A 56-year-old male with unilateral painless diminution of vision.

A 56-year-old male presented to us with painless diminution of vision in the left eye noticed 1 month back. Best-corrected visual acuity was 6/36 in the left eye and 6/9 in the right eye. Anterior segment and intraocular pressure of both eyes were normal. There was no relative afferent pupillary defect in the left eye. The patient was a nonsmoker and there was no history of diabetes or hypertension. The fundus photographs of the right and left eyes are given in Figures 1 and 2, respectively.

Figure 1

Fundus photograph of the right eye

Figure 2

Fundus photograph of the left eye

Questions

Describe the findings in Figures 1 and 2. What is the diagnosis?

What other investigations will you like to do to confirm the diagnosis?

Answers

The right eye shows multiple hard drusen at the macula with focal areas of hypopigmentation. The left eye has a crescent-shaped small zone of increased pigmentation at and just nasal to the fovea. Beyond this further temporally is a crescentic broad zone with well-defined borders with hypopigmentation and prominence of choroidal vessels. Features are suggestive of dry age-related macular degeneration (ARMD) in the right eye and a retinal pigment epithelium (RPE) rip in the left eye.

Fundus fluorescein angiogram (FFA) and optical coherence tomography (OCT) confirm the diagnosis. FFA of the left eye [Figure 3] shows blocked fluorescence at and just nasal to the fovea corresponding to the area of curled RPE. Temporal to this, there is a zone of hyperfluorescence corresponding to the window defect in the crescent-shaped area where the RPE is deficient. FFA was not suggestive of choroidal neovascular membrane in the form of leak even in late phases. The right eye showed multiple focal areas of hyperfluorescence corresponding to the window defects from areas of focal RPE loss and hard drusen. OCT of the right eye was suggestive of drusen. OCT of the left eye [Figure 4] showed a large pigment epithelial detachment (PED) with a large zone of discontinuity of the RPE on the temporal side of the PED. Due to the discontinuity of the RPE temporally, the RPE on the nasal part of the PED appears lax with some infolding. Adjacent to the area of rip, there is some subretinal fluid. There is no evidence of intraretinal fluid or any focal thickening to suggest an associated choroidal neovascular membrane.

Figure 3

Fundus fluorescein angiogram of the left eye shows window defect in the area of absent retinal pigment epithelium. The curled retinal pigment epithelium medial to fovea shows block fluorescence. There was no choroidal neovascularization

Figure 4

Optical coherence tomography of the left eye shows curled torn retinal pigment epithelium, with bare choroid temporal to it

Discussion

RPE tear or rip is a known complication of fibrovascular and serous PED, usually associated with choroidal neovascularization (CNV) and subretinal bleed.[1] It occurs at the junction of attached and detached RPE either spontaneously or secondary to laser, photodynamic therapy, intravitreal anti-vascular endothelial growth factor agents, or steroid in wet-ARMD.[123] Tears may occur following development of a serous PED in the absence of CNV.[1] The tear may happen when the RPE cannot resist the stretch by sub-RPE fluid either from an occult CNV in a PED or from the contractile forces of the fibrovascular tissue intimately associated or entwined with the overlying RPE.[1] Following an RPE tear, the free edge of the RPE retracts and curls toward the mound of fibrovascular tissue. Old age with large irregular PEDs associated with CNV are at higher risk for this complication.[2] Visual prognosis of subfoveal RPE tear is poor.

Conclusion

RPE tear is an important complication of PED. Ruling out CNV is important in all such cases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.