How are contractures characterized in arthrogryposis multiplex congenita (AMC)?

Updated: Jan 03, 2019
  • Author: Mithilesh K Lal, MD, MBBS, MRCP, FRCPCH, MRCPCH(UK); Chief Editor: Maria Descartes, MD  more...
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Answer

Points to consider include the following (see the images below):

  • Distal joints are affected more frequently than proximal joints

  • Observe flexion versus extension, limitation of movement (fixed vs passive vs active), and characteristic position at rest; note the severity of all limitations. Distinguish between complete fusion or ankylosis and soft-tissue contracture

  • Range of motion in the jaw is frequently limited

  • Intrinsically derived contractures are frequently associated with polyhydramnios; the contractures are symmetrical and accompanied by taut skin, pterygia across joints, and a lack of flexion creases; recurrence risk and prognosis depend on etiology

  • Extrinsically derived contractures are associated with positional limb anomalies, large ears, loose skin, and normal or exaggerated flexion creases; patients have an excellent prognosis and a low recurrence risk

The hands of a patient with contractural arachnoda The hands of a patient with contractural arachnodactyly (Beals syndrome). Note the long, thin fingers with interphalangeal joint contractures.
A girl with an autosomal recessive type of multipl A girl with an autosomal recessive type of multiple pterygium syndrome. Note the multiple joint contractures at the knees with marked pterygia, including intercrural webbing, affecting her stance and ambulation.

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