Cancers + HIV + Glaucoma + RD

Leukocoria - what cases have it?

Literally "white pupil"

• A clinical sign where normal red reflex of eye is replaced by a white reflection

• Almost always a pediatric emergency until proven otherwise

Toxocariasis (white puppy + streak)

Coat’s Disease (young boys)

Retinoblastoma (cancer)

Coat’s disease?

Progressive disease (worsens over time)

Starts in young boys <10 yrs old

Vascular abnormality → causing leakage of EXUDATES

• Exudates become thick + plentiful

• Severe enough = cause EXUDATIVE RETINAL DETACHMENT

  • Exudative RD (aka Serous RD) = type of retinal detachment where fluid collects underneath retina without hole or tear in the tissue

  • Leaking blood vessels or an inflamed choroid pumps fluid into subretinal space

  • "Big Three" Causes of Exudative RD:

1. Inflammatory (Vogt-Koyanagi-Harada)

• Patient: Often Hispanic, Asian, or Native American

• Signs: Bilateral exudative RDs, "sunset glow" fundus + systemic signs: hearing loss (tinnitus) or vitiligo (white patches of skin/hair)

2. Neoplastic (Tumors)

• Cause: Choroidal Melanoma or Choroidal Hemangioma

• Logic: The tumor is highly vascular + "leaky" causing fluid to build up around base of mass

3. Vascular (Coats' Disease)

• Patient: Young boys (as we discussed with Leukocoria)

• Logic: The "lightbulb" telangiectatic vessels leak massive amounts of lipid and serous fluid, lifting the retina

Also: optic pits, morning glory syndrome, CSCR

Coat’s disease symptoms?

Poor vision

Coat’s disease signs?

Leukocoria

• HUGE sign

If onset early enough → may have strabismus

Telangectasia (abnormal dilated capilaries)

• "Lightbulb" telangiectasia

Multiple clumped lipid exudates

• Simplest terms: Exudates are "leaky" debris from your blood

  • Specifically - composed of lipids (fats) and proteins that have escaped from damaged/abnormal blood vessels

Exudative retinal detachments also possible

Advanced cases may have: Glaucoma + blind, painful eye

Coat’s Signs Staging:

1. Telangectasia only

2. Exudates

3. Exudative RD

4. Glaucoma

5. End-stage disease

Coat’s disease tx?

***Retinoblastoma MUST BE RULED OUT BEFORE ANY TX

Control leaky blood vessels:

• Laser photocoagulation

• Cryotherapy (tissue freezing)

Glaucoma:

• Tx if present

Retinoblastoma?

MOST common primary intraocular malignancy (cancer that develops inside eye) in kids

• Diagnosis: usually before age of 5 yrs

• Basophilic cells

Retinoblastoma symptoms?

Parents see Leukocoria!!!! (white pupil)

Possible strabmisus

Retinoblastoma signs?

Tumor

• Unilateral

• Dome-shaped yellow-white globular mass w/calcification

• Reduced VA, recurrent uveitis from uvea invaded, psuedo-hypopyon

  • Looks like hypopyon (white layer in anterior chamber) BUT:

    • NOT inflammatory WBCs

    • Malignant cells/tumor cells settling

• Secondary glaucoma w/bupthalmos (globe enlargement)

• IF left untreated = tumor will spread to invade orbit + adjacent bony structures

Retinoblastoma tx?

Confirm diagnosis w/exam under anesthesia:

• B-scan

• MRI

Tx:

• Surgical: Enucleation most common (remove eyeball, leave EOMs + other orbital content intact)

  • VS. Exteneration = complete removal of globe + all eye socket contents

• Chemotherapy

• Good prognosis; 90% survival rate

Primary Ocular Lymphoma (Primary CNS Lymphoma)?

Initially begins in brain!

• Optic nerve is extension of the brain → so this tumor can manifest in eye also

• MOST PATIENTS will DIE wthin 2 yrs diagnosis

Primary Ocular Lymphoma (Primary CNS Lymphoma) symptoms?

Blurry vision

Floaters

NO inflammatory issues (pain/redness/photophobia)

Primary Ocular Lymphoma (Primary CNS Lymphoma) signs?

Vitreous cells

Subretinal + Sub-RPE lesions

Primary Ocular Lymphoma (Primary CNS Lymphoma) tx?

