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LD26.41 Arthrogryposis multiplex congenita

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

exclusions

• COFS syndrome  (LD2B)
• Arthrogryposis multiplex congenita - lissencephaly  (LD2F.1)

code elsewhere

• Arthrogryposis - renal dysfunction - cholestasis  (5C58.0Y)

postcoordination

synonyms

• Arthrogryposis multiplex congenita
• congenital multiplex arthrogryposis
• amyoplasia congenita
• congenital multiple arthrogryposis
• congenital amyoplasia
• AMC - [arthrogryposis multiplex congenita]
• Neurogenic arthrogryposis multiplex congenita
• X-linked distal arthrogryposis multiplex congenita
• Arthrogryposis multiplex congenita with whistling face
• Crisponi syndrome
• Marden-Walker syndrome
• Van den Ende-Gupta syndrome
• Marden-Walker like syndrome

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