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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
  • IJT
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Overview



What is idiopathic juxtafoveal retinal telangiectasia?

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

What causes idiopathic juxtafoveal retinal telangiectasia?

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


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

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.[6]

Some researchers have speculated that diabetes, or pre-diabetes, may be associated with some cases of IJT. [6][7] 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.[6]

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][8] 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.[8]
Last updated: 2/10/2014

References
  1. 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;
  2. Retinal vascular diseases. Digital reference of ophthalmology. http://dro.hs.columbia.edu/juxtafovtel.htm. Accessed 7/23/2012.
  3. Wu J, Ferrucci S. Idiopathic juxtafoveal retinal telangiectasia. Optometry. 2008;
  4. Do D, Haller JA. Coats disease and retinal telangiectasia. Yanoff & Duker: Ophthalmology 3rd ed. Philadelphia PA: Mosby Inc; 2008;
  5. 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.
  6. Watzke RC. et. al. Long-term juxtafoveal retinal telangiectasia. Retina. September, 2005; 25(6):727-735. Accessed 2/10/2014.
  7. 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.
  8. Yanoff & Duker. Ophthalmology, 3rd ed. USA: Mosby, An Imprint of Elsevier; 2008;
  9. Laser Photocoagulation. Retina Vitreous Center. 2010; http://www.retinavitreouscenter.com/procedures_laser_photocoagulation.html. Accessed 7/23/2012.