(+) Biopsy of vitreous - to confirm before any start of tx

Systemic workup with oncologist evaluation:

• MRI brain

• Lumbar puncture

• Bone marrow biopsy

Then:

• Systemic chemotherapy + radiation

Choroidal melanoma?

MOST common primary intraocular malignancy (cancer that develops inside eye) in ADULTS

• Associated with metastasis - most common sites:

  • FIRST + most common site = LIVER

  • Then lungs, bone, skin, CNS

Choroidal melanoma symptoms?

Asymptomatic IF located in periphery

Otherwise - blurry vision, metamorphopsia, VF loss, floaters, photopsia (flashes of light)

Choroidal melanoma signs?

Dome shape elevations:

• May/may not be pigmented

• Overlying yellow/orange lipofuscin

• Indistinct borders

• Serous/exudative retinal detachment

Tumors most likely to metastasis (spread):

• Increased thickness

• Involve ciliary body

• CLOSE proximaty to nerve

• Show growth

Risk Factors: "To Find Small Ocular Melanoma" (TFSOM)

If a lesion has 3 or more of these, there is a 50% chance it is a melanoma.

• T - Thickness: Greater than 2 mm.

• F - Fluid: Subretinal fluid (serous detachment).

• S - Symptoms: Flashes, floaters, or blurred vision.

• O - Orange Pigment: Lipofuscin sitting on the surface (this is a huge red flag).

• M - Margin: The lesion is touching or within 3 mm of the Optic Disc.

Choroidal melanoma tx?

Oncologist for systemic evaluation

• Plaque brachytherapy

• Proton beam therapy

• Enucleation to prevent metastasis

COMs study

• No diff in 5 yr mortality rate for people tx with brachytherapy or enucleation in medium-large sized melanomas

  • THUS: we usually try to save eye with radiation

• Radiation therapy BEFORE enucleation doesn’t improve survival rates

Choroidal metastasis?

Secondary Malignancy!

Most common intraocular malignancy in adults

Cancer started somewhere else (like Breast or Lung) and traveled through the blood to eye

***Most common sites of PRIMARY tumor = women (breast) and men (lung)

Choroidal metastasis prognosis?

Poor ☹

Most people die within 1 yr of diagnosis

Choroidal metastasis signs?

Yellow-white elevated lesions with pigmented clumps + possible overlying serous/exudative RD

Vitreous is usually crystal clear (unlike Primary ocular lymphoma which is 2 yr survival rate)

Choroidal metastasis tx?

Tx underlying cancer with oncologist + chemotherapy

Choroidal nevus?

Typically benign!

• Similar to freckles

• Disappears with Red-free filter (Green one)

  • Not visible with Green (Red-free) filter = NOT in RPE but instead in choroid

  • VS. CHRPE → IS visible with Green (Red-free) filter = in RPE

Choroidal nevus symptoms?

Asymptomatic

Choroidal nevus signs?

Flat, grey-brown spot ANYWHERE in retina

WELL-demarcated borders

***Very small fraction of choroidal nevi CAN grow into choroidal melanomas

• Higher risk nevi:

  • Having symptoms (flashes, floaters, blur, etc.)

  • Close to optic nerve (recall: within 3mm DD)

  • Change/growth

  • Elevation

  • Overlying orange pigment (lipofuscin)

  • Subretinal fluid

Choroidal nevus tx?

Document + monitor with FUNDUS photography!

• Follow up in 3 months first

• Then 1-12 months after depending on risk level

Congenital hypertrophy of retinal pigment epithelium? (CHRPE)

Congenital hypertrophy

• THUS: true CHRPE present at birth

WILL NOT disappear with red-free (green) filter - will still be visible!!!

• THUS: located in RPE

CHRPE symptoms?

Asymptomatic

CHRPE signs?

Singular, flat brown “freckle”

• Not disappearing w/red-free (green) filter

If bilateral, multiple, small CHRPE’s = “bear tracks” (also benign)

• VS. FAP lesions (familial adenomatous polyposis aka Gardner’s syndrome) = more ovoid with comet shape (also bilateral + multiple)

CHRPE tx?

1 solitary CHRPE

• Monitor + photograph for documentation

Any lesions that look like FAP

• Refer for COLONOSCOPY

• Gardener’s Syndrome/FAP (genetic) typically diagnosed in 20s → will lead to colon cancer by 50s

• Ocular looks like: multiple, bilateral CHRPEs

• Systemically: colon polyps, soft tissue tumors (neurofibromas, cysts), skeletal hamartomas

Choroideremia?

