a): Fluorescein angiography correlated with a Spectralis* horizontal section showing an intense exudative reaction. b): SLO-ICG angiography correlated with a Spectralis* horizontal section showing a cluster of small polyps.
a
Polyp
c
Figure 2: Several appearances of polyps:
a): Juxtapapillary polyp with RPE proliferation and marked SRF.
b): Prominent polyp with protrusion into the inner retinal layers, bright hyper-reflective spots, and dense zones. c): Residual polyp after treatment with no adjacent reaction.
The incidence of PCV appears to be higher in some countries, For example in Japan it appears to account for more than 40% cases of macular hemorrhage in the elderly.
Genetic correlation studies demonstrate a relationship with true AMD (Goth 2008, Lee 2008).
In our series of 200 consecutive patients, the incidence of polypoidal choroidal vasculopathy (PCV) was relatively high at 8%.
Contribution of OCT
Time-Domain OCT (Stratus*) clearly demonstrates alterations of the RPE band related to protrusion of polypoidal ectasias
These ectasias induce localized, steeply sloped elevation of the retinal pigment epithelium, in contrast with a serous PED, which is usually gently sloped and relatively flat.
10
The PED cavity is generally optically empty, revealing
Bruch’s membrane away from the RPE, but the cavity may
also have a heterogeneous appearance.
More marked shadowing in the choroid may be observed
in the case of concomitant active neovascularization.
The size of the polyps may be limited to that of drusen
or, on the contrary, can be large and severely deform the
RPE band. Polyps may be observed either within a zone
of serous or serosanguinous PED or, more often, at the
edge of the PED.
SD-OCT sections (Spectralis*) provide more detailed visu-
alization of polyps that can be seen as relatively prominent,
isolated, or multiple and clustered in groups (Figure 1).
Actively evolving polyps are associated with an exudative
reaction that can become intense and sometimes create
large cysts.
A large number of bright hyper-reflective spots adjacent
to polyps are often observed in the outer retinal layers
and even deeper (Figure 2a and b).
The outer retinal layers can be severely altered over the protruding polyps, with displacement of the outer nuclear layer, which is thinned and fragmented. The external limiting membrane and IS/OS interface can be lost and often replaced by a more or less extensive hyper-reflective dense zone (Figure 2b).
Spectral-Domain OCT provides an important advantage over TD-OCT by allowing the acquisition of 3D volume images and dense serial sections. This allows systematic examination of the entire macular region and the detection of even small polyps.
Natural History
The typical natural history of PCV has not been clearly elucidated. PCV has been known to remain asymptomatic or to extend with multiplication of the lesions and progression of the late plaque.
Particularly interesting transitional forms, which are between small polyps and occult CNV, have been observed.
The development of exudative and hemorrhagic complications and choroidal neovascularization is particularly important when they involve the macular region.
Treatment
The treatment of PCV has not been clearly defined, and the course of PCV in response to treatment is variable.
Laser photocoagulation can be used to destroy extrafoveal polypoidal formations and/or photocoagulate feeder vessels in the abnormal vascular network.
However, this treatment modality is not satisfactory due to the subsequent development of new channels, alterations of the choroidal vascular network, and recurrences of polypoidal formations.
Clinical trials of photodynamic therapy have demonstrated fairly good efficacy, particularly in patients who have already developed exudative and hemorrhagic complications.
Intravitreous injections of anti-angiogenic therapy have been used in many cases when these vasculopathies are associated with occult CNV (Figure 2c).
Despite a definite improvement, recurrences and active progression with fibrosis appear to be relatively frequent and may justify combination therapy.
Combined treatments must only be considered when the patient’s vision is at stake, such as in the case of progressive leakage or hemorrhage. Long-term observation is necessary in every case.
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10
TABLE OF CLINICAL CASES
CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY
Macular lesion with an extensive serosanguinous PED and polypoidal choroidal vasculopathy, progressing over several years.
Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy.................................. Page 306
CLINICAL CASE No. 02: POLYPOIDAL CHOROIDAL VASCULOPATHY
Macular lesion with drusen and a vascularized PED, progressing over several years.
Follow-up by SD-OCT after anti-VEGF therapy ...........................................................
Page 314
CLINICAL CASE No. 03: POLYPOIDAL CHOROIDAL VASCULOPATHY
Extensive, exudative macular lesion with several drusen, progressing over several years.
Follow-up by SD-OCT after anti-VEGF therapy ...........................................................
