Abstract
IntroductionArthrogryposis multiplex congenita (AMC) is a rare congenital condition that leads to severe joint contractures and deformities. As painful joint dysplasia and degeneration might develop over time, total joint replacement (TJR) can be a potential treatment option for these patients. The aim of this study is to investigate functional results, implant survivorship and potential complications in patients with AMC who undergo hip or knee arthroplasty.Materials and methodsWe retrospectively identified six TJR in three patients at a single centre performed between 2006 and 2019. The median patient age at surgery was 23 years and the median follow-up period was 69 (IQR 55–99) months. We analysed surgical technique, implant survivorship and complications as well as functional outcome determined by pain reported on the Numerical Rating Scale (NRS), patient-reported outcome scores [Oxford Hip Score (OHS), Harris Hip score (HHS), Oxford Knee Score (OKS)], range of motion and ambulatory status. Depending on data distribution means with ranges and median with interquartile range were compared with the Wilcoxon signed rank test or Student’s t test. The level of significance was defined at < 0.05.ResultsIn hips, the mean range of motion in flexion/extension (52° vs. 85°, p = 0.014) and in rotation (28° vs. 68°, p = 0.02) as well as mean pain score on the NRS (8.5 vs. 0, p = 0.001), OHS (9 vs. 26, p = 0.031) and HHS (17 vs. 52, p = 0.007) significantly improved. In knees, mean range of motion (55° vs. 93°, p = 0.403), mean pain score on the NRS (0 vs. 7) and the OKS (2 vs. 21) also improved. While the ambulatory status did not change, the patients who were wheelchair dependent reported less problems with transfers to a bed or chair and the patient who ambulated reported an improved walking distance. One total knee arthroplasty (TKA) underwent revision for an acute, late infection 155 months following the initial surgery.ConclusionsTJR is a safe procedure in patients with AMC that effectively improves function and reduces pain irrespective of preoperative ambulatory status.
Highlights
Arthrogryposis multiplex congenita (AMC) is a rare congenital condition that leads to severe joint contractures and deformities
Arthrogryposis multiplex congenita (AMC) is a rare congenital symptom complex that describes a collection of aetiologies with several significant joint contractures and consequent deformities [1,2,3]
The mean pain score as determined by the Numerical Rating Scale (NRS) fell to a mean value of 0 postoperatively from a preoperative mean pain score of 8.5 (p = 0.001)
Summary
Arthrogryposis multiplex congenita (AMC) is a rare congenital condition that leads to severe joint contractures and deformities. Implant survivorship and complications as well as functional outcome determined by pain reported on the Numerical Rating Scale (NRS), patient-reported outcome scores [Oxford Hip Score (OHS), Harris Hip score (HHS), Oxford Knee Score (OKS)], range of motion and ambulatory status. Considering that a high percentage of patients with AMC has the potential to ambulate in adulthood and has a close to normal life expectancy [6, 7], surgical intervention to correct deformities or to release contractures should be performed to maintain or regain long-term ability to ambulate and to prevent or relieve pain [2, 7]. Due to the often severe and recurring contractures and deformities [1, 5, 8] some patients may develop degenerative joint changes that cause pain and limited function [9]
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