Total Knee Replacement in Alkaptonuric Ochronosis : Two Case Reports and Literature Review

Total Knee Replacement in Alkaptonuric Ochronosis

Two Case Reports and Literature Review



alkaptonuria, ochronotic arthropathy, total knee replacement


Background and objective: Alkaptonuria is a rare disease characterized by the accumulation of homogentisic acid (HGA). Over time, these patients may develop disabling ochronotic arthropathy. We present 2 cases of patients with end-stage arthropathy treated with total knee arthroplasty (TKA).

Methods:  Both patients complained of disabling knee pain and reported limited walking distance (200-300 m). One had a history of osteotomy for medial knee arthtritis and ignored his underlying condition. The other presented with valgus gonoarthrosis and diagnosis of alkaptonuria.

Results: Intraoperatively, the characteristic dark-blue color in the joint was observed. Both patients evolved favorably after TKA with excellent results according to the Knee Society Scores (KSS) at three years of follow-up.

Conclusion: We believe TKA is the right treatment for patients with end-stage disease because it offers considerable relief from pain and allows patients to recover function.


Karaoglu S., Karaaslan F., Mermerkaya MU. Long-term result of arthroplasty in the treatment of a case of ochronotic arthropathy. Acta Orthop Traumatol Turc 2016;50:584–6.

Millucci L., Giorgetti G., Viti C., Ghezzi L., Gambassi S., Braconi D. Chondroptosis in alkaptonuric cartilage. J Cell Physiol 2015;230:1148–57.

Millucci L., Bernardini G., Spreafico A., Orlandini M., Braconi D., Laschi M. Histological and ultrastructural characterization of alkaptonuric tissues. Calcif Tissue Int 2017;101:50–6.

Li N., Tian W., Yuan Q., He D. Cervical spondylotic myelopathy due to the ochronotic arthropathy of the cervical spine. J Korean Neurosurg Soc 2016;59(1):65-68.

Gil J.A., Wawrzynski J., Waryasz GR. Orthopedic manifestations of ochronosis: pathophysiology, presentation, diagnosis, and management. Am J Med 2016;129(5):536.e1–6.

Ewald FC. The knee socity total knee arthroplasty roentgenographic evaluation and scoring system. Clin Orthop Relat Res 1989;248:9-12.

Harun M., Hayrettin Y., Serhat M., Cuneyt M., Fırat F., Ufuk O. A rare cause of arthropathy: an ochronotic patient with black joints. Int J Surg Case Rep 2014;5:554–7.

Drakoulakis E., Varvitsiotis D., Psarea G., Feroussis J. Ochronotic arthropathy: diagnosis and management—a critical review. Am J Orthop 2012;41:80–3.

Keller J.M., Macaulay W., Nercessian O.A., Jaffe I.A. New developments in ochronosis: review of the literature. Rheumatol Int. 2005;25:81–85.

Raaijmaakers M., Steenbrugge F., Dierickx C. Ochronosis, arthroscopy of a black knee: a case report and review of the literature. Knee Surg Sports Traumatol Arthrosc. 2008;16:182–184.

Laskar F.H., Sargison K.D. Ochronotic arthropathy. A review with four case reports. J Bone Jt Surg. 1970;52:653–666.

Damarla N., Linga P., Goyal M., Tadisina SR., Reddy GS, Bommisetti H. Alkaptonuria: a case report. Indian J Ophthalmol 2017;65:518–21.

Acar M.A., Erkocak O.F., Aydin B.K., Altan E., Senaran H., Elmadag N.M. Pacientes con cadera negra y rodilla negra debido a artropatía ocrónica: reporte de caso y revisión de la literatura. Oman Med J. 2013; 28(6): 448–9.

Couto A., Rodrigues A, Oliveira P., and Seara M. Ochronotic arthropathy - a rare clinical case. Oxf Med Case Reports 2018;9:302-305.

Spencer J.M., Gibbons C.L., Sharp R.J., Carr A.J., Athanasou N.A. Arthroplasty for ochronotic arthritis: no failure of 11 replacements in three patients followed for 6–12 years. Acta Orthop Scand. 2004;75:355–358.

Wu Chean Lee, Tong Leng Tan, Ying Ho Chan. Total Knee Arthroplasty in Ochronosis Arthropathy: A case Report and Systematic Review. Case Reports Orthopedics. 2019;2019:1-8.

Rodriguez J. A., Saddler S., Edelman S., Ranawar C. S. Long-term results of total knee arthroplasty in class 3 and 4 rheumatoid arthritis. The Journal of Arthroplasty. 1996;11(2):141-145.

Ulucay C, Ozler T, Altintas F, Inan M, Onur A, et al. Arthroplasty in ochronosis “tips and pearls in surgery”: case series. J Arthritis. 2013;3;1-3.

Kajino A., Yoshino S., Kohda M., Nagashima S. Comparision of the results of bilateral total knee arthroplasty with and without patellar replacement for rheumatoid arthritis. A follow-up note. The Journal of Bone and Joint Surgery. American Volume. 1997;79(4):570-574.




How to Cite

Pesciallo C, Garabano G, Rodriguez J, Viale G, Tillet F, Del Sel H. Total Knee Replacement in Alkaptonuric Ochronosis : Two Case Reports and Literature Review . Acta Biomed [Internet]. 2022 Jun. 7 [cited 2024 Jul. 13];93(S1):e2022108. Available from: