Ochronotic arthropathy: diagnosis and management: a critical review.

Alkaptonuria, a rare hereditary metabolic disorder, is characterized by accumulation of homogentisic acid in the connective tissues resulting from lack of the enzyme homogentisic acid oxidase. Ochronosis, dark pigmentation of connective tissues, is the musculoskeletal manifestation of alkaptonuria. In this article, we report the case of a 53-year-old man who had ochronotic arthropathy and advanced degenerative changes in the shoulders managed with bilateral total shoulder arthroplasty.