<b>Coates disease</b>
Coates (Coat's) disease
DATE June 14, 2002
Updated: July 16, 2008. See "NEW COATES DISEASE INFORMATION" New!!!! Links added below.
Coats' (Coate's) disease, first described by Dr George Coats in 1908, is an idiopathic eye inflammation usually starting in pediatric patients, which may lead to retinal detachment. This disease remains very poorly understood and the cause of the condition is unknown. Coats' Disease is a peculiar condition of a vascular malformation of the arteries and veins of the retina of the eye. It has been explained as the failure of the blood vessels to form before birth - causes fluid (blood) build-up behind the eye, which in turn causes a build-up or scarring. When the fluid levels are high - the vision is worse. It is not associated with any other type of vascular problems in the body.
Coats' disease is not hereditary.
It is 95% unilateral (in most cases only involves one eye.) This is a rare condition in which the blood vessels inside the eye develop leaks; and is more common in boys than girls. Symptoms generally begin in the first decade of life, sometimes as early as 4-6 months. This is very destructive to the retina (it's like having soap poured on your retina). Because it often affects only one eye, children frequently don't notice any problem because they can see fine with their other eye. Sometimes the first indication that there is a problem is that the pupil turns white from the massive swelling and detachment of the retina. In children with the disease it appears to have quite a rampant course, typically causing severe visual loss. It does also appear in adults but with usually a less severe outcome, again with a male preponderance.
Retinal detachment is not uncommon as a complication of Coats' disease, and retinal detachment is quite rare in young children. It has an increasing breakdown of the blood-retinal barrier, with increasing degeneration of the vessel walls. It will lead to complete retinal detachment and secondary glaucoma. Progressive massive exudation may lead to complicated glaucoma and loss of the eye in advanced cases.
Coats' disease can be treated with various types of eye surgery including laser surgery for cases that are caught early enough. Worse cases need surgery to drain the leaking fluid, seal the blood vessels, and reattach the retina. Also, leaks under the retina cannot be repaired without serious damage to the person's sight. Most patients with this disease end up legally blind in the affected eye. Coats' disease is sometimes very difficult to distinguish from a malignant tumour of the eye called retinoblastoma. If the problem is so severe that it is felt that the eye will never see again, it is sometimes suggested to remove the entire eye to be sure that there is not really retinoblastoma. Treatment in children is usually extremely difficult and mostly unrewarding. The problem being, exudation into and underneath the retina, tending to progress despite various interventions such as laser, cryotherapy and other surgical procedures. In adults the condition is more amenable to laser fortunately.
Coate's Disease and employment :
Coats' Disease is a very unforgiving and severe disease but let me assure that, although your vision may be limited, you career prospects are not. With technology, people with vision loss can aid their remaining vision or substitute the vision they have lost already. For example, someone with limited vision may use a screen magnifier to enlarge the text on the computer monitor, or someone who is totally blind may use speech synthesis to have the computer speak what is on the screen to him or her. For persons who do not work on computers, other accommodations can be made, for example a visually impaired plumber would enlarge his drawings to see blueprints, a salesperson who travels would use public transit and make a schedule so that on particular days, his/her client visits are bunched together in the same area or allow for travel time from place to place. Someone in retail may use a hand magnifier to read labels or price tags or trade duties with a co-worker (i.e. "I'll answer phones for you over lunch break if you could do this certain task For me since I have trouble seeing")
Today more than ever employers are very aware of the skills and strengths that visually impaired and blind persons can bring to their organization. Many employers are looking to diversify and make their workplace more inclusive. The CNIB conducts awareness sessions for employers to teach them what blindness is, and what it isn't. There are also great opportunities for persons looking to return to school. Colleges and universities now have advisors and student service staff who specialize in assisting persons with disabilities. They also allow for accommodations for testing and exams (i.e. longer time allotted for exams when a person is visually impaired or blind) The possibilities are plentiful. The Federal Government is also looking for persons with disabilities to fill numerous positions.
It's never easy to find a good job and it's not easy to find the right "fit" when you have a vision impairment. It may take a little longer to find that "dream job" but people are doing it everyday. Remember, the only person who can limit you is yourself. Being assertive and having the knowledge to go along with it will take you a long way.
"My life with Coates Disease"
From the CNIB Library (The Canadian National Institute for the Blind)
July 20, 1990 and other sources.
