Case 25CMV Retinitis / HIV-AIDS
Clinical Presentation
A 36-year-old man complains of sudden, painless decreased vision in his right eye over 1 week. His medical history is significant for recurrent fevers and lymphadenopathy. The photograph illustrates the right eye.
Learner Questions
- Describe the ophthalmoscopic findings.
- What further history would you obtain?
- What is the differential diagnosis of this type of fundus lesion?
- How would you treat the patient?
Answer Framework
Findings DDx
'PIZZA PIE' / 'SCRAMBLED EGGS AND KETCHUP' retinopathy: yellow-white areas of retinal CMV retinitis (most common infectious retinitis in AIDS, CD4 <50 - pizza pie appearance). necrosis (full-thickness) + interspersed retinal hemorrhages. 'BRUSHFIRE' pattern - active Toxoplasma retinochoroiditis ('headlight in the fog' - fluffy focal lesion +/- adjacent old scar, white leading edge + healed, atrophic, granular retina trailing behind. Lesions advance significant vitritis). ARN (VZV/HSV, peripheral circumferential white necrosis, prominent along vessels. MINIMAL anterior chamber reaction (reflects immune suppression - vitritis, 66% RD risk). PORN (VZV in AIDS, minimal vitritis, rapidly progressive, worst hallmark of CMV retinitis). prognosis). Syphilitic retinitis (ALWAYS test). Candida chorioretinitis ('puff balls' in vitreous, IV drug users).
History Treatment
HIV status, CD4+ count (CMV retinitis almost exclusively at CD4 <50 cells/uL), viral load, ART 1. CMV: intravitreal ganciclovir or foscarnet (immediate high intraocular levels, sight- compliance, prior opportunistic infections (PCP, toxo, MAC), sexual history/IV drug use (HIV threatening lesions). Systemic valganciclovir (900 mg BID x 21 days induction -> 900 mg transmission), transplant recipient or chemotherapy (other immunosuppression), CMV daily maintenance - gold standard). 2. Treat underlying HIV: ART (MOST CRITICAL) - systemic symptoms (fever, diarrhea, esophagitis), duration/progression of visual restoring CD4 >100-150 leads to immune reconstitution -> CMV remission. Watch for IRIS symptoms. (immune reconstitution inflammatory syndrome - paradoxical worsening when starting ART; treat with steroids). 3. Monitor for RD (15-30%; treat with PPV + silicone oil).
Teaching Pearl
CMV retinitis = AIDS-defining illness; almost exclusively at CD4 <50. 'Pizza pie' fundus + brushfire advancing pattern in an immunocompromised patient = CMV retinitis until proven otherwise. Treatment: valganciclovir + ART (the game-changer). Always test for HIV in any atypical uveitis or retinitis in a young person. PORN (VZV in AIDS) = rapidly progressive outer retinal necrosis, minimal vitritis = worst prognosis variant.
Original answer transcript
Findings DDx
'PIZZA PIE' / 'SCRAMBLED EGGS AND KETCHUP' retinopathy: yellow-white areas of retinal CMV retinitis (most common infectious retinitis in AIDS, CD4 <50 - pizza pie appearance).
necrosis (full-thickness) + interspersed retinal hemorrhages. 'BRUSHFIRE' pattern - active Toxoplasma retinochoroiditis ('headlight in the fog' - fluffy focal lesion +/- adjacent old scar,
white leading edge + healed, atrophic, granular retina trailing behind. Lesions advance significant vitritis). ARN (VZV/HSV, peripheral circumferential white necrosis, prominent
along vessels. MINIMAL anterior chamber reaction (reflects immune suppression - vitritis, 66% RD risk). PORN (VZV in AIDS, minimal vitritis, rapidly progressive, worst
hallmark of CMV retinitis). prognosis). Syphilitic retinitis (ALWAYS test). Candida chorioretinitis ('puff balls' in vitreous,
IV drug users).
History Treatment
HIV status, CD4+ count (CMV retinitis almost exclusively at CD4 <50 cells/uL), viral load, ART 1. CMV: intravitreal ganciclovir or foscarnet (immediate high intraocular levels, sight-
compliance, prior opportunistic infections (PCP, toxo, MAC), sexual history/IV drug use (HIV threatening lesions). Systemic valganciclovir (900 mg BID x 21 days induction -> 900 mg
transmission), transplant recipient or chemotherapy (other immunosuppression), CMV daily maintenance - gold standard). 2. Treat underlying HIV: ART (MOST CRITICAL) -
systemic symptoms (fever, diarrhea, esophagitis), duration/progression of visual restoring CD4 >100-150 leads to immune reconstitution -> CMV remission. Watch for IRIS
symptoms. (immune reconstitution inflammatory syndrome - paradoxical worsening when starting
ART; treat with steroids). 3. Monitor for RD (15-30%; treat with PPV + silicone oil).
TEACHING PEARL
CMV retinitis = AIDS-defining illness; almost exclusively at CD4 <50. 'Pizza pie' fundus + brushfire advancing pattern in an immunocompromised patient = CMV retinitis
until proven otherwise. Treatment: valganciclovir + ART (the game-changer). Always test for HIV in any atypical uveitis or retinitis in a young person. PORN (VZV in AIDS) =
rapidly progressive outer retinal necrosis, minimal vitritis = worst prognosis variant.
