Introduction
It is a well-recognized entity seen clinically and called a heterogeneous group of disorders. The juxta foveolar capillary networks of one or both eyes get affected by irregular dilatation of capillaries and differ in appearance, pathogenesis, and management strategies. The term “idiopathic juxta foveolar telangiectasia” was coined by Gass and Oyakawa in about 1982. They proposed the first classification for this clinical entity by dividing it into four groups largely based on the clinical and fluorescein angiographic (FA) features. The classification was further updated in 1993 by Gass and Blodi, which divided IJFT into three distinct categories, and each main group had an individual etiology. Despite the complexities this classification created, it is the most commonly used to date. This condition usually manifests around the fourth to sixth decade of life. Thus, IPT and IJFT apply only when the ectasia is limited to the perifoveal area.
Idiopathic perifoveal telangiectasia (IPT) is known by other terms such as:
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Idiopathic juxta foveolar retinal telangiectasia (IJFT).
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Idiopathic parafoveal telangiectasia.
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Macular telangiectasia.
What Is Peripapillary Telangiectasia?
Classically, three groups of IJFT are recognized. Group I is recognized in males and occurs unilaterally as visible telangiectasia; it causes visual loss due to macular edema. Group II occurs among middle-aged women and men, and telangiectasia is more difficult to detect, leading to vision loss. Group III is very rare, in which the perifoveal capillaries get progressively obliterated and are associated with a medical or neurological disease. IJFT is a descriptive term and represents the disease entities mentioned above.
How Is the Condition Classified?
Predominantly, the classification followed is that of Gass and Boldi. But very recently, a more straightforward classification has been adopted by Yannuzi et al., which serves as a revised and simplified version of Gass and Boldi’s. It proposed the terminology “idiopathic macular telangiectasia,” which has two distinct types: type I or idiopathic “aneurysmal telangiectasia.” This equates to IJFT, group I ( A and B combined). The type II is perifoveal telangiectasia, the most common type of IJFT. Those remaining types, as described by Gass and Boldi, were omitted from this particular classification as they are rare. But all these classifications concur with the already established classification of Boldi and Gass.
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Non-Proliferative Perifoveal Telangiectasia:
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Dilatation of right angle retinal venules.
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Visually, metamorphopsia and mild scotoma.
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Proliferative Perifoveal Telangiectasia:
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Neovascularization of subretina: It happens when new blood vessels are developed.
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Visual loss is quite rapid and severe.
What Causes Peripapillary Telangiectasia, and What Are the Presenting Features?
The cause could be either developmental, congenital, or idiopathic.
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Group I Visible and Exudative Idiopathic Juxta Foveolar Telangiectasia: It is the idiopathic macular telangiectasia type I or the aneurismal telangiectasia, which occurs more predominantly in males, with the mean age of presentation being 40 years. Usually, a two-disk diameter or greater temporal to the fovea region is involved. The characteristic feature is macular edema and lipid deposition, the main cause of vision loss.
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Group II Occult and Non-exudative Idiopathic Juxta Foveolar Telangiectasia:
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Acquired, not congenital.
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Older adults (mean age 55 years).
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Equal in males and females.
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Usually bilateral.
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Early stage, asymptomatic.
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Loss of vision in one eye.
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Loss of retinal transparency.
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In stage two, there is minimal disturbance of the central vision, such as blurring of vision, metamorphopsia, and paracentral positive scotoma.
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In stage III, there is a decrease in vision, which has a slow onset and progression.
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In stage IV, black retinal clumps and epithelial hyperplasia are noted around the para-foveolar right-angled vessel.
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In stage V, the remodeling of retinal capillaries takes place. The outer retina gets atrophied.
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At any stage from 2 to 5, a golden-colored crystal may be seen over the retinal surface, which lies anterior to the retinal vessel over the area of telangiectasia.
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Group III Occlusive Idiopathic Juxta Foveolar Retinal Telangiectasia:
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Rare form.
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Capillary telangiectasia.
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The loss of vision in association with familial and cerebral familial disease.
What Is the Differential Diagnosis?
IJFT Type I:
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Secondary Telangiectasia: Occurs in association with an already known disease.
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Dilated Perifoveal Capillaries: The dilatation of perifoveal capillaries situated in the eyes.
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Coats Disease: This disease is characterized by telangiectasia, exudation, and aneurysm.
Group IIA:
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Macular Hole: A rare eye condition that blurs the central vision.
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Retinal Crystals: Deposited crystals in any part of the retina.
How Is Peripapillary Telangiectasia Diagnosed?
Carefully examining the dilated retinal examination by the eye specialist makes the diagnosis. Optical coherence tomography may also help scan the retina and help visualize the retinal changes commonly seen in this condition, like abnormal fluids under or in the retina. To reveal abnormal or leaky vessels in the retina, fluorescein angiography is used in which a dye is injected into the vein, usually of the arm or hand, before taking the retinal photos and getting the dye circulated through the blood vessels.
What Are the Treatment Options Available?
The treatment options vary depending upon the types encountered:
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IJFT I: Intravitreal steroid injection, laser photocoagulation, and anti-vascular endothelial growth factors are given.
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Group IIA: Very recently, anti-VGEF (Vascular endothelial growth factor) injections, namely Bevacizumab, have been tried. It helps in improving the macular edema. Other modalities used include- ablation, verteporfin PDT, transpupillary thermotherapy, which uses low-watt, and surgery.
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Group III: Since it is very rare, it has been omitted.
Conclusion:
The three groups come under the umbrella of “idiopathic juxta foveolar telangiectasia” and have different appearances, management strategies, and appearances. Introducing newer treatment imaging modalities and functional tests would improve and solve this condition better. Additionally, more studies need to be incorporated, which can also enhance the understanding.
