Total Hip Replacement in Ochronosis: Case Report

Ochronosis is not usual, it is the musculoskeletal manifestation of Alkaptonuria, a condition of abnormal metabolism of Sulphur containing amino acids which result in homogentisic acid accumulation in connective tissues due to homogentisic acid oxidase enzyme deficiency. The urine colour tends to turn into black due to oxidation in air. This is a middle-aged male in his fifties with left sided non traumatic hip pain, x-rays showed OA left hip. Intra op, we noticed the black deposits over the GT and immediately the diagnosis was clear. Urine sample was taken and left in vitro for 24 hours, the colour changed into black. Urinary homogentisic acid level is diagnostic in suspected cases, here no need to perform the test as the diagnosis was clear intra operatively. Ceramic on poly cementless THR was done. The head was preserved in formalin solution for study purposes.