Ocular histopathologic findings in a 46-year-old man with primary hyperoxaluria
T. Sakamoto, K. Maeda, K. Sueishi, H. Inomata and K. Onoyama
Department of Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
A 46-year-old Japanese man with familial primary hyperoxaluria had been
suffering from urolithiasis since the age of 6 years and had been treated
by hemodialysis for 8 years before his death. The alpha-ketoglutarate
glyoxylate carboligase and serine pyruvate transoxylase activities in the
patient's liver had markedly decreased, indicating type I primary
hyperoxaluria. Ocular tissues obtained at autopsy were studied
immunohistochemically and histochemically. The ocular alterations were
characterized by an unusual distribution of oxalate deposits, mainly
located in the outer plexiform layer and the nuclear layer but not in the
retinal pigment epithelium or in the vascularized layer of the sensory
retina. These distributions of the oxalate deposits were determined to be
unrelated to the blood vessels.
