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:
Photoreceptor Bipolar Ganglion cell
rodscotopic vision conephotopic vision Connecting cell between photoreceptor and ganglion

Conduct to brain

Supporting tissue:
Mller cell

Vasculature of retina
inner layer outer layer

central retinal vascular system choroidciliary vascular system choriocapillaries

macula lutea

Retina barrier
Inner barrierbloodretina barrier
dense connection of retinal capillary endothelium

Outer barrierchoroid-retina barrier

zonula occludens between the RPE

RPE- Bruchs membrane- choriocapillaries complex

Symptoms
Visual impairment
Related to lesion site

Metamorphopsia
Flickering Macropsia Micropsia
Retina edema fewer cones stimulated micropsia Vitreous traction to the retina

Signs
Intracellular edema
Retinal artery occlusion: ischemia leads to edema of bipolar cell,ganglion and RNFL

Extracellular edema

Capillary endothelium injuryand then 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
Deep hemorrhage
Between outer plexiform layer and inner nuclear layer .Small round ,dark red

Superficial hemorrhage

Located in nerve fiber layer linestripflame-like bright red

Crescent-shaped hematocele with transverse section Profuse preretinal hemorrhage into the vitreous or hemorrhage of retinal neospastic vasculature

Preretinal hemorrhage

Vitreous hemorrhage

Preretinal hemorrhage

Subretianal 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 sheath

Microaneurysm

Vessel white sheath

Microaneurysm

Changes of RPE
Pigment loss Pigment disorder Osteocyte-like pigment deposition Death or proliferation
atrophy

alteration

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 occlusionCRAO

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 injectionretinal detachment or orbital operation

Clinical manifestation
Symptoms
Sudden painless vision lose of one eye

Signs

Direct light reflex disappear, indirect light reflex normal

Retinal edemacherry-red spot Retina artery narrowmild 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 : 2.Reducing IOP:

antispasm or pushing thrombus to the smaller branch

1massage 2anterior chamber paracentesis 3diamox 500mg st250mg bid NaHCO3 500mg bidtid

3.Oxgen inhalation:
mixture of 95% oxygen &5% carbon dioxide

4.Fibrolytic enzyme: for patients suspect of thrombosis

urokinase 5 00010 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 veinMultiple cotton-wool spots Severe VA decrease Widespread capillary nonperfusion 60% cases present iridal neovascularization.

CRVO Nonischemic

CRVO 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 maculaPRP Laser induced retina-choroid vascular anastomosis

Vasculitis
Idiopathic retinal vasculitis
Eales disease(Retinalperiphlebitis)

Both A. and V. are involved Causes is unclearpatient tuberculin reaction (+) Seen in 20-40 years old men Bilateral peripheral small vessels occlusion recurrent vitreous hemorrhageretinal neovascularization

Ealesdisease: Grey exudate, vessel white shealth and pigment scar is seen near the temperal periferal vein.

Clinical manifestation:
early stage:
no symptoms floaters visual defect

mild hemorrhage:

moderate hemorrhage:
severe hemorrhage:

retinal detachment.

 Chinese medicine Nutrition reinforcement: vitamin Remove causes Corticosteroid laser photocoagulation Surgery: vitrectomy

Coats disease
Or retinal telangiectasiaexternal exudative retinopathy Often seen in boyunilateral Youngsters and adults may also suffer No heredityno relation with systemic angiopathy Etiology: unknown
Malformation and telangiectasia of capillary in external plexiform layer Permeability alteration and vessel rupture Exudation

Clinical manifestation
Visual disturbancestrabismusleucoria
Fundus extensive yellow-white lipid exudation with faring cholesterol crystalcapillary and vein dilatemicroaneurysmcapillary 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

 Etiologyunknown
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 damagehereditymetabolismnutrient factors

Mechanism:
Decreased phagocytosis of RPE leading to dusen.

Drusen can cause damage of Bruchs membraneCNV and fibrocyte proliferation

Destruction of choroidal capillaryBruchs membraneRPE and photoreceptor

Clinical presentation
Visual acuity: decreased VA, metamorphopsia, micropsia Visual field: central scotoma Fundus: Dry:
Wet:

drusen, RPE change gray-yellow CNV under retina of posterior pole associated with dark red subretinal hemorrhagewhich covers CNV sometimes

FFA: CNV leakagebleeding

 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

rhegmatogenoustractionexudative

Rhegmatogenous retinal detachment formation

retinal degeneration liquified vitreous retinal holeRD aging high myopia ocular trauma

Basis

Incentives

 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 tearsby photocoagulation condensationelectric coagulation Scleral buckling Vitrectomy

Retinitis Pigmentosa
Chronic ,progressive,inherited disease
Cone cellrod 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
Onset age Eyes involved Microcirculation abnormality 90%<3 yrs 30%both none

Coats disease
>6-8 yrs 95%single Diffuse microanuerysm

Cholesterol cystal
Calcification B-us

none
+

Subretinal ,obvious
-

Solid tumor Without solid 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