RETINA QUIZ

Is HIV to Blame for This Retinal Condition?

by Mark T. Dunbar, O.D

Multiple cotton wool spots and intraretinal hemorrhages are scattered throughout the posterior pole in both eyes.
A 36-year-old HIV-positive black male presented to the primary-care eye clinic. The primary-care physician at the community health center referred this patient to rule out CMV retinitis. He was first diagnosed with HIV five months prior when he was hospitalized with pneumonia.

The patient's current list of systemic medications included Viracept (nelfinavir mesylate), Bactrim (trimethoprim and sulfamethoxazole), Combivir (lamivudine and zidovudine), Viramune (nevirapine), ibuprofen and an iron supplement. He did not know his CD4 count or his viral load.

The patient also had hypertension, but was not taking any medications for this condition. His visual symptoms consisted only of blurred vision in both eyes for the past three months.

On examination best-corrected visual acuities were 20/20 in the right eye and 20/30 in the left. Confrontation fields were full to careful finger counting. However, in the right eye there was a small, localized area where the peripheral field was most blurry. Motilities were full, and there was no afferent pupillary defect. The anterior segment was completely normal. A mild anterior vitritis was present in both eyes.

The dilated fundus exam showed healthy optic nerves with good rim coloration and perfusion O.U. Scattered throughout the posterior pole in both eyes were numerous cotton-wool spots and intraretinal hemorrhages. Also, there was a small pre-retinal hemorrhage in each eye along the inferior arcades. The peripheral retina in both eyes appeared ischemic with loss of retinal transparency and sheathing of the retinal vessels. His blood pressure at the time of examination was 140/105.

    Quiz
     1. What are the pertinent findings in the fluorescein angiogram?
    a. NVE.
    b. Capillary nonperfusion.
    c. Macular edema.
    d. Necrotizing retinitis.

    2. What is the most likely diagnosis for this patient?
    a. CMV retinitis.
    b. HIV microangiography.
    c. Hypertensive retinopathy.
    d. HIV-related occlusive retinal vasculitis.

    3. How should this patient be managed?
    a. Blood workup.
    b. Panretinal photocoagulation.
    c. Antiviral therapy.
    d. Both a and b.

    4. What is the visual prognosis for this patient?
    a. Excellent.
    b. Fair.
    c. Poor.

Discussion
This patient most likely has HIV-related occlusive vasculitis, a recently reported condition.1 Daniel Roth, M.D., and and Cathleen McCabe, M.D., and Janet Davis, M.D., report two cases in which patients presented with extensive bilateral retinal ischemia in the presence of the human immunodeficiency virus (HIV). Both patients

What are the pertinent findings in this angiogram?
had extensive capillary nonperfusion leading to occlusion of the large retinal vessels and optic atrophy. The final visual acuity on one patient was hand motion O.U. and the other patient at three months was 5/200 O.D. and 20/400 O.S.

Our patient probably has an early form of this disease. There was massive capillary nonperfusion, but no large vessel occlusions at the time of presentation. We initiated an extensive laboratory workup to rule out other conditions such as diabetes, Behçet's disease, systemic lupus erythematosis, syphilis, TB, cardiolipin antibody syndrome, herpetic viral infection, Lyme disease and Bartonella henselae ("cat scratch" disease), among others. It is likely that the workup will fail to reveal a specific etiology since extensive laboratory investigation on the two published patients failed to reveal any causative factors besides HIV.

The cause of the condition remains unknown. HIV has been implicated as a causative factor in systemic thrombotic microangiopathy, a condition reported in HIV infected patients who present with thrombocytopenia and microangiopathic hemolytic anemia.2 Drs. Roth, McCabe and Davis postulate the remarkable selectivity for the retinal circulation in their patients suggests a local phenomenon akin to an infectious or inflammatory process.

If this patient does prove to have this condition, the visual prognosis is grave, based on the outcome of the two reported patients. With such a poor visual prognosis, it was decided that the patient would undergo panretinal photocoagulation in one eye and evaluate the response to the treatment. If he has a good outcome, then the other eye will also undergo laser treatment at a later time.

1. Roth DB, McCabe CM, Davis JL. HIV-related occlusive vasculitis. Arch Ophthalmol. 1999;117:696-698.
2. Hymes KB, Karpatkin S. Human immunodeficiency virus infection and thrombotic microangiopathy. Semin Hematol. 1997:34:117-125.

Answers: 1.b; 2.d; 3.d; 4.c
 

 

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