Cardiac stroke following total knee replacement in an ochronotic arthropathy: Case report and literature review

Alkaptonuria is a rare autosomal recessive disorder caused by defective metabolism of homogentisic acid (HGA) which on accumulation in the connective tissues causes arthritis, darkening of urine and connective tissue pigmentation. Knee is most commonly affected joint whereas pigment deposition is seen in entire body causing cardiovascular, genitourinary, ocular, cutaneous, and musculoskeletal complications. We here report such a case of bilateral ochronotic arthropathy, who was diagnosed to be alkaptonuric only during joint exploration. He sustained a cardiac catastrophic stroke on 3 rd post operative day of the left knee replacement which was done one week after the right knee replacement. With prompt treatment and good hospital care, the patient was revived successfully, without valvulotomy or valvular replacement. The spectrum of clinical manifestations are discussed in the report with emphasis on thorough history and clinical examination of the patient, before taking the patient to the total knee replacement to make accurate diagnosis before hand and be prepared for the complications or catastrophic by a multidisciplinary approach.