MANAGEMENT OF KNEE DEFORMITY IN CLASSICAL ARTHROGRYPOSIS MULTIPLEX CONGENITA (AMYOPLASIA-CONGENITA)

We describe the management of significant knee deformity in 41 knees o f 22 patients suffering from classical arthrogryposis multiplex congen ita (amyoplasia congenital. Follow-up ranged from a minimum of 18 mont hs to 19 years 3 months, with an average 7 years 8 months. Thirteen pa tients showed fixed flexion of the knees at birth and 9 showed fixed e xtension. All were treated initially by physiotherapy and splintage, w hich was successful in all except 1 patient in the extended-knee group , whereas only 7 of 26 knees responded to physiotherapy and splintage alone in the Flexed-knee group. Walking ability in the extended-knee g roup was high; 8 of 9 were community walkers with or without walking a ids and orthoses and only one was a therapeutic walker. By contrast, i n the flexed-knee group, despite posterior release surgery, which some times had to be repeated,only 6 of 13 patients were community walkers at follow-up, 2 were household walkers, 3 were therapeutic walkers, an d 2 had stopped walking in adolescence find prefer-red to use a wheelc hair full time. Long-term splintage is recommended but does not always prevent recurrence of deformity. Bony surgery was used only toward th e 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. Despi te their severe handicap and multiple deformities, this group of child ren show a remarkable determination to walk with or without walking ai ds and orthoses.