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Condition/Disorder Synonyms

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  • Arthrogryposis multiplex congenita (AMC)

  • Amyoplasia

  • Classic arthrogryposis

  • Gordon syndrome

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ICD-9-CM Codes

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  • 728.3 Other specific muscle disorders

  • 754.89 Other specified nonteratogenic anomalies

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ICD-10-CM Code

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  • Q74.3 AMC

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Preferred Practice Patterns

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Key Features

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Description

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  • Children are born with limited range of motion (ROM).

  • Limited room in the uterus for development.

  • Term used to describe two or more congenital, nonprogressive joint contractures.2

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Essentials of Diagnosis

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  • Comprises over 300 different disorders

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General Considerations

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  • Types

    • - Myopathic: Fixed flexion contractures of joints, chest, and spine

      • Amyoplasia: Most common and can have dense fibrous tissue

    • - Arthrogryposis ectodermal dysplasia

      • Cote adamopoulos pantelakis

    • - Arthrogryposis multiplex congenita neurogenic (AMCN)

    • - Arthrogryposis multiplex congenita distal (AMCD)

      • Distal joints more often involved than proximal joints

      • Gordon syndrome: Distal arthrogryposis, type 2A

  • Affects joints bilaterally

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Demographics

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  • About 1 in 3000 to 5100 infants.3

  • Genetic: X-linked recessive origin primarily affects males.3

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Clinical Findings

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Signs and Symptoms

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  • Club foot

  • Hip dysplasia

  • Limited ROM of joints including temporomandibular joint

  • Abnormal development of the muscles, joints, and bones

  • Scoliosis

  • Abnormal development of the spinal cord and central nervous system (CNS)

  • Craniofacial malformations

  • Cardiac, respiratory, or organ anomalies

  • Webbing skin around joints

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Functional Implications

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  • Limits functional mobility

  • Developmental delays with movement

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Possible Contributing Causes

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Differential Diagnosis

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Means of Confirmation or Diagnosis

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Laboratory Tests

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  • Creatine phosphokinase (CPK) levels

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Imaging

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Diagnostic Procedures

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  • Muscle biopsy

  • Electromyography

  • Nerve conduction velocity

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Findings and Interpretation

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  • Abnormal chromosomes with genetic testing

  • Excessive muscle fibrosis on muscle biopsy

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Treatment

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Medical Procedures

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  • Osteotomy

  • Tenotomy

  • Serial casting

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Referrals/Admittance

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  • Admission for surgical correction

  • Orthopedist for muscle and joint development issues

  • Neurologist for spinal cord and CNS development

  • Geneticist

  • Orthotist for bracing and splinting

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