Total Knee Replacement in Alkaptonuric Ochronosis

Acta Biomed. 2022 Jun 7;93(S1):e2022108. doi: 10.23750/abm.v92iS1.10439.

Abstract

Background and objective: Alkaptonuria is a rare disease characterized by the accumulation of homogentisic acid (HGA). Over time, these patients may develop disabling ochronotic arthropathy. We present 2 cases of patients with end-stage arthropathy treated with total knee arthroplasty (TKA).

Methods: Both patients complained of disabling knee pain and reported limited walking distance (200-300 m). One had a history of osteotomy for medial knee arthtritis and ignored his underlying condition. The other presented with valgus gonoarthrosis and diagnosis of alkaptonuria.

Results: Intraoperatively, the characteristic dark-blue color in the joint was observed. Both patients evolved favorably after TKA with excellent results according to the Knee Society Scores (KSS) at three years of follow-up.

Conclusion: We believe TKA is the right treatment for patients with end-stage disease because it offers considerable relief from pain and allows patients to recover function.

MeSH terms

  • Alkaptonuria* / complications
  • Alkaptonuria* / surgery
  • Arthroplasty, Replacement, Knee* / adverse effects
  • Humans
  • Joint Diseases* / surgery
  • Ochronosis
  • Pain / surgery

Supplementary concepts

  • Alkaptonuric ochronosis