An unusual case in total knee replacement surgery: alkaptonuric ochronosis
DOI:
https://doi.org/10.64575/c86hw049Keywords:
Alkaptonuria, ochronosis, total knee arthroplasty, degenerative joint disease, surgeryAbstract
Alkaptonuria is a rare autosomal recessive metabolic disorder caused by homogentisic acid oxidase deficiency, leading to the accumulation of homogentisic acid and ochronotic pigmentation in connective tissues. Although it typically presents with dark urine and arthropathy in later life, it often remains undiagnosed until advanced degenerative changes occur. We report a 54-year-old male patient who underwent total knee arthroplasty due to advanced degenerative arthritis. Intraoperatively, unexpected black pigmentation of the articular cartilage and surrounding connective tissues was observed. The patient had no previous diagnosis of alkaptonuria and was managed with postoperative metabolic evaluation, dietary advice, and follow-up. Ochronosis may remain undetected until late adulthood and can be encountered incidentally during orthopedic procedures. Awareness of this rare entity is crucial for orthopedic surgeons to ensure accurate intraoperative assessment and appropriate postoperative management.
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Copyright (c) 2025 Ahmet Yesevi Sarıaslan, Muhammed Ali Demir, Oğuz Temizyürek (Author)
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