Diseases of Retina

The 4th Affilitated Hospital of China Medical University

Eye Hospital of China Medical University

Introduction
Eyeball structure and retina
Macula lutea

Located 3mm temporally to the optic

papilla, right on the visual axis.
A concave central retinal depression is
called Fovea Centralis

macula lutea contains only cones; 1

cone synapes to 1 bipolar cell,which
synapes to 1 ganglion cell,leading to
the most sensitive vision.
In periferal retina ,600 rods connect to
1 ganglion.
Histology of retina
Internal limiting membrane
Nerve fiber layer
Ganglion cell layer

Inner plexiform layer

Inner nuclear layer

Outer plexiform layer

Outer nulear layer

External limiting membrane

photoreceptor

RPE
Bruchs membrane
Neuroconduction of retina

3 neurons: rod scotopic

vision
Photoreceptor cone photopic
vision
Bipolar Connecting cell between
photoreceptor and ganglion
Ganglion cell

Conduct to brain

Supporting tissue:
Mller cell
Vasculature of retina

inner layer central retinal vascular

system

outer layer choroid ciliary vascular

system

macula lutea choriocapillaries

Retina barrier

Inner barrier bloodretina barrier

dense connection of retinal capillary endothelium

Outer barrier choroid-retina barrier

zonula occludens between the RPE

RPE- Bruchs membrane- choriocapillaries

complex
Symptoms
Visual impairment Related to
lesion site

Metamorphopsia

Vitreous
Flickering traction to
the retina
Macropsia
Retina edema
fewer cones
Micropsia stimulated
micropsia
 Signs
Retinal artery occlusion:
Intracellular edema ischemia leads to edema
of bipolar cell,ganglion and RNFL

Extracellular edemaCapillary endothelium injury and the

exudation

Cystoid macular edema

Henles fibers are radically located;
This pooling forms a flower-petal pattern
Intracellular edema Extracellular edema
Exudates

Hard exudate
Leakage of capillary absorb
deposition of lipid in outer plexiform layer

Cotton-wool spot

Since be calledsoft exudation

Precapillary arteriole occlusion
axoplasmic transport blockedorganelles stack
Cotton-wool spot Hard exudate (Hypertensive
retinopathy)
Heamarrhage
Between outer plexiform
layer and inner nuclear
Deep hemorrhage layer .Small round ,dark
red
Located in nerve fiber
layer
Superficial hemorrhage
line strip flame-
like bright red
Crescent-shaped
hematocele with
Preretinal hemorrhage transverse section
Profuse preretinal
hemorrhage into the
vitreous or hemorrhage
Vitreous hemorrhage of retinal neospastic
vasculature
Preretinal Subretianal hemorrhage
hemorrhage
Deep hemorrhage Superficial hemorrhage
Neovascularization

neovessels,NV

A large area of retinal ischemia

formation of vascular endothelial growth factor
neovascularization

neovessels membrane,NVM

Arise from small veins of optic disc and retina; grow along
retinal surface and into the vitreous
Neovacularizaton of optic disc

Retinal neovascularization
Blood vessel change
1,Atherosclerosis, stenosis,occlusion
2,Tortuous vein, dilated vein ,bead-like change
 A-v cross sign

A-V cross sign

Vessel white Vessel white sheath

sheath

Microaneurysm

Microaneurysm
Changes of RPE

atrophy
Pigment loss atrophy

Pigment disorder
alteration
alteration
Osteocyte-like pigment deposition

Death
Death or
or proliferation
proliferation
Choroidal neovascularization

Inflammation , metabolic deposit of RPE or Bruchs

membrane break

CNV reach RPE or subsensory layer

Classification of retinal
diseases

Vascular diseases
Macular diseases
Retinal detachment
Retinal degeneration
Retinal tumor
Ocular manifestation of general
diseases
Retinal vascular disease

Retinal artery occlusion

Retinal venous occlusion

Diabetic retinopathy

Vasculitis

Coats disease
Central retinal artery
occlusion CRAO
Etiology

Common causes
atherosclerotic thrombosis of cribiform plate
systemic diseases,hemicrania, trauma, blood
coagulation disorder, inflammation, infectious
disease or connective tissue disease

