Ochronotic arthropathy-a rare clinical case

Abstract

Ochronotic arthropathy is a rare condition found in patients with alkaptonuria that results from the accumulation of ochronotic pigment. We present the case of a 65-year-old woman who presented for medical evaluation due to hip and knee chronic pain. The physical and radiographic findings were compatible with an end-stage hip osteoarthritis and knee osteoarthritis. During total hip arthroplasty it was noticed that the articular capsule and the cartilage of the femoral head were black. In the postoperative period she was diagnosed with alkaptonuria. Later, a total knee arthroplasty was performed and once more the presence of black cartilage was noted. Alkaptonuria usually appears after age 30 and is usually asymptomatic until the involvement of the spine, hip, knee and shoulder joints. Therefore, orthopaedic surgeons must be suspicious of an atypical arthropathy in order to not be overwhelmed during surgery with the presence of darkened cartilage.

Figure 1:

Total hip arthroplasty

Figure 2:

Total knee arthroplasty

Figure 3:

Intraoperatively, hip arthroplasty

Figure 4:

Intraoperatively resected head of the femur

Figure 5:

Black ochronotic pigmentation in ear cartilage

Figure 6:

Intraoperatively, the joint surfaces of the ochronotic knee