Arthroplasty in Alkaptonuric Ochronosis

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Hassan Zmerly
Manuela Moscato
Valentina Di Gregori


Ochronotic Alkaptonuria, arthropathy, Joint replacement


Ochronotic arthropathy occurs in patients with alkaptonuria. Alkaptonuria is a rare single-gene disorder characterized by black pigmentation of cartilage and other connective tissues. Premature degenerative arthritis affects the large joints in many of these of patients.   As in other metabolic bone diseases, there are concerns about the quality and strength of affected bones and therefore the suitability and longevity of replacement arthroplasty. We report a case of a patient with sev­eral musculoskeletal manifestations of Alkaptonuria treated with total hip and total  knee  replacement. Perioperative management of these patients may require more careful consideration pertinent to the associated comorbidities of this disorder.

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