Macular Telangiectasia - Signs and Symptoms

Signs and Symptoms

Patients with idiopathic juxtafovealretinal telangiectasia (IJRT) are typically 40 years of age or older. They may have a coincident history of ischemic vascular diseases such as diabetes or hypertension, but these do not appear to be causative factors.

IJRT may present with a wide range of visual impact, from totally asymptomatic to substantially impaired; in most cases however, patients retain functional acuity of 20/200 or better.

Metamorphopsia may be a subjective com-plaint. The key fundal findings in IJRT involve a focal area of diminished retinal transparency (i.e. graying) and/or small retinal hemorrhages just temporal to the fovea. Dilated capillaries may also be noted within this area, and while this is often difficult to visualize ophthalmo scopically, the abnormal capillary pattern is readily identifiable with fluorescein angiography.

Areas of focal RPE hyperplasia, i.e.pigment plaques, often develop in the paramacular region as aresponse to these abnormal vessels. Other signs of IJRT include right angle venules, representing an unusual alteration of the vasculaturein the paramacular area,with vesselstaking an abrupt turn toward themacula as if being dragged.

Also,retinal crystals—fine, refractile deposits in the superficial retinal layers—may be seen within the affected area.

In some forms of the disease,lipid exudates are present.

Although a variety of complex classification schemes are described in the literature, there are essentially two forms of idiopathic juxtafoveal retinal telangiectasia (IJRT): unilateral and bilateral. The unilateral form, described by Yanuzzi as Type 1 or aneurysmal telangiectasia,occurs almost exclusively in males and is asymptomatic until after age 40.

In this group, lipid exudate is common, while pigment plaques and retinal crystals are rare. The bilateral form, also known as Type 2 or perifoveal telangiectasis, occurs equally in males and females.

Type 2IJRT is typically discovered between the ages of 40 and 60 years,with a mean ageof 55–59 years.

In this form of the dis-ease, parafoveal atrophy, right angle venules, RPE pigment plaques and retinal crystals are the prevailing signs.