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