Arthrogryposis Multiplex Congenita

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Arthrogryposis Multiplex Congenita
2012-07-20 13:51:27
Clinical Conditions II

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  1. What is AMC?
    a non-progressive, neurogenic or myogenic syndrome. Born with 2 or more, symmetrical, congenital contractures secondary to lack of or decreased fetal movement with an intact skeleton. Contractures are either flexion or extension. The muscle may just be a fibrous band. 
  2. Cause of AMC?
    UNKNOW, theories: abnormal muscle formation, CNS, lower motor neuron, environment (prenatal virus, drugs, maternal hyperthermia, vascular compromise, low amniotic fluid), unsuccessful termintation of pregnancy, genetic (autosomal dominant, X-linked recessive and mitochondrial inheritance)
  3. Incidence of AMC?
    • Depending on reference source AMC affects 
    • 1 in 5,000 to 10,000 live births or 1 in 3,000 to 6,000 live births. 
  4. How is AMC Diagnosed?
    prenatal level II ultrasound in the first trimester, definitive diagnosis made at newborn exam and radiographs.
  5. What are the 3 types of AMC?
    • Amyoplasia
    • heterogeneous
    • Distal arthrogyposis
  6. What is amyoplasia AMC?
    most commonly recognized, lack of muscle formation or development
  7. Heterogeneous AMC?
    Neuromuscular syndromes, congenital abnormalities, chromosomal abnormalities, contracture syndromes, skeletal dysplasia
  8. Distal arthrogyposis AMC?
    Affects hand and feet
  9. Medical manifestations of AMC?
    • Orthopedic: club feet, hip dislocations (unilateral = bilateral), scoliosis
    • cardiac
    • urinary tract malformation
    • failure of testes to descend
    • cleft palate
  10. Clinical features of AMC?
    Fascial asymmetry, thoracolumbar scoliosis (20%), rigid movements, slow responses, minimum rotation, LE jackknife (55%) LE grog (45%), clubfeet, delayed motor development (independent sitting, 15mo ad independent walking at 2-3 years), ROM restrictions
  11. What is the froglike pattern of AMC?
    UE: shoulder IR, elbow extension, wrist flexion, ulna deviation

    LE: hip abduction, ER, knee flexion, Club feet
  12. What is the jackknifed pattern of AMC?
    UE: shoulder IR, elbow flexion, wrist flexion, ulna deviation

    LE: Hips in flexion and dislocated, knee extension, club feet
  13. PT management of AMC?
    Early intervention is ideal, 0-2 years with periodic followup visits with growth. Need to be creative when problem solving. PROM, positioning, orthotics, strengtheining, collaboration with orthopedic surgeon and OT, recommending and ordering DME.
  14. Prognosis and outcomes of AMC?
    Depends on the severity and location of the jt contractures. Independent living depends on the degree and location of jt contractures. Adults are prone to arthritis secondary to overuse. Uderlying health issues may be the cause of premature death.