Ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare hereditary metabolic disorder characterised by the absence of the enzyme homogentisic acid oxidase and associated with various systemic abnormalities related to the deposition of homogentisic acid pigment (ochronotic pigment). In this report, we describe a 53-year-old, HLA-B27(+) woman with ochronotic arthropathy. In addition to the typical clinical features of the disorder, she had bilateral hip involvement, which was improved by cementless total hip prosthesis.
A 23-year-old, male patient previously operated on four times for a recurring, benign, paranasal mass was admitted to our clinic with pain and swelling under the knee. Physical and radiological examination revealed a malignant process. The histopathological examination of the fine-needle biopsy specimen of the tibia was a malignant mixed tumor. Then. en-bloc resection, free fibular grafting, and fixation with blade plate was performed for one-third of the tibia, conserving the knee joint. Recurrences and metastasis were not observed after surgery and radiotherapy.
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