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- Multiple congenital dislocations of large joints
- Characteristic flat face
- Ligamentous laxity
- Both autosomal dominant & recessive
Clinical
- Flat face, hypertelorism, broad forehead
- Bilateral dislocated knee, hips, elbows + clubfeet
- Look for defects of cardiac valves & septum
Xray
- Knee- tibia anterior to femur, small patella pouch absent cruciate
- Elbow – any pattern of dislocation + radioulnar synostosis
- Extra ossification center of carpus & foot
- C spine instability
Treatment
- Knee surgery precedes hip or feet
- Start with manipulation & casting & surgery at 3-4 months
- ~ 1year address the hip & feet
- with spine instability – requires consideration & possible stabilization
