Case Reports
Case 1
A 32-year-old man presented following right eye trauma with a tennis ball. His vision was hand motions with accurate light projection. The patient had dispersed hyphema and the fundus details were hazy. Digital intraocular pressure (IOP) was high. The patient was prescribed topical lubricant drops and antiglaucoma medications along with 1 mg/kg of oral prednisolone. The next day, his best-corrected visual acuity (BCVA) improved to counting fingers at 1 m with normal IOP. Indirect ophthalmoscopy revealed a grayish elevation at the macula. SD-OCT raster scan with Optovue RTvue-100 (Optovue, Fremont, CA) showed subretinal elevation that was below the RPE and involved the foveal center (Fig. 1). Because the hemorrhage was sub-RPE, we decided to observe the patient. Three days later, BCVA improved to 20/200, hyphema had reduced, and the subretinal hemorrhage was well delineated. A 512 × 128 macular cube scan (Cirrus HD-OCT model 4000; Carl Zeiss Meditec, Dublin, CA) was performed. Advanced analysis using a ray-traced three-dimensional rendering system showed an elevation of the RPE layer with a corresponding elevation of the internal limiting membrane (ILM), whereas the ILM–RPE thickness was normal, indicating that the hemorrhage was below the RPE (Fig. 2). Sequential three-dimensional segmentation maps and high-definition raster scans clearly showed the reducing height of the sub-RPE bleed, which correlated with improving vision (Figs. 3 and 4). On follow-up his visual acuity improved to 20/120 at 7 days, 20/60 at 17 days, and 20/40 at 35 days after injury. Subretinal hemorrhage resolved after 3 weeks, revealing the choroidal rupture. At 3 months, his BCVA was 20/25 with normal IOP without any medication.
Case 2
A 16-year-old boy presented for a second opinion 7 days after left eye trauma with a tennis ball. On initial presentation to another center, his visual acuity was noted to be counting fingers and he was noted to have iritis with Berlin’s edema. The patient was prescribed oral steroids (40 mg daily) with a topical antibiotic steroid eye drop combination administered every 5 hours. The patient sought a second opinion because there was no improvement in visual acuity. On examination, his vision was counting fingers close to face and his IOP was 29 mm Hg. Anterior chamber showed 2+ cells, whereas fundus revealed a reddish elevation at the macula involving the fovea that was suggestive of subretinal hemorrhage (Fig. 5). On three-dimensional segmentation analysis of 512 × 128 macular cube scan, an elevation of the ILM with a flat RPE layer was noted (Fig. 5). ILM–RPE thickness was increased, indicating the blood was between the neurosensory retina and the RPE layer (Fig. 5). The patient underwent 0.5-cc intravitreal SF6 gas injection with prone positioning. Serial three-dimensional OCT imaging at 6 hours, 36 hours, 7 days, and 12 days after injection revealed displacement of the bleed, evidenced by shifting of the elevation and subsequent flattening (Figs. 6 and 7). Fundus examination 1 week after injection revealed choroidal rupture. The site of the choroidal rupture corresponded to the peripapillary RPE elevation seen on the RPE segmentation map (Fig. 5). Three months after pneumatic displacement, his BCVA was 20/50.
Discussion
Submacular hemorrhage is a condition that threatens vision. Natural history and visual prognosis depend on the cause of hemorrhage, the duration of submacular hemorrhage prior to treatment, and the location and thickness of the subretinal bleed.
In a retrospective review of natural history and visual prognosis in 29 eyes with subretinal hemorrhage, Bennett et al.1 reported a mean final BCVA of 20/480 with an average 3-year follow-up. Eyes with age-related macular degeneration had a poor final BCVA of 20/1700, whereas eyes with subretinal hemorrhage from trauma-induced choroidal rupture had a better BCVA of 20/35.
