Block 5 - Retinal Pathology

Front 1

Full-thickness retinal break
May be complicated by proliferative vitreoretinopathy: formation of epiretinal or subretinal membranes by retinal glial cells (Muller cells) or retinal pigment epithelial cells

Back 1

Rhegmatogenous (rhegma = rent or fissure, arising from bursting or fractionating of an organ)

Front 2

Separation of posterior hyaloid from internal limiting membrane
Normal occurrence in aging eye

Back 2

Vitreous detachment leading to a rhegma

Front 3

Retinal detachment without retinal break
May complicate retinal vascular disorders associated with significant exudation and any condition that damages the RPE and permits fluid to leak from choroidal circulation beneath the retina

Back 3

Non-rhegmatogeous

Front 4

May lead to loss of photoreceptor outer segments, gliosis, development of microcystic spaces with the detached retina

Back 4

Chronic retinal detachment

Front 5

In a retinal detachment, what is indicated by the absence of the outer segments of the photoreceptors?

Back 5

chronic retinal detachment

Front 6

Three sclerotomies are made on the outside of the eye, one that allows fluid to infuse into the eye, and two others that are used to introduce instruments into the back part of the eye.
Using a light pipe and a vitreous cutter all of the central and peripheral vitreous is removed and all of the vitreoretinal traction on any of the breaks excised.

Back 6

Vitrectomy

Front 7

What is an adverseffect if vitrectomy?

Back 7

cataracts

Front 8

What is the success rate of 1st time vitrectomy?

Back 8

90%

Front 9

How many patients over 50 will develop cataracts post vitrectomy?

Back 9

virtually ALL

Front 10

sealing the sensory retina to the pigment epithelium and choroid by a freezing probe applied to the sclera

Back 10

cryopexy

Front 11

After a retrobulbar injection or infusion of lidocaine and bupivacaine, cryoretinopexy to the retinal breaks is performed, followed first by suturing of an exoplant to the outside of the sclera that indents the wall of the eye and closes the retinal breaks, and then by drainage of subretinal fluid, if needed (show picture 7). The scleral indentation created by the sutured exoplant not only helps close the retinal break and allow the chorioretinal retinal adhesion to form, but also helps reduce the vitreoretinal traction present.

Back 11

scleral buckle

Front 12

Scleral buckle placement leads to successful retinal reattachment approximately ............. of the time after one surgery. In cases of failure, most patients are treated with ..............

Back 12

80-90% of the time

vitrectomy

Front 13

Branch of Opthalmic  Posterior Ciliary Arteries supply choroid without passing through optic nerve
Supply MACULA and Anterior Optic Nerve
Supply outer retina and RPE.

Back 13

Uveal blood supply

Front 14

Branches of Central Retinal artery (distal Ophthalmic)
Supply Inner retina

Back 14

retinal blood supply

Front 15

A focal swelling of the nerve fiber layer is occupied by numerous red to pink cytoid bodies (arrowheads), bulbous ends of severed axons.
Hemorrhage (arrows) surrounding the nerve fiber layer infarct is a variable and inconsistent finding.

Back 15

Cotton Wool Spots

Front 16

Thickening of the basement membrane of epithelium of pars plicata of ciliary body is reliable histologic marker of ...... ....... in the eye

Back 16

Diabetes Mellitus

Front 17

aldose reductase
High CHO-------------> Sorbitol

Back 17

Sorbitol causes a fibrotic scar response via osmotic damage

Front 18

Plasma (with proteins) leaked from damaged vessels, fluid reabsorbed, which left the proteins in retina

Back 18

Hard Exudates

Front 19

Keywords: Microaneurysms, dot-blot hemorrhages, Cotton wool spots, Hard Exudates

Back 19

Keywords for Diabetic Retinopathy

Front 20

When should diabetics get optho consult?

Back 20

DM II immediately

DM I at 3 y.o.

Front 21

Upregulation of vascular endothelial growth factor (VEGF) and retinal angiogenesis
Basement membrane of retinal vessels is thickened
Leaky vessels macular edema, hemorrhagic exudates in outer plexiform layer

Back 21

Retinal vascular disease in DM

Front 22

Dot Blot Hemorrhages
Cotton Wool Spots
Hard Exudates
No neovascularization

Back 22

No-proliferative DM Retinopathy

Front 23

Vessel disease leads to peripheral retinal ischemia
Ischemic Tissue produces VEGF.
VEGF = Neovascularization = new vessels sprouting from existing vessels on surface of optic nerve head, peripapillary, or peripheral retina

Back 23

Proliferative Diabetic Retinopathy

Front 24

What is the compositio of hard exudates?

