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Qian LI, Xiaoyan Peng; Distinct Features of Intraretinal Cystoid Changes in Patients of Inherited Retinal Degenerations. Invest. Ophthalmol. Vis. Sci. 2018;59(9):1520.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate the imaging features of intraretinal cystoids changes (IRCs) in patients with inherited retinal degenerations (IRDs).
Totally 67 eyes of 40 cases in our IRDs database showing IRCs on OCT were further analyzed for their fundus photos, macular structures on OCT and vascular changes on fluorescein angiography (FA).
IRCs were shown in patients diagnosed as retinitis pigmentosa (RP, 29 eyes of 17 cases), Bietti crystalline dystrophy (BCD, 23 eyes of 15 cases), autosomal recessive bestrophinopathy (ARB, 14 eyes of 7 cases) and cone dystrophy (CD, 1 eye of 1 patient). In RP patients, visible IRCs were displayed in the outer portion of the retina (outer nuclear layer [ONL] and inner nuclear layer [INL]), showing a typical “honeycomb” appearance with enlarged/coalesced cavities in ONL of the fovea and smaller cysts in the INL in the parafovea region. The outer retinal structures including ONL, outer limiting membrane (OLM), ellipsoid zone (EZ) and retinal pigment epithelium (RPE) in the foveal and parafovea regions could be preserved, partially preserved or completely loss, but extensive disruptions of the outer retina was shown in the perifoveal region in all cases. On FA petaloid hyperfluorescence was demonstrated in macula, while extensive retinal and choroidal vascular leakages, telangiectasia and neovasculature were noted in the peripheral. Comparably in BCD patients, the cavities of IRCs were seen sporadically distributed in the inner layers (INL and ganglion cell layer [GCL]), fewer in amount and smaller in size as well, meanwhile the outer retinal and choriocapillary structures were severely lost throughout the macula region. No remarkable peripheral leakage or vascular alteration was found on FA in those cases. In ARB patients IRCs were all displayed bilaterally as the ‘honeycomb’ pattern in ONL and INL, with subretinal fluid and hyperreflective doposits, whereas outer retinal layers being preserved. No sign of peripheral vasculopathy was noted.
IRCs were seen mainly in RP, BCD and ARB, but showed varied morphologic features among those entities, implying potentially different etiology and treatment strategies. RPE and photoreceptor’s death, vascular response, inflammatory and genetic causes might contribute to the formation of the IRCs.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
Varied features of IRCs on SD-OCT
Choroidal leakage and peripheral vascular changes in an RP case with macular edema.
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