Which patient group is most frequently affected by Coats disease?

Young males, usually in one eye Coats disease is a telangiectatic retinal disease of unknown etiology that frequently affects unilateral eyes of young males.

Elderly women with hypertension

Post-menopausal women, bilaterally

Middle-aged diabetics, bilaterally

What did fundus fluorescein angiography reveal in this case of Coats disease?

A pigment epithelial detachment with a smokestack leak

Diffuse late staining of the optic disc only

Widespread blockage from preretinal haemorrhage

What did OCT demonstrate in this patient with Coats disease?

Cystoid macular edema OCT performed subsequently revealed cystoid macular edema in the affected right eye, while the left eye was normal on all imaging.

Vitreomacular traction with a taut membrane

Outer retinal atrophy with RPE loss

In the original classification of Coats disease, what defined Group I?

Massive subretinal exudate alone Group I demonstrated massive subretinal exudate alone, whereas Group II added intraretinal and subretinal haemorrhage with retinal vascular dilatations.

Retinal arteriovenous malformations

Total exudative retinal detachment

Vitreous haemorrhage with traction

Group III of the original Coats classification was later recognised by Von Hippel as which entity?

Angiomatosis retinae Group III included subretinal exudate with retinal arteriovenous malformations, which Von Hippel later recognised as a separate entity called angiomatosis retinae.

Familial exudative vitreoretinopathy

Retinal capillary haemangioblastoma of the disc

Which definition of Coats disease was used in the large study of 117 patients?

Bilateral retinal neovascularisation with fibrous traction

Peripheral avascular retina with a demarcation ridge

Subretinal fluid following a peripheral retinal break

In which situation is observation appropriate management for Coats disease?

Extensive exudative retinal detachment

Thick subretinal fluid under the macula

Any eye with documented macular edema

What laser settings are recommended for the telangiectatic vessels in Coats disease?

Low power, long duration, large spot burns Telangiectatic vessels and aneurysms are treated with low power, long duration, large spot burns, with scatter laser applied to peripheral capillary non-perfusion.

Short duration, high power, small spot burns

Micropulse subthreshold grid without visible burn

Single high-energy YAG pulses per aneurysm

When is cryotherapy preferred over laser in the management of Coats disease?

When subretinal fluid is thick enough that cryo reaches the retina Cryotherapy is used for exudation with subretinal fluid of such thickness that the cryo reaction can still reach the retina; laser suits eyes with no or minimal subretinal fluid.

When rubeosis iridis has already developed

When the media are perfectly clear

When exudation is confined to the fovea

Which indication for enucleation in Coats disease is listed alongside a painful blind eye?

Retinoblastoma that cannot be ruled out Enucleation is reserved for a painful blind eye, or when retinoblastoma cannot be excluded by imaging and clinical examination.

Persistent cystoid macular edema

Intraocular pressure above 30 mm Hg

What limits the use of anti-VEGF therapy in Coats disease?

The safety profile in children is unclear Anti-VEGF therapy has been promising in Coats disease, but its safety profile in children is as yet unclear; intravitreal triamcinolone carries glaucoma and cataract risks.

It reliably worsens the subretinal exudation

It cannot be combined with laser treatment

It requires general anaesthesia in every case

Coats disease - Ophthalmology Education - Coats disease Educate.choroida.com

CASE REPORT

Case report of Coats disease 25-year-old male patient presented with a gradual diminution of vision in his right eye, for the past 6 months. Ocular examination on presentation revealed a vision of 6/60 in his right eye with no improvement.

The vision in his left eye was normal i.e. 6/6. The intraocular pressure (IOP) of the right and left eye, measured using a non-contact tonometer (NCT), was 14 mm of Hg and 13 mm of Hg, respectively.

Slit-lamp examination revealed no specific findings in the anterior segment and no rubeosis. Dilated indirect fundoscopy revealed subretinal exudation extending into the fovea. Fundus Fluorescein Angiography (FFA) revealed abnormal inferotemporal vessels with aneurysmal dilatation and telangiectasia, which leaked profusely.

Peripheral to the abnormal vasculature was an area of capillary non-perfusion. Optical Coherence Tomography (OCT) done subsequently revealed cystoid macular edema. Left eye examination including fundoscopy, FFA, and OCT was all within normal limits (WNL). The patient was diagnosed as a case of Coats’ disease

DISEASE of Coats disease

It is a telangiectatic neovascular disease of the retina of unknown etiology that frequently affects unilateral eyes of young males.

George Coats in 1908 described the histopathological features of enucleated eyes with massive exudation. He divided the morphology into 3 groups:

Group I demonstrated massive subretinal exudate alone.
Group II consisted of eyes with massive subretinal exudate, intra and subretinal hemorrhage, and retinal vascular dilatations.
Group III included eyes with subretinal exudate and retinal arteriovenous malformations. Later, Von Hippel recognized group 3 as a separate entity called angiomatosis retinae.

