NBEO Part 1 Systemic/Ocular Disease Q

What is the "triad" for Histoplasmosis

There are really 4!
1) PPA peripapillary atrophy of the optic nerve
2) Multifocal lesions in the periphery
3) Maculopathy
4) No cells in the vitreous!!!

What is the triad for RP?

- Retinal bone-spicule pigmentation (pigment clumps in periph)
- Arteriolar attenuation
- Waxy Optic Disc pallor

CSME criteria (3):

Only has to fit 1 of the following.

- Retinal thickening within 500 um (1/3 DD) or foveal center
- Hard exudate w/i 500 um of foveal center w/ adjacent thickening
- Retinal thickening 1 DD (1500 um) within 1 DD of foveal center)

Severe NPDR criteria (3):
Very severe NPDR criteria:

Severe NPDR has to fit 1 of the criteria.
Very severe has to fit 2 of the criteria:
4:2:1 rule
4: Retinal hemorrhage in 4 quadrants
2: Venous beading in 2 quadrants.
1: IRMA (intravascular retinal abnormalities) in 1 quadrant

What are the "High Risk Characteristics" of PDR? (2)

Only has to fit 1 of the criteria:

- Neovascularization disc > 1/4 DD
- NVD or NVE with vitreous or preretinal hemorrhage.

What is the triad for Congenital Syphilis? (3)

- Hutchinson's teeth
- Saddle bridged nose
- Flat forehead

What are the 3 C's and what disease do they apply to?

This applies to Congenital Toxoplasmosis.
- Cough
- Convulsions
- Chorioretinitis

Name 5 Granulomatous diseases:

- Sarcoidosis
- TB
- Syphilis
- HIV?
- Herpes?

What are the stages of Hypertensive Retinopathy using the Keith Wegener Barker Classification? (4)

Stage 1: Mild to moderate diffuse narrowing

Stage 2 = Stg 1 + focal constriction (Arteriovenous nicking); Exaggerated light reflex

Stage 3: Stg 2 + Retinal hemes, CWS, Hard exudates (macular star pattern), Retinal edema

Stage 4: Stg 3 + papilledema (malignant HTN) => hospitalize

What are Elschnig spots?

Choroidal infarctions associated with severe HTN.

What is Watzke-Allen sign?

Test associated with macular hole, first seen in Stage 3

What are the stages for Macular Hole? (4)

Stage 1: impending hole, loss of foveal depression, with YELLOW SPOT or RING

Stage 2: Round, small full-thickness hole with PSEUDO-operculum

Stage 3: Large, full-thickness hole with operculum. WATZKE-ALLEN sign now apparent.

Stage 4: Stage 3 + PVD

What starts a macular hole?

Posterior vitreous traction

What is Pavingstone Degeneration? Is it risky?

What are Lacquer Cracks? Is it risky?

-Discrete, circular areas of yellow white chorioretinal atrophy in the retinal periphery. AKA cob. It has no clinical importance, and will not lead to retinal detachment or other retinal pathology.

-Fine, yellow irregular lines that represent breaks in BRUCH's membrane. It can lead to choroidal neovascularization and lead to severe vision loss. It is found an early finding in pathological myopia.

What is the hallmark for pathological myopia?

What are some other signs?

Posterior staphyloma.

- Oblique insertion, Fuch's spots, lacquer cracks, macular holes, premature cataracts, peripheral retinal degeneration.

CRVO vs BRVO.
1) What type of heme?
2) Hemes in how many quadrants?

1) Intravitreal hemes:
2) CRVO: All 4. BRVO: 1 quadrant, usually superior/temporal

What is a Hollenhorst plaque? Small or large?

What is a calcific emboli? Small or large?

Hollenhorst plaques and Calcific plaques are associated with CRAO and BRAO.

Hollenhorst plaques = Carotid Emboli; Small. BRAOs usually occur because of Hollenhorst plaques.

Calcific emboli come from calcified heart valves; Large.

What is Amaurosis Fugax?

Transient blackout or blur of vision. Usually occurs from carotid artery emboli (Hollenhorst plaques?)

What is associated with Gardner's Syndrome?

Bilateral, multifocal (4 or more) CHRPE.

What are the differentials for leukocoria? (4)

- ROP
- Toxocariasis
- Coat's disease
- Retinoblastoma

What are the symptoms in Dry ARMD?

What are the signs (4)? What is the hallmark?

- metamorphopsia, gradual vision loss (months to years), blurred vision
- most DO NOT have vision loss.

-HALLMARK sign: Drusen
- Associated signs: motting, granularity, geographic atrophy, focal hyperpigmentation

12% of all dry ARMD patients develop severe vision loss (def: >6 lines). Most of these result from geographic atrophy.

What are the symptoms in Wet ARMD?

What are the signs?

- metamorphopsia, central scotoma, rapid vision loss

Drusen associated with subretinal fluid (blood or plasma)

What are the risk factors of progressing from Dry ARMD to Wet ARMD (as established by The Macular Photocoagulation Study Group) (4)

- multiple soft drusen (NOT HARD DRUSEN)
- focal hyperpigmentation
- HTN
- Smoking

What is Usher's Syndrome?

Is is the most common associated systemic condition with Retinitis Pigmentosa (5% of RP patients).

What is the most common symptom of RP? What are some of the others?

What is the mode of inheritance, if inherited?

- Night Blindness

- Peripheral vision loss (Early stage: in dim light only)

- It can be inherited or non-inherited. If inherited, it is most commonly AD

What is the mode of inheritance for Stargardt's patients?

Typically autosomal recessive.

