Proximal translation of the radius following arthroplasty of the distal radioulnar joint in Hajdu-Cheney syndrome

Abstract

Several surgical procedures for derangement of the distal radioulnar joint have been developed such as the Darrach, Sauve ´-Kapandji (S-K), and Bowers procedures. 1,3 After arthroplasty of the distal radioulnar joint, limitation in the range of motion and wrist pain have been well documented; however, few reports related to problems of the elbow after these surgical procedures have been documented. We report asymptomatic marked proximal translation of the radius at the elbow joint after treatment of dorsal dislocation of the distal radioulnar joint by the S-K procedure in a patient with Hajdu-Cheney syndrome, an idiopathic skeletal dysplasia with osteoporosis characterized by short stature, joint laxity, short clubbed fingers and toes, premature loss of teeth, and defective vision and hearing. 5,9,13 CASE REPORT The patient was a 46-year-old woman, the first-born and the product of a full-term normal delivery. There was no family history of bone disease, and the development of intelligence was normal. In the patient’s late childhood she was diagnosed as having Hajdu-Cheney syndrome on the basis of short stature, osseous dysplasia with osteoporosis, short clubbed fingers and toes, premature loss of teeth, defective hearing, acro-osteolysis, wide cranial sutures, and multiple wormian bones. The patient was right-handed and had experienced mild pain in the right wrist and an inability to extend the little finger 6 months before the patient visited us. Although the wrist pain had not increased, she later experienced an inability to extend the middle and ring fingers. Physical examination revealed dorsal displacement and tenderness of the distal end of the ulna. Full range of motion in extension and flexion of the wrist and elbow and in pronation of the forearm was noted. However, supination of the forearm was limited to 60° because of wrist pain. Active motion of the thumb and index finger was not limited. The patient could fully flex all joints of the middle, ring, and little fingers and extend their distal and proximal interphalangeal joints. However, extension of their metacarpophalangeal joints was restricted to 50° (Figure 1). Passive range of motion of these fingers was fully maintained. Tenodesis effects of the extensor tendons of the fingers were not detected. Grip strength in the right hand was 12 kg, and that in the unaffected left hand was 19 kg. General joint laxity was noted. The anteroposterior radiograph of the hand showed osteolysis of the distal phalanges, positive variance and deformity of the ulna, and dissociation of the distal radioulnar joint (Figure 2). The radiograph of the forearm showed slight bowing deformity of the ulna and incongruity of the humeroradial joint; however, discrepancy with regard to the length of these paired bones was not evident (Figure 3). The patient was treated surgically. Very mild tenosynovitis and rupture of the extensor tendons, extensor digitorum communis (III, IV, and V) and extensor digiti minimi, were observed. The ruptured extensor tendons were reconstructed with a palmaris longus tendon graft.3 Dorsal dislocation of the distal radioulnar joint was managed by the S-K procedure. 1 A bone segment of the ulna was excised in order to create a space for a pseudoarthrosis site, and articular surfaces between the radius and ulna were excised to cancellous bone. The head of the ulna was opposed to the radius and pinned to it with 2 Kirschner wires. The patient started active finger exercises at 3 weeks after surgery. By 6 months after surgery, there was no pain or limitation in the range of motion of the wrist or fingers. Two years after surgery, she had no complaint with regard to the wrist, fingers, and elbow, with full functional recovery. Radiographs of the wrist showed complete arthrodesis of the distal radioulnar joint and a pseudoarthrosis of the ulna (Figure 4). However, radiographs of the elbow showed marked anterior dislocation of the radial head and proximal translation of the radius (Figure 5).