X-linked recessive inheritance (genetic)

• MEN affected but WOMEN are carriers

• Men age 20-30 yrs → progresses to legal blindness by age 50-60 yrs

Choroideremia symptoms?

Night blindness (nyctalopia)

Light sensitivity (photophobia)

Other causes of Night blindness (nyctalopia)?

Choroidemia ✔

Birdshot Chorioretinopathy ✔

Thioridazine Retinopathy

Retinitis Pigmentosa

Gyrate atrophy (progressive myopia) ✔

Enhanced S-cone Syndrome/Goldmann-Favre Syndrome

Choroideremia signs?

Peripheral atrophy of choriocapillaries

• Revealing underlying choroidal vessels

• Progressive

• Spares macula till late in disease

Choroideremia tx?

No treatment ☹

Low vision consult + genetic testing

Central Areolar Choroidal Dystrophy?

Autosomal Dominant inheritance (genetic)

• Starts 30-40 yrs → progresses to severe vision loss by age 60-70 yrs

Central Areolar Choroidal Dystrophy symptoms?

Blurry vision

Central scotoma

Central Areolar Choroidal Dystrophy signs?

Early stages:

• Hypopigmentation of macula → progress to geographic atrophy with well-defined borders, slowly enlarging to reveal underlying choroidal vasculature

• Decreasing vision from 20/25 → 20/200

• Bilateral + symmetric

• Almost looks like choroidermia inverted (now affecting macula first)

Central Areolar Choroidal Dystrophy tx?

No treatment ☹

Low vision consult + genetic testing

Choroidal Detachment?

Choroid separated from sclera

Looks like a thick, smooth, brown, dome-shaped elevation (VS. RD =

Bullous orange-brown peripheral elevation

2 types! BOTH look same on DFE!!

• Serous

• Hemorraghic

RD vs CD (detachments)?

• Think RD if: "Flashes and floaters," "High myope," "History of lattice degeneration," or "Recent trauma"

• Think CD if: "Recent Glaucoma Surgery," "Low IOP (Hypotony)" or "Inflammatory disease"

Serous Choroidal Detachment (Choroidal Effusion)?

LOW IOP (hypotony) secondary to puncture wound to globe

• Or after glaucoma surgery like a trabeculectomy

WILL transilluminate

• Placing bright light (transilluminator) against sclera + looking through pupil to see if light shines through mass

Symptoms?

• Asymptomatic if peripheral or mild

• Severe vision loss if severe (i.e. kissing)

  • Kissing Choroids: If detachment is massive, the two sides of choroid can actually touch in middle of vitreous

Tx?

• Close wound

• Prevent further IOP decrease

• Bandage CL, Suture, Cyanoacrylate glue

Hemorrhagic Choroidal Detachment?

HIGH IOP secondary to hemorrhaging during ANT SEG surgery

• Blood pools between choroid + sclera (limited area in globe) → pressure increases → anterior chamber narrows as iris pushes forward

• WILL NOT transilluminate

Symptoms?

• SEVERE pain

• RAPIDLY decreased vision

• Red + inflammed eye

Tx?

• Close wound to prevent further hemorrhaging

• Cycloplegic to prevent synechiae

• Sclerotomy (surgical scalera cutting) possibly - to allow blood drainage

• Ocular hypotensives possibly - to reduce IOP

Any remaining choroid issues haven’t gone over yet?

Candidas (other flashcard set - sick/IV patients)

Serpinginous Choroidopathy (similar presentation to TB which is an infection, gran panuveitis, antibiotics, RICE)

Birdshot Chorioretinopathy (Granulomotous, white dot syndrome)

Serpinginous Choroidopathy?

Idiopathic

MEN - 50-60 yrs old

HLA-B7

• ALSO think Presumed Ocular Histoplamosis!

***TB can cause similar fundus appearance - RULE OUT***

Serpinginous Choroidopathy symptoms?

Central blurry vision

Scotoma that may be unilateral or bilateral

Serpinginous Choroidopathy signs?

Recurrent disease

Lesions:

• Begin at optic nerve

• Extend in SERPENTINE manner towards macula

ACTIVE lesions = grey-white color

DORMANT lesions = atrophy + scar

CNVM = develops in 25% of cases

Serpinginous Choroidopathy tx?

