2. • Lee Jampol et al in 1984
• multiple white dots, vitreal cells, RPE granularity in the macula, and reduced
visual acuity.
• Fluorescein angiography showed leakage from disc capillaries and zones of late
staining.
• Good visual recovery was the rule
• ERG-photoreceptor activity was profoundly affected
• Only profound ischemia of the outer retina due to choriocapillaris non perfusion,
its source of oxygen, can account for such an effect both on the photoreceptors
and secondarily on the RPE
3. CLINICAL PRESENTATION
• M:F :: 1:3
• Flu like symptoms or URTI precedes in 50 % cases.
• unilateral
• Presents- photopsia ,scotoma
• Recovers in 6-12 weeks
• Fundus-numerous faint white dots in the mid-periphery seen at disease onset.
These dots can disappear very rapidly and can be missed by the clinician if the
patient does not consult at an early stage
• convalescent stage the macula takes a granular aspect.
• Inflammation is usually moderate and limited to the vitreous but the optic disc
can be involved.
4. ICGA
• Choroidal capillary non-perfusion.
• Numerous hypofluorescent dark areas predominantly situated in the mid-
periphery with tendency to confluence of the lesions.
• In most cases hypofluorescence is also present around the optic disc where it is
usually confluent forming a more or less large zone which probably explains the
more or less important enlargement of the blind spot.
• these dark dots are much more clearly delineated in the late phase
5. ICGA. Classical signs of confluent geographic
hypofluorescent areas indicating choriocapillaris
perfusion pathology
FA. Shows early choroidal
filling abnormalities and disc hyperfluorescenc on late
frames
6. FFA
• Signs can be absent or very minimal, consisting of faint patchy areas of late
hyperfluorescence (staining).
• They can also be very pronounced in some cases.
• There can be an associated disc hyperfluorescence.
7. FUNDUS AUTOFLUORESCENCE (FAF)
• Fundus autofluorescence (FAF) is principally produced by the lipofuscin present in the
RPE cell.
• This material is originating from the photoreceptor outer segments and its degradation
process or accumulation in the RPE cell lysosomes seems to be an indicator on the
quality of the RPE cell metabolism.
• Hyperautofluorescence is much more widespread than the ICGA lesions indicating that
the RPE is suffering also in areas that do not show the classical signs of non perfusion
seen on the ICGA
8. FAF is widespread on the involved side and
goes beyond the lesions seen on ICGA
9. OCT
• changes at the level of the outer
retinal in most cases of acute and
active choriocapillaritis such as
MEWDS.
• Irregularities at the level of the RPE,
a hypereflective layer probably
indicating liquid as well as
disorganisation of mainly the outer
retina
12. ACUTE IDIOPATHIC BLIND SPOT
ENLARGEMENT (AIBSE)
• 1988-Fletcher et al
• peripapillary scotoma - symptomatic enlargement of the blind spot objectively
identified by visual field testing
• Visual acuity, color vision, pupillary responses, funduscopy and fluorescein
angiography were all normal.
• focal ERG indicating retinal dysfunction around optic disc
• Most probably AIBSE and MEWDS are the same disease with the exception of the
usual fundus findings that were not found-may be the clinical lesions subsided or
subclinical.
• ICGA- choriocapillary non perfusion in peripapillary area
13. Acute Posterior Multifocal Placoid
Pigment Epitheliopathy (APMPPE)
• 1968 - J. Donald M. Gass
• rapid loss of central vision associated with multifocal, yellow-white placoid
lesions occurring in one eye, with sequential involvement of the second eye,
followed by resolution of the fundus lesions and visual improvement over weeks
or months thereafter
• choriocapillaris nonperfusion as the primary event
• preceded by a febrile, infectious or flue-like episode in at least half of the patients
14. DISEASE MECHANISM AND
PATHOPHYSIOLOGY
• Most probably nonperfusion is secondary to a vasculitis touching arterioles or
precapillaries at the origin of the area of nonperfused choriocapillaris
• As the involved nonperfused zones are much larger, outer retinal ischaemia is
more pronounced than in MEWDS explaining the more pronounced reactional
fluorescein exudation from capillaries of the inner retina
15. CLINICAL FEATURES
• visual disturbance, photopsia and scotoma
• Bilateral, may be asymmetric
• Classically absent anterior segment signs ,sometimes frank anterior uveitis
Yellow-white plaques of fundus discoloration in posterior pole during active phase of disease (left picture) having
resolved at convalescent stage of disease (right picture)
16. • Rarely ,recurs
• Sometime sit may start as APMPPE, later take the form of serpigenous choroiditis
• Fundus-acute phase are characterised by deep plaques of yellow-white discoloration of the
fundus of diverse size, sometimes confluent and geographic in aspect.
• Serous retinal detachment can rarely be seen in severe cases resembling Vogt-Koyanagi-
Harada disease that should be excluded
• Convalscent phase-mottled RPE or frank chorioretinal scars
17. ICGA
• unevenly sized geographic hypofluorescent areas randomly scattered in the
posterior pole present in the early and intermediate phase of angiography but
very distinctly visible in the late ICG angiographic phase
• On follow-up angiograms these signs resolve almost completely with the
exception of a few areas of persisting hypofluorescence due to chorioretinal
atrophy
18. Indocyanine green angiography in healed stage of APMPPE/AMIC.
Hypofluorescent areas persist with reduced surface representing
retinochoroidal scars
19. FFA
• Initial hypo-later hyper
In the early phase of FA (left frame) there is patchy choriocapillaris nonperfusion (corresponding to
hypofluorescent areas on ICGA) leading to ischaemia of the outer retina.
The capillaries of the retina react to this outer retina ischaemia by increased permeability leading to leakage
(middle frame).
Sometimes, when ischaemia is very pronounced this can lead to massive pooling having the aspect of serous
retinal detachments similar to that pound in VKH disease (right frame)
20. FAF
• there are widespread zones of choriocapillaris involvement, FAF is very often decreased within the active
lesions being sometimes visible on the border of active or progressing lesions.
• In these conditions bright FAF is often present in the center scarred lesions, but this type of autofluorescnce is
not reflecting RPE dysfunction but accumulation of cellular fluorophore debris.
in acute phase of a case of APMPPE/AMIC.
Hyperautofluorescence is mainly present at the rims of the
nonperfused areas but less so inside the areas of disease
activity, indicating mostly nonfunctioning RPE cells, wheras in
the rims RPE cells are still metabolically active although in a
slowed fashion explaining the hyperautofluorescence
in healed phase of APMPPE/AMIC.
Hyperautofluorescent rims around affected areas
have disappeared. However there are
fluorophores in the center of the scars
21. OCT
• showing inhomogeneity of outer retina and sometimes hyporeflective layer,
probably indicating subretinal fluid.
OCT- focal areas of RPE elevation and atrophy, associated with disruption and loss of overlying
photoreceptor outer segments. There are also small pockets of subretinal fluid. Focal hyper-
reflectivity of the photoreceptor IS/OS junction is also noted.There is patchy loss of the outer
plexiform layer, though the inner retinal layers remain intact
22. ERG
• moderate and transient abnormalities in APMPPE.
• This is in contrast to MEWDS where ERG shows abnormalities indicating outer
retinal dysfunction in up to 80% of cases.
• This is possibly explained by the fact that lesions in MEWDS are diffuse whereas
in APMPPE they are usually more limited and focal.
