Arthrogryposis

Background: Arthrogryposis, or arthrogryposis multiplex congenita, comprises nonprogressive conditions characterized by multiple joint contractures found throughout the body at birth. The term currently is used in connection with a very heterogeneous group of disorders having the common feature of multiple congenital joint contractures.

Pathophysiology: The major cause of arthrogryposis is fetal akinesia (ie, decreased fetal movements) due to fetal abnormalities (eg, neurogenic, muscle, or connective tissue abnormalities; mechanical limitations to movement) or maternal disorders (eg, infection, drugs, trauma, other maternal illnesses). Generalized fetal akinesia also can lead to polyhydramnios, pulmonary hypoplasia, micrognathia, ocular hypertelorism, and short umbilical cord.

During early embryogenesis, joint development is almost always normal. Motion is essential for the normal development of joints and their contiguous structures; lack of fetal movement causes extra connective tissue to develop around the joint. This results in fixation of the joint, limiting movement and further aggravating the joint contracture. Contractures secondary to fetal akinesia are more severe in patients who are diagnosed early in pregnancy and in those who experience akinesia for longer periods of time during gestation.

*Taken from www.emedicine.com