Management of knee deformity in classical arthrogryposis multiplex congenita (amyoplasia congenita)

We describe the management of significant knee deformity in 44 knees of 22 patients suffering from classical arthrogryposis multiplex congenita (amyoplasia congenita). Follow-up ranged from a minimum of 18 months to 19 years 3 months, with an average 7 years 8 months. Thirteen patients showed fixed flexion of the knees at birth and 9 showed fixed extension. All were treated initially by physiotherapy and splintage, which was successful in all except 1 patient in the extended-knee group, whereas only 7 to 26 knees responded to physiotherapy and splintage alone in the flexed-knee group. Walking ability in the extended-knee group was high; 8 of 9 were community walkers with or without walking aids and orthoses and only one was a therapeutic walker. By contrast, in the flexed-knee group, despite posterior release surgery, which sometimes had to be repeated, only to 6 of 13 patients were community walkers at follow-up, 2 were household walkers, 3 were therapeutic walkers, and 2 had stopped walking in adolescence and preferred to use a wheelchair full time. Long-term splintage is recommended but does not always prevent recurrence of deformity. Bony surgery was used only toward the end of growth or in one case when very severe deformity necessitated its use at an early age and it subsequently had to be repeated. Despite their severe handicap and multiple deformities, this group of children show a remarkable determination to walk with or without walking aids and orthoses.