Michael Hayden (2002)
Employment Accommodation Consultant, Ontario & Quebec
CNIB National Office
Tel: 416-480-7431
Fax: 416-480-7677
http://cnib.workopolis.com
NEW COATES DISEASE INFORMATION
As of 02/25-2003
Healthwise © This information is not intended to replace the advice of a Doctor.
Healthwise disclaims any liability for the decisions you make based on this information.
http://www.healthwise.net
This is the moist current information that NORD (National Organization for Rare Disorders, Inc.)
has available at this time.
***Alternate names for Coats' Disease: Coats Syndrome and Retinal Telangiectasis.
Related Disorders: Retinoblastoma, Norrie Disease & Persistent Hyperplastic Primary Vitreous (PHPV)
SYMPTOMS:
The specific symptoms present in each case of Coates Disease depend on the number and size of the blood vessels involved. Some affected individuals may display few or no symptoms (asymptomatic), while others may have a more severe form of the disorder.
The most common symptoms associated with Coats disease, include decreased clearness of clarity of vision (visual acuity), crossed eyes, (strabismus) and / or the development of a white mass in the pupil area behind the lenses of the eyes so that the pupil appears white (leukokoria of "cat's eye" reflex).
Additional symptoms that may occur as Coats disease progresses include elevated fluid pressure within the eye (glaucoma), clouding of the lens of the eyes (cataract), reddish discoloration in the iris due to the growth of new blood vessels in the iris (rubeosis iridis), shrinking of the affected eyeball (phthisis bulbi), and / or inflammation of the membrane covering most of the back of the eyeball (uveitis).
Symptoms may develop due to a developmental malformation, known as Telangiectasia, of blood vessels of the nerve-rich membrane lining the eyes (retina). Telangiectasia occurs when there is abnormal widening (dilation) of groups of small blood vessels, often resulting in the leakage of various substances (e.g., fluids or cells) from the affected blood vessels. When this occurs in the retina, it is termed Exudative Retinopathy.
Causes:
The exact cause of Coats disease is not known. Some cases of Coats disease have been associated with a mutation of the Norrie disease gene located on the short arm (p) of the X chromosome (Xp11.4).
Some researchers believe that Coats disease represents a spectrum of disorders instead of one distinct disorder. This disease spectrum may be subtypes of Idiopathic Retinal Telangiectasia with exudation.
Related Disorders:
Symptoms of the following disorders can be similar to those of Coats disease. Comparisons may be useful for a differential diagnosis.
Retinoblastoma is an extremely rare malignant tumour that develops in the nerve-rich layers of one eye or both eyes (retinas), most commonly in children under the age of three years. The most typical finding associated with Retinoblastoma is the appearance of a distinctive white mass in the pupil area behind the lens of the eye, the so called "cat's eye reflex" (leukokoria). In addition, the eyes may appear crossed (strabismus). In some affected children, the eye(s) may become red and/or painful. The presence of the tumour may cause a rise in the pressure in the eyeball (glaucoma). In most cases, Retinoblastomas occur spontaneously for no apparent reason (sporadic); however, approximately 20 percent of cases are transmitted as an autosomal dominant trait.
Norrie Disease is a rare inherited neurodevelopmental disorder characterized by blindness in both eyes. (Bilateral) at birth (congenital). In some cases, children with this disorder may also experience varying degrees of mental retardation. Additional symptoms associated with Norrie disease may include mild to profound hearing loss and additional eye abnormalities. The lenses of the eyes may shrink (phthisis bulbi). Norrie disease is inherited as an X-linked recessive trait.
Persistent Hyperplastic Primary Vitreous (PHPV): is a developmental disorder affecting the eye that is present at birth (Congenital). The disorder is characterized by abnormalities of certain eye structures and loss of vision. Specific symptoms include abnormally small eyes (microphthalmia), cataracts, and/or the formation of a white membrane or mass in the pupil area behind the lens of the eyes (leukokoria).
Treatment:
The treatment of Coats disease is directed toward the specific symptoms that are apparent in each individual. A procedure that uses extreme cold to destroy tissue (cryotherapy), and/or a procedure that uses intense, focused light (e.g. laser therapy) to heat and destroy tissue (photocoagulation) are often used to treat abnormal blood vessels (Telangiectasia) associated with Coats disease.
Surgery to reattach the retina may be necessary in some cases. Drainage of sub retinal fluid and/or surgical removal of the transparent substance (vitreous body) that fills the eyeball between the lens and the retina (vitrectomy) may also be used to treat Coats disease. Procedures where body tissues (e.g. Telangiectasia) are heated and destroyed (diathermy) have also been used to treat Coates disease.
