Idiopathic Juxtafoveolar Retinal Telangiectasia (JXT)

Patient was a 53 yo, white female returning for a routine exam. She has a positive family history of macular degeneration. Her BCVA was 20/20 OU and amsler grid was WNL OU; we did find a small aneurysm on the temporal edge of the avascular zone of the fovea. Based on this finding, we diagnosed our pt with juxtafoveolar retinal telangiectasia (JXT). She will rtc in 6 mo. for a follow-up, and she was advised to complete the amsler grid once a week.

When I looked up JXT, I found info about other retinal telangiectasias, so I thought I would briefly describe the other ones too. Retinal telangiectasias are a group of rare, idiopathic, congenital retinal vascular anomalies affecting the capillary bed. The conditions are characterized by dilation and tortuosity of retinal vessels and formation of multiple aneurysms, with different degrees of leakage and lipid exudates. Idiopathic JXT, Lebers Miliary Aneurysms, and Coats' Disease are three types of retinal telangiectasias.

Idiopathic Juxtafoveolar Retinal Telangiectasia (JXT) JXT is commonly characterized by slow vision loss beginning in adulthood. Dilated and kinked vessels, along with microaneurysms, in the parafoveal capillary network are the hallmark of the condition. Current convention divides patients into one of three sub-categories of disease. In group 1 the telangiectasis appears to be caused by retinal capillary leakage. In group 2 the telangiectasis is most likely caused by capillary diffusion abnormalities. Capillary occlusion is suspected of causing the telangiectasis in group 3 patients. Group 1 patients are primarily male. The telangiectasia is unilateral in most cases and easily visible. It is suspected this form of JXT may be a mild presentation of Coats disease. Group 2 patients are characterized by retinal juxtafoveolar telangiectasia, minimal exudation, superficial retinal crystalline deposits, and right-angle venules. As the disease progresses, intraretinal pigment plaques and subretinal neovascularization develop. Group 3 patients are diagnosed with bilateral, easily visible telangiectasia, minimal exudation, and capillary occlusion. Gass and Blodi (1993) postulate this category of disease to be related to systemic conditions, such as gouty arthritis or hypoglycemia.

The DDx for idiopathic JXT includes retinal vascular disorders (such as BRVO), diabetic retinopathy, sickle-cell retinopathy, Eales' Disease, and retinopathy of prematurity. Based on the information available, I suspect may pt falls into group 2 because her case is not bilateral (group 3) nor easily visible (group 1). Lebers Miliary Aneurysms This condition represents a more severe form of telangiectasia. Patients often present in middle-age with a drop in VA. Fundus findings include lipid deposition at the macula and dilation of arterioles and venules.

Cryotherapy or photocoagulation may be needed to destroy the area of vascular abnormality. Coats' Disease This is a very severe, unilateral form of retinal telangiectasia that is most often seen in boys before age 10. Patients often present with strabismus or leukocoria; the DDx includes retinoblastoma and retinopathy of prematurity. The disease is characterized by large areas of dilated, tortuous retinal blood vessels; these vessels overlie intra- and sub-retinal yellow exudate at the posterior pole and in the periphery. Spontaneous resolution is rare; the disease often progresses to massive sub-retinal exudation, exudative retinal detachment, rubeosis iridis, secondary cataract and secondary glaucoma.

Tx includes cryotherapy and photocoagulation to prevent retinal detachment.

Sources Gass, J. D. M., and B. A. Blodi. Idiopathic Juxtafoveolar Retinal Telangiectasis. Ophthalmology 1993:100:1536-1546. Kanski, J.J. 1997. Clinical Ophthalmology. Oxford: Butterworth-Heinemann.