- A range of inflammatory diseases of the retina, RPE and choroid
- There are three well defined/distinct clinical syndromes:
    - AMPEE
    - Birdshot
    - Serpiginous chorioretinopathy

- The **remaining** white dot syndromes exist on a spectrum
- All tend to affect young women (typically myopes) except Serpiginous Choroidopathy 
 and Birdshot which affect the middle-aged.

## Acute posterior multifocal placoid pigment epitheliopathy (AMPPE)

- Young adults
- Usually **bilateral**
- Flu-like prodrome
- Possibly related to patchy choroidal ischaemia/vasculitis

### Clinical features

- Reduced vision sequentially
- Post-equatorial placoid lesions of the RPE: creamy white then fading
- Later pigmentary changes
- Mild vitritis

### Tests

- FFA: early hypofluorescence and late hyperfluorescence (‘**block early, stain late**’)
- ICG: hypofluorescence of placoid lesions
- OCT: dome elevation of the placoid lesions at the outer retina
- OCTA: islands of altered flow and non-perfusion
- AF: hypofluorescence in acute phase

### Natural history

- Self-limiting within 2-3 months
    - Rarely progresses to a serpiginous choroiditis requiring steroids.

- Associated with **CNS vasculitis**: any neurological symptoms (such as **headache** require prompt neurology evaluation.

## Birdshot chorioretinopathy aka vitiliginous chorioretinitis

- Bilateral
- Unknown aetiology
- Middle-aged Caucasians
- F>M slightly
- **HLA-A29 association**

### Ocular features

- Reduced vision
- Reduced colour vision
- Floaters
- Night blindness
- Oval cream-coloured lesions radiating from the optic disc to the equator associated with choroidal vessels
- Moderate vitritis
- Vasculitis
- CMO
- Lesions become atrophic with time
- CNV
- Optic atrophy

<h3 id="tests-1">Tests</h3>

- HLA testing to identify HLA-A29 (>95% patients): if negative consider sarcoidosis which can appear similar
- OCT: CMO and outer retinal changes
- OCTA: disruption to the RPE with reducing underlying choroidal blood flow
- FFA: early hypofluorescence of lesions with late hyperfluorescence. Hyperfluorescence of the disc, retinal vessel leakage, CMO
    - May show **‘quenching’**: quick fading of the dye from retinal circulation

- ICG: hypofluorescent spots showing inactive lesions (more lesions seen on ICG than FFA)
- VF
- ERG: reduced b-wave amplitude and latency
- EOG: reduced Arden index


<HotTopic>

**Electrodiagnostics** can play a useful role in diagnosis of Birdshot

</HotTopic>

### Management

- Corticosteroids and long-term immunosuppression with MMF

## Serpiginous choroidopathy

- Rare
- Bilateral
- Middle aged
- **Resembles AMPEE** but has much worse prognosis
- Associated with TB and syphilis (or these cause a similar picture)

<h3 id="clinical-features-1">Clinical Features</h3>

- Reduced vision
- Asymptomatic until macular involvement
- Serpiginous, placoid or geographic lesions at the RPE/inner choroid level: greyish-yellow, spread **centrifugally from the disc**
    - Become atrophic over months with irregular depigmentation/pigmentation

- Mild vitritis
- CNV: **rare**
- Subretinal scarring

<h3 id="tests-2">Tests</h3>

- OCT: hyperreflective outer retina and RPE, choroidal hyperreflectivity (‘reverse shadowing’)
- AF: demarcates lesions with hyperfluorescent active edge and hypofluorescent inactive lesions due to loss of RPE
- FFA: **early hypofluorescence and late staining (and early hyperfluorescence at the edge of lesions)**
  - ICG: hypofluorescence

<h3 id="management-1">Management</h3>

- Corticosteroids/immunosuppression in the acute phase eg mycophenolate, cyclosporine, azathioprine, prednisolone
- IVT anti-VEGF

## Multiple evanescent white dot syndrome (MEWDS)

- Rare
- Typically affects **young myopic** women
- **Unilateral**
- Flu-like prodrome

<h3 id="ocular-features-1">Ocular Features</h3>

- Reduced vision
- Scotomata
- **Photopsia**
- Transient RAPD
- White dots at the level of the outer retina/RPE
- Orange-white dots at the fovea (‘**foveal granularity**’)
- Mild vitritis

<h3 id="tests-3">Tests</h3>

- OCT: attenuation of the IS-OS junction (ellipsoid zone) resolves within weeks/months. In the right clinical context, disruption of the normal IS-OS and RPE layers is effectively diagnostic of MEWDS
- FFA: **wreath-like punctate hyperfluorescence** corresponding to white dot areas with late staining, disc leakage
- ICG: hypofluorescent dots around optic disc
- ERG: reduced **a-wave**

<h3 id="natural-history">Natural history</h3>
- Self-limiting within 2-3 months


## Acute retinal pigment epitheliitis (ARPE, Krill disease)

- Rare
- Unilateral
- Young adults
- Self-limiting within 1-3 months
- Slightly reduced vision
- Metamorphopsia
- Foveal pigment spots with yellow-white halo
- FFA: hyperfluorescent spots
- OCT: hyperreflective accumulations within the RPE

## Acute zonal occult outer retinopathy (AZOOR)

- Rare
- May be bilateral (60% eventually involve second eye) especially in males
- Affects young, myopic females (like MEWDS)
- Flu-like prodrome

