Coats disease

Dr. Md.Mominul IslamIspahani Islamia Eye Institute And Hospital

Dr. Md. Mominul Islam Fellow (Vitreo-Retina)Ispahani Islamia Eye Institute And Hospital Dhaka Bangladesh

Introduction

• Idiopathic • Characterized by: Telangiectatic aneurysmal retinal

vessels with sub-retinal exudation and fluid

History

Scottish ophthalmologist Coats

Massive sub retinal Exudation No significant vascular abnormalities

Massive sub retinal Exudation No significant vascular abnormalities Internal Hemorrhage

Massive sub retinal Exudation No significant vascular abnormalities Internal Hemorrhage

Massive sub retinal Exudation Frank retinal arterioles and venous malformation

Massive sub retinal Exudation Frank retinal arterioles and venous malformation

Group I Group II Group III

Histopathology

Clinical presentation

• Painless ophthalmic condition• Male affected 3 times more 3:1• No racial predilection

On examination

Decrease Visual acuity Corneal Oedema Strabismus Leukocoria Heterochromia Iris neovascularization

Tealangiectesia Intraretinal exudation Exudative RD Partial RD Total RD Retinal hemorrhage Retinal macrocyst

Ophthalmoscopic picture

Staging of Coats Disease

Am J Ophthalmol 2001;131:572–83

Systemic conditions

• Muscular dystrophy• Turner syndrome• Alport syndrome• Aplastic anemia

Ocular conditions that can simulate

Juvenile Coats disease

Retinoblastoma Retinal detachment Congenital cataract Norrie disease Persistent hyperplastic

primary vitreous Ocular toxocariasis

At any age Branch retinal vein

occlusion Vasculitis Ocular toxoplasmosis Type 1 idiopathic

juxtafoveolar telangiectasis

Diagnostic Testing

Treatment

The goal of treatment mainly is to close telangiectesia so that further leakage will not

occur

Treatment (contd)

Stage I• Documentation (CFP and FFA)• Follow up conservatively • Intervention (if sub-retinal fluid and

exudation develop)

Treatment (contd)Stage II to IV:

• Laser photocoagulation• Cryotherapy• Surgical Intervention

Repair Traction Hemorrhage RRDUSE of PDT in combination with IVB for adult coats. disease

Ablative therapies

Laser Photocoagulation

• Less severe cases of exudation• With or without RD• Vascular leakage• Non perfusion• NVE

Laser Photocoagulation

• Less severe cases of exudation• With or without RD• Vascular leakage• Non perfusion• NVE

Cryotherapy

• Laser is ineffective • Extensive sub-retinal exudation• RD• Drain sub retinal exudation

Pharmacologic therapiesIntravitreal Triamcinolone acetonide

Effective in macular Oedema Sub retinal exudation

Intravitreal Anti VEGF

• Surgical Intervention

Repair Traction Hemorrhage RRD End stage NVE Painful Blind eye

Outcome• Telangiectesia resolved (mean interval 15 months following

treatment) Completely 47% Partially 53%• Inactive telangiectesia and Old exudation (17 months following

Treatment)- 45%• Recurrence of Leakage and New telangiectesia (in 10 years)-7%• Most cases Stabilize /Improve- 76% Progressively worsening -8% Required Enucleation – 20%

Take Home Massage

• Coats disease is a serious eye disease• Repeated treatment are needed to stabilize

the affected eyes• Lifelong and serial monitoring required.• Careful distinction of coats disease from

retinoblastoma is important