Abstract
Arthrogryposis multiplex congenita (AMC) presents complex therapeutic challenges in upper limb reconstruction, in which establishing an optimal positioning-reaching-grasping axis is critical for patient independence. However, clinical evidence is often limited by low-level data and significant phenotypic heterogeneity, leaving management strategies fragmented. This review synthesizes current evidence to provide a strategic framework for managing complex upper extremity deformities in AMC. A selective literature search was conducted via the MEDLINE database in April 2026, focusing on advancements published between 2020 and 2025. Key keywords included "arthrogryposis", "amyoplasia", and "upper limb deformities", analyzing the multi-joint therapeutic hierarchy, nonoperative timelines, and surgical sequencing. The evidence identifies a shift toward a functional kinetic chain approach, prioritizing the restoration of self-care autonomy over simple anatomical realignment. Effective management begins with early, nonoperative mobilization to leverage neonatal tissue plasticity. Surgical strategy is often conceptualized as a hierarchical framework, typically addressing passive constraints through joint release or osteotomy to optimize the limb's spatial positioning before considering active motion reconstruction via functional tendon or muscle transfers. The therapeutic logic emphasizes the critical hand-to-mouth and personal hygiene pathways, in which the timing of intervention is tailored to the child's developmental milestones and compensatory patterns, ensuring that each segmental correction supports the overall synergy of the limb. Early, persistent nonoperative intervention serves as the foundational pillar, while surgical strategies must be tailored to the child's adaptive patterns. Future care should move toward precision functional restoration, supported by genomic insights, and standardized multicenter outcome measures.