Arthrogryposis Multiplex Congenita

¹ Shriners' Hospital for Crippled Children, Chicago

1. The etiology and pathology of arthrogryposis are not well understood, and there is no general agreement concerning them. Until the true nature of the disorder is known there is little advantage is using other names for the condition.

2. The occurrence of the disease in one member of each of four sets of identical twins, two of which are in this series, seems to exclude hereditary factors as the cause, and to cast doubt on theories of intra-uterine mechanical, circulatory, or toxic causes. The negative family histories further tend to exclude the theory that heredity is a cause.

3. The primary pathological site may be either in the spinal cord or in the muscles. It is believed that the fibrosis around the joints and in the subcutaneous tissues is a part of the pathological process and not merely secondary to intra-uterine inactivity.

4. Mental deficiency is infrequent in patients with arthrogryposis. When present , it does not appear to be more than coincidental. It is not directly related to arthrogryposis.

5. Deformities are difficult to correct and to maintain in correction. Function may be poor because of stiffness or lack of muscle power even when the deformity has been overconme. Stiff joints must be placed in the position of function, and unstable limbs must be braced for weight-bearing. If these things are accomplished, results will be satisfactory.

6. Patients with arthrogryposis, even though severely handicapped, generally make a satisfactory adjustment and live successful lives. Severe pain is not often present despite the poor mechanics many of these people must endure.

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