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Arthrogryposis of the upper extremity
The Orthopedic Clinics of North America
|April 1, 1976
Summary
Arthrogryposis multiplex congenita (AMC) upper extremity deformities can be significantly improved with early, tailored interventions. Nonoperative treatments like serial casting are primary, with surgery reserved for non-responders, offering functional gains.
Area of Science:
- Orthopedics
- Pediatric Surgery
- Rehabilitation Medicine
Background:
- Arthrogryposis multiplex congenita (AMC) presents with congenital contractures affecting upper extremities, impacting patient function.
- Careful patient selection is crucial, considering disease severity and social factors, to determine appropriate treatment pathways.
Observation:
- Nonoperative management, including serial corrective casting initiated at birth, is the initial treatment of choice for AMC upper extremity deformities.
- Serial casting requires frequent adjustments initially, with decreasing frequency as deformities correct.
Findings:
- Surgical intervention becomes necessary for patients unresponsive to nonoperative measures.
- Common surgical procedures include soft tissue releases (e.g., posterior capsulotomy) and triceps lengthening around the elbow, often with tendon transfers to restore active motion.
- Osteotomies are indicated for shoulder deformities, while bone operations are preferred over soft tissue procedures for wrist and hand deformities due to higher success rates.
Implications:
- Despite severe limb involvement, patients with AMC often possess normal sensation and average to above-average intelligence, facilitating rehabilitation.
- Improvements in extremity positioning and reduction of deformities through these interventions can significantly enhance overall patient functioning and quality of life.