146
vasculopathy (PCV)
(30)
. PCV is a peculiar form of cho-
roidal neovascularization, characterized by the presence
of orange, aneurismal, polyp-like round dilatations at the
border of a branching vascular network from choroidal
origin.
Although PCV afflicts more frequently middle-
age black and Asian populations, its clinical spectrum is
expanded to whites, where it has been found to be pres-
ent in 8-13% of patients with concomitant AMD lesions.
In these cases, when the manifestations attributable to
both PCV and AMD are present, some authors consider
PCV as a subtype of CNV in AMD
(30,31)
. Hemorrhagic
manifestation are common in patients with PCV. Serous
PED associated with PCV show frequently a blood level
in the lower portion of the detachment. ICGA is the state-
of-the-art examination to distinguish the typical features
of the two vascular components. The vascular network
is characterized by the presence of one or more aneuris-
mal lesions that show a bright fluorescence since the early
phases, followed in the late phases by a clearing of the dye,
called “wash-out”, typical of this disease (Fig. 3).
Nevertheless, some polyp-like structure can actively
leake showing late staining of their walls and surround-
ing exudation. The polypoidal lesions are usually located
at the margin of the serous PED
(32)
. Recognition of these
lesions appears fundamental because clinical course,
prognosis and treatment response of PCV and RAP are
different from that of CNV.
Optical coherence tomography (OCT) provides images
that allows an exact correlation with both the angio-
graphic exams findings. In OCT cross-sectional scans,
serous PED appears as an optically empty dome-shaped
elevation of the external high reflective band – the RPE -,
that steeply detaches fromBruch’smembrane
(33)
.The over-
lying retina, usually adherent to the bullous PED, at the
margins of the lesion can be slightly detached from the
underlying RPE. More additional information can be
provided by OCT in vascularized PED
(34)
. The tomo-
graphic sections, guided by FA and ICGA in the area
corresponding to the CNV, show a smoother elevation of
the RPE, continuous with the serous detachment, with
a deeper backscattering, due to the presence of the fibro-
vascular tissue. Hyporeflective areas of homogeneous
optically empty spaces referable to fluid accumulation
are frequently present in the intraretinal and subretinal
spaces
(35)
.
Intraretinal optically empty spaces are more
pronounced when the serous PED is associated with a
RAP, specially with cystic shape. By positioning the scan
line corresponding to the “hot-spot”, the neovascular
abnormality is represented as a dense or hyperreflective
pre-epithelial zone in the inner retinal layers, where the
outer hyperreflectant layers are no longer detectable
(36)
.
The RPE close to that lesion shows frequently effractions
or interruptions in its hyperreflective layer
(37)
.The retinal
topographic measurement sustains an increased retinal
thickness. Even though it has been hypothesized that the
intraretinal neovascularization should be connected to
the choroid with an anastomotic vessel, no clear enough
images that can bear its presence in the optical free area
under the PED can be detected by OCT (Fig. 4-A).
In eyes with serous PED and PCV, the polypoidal ecta-
sies show a sharp protrusion of RPE, similar to the
PED but steeply sloped. The polyps cavity, usually opti-
cally empty, is contiguous to irregular RPE elevation,
expression of the occult neovascular component of the
lesion
(38,39)
. Subretinal and intraretinal fluid, observed as
hypofluorescent optical empty areas, are related to the
PCV’s activity (Fig. 4-B).
Serous PED natural course depends on the presence or
not of the neovascular component
(40)
. In pure serous
PED there is generally a slow enlargement of the lesion,
with a minimal progression of visual loss over a long
period (months or years). However, many of them can
subsequently develop neovascularization, that wors-
ens their prognosis
(8)
. Different natural course occurs
indeed in vascularized PED, and it’s related to the type of
newvessels associated. The most common acute compli-
cation is the tearing of the RPE
(6,41-43)
. It usually occurs at
the edge of the PED, at the intersection of the detached
and attached RPE. Clinically, a RPE tear or rip appears
as a well-defined area of bare choroid, contiguous to a
darker hyperpigmented rugate area, that corresponds
to the mound of the RPE that has torn away
(44,45)
. The
ripped RPE usually rolls towards the occult CNV, and
its propensity to tear can be predicted by the observation
of pre-tear characteristics, such as an increase in the size
and a modification in the shape, the presence of small
holes at the PED margins, the presence of hemorrhages
or subretinal fluid, but the most noteworthy aspect is
the irregular filling of the PED visible at the FA
(46)
. RPE
tears occurs either spontaneously, either after a treat-
ment, formally laser photocoagulation, photodynamic
therapy and intravitreal injection of steroids or anti-
VEGF agents
(47- 57)
. The exact pathogenesis of RPE tears
is poorly understood. Concerning PEDs’ natural course,
it has been hypothesized that tangential shearing forces
in the PED can cause the break of the RPE basement
membrane at the edge of the detachment; however it is
more likely the result of several variables, where the pres-
ence of a CNV plays a major role. When RPE tear occurs
after a treatment, various other causal relationships have