CLINICAL CASE No. 01: Polypoidal Choroidal Vasculopathy
Macular lesion with extensive serosanguinous PED and polypoidal choroidal vasculopathy, progressing over several years. Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy
A 73-year-old man presented with slowly progressive decline of visual acuity in his left eye, which interfered with his daily activities. He had a central scotoma in his other eye, existing for several years.
VA RE: 20/400 - VA LE: 20/50.
Biomicroscopic Examination
Central macular discoloration and inferior temporal
bullous detachment extending well into the periphery; no
lipid exudates (Figure 3a and b).
Autofluorescence
A vast hyper-fluorescent halo is visible in the macular
region and spreading inferiorly and beyond the temporal
10
vessels. The central zone and xanthophyll pigment were
spared (Figure 3c).
Fluorescein Angiography
▬In the superior half, a zone of irregular hyper-fluo- rescence suggestive of alteration of the RPE, with no fluorescein leakage. A series of small, rounded, foveal and temporal lesions that became hyper-fluorescent at the late phases.
▬In the inferior half, progressive filling of the well-de- marcated bullous PED with pooling of dye, except in the inferior sector, which remained hypo-fluorescent with a horizontal limit. This suggested a blood-filled fluid level (Figure 3e and f).
SLO-ICG Angiography
a): Early phase: fine, abnormal, juxtafoveal and inferior temporal network at the superior edge of the dark, hypofluorescent zone corresponding to the inferior PED.
b): Late phase: progressive and increasingly dense filling of a series of polypoidal formations, forming a group, like a bunch of grapes.
These high-contrast polyps were clearly visible adjacent to the well-demarcated, dark PED (Figure 3g and h).
Suggested Diagnosis:
Macular polypoidal choroidal vasculopathy with exten-
sive serosanguinous PED, progressing over several years.
Contribution of OCT (Stratus*)
Horizontal section
In the center of the fovea anterior to the RPE, a localized zone of hyper-reflectivity suggested moderate fibrosis.
Immediately adjacent to this lesion, the RPE was elevated with a moderately reflective cavity and a wavy contour, corresponding to the polypoidal formations.
Slight shadowing was observed with no increase in retinal thickness (Figure 4).
Vertical section
Multiple irregularities of thickness and reflectivity were observed in the RPE with slight elevation in the central zone.
Anterior to the RPE, increased retinal thickness and diffuse intraretinal fluid with a few cysts (Figure 4).
Diagnosis
The diagnosis was affirmed by the presence of
poly-
OCT examination
alone could not provide a diagno-
poidal formations
in the superior part of this PED.
sis at this stage, but it did confirm the presence of a
The existence of an exudative and serosanguinous
moderately reflective PED
with a minor intraretinal
reaction with recent decline of VA justified immediate
exudative reaction.
treatment.
Correlation with angiographies
confirmed the diag-
nosis of a lesion comprising:
Although treatment of this disease has not been
▬
An atrophic zone in the superior and foveal part, and
clearly established, therapy by
intravitreous injections
▬
A vast, inferiorly eccentric serosanguinous PED.
of anti-VEGF
was offered.
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CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY
Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy.
a
b
c
d
Bullous Serous PED
e
f
g
h
Polyps
Sero-hemorragic PED
Figure 3: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 20/50.
a): Color fundus photograph. b): Monochromatic red-free fundus photograph. c): Autofluorescence. d): Infrared. e and f): Fluorescein angiography. g and h): SLO-ICG angiography
Polyps
Fibrosis
Figure 4: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 20/50.
Stratus* horizontal and vertical sections.
The horizontal section in the center of the fovea, anterior to the RPE, reveals a localized zone of hyper-reflectivity suggestive of moderate fibrosis. Adjacent to this lesion, elevation of the RPE with a moderately reflective cavity and a wavy contour corresponded to the polypoidal formations.
The vertical section shows multiple irregularities of thickness and reflectivity of the RPE. Anterior to the RPE, increased retinal thickness with several small cysts.
CLINICAL CASE No. 01: Polypoidal Choroidal Vasculopathy
Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy.
Treatment by monthly intravitreous injections of antiVEGF (Lucentis*) was continued for more than 2 years with follow-up by visual acuity, OCT, fluorescein angiography, and ICG angiography. The response to treatment was favorable but often incomplete and marked by several recurrences.
After the 8th injection, the OCT horizontal section showed a satisfactory appearance of the fovea with restoration of a normal profile and resolution of edema. This was accompanied with a gain in visual acuity.
Fluorescein Angiography
During the first year, the patient was followed by conventional Stratus* TD-OCT (Figure 5).
During the second year, follow-up was based on simultaneous angiography and SD-OCT (Spectralis*) (Figure 5).