Chronic + relapsing course

TX starts with steroids (oral or local) → transition to immunomodulating meds (steroid-sparing)

If CNVM - tx with Anti-VEGF

Birdshot Chorioretinopathy (Vitiliginous Chorioretinitis)?

White dot syndrome!

Bilateral, chronic chorio-retinal inflammatory disease

White females - 40-60 yrs

HLA-A29

Granulomatous Posterior Uveitis (***NO KPs/mutton fat/busacca)

Birdshot Chorioretinopathy symptoms?

Blur

Photopsia (flashes)

Nyctalopia (night blindness)

Birdshot Chorioretinopathy signs?

Multiple, creamy yellow-white spots in fundus periphery

• SPARE macula

• < or equal to = size of optic disc

May have Vitritis, Retinal vasculitis, Cystoid Macular Edema

Atrophy + choroidal depigmentation → advanced cases

Birdshot Chorioretinopathy tx?

TX starts with systemic steroids → transition to immunomodulating meds (steroid-sparing)

Periocular + IV steroids = for CME especially

Gyrate Atrophy?

Autosomal Recessive

Genetic mutation of ornithine aminotransferase (OAT)

• Causing urine + plasma ornithine levels increased: 10-20x greater than norm = toxic to RPE and Choroid

Onset → first 10 yrs of life

• Afflicts entire retina between 40-50 yrs without treatment

Gyrate Atrophy symptoms?

Nyctalopia (night blindness)

Constricted visual field

Gyrate Atrophy signs?

Progressive myopia

Scalloped chorioretinal atrophy peripherally

• Enlarge + migrate posteriorly

• Effects macula last

Gyrate Atrophy tx?

Argine + Protein = make ornithine

• THUS: we restrict these in diet to lower levels of ornithine

Also take Vitamin B6 supplement

Pathological Myopia/Myopic Degeneration?

High grade + progressive myopia

Hereditary component

Eyeball will not stop elongating

Pts > -6.00D myopia or > 26mm axial length

***NOTE: NOT every high myopia patient has pathological myopia

• JUST ones where myopia CONTINUES to progress + present certain signs

Pathological Myopia/Myopic Degeneration symptoms?

May complain decreased vision

Pathological Myopia/Myopic Degeneration signs?

Tilted disc

• Stretching makes optic nerve look "cupped" or pale → can mimic glaucoma even if IOP normal

• PPA (RPE pulls away from the nerve)

Lacquer cracks

• Mechanical breaks in Bruch's Membrane

• Look like yellow-white "lightning bolts" radiating from nerve

• Lacquer Cracks = "Mechanical Stretching" (Fine, irregular, yellow-white "lightning bolt" lines. Usually found in Macula or radiating from Optic Nerve) VS. Angioid Streaks = "Chemical Brittleness" (Thicker, darker, "vessel-like" streaks radiating from Optic Nerve)

CNVM

• While lacquer crack is a physical break in Bruch's Membrane - (think of Bruch's as "fence" keeping choroidal blood vessels out of retina) → once the fence "cracks" from stretching, the vessels can keep growing through gap then leak

• New, fragile, "garbage" blood vessels

• Start in Choroid (layer under retina) → Break: Grow upward through a break in Bruch’s Membrane (the "fence") → Leak: Because these new vessels are poorly built, they leak fluid (edema), lipids (exudates), and blood (hemorrhage) into subretinal space

• Look: Small, grayish-green "tuft" or a small red subretinal hemorrhage

• Symptom: Metamorphopsia (wavy lines) or new scotoma (blind spot)

  

Fuchs’ spots (dark spots from RPE hyperplasia)

• If lacquer crack bleeds → leaves a pigmented scar in macula

Posterior staphyloma (outpouching of post seg as white circles = visible choroid)

• NOT same as gyrate atrophy = this usually has high myopia too, BUT this atrophy isn't caused by the refractive error— it's metabolic toxic buildup

  

Retinal holes and detachments

PVD

Lattice degeneration

Pathological myopia tx?

Contact lenses preferred over glasses

• Less image minification and prismatic effects

Glasses wearers - recommend polycarb

CNVM → Anti-VEGF

Retinal holes + detachments → tx accordingly

Chorioretinal atrophy → no tx

May benefit from Low Vision devices

CNVM VS. NVD/NVI/PDR neo

Who gets CNVM?

If the pathology is Under the retina (Choroid/Bruch's), it's CNVM

If it's In/On retina, it's Neovascularization (NV).

