Reconstruction of forearm deformity by distraction osteogenesis in children with relative shortening of the ulna due to multiple cartilaginous exostosis.

BACKGROUND

In patients with multiple cartilaginous exostosis, distal ulnar osteochondromas frequently cause forearm deformities, with relative ulnar shortening, wrist joint deviation, and varus bowing. Progressive deformation often leads to pain, functional impairment, and cosmetic problems. Surgical ulnar lengthening is necessary to restore the carpal balance. The results of fixator-controlled ulnar lengthening were investigated in this study, using appropriate clinical and radiologic parameters and focusing on medium-term functional and structural outcomes.

METHODS

Twelve children (3 boys, 9 girls; mean age 9.8 y) with multiple cartilaginous exostosis-induced ulnar shortening treated with fixator-controlled ulnar callotasis were evaluated retrospectively based on clinical and radiographic examinations preoperatively, after fixator removal, and at a follow-up investigation. Subjective symptoms and objective joint function were assessed clinically, whereas the extent of ulnar shortening, radial articular angle, carpal slip, and radial head dislocation were determined radiographically.

RESULTS

The average follow-up period was 24.6 months. The mean ulnar shortening and radial articular angle improved significantly, from 14.3 mm or 38.7 degrees preoperatively to 1.7 mm or 25.6 degrees after fixator removal and showed a slight but significant increase to 5.2 mm or 30.1 degrees at the follow-up. Carpal slip and radial head dislocation remained unchanged. With the exception of radial abduction, no notable functional advancement was observed. One unintended ulnar overlengthening with a subsequent ulnocarpal impaction syndrome, one premature callus consolidation, and two fixator dislocations were noted.

CONCLUSIONS

In agreement with literature reports, carpal balance can be restored over the medium term. However, mild recurrences of ulnar shortening and radial malformation were observed during further development. To prevent deformity progression in immature patients, surgery should be carried out early. The optimal timing of surgery needs to be calculated precisely to take advantage of the high remodeling potential and an acceptable degree of recurrent deformity. Ulnar lengthening is necessary, but overcorrection is inadvisable due to possible ulnocarpal impaction syndrome. As significant remodeling effects on the radius were observed, simultaneous radial correction procedures are not recommended a priori.