Arthrogryposis
Curved joints
Classical Arthrogryposis multiplex congenita
limbs involved , muscles absent or deficient
Arhtrogryposis associated with neuropathic (brain, spinal cord, perpheral nervous system - nerves )
or myopathic (congenital muscular dystrophy )
Arthrogryposis associated with other syndromes or anomalies such as diastrophic dysplasia ,
Aetiology - Unknown
Fetal Akinesia
Hereditary
On examination
Involved limbs - tubular shape, thin subcuatneous tissue absent skin crease
symmetrical , severe distally typically hands and feet
dislocated hip joints , ocasionally knees
trunk - rarely affected , scoliosis
muscle groups absent
Normal IQ
Management
Paediatrician , Orthopaedic surgeon, geneticist , psychologist , physiotherapist , ocupational
therapist
Orthopaedic management
Optimize function , keep children independent ,
Goal - improve function of the limb, rather than a joint,
e.g. elbow extension deformity ;
PT
Tendon transfer
Time
Within the first year -
splint , stretching
correct major defomities (e.g. Hip dislocation - ORIF in first year )
Later , limited to maintain mobility and imrove function ; Tendon transfer
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