Total knee arthroplasty in ochronosis: a case report and critical review of the literature.

Alkaptonuria is an autosomal recessive disorder caused by the deficiency of homogentisate 1.2 dioxygenase activity. The clinical presentation shows an ochronotic pigment which is deposited in all connective tissues, including in cartilage, particularly. The knee is the most common site of peripheral abnormality. There is currently no definitive cure for alkaptonuric ochronosis. In this article, we present a 69-year-old male case who underwent bilateral cemented total knee arthroplasty simultaneously. Our results during two-year follow-up were satisfactory. A critical review of the literature revealed no uniformity in reporting such cases.