Arthrogryposis Multiplex Congenita (Amyoplasia)


Arthrogryposis multiplex congenita, or amyoplasia, is a distinct form of arthrogryposis characterized by multiple joint contractures in the upper and lower extremities and markedly decreased muscle development. This syndrome, which is present at birth, is not progressive, although deformity of an affected joint may worsen over time. The cause of amyoplasia remains unknown, but the musculoskeletal implications are profound. Initial treatment ideally begins at birth, with physical therapy and splinting to improve the contractures; however, surgical intervention is often necessary. Soft-tissue releases in conjunction with splinting may improve joint position. In patients with more severe contractures, osseous surgery may be necessary. Much additional research is needed to clarify which interventions yield the greatest benefit in each situation. Nevertheless, at present it is encouraging to note that through a combination of multidisciplinary care and the patients' own intelligence and adaptability, many patients with amyoplasia are indeed able to gain an increased level of function and develop a measure of independence into adulthood.

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