ICD-11

Any syndrome caused by failure of elastic tissue to correctly develop during the antenatal period. These syndromes are characterised by the presence of multiple joint contractures, where elastic tissues are replaced by inelastic tissues, which results in fixation of the joint.

Arthrogryposis multiplex congenita, comprises nonprogressive congenital conditions characterised by multiple joint contractures. The term is currently used in connection with a very heterogeneous group of disorders that all include multiple congenital joint contractures. The major cause of arthrogryposis is fetal akinesia due to fetal abnormalities (e.g. neurogenic, muscle, or connective tissue abnormalities; mechanical limitations to movement) or maternal disorders (e.g. infection, drugs, trauma, other maternal illnesses). Generalised fetal akinesia can also lead to polyhydramnios, pulmonary hypoplasia, micrognathia, ocular hypertelorism, and short umbilical cord. Lack of fetal movement causes extra connective tissue to develop around the joint, limiting movement and further aggravating the joint contracture.