Tears of the retinal pigment epithelium (RPE) were first described in 1981 by Hoskin et al1 as a complication of detached pigment epithelium in patients with age-related macular degeneration. Since then, RPE tears have also been described in patients with chorioretinal scarring in retinal detachments, with subretinal neovascular membranes, following glaucoma surgery, and after laser photocoagulation of pigment epithelium detachments.2- 4 We describe a patient who developed an RPE tear following blunt trauma to the eye. Ultra-high resolution optical coherence tomography was performed, and it provided unprecedented visualization.
A 43-year-old woman reported falling and hitting her head and left eye on a wooden rail 1 week prior to her initial visit. After the swelling in her left eye subsided, she noticed decreased vision in that eye. At the time, the best-corrected visual acuities were 20/100 OD and 20/70 OS. Ocular history was significant for amblyopia in the right eye. Amsler grid testing of the left eye revealed areas of waviness and a scotoma in the center of the grid. Intraocular pressures were 15 mm Hg OU. Dilated fundus examination results of the right eye were normal whereas the left eye showed a well-demarcated area of RPE loss in the macula that was elevated with fluid. A scroll of pigmented RPE was noted infranasally (Figure 1A), and a horseshoe tear with surrounding subretinal fluid was noted superotemporally (located outside of the photographic field). Fluorescein angiography showed an early window defect from the lost RPE measuring several disc areas in size and involving the entire temporal and superior macula. A band of blocked fluorescence on the nasal margin was consistent with the scroll of RPE (arrow in Figure 1B). Ultra-high resolution optical coherence tomography was performed using a standard protocol. The horizontal temporonasal scan revealed a large area of subretinal fluid in the fovea. The area of RPE distortion in the nasal region (arrow in Figure 2) corresponds to the clinical finding of scrolled RPE as a result of retraction and folding of the RPE following the tear.
Following blunt trauma to the left eye, the patient underwent fundus photography and fluorescein angiography. A, Color fundus photograph of the left eye at the initial visit showing a well-demarcated hypopigmented area corresponding to loss of retinal pigment epithelium and exposed Bruch's membrane. A hyperpigmented strip of rolled-up pigmented retinal pigment epithelium was noted infranasally. B, Late-stage fluorescein angiography (59 seconds) of the left eye showing mottled hyperfluorescence in the shape of a large crescent involving the macula and extending temporally. A band of blocked fluorescence (arrow) on the nasal margin corresponded to the scroll of retinal pigment epithelium found on clinical examination.
A 6-mm horizontal temporonasal scan with ultra-high resolution optical coherence tomography was performed through the center of the fovea, showing a large accumulation of subretinal fluid. The arrow indicates the distorted retinal pigment epithelium in the nasal region. This is thought to be due to the retraction and folding inward of the retinal pigment epithelium following the tear.
Given the presence of the horseshoe tear with localized retinal detachment, the patient underwent laser treatment without any complications. She was informed that no successful therapy for RPE tears was available. Several options were discussed, including surgery to unroll the RPE and autologous iris pigment epithelial cell transplant. Surgery to drain the subretinal fluid was also considered.
Although the subretinal fluid had almost completely resolved spontaneously 5 weeks later, the patient perceived no improvement in vision. In fact, the best-corrected visual acuity had worsened to 20/300 OS. Ultra-high resolution optical coherence tomography revealed disruption of the inner and outer segments of the photoreceptors as well as thinning of the outer nuclear layer centrally. These findings may help explain the poor vision in this eye.
Retinal pigment epithelial tears are rarely caused by trauma. It is thought that the force applied must fit into an extremely narrow window. If the force is too strong, both the pigment epithelium and Bruch's membrane will be torn, and the commonly seen choroidal rupture will occur. If the force is too small, no tears will occur. Only if the force is strong enough to tear the pigment epithelium but not strong enough to tear Bruch's membrane will a traumatic RPE tear result.2,3 In experimental studies, Korte et al5 found that 1 week after an RPE tear, a layer of flattened, depigmented cells had replaced the area stripped of RPE. Although these cells may physically reconstitute the outer blood-retinal barrier, they may not be able to take the active role of normal, healthy RPE cells. Korte and colleagues have also shown that choriocapillaris death occurs as early as 11 weeks after RPE removal. Progressive deterioration in the visual acuity in our patient was noted as early as 8 weeks after the initial traumatic event, correlating well with experimental studies and other clinical reports.6
Correspondence: Dr Duker, Department of Ophthalmology, Tufts–New England Medical Center, New England Eye Center, 750 Washington St, Box 450, Boston, MA 02111-1533 (email@example.com).
Financial Disclosure: None.
Funding/Support: This work was supported in part by grants RO1-EY11289-16, RO13178, and P30-EY13078 from the National Institutes of Health, Bethesda, Md, ECS-0119452 from the National Science Foundation, Arlington, Va, F49620-98-1-0139 from the Air Force Office of Scientific Research, Arlington, and F49620-01-1-0186 from the Medical Free Electron Laser Program, Washington, DC, and by Carl Zeiss Meditec, Inc, Dublin, Calif.
Chan A, Duker JS, Ko TH, Schuman JS, Fujimoto JG. Ultra-High Resolution Optical Coherence Tomography of Retinal Pigment Epithelial Tear Following Blunt Trauma. Arch Ophthalmol. 2006;124(2):281-283. doi:10.1001/archopht.124.2.281