A patient presents with pigmentary changes and retinal thinning. What is your diagnosis?
Analysis
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
OCT Tip
In a patient with perivascular pigment consider paravenous pigmentary chorioretinal atrophy - but rule out inflammatory and infectious causes.
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