This document discusses Coats' disease, a rare eye condition characterized by abnormal blood vessels in the retina. It presents in children, more often in boys than girls, and causes vision loss. Symptoms include blurred vision and eye swelling. Examination shows leaking blood vessels, fluid buildup, and retinal detachment. Treatment depends on the stage, ranging from close monitoring to laser therapy, cryotherapy, surgery, and in severe cases, eye removal. The goal is to preserve vision or the eye's structure.

1. Dr. Lanin Chen
Resident, Dept. of Ophthalmology
D. Y. Patil Medical College, Navi Mumbai.

2. INTRODUCTION
 First described by George Coats in 1908.
 Coats’ disease is an idiopathic condition
characterized by retinal vascular changes and
exudation caused by a defect in retinal
vasculature development.
 Increased permeability of abnormal retinal
vessels causes leakage into the intraretinal
and subretinal spaces.
 Males: Females :: 3:1
 80% are unilateral
 2/3rd cases present before 10 years of age
 No predilection for race

3. CLASSIFICATION
Shields, JA et al. Classification and Management of Coats Disease: the 2000 Proctor Lecture. Am J
Ophthalmology. 2001;31(5):572-583.

4. CLINICAL FEATURES
SYMPTOMS
 Blurred vision
 Photopsia
 Floaters
 Red eye effect
SIGNS
 Leucocoria
 Strabismus
 Corneal Oedema
 Iris
neovascularization
 Heterochromia

5. FUNDUS EXAMINATION
 Telengiectasia
 Intraretinal
exudation
 Exudative RD
 Partial RD
 Total RD
 Retinal hemorrhage
 Retinal macrocyst

6. DIFFERENTIAL
DIAGNOSIS
 Retinoblastoma
 Retinal detachment
 Persistent hyperplastic primary vitreous
 Congenital cataract
 Norrie’s disease
 Eale’s disease
 Vasculitis
 Tumour with exudation
 Idiopathic juxtafoveal telangiectasia

7. DIAGNOSTIC TESTS
 FFA
 USG - Hyperechoic mass in the posterior vitreous without
posterior acoustic shadowing. Vitreous and subretinal
hemorrhage may be seen.
 CT Scan - The globe appears hyperdense compared to the
normal vitreous due to the proteinaceous exudate, which may
obliterate the vitreous space in advanced disease. The anterior
margin of the subretinal exudate enhances with contrast. Since
the retina is fixed posteriorly at the optic disc, this enhancement
has a V-shaped configuration.
 MRI - The subretinal exudate shos high signal intensity on both
T1 and T2 weighted images. The exudate may appear
heterogenous if hemorrhage or fibrosis is present. The
subretinal space does not enhance with gadolinium constrast.
Mild to moderate linear enhancement may be seen between the
exudate and the remaining vitreous.

8. FFA

9. TREATMENT
 The major goal of treatment in Coats' disease is
to preserve or improve visual acuity or, when
this is impossible, to preserve the anatomical
integrity of the eye.
 Treatment generally consists of
photocoagulation, cryotherapy, and in severe
cases, retinal reattachment surgery.
 Anti-VEGF injections have been successfully
used as part of combination therapy in case
series but there is a risk of vitreo-retinal traction.

10. TREATMENT MODALITIES
 Stage 1 – periodic observations / laser
photocoagulation
 Stage 2 – Laser photocoagulation /
cryotherapy
 Stage 3A – Retinal re-attachment surgery /
laser photocoagulation
 Stage 3B – Retinal re-attachment surgery
/Cryotherapy
 Stage 4 – Enucleation
 Stage 5 – No aggressive treatment

11. TREATMENT
 Intravitreal triamcinolone acetonide
 Effective in macular oedema and subretinal
exudation.
 Intravitreal Anti-VEGF agents
 Reduces subretinal fluid and macular
exudation.
 Surgical Intervention
 in cases of traction, hemorrhage, retinal
detachment, painful blind eye.

12. REFERENCES:
1. Shields, JA et al. Classification and Management of
Coats Disease: the 2000 Proctor Lecture. Am J
Ophthalmology. 2001;31(5):572-583.
2. Kanski, Jack J. Clinical Ophthalmology: A Systematic
Approach. 6th ed. Elsevier Ltd., 2007.