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$Title{Coats' Disease}
$Subject{Coats' Disease Exudative Retinitis Hyperlipemic Retinitis}
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Copyright (C) 1991 National Organization for Rare Disorders, Inc.

837:
Coats' Disease

** IMPORTANT **
It is possible that the main title of the article (Coats' Disease) is not
the name you expected.  Please check the SYNONYMS listing to find the
alternate name and disorder subdivisions covered by this article.

Synonyms

     Exudative Retinitis

Disorder Subdivisions:

     Hyperlipemic Retinitis

General Discussion

** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

Coats' Disease is a rare eye disorder that usually presents itself during
the first ten years of a child's life.  This disorder is characterized by a
white or yellowish area encompassing the small blind spot on the surface of
the retina (optic disk).  Inflammation may result from an excess of fluid
beneath the retina.  Loss of vision and retinal detachment may occur.  Coats'
Disease is usually found in one eye only and normally progresses slowly.

Hyperlipemic Retinitis is the adult form of Coats' Disease.  Information
on Hyperlipemic Retinitis can be found in the disorder subdivision section of
this report.

Symptoms

The main symptoms of Coats' Disease are widening of capillaries and small
vessels of the retina (retinal telangiectasia), reflection from a white mass
within the eye giving the appearance of a white pupil (white fundus reflex),
and a condition in which the eye is crossed (strabismus).

Early Coats' Disease is characterized by large, yellowish areas in the
retina as well as beneath it.  There may also be dilated blood vessels around
the outer surface of the retina.

In the majority of cases, this disorder progresses for many years.
Eventually detachment of the retina, the development of a fiberlike tissue
behind the lens of the eye (Retrolental mass), a loss in the clearness of the
lens (secondary cataract), reddish discoloration due to the forming of new
blood vessels on the iris of the eye (rubeosis iridis), swelling under the
membrane that covers most of the back of the eyeball (uveitis), pressure
within the eye (secondary glaucoma) and shrinking of the eyeball may also
occur with this disorder.  In a few cases this disorder has regresses for no
apparent reason.

Disorder Subdivision
Hyperlipemic Retinitis is the adult form of Coats' Disease.  The symptoms
of this disorder are the same as those in Coats' Disease with the exception
of adults having a history of inflammation of the middle layer of the eye and
excessive cholesterol in the blood.

Causes

The exact cause of Coats' Disease is not known.

Affected Population

Coats' Disease affects males more often than females.  This disorder usually
occurs during childhood.  When it occurs during adulthood it is called
Hyperlipemic Retinitis.

Therapies:  Standard

Coats' Disease may be treated with heat targeted to the retinal tissue which
is generated by electric currants (diathermy).  Corticosteroid drugs may also
be used.  These methods have been used with limited effects.
Photocoagulation and cryotherapy (freezing) may also be of some value.

Hyperlipemic Retinitis (the adult form of Coats' Disease) usually does
not respond to photocoagulation or diathermy but treatment with
anticholesterolemic drugs and a low cholesterol diet may be helpful.

Therapies:  Investigational

This disease entry is based upon medical information available through August
1991.  Since NORD's resources are limited, it is not possible to keep every
entry in the Rare Disease Database completely current and accurate.  Please
check with the agencies listed in the Resources section for the most current
information about this disorder.

Resources

For more information on Coats Disease, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     203-746-6518

     National Retinitis Pigmentosa Foundation
     1401 Mt. Royal Avenue, 4th Floor
     Baltimore, MD  21217
     (800) 638-2300
     (301) 225-9400

     National Federation of the Blind
     1800 Johnson Street
     Baltimore, MD  21230
     (301) 659-9314
     (800) 638-7518

     American Council of the Blind, Inc. (ACB)
     1155 - 15th St., NW, Suite 720
     Washington, D.C.  20005
     (202) 467-5081
     (800) 424-8666

     American Foundation for the Blind (AFB)
     15 W. 16th St.
     New York, NY  10011
     (212) 620-2000
     Regional offices:
     Atlanta, GA  (404) 525-2303
     Chicago, IL  (312) 245-9961
     Dallas, TX  (214) 352-7222
     San Francisco, CA  (415) 392-4845

     National Association for Parents of the Visually Impaired, Inc. (NAPVI)
     P.O. Box 180806
     Austin, TX  78718
     (512) 459-6651

     NIH/National Eye Institute
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5248

References

CLINICAL OPHTHALMOLOGY, 2nd Ed.; Jack J. Kanski, Editor; Butterworth-
Heinemann, 1990.  Pp. 336.

COATS'-TYPE RETINITIS PIGMENTOSA:  J.A. Khan, et al.; Surv Opthalmol
(Mar-Apr, 1988, issue 32(5).  Pp. 317-32.