Occasionally seen in
retrobulbar injection retinal detachment or
orbital operation
Clinical manifestation
Sudden painless vision
Symptoms lose of one eye

Direct light reflex disappear,

Signs indirect light reflex normal

Retinal edema cherry-red spot

Retina artery narrow mild hemorrhage
Normal eye fundus CRAO
21s after injection of
fluorescein ,a complete
absence in filling
central retinal artery,
except segment of
inferior temperal
branch and macular
branch FFA of CRAO
Treatment

Target: to reestablish retinal

circulation & function

Timing: the earlier the better

Drugs: vasodilator( tropical or

systemic)+ reduce IOP
1.Vasodilator :
antispasm or pushing thrombus to the
2.Reducing IOP:
smaller branch
1 massage
2 anterior chamber paracentesis
3 diamox 500mg st 250mg bid
NaHCO3 500mg bid tid
3.Oxgen inhalation:
mixture of 95% oxygen &5% carbon dioxide

4.Fibrolytic enzyme: for patients suspect of thrombosis

urokinase 5 000 10 000U iv qd
Prognosis

Depends on site ,severity and

duration.

Irreversible after 4 hrs

Central retinal vein
occlusion
Clinical manifestation
Non-ischemic type

Mild fundus change :

retinal hemorrhage and
tortuous vein
Mild VA decrease
capillary nonperfusion
rare
Visual field defect
(retinal hemorrhage)
 Ischemic type

More common
Extensive retinal
hemorrhage and
tortuous
vein Multiple
cotton-wool spots
Severe VA decrease
Widespread capillary
nonperfusion 60%
cases present iridal
neovascularization.
CRVO CRVO
Nonischemic ischemic
Branch retinal vein
occlusion

BRVO BRVO
FFA
Treatment

Chinese medicine
Anitplatelet or antithrombotic drugs: unknown
therapeutic effects
Systemic examination to find out causes
Corticosteroid if vasculitis exist

Grid pattern photocoagulation of macula PRP

Laser induced retina-choroid vascular

anastomosis
Vasculitis

Idiopathic retinal vasculitis

Eales disease(Retinalperiphlebitis)

Both A. and V. are involved

Causes is unclear patient tuberculin
reaction (+)
Seen in 20-40 years old men
Bilateral peripheral small vessels
occlusion recurrent vitreous
hemorrhage retinal neovascularization
Ealesdisease:
Grey exudate, vessel
white shealth and
pigment scar is
seen near the
temperal periferal
vein.
Clinical manifestation:

early stage: no symptoms

mild hemorrhage: floaters
moderate hemorrhage: visual defect
severe hemorrhage: retinal detachment.
 Chinese medicine
Nutrition reinforcement:
vitamin
Remove causes
Corticosteroid
laser photocoagulation
Surgery: vitrectomy
Coats disease
Or retinal telangiectasia external exudative
retinopathy

Often seen in boy unilateral

Youngsters and adults may also suffer

No heredity no relation with systemic angiopathy

Etiology: unknown

Malformation and telangiectasia of capillary in external

plexiform layer Permeability alteration and vessel
rupture
Exudation
Clinical manifestation

Visual
disturbance strabismus leucoria

Fundus
extensive yellow-white lipid exudation
with faring cholesterol crystal capillary
and vein dilate microaneurysm
capillary nonfusion Secondary
glaucoma, exudative RD, uvitis,
complicated cataract

Rare neovascularization
Differential diagnosis

RB

ROP

FEVR
Treatment

Photocoagulation or
cryocoagulation of capillary dilation
Macular diseases

Central serous chorioretinopathy

Age-related macular degeneration

Central exudative chorioretinopathy

Central serous
chorioretinopathy

Often seen in 20-45 years old men

Self-limitted
Related to stress reaction
 Etiology unknown

Anxiety,allergy,infection,
insufficient reflux of choroid
vein,thermal regulation
dysfunction
Clinical manifestation
Metamorphopsia

blurred vision,
micropsia
 OCT

serous
detachment of the
sensory retina in
the macular area

Subretinal yellow
deposit in the
macular area
FFA shows dye leakage under RPE
 Treatment
80% -90%undergo spontaneous
recovery

