Arthrogryposis

• Diffuse group of syndromes

1. Arthrogryposis – involves all four extremities
   • Include arthrogryposis multiplex congenita
   • Larsen Syndrome
2. Distal Arthrogryposis
3. Pterygia Syndromes

Arthrogryposis Multiplex Congenita

Features

• Featureless tubular limbs
• Absent skin creases
• Arms adducted & internally rotated
• Elbow ususally extended
• Hips flexed abducted & externally rotated
• Knee usually extended
• Feet – clubfeet
• Stiff firm inelastic block to motion
• 2/3 involve all 4 extremities
• 1/3 lower extremities predominate
• major feeding difficulty may occur secondary to stiff jay
• intelligence normal
• clinical exam is best method for diagnosis

Aetiology

• unknown

Natural History

• unknown
• 1/3 scoliosis
• ¼ non walkers

Treatment

• goals
  • lower extremity = ambulation
  • upper extremity – ROM for self care
• early ROM & casting/splinting
• Hips
  • Unilateral dislocation can be treated
  • Leave bilateral dislocation alone
• Knee
  • Usually kneeis addressed prior to hip & feet
  • Manipulate & cast
  • Surgery to follow
• Foot
  • Usually requiring open procedure, rarely vertical talus
• Upper extremity
  • Consideration to procedures which would get flexion