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Neovascular Phenotypes:
Polypoidal Choroidal Vasculopathy
12
Polypoidal choroidal vasculopathy (PCV) was described
for the first time in 1982
(1).
Different names were pro-
posed like posterior uveal bleeding syndrome
(2)
or multi-
ple recurrent retinal pigment epithelium detachments in
black women
(3)
. It has a characteristic imaging expression
on indocyanine green angiography (ICG), peculiar char-
acteristics in optical coherent tomography (OCT) and
apparently different responses to treatments when com-
pared to occult or classic choroidal neovascularization.
Diagnosis is based on ICG and confirmed with fundus
characteristics and OCT findings. The primary abnor-
mality involves the choroidal circulation and the char-
acteristic lesion is an inner choroidal vascular network
of vessels ending, in the great majority of cases, in an
aneurismal dilatation. Clinically a reddish orange, spher-
oid, polyp-like structure may be observed. The natural
course of the disease often follows a remitting-relapsing
course, and clinically, it is associated with chronic, mul-
tiple, recurrent serosanguineous detachments of the reti-
nal pigment epithelium and neurosensory retina with
long-term preservation of good vision
(4)
. A more recent
knowledge has expanded the spectrum of PCV allowing
us a clear characterization of PCV as a distinct subtype of
exudative age-related macular degeneration (AMD) eas-
ily differentiated from other diseases and other subtypes
of choroidal neovascularization associated with AMD.
2. Epidemiology
PCV is usually diagnosed in patients between 50 and 65
years old but the age of diagnoses may range between
20 and more than 80 years. Most patients with PCV are
likely to have AMD signs. Prevalence of PCV in patients
with AMD signs, ranged between 4.8% and 23% in dif-
ferent series and different countries
(3-9)
. It is considered
to be more prevalent in Asian population
(5)
and African
American than in Caucasian
(3,4,6,7)
as it seems to preferen-
tially affect pigmented individuals. Preference for female
gender is referred in Caucasian
(4,6,7)
whilst in Asian popu-
lation male are more affected
(5,8)
. Bilateral involvement is
common and may be as high as 86%
(6)
.
3. Pathogenesis
The pathogenesis of PCV is not completely understood.
It is widely accepted to be originated at the inner cho-
roidal level. Polyps may develop from a choroidal vascu-
lar network or from a plaque of occult new vessels
(4,7,8)
.
Yuzawa et al.
(8)
described the filling of PCV lesion simul-
taneously with the surrounding choroidal arteries sug-
gesting that PCV lesions grow from inner choroidal ves-
sels. Few clinicopathological studies have been reported.
MacCumber et al.
(9)
examined an enucleated high myo-
pic eye with rubeosis and vitreous haemorrhage from
a diabetic patient without diabetic retinopathy, with
high blood pressure and history of multiple, bilateral,
recurrent neurosensory and pigment epithelium detach-
ments. Bruch’s membrane was crossed by choroidal
vessels and an extensive fibrovascular proliferation was
disclosed within Bruch’s membrane and the inner reti-
nal space. They did not observe choroidal saccular dilata-
tions except that some choroidal veins were quite large.
Inflammation was expressed by the presence of B and
T lymphocytes at the level of choroid and fibrovascular
tissue and the expression of intercellular adhesion cyto-
kines like ICAM-1 was shown. Lafaut et al.
(10)
reported
the histopathologic features of surgically removed sub-
macular tissue from an elderly patient with a pattern of
polypoidal choroidal vasculopathy on indocyanine green
angiography. A thick fibrovascular membrane located
on the choroidal side of the retinal pigment epithelium
(RPE) was described. The RPE layer was discontinuous
1. Introduction
Author:
Rufino Silva, MD, PhD
Coimbra University Hospital - Coimbra, Portugal
