Ochronotic Arthropathy Effectively Treated with Staged Bilateral Total Hip and Knee Arthroplasty: A Case Report
Abstract
Homogentisic acid deposition within connective tissues causes ochronotic arthropathy, a rare musculoskeletal consequence of alkaptonuria that causes progressive degenerative changes in the joints. Significant impairment and unique intraoperative challenges during surgical management may result from advanced involvement of weight-bearing joints. We present the case of a 53-year-old adult who arrived with radiating hip and knee pain, stiffness, and limited mobility. The patient's history of dark urine prompted a biochemical analysis that showed high levels of homogentisic acid. It's interesting to note that there were no traditional outward signs of ochronosis, such as bluish-black scleral discolouration (Osler's sign) or auricular cartilage pigmentation. Both the hip and knee joints showed significant degenerative changes on radiographs. A phased surgical approach was chosen due to the advanced arthropathy, starting with bilateral total hip replacement and progressing to bilateral total knee replacement. Pathognomonic symptoms of ochronotic arthropathy, i.e. diffuse blackish-brown pigmentation of the articular cartilage and subchondral bone, were observed during surgery. Following surgery, the patient experienced a smooth recovery and reported long-lasting pain relief along with a return to his normal level of mobility and independence. The Harris Hip Score (HHS) increased from 31 to 93, and the Knee Society Score (KSS) increased from 40 to 93 at the 12-month follow-up,…
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