فهرست نشریات علمی دسترسی آزاد ایران

Ochronosis or black joints disorder is a rare autosomal recessive disorder caused by deficiency of homogentisic acidoxidase. 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 todevastating complications. We present a 57 year-old woman with Ochronosis who successfully underwent cementedcruciated substituted knee replacement and cementless hip replacement. Proper orthopaedic and anesthetic preoperativepreparation, soft tissue specially patella tendon management throughout operation and meticulous bleedingcontrol 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