Common CNVM Causes (CHESS):

• C: Choroidal Rupture (Trauma).

• H: Histoplasmosis (POHS).

• E: Exudative AMD (Wet AMD).

• S: Serpiginous Choroiditis.

• S: Sight-threatening Myopia (Pathologic Myopia/Lacquer Cracks).

Hard exudates are simply lipids and proteins left behind when watery part of blood (serum) leaks out of vessel + gets reabsorbed

• "Salt" left behind after "seawater" evaporates

---

"Basement" Leak (CNVM in AMD/Myopia)

• Source: Fragile new vessels growing from Choroid up through Bruch's Membrane

• Location: Fluid/exudates sit under retina (subretinal)

• Look: Localized, dirty-gray-green "tuft" with exudates/blood gathered right around that specific spot in Macula

---

"Wall" Leak (Diabetes & HTN)

• Source: Pre-existing Retinal Capillaries that have become leaky due to high sugar (Diabetes) or high pressure (HTN)

• Location: Fluid/exudates sit inside retina (Intraretinal)

• Look:

  • Diabetes: Exudates often form Circinate Ring (circle of yellow spots) around leaking microaneurysm

  • Summary: The Diabetic "Leak" Timeline

    1. Sugar damages pericytes → Capillaries get weak.

    2. Weak capillaries → Microaneurysms → CME and Dot/Blot Hemes.

    3. Capillaries close up → Retina loses oxygen (Ischemia).

    4. Ischemia → VEGF is released.

    5. VEGF → Neovascularization → Vitreous Hemorrhage

       1. Vitreous Hemorrhage or Tractional RD

  • HTN: In severe cases (Grade 4), you see Macular Star

    • Fan-like spray of exudates radiating from fovea because fluid is being pushed into Henle's layer of retina

    • Also in HTN, pressure so high it pops superficial capillaries in Nerve Fiber Layer (NFL) = why HTN gives you Flame Hemorrhages, whereas Diabetes gives you Dot and Blots

---

***Blood-retinal barrier in a diabetic is failing in inner retina (the capillaries)

• Oxygen problem (ischemia) is happening in retina itself, so new vessels grow on top of retina (NVD/NVE).

• NVD/NVE (Diabetes/CRVO): These vessels grow on top of retina or into vitreous

  • Visual clue: They look like "frond" of sea anemone or tangled "fan" of fine, red vessels

    • Follow retinal veins

  • Vessel color: Bright red

  • “Top”

***In AMD or Pathologic Myopia, damage is to Bruch's Membrane (the basement floor)

• Because floor is cracked, vessels from the basement (Choroid) can grow up into the room.

• CNVM (AMD/Myopia): These vessels grow under retina

  • Visual clue: You usually don't see individual vessels. Instead, you see a dirty-gray, greenish, or yellowish "tuft" or a mound. Looks like something is pushing retina up from basement

  • Vessel color: Subdued gray/green/dark

  • “Bottom”

  • In AMD, you won’t see "cracks" like angioid or lacquer; you’ll see Drusen (yellow pebbles) instead

  • Localized fluid (subretinal on OCT) and "random" hard exudates clustered right around lesion because it is a leaky "faucet"

CNVM vs Diabetic/HTN neo vs Coats

Coats CNV vs. Diabetics?

CNVM are similar because both involve Retinal vessels (not choroidal; but Intraretinal) becoming leaky. However, the pattern and cause are different:

• "How" (Similarity): Both cases, the blood-retinal barrier is broken. Serum leaks out of retinal capillaries, leading to lipid formation = mix of Hard Exudates/Edema (fluid).

• "Where" (Difference):

  • Diabetes: Leaks are usually small, scattered microaneurysms across posterior pole. "Dot + Blot" hemorrhages and circinate rings of exudates.

  • Coats': Leaks are massive, localized "clusters" of Telangiectatic vessels (often in temporal periphery). Like one giant broken fire hydrant vs. hundred tiny dripping faucets.

The Difference from “CHESS” CNVM: Comes from under the floor (the choroid)

• VS. the "lightbulb" vessels in Coats' are part of retina's own plumbing.

