|
CHR |
Coats disease |
von Hippel-Lindau disease |
Sex |
No sex predilection |
Male: female=3:1 |
No sex predilection |
Average age at diagnosis |
25 years old |
10 years old |
25 years old |
Family history |
20% |
No |
Yes |
Affected eye |
92% unilateral |
95% unilateral |
45% unilateral |
Symptom |
Usually asymptomatic |
Decreased visual acuity
Leukocoria
Strabismus |
Decreased visual acuity |
Fundus finding |
1. Clusters of thin-walled saccular aneurysms filled with dark venous blood isolated from the vascular tree
2. White fibroglial tissue on the surface of the tumor. |
1. The aneurysms placed along retinal vessels,
2. Progressive vascular dilatation
3. Massive intraretinal and subretinal exudation
4. Subretinal fluid |
Progressive dilation of the afferent and efferent feeder vessels
Large isolated angioma
Exudation |
Retinal vascular circulation |
Normal |
Aberrant arteriovenous communicating channels |
Abnormal feeder vessels, dilated capillary bed around the tumor |
Retinal exudation |
Rare |
Common and progressive |
Common, may progressive |
Retinal detachment |
Rare, proliferative |
Sometimes, exudative |
Sometimes, proliferative |
FFA |
Plasma-erythrocytic separation; No leakage |
Capillary nonperfusion
Leakage from the lesion |
Slow retinal circulation; Leakage from angioma |
Progress |
Usually stable |
Progress |
Progress |
Visual prognosis |
Good |
Poor |
Poor |
Treatment |
Observation |
Photocoagulation
Cryotherapy |
Photocoagulation
Cryotherapy |
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