Shriners Hospitals — Orthopaedic Hospitals


Your child has been diagnosed with Arthrogryposis


This information was provided by the Spokane Shriners Hospital
911 W. 5th Avenue
Spokane, Washington 99210-2472
Telephone (509) 623-0400


What is arthrogryposis?


Arthrogryposis (Arthrogryposis Multiplex Congenita) is a term describing the presence of a muscle disorder that causes multiple joint contractures at birth. A contracture is a limitation in the range of motion of a joint.

In some cases, few joints may be affected and the range of motion may be nearly normal. In the "classic" case of arthrogryposis, hands, wrists, elbows, shoulders, hips, feet and knees are affected. In the most severe cases, nearly every body joint may be involved, including the jaw and back. Frequently, the contractures are accompanied by muscle weakness, which further limits movement. Arthrogryposis is relatively rare, occurring in approximately one in 3,000 births.


Can arthrogryposis occur again in the same family?


In most cases, arthrogryposis is not a genetic condition and does not occur more than once in a family. In about 30% of the cases, a genetic cause can be identified. The risk of recurrence for these cases varies with the type of genetic disorder.


What causes arthrogryposis?


Research has shown that anything that prevents normal joint movement before birth can result in joint contractures. The joint itself may be normal. However, when a joint is not moved for a period of time, extra connective tissue tends to grow around it, fixing it in position. Lack of joint movement also means that tendons connecting to the joint are not stretched to their normal length; short tendons, in turn, make normal joint movement difficult. (This same kind of problem can develop after birth in joints that are immobilized for long periods of time in casts.)

In general, there are four causes for limitation of joint movement before birth:

  1. Muscles do not develop properly (atrophy). In most cases, the specific cause for muscular atrophy cannot be identified. Suspected causes include muscle diseases (for example, congenital muscular dystrophies), maternal fever during pregnancy, and viruses, which may damage cells that transmit nerve impulses to the muscles.
  2. There is not sufficient room in the uterus for normal movement. For example, the mother may lack a normal amount of amniotic fluid, or have an abnormally shaped uterus.
  3. Central nervous system and spinal cord are malformed. In these cases, a wide range of other conditions usually accompanies arthrogryposis.
  4. Tendons, bones, joints or joint linings may develop abnormally. For example, tendons may not be connected to the proper place in a joint.

What is the treatment?


For most types of arthrogryposis, physical therapy has proven very beneficial in improving the range of motion of affected joints. Parents are encouraged to become active participants in a therapy program and to continue therapy at home on a daily basis.

Splints can be made to augment the stretching exercises to increase range of motion. Casting is often used to improve foot position. However, emphasis should be placed on achieving as much joint mobility as possible. Some type of removable splint (perhaps a bi-valve cast) may be used on knees and feet so that the joints can be moved the muscled exercises periodically. In som cases, merely wearing a splint at night may be sufficient.

Surgery may be used to treat the congenital deformities that frequently occur in conjunction with arthrogryposis or should be viewed as a supportive measure once physical therapy has achived maximum results but more range of motion is needed. Surgeries are commonly performed on feet, knees, hips, elbows and wrists to achieve better position or greater range of motion. In some cases, tendon transfers have been done to improve muscle function. Congenital deformities of the feet, hips and spine may require surgical correction at or about one year of age.


What is the outlook?


There is a wide variation in the degree to which muscles and joints are affected in those with arthrogryposis. In some cases, arthrogryposis may be accompanied by other conditions, such as central nervous system disorders, which complicate the picture. However, in most cases, the outlook for those with arthrogryposis is a positive one. Unlike many other conditions, arthrogryposis is non-progressive; it does not worsen with age. Furthermore, with physical therapy and other available treatments, substantial improvement in function is usually possible. Most people with arthrogryposis are of normal intelligence and are able to lead productive, independent lives as adults.

For more information:
AVENUES is a national support group for individuals, families, and medical professionals, and can be reached at:
   P.O. Box 5192
   Sonora, CA 95370
   Phone: (209) 928-3688
   E-Mail: avenues@sonnet.com
   Website: http://www.sonnet.com/avenues


All information provided on this Web site is for informational purposes only and should not be relied upon for medical diagnosis, prognosis or treatment for any specific condition or individual. Always seek the advice of your physician or other healthcare provider with any questions you may have regarding a medical condition. The information found here is not meant to substitute for the advice of a qualified physician. If you have, or think you have, a medical emergency, dial 911 or call your doctor or local emergency services agency immediately.


March 28, 2003




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