What is Arthrogryposis?
Arthrogryposis is a rare condition that is congenital in nature. It derives itself from a Greek term that means ‘joints that are hooked or curved’. Being congenital, this implies that it is a condition that exists at the time of birth. It may develop in the womb or during the first month of birth.
It is formally called Arthrogryposis Multiplex Congenita. This disorder presents itself by way of contractures that affect many or multiple joints. There can also be the presence of muscle weakness and possibly fibrosis. It usually affects both sides of the body with a few differences per extremity.
There are different classes of this disease. Some do not affect more than a few odd joints. Those who exhibit less damage may have more control of movement. They usually affect the wrists, elbows, shoulders, hands, feet, hips and knees. There may be a few cases where all the joints are involved.
Arthrogryposis Symptoms
There are a variety of symptoms that can prelude to this condition. The most common factors are the following:
Difficulty with or inability of the patient to perform:
- Internal shoulder rotations
- Pronation and extension of the elbow
- Wrist movements ( affects the volar and ulnar joints)
- Opening and closing movements with the hand (flexed fingers with the thumb in palm)
- Hip movements ( area is flexed or in an abducted position that may be dislocated)
- Knee movements (flexion is observed)
Various possible complications
- Clubfoot
- Scoliosis
- Respiratory Issues
- Hypoplasia of the Lung
- Retarded Growth
- Face and Jaw Irregularities
- Hernias in the Abdomen
- Hemangiomas in the Face (usually located in the midfacial area)
Presently, there are no special tests that can help diagnose arthrogryposis. To determine the presence of the condition would require a patient to take various tests. This is done to rule out other possible factors. This is usually not related to genetics therefore, it cannot passed on to future offspring. There is a rare form of the disease that is autosomal by nature and can be inherited.
Arthrogryposis Causes
Most physicians and scientists agree that the cause of Arthrogryposis is not known. They have a few theories regarding the condition.
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- Fetal hyperthermia is a plausible cause. This involves an increase in temperature of the fetus. This may be due to a prenatal infection that causes fever in the host or mother.
- Prenatal viruses have also been considered. Many forms of infectious agents are being studied to determine etiological significance with regard to arthrogryposis.
- Vascular compromise of the fetus is a popular theory. The decrease in blood flow may be credited to impingement and other factors. This may cause delayed development to the muscles and tissues of the unborn child.
- Developmental inconsistencies of both muscle and tissue is another probable factor. This may be linked to genetics or due to extrinsic factors. The tendons and the joint linings may be abnormally shaped. This could possibly lead to this condition.
- Decreased levels of amniotic fluid has also been considered as a plausible theory. This would mean a lack of nutrients and hydration for the child.
- Uterine septum is a condition in which the uterus is shaped abnormally. There is usually a longitudinal septum. The outward appearance may appear normal in shape. This is a congenital malformation that affects a few percent of women.
- Malformation of the CNS (Central Nervous System) and the spinal cord is a theory that is linked to genetics and exposure to foreign contaminants.
- Anything that prevents the movement of joints may cause extra tissue to develop around the area. That is a highly probably causative factor. This is common in babies that are born prematurely and require arm or leg straps to prevent them from tinkering with their oxygen tubes.
- Akinesia or a decrease in fetal mobility may also be a cause of AMC.
Arthrogryposis Treatment
This is a condition that cannot be reversed. Patients usually undergo physical therapy to help them cope with the demands of life. Every patient will respond differently to treatment. This is due to the fact that arthrogryposis comes in a variety of forms. This is why therapists have developed many types of exercises to help patients with their specific areas of concern. The therapists who help them deal with their condition are occupational therapists and physicals therapists. For emotional problems, they can seek the help of psychiatrists.
Stretching Exercises
These are performed to help ease out the tension in the muscle group involved. Other procedures like casting and the use of splints on the affected part are used in this process.
Strengthening Exercises
These are done to help the muscle tissue bear greater loads. It is very important in those who have lower extremity arthrogryposis.
Activity of Daily Living (ADL) Exercises
The fine motor skills of the patient are honed to help him or her cope with the real world.
Orthopedic Surgery
This can be performed in those who do not respond well to therapy and those with far complicated malformations. The patient with a hip dislocation or contracture, those with clubfoots or hernias may benefit from this approach. They surgeon will opt for an osteotomy or the Ilizarov external fixator for the procedure. It should be noted that in some cases, not even surgery can fix the problem.
Arthrogryposis Prognosis
Parents of children who show faint symptoms of AMC should seek the advice of a pediatrician or their family physician immediately. Stimulate movement in the area by engaging your child in activities that promotes movement of the specific site.
Although it is a severe condition, patients who have Arthrogryposis Multiplex Congenita can be taught to perform almost like regular individuals. They may be unable to do a few tasks that require complex gestures or movement of the affected area. Despite that, they should be able to live a full and useful existence. With the help of physical therapy and perhaps surgical procedures in major cases, they will be equipped to handle the challenges of the real world with but a few minor complications. They are expected to handle living an independent life that is both rewarding and productive. Since most of them have normal speech and cognitive abilities, they will not have a hard time adjusting to society.
Anthrogryposis Pictures
