Abstract
Introduction: Arthrogryposis congenita multiplex (AMC) is a multifactor-determined limb affection characterized by joint stiffness and motor impairment. Upper and/or lower limbs may be involved with variable severity; upper limb treatment strategy concentrates on joint stiffness first and is limited by the availability of active muscles. In specific cases, early nerve and/or muscle transfers show to be very helpful. Materials and Methods: Since 2013, 6 AMC children have been operated to improve upper limb function, using early nerve transfers to reanimate elbow flexion and/or shoulder abduction. When there was no target muscle present, early muscle transfers were performed. All cases are analyzed retrospectively with the assessment of active and passive range of motion and video motion analysis. Results: Nerve transfers allowed to achieve otherwise unexpectable elbow flexion and shoulder abduction/external rotation. In this 2-year follow-up, the involved and operated upper limb achieved better functional integration. Discussion: This early microsurgical strategy does apply to specific cases with limited joint contracture and seen before 1 year. The treatment allows good muscle targeting and improved function, if muscle tissue is found to be neurotized or moved as a pedicled functional flap. Although etiology of AMC remains unclear and multifactorial, our strategy applied to selected cases highly improved upper limb function and thus should be considered in an (early) treatment algorithm.
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