RP vs Stargardt's
Inheritance:
Most common:

RP: AD, Most common retinal dystrophy
Stargardt: AR, Most common macular dystrophy

What are the signs and symptoms of a patient with Stargardt's disease?

Early - decreased vision out of proportion with fundus appearance. 20/40, although fovea is affected. Rapid Vision loss, color vision abnormalities.

Middle - bilateral yellow flecks in a pisciform (fish-tail) configuration @ the posterior pole and mid-periph. Non-specific RPE mottling of the macula may also be apparent.

Late - classic "beaten bronze" macular pattern (Bull's Eye maculopathy) and "salt and pepper" pigmentary changes may appear in periphery.

What is the typical patient profile for Stargardt's?

Onset: 1st or 2nd decade of life. Presents between 6 and 20.

What is Fundus Flavimaculatus?

Fundus Flav and Stargardt's are variants of the same disorder. Fundus flav is reserved for patients w/o macular dystrophy; It often presents later in life (4th and 5th decade); patients are commonly asymptomatic. Vision loss can still occur if fleck-lesions involve the macula.

What diseases are associated with night blindness (Nyctalopia)?

Mnemonic: Color: RG

Choroideremia
RP
Gyrate atrophy

What are the DDX for "Bull's eye maculopathy"?

What has a bull's eye pattern on the LENS capsule?

Mnemonic: PS THC - PasS (the) THC

Progressive cone dystrophy
Stargardt's disease
Thioridazine, Hydroxychloroquine, Chloroquine (drug toxicity)

On lens capsule? Pseudoexfoliation Syndrome

Which disease has the "scrambled-egg" appearance?

Best's Disease

What are the following terms associated with:
1) Tobacco dust
2) Schaeffer's sign
3) Asteroid hyalosis
4) Synchisis scintillans

1) Retinal detachment
2) aka Tobacco dust
3) Calcium soaps, does not cause decrease in VA.
4) Hyperlipidemia, cholesterol

---------- are associated with vitreoretinal traction, and therefore have a (high/low) risk for subsequent detachment.

Retinal tears; high risk

Chronic atrophy => --------- holes
Vitreoretinal traction => --------- tears

- Atrophic holes
- Retinal tears

What are the 2 types of retinal tears? Which one has increased risk or retinal detachment?

- Flat tears = uneven vitreous traction; vitreous stays attached to thte flap => INCREASED risk
- Operculated tears = even, symmetric tear; vitreous pulls away and traction no longer exists => decreased risk.

-- % of patients with lattice degeneration => RD

1%

Non-rhegmatogenous RD include 2 types:
PDR => ------- RD
ARMD => ------- RD

========> Tractional RD
========> Serous RD

In reference to Age-related Degenerative Retinoschisis:

Splitting of the ---- layer => elevation of the inner retina => mimicking retinal detachment

Outer Plexiform Layer

What vascular birthmarks are associated with ipsilateral glaucoma? What systemic condition is associated (rarely) with this birthmark?

Port-wine Stain

Sturge-Weber Syndrome (5%)

Sampaolesi's Line:
Posterior Embryotoxin:
Arcus:
Limbal Girdle of Vogt:

Sampaolesi's Line: Increased pigmentation anterior to Schwalbe's line, associated with Pseudoexfoliation Syndrome

Posterior Embryotoxin: anterioriorly displaced Schwalbe's line

Arcus: has clear zone from limbus, associated with aging or cholesterol

Limbal Girdle of Vogt: May or may not have a clear zone from limbus. Age related condition = corneal degeneration; No therapy

In Pseudoexfoliation syndrome, the white flakes will deposit onto which ocular structures?

Pupil margin, lens capsule (Bull's eye pattern), TM

What are some common symptoms of patients with pigmentary dispersion syndrome?

- Blurred vision (after exercising or dilation)
- Halos (after exercising or dilation)
- Transillumination defects (TIDs)
- Krukenberg's spindle (vertically oriented pigment lines)
- Trabecular hyperpigmentation

What are the DDX for Normal Tension Glaucoma patient presenting with optic neuropathies?

MASH V

Myocardial Infarction
Anemia
Syphilis
Hemorrhagic shock
Vasculitis

What are the 2 mechanisms for Primary Angle Closure Glaucoma?

Pupillary Block and Plateau Iris

What are the risk factors for patients with Pupillary Block?

Anatomically narrow angles
Hyperopia

What are some SYMPTOMS of a patient with acute angle closure?

Vomiting, intense ocular pain, headaches, halos, nausea, progressive vision loss

What are some signs of a patient with acute angle closure?

hazy cornea, mid-dilated pupil that responds poorly to light, ciliary flush, glaucomflecken, occluded angle

What is the greatest threat to vision loss in acute angle closure?
What is subacute angle closure glaucoma?
Is acute or subacute more common?

CRAO
Angle closure w/o symptoms; subacute more common.

What is rubeosis of the iris?

The most important sign to recognize for prevention of Neovascular glaucoma. It can cause secondary angle closure because the fibrovascular membrane impedes outflow 360 deg of peripheral anterior synechiae.

In uveitic glaucoma, what two ocular findings can lead to secondary angle closure?

Peripheral anterior synechiae (PAS), and posterior synechiae (PS)

What are the two prominent types of inflammatory glaucoma?

Glaucomatocyclitic Crisis: aka Posser-Schlossman syndrome; elevated IOP w/ open AC angle; recurrent unilateral attacks => burn out over time.
Fuch's Heterochromic Crisis: chronic non-gran anterior uveitis => glaucoma, cataracts