Upper Limb Reconstruction in Arthrogryposis Multiplex Congenita: A Systematic Review

Area of Science:

• Orthopedic Surgery
• Pediatric Orthopedics
• Congenital Limb Deformities

Background:

• Arthrogryposis multiplex congenita (AMC) presents significant upper limb challenges, impacting patient function.
• Effective reconstructive strategies are crucial for improving quality of life in AMC patients.

Purpose of the Study:

• To synthesize and evaluate nonsurgical and surgical upper limb reconstruction strategies for AMC.
• To develop an evidence-informed treatment algorithm for upper limb deformities in AMC.

Main Methods:

• A PRISMA-aligned systematic review of MEDLINE/PubMed was conducted.
• Included studies: randomized trials, cohort studies, and case series (≥5 patients/limbs).
• Risk of bias assessed using ROBINS-I informed domains.

Main Results:

• Thirty-two studies (412 patients, 589 procedures) were analyzed.
• Posterior elbow release improved flexion and reach; muscle transfers restored active elbow flexion.
• Osteotomies corrected wrist and humeral deformities; thumb reconstruction addressed thumb-in-palm deformity.

Conclusions:

• Upper limb reconstruction enhances functional independence in AMC by optimizing joint positioning and restoring motion.
• Supported strategies include elbow release, osteotomies, and thumb stabilization.
• Evidence is limited by study design and outcome heterogeneity.