Back 24

blood protein spilled by leaky vessels

Front 25

posterior vitreous detachment might exert traction on new vessels --->........

Back 25

vitreous hemorrhage

Front 26

Blush of thin-walled vessels not accompanied by a fibrous stroma

Back 26

Retinal neo vascularization

Front 27

Neovascularization occurs inbetween the ......... ........ ........ and the posterior hyaloid membrane.

Back 27

Internal Limiting Membrane

Front 28

What is the purpose and consequence of Pan-photo coagulation?

Back 28

Kill the peripheral Ischemic Tissue = decrease level of VEGF = decrease neovascularization

Zapped retina does NOT function

Front 29

...................... leads to can lead to macular edema.

Back 29

Neovascularization

Front 30

Monoclonal Antibody that recognizes and blocks VEGF.
Used in Colorectal, lung, breast, renal cancer.
Used in Proliferative Retinopathy, “Wet” Macular Degeneration, Macular Edema and ROP.

Back 30

Avastin (bevacizumab)

Front 31

WHat is the premature birth cut off for risk of ROP?

Back 31

> 32 weeks not at risk

Front 32

What is the best treatment for ROP?

Back 32

Avastin

Front 33

Proliferative
-Retinal neovascularization “Sea Fans”

Nonproliferative
-Intraretinal angiopathic change

Final common pathway in both is vascular occlusion
↓ O2 saturation in peripheral retina --> red cell deformity --> microvascular occlusion

Complications: neovascularization (retinal angiogenesis) due to VEGF release, hemorrhage, traction
-->retinal detachment

Back 33

Sickle Retinopathy

Front 34

Heart or ulcerated plaque in carotid arteries can send an emboli  central retinal artery

Back 34

Hollenhorst plaques

Front 35

Central retinal artery occlusion --> cherry-red spot of fovea and foveola seen on exam as retina becomes opaque WHY IS THIS?

Back 35

exposure of the Choroid which is dark

Front 36

In a central retinal artery occlusion, where is the likely pupillary defect, afferent or efferent?

Back 36

Afferent

Front 37

"Blood and Thunder Fundus"

Back 37

Retinal Vein Occlusion

Front 38

↓ IOP via massage, acetazolamide in hopes of breaking up intravascular clot

Injection of thrombolytic agents into ophthalmic artery

Have approximately 1 hour to fix

Back 38

Tx of Central Retinal Artery Occlusion

Front 39

No effective acute treatment

Complication from macular edema and neovascularization treated with photocoagulation, intraocular triamcinolone, and Avastin.

Back 39

Tx of Central Retinal Vein Occlusion

Front 40

A 75-year-old man presents with the gradual loss of vision in both eyes. He has trouble seeing when he drives and tries to read. Exam reveals the presence of drusen, with round patches of geographic atrophy of the retina.

Back 40

Dry AMD

Front 41

A 86-year-old man presents with acute decrease in vision in the right eye with distortion of straight lines. Dilated retinal exam reveals subretinal hemorrhage and neovacularization appearing greenish-grey.

Back 41

Wet AMD

Front 42

Retinal pigment epithelium (RPE)

Bruch’s membrane (innermost choroidal layer)
-Contains basement membrane of RPE

Choriocapillaris
-Innermost layer of the choroidal vasculature

Back 42

Anatomy involved in Macular Degeneration

Front 43

Discrete deposits in Bruch’s membrane known as drusen (German Druse- stony nodule, geode) which consists of amorphous granular material, collagen etc.

Atrophy of the RPE

10-20% develop choroidal neovascular membranes

Back 43

Atrophic (dry) Macular Degeneration

Front 44

Choroidal neovascular membranes-->leak-->subretinal fluid

Back 44

Exudative (wet) Macualr Degeneration

Front 45

To what cancer is high dose beta carotene linked?

