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The Journal of Bone and Joint Surgery (American) 85:2097-2102 (2003)
© 2003 The Journal of Bone and Joint Surgery, Inc.

Total Knee Arthroplasty in Patients with Diastrophic Dysplasia

Ilkka Helenius, MD, PHD1, Ville Remes, MD, PHD2, Martina Lohman, MD, PHD2, Kaj Tallroth, MD, PHD, FICA2, Mikko Poussa, MD, PHD2, Miia Helenius, DDS, MB2 and Timo Paavilainen, MD, PHD2

1 Arhipanpolku 8 b A, Helsinki FIN-00420, Finland. E-mail address: ilkka.helenius{at}helsinki.fi
2 ORTON Orthopaedic Hospital, Invalid Foundation, Tenholantie 10, Helsinki FIN-00280, Finland

Investigation performed at the ORTON Orthopaedic Hospital, Invalid Foundation, Helsinki, Finland

In support of their research or preparation of this manuscript, one or more of the authors received grants or outside funding from the Sivia Kosti Foundation, Instrumentarium Research Foundation, Foundation for Pediatric Research, Ulla Hjelt Fund, Pär Slätis Joint Surgery Foundation, and Emil Aaltonen Foundation. None of the authors received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

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.


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