Document Type: CASE REPORT

Authors

1 Department of Trauma and Orthopaedic, Shahid Modarress Hospital, Saveh, Markazi, Iran

2 Department of Orthopaedic Surgery and Department of Sports Medicine, Iran University of Medical sciences, Tehran, Iran

Abstract

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

Keywords

Main Subjects

1. 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(8):554-7.
2. Ozmanevra R, Güran O, Karatosun V, Günal I. Total
knee arthroplasty in ochronosis: a case report and
critical review of the literature. Eklem Hastalik
Cerrahisi. 2013; 24(3):169-72.
3. 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(5):584-6.
4. Acar MA, Erkocak OF, Aydin BK, Altan E, Senaran H,
Elmadag NM. Patients with black hip and black knee
due to ochronotic arthropathy: case report and review
of literature. Oman Med J. 2013; 28(6):448-9.
5. Siavashi B, Zehtab MJ, Pendar E. Ochronosis of hip
joint; a case report. Cases J. 2009; 16(2):9337.
6. Mohapatra S, Biswas T, Das S, Saxena A. Ochronosis
of the hip joint: differential diagnosis of inflammatory
arthropathy with lytic lesion. Indian J Clin Biochem.
2014; 29(1):112-3.
7. Cebesoy O, Isik M, Subasi M, Kaya A, Bilgin F, Kaya O.
Total hip replacement for an ochronotic patient: a
technical trick. Am J Case Rep. 2014; 15(1):27-30.
8. Ulucay C, Ozler T, Altintas F, Inan M, Onur A.
Arthroplasty in ochronosis “tips and pearls in
surgery”: case series. J Arthritis. 2013; 3(116):2.
9. Manoj Kumar RV, Rajasekaran S. Spontaneous tendon
ruptures in alkaptonuria. J Bone Joint Surg Br. 2003;
85(6):883-6.
10. Alajoulin OA, Alsbou MS, Ja’afreh SO, Kalbouneh HM.
Spontaneous Achilles tendon rupture in alkaptonuria.
Saudi Med J. 2015; 36(12):1486-9.
11. Sahoo MM, Mahapatra SK, Sethi GC, Dash SK. Patellar
ligament rupture during total knee arthroplasty in
an ochronotic patient. Acta Orthop Traumatol Turc.
2014; 48(3):367-70.
12. Fisher AA, Davis MW. Alkaptonuric ochronosis with
aortic valve and joint replacements and femoral
fracture: a case report and literature review. Clin Med
Res. 2004; 2(4):209-15.

13. Wauthy P, Seghers V, Mathonet P, Deuvaert FE. Cardiac
ochronosis: not so benign. Eur J Cardiothorac Surg.
2009; 35(4):732-3.
14. Ogata J, Tamura K, Miyanishi K, Minami K, Haranishi Y.
Anesthesia in a patient with alkaptonuric ochronosis
for total hip arthroplasty. Masui. 2008; 57(4):439-42.
15. Pandey R, Kumar A, Garg R, Khanna P, Darlong
V. Perioperative management of patient with
alkaptonuria and associated multiple comorbidities.
J Anaesthesiol Clin Pharmacol. 2011; 27(2):259-61.