Coats’ disease is a rare eye condition in which abnormal blood vessels form and grow behind the retina (the light-capturing membrane at the back of the eye). These abnormal vessels have weak walls and leak fluid into the back of the eye. Over time, the fluid accumulates, pushing on the retina and causing it to swell. The retina may start to detach, causing a partial loss of vision. If the retina completely detaches, vision may be completely lost in the affected eye.
Coats’ disease is found more commonly in males than in females. Symptoms can begin in children as young as 12 months. If the condition is found and treated before the vessels have leaked a significant amount of fluid, there is a good chance much of the vision can be restored. Once the retina has completely detached, the likelihood of vision restoration is very low. Glaucoma can develop, leading to a significant amount of eye pain and the need to remove the damaged eye (enucleation).
Retinoblastoma is a rare type of eye cancer. Though it is the most common type of ocular tumor in children, the American Cancer Society estimates only about 300 cases occur annually in the U.S. The cancer is most common in infants and young children, with an average age at diagnosis of two. Boys and girls are affected equally.
Researchers have determined that retinoblastoma is caused by a mutation in a gene called RB1, causing some of the fetal cells that form the retina to grow rapidly out of control. In about 25 percent of cases, this gene mutation is inherited from one of the parents. In these children, both eyes are usually affected. In the remaining 75 percent of cases, the BR2 gene mutation occurs sporadically. For these cases, only one eye is usually affected.
The danger with retinoblastoma is two-fold. First, the tumor can cause vision problems, eye pain, and, eventually, loss of vision. Second, the cancer can spread beyond the eye to the eye socket and then to rest of the body.
“Eye Glow” Warning
The sooner Coats’ disease, retinoblastoma and other potentially serious eye conditions can be detected, the greater the likelihood of preventing blindness, loss of the eye or death. One way to spot a possible problem is with a flash camera.
Thomas Lee, M.D., Retina Specialist with Children’s Hospital Los Angeles, says sometimes parents notice “red eye” in a flash photo of a child. That occurs because in a dark setting, the pupils of the eye dilate (open). When the camera flash goes off, the light enters the eye and reflects off the retina, causing the appearance of the red eyes. This is a normal phenomenon. On the other hand, a yellow or whitish glow in the eyes is not normal. These colors may be a reflection of some kind of abnormality in the eye. Lee says, if a child’s picture has an abnormal or unusual eye glow, an eye exam is highly recommended.
The “Know the Glow” campaign aims to spread the word about using flash photography to detect possible eye problems in children. In addition to Coats’ disease and retinoblastoma, an abnormal eye glow can be an indication of 14 other conditions, such as amblyopia, cataract, refractive errors (like nearsightedness and farsightedness), retinopathy and eye trauma. To access information about the “Know the Glow” campaign, go to http://www.knowtheglow.org.
For information on Coats’ Disease, go to http://www.coatsdisease.org
For information on retinoblastoma:
American Cancer Society, http://www.cancer.org
Eye Cancer Network, http://www.eyecancer.com
National Cancer Institute, http://www.cancer.gov
National Eye Institute, http://www.nei.nih.gov
Arean, Carolina, M.D., et al., “Expression of Vascular Endothelial Growth Factor in Retinoblastoma,” Archives of Ophthalmology, February 2010, Vol. 128, No. 2, pp. 223-229.
Gilles, Mark. Ph.D., et al., “Familial Asymptomatic Macular Telangiectasia Type 2,” Ophthalmology, December 2009, Vol. 116, No. 12, pp. 2422-2429.
Shields, Carol, M.D., et al., “Retinoblastoma Regression Patterns Following Chemoreduction and Adjuvant Therapy in 557 Tumors,” Archives of Ophthalmology, March 2009, Vol. 127, No. 3, pp. 282-290.
Weiss, Avery, M.D., et al., “Retinal Function and Corresponding Pathology in Advanced Retinoblastoma,” Archives of Ophthalmology, November 2008, Vol. 126, No. 11, pp. 1507-1512.