Back 45

Lung cancer

Front 46

-Hereditary, progressive retinal degeneration with pigment infiltration that primarily affects photoreceptors (rods and cones) and pigment epithelium function

-Night blindness(nyctalopia) and peripheral vision are lost progressively --> constricted visual field

-Can start in first or second decade of life

-Most patients meet criteria for legal blindness by age 40, due to narrowing of visual fields

Back 46

Retinitis Pigmentosa

Front 47

Bone spicules are caused by....

Back 47

RPE migration into the innner retina following photreceptor loss.

Front 48

RPE migration into the innner retina following photreceptor loss.

Back 48

Abetalipoproteinemia (Bassen-Kornzweig syndrome)

Front 49

Enzyme that breaks down phytanic acid is absent. Patients develop RP, cerebellar ataxia, and peripheral polyneuropathy

Back 49

Phytanic acid oxidase deficiency (Refsum disease)

Front 50

Hereditary syndrome with RP, ataxia, and vitamin E deficiency

Back 50

Alpha-tocopherol transport protein deficiency

Front 51

RP and congenital sensorineural deafness.

Back 51

User Syndrome

Front 52

Questionable benefit of Vitamin A (palmate form)

Omega-3 fatty acid

Gene therapy

Retinal cell transplantation

Retinal prosthesis

Back 52

Possible treatments for RP

Front 53

What are possbile treatments for choroidal melanoma?

Back 53

Plaque brachy therapy to enucleation

Front 54

Most common primary intraocular malignancy of childhood (1 in 15,000 to 16,000 live births)

95% diagnosed before age 5
Sporadic and hereditary forms (germline mutation)

13q14, encodes a nuclear protein that acts as a tumor suppressor

25% cases are bilateral

Presents most commonly with leukocoria (54%) and strabismus (19%)

Back 54

Rentinoblastoma

Front 55

What are two possible therapies for RB besides enucleation?

Back 55

External beam radiotherapy, and Plaque radiotherapy

Front 56

The characteristic pathologic finding is a chalky, white-gray retinal mass with a soft, friable consistency

Back 56

RB

Front 57

Which parent contributes the defective RB gene?

Back 57

mom

Front 58

Bilateral RB is always...

Back 58

inherited

Front 59

Exudative Retinitis

Unilateral Leukocoria in a 11 year old male

Treatment: Laser therapy and Cryotherapy, to Enucleation

Back 59

Coats Disease

Front 60

a whitish or pinkish fibrovascular plaque may be present on the posterior surface of the lens. Depending on how large the plaque is, drawn-in ciliary processes can also be seen. The lens is usually clear, but a cataract can develop over time.
2. Persistent pupillary membranes and Mittendorf dots are often observed.

Back 60

Anterior form of PFV

Front 61

a stalk of condensed, or hyperplastic, vitreous attaches at the optic nerve and extends into the vitreous. From there, it can be followed anteriorly to the posterior lens surface or just adjacent to it.

Back 61

posterior form of PFV

Front 62

If only the anterior extension of the primary vitreous remains...

Back 62

microphthalmia, retrolental membrane, and a high incidence of glaucoma.

Front 63

If the fibrovascular stalk attached to the optic nerve remains....

Back 63

retinal fold, traction and detachment.

Front 64

The tertiary vitreous is secreted last and condenses to form the .....

Back 64

suspensory ligaments of the lens (zonules).

Front 65

The hyaloid artery and its derivatives, the vasa hyaloidea propria and tunica vasculosa lentis, develop from mesodermally derived cells during formation of the primary vitreous.
Following this.....

Back 65

the inner retina begins liberating collagen, and Mueller cells and hyalocytes secrete the hyaluronic acid of the secondary vitreous. Formation of the secondary vitreous displaces the primary vitreous centrally, the condensed junction of which forms Cloquet's canal.

Front 66

Formation of the secondary vitreous is completed by the end of the first trimester, whereupon the vascular components of the hyaloid system begin a slow process of involution regulated in part by .....

Back 66

macrophage-mediated apoptosis.

Front 67

The primary vitreous begins to develop at about 3 weeks of age and is complete by about .......

Back 67

9 weeks.

Front 68

When does the abnormality of PHV occur?

