Case Study: November 2006

During the routine cut-up of an enucleation specimen for suspected posterior pole uveal melanoma, from a male adult aged 60 years old, some well-defined, white patches were noticed in the vicinity of the antero-inferior choroid / retina. The axial length of the eyeball was 24mm.

Figure 1 is a macro photo of these white patches.

Figure 2 is a low power micro of one of these white patches. The white patch lies between the 2 arrows.

Figure 3 is a higher power of the white patch.
Questions

1. What is the likeliest explanation for these white patches?

2. What do figures 2 and 3 show?

Click to show answers
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1. This is a nice example of confluent ‘paving stone’ or ‘cobblestone’ degeneration.

2. Retinochoroidal adhesion, neural retinal atrophy, gliosis and loss of photoreceptors. Intact Bruch’s (not readily apparent on this H and E), choroidal atrophy with loss of the choriocapillaris and absent RPE.

For a good summary of cobblestone degeneration, see Yanoff and Fine’s Ocular Pathology p401; Bill Lee’s book-p228.

Some quick facts about cobblestone degeneration:

• Degenerative process-increase with age and with axial length of eye (hence commoner in myopic eyes).
• Often bilateral
• Present in 25% of autopsy cases.
• Located between the ora serrata and equator and separated from ora by normal retina.
• Commonly located in infero-temporal quadrant.