A patient presents with pigmentary changes and retinal thinning. What is your diagnosis?

Tip #223


In this image, there is thinning of the deep retina. There is loss of the outer nuclear layer; the external limiting membrane and ellipsoid are absent. There is a reverse shadow of the choroid. This area corresponded to areas of hypoautofluorescence on FAF and peri-vascular pigment (video).

The patient was treated for pigmented paravenous retinochoroidal atrophy (PPRCA).

In this tip's accompanying 12 min. video, we'll review:

  • The differential diagnosis of this case
  • How PPCRA is diagnosed
  • Which lab tests should be ordered
Tip #223 Analysis


In a patient with perivascular pigment consider paravenous pigmentary chorioretinal atrophy - but rule out inflammatory and infectious causes.

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