• "Sea of Yellow": Because these vessels are so high up and leak SOOO massively, hard exudates (lipids) often settle deep into the outer plexiform layer or even subretinal space, which is why you see massive yellow "mountain" pushing retina forward

---

Feature	Coats' Disease	Diabetic Retinopathy
Vessel Look	"Lightbulbs" (Telangiectasia)	Microaneurysms / IRMA
Exudates	Massive / Sheets of Yellow	Small / Circinate Rings
Laterality	Unilateral (85%+)	Bilateral
Patient	Young Boys (avg. age 8-10)	Adults with high blood sugar

---

Coats' and Serous (Exudative) RD

Serous RD

Think of it this way: In Coats, "leaky lightbulbs" dump so much fluid that RPE (pump that keeps retina dry) can't keep up

• Fluid builds up under retina until it literally floats retina off back of eye

• No hole or tear = Exudative/Serous RD

• Diabetes, RD is usually Tractional (pulled by scars)

---

Summary: The "Vessel Vocabulary" for your Boards

• Telangiectasia ("Lightbulbs"): Think Coats' Disease. (Also Leber's Miliary Aneurysms + Von Hippel-Lindau (Retinal Hemangioblastoma) + MacTel).

• Microaneurysms ("Red Dots"): Think Diabetes.

• CNVM ("Gray/Green Mound"): Think AMD, Myopia, POHS.

• NVE/NVD ("Red Sea Fans"): Think Proliferative Diabetes (PDR) or Vein Occlusion.

Enhanced S-cone Syndrome/Goldmann-Favre Syndrome?

Extremely RARE

Autosomal Recessive degeneration

Begins early childhood

Goldmann-Favre Syndrome is SEVERE form of Enhanced S-cone Syndrome

Enhanced S-cone Syndrome/Goldmann-Favre Syndrome symptoms?

Nyctalopia (night blindness)

Blue light sensitivity

Blurry vision

Enhanced S-cone Syndrome/Goldmann-Favre Syndrome signs?

Goldmann-Favre Syndrome presents as:

→ Bilateral retinal pigmentary + vitreous degeneration with macular retinoschisis (& sometimes peripheral retinoschisis)

• sometimes also CME if fluid accumulates within splitting layers (esp for cases like genetics where retina is “sick”)

Enhanced S-cone Syndrome/Goldmann-Favre Syndrome tx?

If CME → topical CAIs (e.g., Dorzolamide or Brinzolamide; these uniquely help with both IOP lowering & CME issues)

Progressive issue → condition likely to get worse despite tx (“prognosis gaurded”)

• In Gyrate Atrophy or Retinitis Pigmentosa (RP), the CME isn't a one-time event (like after cataract surgery)

  • Actual "machinery" of retina is slowly dying

• Treatment Limit: CAI drops treat the symptom (the fluid), but don't fix the genetic mutation (broken enzyme)

• Progressive Nature: Since RP + photoreceptors keep degenerating over decades, vision will likely keep declining even if you keep macula "dry" for few years

Retinoschesis?

Splitting layers within the retina

• VS. Retinal Detachment = retina is detached from RPE/Choroid

• Dome-shaped lesions in retinal periphery

• May look very similar to RD

Any visual field defects from Retinoschesis?

YES! Intra-retinal visual pathway disrupted:

• Absolute VF defect with sharp borders

  • VS. RD = Relative VF defects

How to distinguish RD from Retinoschesis?

HVF testing

Scleral indentation

OCT

***NOT Fluorescein Angiography

Retinoschesis:

• NO Tobacco Dust/Shafer’s sign (may see on RD)

• Rarely → can have associated Rhegmatogenous RD + Vitreous hemorrhages

Retinitis Pigmentosa?

What else for this section?

RDs

Breaks/holes

lattice/snail

retinoschesis

myopia - lead to RDs (all myopia stuff)

RP

star

best/vitilli

fam

leber

Maculopathies?

Any pathological change/disease - specifically affecting macula

• Central part of retina responsible for detailed, color, "straight-ahead" vision

• Look for loss of Visual Acuity (VA) and central distortions (metamorphopsia)

6 maculopathies:

1. Macular Telangectasia

2. Macular Hole

3. Cystoid Macular Edema (CME)

4. Central serous chorioretinopathy (CSCR)

5. Age-related Macular degeneration (ARMD)

6. Epiretinal membrane (ERM)

Macular Telangectasia?

Telangectasia = abnormally dilated capillaries

• Usually caused by another retinal disease

• Rarely idiopathic

***IF ever rarely idiopathic? Macular Telangectasia

• 3 types!

