A 78-year-old, white male presented with hazy vision and symptoms of floaters in his right eye for the past month. He denied any pain or discomfort. He had been followed on an annual basis for several years with intermediate stage, dry macular degeneration. His last visit was 7 months earlier at which time he was 20/20 in each eye despite incipient cataracts and macular drusen.
On examination, his best corrected visual acuity was 20/20 in each eye. Extraocular motility was full and cover test was ortho. Confrontation visual fields were full to careful finger counting OU and the pupils were equally round and reactive; there was no APD.
His anterior segment exam was significant for early NS and cortical cataracts OU. The anterior chamber was deep and quiet without cell or flare.
On dilated fundus exam, the anterior vitreous was clear OU. In the temporal retina of the right eye a large circular lesion beyond the arcade was clearly visible. It had granular, feathery borders with scattered areas of hemorrhage within the lesion. There was some vitreous haze overlying. The left eye was completely normal with the exception of intermediate size macular drusen.
Categorically, this appeared to be an infectious process, either a retinitis or possibly a retinochoroiditis, but the absence of vitreous cells doesn’t really fit what you usually see with an infectious process. The most common infectious process involving the retina and choroid is Toxoplasmosis but with a clear vitreous and no “headlights in the fog,” appearance, Toxoplasmosis was not likely. So what’s going on with our patient? Might this represent some other infectious process?
The answer, of course, lies in the history. There were a few key facts that were purposely omitted; the main one being that our patient has been HIV+ since 1985. He has been on various HAART (highly active antiretroviral therapy) treatment regiments over the years until he became completely resistant to HART therapy at which time his CD4 counts slowly declined and his viral load increased. By December 2020 his CD4 count had dropped to 63 with viral load being 89800 copies. It continued to declined and by December 2021, the CD4 was 21 and the viral load was 410,000. A new treatment regimen was started in January 2022 but it didn’t help and by Feb the CD4 count dropped to 10.
As the CD4 count declined he started to develop various opportunistic infections such as oral thrush and by June 2022 he started to notice an increase in floaters which was when CMV retinitis in the right eye was diagnosed. Even though it was not sight threatening, treatment was recommend. He was started on oral Valgancyclovir 900 mg twice daily. If no improvement by six weeks, then intravitreal injections would be considered.
In the early days of the AIDS epidemic, CMV retinitis was very common but in the era of HAART therapy it is extremely rare. When it is seen, it's usually in patients that are not on HAART therapy and are severely immunocompromised. Often these are socioeconomically compromised individuals. Fortunately today, the vast majority of HIV+ patients receive HAART therapy and are able to live healthy and productive lives.
Our patient is unique in that he has been HIV+ for over 35 years and has lived a long productive live. Unfortunately now he has become pandrug resistant, and as a result is slowly wasting away and starting to develop opportunistic infections.
CMV retinitis is the most common ocular complication seen in AIDS patients and may develop anywhere in the retina. Posterior pole lesions have a characteristic white, hemorrhagic appearance with retinal necrosis and edema, while the peripheral lesions are more indolent and nonhemorrhagic as is seen in our patient. In addition, vitreous cells may be present but because these patients are so immunocompromised their immune system is not strong enough to mount an immune response, thus a minimal vitritis. Our patient did in fact have a few vitreous cells when we went back and looked more carefully, which probably accounts for the floaters.
CMV retinitis develops in patients whose CD4+ lymphocyte T-Cell (CD4) counts are below 50, and usually even lower than that. In the early days of the AIDS epidemic, CMV was common, developing in up to 40% in AIDS patients. With the advent of HAART therapy, CMV has virtually disappeared, as there has been a dramatic 55-95% decline in the number of CMV retinitis cases1.
Our patient was treated on the standard protocol of ValGanciclovir (a prodrug of ganciclovir) 900 mg twice daily. He was seen one month later with significant improvement of the CMV retinitis. He continued to improve until there was complete resolution. One year later, the patient is still alive and maintaining excellent visual function.
1 Jabs DA, Van Natta ML et al. Characteristics of patients with cytomegalovirus retinitis in the era of highly active antiretroviral therapy. Am J Ophthalmol 2002;133:48-61