COAT’S DISEASE

COAT’S Disease

• Presence of

– vascular dilatations (retinal telangiectasia),

• Ectatic arterioles,

• Microaneurysms,

• Venous dilations (phlebectasias), and

• Fusiform capillary dilatations,

– frequently associated with exudative retinal detachment.

• Retinal Capillary nonperfusion• Neovascularization is distinctly UNUSUAL • Vessels are incompetent

– Leakage of serum and other blood components and accumulate under the retina

– Any portion of the peripheral retina and macula can be involved

• Variation is wide – Mild retinal vascular abnormalities– Minimal exudations– Extensive areas of telangectasias associated with massive

leakage and ERD (children with coat’s dse)

"Light bulb” Aneurysms

Histopathology of Coat’s Disease

COATS• MALE (85%)• YOUNG

– Faster Progression in children younger than 4 yo– Massive Exudation with ERD apposing the lens

• DDX of leukocoria? ----– Retinoblastoma– Retinoma– Retinal Dysplasia – Coloboma– Myelinated Nerve Fiber layers – Astrocytic hamartroma– Granuloma – PFV– TRAUMA– Endophthalnmitis– ROP– FEVR

• UNILATERAL• Gradual Progression w increasing Exudation

COATS in ADULTS

• Patients with peripheral areas of leakage typically present with lipid deposition in otherwise angiographically normal macula.

• Similar findings in adults represent LATE DECOMPENSATION of preexisting vascular anomalies– DDX

• ROP

• Dominant (Familial) Exudative Vitreoretinopathy

• Capillary Hemangioma ( VonHL)