Orphanet Journal of Rare Diseases (Oct 2023)

The favorable outcome of Bernese periacetabular osteotomy for the hip osteoarthritis in multiple epiphyseal dysplasia

  • Yao-Yuan Chang,
  • Chia-Che Lee,
  • Sheng-Chieh Lin,
  • Ken N. Kuo,
  • Jia-Feng Chang,
  • Kuan-Wen Wu,
  • Ting-Ming Wang

DOI
https://doi.org/10.1186/s13023-023-02920-1
Journal volume & issue
Vol. 18, no. 1
pp. 1 – 7

Abstract

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Abstract Background Multiple epiphyseal dysplasia (MED) is a rare congenital bone dysplasia. Patients with MED develop secondary hip osteoarthritis as early as the third to the fourth decade. Currently, there is no consensus on the prevention of the progressive hip osteoarthritis secondary to MED. The Bernese periacetabular osteotomy (PAO) is a joint-preserving surgery to reshape acetabulum and extend femoral head coverage. However, there is no documentary evidence for the effect of the procedure on MED hips. Patients and methods We analyzed the preliminary outcomes following the Bernese PAO in 6 MED hips. The average age at the time of surgery was 14.3 years (range from 11.4 to 17.2 years). For our study interest of time efficiency, radiographic parameters were analyzed preoperatively and 1 year postoperatively. The hip function was evaluated by the Harris Hip Score (HHS) before and after surgery. Results The mean follow-up time was 1.7 years. The mean lateral center–edge angle increased from 3.8° to 47.1° (p = 0.02), anterior center–edge angle increased from 7.3° to 35.1° (p = 0.02), and acetabulum index decreased from 27.8° to 14.6° (p = 0.04). The femoral head coverage ratio increased from 66.8% to 100% (p = 0.02). The post-operative anteroposterior pelvic radiograph demonstrated all preoperative broken Shenton lines were reversed. The mean HHS improved from 67.3 to 86.7 (p = 0.05). Conclusion Bernese PAO is a feasible treatment for hip disorders in MED patients. It reshapes acetabular and femoral morphology efficiently. In our study, the preliminary results showed the procedure not only improved radiographic outcomes but also hip function.

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