Arthrogryposis – a congenital disease in which marked stiffness of the joints due to development of contractures and wasting of muscles of strong damage of motoneurons of the spinal cord.
The disease is diagnosed in one of 3 thousand newborns. The reasons are not yet fully understood, but it is clear that it is not inherited. All cases – the result of completely random mutations.
However, there are some predisposing factors that can cause the disease in the fetus.
Noteworthy among them are:
- The viral infection.
- The bacterial disease.
- The use of medicines.
- The influence of toxic substances.
- Limited space in the womb due to some anomalies.
- Disruption of the placenta.
How is
Arthrogryposis in children develops for unknown reasons. While there are two versions, but none of them and not confirmed.
The first is called myogenic. Through it under the influence of certain teratogenic factors disrupts the development of muscle tissue. Because of this start different negative changes in joints, and ligaments, and nervous system.
According to the second theory, neurogenic, the pathological process starts in the primary lesion of the spinal cord, and all other symptoms are secondary.
Classification
Congenital arthrogryposis is possible to classify on various signs. As the prevalence of it can be generalized that most common, with involvement of only the upper extremities, with involvement of only the lower extremities, distal.
Contractures that develop in the joints, can also be different. There are abductor, adductor, flexor, extensor, and some other species.
According to the severity of the disease can be mild, moderate or severe degree.
How does
Under the generalized form of the stiffness of the shoulders, elbows, knees and hip joints observed in all children. The deformation of the hands, feet, face, spine, full hypotension or atony of the muscles.
If the affected only the lower extremities, then there are contractures of the hip joints. Some diagnosed dislocation of the hips. Can be flexor or extensor contractures in the knees, the feet deformation.
If only the affected upper limb, marked contractures in the shoulders, elbows, the area of the wrist and the first finger.
In addition, arthrogryposis, upper and lower extremities can be supplemented by the following symptoms:
- Constant respiratory disease.
- Clubfoot.
- Clubhand.
- Dislocations.
- The appearance of hemangiomas on the body.
- Syndactyly of the fingers.
Moreover, congenital multiple arthrogryposis is diagnosed and the symptoms that are uncharacteristic. For example, there is not observed the symmetry of the deformation of the bones or damage internal organs. The intelligence of child is not reduced, but overall health never suffers. Throughout life and rapid progression of the disease.
Diagnosis
Arthrogryposis in newborn never in doubt in the diagnosis. However, in some cases, confirmation is required to pass some examination.
Examines the medical history of the child, mothers are asked, how was the pregnancy. Then going to the life history of the family and blood tests and urine of the baby as needed.
After that, the pediatrician conducts a thorough examination of the child and, if necessary, consultation and other doctors. If necessary, the doctor may refer the baby to the x-ray of the spine, ultrasonography of the hands and feet, and a CT or MRI.
Arthrogryposis of the lower extremities can be detected at the early stage of fetal development. this is done with ultrasound.
How to get rid of
Treatment of arthrogryposis is only symptomatic. To cure the disease does not always work, especially if both the affected arms and legs.
To restore the shape of the joints you can use plastering, which should be held every week. After a full course the doctor must choose the proper ones.
Electrical stimulation with arthrogryposis included in the list of physiotherapy. Also, the patient can obtain medicinal electrophoresis, applications with paraffin and ozokerite, salt warmers, phonophoresis. Mandatory common courses of massage, exercise therapy.
After you manage to eliminate all visible curvature of the bones, surgery is performed to restore the muscles and tendons.
Treated or not arthrogryposis? If it’s generalized form, the prognosis is always unfavorable here. If local, for example, damaged hands and feet, then it is very likely to return the limb to normal. Disability will also depend on these parameters.
Prevention has not been developed so far, as there is no accurate ideas about what generates disease. The only thing required is the complete elimination of those factors which may affect development of the fetus.