Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. A group of disorders characterized by congenital limb contractures manifesting as limitation of movement of multiple limb joints at birth that is usually non-progressive and may include muscle weakness and fibrosis. This disorder is always associated with decreased intrauterine fetal movement which leads secondarily to the contractures.
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Amyoplasia congenita is a syndrome characterized by multiple specific congenital joint contractures, associated with substitution of muscular tissue by fibrosis and adipose tissue. There may be a higher prevalence with twin pregnancies 1. The term a-myo-plasia essentially derives from the Latin meaning no-muscle-development. Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys.
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AMC: amyoplasia and distal arthrogryposis
This is a rare disorder occurring in 1 out of every 3, live births. The incidence of true amyoplasia occurs in 1 out of every 10, live births. Arthrogryposis, also called arthrogryposis multiplex congenita AMC , involves a variety of non-progressive conditions that are characterized by multiple joint contractures stiffness and involves muscle weakness found throughout the body at birth. This disorder should be considered a symptom complex or a descriptive term rather than a disease and an underlying cause should be sought when possible. The most common type of arthrogryposis is called amyoplasia in which the hands, wrists, elbows, shoulders, hips, feet and knees are affected.
Amyoplasia congenita is a diagnostic subgroup of children with arthrogryposis multiplex congenita AMC. AMC is a relatively rare syndrome characterized by multiple joint contractures at birth. Amyoplasia congenita is the most common type of this syndrome with an occurrence rate of 1 in 10, live births, and mainly refers to the disorders with limb involvement. In this report, the author presents a premature baby with amyoplasia congenita, whose hips showed flexion, abduction, and external rotation contractures. The knees showed fixed extension contractures, so that his lower extremities were cylindrical with absent skin creases at birth. The syndrome arthrogryposis multiplex congenita AMC is characterized by the presence of multiple contractures in several body areas present at birth. Here the author describes a case of amyoplasia congenita involving only the lower extremities in a premature baby.
Amyoplasia is a condition characterized by a generalized lack in the newborn of muscular development and growth, with contracture and deformity at most joints. It is the most common form of arthrogryposis. It is characterized by the four limbs being involved, and by the replacement of skeletal muscle by dense fibrous and adipose tissue. So amyoplasia may also include an intermediate common pathway, rather than the primary cause of the contractors. Amyoplasia results when a fetus is unable to move sufficiently in the womb. Mothers of children with the disorder often report that their baby was abnormally still during the pregnancy.