
Arthrogryposis multiplex congenita - a rare congenital stiff joints syndrome
Author(s) -
Gordana Velisavljev-Filipović
Publication year - 2006
Publication title -
medicinski pregled
Language(s) - English
Resource type - Journals
eISSN - 1820-7383
pISSN - 0025-8105
DOI - 10.2298/mpns0608375v
Subject(s) - medicine , arthrogryposis multiplex congenita , arthrogryposis , surgery
Arthrogryposis multiplex congenita is not a disease but a term describing multiple congenital contractures. Etiological factors include neurological and primary myogenic diseases. This rare syndrome is present at birth and is characterized by reduced mobility of many joints. The contractures involve two or more joints with ankylosis. The accompanying musculature is hypoplastic, but multiple pterygia are also present. Arthrogryposis multiplex congenita is a heterogeneous group of disorders with the incidence rate of 6.2/1 liveborn infants. The true incidence cannot be established, because many cases result in spontaneous miscarriages or stillbirth. More than 90% of cases are associated with birth defects. The cause of this syndrome is unknown. Many forms are not hereditary, though there are hereditary forms as well.