year old male presented to us with complaints of poor vision in both eyes since childhood. He did not use any spectacles till presentation.
On examination, he was orthophoric and his unaided and best corrected visual acuity was FC 1/2
m, 20/70, N18 (+14DS/-2DCx10) and FC2m PR accurate, 20/200, N36 (+14DS/-1.5DCx1100
) in the right and left eye, respectively. Slit lamp showed shallow anterior chamber, intraocular pressure (IOP) by Goldmann applanation tonometry of 20
mm and 18
mm Hg and closed angles on 4 mirror gonioscopy in both eyes. Lens was clear in both eyes. His axial length (15.3
mm), corneal diameter and anterior chamber depth were suggestive of nanophthalmos. Central corneal thickness measured 555 microns and 554 microns in the right and left eye, respectively. Review history did not reveal any family history in siblings.
On a provisional diagnosis of pure non-familial nanophthalmos, he received prophylactic peripheral laser iridectomy (LPI) in both eyes. Dilated fundus examination showed crowded discs with obliterated cup and dilated engorged non-tortuous vessels in both eyes (Figures
&). There were prominent internal limiting striae radiating from the optic nerve to ½ disc diameter beyond the fovea associated with subretinal deposits in both eyes (Figure
). There was a cuff of subretinal fluid along the superotemporal arcade with underlying yellow subretinal deposits. There was a pigmented scar in left eye inferotemporal to macula in left eye.
Fundus photograph of the right and left eye with macular striae (black arrow) in adult nanophthalmos (inset showing red free photos).
Fundus Flourescein angiography of right eye (A) and Left eye (B) and corresponding Cirrus SD-OCT images (C and D) showing ILM folds in both eyes and juxtafoveal retinal pigment epithelial detachment in the right eye.
Fundus fluorescein angiography, FFA, revealed hyperfluorescence due to transmission defect at macula in both the eyes,dye pooling in PED in juxtafoveal region in the right eye (Figure
) and leak along superotemporal arcade in left eye.
Full field flash ERG done showed normal scotopic and photopic response. Humphrey visual fields 24-2 showed peripheral artefacts in both eyes. Cirrus SD-OCT horizontal line scan passing across the fovea showed extensive corrugations involving the inner limiting membrane (ILM) suggestive of ILM striae causing distorted foveal contour in both eyes (Figure
A large serous PED was seen encroaching the fovea in the right eye with folds in the RPE-choriocapillary layer. The left eye showed normal vitreoretinal interface and normal intraretinal layer with no folds in the REP-choriocapillary complex.
In view of the above OCT findings, we advised him spectacles with rehabilitative support with low vision devices with advice for periodic follow up.