Ochronotic Surprise during Total Knee Replacement! A Case Report

Introduction:Alkaptonuria is a rare autosomal recessive metabolic disorder characterized by accumulation of homogentisic acid (HGA) due to an inherited deficiency of the enzyme HGA oxidase. Unlike rheumatoid arthritis which affects the small joints of the hands and feet, ochronotic arthropathy predominantly involves the large weight-bearing joints such as hips, knees, and spine. The knee is the most common joint to be affected. Ochronotic arthropathy is usually managed conservatively, but for severely affected hip and knee joints, replacement is considered. Case Report:This report describes a case of 57-year-old male who presented with bilateral knee osteoarthritis who was incidentally diagnosed with ochronosis intraoperatively during total knee arthroplasty, its challenges faced and post-operative functional outcome after 18 months follow-up. Conclusion:This case report highlights the need for high index of suspicion for preoperative diagnosis of ochronotic arthropathy. Difficult spinal anesthesia should be anticipated. Friable and stiff tendon due to ochronotic involvement can put the extensor tendon at risk of rupture during retraction of patella intraoperatively. Post-operative active quadriceps rehabilitation should be done with caution due to friable tendon. Keywords:Alkaptonuria, ochronosis, ochronotic arthropathy, autosomal recessive, total knee arthroplasty.

The Black Knee – A Case Report

Introduction: Ochronotic arthropathy in patients with alkaptonuria is a rare hereditary disorder. The altered metabolism causes the homogentesic acid derivatives to deposit in various connective tissues causing characteristic pigmentation. Due to the close clinical resemblance to that of a degenerative disorder, diagnosis of ochronotic arthropathy usually occurs intraoperatively. We report arthroscopic findings of a 50-year-old female with ochronotic arthropathy. Case Report: A 50-year-old woman came with complaints of pain and swelling in the left knee. Clinical examination and MRI findings were correlated to reveal a tear of lateral meniscus. On arthroscopic examination, the blackish pigmentation of the meniscus and the articular cartilage led to the diagnosis of ochronotic arthropathy. Conclusion: Arthroscopy plays an important role in diagnosis and treatment of patients with ochronotic arthropathy. The characteristic arthroscopic finding may aid diagnosis even in patients who do not have other systemic manifestations. Timely arthroscopic intervention can help delay the disease progression. Keywords: Alkaptonuria, Ochronotic arthropathy, Ochronosis, Arthroscopy.

Black Femoral Head Due to Ochronotic Arthropathy: A Case Report

Background: Ochronotic arthropathy is a rare complication in patients with alkaptonuria (AKU) that occurs as a result of ochronotic pigment accumulation in the joints. Case Report: This case report presents a 54-year-old patient with severe degenerative arthritis who underwent total hip arthroplasty on both sides within an interval of three years. The diagnosis was made by observation of a dark capsule and femoral head during the second surgery. The surgical treatments significantly reduced the pain and improved the range of motion (ROM). Conclusion: Orthopedic surgeons must be vigilant about clinical manifestations of this rare condition, before and during the surgery. Arthroplasty is a favourable therapeutic option in patients suffering from ochronotic arthropathy.

Case Report: Ochronotic Arthropathy

Ochronosis is a rare metabolic disease caused by the deficiency of the homogentisic acid oxidase enzyme. It gives clinical findings related to the accumulation of homogentisic acid in soft tissues and excretion in urine. Patients with chronic arthropathy usually have some joint pain and inflammatory back pain. Although axial involvement radiologically resembles ankylosing spondylitis (AS), it is differentiated by the absence of typical syndesmophytes, facet involvement, sacroiliac erosion and fusion.Although there is no effective treatment for ochronosis disease; our patient was given a protein-poor diet, vitamin C supplementation (100 mg/kg/day) and analgesic treatment for symptoms recommended in the literature; and a reduction in joint complaints was observed. In this case, the aim is to diagnose the rare ochronotic arthropathy followed with the diagnosis of AS and to show that both axial and peripheral joint involvement can be together in this disease.