Case report first published in Reivew of Optometry, Vol. No: 140:06 Issue: 6/15/03
Retinal Telangiectasis In HIV-Positive Woman
Idiopathic, bilateral disease is typically found only in men. Fluorescein angiography helped differentiate the diagnosis in this patient.
Thomas J. Riley, O.D.
Presentation
A 53-year-old white female was referred so that we could rule out retinopathy secondary to or associated with HIV. She complained of fluctuating decreased vision in her right eye, and was concerned that she would not pass the vision exam for her driver’s license.
The patient’s ocular history was unremarkable. She was diagnosed with HIV eight years earlier. Her current viral load was undetectable, and her CD4 count was 300. Her medical history also was significant for recent onset of non-insulin dependent diabetes mellitus, which was stable, and depression. Family history was noncontributory.
She was taking Crixivan (indinavir, Merck) lamivudine, and Zerit (stavudine, Bristol-Myers Squibb) for her HIV; Humulin N (insulin NPH, Eli Lilly) and Novolin N (insulin NPH, Novo Nordisk) for her diabetes; and Zoloft (sertraline, Pfizer) for her depression. She reported no known allergies.
Diagnostic Data
Entering corrected distance visual acuity was 20/60 O.D. and 20/25 O.S. Pinhole showed an improvement to 20/40 O.D. and 20/20 O.S. Pupils were equal, round and reactive to light with no afferent pupillary defect. Amsler grid testing revealed metaphotopsia in the central and inferior 5-10 degrees O.D. Manifest refraction uncovered myopia with astigmatism, and presbyopia O.U., yielding best-corrected visual acuity of 20/40 O.D. and 20/20 O.S. All other visual findings were unremarkable.
Dilated fundus exam revealed 0.3-0.3 cup-to-disc ratios O.U. The macula of the right eye had mild to moderate edema, scattered microaneurysms and RPE changes superior to the fovea. All other findings were unremarkable.
We sent the patient for a retinal consult. Fluorescein angiography O.D. showed normal arterial filling with early punctate hyperfluorescence that appeared to be above the level of the pigment epithelium and deep to the major retinal vessels. The vessels diffusely increased in size and intensity, involving the fovea, through the late stages.
Diagnosis
This patient has group 1A unilateral, idiopathic, focal juxtafoveolar telangiectasis in the right eye.
Treatment and Follow-Up
We referred the patient for cranial computed tomography with and without contrast to rule out cerebral telangiectasis. The CT was negative for any abnormality.
Focal argon laser was contraindicated given the proximity of the telangiectasis to the avascular foveal region. Laser treatment would likely cause the patient to lose vision due to scarring.
We educated the patient about her condition and advised continued monitoring for any changes. We have seen her several times since this initial visit. Vision has remained 20/50 O.D., and the macula has not changed. We continue to see her every six months.
Diagnosis
This patient has group 1A unilateral, idiopathic, focal juxtafoveolar telangiectasis in the right eye.
Treatment and Follow-Up
We referred the patient for cranial computed tomography with and without contrast to rule out cerebral telangiectasis. The CT was negative for any abnormality.
Focal argon laser was contraindicated given the proximity of the telangiectasis to the avascular foveal region. Laser treatment would likely cause the patient to lose vision due to scarring.
We educated the patient about her condition and advised continued monitoring for any changes. We have seen her several times since this initial visit. Vision has remained 20/50 O.D., and the macula has not changed. We continue to see her every six months.
Discussion
The retinal telangiectasias are rare, idiopathic anomalies of the retinal vasculature. They are characterized by dilation and tortuosity of retinal vessels, formation of multiple aneurysms, varying degrees of leakage and deposition of lipid exudates.1 J. Donald Gass, M.D., used the term “idiopathic juxtafoveolar telangiectasis” in 1982 to describe a localized macular form of retinal telangiectasis.2 This condition falls into six subgroups (below).
The pathogenesis of juxtafoveolar telangiectasis is unknown.3 It may be due to chronic venous stasis caused by obstruction of the retinal veins as they cross retinal arteries on both sides of the horizontal raphe.4 Primary retinal telangiectasis is rarely associated with vascular anomalies elsewhere in the body, although congenital telangiectasis of the cerebral blood vessels is occasionally found.5
The primary condition is not associated with any systemic or ocular disease, and should be distinguished from secondary telangiectasis.1 Consider several pathological conditions in differentiating idiopathic and secondary retinal telangiectasis.2
Abnormal dilation of the parafoveal capillary network may be associated with ocular inflammatory conditions, diabetes, hypertension, carotid artery occlusion or retinal arterial macroaneurysm; it may also occur after retinal venous occlusion, or ocular or periocular radiation.6
Fluorescein angiography can help define this structural and permeability alteration in the affected vessels, and demonstrate the extent of the extravascular leakage of serous exudate into and beneath the retina.3 The angiography shows dilated and kinked microvascular channels, microaneurysms and capillary non-perfusion near the fovea.1 Dye leakage is largely confined to the dilated vessels. When intraretinal serous exudation is extensive, fluorescein stains the extravascular fluid pooled in the outer layers to produce a characteristic angiographic pattern of cystoid macular edema.5 Retinal function remains intact in an area of telangiectasis if the permeability of the retinal vessels is relatively normal.5
Laser photcoagulation may improve or stabilize visual acuity for central macular edema, particularly in unilateral cases.7 In bilateral cases, though, the minimal amount of exudation and confinement of the telangiectasis to within 1 disc diameter of the center of the foveola reduce the chances of a favorable outcome.3
Our patient’s unilateral Type 1A idiopathic juxtafoveolar retinal telangiectasis was somewhat unusual in that most patients who suffer from retinal telangiectasis are males, and most patients have the bilateral form. Fortunately, her vision has remained stable.
Dr. Riley is chief of optometry at the Veteran Affairs Medical Center in Spokane, Wash.
1. Kanski JJ. Clinical Ophthalmology: A Systemic Approach. 3rd ed. Oxford: Butterworth-Heinemann, 1994:377-8.
2. Terasvirta M, Tuovinen E. Idiopathic uniocular juxtafoveolar retinal telangiectasis in a female patient. Acta Ophthalmol Suppl 1985;173:60-1.
3. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 1982 May;100(5):769-80.
4. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update and classification and follow-up study. Ophthalmology 1993 Oct;100(10):1536-46.
5. Gass JD. Stereoscopic Atlas Of Macular Diseases Diagnosis and Treatment. 4th ed. St. Louis: Mosby-Year Book, 1997:494-512.
6. Isaacs TW, McAllister IL. Familial idiopathic juxtafoveolar retinal telangiectasis. Eye 1996;10(Pt 5):639-42.
7. Lowe MA, Akduman L, Olk RJ. Laser photocoagulation and glucose metabolism in idiopathic juxtafoveolar retinal telangiectasis. Ophthalmic Surg Lasers 1998 Feb;29(2):126-39.
8. Alexander LJ. Primary Care of the Posterior Segment. 2nd ed. Norwalk, Conn: Appleton and Lange, 1994:192-3.
