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CHRPE

Introduction

CHRPE is typically associated with genetic defects, and systemically can be associated with familial adenomatous polyposis (FAP).
- Considerably rare, with an approximate prevalence of 1.2%
- Presents in 3 forms, either alone, or in a group known as bear tracks, or following neither.

Dead Giveaways

Histology:

  • Characterised by grouped pigmentation, present in a monolayer of hypertrophised RPE cells.
    Characterised by grouped pigmentation, present in a monolayer of hypertrophised RPE cells.

Red-Green Fundoscopy Channels:

  • Whilst this is not a distinct feature of CHRPE, this technique can be used to identify RPE lesions

  • Red separation (green-free) penetrates deeper into the eye, revealing the choroidal and RPE layers

  • Green separation (red-free) penetrates not as much into the eye, revealing the RPE and retinal layers

  • Blue light will view the anterior retinal layers

  • Hence, if a lesion appears in both red and green separation channels, it must be present in the RPE (overlapping layer).

    • Or, it could simply be in multiple layers

Present in both green and red separation channels
Present in both green and red separation channels

diagnostic features

Signs of different CHRPE

Solitary:

  • Single flat round lesion

  • Note the single big lesion
    Note the single big lesion

Multifocal (Bear Tracks):

  • A group or cluster of pigmentary lesions

  • Typically referred to sometimes as Congenital Grouped Lesions of the RPE

  • Consecutive clusters of pigmentary lesions that looks like animal bear tracks
    Consecutive clusters of pigmentary lesions that looks like animal bear tracks

Atypical

  • Typically smaller lesions of variable shape, and doesn't follow the previous two presentations

2025, made by Eric Qin. UNSW. SOVS

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