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Ambulation in severe arthrogryposis.

M M Hoffer, S Swank, F Eastman

    Journal of Pediatric Orthopedics
    |July 1, 1983
    PubMed
    Summary
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    Functional ambulation was achieved by 22/36 severely affected arthrogrypotic patients. Key requirements included specific hip and knee motion ranges, adequate muscle strength, and supportive devices or orthotics for mobility.

    Area of Science:

    • Orthopedics
    • Rehabilitation Medicine
    • Pediatric Neurology

    Background:

    • Arthrogryposis multiplex congenita (AMC) is a rare congenital disorder characterized by joint contractures affecting multiple body parts.
    • Severely affected individuals often face significant challenges in achieving functional ambulation.
    • Understanding the specific physical requirements for ambulation in AMC is crucial for effective treatment planning.

    Purpose of the Study:

    • To identify the key physical parameters enabling functional ambulation in severely affected arthrogrypotic patients.
    • To establish the relationship between joint range of motion, muscle strength, and assistive devices for successful ambulation in this population.

    Main Methods:

    • Retrospective analysis of 36 severely affected arthrogrypotic patients.

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  • Assessment of functional ambulation status.
  • Measurement of hip and knee range of motion (degrees of extension).
  • Evaluation of hip extensor and quadriceps muscle strength (Medical Research Council scale).
  • Documentation of upper extremity function and use of orthotic substitutes.

    • Main Results:

    • Functional ambulation was achieved in 22 out of 36 patients (61%).
    • Required hip motion: within 30° of full extension; knee motion: within 20° of full extension.
    • Adequate muscle strength (hip extensors grade 4, quadriceps grade 3) or crutchable upper extremities were necessary.
    • Foot and spine deformities presented additional challenges to ambulation.

    Conclusions:

    • Achieving functional ambulation in severe arthrogryposis is feasible with specific joint mobility and muscle strength.
    • Assistive devices and orthotics play a vital role in compensating for functional deficits.
    • Addressing associated deformities is important for optimizing ambulatory outcomes in arthrogrypotic patients.