• Similar to Coat’s disease (progressive, vascular abnormalities are = leaky → hard exudates deposit

Macular Telangectasia symptoms?

Progressive loss of central vision

Macular Telangectasia signs?

Macular findings:

• Atrophy

• Exudates

• Pigmentary changes

• Hemorrhage from CNVM

Macular Telangectasia 3 types?

1. MacTel Type 1 = essentially a localized, adult-onset version of Coats' Disease (vascular issue)

• Exudates found in perifovea + periphery

• Middle-aged men

• “Form frust” (incomplete or “mini version”) of Coat’s disease

• Unilateral

  

2. MacTel Type 2 is "dry" and has no exudates - cellular problem

• MORE common than Type 1

• WORSE visual prognosis than Type 1

• Neurodegenerative changes temporal to fovea center w/ILM drape on OCT (on OCT scan, will be large, empty-looking cavities/hyporeflective spaces in middle layers of retina)

• Can progress to "Wet" phase where secondary CNVM causes exudates + hemorrhages you’d normally see in Wet AMD (now needs Anti-VEGF)

• Atrophy + pigmentary changes → advanced cases

• Müller cells die!!! These cells - responsible for holding onto macular pigment (lutein + zeaxanthin)

  • Hyper-autofluorescence on FAF (due to loss of macular pigment)

  • Hyporeflective on OCT

  • Fluorescein Angiography (FA) = Late temporal leakage (diffuse "blush")

    • Invasive test - injecting dye (Sodium Fluorescein) into patient’s arm

  • OCT-A = Missing capillary network in deep plexus

3. MacTel Type 3: Occlusive Telangectasia

• RARE

• 60s age

• Capillaries occluded

• VERY poor visual prognosis

• If you see

• question about MacTel mentioning "Systemic association" or "Capillary occlusion," think Type 3

MacTel tx?

Anti-VEGF injections for associated CNVM

The "Dye" (FA) vs. The "Glow" (FAF)

Condition	FA (Dye) Keyword	FAF (Glow) Keyword
Stargardt's	Silent Choroid (Blackout)	Pisciform Flecks (Bright)
Best's	Masking (Dark hole)	Egg Yolk (Very Bright)
Plaquenil	Window Defect (Ring) "Bull’s Eye Maculopathy"	Stressed Ring (Bright) Hyper-FAF Ring
CME	Petaloid Leak	Usually normal / subtle
MacTel 2	Temporal Blush (late leak)	Hyper-fovea (Lost pigment)

CSCR

Smokestack / Inkblot

Descending Tracts (Gravity)

Exudates?

	Hard Exudates	Soft Exudates (CWS)
Material	Lipids / Proteins	Infarct/Collection of Swollen Axons (Debris)
Problem	Leaky Vessels (Wet)	Clogged Vessels (Dry)
Looks	Yellow, Shiny, Sharp borders	White, Fluffy, Fuzzy borders
Location	Deeper (Outer Plexiform Layer)	Superficial (Nerve Fiber Layer)

• Presence of exudates tells you the severity of a disease

Hard:

Condition	Recognition / Board Clue	Treatment	Fluid Name
Diabetes (CSME)	Circinate rings of yellow dots	Anti-VEGF or Focal Laser	Macular Edema/CSME/DME
Coats' Disease	Massive "Puddles"; Young boy; Lightbulb vessels	Laser/Cryo to vessels	Exudative RD
HTN Retinopathy	Bilateral Macular Star + AV Nicking + High BP	BP Control	Macular Edema
Cat Scratch (CSD)	Unilateral Macular Star + Swollen Nerve + Fever	Antibiotics (Azithromycin)	Serous Fluid
RAM (Macroaneurysm)	Single localized ring on one artery	Observation or Laser	Localized Edema
Von Hippel-Lindau	Exudates near a red "orange" tumor	Laser/Cryo to tumor	Exudative RD

Soft:

Condition	Recognition / Board Clue	Treatment
Pre-Proliferative DR	>1 CWS (4-2-1 rule); Dot/Blot hemes.	Monitor closely for Neo.
HIV Retinopathy	CWS in a patient with a low T-cell count.	Manage the HIV (HAART).
HTN (Grade III)	CWS + Hard Exudates + Hemorrhages.	Urgent BP control
CRVO / BRVO	CWS + "Blood and Thunder" (Massive hemes)	Anti-VEGF for edema