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A 68-year-old Hispanic female first presented in April 2018 for a comprehensive eye exam.  She had a history of Type 2 diabetes for many years and hypertension (controlled on Metformin and Metoprolol). Her best-corrected visual acuity was 20/20 in each eye and her eye exam seemed to be unremarkable until on her dilated fundus exam in the right eye she was found to have a large “blot” hemorrhage temporally at 10:00. There were no other findings associated with the retinal hemorrhage. It was thought this was likely from an old retinal vein occlusion (RVO) that had resolved and the hemorrhage was what had remained. A 1-year follow-up was recommended.

She didn’t come in again until 2 ½ years later and the retinal hemorrhage was still present, but perhaps now even a little bit larger. There now was an area of whitening superior to the hemorrhage that was either exudate or dehemoglobinized blood. There was no fluid accumulation and no exudative retinal detachment.  

Clearly, this is not a simple retinal hemorrhage. If that were the case the hemorrhage would have resolved. Instead, this may be retinal neovascularization or even a small retinal angiomatous mass. If this is neovascularization, a resolved RVO was still high on the differential diagnosis. Other possibilities included a retinal angiomatous mass from a vasoproliverative tumor (VPT). A VPT is an uncommon benign vascular tumor that often occurs in healthy patients but can be associated with other chorioretinal conditions.  Usually, with VPT, a dilated afferent feeder vessel will be present with an efferent drainage vein coming out of the mass. That was not evident in this patient.  

The patient was referred and seen by a retinal specialist 3 months later (Figure 1) who agreed this was most likely a retinal hemorrhage from a resolved distal RVO and elected to see the patient again four months later. Four months later the hemorrhage was still present (Figure 2) with adjacent dehemoglobinized blood. Wide-field fluorescein angiography was performed (Figures 3-6).  

Figure 1: March 12, 2021, Initial visit with the retinal specialist. This was over 2 ½ years after initial exam.

 

Figure 2: 7/7/21; 3 months later and the peripheral lesion is still present. What does the whitening around the lesion represent? A wide-field fluorescein angiogram was performed on this visit; note the findings in the peripheral retinal.

 


Figure 3-5: Mid and late phase FA of the right eye. Note the capillary dropout that represents peripheral ischemia extending from 6:00 all the way up to 12:00.
Figure 6: Nasal retina in the right eye.

 

Figure 7-8: This is the FA in the LE – note the changes in the peripheral retina. This was a surprise.

The FA shows a focal area of hyperfluorescence that actually represents neovascularization. What’s surprising is the extremely large area of capillary nonperfusion that involves most of the peripheral retina beginning a 6:00 and extending up to 12:00. On the FA there is extensive “pruning” of the retinal vessels that stop at the junction of perfused and nonperfused retina. There are also several areas of focal leakage that may also represent small “buds” of neovascularization. In the nasal retina, there are even some peripheral vascular changes and ischemia. Even more surprisingly, the left also has areas of capillary nonperfusion with leakage of the retinal vessels temporally (Figures 7-8). 

Clearly, this is not a unilateral process. A resolved peripheral “ischemic” RVO involving both eyes is not likely. Other conditions that manifest bilaterally now need to be considered.  This looks suspicious for sickle cell retinopathy, though she is not African American, but she is Hispanic. This could also be a manifestation of her hypertension or diabetes. Blood work was recommended but she declined and careful follow-up was recommended.  

She presented again 9 months later on September 22, 2022, and not surprising the area of peripheral neovascularization in the right eye was still present (Figure 9) but is now even larger. There was associated hemorrhage but it did not extend beyond the area of neovascularization. There were some smaller sclerosed vessels adjacent to the lesion.  There were no other areas of neovascularization present. In the left eye temporally (Figure 10) there were several small focal areas of whitening that was present in both eyes that were originally thought to be drusen but in fact might represent old microvascular changes, perhaps resolved buds of NV. 

Figure 9: This is the right eye on 9/22/2022.
Figure 10: This is the left eye on 9/22/22

At this point, we still don’t know what she has. Systemic conditions such as Sickle Cell retinopathy or systemic vascular disease remain high on the differential diagnosis list.  

Does she need to be treated? She is not symptomatic and still enjoys 20/20 VA. We will continue to observe her. She will see the retinal specialist in a few months for follow-up.     

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