Novel Approach to Improving Knee Range of Motion in Arthrogryposis with a New Working Classification.

David S Feldman,Troy J Rand,Aaron J Huser

doi:10.3390/children8070546

Abstract

Arthrogryposis multiplex congenita (AMC) is a rare condition defined as contrac-tures in multiple joints. Surgical interventions for severe knee flexion contractures have included posterior release, distraction and extension with external fixation and distal femoral extension osteotomies. These operations have been able to achieve knee extension, but not increase the range of motion. The purpose of this study was to review our experience with peroneal nerve decompression, posterior knee release and proximal femoral shortening. We retrospectively reviewed the medical charts and radiographs of all patients with a diagnosis of arthrogryposis who underwent aforementioned procedure. There were 39 patients with 73 knees included in the analysis with a mean follow-up of 21 months. The mean preoperative arc of motion was 45° and last followup arc of motion was 79° (p < 0.0001). The mean last followup flexion contracture was 8° (p < 0.0001). Additional subanalyses were performed on those with followup greater than 24 months and those with flexion contractures >60°; there were no differences found in these groups. This study demonstrates that it is possible to achieve a functional range of motion of the knees in patients with AMC while improving ambulatory function.

Highlights

Arthrogryposis multiplex congenita (AMC) is a rare condition defined as contractures in multiple joints affecting 1:3000–5000 live births [1,2] Over 400 different conditions have been described that include multiple joints with contracture and that fall under the umbrella of AMC [1]
It has been demonstrated that treatment can redirect the arc of motion in AMC; it has not been shown that treatment can increase the total arc of motion [4,5,6,7,8,9,10,11,12]
The ability to achieve an increased range of motion in patients with AMC and a functional range of motion has not been previously demonstrated in the literature [4,7,12]

Summary

Introduction

Arthrogryposis multiplex congenita (AMC) is a rare condition defined as contractures in multiple joints affecting 1:3000–5000 live births [1,2] Over 400 different conditions have been described that include multiple joints with contracture and that fall under the umbrella of AMC [1]. Hip and knee flexion contractures in arthrogrypotic patients can be the limiting factors in a patient’s ability to ambulate [3]. Treatment of these contractures have included: non-operative therapy, osteotomies of the hips/knees, posterior releases of the knee and gradual soft-tissue lengthening with external fixation [4,5,6,7,8,9,10,11,12]. If a patient has a knee flexion contracture of 90◦ with an arc of motion of 15◦, redirection of this arc so that the patient can stand may take away their ability to sit. The goal of our treatment is to increase the range of motion to be more inclusive of activities rather than to trade standing for sitting

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