advertisement

Coats Disease

67 %
33 %
advertisement
Information about Coats Disease

Published on July 2, 2008

Author: ophthalmologyweb

Source: slideshare.net

advertisement

Doheny Eye Institute Grand Rounds Presenter Irina Bykhovskaya, MD Discussant Dr. SriniVas Sadda 3/9/2007

Patient Presentation HPI: 16 yo HM from juvenile hall c/o “black cloud” OS x 8 months POHx: h/o “fluid in the eye” and laser tx OS at age 8 yo PMH / FHx – unremarkable SLE: LLL/S/C/K/AC/I/L – wnl OU

HPI: 16 yo HM from juvenile hall c/o “black cloud” OS x 8 months

POHx: h/o “fluid in the eye” and laser tx OS at age 8 yo

PMH / FHx – unremarkable

SLE: LLL/S/C/K/AC/I/L – wnl OU

Fundus Photos lipid-rich, yellow subretinal exudate in the posterior pole OS

Fundus Photos OS

Red-Free Fundus Photos

Fluorescein Angiogram

Fluorescein Angiogram

Coats’ Disease Idiopathic condition Telangiectatic and aneurismal vessels with intraretinal and subretinal exudates 1 st described by Coats in 1908

Idiopathic condition

Telangiectatic and aneurismal vessels with intraretinal and subretinal exudates

1 st described by Coats in 1908

Etiology Vascular: Thickening of endothelial basement membrane of the characteristic telangiectatic vessels due to deposition of acid-Schiff (PAS)-positive material

Vascular:

Thickening of endothelial basement membrane of the characteristic telangiectatic vessels due to deposition of acid-Schiff (PAS)-positive material

Clinical Presentation Painless Males : Females = 3:1 80% unilateral 2/3 cases present before age 10 Diagnosed at a median age of 5 years No predilection for race or laterality Poor vision, strabismus, leukocoria Visual acuity at presentation 20/200 to no light perception No genetic transmission No systemic associations

Painless

Males : Females = 3:1

80% unilateral

2/3 cases present before age 10

Diagnosed at a median age of 5 years

No predilection for race or laterality

Poor vision, strabismus, leukocoria

Visual acuity at presentation 20/200 to no light perception

No genetic transmission

No systemic associations

Clinical Picture Localized, lipid-rich, yellow subretinal exudate Associated vascular anomalies: sheathing, telangiectasia, tortuosity, aneurismal dilatations, zones of capillary drop out, and occasionally neovascularization

Localized, lipid-rich, yellow subretinal exudate

Associated vascular anomalies: sheathing, telangiectasia, tortuosity, aneurismal dilatations, zones of capillary drop out, and occasionally neovascularization

Clinical Course Variable but generally progressive with acute exacerbations and quiescent stages Complications: subretinal CMV serous RD hemorrhagic macrocysts secondary iridocyclitis Cataract neovascular glaucoma

Variable but generally progressive with acute exacerbations and quiescent stages

Complications:

subretinal CMV

serous RD

hemorrhagic macrocysts

secondary iridocyclitis

Cataract

neovascular glaucoma

FA Reveals localized anomalies in retinal vasculature Telangiectasia, aneurisms, beading of vessel walls, and vascular communication channels Breakdown of blood-retinal barrier  Early and persistent leakage Microvascular anomalies: Diffuse loss of capillary bed or areas of complete capillary nonperfusion surrounded by areas of arteriolar and venular anomalies

Reveals localized anomalies in retinal vasculature

Telangiectasia, aneurisms, beading of vessel walls, and vascular communication channels

Breakdown of blood-retinal barrier  Early and persistent leakage

Microvascular anomalies: Diffuse loss of capillary bed or areas of complete capillary nonperfusion surrounded by areas of arteriolar and venular anomalies

Histopathology Loss of vascular endothelium and pericytes All levels of the retinal vascular circuit are involved Proteinaceous subretinal exudates with prominent cholesterol clefts and prominent histiocytes Total exudative retinal detachment

Histopathology cont’d Subretinal fluid with cholesterol clefts and lipid-laden macrophages Telangiectasic retinal vessels Intraretinal cholesterol deposition triggering a giant cell reaction foreign-body type

Differential Diagnosis Retinoblastoma Retinal detachment Persistentt hyperplastic primary vitreous Congenital cataract Norrie’s disease Familial exudative vitreoretinopathy Eales’ disease Vasculitis Tumor accompanied by exudation Diabetic vasculopathies BRVO Idiopathic juxtafoveal telangiectasis

Retinoblastoma

Retinal detachment

Persistentt hyperplastic primary vitreous

Congenital cataract

Norrie’s disease

Familial exudative vitreoretinopathy

Eales’ disease

Vasculitis

Tumor accompanied by exudation

Diabetic vasculopathies

BRVO

Idiopathic juxtafoveal telangiectasis

Diagnosis and Ancillary Testing Echography - Ultrasonography typically shows a retinal detachment but no solid mass CT or MRI High-resolution Doppler ultrasound - to differentiate from retinablastoma Computed Tomography. Total exsudative retinal detachment in the right eye.

