Alcaptonuria is an inherited hereditary metabolic disorder, which is associated with various systemic abnormalities and related to the accumulation of homogentisic acid and a derived melanine-like pigment in the connective tissues; the latter is termed ochronosis. We present the arthroscopic findings in the shoulder of a 58-year-old female with ochronotic arthropathy and discuss the role of arthroscopy in the diagnosis and management of this rare metabolic disorder.

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HighlightsCase 1: massive calcific deposits in the context of the supraspinatus muscle belly.Case 2: calcific deposits at the biceps anchor.Case 3: calcific deposits in the cranial intra-articular trait of the subscapularis tendon.These 3 atypical cases were not responsive to conservative treatment.Arthroscopic removal of the deposits ensured an optimal clinical outcome.

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