A study on joint involvements of the children suffering from arthrogryposis multiplex congenita attended at a tertiary care centre of eastern India

Abstract

Background: Arthrogryposis multiplex congenita (AMC) is a disorder which present at birth and characterized by multiple joint contractures and defective muscles, with normal sensations. This condition was first described by Otto in 1841. In 1923 Stern first used the term AMC in 1932. The objective of this review is to summarize the demography related to arthrogryposis, especially its age of presentation, gender predominance, joints involved, whether both upper limb and lower limb or only lower limb affected.Methods: Total 30 patients in the age group new born to 18 years included in the study. It is a combination of retrospective and prospective observational study. The following parameters are analysed in the study, Age at presentation, gender, type and severity of joints affected.Results: In our study, 8 (26.7%) patients were <12 months old, 13 (43.3%) patients were 12-60 months old, 8 (26.7%) patients were 61-120 months old and 1 (3.3%) patient was >120 months old. 9 (30.0%) patients were female and 21 (70.0%) patients were male. 27 patients have combined upper and lower limb involvement and 3 patients has only lower limb involvement. In our study, 2 (6.7%) patients had progressive upper limb and 28 (93.3%) patients had regressive upper limb.Conclusions: Most common joints which are affected in AMC are shoulder, elbow, wrist, MCP, IP, hip, knee, ankle and the most common position of these joints are internal rotation of shoulder, extended elbow joint, flexed wrist joint, extension of MCP joints, extension of IP joints, flexed hip joint, flexed knee joint and presence of club foot and hip dysplasia (DDH).