Echography

- Ultrasonography typically shows a retinal detachment but no solid mass

CT or MRI

High-resolution Doppler ultrasound

- to differentiate from retinablastoma

Treatment Focal laser photocoagulation to close areas of vascular leakage to allow resorption of exudate Near yellow wavelength 100 to 500 microns Cryotherapy if retina is exudatively detached Subretinal fluid drainage and vasoablation +/- scleral buckle

Focal laser photocoagulation

to close areas of vascular leakage to allow resorption of exudate

Near yellow wavelength

100 to 500 microns

Cryotherapy if retina is exudatively detached

Subretinal fluid drainage and vasoablation +/- scleral buckle

References Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol. 2001;131:561–571. Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina. 2002;22:80–91. Shields JA, Parsons HM, Shields CL, Shah P. Lesions simulating retinoblastoma. J Pediatr Ophthalmol Strabismus. 1991;28:338–340. Bruno F Fernandes, Alexandre N Odashiro, Shawn Maloney, Moyses E Zajdenweber, Andressa G Lopes, and Miguel N Burnier, Jr. Clinical-histopathological correlation in a case of Coats' disease. Diagn Pathol. 2006; 1: 24. L. Smithen, G. Brown, A. Brucker, L. Yannuzzi, C. Klais, R. Spaide. Coats’ Disease Diagnosed in Adulthood. Ophthalmology, Volume 112, Issue 6, Pages 1072-1078 Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary vitreous: role of MR imaging and CT. Radiol Clin North Am 1998;36:1119-1131

Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol. 2001;131:561–571.

Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina. 2002;22:80–91.

Shields JA, Parsons HM, Shields CL, Shah P. Lesions simulating retinoblastoma. J Pediatr Ophthalmol Strabismus. 1991;28:338–340.

Bruno F Fernandes, Alexandre N Odashiro, Shawn Maloney, Moyses E Zajdenweber, Andressa G Lopes, and Miguel N Burnier, Jr. Clinical-histopathological correlation in a case of Coats' disease. Diagn Pathol. 2006; 1: 24.

L. Smithen, G. Brown, A. Brucker, L. Yannuzzi, C. Klais, R. Spaide. Coats’ Disease Diagnosed in Adulthood. Ophthalmology, Volume 112, Issue 6, Pages 1072-1078

Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary vitreous: role of MR imaging and CT. Radiol Clin North Am 1998;36:1119-1131

Add a comment

Related pages

Coats' disease - Wikipedia, the free encyclopedia

Coats’ disease, (also known as exudative retinitis or retinal telangiectasis, sometimes spelled Coates' disease), is a rare congenital, nonhereditary eye ...
Read more

Coats' Disease: Get the Facts about This Rare Eye Condition

Coats' disease is a rare eye condition that typically progresses to vision loss or blindness in one eye. Read about symptoms, signs, diagnosis, treatment ...
Read more

Morbus Coats – Wikipedia

Der Morbus Coats ist eine seltene angeborene Augenkrankheit mit sichtbaren Erweiterungen und Veränderungen ... Coats Disease. Orphanet Encyclopedia. Sept ...
Read more

Coats disease | Disease | Your Questions Answered ...

Coats disease is an eye disorder characterized by abnormal development of the blood vessels in the retina (retinal telangiectasia). Most ...
Read more

Coats' Disease — AAPOS - American Association for ...

What is Coats' disease? Coats’ disease is caused by a problem with the arteries and veins (blood vessels) inside the eye that provide blood and oxygen to ...
Read more

Coats disease | Disease | Overview | Genetic and Rare ...

A collection of disease information resources and questions answered by our Genetic and Rare Diseases Information Specialists for Coats disease
Read more

Coats Disease - WebMD - Better information. Better health.

Important It is possible that the main title of the report Coats' Disease is not the name you expected. Please check the synonyms listing to ...
Read more

Coats' Disease Symptoms, Causes, Treatment - What is the ...

What is the prognosis of Coats' disease? What research is being done on Coats' disease?
Read more

Coats' disease | RNIB | Supporting people with sight loss

About Coats' diseaseCoats’ disease, also known as Exudative Retinitis, is an uncommon eye condition which affects the smaller blood vessels (capillaries ...
Read more