Alkaptonuria is a rare metabolic disease caused by a partial or total deŞciency of homogentisic acidoxidase, which results in excess homogentisic acid (HGA) levels. Homogentisic acid and its oxidationproducts can accumulate in hyaline cartilage, tendons, and ligaments. A 55-year-old male was admittedcomplaining of worsening chronic pain in his left knee. A radiographic evaluation showed tricompartmental end- stage osteoarthritis. A cemented total knee replacement was performed. At the 10year follow-up, he had returned to full activity, had no knee pain, and was very satisŞed with theoutcome. No abnormality was observed in the femoral, tibial, or patellar components on radiography. Webelieve that total knee replacement is a good option in a patient with marked degenerative arthritissecondary to ochronotic arthritis.© 2016 Publishing services by Elsevier B.V. on behalf of Turkish Association of Orthopaedics andTraumatology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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