We performed a clinicopathological study on the eyes of a 3-year-old girl with primary hyperoxaluria type I. An examination one year before death disclosed a slightly diminished visual acuity in both eyes with black, geographic central macular, subretinal patches. Calcium oxalate was deposited predominantly in the retinal pigment epithelium of the posterior pole, where these cells were markedly hyperplastic and hypertrophied round foci of oxalate crystals. Oxalate crystals were exceedingly sparse in other ocular structures and when present were not associated with an apparent tissue reaction in these other locations. A collagenous layer was evident between parts of the retinal pigment epithelium and the neurosensory retina, which contained occasional perivascular clumps of melanin laden cells. The predominant deposition of oxalate in the retinal pigment epithelium, with the exuberant response of these cells around the crystals, gives a clue to the pathogenic mechanisms of primary hyperoxaluria.
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