Ocular Findings in Hereditary Ochronosis

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Abstract
No feature of hereditary ochronosis is more prominent than the pigmentation that occurs in the eye. Nonetheless, the pathology of affected eyes has received only slight attention. A review of the literature revealed only a single report11 describing the gross examination of an enucleated ochronotic globe, 2 recorded biopsies of involved conjunctiva and cornea,18,20 and 1 account of the histological examination of an ochronotic globe.19 This report describes the findings of hereditary ochronosis, including gross and microscopic features of the eyes, in an 83-year-old man, who died of myocardial infarction. The ochronotic lesions demonstrate the destructive features of the disease and emphasize the diverse and widespread pigment deposition. Report of Case An 83-year-old retired architect entered the University of California Medical Center for evaluation of abdominal pains and nausea of 6 months' duration. For most of his life this patient had noted that his urine was yellow

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