Abstract
INTRODUCTION: Alkaptonuria is a rare genetic disorder characterized by the absence of the enzyme homogentisate 1,2 dioxygenase, which leads to the accumulation of homogentisic acid in connective tissues and blood, resulting in Ochronosis and osteoarthropathy. CASE REPORT: We present a case report of a 56-year-old male with ochronotic arthropathy and quadriceps tendon rupture who underwent a total hip replacement. The patient exhibited right hip pain, difficulty walking, and restricted movement at the hip and spine. Radiographic and magnetic resonance imaging findings confirmed significant arthritic changes in the right hip joint and a mid-substance tear in the quadriceps tendon. Intraoperatively, dark pigmentation was observed in the affected tissues, consistent with the diagnosis of Ochronosis. The literature review indicates that joint replacement surgery is the treatment of choice for ochronotic arthropathy, with patients experiencing good functional recovery. The prosthesis's long-term survival rate is comparable to primary arthroplasty, and the optimal surgical approach regarding capsule preservation remains a topic of debate. CONCLUSION: Joint replacement is a feasible treatment option for patients with ochronotic arthropathy. Understanding the spine-pelvic relations and other potential complications can help surgeons plan for better recovery and functional outcomes.