Ochronosis or black joints disorder is a rare autosomal recessive disorder caused by deficiency of
homogentisic acid oxidase. Orthopaedic manifestations are common and mostly involve spine and large joints such as knee and hip.
Arthropathy is progressive and will eventually leads to
arthroplasty. Not being familiar with this disorder might lead to devastating complications. We present a 57 year-old woman with
Ochronosis who successfully underwent cemented cruciated substituted knee replacement and cementless hip replacement. Proper orthopaedic and
anesthetic pre-operative preparation, soft tissue specially patella tendon management throughout operation and meticulous
bleeding control during surgery are crucial. The results of the knee and the hip replacement surgery in this patient are satisfactory, after 24 months and 18 months follow-up, respectively. If Orthopaedic surgeons and anesthesiologists are well prepared, the outcome of
joint replacement in
Ochronosis patients will be as satisfactory as patients with primary
osteoarthritis.