Ch 11. Inflammatory Diseases Of the Retina

Question 1

Given that this is a young Japanese patient that presented with acute vision loss after a flu-like prodrome, with mayor vessel perivascular infiltration and disc edema what is the most likely diagnosis?

Answer 1

Frosted branch angiitis.

Question 2

Name causes for frosted branch angitis

Answer 2

1. idiopathic
2. Secondary: including cytomegalovirus retinitis, Behçet’s disease, lupus erythematosus, Epstein–Barr virus retinitis, syphilis, toxoplasmosis, herpetic retinitis, human immunodeficiency virus (HIV) positivity, Hodgkin’s disease, rapidly progressive glomerulonephritis, staph and streptococcal endophthalmitis.

Question 3

What is the name for the inflamatory reaction induced by initial ATB treatment of ocular syphilis?

Answer 3

Jarisch-Herxheimer

Question 4

The following statement and image refer to,

“typically affects young healthy male or female patients (average age approximately 25 years), who develop rapid loss of vision in one or both eyes secondary to multiple postequatorial, circumscribed, flat, gray subretinal lesions involving the retinal pigment epithelium”

Answer 4

ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY

(APMPPE)

Question 5

To what potentially fatal complication is APMPPE associated ?

Answer 5

Cerebral Vasculitis, with a 95% fatality rate if not promptly treated.

Question 6

Laterality of APMPPE

Answer 6

Usually bilateral, the second eye is usually involved within a few days or weeks after the first.

Question 7

T/F

APMPPE has a very poor visual prognosis, most patients end up with vision wost than 20/40.

Answer 7

False, it has a great prognosis, with most patients returning to 20/20.

APMPPE is dangerous not for its visual prognosis, but its association to cerebral vasculitis, which does have a high mortality rate.

_{Many patients can describe a paracentral scotoma when carefully examined.}

Question 8

How are APMPPE plaques imaged in the FA?

Answer 8

They are hypo-fluorescent in early stages (blockage) and stain during late stages

Question 9

APMPPE and Serpiginous Choroiditis may present similar in early stages but have entirely different clinical courses. Name differences between them.

Answer 9

Question 10

This 43-year-old patient’s illness has been characterized by a multifocal chronic/recurrent choroidal inflammation that began at the nerve and has spread centrifugally. What is the most likely dx?

Answer 10

Serpiginous Choroiditis

Question 11

What difference do the acute Serpiginous Choroiditis plaques have when compared to APMPPE ones?

Answer 11

They are both early-hypo, late hyper… fluorescent.

But SC ones stain from the periphery, so they present a halo appearance during “early” late stages.

_{<em>This is because APMPPE is not associated with choriocapillaris shut down, while SC affects primarily the inner half of the choroid. </em>}

Question 12

If a patient under treatment for serpiginous choroiditis is not responding to steroids, what other Dx must be ruled out (if not before starting Tx)

Answer 12

Tubercular Serpiginous Choroidopathy

Question 13

What is the visual prognosis of acute idiopathic maculopathy?

Answer 13

Excelente.

Question 14

This young patient presents a couple of weeks after having a flu-like illness, with unilateral paracentral scotomas, photopsia, vitritis, and this funduscopic image.

Name the most likely Dx.

Answer 14

MEWDS

_{An enlarged blind spot will most likely be present if this is the correct dx and must be looked for.}

Question 15

Is vitritis present in MCP?

Answer 15

In its original description, yes, this was actually one of the ways to tell the difference from POHS.

Nevertheless, it is now clear that it may not have cells at the time of clinical assessment.

Question 16

Is MCP necessarily a panuveitis?

Answer 16

Not necessarily, anterior segment inflammation is present in 50% of patients.

Question 17

Laterality and gender predilection of MCP?

Answer 17

Mostly bilateral (but asymmetric in presentation)

Female predilection.

Question 18

Which HLA is useful in distinguishing between POHS and MCP?

Answer 18

HLA-DR2 (positive in POHS)

Question 19

Characterize PIC

Answer 19

A clinical syndrome characterized by

• Moderate myopia
• blurred vision, photopsia, and scotomas
• POHS-like lesions mostly in the posterior pole.
• NO vitritis or anterior Uveitis
• Bilateral
• Very good prognosis in 50% of cases (those that does not develop CNV)

Question 20

PIC and MCP are thought to be part of a disease spectrum, name differences between them.

Answer 20

• Vitritis: Frequent in MCP
• Pan-uveitis/anterior segment inflamation: present in MCP
• PIC is confined to the posterior pole

Question 22

treatment for PIC?

Answer 22

• If lesions are active/inflamed, or in a CNV associated with an active lesion: steroids and/or IMT.
• In CNV that develops in a scar, anti-VEGF should be used.

Question 23

This young female patient presented with a history of rapid bilateral central vision loss, after a flu-like illness. In the amsler grid, she can clearly define a central-paracentral scotoma with a petaloid pattern.

Funduscopy shows multiple oval paracentral graish foci of oval discoloration.

FA shows a central pattern of hypofluerescence in a petaloid shape that correlates to a sharply deliniated photoreceptor loss in the OCT.

Name de diagnosis.

Answer 23

AMN

Question 24

Name possible predisposing factors that have been reported in association with AMN

Answer 24

• Flu-like illness
• IV sympathomimetics or iodine-containing contrast agents.
• Anaphylactic shock and shock in general.
• Oral contraceptives
• Trauma
• Headache and migraine.
• Postpartum hypotension.

Question 25

Name the differences in OCT between AMN and PAMM

Answer 25

PAMM Involves the inner nuclear and outer plexiform while AMN involves the photoreceptors and RPE.

(in both, the acute case will show hyperreflective bands corresponding to the affected layers, and then in the follow-up, atrophy of these layers will show in the OCT)