Arthrogryposis

Question 1

What is arthrogyposis?

Answer 1

• Contractures in 2 or > body areas
• Non-progressive
• It is a clinical finding, not a diagnosis

Question 2

Contributing factors to arthrogryposis

Answer 2

• Fetal hyperthermia - maternal fever > 100 degrees
• Vascular compromise
• Fetal crowding
• Metabolic disturbances
• Neuromuscular, skeletal or connective tissue disturbances

Question 3

Types of arthrogryposis

Answer 3

• Amyoplasia “classic”
• Neuropathic - lethal, ant. horn is often degenerated
• Heterogeneous - neuromuscular, congenital anomolies, etc

Question 4

Characteristics of arthrogryposis

Answer 4

• Severe joint contractures
• Muscle weakness/fibrosis - damaged ant. horn cells, fatty infiltrates
• Decreased/absent muscle development
• Typical cognition
• May have cardiac and respiratory disorder

Question 5

Typical presentations of arthrogryposis

Answer 5

• “Jack-knifed” - Hip flex, knee ext, clubfeet, shoulder IR, elbow flex, wrist flex and ulnar dev.
• Frog legges - hip abd/er, knee flex, club feet, shoulder IR, elbow ext, wrist flex and ulnar dev

Question 6

Typical interventions for arthrogryposis

Answer 6

• Surgery for hip dislocation (reduce if unilateral)
• Wedge osteotomy to alter knee flexion deformity
• Ponsetti surgery for clubfoot
• Soft tissue lengthening
• Muscle transfers

Question 7

Typical interventions for arthrogryposis

Answer 7

• Splinting/stretching
• Strengthening
• Standing program
• Mobility skills - moving toward independence
• Orthoses and adaptive equipment