Genetic counselling May be of benefit for affected individuals and their families. Other treatment is symptomatic and supportive.
NEW INFORMATION 2006
Origin of ghost cell in Coats' disease
Y Takei
A morphological study of Coats' disease was performed with light and electron microscopy. The choroid was intact throughout. The pigment epithelial cells showed proliferative changes, being thickened in two or three rows. The transitional form of ghost cell was observed in contact with the pigment epithelial layer, then migrated toward the subretinal space, and finally invaded the detached retina. It was concluded that the origin of ghost cell in Coats' disease was the pigment epithelium.
http://www.iovs.org/cgi/content/abstract/15/8/677
RESOURCES
CNIB Canadian National Institute for the Blind.
CNIB Peterborough Ontario Canada
Haliburton/Kawartha/Pine Ridge
Doug Downer, District Manager
236 King St., Peterborough K9J 7L8
Tel: (705) 745-6918 Fax: (705) 745-9899
Fran Daly, Administrative Secretary
NORD -National Organization for Rare Disorders, Inc
P.O. Box 1968,
Danbury, CT 06813-1968
Tel: (203) 744-0110
Email: orphan@rarediseases.org
Internet: www.rarediseases.org
National Association for Visually Handicapped
22 West 21st. Street
New York, NY 10010
Tel: (212) 889-3141
Fax: (212) 727-2931
Email: staff@navh.org
Internet: www.navh.org
Foundation Fighting Blindness
11435 Cronhill Dr.
Owings Mills, MD 21117-2220
USA
Fax: (410) 363-2393
Tel: (800) 683-5555
TDD: (800) 683-5551
Email: jchader@blindness.org
Internet: www.blindness.org
National Association for Parents of the Visually Impaired
P.O. Box 317
Watertown, MA 02472
Tel: (617) 972-7441
Fax: (617) 972-7441
Tel: (800) 562-6256
Email: napvi@perkins.pvt.k12.ma.us
Internet: www.spedex.com/napvi
National Federation of the Blind
1800 Johnson St.
Baltimore, MD 21230
Tel: (410) 659-9314
Fax: (410) 685-5653
Email: nfb@iamdigex.net
Internet: www.nfb.org
American Council of the Blind
1155 15th. St.
Suite 720
Washington, DC 20005
Tel: (202) 467-5081
Fax: (212) 467-5085
Tel: (800) 424-8666
Email: nfb@acb.org
Internet: www.acb.org
American Foundation for the Blind
11 Penn Plaza
Suite 300
New York, NY 10001
Tel: (212) 502-7600
Fax: (212) 502-7777
Tel: (800) 232-5463
TDD: (212) 502- 7662
Email: afbinfo@afb.org
Internet: www.afb.org
Blind Children's Fund
311 W. Broadway
Suite 1
Mt. Pleasant, MI 48858
Tel: (989) 779-9966
Fax: (989) 779-0015
Email: bcf@blindchildrensfund.org
Council of Families with Visual Impairment
6212 West Franklin St.
Richmond, VA 23226
Tel: (804) 288-0395
NIH/National Eye Institute
Building 31 Rm. 6A32
31 Center Dr. MSC 2510
Bethesda, MD 20892-2510
USA
Tel: (301) 496-5248
Fax: (301) 402-1065
Email: 2020@nei.nih.gov
Internet: www.nei.nih.gov/
Selected Anomalies and Diseases of the Eye
Short descriptions of the eye condition, implications, and related websites for further information.
http://www.tsbvi.edu/Education/anomalies/index.htm
Blindness/Visual Impairment
Links to over 700 sites on blindness and vision impairment.
http://blindness.about.com/health/blindness/mbody.htm
Eyeatlas
An online atlas of Ophthalmology- descriptions and slides of eye diseases.
http://www.eyeatlas.com
University of Florida
Assorted slides of eye diseases and anomalies.
http://www.medinfo.ufl.edu/year2/ophthal
Other Vision Related Websites
http://www.svrc.solind.net.au/visionrelated.html
http://www.djo.harvard.edu/GR/Kapusta073097/Kapusta073097Dx.html
http://med-aapos.bu.edu/iHound.ihnd
Gislin Dagnelie, Ph.D.
Assistant Professor of Ophthalmology/.
Director of Low Vision Services.
Lions Vision Research & Rehab Center.
Johns Hopkins Univ. Sch. of Medicine.
