Cardiac ochronosis: not so benign

Wauthy, Pierre; Seghers, Valérie V.; Mathonet, Perrine; Deuvaert, Frank

doi:10.1016/j.ejcts.2008.12.032

Cited by 17 publications

(15 citation statements)

References 3 publications

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“…Other body locations include the eyelids, sclera, foreheads, cheeks, axillae, genitals, nail beds, buccal mucosa, larynx, tympanic eardrum and nasal tip. Simultaneously, this discoloration can occur in skin, tendons, ligaments, costochondral junctions, sclera, heart valves, the intima of blood vessels and cause lumbar intervertebral disc calcification and disc space narrowing [3] , [4] , [5] .…”

Section: Introductionmentioning

confidence: 99%

Total knee arthroplasty in ochronosis

Patel¹

2015

Arthroplasty Today

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Alkaptonuria is disorder of tyrosine metabolism due to deficiency of homogentisic oxidase characterized by excretion of homogentisic acid in urine, deposition of oxidized homogensitate pigments in connective tissues and articular cartilages (ochronosis). The result is dark pigmentation and weakening of the tissues resulting in chronic inflammation and osteoarthritis. Management of alkaptonuric ochronic osteoarthritis is usually symptomatic and replacements have comparable outcomes to osteoarthritis in patients without ochronosis. I report a case of a patient with ochronosis of knee treated with total knee replacement and report operative pearls for surgery in this rare disorder.

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Section: Introductionmentioning

confidence: 99%

Total knee arthroplasty in ochronosis

Patel¹

2015

Arthroplasty Today

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“…Patients with ochronosis may have cardiac problems, which are coronary or valvular calcifications, and may have dyspnea and diminished vital capacity [16,17]. Therefore spinal anesthesia could be a good choice but degenerative changes such as narrowed disk spaces and spine fusions may challenge the regional anesthesia techniques [18].…”

Section: Discussionmentioning

confidence: 99%

Arthroplasty in Ochronosis “Tips and Pearls in Surgery”: Case Series

Ozler¹

2014

J Arthritis

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Introduction: The aim of this paper is to get the surgeon familiar with black cartilage and massive synovitis while performing a total joint replacement operation including preoperative, intraoperative and postoperative tips and pearls. Case presentation: Two Caucasian male patients, one with knee osteoarthritis and one with both knee and hip osteoarthritis. Conclusion: The black cartilage and softened bone does not heal different from the normal bone, therefore internal fixation of the fractures and cement less fixation of the prosthesis will not fail. Caution should be paid for excessive bleeding after total synovectomy. Patients have to be informed about consecutive operations and recurrent postoperative effusions.

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“…[6] Other manifestations of this disease include pigment deposition, aortic or mitral valve calcification or regurgitation, and occasionally aortic dilatation, renal stones, prostate stones, and tendon rupture. [7–9] Progressive ochronotic arthritis always shows articular space narrowing, bone sclerosis, and effusion. [10] Although many clinical features were reported on the AKU disease, there were still lack of physiopathological mechanism studies.…”

Section: Introductionmentioning

confidence: 99%

Ochronotic arthritis and ochronotic Achilles tendon rupture in alkaptonuria

et al. 2019

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Introduction: Alkaptonuria (AKU) is a rare disease caused by deficiency of homogentisate 1,2-dioxygenase which results in deposition of homogentisic acid (HGA). Ochronotic arthritis, the deposition of excess oxidized HGA in the connective tissues, causes pigmentation and degeneration of the joint tissues ultimately resulting in chronic inflammation and osteoarthritis. The ochronotic arthritis has similar clinical features with osteoarthritis. There is currently no specific treatment for AKU and management is usually symptomatic. In severe cases, total joint arthroplasty is the major treatment approaches. It is rarely reported in China. Patient concerns: Here we reported a case of a patient with bilateral knee pain for more than 1 year. He complained of a 20-year history of chronic, nonspecific low back pain and stiffness. His urine was black since he was a child. Six years after the knee surgery, his Achilles tendon ruptured. Diagnosis: Specific radiographic and magnetic resonance imaging manifestations were observed. Darkly pigmented full-thickness cartilage and subchondral bone were found during the operation. Histological investigation also manifested dark stains in meniscus and synovial tissues. Black-denatured tendon tissue was also found during the operation. The patient was diagnosed as AKU. Interventions: Total knee arthroplasty and Achilles tendon repair were operated separately after the disease was diagnosed. Outcomes: The patient recovered very well after the second surgery. He returned to full activities, described no knee pain, and presented to the clinic walking without any aid. Physical examination revealed 0 to 20 of plantar flexion and 0 to 15 of dorsiflexion of the ankle. Conclusions: Ochronosis is a very rare disease in Asia. This paper supplies new information for study of this disease. The mechanism is still unknown right now. Further studies will be necessary.

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Cardiac ochronosis: not so benign

Cited by 17 publications

References 3 publications

Total knee arthroplasty in ochronosis

Total knee arthroplasty in ochronosis

Arthroplasty in Ochronosis “Tips and Pearls in Surgery”: Case Series

Ochronotic arthritis and ochronotic Achilles tendon rupture in alkaptonuria

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