Total Knee Arthroplasty in Patients with Diastrophic Dysplasia

Background: Diastrophic dysplasia results in severe disproportionate short stature, generalized joint deformities, and early osteoarthritis. The knee joint often has an abnormal valgus position and is unstable, and degeneration of all joint compartments occurs, even during growth. The purpose of our study was to evaluate the clinical and radiographic results of total knee arthroplasty in a series of patients with diastrophic dysplasia.

Methods: Between February 1992 and March 2000, twenty-one primary total knee arthroplasties were performed on fourteen consecutive patients with severe osteoarthritis due to diastrophic dysplasia. The patients were followed prospectively with clinical examinations, determination of the Hungerford knee scores, and radiographs. Preoperatively, ten knees had chronic dislocation of the patella. The mean duration of follow-up was 3.4 years.

Results: The mean Hungerford knee pain and total scores improved from 5.8 points and 46 points preoperatively to 50 points and 83 points, respectively, at the final follow-up examination (p < 0.001 for both comparisons). Two knees required a distal femoral corrective osteotomy because of metaphyseal angulation. None of the total knee arthroplasties had to be revised during the follow-up period. Six complications were recorded.

Conclusions: Total knee arthroplasty substantially improved the function of patients with diastrophic dysplasia. Additional peripatellar procedures were commonly needed, and complications were frequent.

Level of Evidence: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.