Tuesday, September 11, 2007

Madelung Deformity

Otto W. Madelung described the wrist deformity bearing his name in 1878 as ”Die spontane subluxation der hand nocte vorne” or “spontaneous forward subluxation of the hand.” Several authors prior to Madelung, including Dupuytren in 1834, Nelaton in 1847, and Malgaigne in 1855, had described entities termed carpus curvus, radius curvus, progressive subluxation of the wrist, manus valgus, manus furca, and idiopathic progressive curvature of the radius. However, Madelung first accurately described it clinically and proposed both an etiology and treatment. photo credit

Madelung deformity (MD) of the wrist is characterized by a growth disturbance in the ulnar (small-finger side) and volar (palm-side) half of the distal (near the wrist) radial physis (growth plate of the radius). As the bone grows, the carpal end of the radius curves in a volar and ulnar direction rather than straight. The distal articular surface of the radius slants in a volar and ulnar direction to a greater degree than normal, the carpus (wrist) and hand shift volarward, and the ulna remains dorsally.

It occurs predominantly in adolescent females. One third of cases of MD are transmitted in an autosomal dominant fashion. The condition has a variable expression and 50% penetrance. MD is bilateral in 50% of cases. There is an associated with mesomelic dwarfism and Turner's Syndrome.

MD has been classified into four different etiologic groups:

  1. Posttraumatic--has been found following repetitive trauma or following a single event that disrupts growth of the distal radial ulnar-volar physis.

  2. Dysplastic--Bone dysplasias associated with MD include multiple hereditary osteochondromatosis, Ollier disease, achondroplasia, multiple epiphysial dysplasias, and the mucopolysaccharidoses (eg, Hurler and Morquio syndromes). The most important dysplasia associated with MD, however, is dyschondrosteosis

  3. Chromosomal or genetic (Turner syndrome)
  4. Idiopathic or primary

Symptoms usually begin during adolescence in girls aged 10-14 years. Patients experience increasing deformity and pain in the wrist with decreased range of motion. On physical examination, the hand is translated volarly to the long axis of the forearm. The ulna, being relatively unaffected, abuts the carpus and becomes prominent dorsally relative to the carpus and hand. Range of motion is decreased, with a limitation of supination, dorsiflexion, and radial deviation. Pronation and flexion usually are normal, but may be decreased.

The diagnosis is confirmed with PA and lateral X-ray views of the forearm and wrist. Several distinct radiographic features of MD exist (described here). Because the deformity is 3-dimensional, a better understanding may be obtained with tomograms or CT scans.

Nonoperative management may be helpful in skeletally mature individuals with MD and mild-to-moderate short-term wrist pain. This management may include anti-inflammatories and appropriate splinting to relieve joint irritation from overactivity.

Operative treatment can be divided into those procedures that

1. Correct the primary deformity of the radius

  • Vickers Physiolysis--In 1992, Vickers and Nielsen described the lesion in the volar and ulnar distal radius as both bony and ligamentous, and they stated that it is an inherent failure of focal growth and structural tethering of further growth. They described an ulnar-volar release for MD of the physis, called physiolysis. This then allows normal and compensatory growth to correct the deformity. The deformity must be noticed early when significant growth remains.

2. Those that attempt to decrease pain and increase range of motion by making a compensatory change in the ulna

  • Osteotomy of radius--If the deformity has progressed in an older child and remaining growth is insufficient, several procedures can be used to correct the position of the distal radiocarpal joint surface. They usually consist of a biplane osteotomy, either closing or opening wedge, which corrects the position of the joint surface and brings the radius and ulna into a more proper position. If a positive ulnar variance remains, an ulnar shortening procedure can be performed.

  • Radioulnar length adjustment--In MD, the ulna grows normally and becomes longer than the radius. Because the radius is volar, the ulna appears to be subluxed dorsally. The incongruence at the distal radioulnar joint and the impingement of the radius on the ulna in supination may cause pain and contribute to decreased range of motion in supination. In order to allow unrestricted rotation, several ulnar procedures have been described. These include 1) ulnar shortening, 2) ulnar head resection and a DRUJ arthrodesis, and 3) ulnar pseudoarthrosis. Some have advocated both radial and ulnar procedures.

  • Ulnar resection--The Darrach procedure long has been a treatment option for MD. This construct in isolation may leave the carpus unstable, especially in light of the increased ulnar and volar slope of the radial articular surface. The carpus therefore tends to slide off of the ulnar side of the wrist. Several authors have devised procedures to solve this problem. The Sauve-Kapandji (Lauenstein) procedure may be a viable option for MD as the ulnar head is preserved and there is less chance for ulnar migration of carpus.

3. Those that address both
  • In 2000, Carter and Ezaki reported a combined procedure using a Vickers ligament release and a dome-shaped osteotomy of the radius to correct all of the aspects of the radial deformity, including the radial and volar translation of the distal metaphysis. It not only corrects the deformity but also decreases pain and increases range of motion.

Postoperative management will depend upon what is done surgically. Six to 8 weeks of cast immobilization is necessary following an osteotomy of the radius or ulna to allow for bony healing. Hand therapy is necessary in children who are not able to regain range of motion on their own after 2-3 months without restrictions. These children should be monitored until surgical issues are resolved and then yearly until skeletal maturity is reached. Following a physiolysis procedure, yearly radiographs can be used to document improvement in the position of the distal radial articular surface with growth.

References

  • Madelung Deformity by Paul M Lamberti, MD--eMedicine Article
  • Carter PR, Ezaki M: Madelung's deformity. Surgical correction through the anterior approach; Hand Clin 2000 Nov; 16(4): 713-21 [Medline]

  • Vickers D, Nielsen G: Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg [Br] 1992 Aug; 17(4): 401-7 [Medline]

  • Madelung's Deformity--Wheeless' Online Textbook of Orthopedics

  • Madelung's Deformity--Children's Hospital Boston

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