Federal State Budgetary Institution
THE TURNER SCIENTIFIC RESEARCH INSTITUTE FOR CHILDREN’S ORTHOPEDICS
under the Ministry of Health of the Russian Federation
Parkovaya 64-68, Pushkin,
Saint-Petersburg, Russia 196603
Tel.: +7 812 465 2857
www.rosturner.ru




















Arthrogryposis


The head of arthrogryposis clinic
      Olga AGRANOVICH
     MD, PhD, orthopedic and trauma surgeon


     Arthrogryposis, or arthrogryposis multiplex congenita (AMC) is a congenital nonprogressive limitation of movement of two or more joints in different body areas. A contracture is the limitation of movement of a specific joint, in other words, a joint that does not have a full range of movement.
     Arthrogryposis is relatively rare. It has been estimated to occur once in every 3000 live births.
     Arthrogryposis is not a problem in the formation of the joint or limb (the formation of organs and systems of the human body occurs in the first 8 weeks of pregnancy and is called embryogenesis), but rather it is a problem during fetal life (i.e., after 8 to 10 weeks of the pregnancy). The joint is likely to be normal, but lack of movement is associated with the development of extra connective tissue around the joint. This extra connective tissue fixes the joint in place and limits movement even more. Because the affected joint has not moved normally during fetal life, the tendons around the joint may not have stretched to their normal length, and this makes normal joint movement (and physical therapy) after birth even more difficult. Over a period of time if the joint is not used, the surfaces at the end of the bones within the joint begin to assume a different and flattened contour with more acute edges. This may lead to still further difficulty with achieving the full range of movement of that joint. Limitation of movement during a pregnancy there is also limitation of growth of the limb. Amyoplasia is the most common type of arthrogryposis. In the older medical literature, it is called "classical arthrogryposis." The term means, a, no, myo, muscular, and plasia, growth. Amyoplasia has an incidence of 1 in every 10,000 live borns. It represents one third of all cases of liveborns with arthrogryposis. Amyoplasia is characterized by typical symmetric positioning of the limbs with severe equinovarus feet and extended elbows, absent muscle tissue with fibrotic replacement, midfacial haemangioma, and normal intelligence. Some individuals have only legs involved and more rarely only arms involved. Most affected individuals have all four limbs involved but the trunk spared. About 10% of the individuals with amyoplasia have abdominal structural anomalies. Amyoplasia is considered a sporadic disorder. Another form of arthrogryposis is distal arthrogryposis. These forms involve the distal joints and include 9 types (all have autosomal dominant inheritance).
     The aim of treatment for children with arthrogryposis is to help the child gain the maximum function and independence possible.

There are our results of treatment


before operation


   restoration of elbow flexion 1 year after operation
    
    before operation


     4 years after operation


    
    before operation


      restoration of upper limb function 6 months after
      operation

    
    before operation


     3 years after operation



before operation


     4 years after operation

before operation


     2 years after operation


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