Arthrogryposis multiplex congenita

Description

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.

Excludes

• LD2B – COFS syndrome
• LD2F.1Y – Arthrogryposis multiplex congenita - lissencephaly

Exclusions from above levels

• 8C70 – Arthrogryposis due to muscular dystrophy
• http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism