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The Journal of Bone and Joint Surgery (American) 85:441-447 (2003)
© 2003 The Journal of Bone and Joint Surgery, Inc.

Scientific Article

Total Hip Arthroplasty in Diastrophic Dysplasia

Ilkka Helenius, MD, PhD, Ville Remes, MD, PhD, Kaj Tallroth, MD, PhD, Jari Peltonen, MD, PhD, Mikko Poussa, MD, PhD and Timo Paavilainen, MD, PhD

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

Ilkka Helenius, MD, PhD
Arhipanpolku 8 b A, Helsinki FIN-00420, Finland. E-mail address: ilkka.helenius{at}helsinki.fi

Ville Remes, MD, PhD
Kaj Tallroth, MD, PhD
Mikko Poussa, MD, PhD
Timo Paavilainen, MD, PhD
ORTON Orthopaedic Hospital, Invalid Foundation, Tenholantie 10, Helsinki FIN-00280, Finland

Jari Peltonen, MD, PhD
Hospital for Children and Adolescents, Helskinki University Central Hospital, P.O. Box 281, Helsinki FIN-00029 HUS, 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, Par Slatis Joint Surgery Research Foundation, Ulla Hjelt Foundation, and Foundation for Pediatric Research. 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 growth failure, multiple joint deformities, and early osteoarthritis of the hips. Mortality is increased in early childhood, but thereafter life expectancy is normal. Because of severe flexion deformities, resting pain, and diminished movements of the hip joints, total hip arthroplasty is indicated at an early age. The purpose of our study was to evaluate prospectively the midterm results of total hip arthroplasty in a consecutive series of patients with diastrophic dysplasia.

Methods: Between 1982 and 1996, forty-one total hip replacements were performed in twenty-four consecutive patients with diastrophic dysplasia (mean age, forty-one years) at our hospital. The patients were followed prospectively for a minimum of five years with clinical examination, determination of Harris hip scores, and radiographs. Twenty-two patients (thirty-eight hips) were examined clinically and radiographically at the time of follow-up, and the remaining two patients (three hips) were contacted only by telephone. The mean duration of follow-up was 7.8 years.

Results: The mean Harris hip score increased from 44 points (range, 25 to 66 points) before the operation to 70 points (range, 37 to 89 points) at the final follow-up examination (p < 0.001). Ten complications (24%) were recorded. Five (12%) of the forty-one hips required revision because of aseptic loosening of the acetabular component at a mean of 9.4 years after the primary operation. No revisions were due to aseptic failure of the femoral component.

Conclusions: Implant survival was good and the Harris hip scores increased significantly after total hip arthroplasty in patients with diastrophic dysplasia. However, shortening femoral osteotomy and transposition of the greater trochanter, adductor and flexor tenotomies, and modification of the femoral stem were frequently needed. Total hip arthroplasty is recommended for patients with diastrophic dysplasia and severe degeneration of the hip joints, even for those who are relatively young.

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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