ISOLATED MADELUNG’S DEFORMITY. LONG- TERM FOLLOW-UP STUDY OF FIVE PATIENTS TREATED SURGICALLY.

Abstract

Five patients with isolated Madelung’s deformity were reviewed with an average follow-up of 34 years after surgery. All the patients were female and their average age at surgery was 12.7 years, whereas average age at follow-up was 53 years. The deformity was bilateral in 4 patients and unilateral in 1.

At diagnosis deformity, pain and limitations of the range of motion were present in all the wrists except 2, which were painless but presented marked functional impairment. In all the patients the typical radial deviation of the hand, was observed, with dorsal prominence of the distal end of the ulna. The x-rays showed, in anteroposterior view, the V-shaped arrangement of the first carpal row, with the lunate at the apex of the V and the marked obliquity of the articular surface of the radius toward the ulnar side. In the lateral view, the articular surface of the radius was markedly angulated anteriorly, the ulna was subluxated posteriorly and carpal bones were translated anteriorly. In no patient did we observe growth disturbance of the other bones or deformities typical of osteochondrodysplasias. In some cases the deformity progressed rapidly, whereas in the others the progression was slow. Surgical correction was sought by both the family and the patients mainly for functional reasons, although cosmetic improvement was also expected.

The operation consisted of closing-wedge osteotomy of the distal radial metaphysis and either shortening osteotomy or resection of the distal ulna. At the operation all the patients had passed the adolescence growth spurt, although in 5 out of the 9 wrists growth plates were still open.

At follow- up, all the patients were satisfied with the results of the operations and the range of motion of the wrists was improved. Some residual radiographic abnormalities were present in 4 wrists, but all patients were mostly pleased with the absence of pain and improvement of wrist cosmesis. No radiographic osteoarthritis was present in any of the operated wrists, although 4 of the 5 patients were over 55 years of age.

Conclusions: An association of distal radial wedge-closing osteotomy and either distal ulnar hand resection or shortening distal ulnar osteotomy to correct Madelung’s deformity gave very good results in line with other reports. The limited number of our cases may be compensated by the very lengthy long-term follow-up of our series that showed how the results of these corrective operations do not deteriorate in time; moreover no radiographic osteoarthritis was present even almost 50 years after the operation.