The pretreatment duration of the subretinal hemorrhage correlates inversely with final visual acuity. In an interventional study, Hattenbach et al.2 found that final visual acuity was better in eyes with subretinal hemorrhage of less than 2 weeks’ duration before treatment. Similar findings were reported by Lewis3 after surgical evacuation and the final BCVA was worse in patients with hemorrhage lasting longer than 7 days. Toxicity due to iron could be partially responsible for retinal damage apart from trauma itself, as shown in experimental studies.4
Prognosis also depends on the thickness and location of the bleed. Increased thickness of subretinal hemorrhage has been correlated with poorer final visual acuity.1,5,6 This is because a larger separation between the photoreceptors and the RPE correlates with a greater diffusion barrier, therefore resulting in a higher chance of permanent photoreceptor damage. The larger the separation between the photoreceptors and the RPE, the greater the diffusion barrier will be, resulting in a higher chance of permanent photoreceptor damage.
Although studies have looked at both the location (foveal involvement) and the clinical assessment of hemorrhage thickness to prognosticate outcome, the exact anatomical location has not been investigated directly with ancillary testing. Submacular hemorrhage may be present between the photoreceptors and RPE or below the RPE. Clinical examination or fluorescein angiography may not differentiate the type of submacular hemorrhage with certainty. In our cases, we were able to accurately diagnose the exact anatomical location of the subretinal bleed using three-dimensional segmentation analysis of Cirrus HD-OCT. This differentiation is important because both types of hemorrhage may significantly reduce vision; however, prognosis and approach to their management would differ. In patients with hemorrhage between the neurosensory retina and RPE, immediate management, including either surgical evacuation or gas injection to displace the blood away from the fovea, needs to be initiated. In patients with a sub-RPE bleed, it may be difficult to displace the blood because it is a closed space. Furthermore, sub-RPE blood cannot be removed with surgery without creating iatrogenic damage to the RPE with resultant poor vision. Hence, observation would be prudent in such cases.
Both our patients had similar clinical presentation. The maximum foveal elevation on high-definition raster scan was higher in the first case (353 microns) compared with the second case (104 microns), and should therefore have had a poorer prognosis without therapeutic measures to displace the blood. However, the visual recovery started as early as 3 days after injury and continued to improve, which correlated with a decrease in the thickness of the sub-RPE bleed. Chaudhry et al.7 reported a similar case of spontaneous resolution of large traumatic submacular hemorrhage. The case reported by Chaudhry et al.7 and our first case defy our logic based on animal studies4 that have revealed irreversible iron-related damage happening within 24 hours of the onset of submacular hemorrhage. One possible explanation is that the photoreceptors were not directly exposed to the iron-induced damage because the blood was sub-RPE.
To the best of our knowledge, use of SD-OCT with automatic three-dimensional segmentation analysis to differentiate submacular hemorrhage types and its serial use to objectively monitor hemorrhage resolution following observation/gas injection has not yet been reported. Apart from establishing the anatomical location of the bleed, utility of an RPE-fitted slab to visualize retinochoroidal interface was demonstrated. This feature allowed en face visualization (C-scan) of the choroidal rupture in both cases that was hidden to clinical evaluation due to hemorrhage (Fig. 8A). C-scan imaging of the outer segment/inner segment junction was also done at the final visit, which revealed reduced density of photoreceptors in the area immediately above the hemorrhage. C-scan of the RPE and sub-RPE level revealed a relatively intact RPE with altered sub-RPE in case 1, whereas the RPE uniformity was altered but the sub-RPE appeared intact in the second case (Figs. 8B, 8C, and 8D).
OCT was also used to guide the duration of face-down positioning in the second case. Prone positioning was maintained for 15 days until the subretinal elevation was nearly flat on OCT.
Based on our experience in these two cases, we infer that SD-OCT has an important role in managing submacular hemorrhage in patients. Combined with the ability for automatic segmentation, SD-OCT clearly improves the clinical evaluation of chorioretinal disease by adding relevant new parameters to diagnose the exact anatomical location of lesions and to monitor the patient’s progress toward treatment. Further experience with a larger number of patients is desirable.