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The knee in arthrogryposis multiplex congenita
1Department of Surgery and Orthopedics, University of Helsinki, Finland.
Journal of Pediatric Orthopedics
|March 1, 1990
Summary
Arthrogryposis multiplex congenita (AMC) knee deformities often require surgery despite initial nonoperative treatment. Flexion contractures are challenging, and extension contractures increase degenerative arthritis risk.
Area of Science:
- Orthopedics
- Pediatric Orthopedics
- Musculoskeletal Disorders
Background:
- Arthrogryposis multiplex congenita (AMC) is a rare neuromuscular disorder characterized by congenital joint contractures.
- Muscular imbalances are common in AMC patients and correlate with specific deformity types.
Purpose of the Study:
- To analyze the long-term functional, radiological, and ambulatory outcomes in patients with AMC knee deformities.
- To evaluate the effectiveness of nonoperative and operative management strategies for AMC knee contractures.
Main Methods:
- Retrospective analysis of 30 patients (58 knees) with AMC.
- Long-term follow-up (1-36 years) assessing functional, radiological, and ambulatory status.
- Correlation of muscular imbalance with deformity type and treatment outcomes.
Main Results:
- Nonoperative treatment was primary, but many patients required surgery for residual symptoms.
- Treatment of flexion contractures yielded less favorable outcomes compared to extension contractures.
- A higher risk of degenerative arthritis was observed, particularly in the extension-contracture group.
Conclusions:
- Long-term management of AMC knee deformities presents significant challenges.
- Flexion contractures are particularly difficult to treat effectively.
- Extension contractures are associated with an increased risk of developing degenerative arthritis later in life.