Upper Limb Reconstruction in Arthrogryposis Multiplex Congenita: A Systematic Review.

Abstract

The goal of this study is to synthesize and critically evaluate nonsurgical and surgical strategies for upper limb reconstruction in arthrogryposis multiplex congenita across the shoulder, elbow, forearm, wrist, and thumb, and to develop an evidence-informed treatment algorithm. A Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA)-aligned systematic review was conducted using MEDLINE/PubMed from database inception to February 28, 2026, with backward and forward citation tracking. Eligible studies included randomized trials, cohort studies, and case series of at least five patients or limbs reporting upper limb-specific outcomes in arthrogryposis multiplex congenita. Extracted variables included phenotype, intervention, range of motion, functional outcomes, patient-reported outcome measures, radiographic findings, complications, reoperations, and follow-up. Risk of bias was assessed using Risk Of Bias In Non-randomized Studies of Interventions (ROBINS-I)-informed domains. Thirty-two studies met inclusion criteria, representing 412 patients who underwent 589 upper limb procedures. Thirteen joint-specific interventional studies with detailed procedure-linked outcomes formed the basis for structured narrative synthesis and treatment algorithm development. Posterior elbow release consistently improved passive elbow flexion and functional hand-to-mouth reach. Muscle transfer procedures, including latissimus dorsi, pectoralis major, Steindler flexorplasty, and selective triceps transfer, restored clinically meaningful active elbow flexion in selected patients with adequate passive motion and donor strength; however, outcomes depended heavily on donor muscle quality and reconstructive selection. Distal humeral external rotation osteotomy improved hand orientation and bimanual grasp, whereas carpal wedge osteotomy reliably corrected rigid wrist flexion deformity by recentering the wrist into a functional arc. Thumb-in-palm deformity was most effectively addressed using first-web reconstruction and metacarpophalangeal stabilization, with emerging support for severity-based staged reconstruction. Upper limb reconstruction in arthrogryposis multiplex congenita improves functional independence primarily by repositioning joints into usable arcs and selectively restoring active motion. Posterior elbow release and individualized elbow flexion reconstruction procedures, humeral rotational osteotomy, wrist realignment osteotomy, and thumb stabilization represent the most consistently supported strategies, although the evidence remains limited by retrospective designs and heterogeneous outcome reporting. Therapeutic IV.