Spontaneous retinal pigment epithelial tear
Dr. Shashank Somani, Dr. Sarang Lambat, Dr Prabhat Nangia, Dr. Vinay Nangia
Suraj Eye Institute, 559, New Colony, Sadar, Nagpur- 440001.
A male, 74 years of age, presented with blurring of vision in left eye (LE) since 2 years. On examination his best corrected visual acuity (BCVA) was 6/18, N10 in right eye (RE) and 6/36p, N36 in the left eye (LE). His anterior segment examination was normal in both eyes with presence of immature cataract. His intraocular pressure (IOP) was 16 in both eyes.
A retinal pigment epithelial tear can occur due to various eye conditions. It occurs more commonly in a vascularised PED due to neovascular AMD. It can also arise after treatment of a choroidal neovascular membrane with laser photocoagulation, transpupillary thermotherapy or photodynamic therapy with verteporfin. The rate of spontaneous tear in a vascularized pigment epithelial detachment is reported to be between 10% and 12.5 %. With the use of anti-VEGF agents, the incidence of RPE tear is increasing and has been found to be as high as 33%. It can lead to a further decrease in visual acuity despite treatment and hence it is important to be able to identify an RPE tear when it occurs.
Size of the PED and basal diameter are recognized risk factors for the development of RPE tear. Studies have reported that the tear happens when the neovascular process is fresh and in one of the studies, it was found that there is an increased chance of tear formation if the duration of the PED is less than 4.5 months. Increased hydrostatic pressure and CNVM contraction under the RPE are the most acceptable mechanisms responsible for the formation of the RPE tears. Mouallem and Sarraf et. al postulated that a double RPE tear may occur on two opposite sides of a vascularised PED because of contractile forces by a choroidal neovascularization adherent to the posterior surface of the detached RPE. The CNV spans a larger area below the RPE and the hydrostatic pressure is not completely released with a single tear on one side. The edge opposite to the tear probably still has some susceptibility and it may get torn with further injection and subsequent CNV contraction.
Our patient presented with a significantly reduced vision of 6/36 in the left eye. The left eye fundus photograph (Fig 2) gave an impression of large areas of geographic atrophy with significant pigmentation in between the two main patches. The RE also showed a small patch of geographic atrophy associated with drusen and reticular pseudodrusen ( Fig 1, and Fig 3 a and b)
Initially, the appearance of the elevated area seen in Figure 4b (blue arrow) led to the suspicion that this was a partially active choroidal neovascular membrane. The patient underwent an anti-VEGF injection, however, there was no response.
The patient was diagnosed to have a spontaneous retinal pigment epithelial tear, which is known to occur in conditions that involve diseases affecting the retinal pigment epithelium. Initially, the two areas Fig 2 (pink arrow) and Fig 4a appeared to be patches of geographic atrophy. However, on further assessment, it appeared that these areas lacked retinal pigment epithelium. The retinal pigment epithelium had retracted inferiorly from the superior bare patch and superiorly from the inferior bare patch. ( fig 6a blue arrow and Fig 6b blue arrow). This resulted in the classic dividing band of retinal pigment epithelium in between the two bare patches ( Fig 6a blue arrow, Fig 2 yellow arrow) which consisted of rolled-up RPE. ( Fig 6b blue arrow). The Retinal pigment epithelium had also retracted temporally and appeared as a rolled-up pigmented edge ( Fig 2 and fig 7b blue arrow).
The RPE tear had a maximum width of 3738 microns (fig 7b). The Rolled up RPE when measured segmentally appeared to be 622 microns in length (Fig 7b) This indicates that the RPE not only retracts but may also have contractile properties that allow it to shrink in linear dimension besides folding up (Fig 7b).
Autofluorescence showed the presence of dark patches representing the areas devoid of the retinal pigment epithelium. The FFA shows the two areas of bare RPE lighting up like a flare. ( Fig 9b green arrow). The band of RPE in between and on the temporal sides showed blocked fluorescence (Fig 9b red arrow).
En-face imaging showed the presence of medium and large choroidal blood vessels in the area of RPE loss and a dark web-shaped patch of pigment (Fig 10a). On OCTA the choroidal Haller’s and Sattler’s layer was visible in the choroid in the area bare of RPE and also in the area nasal to it. However, choroid vessels were not imaged in the area where RPE had bunched up (Fig 10b) probably because of the nature of the RPE.
Changes of geographic atrophy with loss of outer retinal layers including the photoreceptors and outer nuclear layer with a downward movement of the outer plexiform layer are seen in our patient. There is also a loss of the outer plexiform layer (Fig 6b and Fig 7b). The presence of geographic atrophy in the RE is indicative that a similar process must have been set in the left eye. However, in the left eye, it was a precursor and may have contributed through a degenerative process to the retinal pigment epithelial tear.
The prognosis in these patients would be very guarded. The case is unusual because the retinal pigment epithelial tear occurred in the absence of classical predisposing factors and also because of the direction of RPE movement following the tear ( Fig 8 )
- Clemens CR, Eter N. Retinal Pigment Epithelium Tears: Risk Factors, Mechanism and Therapeutic Monitoring. Ophthalmologica. 2016;235(1):1-9. doi: 10.1159/000439445. Epub 2015 Oct 22. PMID: 26489018. DOI: 10.1159/000439445
- Ersoz MG, Karacorlu M, Arf S, Sayman Muslubas I, Hocaoglu M. Retinal pigment epithelium tears: Classification, pathogenesis, predictors, and management. Surv Ophthalmol. 2017 Jul-Aug;62(4):493-505. doi: 10.1016/j.survophthal.2017.03.004. Epub 2017 Mar 21. PMID: 28336128. DOI: 10.1016/j.survophthal.2017.03.004 .
- Sarraf D, Reddy S, Chiang A, Yu F, Jain A and al.A new grading system for retinal pigment epithelial tears. Retina. 2010 Jul- Aug;30(7):1039-45. doi: 10.1097/IAE.0b013e3181cdf366
Dr. Sarang Lambat
Suraj Eye Institute
Email – firstname.lastname@example.org
Q 1 – One or more of the following are true for retinal pigment epithelial tears.
- All patients with Choroidal neovascularization will get a retinal pigment epithelial tear
- Retinal pigment epithelial tears are often associated with intravitreal Anti VEGF injections
- Retinal pigment epithelial tears always heal by sliding and proliferation of the epithelium
- One can predict which patient with CNV will develop a RPE tear.
Q 2 – RPE tears may occur in one or more of the above.
- Central serous retinopathy
- Traumatic chorioretinopathy
- Glaucoma surgery
- High myopia
- Geograpic atrophy
- Toxemia of pregnancy