550 N. Broadway, 6th floor.
Baltimore, MD 21205-2020 USA.
http://www.focusnewsletter.org/frequent.htm.
American Journal of Ophthalmology 2001;131:572-583 and 561-571
National Library of medicine.
http://gateway.nlm.nih.gov/gw/Cmd
Canadian Ophthalmological Society
1525 Carling Avenue, Suite 610
Ottawa, Ontario, Canada K1Z 8R9
(800) 267-5763 or (613) 729-6779
fax (613) 729-7209
Eye Clinic at St. Michael's Hospital in Toronto.
The Hospital for Sick Children
Jamie Morillo Resource Centre Assistant The Centre for Health Information and Promotion
Toronto, Ontario, Canada
p: (416) 813-7654 ext. 8827
f: (416) 813-6715
jamie.morillo@sickkids.ca
National Library of medicine.
David McKay
Level 5, 231 Macquarie Street
Sydney
NSW 2000
Australia
61-2-92334840
http://www.rbas.org.uk/
The Royal Blind Asylum and School in Scotland
PO Box 500
Gillespie Crescent
Edinburgh EH10 4HZ
General enquiries
J B M Munro,
Secretary and Treasurer
email: enquiries@rbas.org.uk
Tel: 0131 229 1456
Fax: 0131 229 4060
Ophthalmology Links
Vision Related Web Sites
WEB MD
Wills Eye Hospital
Blindness Related Resources
Accessdome.com
The Blind and Visually Impaired Ring
I have provided some links to images of the human retina.
Medical Encyclopedia
Simple Anatomy of the Retina
Parents.org Coates disease
Google search for photo's of the retina
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There will be more medical information and photographs on Coates (Coat's) Disease as I find it. If you have additional information, related links and/or photographs, PLEASE email me. I would also like to add personal encounters with this disease as well as my own. See My life with Coates disease.
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New!!!! Links added June 13, 2006
The Macular Degeneration Community
http://www.mdsupport.org/list.html
Smilechild magazine UK
http://www.smilechild.co.uk/magazine/generator.asp?article=73
Discussion Area
http://www.smilechild.co.uk/da/messages.asp?page=1&gototopic=coates+disase
Lasik Surgery for Coate's
http://www.lasikcorner.com/links/coates-disease-eye.html
Coats' Disease and Leber's Miliary Aneurysm
http://www.revoptom.com/handbook/41.htm
Retinal cryotherapy
http://www.umdnj.edu/eyeweb/faqs/cryo.html
Human Molecular Genetics
http://hmg.oxfordjournals.org/cgi/content/full/8/11/2031
Coats’ disease in a vegetarian female
http://bjo.bmjjournals.com/cgi/content/full/88/7/970
Informational website for parents of children diagnosed with Coats' Disease.
http://www.coatsdisease.org/index.html
Coats disease by Doctor Alessandra Del Longo
http://www.coatsdisease.org/index.html
As of September 2005, there is a clinical trial for Coats' Disease recruiting patients over 18 years old
http://www.clinicaltrials.gov/ct/show/NCT00211315;jsessionid=B9BE70FD21BDDE367C988308280A9216?order=1
Retinal Detachment Repair
http://www.eyemdlink.com/EyeProcedure.asp?EyeProcedureID=52
Physical anatomy of the retina with descriptions of diseases
http://en.wikipedia.org/wiki/Retina#Retinal_anatomy
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New Links!!!! July 16, 2008
Eyes Apart
http://www.eyesapart.com/2006/11/11/coats-disease/#comment-19445
Wikidoc Coats disease
http://www.wikidoc.org/index.php/Coats_disease
Coats' Disease
Our journey since our son was diagnosed with Coats' Disease.
http://coatsdisease.blogspot.com/
Coates Disease Message Board
http://www.coatsdisease.org/board/board_topic/2684224/341732.htm
Coats' Disease Resources - Discussion Forum
http://www.coatsdisease.net/
http://www.coatsdisease.net/phorum/list.php?2
Eyes Apart
http://www.eyesapart.com/2006/11/11/coats-disease/#comment-19445
Wikidoc Coats disease
http://www.wikidoc.org/index.php/Coats_disease
Coats' Disease
Our journey since our son was diagnosed with Coats' Disease.
http://coatsdisease.blogspot.com/
Coates Disease Message Board
http://www.coatsdisease.org/board/board_topic/2684224/341732.htm
Coats' Disease Resources - Discussion Forum
http://www.coatsdisease.net/
http://www.coatsdisease.net/phorum/list.php?2
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