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CONDITION/DISORDER SYNONYMS

  • Arthrogryposis multiplex congenital (AMC)

  • Amyoplasia

  • Classic arthrogryposis

ICD-9-CM CODES

  • 728.3 Other specific muscle disorders

  • 754.89 Arthrogryposis multiplex congenita

ICD-10-CM CODE

  • Q74.3 Arthrogryposis multiplex congenita

PREFERRED PRACTICE PATTERNS1

  • 4A: Primary Prevention/Risk Reduction for Skeletal Demineralization

  • 5B: Impaired Neuromotor Development

PATIENT PRESENTATION

A 2-year-old female child is referred to outpatient physical therapy with a diagnosis of arthrogryposis multiplex congenital—myopathtic type. The past medical history includes the following: no familial history of arthrogryposis and an uneventful pregnancy of her mother but the mother reports experiencing decreased fetal movements at about 26 weeks gestation. The infant was born via C-section at 36 weeks gestation with bilateral equinovarus, knee extension contractures, dislocated right hip, bilateral shoulder ankylosis, bilateral elbow flexion, wrist flexion contractures, and ulnar deviation. Surgical history includes: club feet corrected at one month of age, knees and hips corrected at 5 months of age, right elbow corrected into extension. The shoulders and wrists have not been surgically corrected and the left elbow remains in flexion.

Upon examination, the child exhibits delays in gross and fine motor areas, and is dependent for transfers in and out of adaptive stroller. She is unable to stand, has decreased strength and endurance in her lower extremities for functional activities, and decreased active range of motion at hips and knees. She has sufficient passive knees and hips range of motion to allow sit independently in a child sized chair. The child can roll from supine to prone and can assume long sitting independently from supine.

The general therapy goal is to facilitate and promote independent function and mobility.

KEY FEATURES
Description

  • Term used to describe two or more congenital non-progressive, joint contractures2

FIGURE 230-1

Clinical appearance of congenital right clubfoot. (From Skinner HB. Current Diagnosis & Treatment in Orthopedics. 4th ed. www.accessmedicine.com. Copyright © The McGraw-Hill Companies, Inc. All rights reserved.)

Essentials of Diagnosis

  • Comprises over 300 different disorders

General Considerations

  • Types

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

      • Amyoplasia: most common

    • Neuropathic: Fixed contractures

    • Distal

  • Categorization based on presence of disorders

    • mainly limb involvement

    • limb involvement plus other body area(s)

    • limb involvement and central nervous system dysfunction

  • Affects joints bilaterally

  • Distal joints more often involved than proximal joints

Demographics

  • 1 in 3000 to 5100 infants3

  • X-linked recessive origin primarily affects males3

CLINICAL FINDINGS

SIGNS AND SYMPTOMS

  • Limited range of motion of joints including temporomandibular joint

  • Muscle weakness

  • Scoliosis

  • Craniofacial malformations

  • Cardiac, respiratory, or organ anomalies

Functional Implications
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