Morphometric analysis of the extramacular retina from postmortem eyes with retinitis pigmentosa.
OBJECTIVE
To evaluate the degree of inner retinal preservation in the extramacular regions of postmortem retinitis pigmentosa (RP) eyes.


METHODS
Eighteen RP retinas and 11 age-matched healthy retinas were sectioned for morphometric analysis by light microscopy. The 18 RP retinas were classified by disease severity and mode of inheritance. Cell nuclei in the outer nuclear layer (ONL), inner nuclear layer (INL), and ganglion cell layer (GCL) were counted in adjacent 125-microm segments from an area spanning the region between 4 mm and 10 mm from the fovea.


RESULTS
A mixed-effects model showed a decrease in mean cell counts for each of the cell layers when the severity groups and inheritance types compared with those of control retinas. There was no statistically significant difference in the number of nuclei preserved in the INL and GCL in the moderate group compared with the severe group. Results from the INL counts for the different inheritance types of RP showed a higher overall mean percentage of cells was preserved for the autosomal dominant RP (ADRP) group when compared with the X-linked (XLRP) and simplex RP groups. Analysis of the GCL counts revealed significantly more counts only in the ADRP group compared with the XLRP group; the other group comparisons were not significant.


CONCLUSIONS
Retinitis pigmentosa results in cell loss in all retinal layers, with the most profound loss in the ONL, followed by the GCL and then the INL. The preservation of the INL and GCL in the extramacular region is less than that previously reported for the macular region of the same retinas.
Humayun
M S
MS
Wilmer Ophthalmological Institute at Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Prince
M
M
de Juan
E
E
Barron
Y
Y
Moskowitz
M
M
Klock
I B
IB
Milam
A H
AH
D000368:Aged; D000369:Aged, 80 and over; D002452:Cell Count; D002467:Cell Nucleus / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012160:Retina / Q000473:pathology*; D012165:Retinal Ganglion Cells / Q000473:pathology; D012174:Retinitis Pigmentosa / Q000145:classification / Q000235:genetics / Q000473:pathology*