How is arthrogryposis multiplex congenita (AMC) treated?

Updated: Nov 11, 2020
  • Author: Mithilesh Kumar Lal, MD, MBBS, MRCP, FRCPCH, MRCPCH(UK); Chief Editor: Maria Descartes, MD  more...
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Answer

Family and everyone else involved with the patient’s treatment should participate in a general management plan, with this plan involving intensive and aggressive physiotherapy, bracing, and surgical interventions. [37]  There are, however, no guidelines for the care of affected individuals.

Affected babies should be delivered in tertiary care centers.

No completely successful approach to treat arthrogryposis has been found. Goals include lower-limb alignment and establishment of stability for ambulation and upper-limb function for self-care. A literature review by Gagnon et al found that rehabilitation was the most frequently reported form of conservative treatment for arthrogryposis. [38]

Early, gentle manipulation soon after birth improves passive and active range of motion. This is especially true in the case of the inherited distal arthrogryposes, in which prolonged immobilization associated with casting may be undesirable. Late manipulation is of little value.

Early, vigorous physical therapy to stretch contractures is very important in improving joint motion and avoiding muscle atrophy. Patients with amyoplasia or distal arthrogryposis respond well to physical therapy, with excellent functional outcome. However, physical therapy may actually be harmful in patients with conditions of bony fusion such as diastrophic dysplasia, because it may lead to joint ankylosis. Recurrence of deformities following stretching is common, and surgery is often indicated.

Splinting combined with physical therapy appears preferable to continuous casting. Night splinting after surgical procedures is indicated to maintain increased range of motion.

Feeding assistance and intubation is needed in patients with severe trismus.

See Deterrence/Prevention for information on recurrence risk.


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