Unilateral Pigmentary Retinopathy

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File number: 133820

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    Appears in
    Dystrophies
    Condition/keywords
    unilateral, retinitis pigmentosa, montage
    Imaging device
    Fundus camera
    Zeiss Visucam 224
    Description
    Montage fundus photographs of a 47 year old female presenting with unilateral vision loss in the left eye. Fundoscopy revealed extensive intraretinal pigment clumps, waxy disc pallor, and marked vessel attenuation in the left eye with a normal fundus in the right. Electroretinography showed unilateral reduction in rod and cone function. Unilateral pigmentary retinopathy, an uncommon variant of retinitis pigmentosa (reported incidence ˜ 5%) presents with RP-like changes in one eye, the fellow eye being completely normal. Proposed causes include lyonization and somatic mosaicism. Conditions which mimic RP should be excluded, and any diagnoses should be supported with electrodiagnostic tests and autofluorescence imaging. Management parallels RP, focusing on cataract and macular complications and long-term follow-up to monitor possible bilateral progression.

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