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Ocular involvement in primary hyperoxaluria.

T A Meredith, J D Wright, J A Gammon

    Archives of Ophthalmology (Chicago, Ill. : 1960)
    |April 1, 1984
    PubMed
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    Primary hyperoxaluria can cause calcium oxalate crystal deposition in the eyes. Advanced imaging and autopsy revealed widespread crystal distribution and a unique macular lesion in affected patients.

    Area of Science:

    • Ophthalmology
    • Nephrology
    • Genetics

    Background:

    • Primary hyperoxaluria (PH) is a rare genetic disorder.
    • It leads to excessive oxalate production and deposition in various organs.
    • Ocular manifestations in PH are not well-characterized.

    Observation:

    • Two cases of ocular involvement in PH are presented: one infant and one teenaged patient.
    • Autopsy in the infant revealed extensive calcium oxalate crystal deposition in ocular tissues using special staining.
    • The teenaged patient showed widespread retinal crystal deposits with a periarterial predilection on fundus examination and fluorescein angiography.

    Findings:

    • Histopathology confirmed wider ocular calcium oxalate crystal deposition than previously recognized.
    • Fluorescein angiography highlighted a specific retinal pattern of crystal deposition.

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  • A unique acquired black macular lesion was observed in the teenaged patient.

    • Implications:

    • This study underscores the significant ocular impact of primary hyperoxaluria.
    • Enhanced diagnostic techniques are crucial for identifying ocular involvement in PH.
    • Further research is needed to understand the pathogenesis of these unique ocular findings and develop targeted treatments.