Analysis of the course over the first year
Visual acuity slowly improved, providing the patient with 10 more comfortable vision, although it remained at a relatively low level after each injection. Visual acuity stabi-
lized at 20/100 or 20/63 throughout this period.
OCT
On the first follow-up examination, retinal thickness gradually decreased. Intraretinal fluid resolved, and the foveal depression became more clearly visible (Figure 5a).
IVT injections were regularly repeated due to persistence of the angiographic signs and development of progressive macular fibrosis. This justified combined IVT and photodynamic therapy (Figure 5b).
After the 6th injection, a recurrence was observed with return and extension of a serosanguinous detachment, structural changes of the polyps, and formation of cystoid macular edema (Figure 5c).
All of the abnormal choroidal vascular network persisted with changes in the structure and number of polyps but with no increase in the surface area of the lesion.
Fluorescein angiography demonstrated alteration of the RPE in the foveal region and persistence of the pigment epithelium detachment (Figure 5a-d).
ICG Angiography
The choroidal vascular network and the polyps were clearly visualized, allowing analysis of morphological changes as well as extension and recurrence of the serosanguinous PED (Figures 5, 6, and 7).
During the second year
The lesions gradually healed: the PED became flattened and organized, bright hyper-reflective spots decreased, and the outer nuclear layer became more clearly visible.
However, the foveal zone presented features of RPE atrophy and a small band of fibrosis (Figure 7).
At the end of the second year, this patient developed exudative phenomena with degenerative cystoid edema and an epiretinal membrane (Figure 8).
At this Stage
Lasting stabilization of the lesion with limited im-
Persistence of the choroidal vascular abnormalities,
provement but acceptable visual acuity justified
polyps, fibrosis, cystoid edema, and extrafoveal PED
suspension of IVT injections (
Figure 5
). This im-
justified continued follow-up throughout the sec-
provement was slow and only partial, marked by
ond year with a poor long-term prognosis (
Figures
an episode of recurrence that responded to treat-
7, 8, and 9
).
ment.
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CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY
Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy.
FA
a
3rd month
ICG
After IVT-3
Figure 5: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 1.5/10.
a): After the 3rd IVT injection: stabilization of the lesions, decreased exudation, persistence of the polyps, and serosanguinous PED.
FA
b
7th month
ICG
Fibrosis
After IVT-5 + PDT
Figure 5: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 1.5/10.
b): After the 5th IVT injection: retinal reattachment but subfoveal fibrosis and persistence of the polyps.
FA
c
1
st
year
ICG
Cystoid spaces
After IVT-7
Figure 5: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 2/10.
c): After the 7th IVT injection : slight functional improvement despite the development of cystoid macular edema.
FA
d
13th month
ICG
Recurrence with hemorrhage
After IVT-9
Figure 5: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 3/10.
d): After the 9th IVT injection: improvement of VA with retinal reattachment despite a recurrent hemorrhagic PED.
CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY Follow-up by TD-OCT and SD-OCT after anti-VEGF therapy.
________________
ENLARGED IMAGES
FA
ICG
Polyps
Sero hemorragic PED
a
3rd month
10
After IVT-3
FA
ICG
d
13th month
Recurrence with hemorrhage
After IVT-9
Figure 6: Polypoidal choroidal vasculopathy with serosanguinous PED.
Enlarged views of Figures 5a and d.
Chapter 10 · Polypoidal Choroidal Vasculopathy
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CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY
FOLLOW-UP AFTER TREATMENT
a
b
c
Fibrosis
Abnormal choroidal network
d
PED Polyp
e
Progressive Scarring
Fibrosis
Outer Nuclear Layer
.…RPE Atrophy .…
Figure 7: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 3/10.
a-e): During the first year: progressive healing: atrophy of the RPE, fibrosis and organization of the PED. Marked alterations of the outer nuclear layer, external limiting membrane, and IS/OS interface.
CLINICAL CASE No. 01: POLYPOIDAL CHOROIDAL VASCULOPATHY
FOLLOW-UP AFTER TREATMENT
a
b
c
d
10
Hyper reflective dots
Fibrotic PED
e
Residual Polyp
Figure 8: Polypoidal choroidal vasculopathy with serosanguinous PED. VA: 3/10. a-e): At the end of the second year: recurrence and deterioration of the SD-OCT signs:
▬Flat but hyper-reflective PED suggesting fibrosis;
▬Intraretinal fluid with cysts and bright hyper-reflective spots;
▬Central atrophy and loss of the outer nuclear layer in the foveal zone;