<h3 id="ocular-features-2">Ocular Features</h3>

- Acute scotomata (zonal ** field loss**) worse in bright conditions
- **Photopsia**
- Fundal lesions: on OCT/AF these have a demarcating line at the level of the outer retina in a trizonal pattern of sequential involvement: outer retina, RPE and choroid
- Mild vitritis
- Retinal/choroidal atrophy and RP-like pigmentation

<h3 id="tests-4">Tests</h3>
- ERG: variably abnormal in a patchy distribution
- EOG: loss of light rise
- FFA: may be normal initially

<h3 id="management-2">Management</h3>
- Best treatment is unclear
- Tends to stabilize by 6 months but causes visual field defects corresponding to areas of RPE atrophy



## Multifocal choroiditis with panuveitis (MCP) and punctate inner choroidopathy (PIC)

- Inflammatory conditions of the choroid and retina
- Bilateral
- Grey or yellow-white lesions
- MCP: visible inflammation (especially vitritis)
- PIC: **no** visible inflammation. Tends to affect younger patients
- F>M
- There may be a viral aetiology

<h3 id="clinical-features-2">Clinical Features</h3>
- Reduced vision
- Scotomata
- Photopsia
- MCP: choroidal lesions, vitritis, anterior uveitis, CMO, subretinal fibrosis, CNV (**CNV** is the most common visually significant complication of MCP)
- PIC: **quiet eye**, inner choroidal/retinal lesions, yellow-white with fuzzy borders then become atrophic and pigmented scars similar to POHS, serous RD, CNV (visual prognosis is very good unless CNV develop)

<h3 id="tests-5">Tests</h3>
- OCT
- AF: reveals active lesions at an early stage with hyperfluorescent spots with hypofluorescent centre
- OCTA
- FFA: early punctate hypofluorescence and late hyperfluorescence of lesions, may reveal CNV
- ICG: hypofluorescent lesions

<h3 id="management-3">Management</h3>

- IVT anti-VEGF for CNV
- Intravitreal corticosteroids
- Immunosuppression may have a role
- Rescue corticosteroids may be used in CMO


A range of inflammatory diseases of the retina, RPE and choroid There are three well defined/distinct clinical syndromes: AMPEE Birdshot Serpiginous…

Uveitis

White dot syndromes

Intro

FRCOphth Part 2 Study Notes

Tumours, masses and neoplasia

Tumours overview

Uveitis background

Anterior uveitis

Intermediate uveitis

Uveitis in JIA

Uveitis with JIA

Retinal vasculitis

Sarcoidosis

Behcet's disease

Toxoplasmosis

Viral uveitis

Strabismus

Overview

Esotropia

Fungal uveitis

Mycobacterial uveitis

Adnexal masses

Spirochaetal uveitis (including Syphilis)

Exotropia

Melanoma

Nematode uveitis

Retinoblastoma

Retinoblastoma (for Part 2)

Incomitent

Voyt-Koyanagi-Harada syndrome

Conjunctival intraepithelial neoplasia

Vertical deviations

Sympathetic ophthalmia

Orbit and ocular adnexae

Orbit

AV Malformations and hamartomas

Arteriovenous malformations and hamartomas

Restriction syndromes

Paediatrics

Leucocoria

Congenital Cataract

Paediatric Glaucoma

Retinopathy of prematurity

Retinopathy of Prematurity

ROP-like disorders

Congenital nasolacrimal duct obstruction

Aniridia: Short Arm 11 Deletion (11p13)

Choristomas

Management Of Amblyopia

Alphabet patterns

Scleritis

Iris Nodules

Meningioma

Strabismus surgery

Optic nerve glioma

Lymphoma

Extraocular Muscles

Rhabdomyosarcoma

Idiopathic orbital inflammation

Orbital neuroblastoma

Anterior Persistent Foetal Vasculature

Lacrimal system

Metastatic lesions

Surgical Retina

Retinal Detachment

Vitreoretinal Interface And Macular Hole

Epiretinal Membrane

Medical Retina

Diabetic Eye Disease

Orbital Blood Vessels

Retinal Vascular Disease

Macular Degeneration

Central Serous Chorioretinopathy (CSCR)

Sickle Cell Retinopathy

Retinitis Pigmentosa

Albinism

Retinal Telangiectasias

Miscellaneous Retinopathies

Atropine And Myopia Progression

Pharmacology

Side Effects Of Systemic Drugs

Foetal Alcohol Syndrome

Neuro-ophthalmology

Optic Neuropathy

Papilloedema

Idiopathic Intracranial Hypertension

Chiasmal Disorders

Retrochiasmal Disorders

Migraine

Paranasal Sinuses

Supranuclear Motility Disorders

Third Nerve Palsies

Fourth Nerve Palsies

Sixth Nerve Palsies

Seventh Nerve Palsies

Anisocoria

Myasthenia Gravis

Myopathies

Blepharospasm

Functional Visual Loss

Lacrimal Glands

Phakomatoses

Developmental diseases

Tear Film

Eyelids

Blinking

Lid Malpositions

Anterior segment - ocular surface

Conjunctiva

Conjunctivitis

Sclera

Pigmented Conjunctival Lesions

Squamous Cell Carcinoma Of The Conjunctiva

Ocular Cicatricial Pemphigoid

Cornea

Corneal Structure And Development