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. 2014;5(8):554-7.
doi: 10.1016/j.ijscr.2014.06.015. Epub 2014 Jun 27.

A rare cause of arthropathy: An ochronotic patient with black joints

Mutlu Harun  1 , Yaldız Hayrettin  2 , Mutlu Serhat  3 , May Cuneyt  4 , Fidan Fırat  5 , Ozkaya Ufuk  6
Affiliations

A rare cause of arthropathy: An ochronotic patient with black joints

Mutlu Harun et al. Int J Surg Case Rep. 2014.

Abstract

Introduction: Alkaptonuria is an autosomal recessive disorder of metabolism. The pathogenesis of alkaptonuria includes chronic inflammation, degeneration, and eventually osteoarthritis. Ochronotic arthropathy is a rare condition found in patients with alkaptonuria.

Presentation of case: A 60-year-old female presented for evaluation after a 10-year history of low back pain, right hip pain, and bilateral knee pain. A cementless right total hip and a cemented left knee replacement were performed. Intraoperatively, the joint surfaces, neighboring ligaments, and tendons were black with pieces of black cartilage tissue. Histological sections of bone and soft tissue demonstrated classic findings of ochronosis, including multiple pigmented areas, reactive giant cells, and a thickened, inflamed synovium.

Discussion: The management of ochronotic arthropathy in alkaptonuria patients is usually conservative, but replacement surgery is offered for severely affected hip and knee joints. A few reports of the surgical treatment of ochronotic arthropathy have been published. This report describes a case of ochronotic hip and knee arthritis treated with total hip and knee arthroplasties.

Conclusion: Joint replacement has excellent outcomes in a patient with significant degenerative arthropathy due to ochronosis.

Keywords: Alkaptonuria; Black cartilage; Joint replacement; Ochronosis.

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Figures

Fig. 1
Fig. 1
Preoperative anteroposterior radiograph of the hips.
Fig. 2
Fig. 2
Postoperative anteroposterior radiograph of the right hip showing cementless total hip prosthesis.
Fig. 3
Fig. 3
(a) Preoperative anteroposterior radiograph of the knees. (b) Preoperative lateral radiograph of the left knee.
Fig. 4
Fig. 4
(a) Intraoperatively the joint surfaces of the ochronotic knee. (b) Intraoperatively resected portion of the tibial plateau.
Fig. 5
Fig. 5
(a) Microscopic examination revealed “synovial stroma, pigmented macrophages, inflammatory cells, and broken glass as material accumulation” (H&Ex10). (b) Microscopic examination revealed “synovial tissues with pigmented cartilage fragments” (H&Ex20).
Fig. 6
Fig. 6
(a) Postoperative anteroposterior radiograph of the cemented left knee prosthesis. (b) Postoperative lateral radiograph of the cemented left knee prosthesis.
See this image and copyright information in PMC

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