hypertrophy of the palmar ligaments, including the radiotriquetral and the short radiolunate ligaments and an anomalous volar ligament(Vicker’s ligament)

Clinical Features:

 Often bilateral, hence disability may not be identified early and hence late presentation is common
 Often seen for the first time in adolescence.
 Females>males.
 Early cases: mild symptoms of ulnocarpal impaction with power grip activities, and distal radioulnar joint incongruity with forearm rotation
 Flexion may be increased; other movements are restricted and may be painful.
 May be associated with Dyschondrosteosis (Leri Weil syndrome), Turner’s syndrome, Achondroplasia, Ollier’s disease

Vender and Watson Classification:
a) Post traumatic
b) Dysplastic
c) Genetic
d) Idiopathic



X-ray:
 Steep ulnar slope and deficient ulnar margin of radius
 Lunate uncovered.
 The carpus subluxates ulnar and palmarward and appears wedge shaped (lunate lies at the apex of the wedge)
 Increased width between the distal radius and ulna.
 Relatively long ulna compared to radius (positive ulnar variance).
 Decreased carpal angle.
 Triangularization of the distal radial epiphysis.
 Carpus migrates more proximal into the increasing diastasis between the radius and the ulna

Treatment

• In recent or acute cases, dorsiflexion of the wrist-maintained by a full arm plaster for 4 weeks.

Indications for surgery: Acute pain and deformity

Early presentation:
• In early-detected cases distal radial epiphysiolysis is done (Vickers and Nielsen et al.)
• Epiphysiolysis involves resection of the abnormal volar, ulnar physeal region of the radius and fat interposition. At the same time, any aberrant, tethering anatomic structures are excised
• Early presentation with marked deformity and complete lack of a lunate fossa for carpal support, needs combined radial and ulnar osteotomies. Alternatively ulnar and radial epiphysiodesis maybe done

Late presentation:
• Osteotomy of the lower end of the radius may be done. Options include dome osteotomy, dorsal radial closing-wedge osteotomy, or volar opening-wedge radial osteotomy and bone grafting
• Ulnar shortening procedure like the Suave-kapandji maybe useful, though there may already be deterioration of the articular cartilage, wrist ligaments, or triangular fibrocartilage, resulting in continued pain and limitation of motion postoperatively..

Ref:
1. Vickers D, Nielsen G. Madelung's deformity: treatment by osteotomy of the radius and Lauenstein procedure. J Hand Surg [Am] 1987;12(2):202-204
2. Ranawat CS, DeFiore J, Straub LR. Madelung's deformity. An end-result study of surgical treatment. J Bone Joint Surg Am 1975;57(6):772-775