Back 68

4-6 mos gestation

Front 69

(1) the hyaloid artery, (2) the vasa hyaloidea propria, which is derived from the vessels that pass into the peripheral parts of the vitreous and enter the vascular coats of the lens at its equator, and (3) the anterior ciliary vessels, which originate from the major arterial circle of the iris.

Back 69

the vascular channels forming the tunica

Front 70

The anterior side is supplied by
the ...

Back 70

Ciliary system

Front 71

The posterior side is supplied by the ....

Back 71

Hyaloidean system

Front 72

Early, can see loss of spontaneous venous pulsations
Splinter hemorrhages in the nerve fiber layer, at or beyond the disc margin, may be seen early

Back 72

Early papilledema

Front 73

Disc becomes elevated, the cup is obliterated, and the disc margins become obscured.

Blood vessels are buried as they course the disc.

Serpentine engorgement of retinal veins is evident, and the disc appears hyperemic.

Edema extends into the retina, giving the appearance of an enlarged optic nerve head.

Multiple flame hemorrhages and cotton wool spots, resulting from nerve fiber infarction, appear

Back 73

Fully developed papilledema

Front 74

*NORMAL MRI.

Symptoms:

Headache
Nausea
Vomiting
Pulsitile Tinnitus
Altered Vision

Back 74

You're probably dealing with pseudotumor cerebri

Front 75

Lumbar Puncture: *>25mm HG

Papilledema

Back 75

these happen with pseudo tumor cerebri in an absence of hemorrhage or mass

Front 76

Female
*Obese
Doxycycline
OCPs
Pregnancy

Back 76

risk factors for pseudotumor cerebri

Front 77

What is the treatment for pseudotumor cerebri?

Back 77

acetazolamide

Front 78

Inflammatory, demyelinating condition of the optic nerve that causes acute, usually monocular (90%), visual loss and eye pain
Presenting feature of Multiple Sclerosis in 15-20% of patients and occurs in 50% at some time during the course of their illness

Back 78

Optic Neuritis

Front 79

Perivascular cuffing
Edema in the myelinated nerve sheaths
Myelin breakdown
Inflammation of retinal vascular endothelium retinal vein sheathing
Myelin loss exceeds axonal loss
Believed to be immune mediated
T cell activation  cytokine release  inflammation
B- cell activation against myelin basic protein in CSF of patients

Back 79

Optic Neuritis

Front 80

Perivascular cuffing

Edema in the myelinated nerve sheaths

Myelin breakdown

Inflammation of retinal vascular endothelium--> retinal vein sheathing

Myelin loss exceeds axonal loss

Believed to be immune mediated
-T cell activation --> cytokine release --> inflammation
-B- cell activation against myelin basic protein in CSF of patients with optic neuritis

Back 80

Optic Neuritis

Front 81

An atrophic, internally disorganized eye

Trauma
Instant increase in ocular pressure -->stretching of globe with pressure to iris --> blood in the anterior chamber from tears in vessels of ciliary body or iris = hyphema

Intraocular inflammation

Chronic retinal detachment

Back 81

pthisis bulbi

Front 82

Ciliochoroidal effusion
Exudate or blood in potential space between ciliary body and sclera and choroid and sclera

Cyclitic membrane
Extends across the eye from one aspect of ciliary body to other

Chronic retinal detachment

Optic nerve atrophy

Back 82

Characteristics of Pthisis Bulbi

Front 83

Osseous metaplasia of retinal pigment epithelium (eye bones)

Thickened sclera, especially posteriorly

Hypotony
Low intraocular pressure, which causes the normal pull of extraocular muscles to render the eye square rather than round

Back 83

Characteristics of Pthisis Bulbi

Front 84

A 28 yr old male with HIV presents to your clinic with complaints of altered vision. He has not been compliant with his medications. Routine CD4+ count is 45 cells/mm3.
Visual acuity: 20/30 OD; LP in OS.
Visual field defects do not follow any pattern.

Back 84

CMV retinitis

Front 85

Classically seen in CD4+ counts less than 50.

Symptoms:
Blurry vision to central/peripheral loss.

Signs:
Perivascular cuffing
Hemorrhage

Treatment:
Intravitreal ganciclovir implant.

Back 85

CMV retinitis

Front 86

Fluorescein strip (orange) stain of eye.

Leaking Vitreous will create a lighter colored (green) flow.

Significant for piercing trauma.

Back 86

Seidel Test