CHST3

Management

Congenital joint dislocations, early onset arthritis and spinal deformities are seen in all patients and need appropriate management.
Medical-management for arthritis.
Surgical-surgical correction is often only partially successful and multiple procedures are needed. The outcome of surgery is good without recurrence, and surgically treated patients were reported to have better and more comfortable movement compared with the patient without amelioration.
In some patients knee dislocation may resolve spontaneously few days after birth.
Physical therapy has not been effective.
Agents/circumstances to avoid: Activities with a high impact on joints (e.g., jogging) and obesity.
Echocardiographic evaluation and surveillance of all cases is necessary as untreated cardiac complications can lead to early death.