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Genetic and Rare Diseases Information Center (GARD)

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Idiopathic juxtafoveal retinal telangiectasia


Other Names for this Disease

  • Idiopathic juxtafoveal retinal telangiectasis
  • Idiopathic juxtafoveal telangiectasis
  • Idiopathic macular telangiectasia
  • IJT
  • Parafoveal telangiectasis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My girlfriend has this disease. Can you please provide some basic information on this disease including treatment and possible outcomes? How long does it take for eye sight to deteriorate?

Our Answer

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What is idiopathic juxtafoveal retinal telangiectasia?

Idiopathic juxtafoveal retinal telangiectasia (IJT) refers to a group of eye conditions characterized by dilated or twisting blood vessels (telangiectasia) and defective capillaries (tiny blood vessels) near the fovea in the retina. The fovea has the biggest number of special retinal nerve cells, called cones, which enable sharp, daytime vision. In IJT, the telangiectasias cause fluid or crystal buildup and swelling, impairing reflection of light. This results in progressive vision loss.[1][2][3][4] It may be congenital (present at birth) or can develop during the lifetime (acquired).[2] The different types of IJT are distinguished by their features and treatment options.[5] Laser photocoagulation maybe helpful in treating vision loss for individuals with certain types of IJT.[2]
Last updated: 2/10/2014

What are the signs and symptoms of idiopathic juxtafoveal retinal telangiectasia?

Signs and symptoms of idiopathic juxtafoveal retinal telangiectasia may include slow loss of vision, distorted vision, trouble reading, and scotomata (a spot in the visual field in which vision is absent or deficient).[1]
Last updated: 7/24/2012

When does eye sight typically deteriorate for individuals with idiopathic juxtafoveal retinal telangiectasia (IJT)?

Individuals with IJT may be divided into four categories based on a number of characteristics: groups 1A, 1B, 2, and 3.[6] The age of onset of symptoms, progression of the disease and potential for treatment differ amongst these categories.

In Group 1A disease, which typically occurs in men and affects only one eye, symptoms typically develop at an average age of 40 years. Treatment with photocoagulation of areas of leakage may help restore visual acuity for these individuals.[6]

Group 1B is usually found in middle-aged men who have blurring caused by a tiny area of capillary telangiectasia. Photocoagulation usually is not considered for these individuals because of the proximity of the leakage to the fovea and the good prognosis without treatment.[6]

Group 2 disease, which consists of bilateral, acquired, idiopathic parafoveal telangiectasia typically affects individuals in the fifth and sixth decades of life; mild blurring of vision occurs in one or both eyes. In these individuals, slow loss of visual acuity over many years is produced by atrophy (wasting away) of the central fovea; individuals also may develop choroidal neovascularization (creation of new blood vessels in the choroid), hemorrhagic macular detachment, and retinochoroidal anastomosis (reconnection of blood vessels). Photocoagulation may be of benefit, but in most cases, the abnormal lesions are so close to the fovea that treatment is problematical.[6]

Group 3 disease, bilateral idiopathic perifoveal telangiectasia with capillary occlusion, is a rare variant in which adults experience loss of vision because of progressive obliteration of the capillary network, which begins with telangiectasia.[6]
Last updated: 7/24/2012

What causes idiopathic juxtafoveal retinal telangiectasia?

The exact, underlying cause of idiopathic juxtafoveal retinal telangiectasia (IJT) is not known. IJT has been reported in some siblings (including twins) and other family members of affected people. This suggests there may be a genetic component to IJT; however, no specific gene has been proven to cause the condition.[5] Researchers have considered that changes in the ATM gene may interact with other genes or environmental factors to predispose a person to developing IJT.[7]

Some researchers have speculated that diabetes, or pre-diabetes, may be associated with some cases of IJT. [7][8] However, to our knowledge, this association has not been proven. Others have suggested there may be a developmental cause, such as abnormal formation of vessels in the eye, which could cause abnormalities of the vessels in adulthood.[7]

Certain types of IJT may occur in association with other conditions, including polycythemia (abnormal increase in blood volume), hypoglycemia, ulcerative colitis, multiple myeloma and chronic lymphatic leukemia.[2]
Last updated: 2/10/2014

How might idiopathic juxtafoveal retinal telangiectasia (IJT) be treated?

Laser photocoagulation of areas of leakage may be helpful in treating vision loss in people with certain subtypes of IJT, such as Group 1A.[2][6] A laser is a powerful beam of light which can be focused on the retina. Small "bursts" of the laser can be used to seal leaky blood vessels, destroy abnormal blood vessels, seal retinal tears, and destroy abnormal tissue in the back of the eye.[9] Photocoagulation usually is not considered for people with people in Group 1B because of the closeness of the leakage to the fovea, and the good prognosis without treatment. It may benefit people in Group 2 but in most cases, the abnormal lesions are so close to the fovea that treatment is difficult.[6]
Last updated: 2/10/2014

References
  • Schmitz-Valckenberg S, Fan K, Nugent A, Rubin GS, Peto T, Tufail A, Egan C, Bird AC, Fitzke FW. Correlation of functional impairment and morphological alterations in patients with group 2A idiopathic juxtafoveal retinal telangiectasia. Arch Ophthalmol. 2008;
  • Retinal vascular diseases. Digital reference of ophthalmology. http://dro.hs.columbia.edu/juxtafovtel.htm. Accessed 7/23/2012.
  • Wu J, Ferrucci S. Idiopathic juxtafoveal retinal telangiectasia. Optometry. 2008;
  • Do D, Haller JA. Coats disease and retinal telangiectasia. Yanoff & Duker: Ophthalmology 3rd ed. Philadelphia PA: Mosby Inc; 2008;
  • Nowilaty SR, Al-Shamsi HN, Al-Khars W. Idiopathic juxtafoveolar retinal telangiectasis: a current review. Middle East Afr J Ophthalmol. July, 2010; 17(3):224-241. Accessed 2/10/2014.
  • Yanoff & Duker. Ophthalmology, 3rd ed. USA: Mosby, An Imprint of Elsevier; 2008;
  • Watzke RC. et. al. Long-term juxtafoveal retinal telangiectasia. Retina. September, 2005; 25(6):727-735. Accessed 2/10/2014.
  • Tasca J, Ann Grogg J. Idiopathic juxtafoveolar retinal telangiectasia: a review and case report. Clin Eye Vis Care. March 1, 2000; 12(1-2):79-87. Accessed 2/10/2014.
  • Laser Photocoagulation. Retina Vitreous Center. 2010; http://www.retinavitreouscenter.com/procedures_laser_photocoagulation.html. Accessed 7/23/2012.
Other Names for this Disease
  • Idiopathic juxtafoveal retinal telangiectasis
  • Idiopathic juxtafoveal telangiectasis
  • Idiopathic macular telangiectasia
  • IJT
  • Parafoveal telangiectasis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.