title: Total knee arthroplasty in patients with diastrophic dysplasia.
abstract: 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.
authors: ORTON Orthopaedic Hospital, Invalid Foundation, Helsinki, Finland. ilkka.helenius@helsinki.fi
authors: Ilkka
authors: I
authors: Helenius
authors: Ville
authors: V
authors: Remes
authors: Martina
authors: M
authors: Lohman
authors: Kaj
authors: K
authors: Tallroth
authors: Mikko
authors: M
authors: Poussa
authors: Miia
authors: M
authors: Helenius
authors: Timo
authors: T
authors: Paavilainen
mesh_terms: D019645:Arthroplasty, Replacement, Knee / Q000379:methods*; D001848:Bone Diseases, Developmental / Q000601:surgery*; D006801:Humans; D007719:Knee Joint / Q000601:surgery*; D020127:Recovery of Function; D016896:Treatment Outcome