A large study of 117 patients took the definition of Coats disease as ‘idiopathic retinal telangiectasia and intraretinal or subretinal exudation without appreciable signs of vitreoretinal traction.

Test your knowledge 12 questions from this article

MANAGEMENT of Coats disease

Observation- In minimal telangiectatic vessels with little or no exudation and no imminent threat to vision, or in painless and comfortable blind eyes

Laser treatment (low power, long duration, large spot burns to the telangiectatic vessels and aneurysms, also laser the peripheral capillary nonperfusion as per scatter laser settings) – in telangiectasia with exudation but no or minimal subretinal fluid

Cryotherapy- for exudation with the subretinal fluid of such thickness that cryo reaction can reach the retina.
Vitreoretinal surgery- for extensive retinal detachment not allowing cryo or laser.
Enucleation- Painful blind eye or when retinoblastoma can not be ruled out by imaging and clinical examination.
Anti-VEGF therapy- has been promising but the safety profile for children is as yet unclear.
Intravitreal triamcinolone acetonide- may reduce the exudation, but risks of glaucoma and cataract are there.

Fundus photography is superior to fundus analysis as it enables intraocular pathologies to be photo captured and encrypted information to be shared with colleagues and patients.

Recent technologies allow smartphone-based attachments and integrated lens adaptors to transform the smartphone into a portable fundus camera, Slit lamp examination

REFERENCES

Coats G. Forms of retinal diseases with massive exudation. R Lond Ophthalmol Hosp Rep. 1908;17:440-525
Sigler EJ, Randolph JC, Calzada JI, Wilson MW, Haik BG. Current management of Coats disease. Surv Ophthalmol. 2014 Jan-Feb;59(1):30-46. doi: 10.1016/j.survophthal.2013.03.007. Epub 2013 Oct 15. Review. PubMed PMID:24138893.
Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: the 2000 Proctor Lecture. Am J Ophthalmol. 2001 May;131(5):572-83. PubMed PMID: 11336931.
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 May;131(5):561-71. PubMed PMID: 11336930.

Test yourself

A few questions straight from this article.

1 / 12 0 correct

Which patient group is most frequently affected by Coats disease?
Coats disease is a telangiectatic retinal disease of unknown etiology that frequently affects unilateral eyes of young males.
What did fundus fluorescein angiography reveal in this case of Coats disease?
FFA showed abnormal inferotemporal vessels with aneurysmal dilatation and telangiectasia that leaked profusely, with capillary non-perfusion peripheral to them.
What did OCT demonstrate in this patient with Coats disease?
OCT performed subsequently revealed cystoid macular edema in the affected right eye, while the left eye was normal on all imaging.
Who first described the histopathological features of eyes with massive exudation in 1908?
George Coats described in 1908 the histopathological features of enucleated eyes with massive exudation and divided the morphology into three groups.
In the original classification of Coats disease, what defined Group I?
Group I demonstrated massive subretinal exudate alone, whereas Group II added intraretinal and subretinal haemorrhage with retinal vascular dilatations.
Group III of the original Coats classification was later recognised by Von Hippel as which entity?
Group III included subretinal exudate with retinal arteriovenous malformations, which Von Hippel later recognised as a separate entity called angiomatosis retinae.
Which definition of Coats disease was used in the large study of 117 patients?
The 117-patient study defined Coats disease as idiopathic retinal telangiectasia with intraretinal or subretinal exudation and no appreciable signs of vitreoretinal traction.
In which situation is observation appropriate management for Coats disease?
Observation suits minimal telangiectatic vessels with little or no exudation and no imminent threat to vision, or painless comfortable blind eyes.
What laser settings are recommended for the telangiectatic vessels in Coats disease?
Telangiectatic vessels and aneurysms are treated with low power, long duration, large spot burns, with scatter laser applied to peripheral capillary non-perfusion.
When is cryotherapy preferred over laser in the management of Coats disease?
Cryotherapy is used for exudation with subretinal fluid of such thickness that the cryo reaction can still reach the retina; laser suits eyes with no or minimal subretinal fluid.
Which indication for enucleation in Coats disease is listed alongside a painful blind eye?
Enucleation is reserved for a painful blind eye, or when retinoblastoma cannot be excluded by imaging and clinical examination.
What limits the use of anti-VEGF therapy in Coats disease?
Anti-VEGF therapy has been promising in Coats disease, but its safety profile in children is as yet unclear; intravitreal triamcinolone carries glaucoma and cataract risks.

Coats disease

CASE REPORT

DISEASE of Coats disease

MANAGEMENT of Coats disease

REFERENCES

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