Document Type: CASE REPORT
Department of Trauma and Orthopaedic, Shahid Modarress Hospital, Saveh, Markazi, Iran
Department of Orthopaedic Surgery and Department of Sports Medicine, Iran University of Medical sciences, Tehran, Iran
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 preoperative
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.
Level of evidence: V