Ochronosis is connective tissue manifestation of Alkaptonuria. Joint involvement especially hip and knee destruction is seen. The cartilage is pigmented and destroyed. A 55years old male (Figure 1) with a previous total arthroplasty on both shoulders, has recently develop a hip pain. He was affected of alkaptonuria (AKU). We proceed with a total hip arthroplasty with cement less cup and modular...

OBJECTIVE To review the cases sent to the section of histopathology, Aga Khan Universty (AKU) for second opinion and see whether there are significant differences in the original outside diagnosis and the subsequent diagnosis submitted by us. METHODS A retrospective study of all consecutive cases for second opinion in the form of paraffin blocks from 1st Novemver 2001 to 31st July 2002. The p...

Alkaptonuria is an autosomal recessive metabolic disorder characterized by joints and spine involvement, ochronosis and presence of homogentisic acid in urine and its deposition in cartilage, intervertebral disc and other connective tissues, leading to disabling arthritis in elderly individual.

Four cases of operative treatment for ochronosis arthropathy in patients with alcaptonuria are presented. In 3 hip joint and 1 patient knee were affected. Prior to surgery all treated conservatively. Two successfully underwent total replacement. Intertrochanteric femur osteotomy was performed case. the fourth arthroplasty allograft from rib cartilage failed due suppurative arthritis resulted re...