Corticosteroid is forbidden

Laser photocoagulation may be

considered when the leakage more than
200um away from macula
Age-related macular
degeneration
The incidence increase with each decade over age 50
Main blind-causing disease in elderly
Severe central visual loss
Etiology:
Long-term chronic macular light damage heredity metabolism
nutrient factors

Mechanism:
Decreased phagocytosis of RPE leading to dusen.
Drusen can cause damage of Bruchs membrane CNV and fibrocyte
proliferation
Destruction of choroidal capillary Bruchs membrane RPE and
photoreceptor
Clinical presentation
Visual acuity: decreased VA, metamorphopsia,
micropsia

Visual field: central scotoma

Fundus:
Dry:
drusen, RPE change
Wet:
gray-yellow CNV under retina of posterior pole
associated with dark red subretinal hemorrhage which
covers CNV sometimes

FFA: CNV leakage bleeding

 Nonexudate:
Drusen
RPE atrophy
Degeneration of photoreceptor
Choroid capillary atrophy
 Exudate:
Dursen
Damage of Bruchs membrane
CNV
Disciform scar formation under
macula, bleeding and leakage of
CNV
Exudative AMD
 Treatment
Anti-oxidation drug
CNVM located greater than 200 microns from
the center of the foveal avascular zone (FAZ)
Photodynamic therapy PDT
TTT
Surgically remove CNV
Macular translocation
Retinal detachment

Retinal detachment denotes

separation of the sensory retina from the
underlying RPE

There are three main types

rhegmatogenous traction exudative
Rhegmatogenous retinal
detachment formation

retinal degeneration
Basis liquified vitreous
retinal holeRD
aging
Incentives high myopia
ocular trauma
 Clinical manifestation:

Flashes of light, floaters, a curtain or

shadow moving over the field of vision,
peripheral and/or central visual loss.
Normal IOP, then low IOP
Elevation of the retina and a flap tear or
break in the retina
Treatment

Close the tears by

photocoagulation condensation
electric coagulation
Scleral buckling
Vitrectomy
Retinitis Pigmentosa

Chronic ,progressive,inherited disease

Cone cell rod cell and RPE distrophy
Inheritance pattern: AD, AR,X-link
Onset age: childhood,
Bilateral eyes involved
Clinical manifestation
Constriction of visual field
Fundus :
optic disc waxcolor, "bone spicule" pigment
deposition
Nyctalopia is the first symptom
FFA:
window defect, blocked fluorescein in pigment
deposition at early stage, hypofluorescence and the
fluorescein of choroid is seen at late stage
optic disc waxcolor,
bone spicule" pigment
deposition
Treatment

Genetic counselling
Avoiding sunlight and UV
Vasodilator, Vitamins
Suppliment of taurine
Low vision aids
Grid laser coagulation is used with caution
for CME
Retinal tumor

Retinoblastoma

The most common primary intraocular

malignant tumor in children,of which
90%younger than 3 yrs

Heritable (AD)or sporadic

Etiology
parents are affected or gene carriers ,or germ
cell mutation
-----autosomal dominant 40%
patient with retinoblast mutation
-----uninherited 60%
patient with autosomal chromosome mutation
----- RB ,complicated with intelligent and
growth retardation.
Study shows:
loss of or unactivated RB gene is the key
factor of RB
Staging

intraocular stage

glaucomatous stage

extraocular stage

metastasis stage
 Differential diagnosis
RB Coats disease
Onset age 90%<3 >6-8 yrs
yrs
Eyes involved 30%both 95%single
Microcirculati none Diffuse
on microanuerysm
abnormality
Cholesterol none Subretinal
cystal ,obvious
Calcification + -
B-us Solid Without solid
tumor tumor
RB Coats disease
Treatment
Small tumor localized in central retina
can be effectively treated with
photocoagulation
Small tumor localized in periferal retina
can be treated with cryotherapy
Moderate localized tumor:Plaque
radiation therapy
Big tumor: Enucleation
Extraocular stage: enucleation+
chemotherapy + radiation (bad
prognosis)
Metastasis : no specific treatment
 Right afterLaser
photocoagulation

6 moths after
photocoagulation
 RB

After plate radiation therapy