Pavingstone Chorioretinal Atrophy (Primary Chorioretinal Atrophy)

Overview

Pavingstone chorioretinal atrophy (also known as Primary chorioretinal atrophy or Cobblestone chorioretinal atrophy) is a benign retinal thinning, involving loss of the RPE and absence of photoceptors, that occurs in the peripheral retina. A strong adhesion between the retina and choroid exists in these areas. It is found more commonly in eyes with longer axial length, is often bilateral, and prevalence increases with age.

Case Examples

  • Case 1

    An 89-year-old female with visual acuities of 6/9+2 (20/30+2) and 6/9-2 (20/30-2) and no night vision problems or family history of inherited retinal dystrophies.

    Retinal photo (right eye, nasal retina)

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    Retinal photo (right eye, nasal retina)

    Circumscribed areas of chorioretinal atrophy are present in the nasal periphery, some forming confluent areas of atrophy.

    Optomap pseudocolour image (right eye)

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    Optomap pseudocolour image (right eye)

    Optomap shows numerous areas of chorioretinal atrophy in the nasal mid-periphery, some confluent, with increased visibility of underlying choroidal vasculature. Incidental findings of drusen and areas of hyperpigmentation were also present in the superior and inferior mid-periphery.

    Green separation image (right eye)

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    Green separation image (right eye)

    Optomap green separation images shows numerous areas of chorioretinal atrophy in the nasal mid-periphery, some confluent, with increased visibility of underlying choroidal vasculature. Incidental findings of drusen and areas of hyperpigmentation were also present in the superior and inferior mid-periphery.

    Spectralis OCT volume (1) and line scans (2) - right eye, nasal retina

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    Spectralis OCT volume (1) and line scans (2) - right eye, nasal retina

    OCT shows thinning of the retina, loss of the RPE, relatively thinner choroid and hyper-transmission of the OCT signal through the choroid and sclera within areas of pavingstone chorioretinal atrophy.

  • Case 2

    A 54-year-old Caucasian male with high myopia in the right eye (-9.50DS).

    Optomap pseudocolour (top) and green separation (bottom) images

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    Optomap pseudocolour (top) and green separation (bottom) images

    Areas of well-demarcated but confluent chorioretinal atrophy, some with hyperpigmented borders, in the inferior peripheral retina. There is greater visibility of the choroidal vasculature. The white appearance of the lesion arises from the loss of RPE and retinal atrophy resulting in greater visibility of the underlying sclera.

  • Case 3

    A 65-year-old Caucasian male with high myopia in the right eye (-10.50DS).

    Optomap pseudocolour (1), green separation (2) and fundus autofluorescence (3) images - right temporal retina

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    Optomap pseudocolour (1), green separation (2) and fundus autofluorescence (3) images - right temporal retina

    Areas of well-demarcated but confluent chorioretinal atrophy, some with hyperpigmented borders, in the temporal peripheral retina. There is greater visibility of the choroidal vasculature. The white appearance of the lesion arises from the loss of RPE and retinal atrophy resulting in greater visibility of the underlying sclera.

Differential Diagnosis

Atrophic retinal holes

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Lattice degeneration

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Enclosed oral bay

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Toxoplasmosis

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References

Alexander L. Primary Care of the Posterior Segment, 3rd ed. McGraw-Hill, 2002.