LOWER LIMB SOFT TISSUE RELEASE IN CHILDREN WITH DUCHENNE DE BOULOGNE DISEASE: 12 YEARS FOLLOW-UP

Abstract

Introduction: The progression of Duchenne de Boulogne disease, coupled with growth leads to contractures consisting of an asymmetric flexion of the pelvis and knees and equinovarus of both feet. Once these can no longer be managed conservatively surgery becomes necessary.

Material & Methods: Thirty four boys who were on average of 11 years old underwent releases of significant hip flexion (av.32°), knee flexion (av.34°) contractures and 20° of foot equinus. These contractures prevented the patients from standing and walking. Per and postoperative epidural anaesthesia was used for the surgical procedures and post-operative splints were not used. On the third post-operative day the patients were transferred to a Paediatric Rehabilitation Centre where progressive skin traction, passive mobilization and postural splinting were used. The patients soon progressed to an upright position. The mean length of time in the specialized Centre was nine and a half weeks.

Results: With a follow-up of 12 years, 15 patients have had a good functional result with a good alignment of both lower limbs. Four children had a residual flexion contracture of the hip of 20°, which was attributed to inadequate care. 10 children gave up their postural management and had a poor result with knee and hip flexion contractures of 60°. 5 patients were lost to follow up.

Conclusion: This treatment was undertaken after the families and their children were fully informed of the procedure. In the group of 15 excellent results, the patients were satisfied as they became free of pain. They had a beneficial upright stance, and a good tolerance of the splints. Their quality of life and their psychology improved. Concerning the second group, the 19 patients had a lack of motivation for such treatment and/or true difficulties to cope with this treatment and their results were correspondingly poor.