1. Polypoidal Choroidal
Vasculopathy
Dr. Md Mominul Islam
Fellow Vitreo - Retina

2. Introduction
 PCV is an exudative maculopathy with features similar
to neovascular AMD with hemorrhage, pigment
epithelium detachment and neurosensory
detachment.
 Pathogenesis mostly unknown
 It remains controverial as to weather PCV represents a
sub type of neovascular AMD

4. History
• In 1982 By Yannuzzi et al
• At annual meeting of American academy of
ophthalmology as :-
 Polypoidal , subretinal, vascular lesions associated
with serous and hemorrhagic detachments of the RPE
in a series of patients (10/11 patients were woman).
 The entitity was initially called Idiopathic
polypoidal choroidal vasculopathy.

5. History (contd) In 1984 Kleiner et al Described as:-
 A peculiar hemorrhagic disorder of the macula and
sub retinal pigment epithelium bleeding in middle
aged black woman ,which they term posterior uveal
bleeding syndrome.
 Later a study from the same group of authors showed
an expanded clinical spectrum for PCV

6. Pathogenesis
 It is a primary abnormality of the choroidal
circulation, characterized by :-
 An inner choroidal vascular network of vessels ending
in an aneurysmal bulged or outward projection often
visible as a reddish – orange, spheroid ,polyp like
structure.
 PCV primarily involves the inner choroidal vasculature
that is well differentiated from the middle and larger
choroidal vessels by histology.

7. Histopathological Features
 Kuroiwa reported from surgically excised specimens
from 5 patients with PCV had been diagnosed by ICGA
:--
 Results - arteriosclerosis appears to be an important
pathological feature in the choroidal vessels of PCV
subjects.

8. Histopathological Features (Contd)
In another histopathologic group :
• Nakashizuka et al, who also examined from surgically
excised specimens from 5 patients with PCV
Results :- little granulation tissue from any of the
specimens.
On the other hand all the specimen exhibited:-
• Massive exudative change and leaking ,all the vessels
exhibited hyalinization and chorio-capillaris had
disappeared ,in which RPE had been preserved.

9. Immuno ChemistryThis group demonstrated that :
• PCV is not the same as choroidal neovascularization.
• CD-34 is a marker of vascular endothelial growth factor
• CD-34 staining revealed
 Discontinuity in the vascular
endothelium.
 Smooth muscle actin was negative in
hyalinized vessels
 There was disruption and injury of
smooth cells causing dilation of vessels

10. Demography
Prevalence of PCV in presumed neovascular AMD
• US -7.8%
• Belgian – 4.0%
• Italian - 9.8 %
• Greek – 8.2%
• Japanese – 23.0-54.7%
• Chinese – 22.3%
• Korean – 24.6%

11. Demography (contd)
 Varies with age
 Predominantly middle aged ,one or two decade earlier
than classical AMD
 Commonly occur both gender ( more in Asian man
than woman)
 More prevalent in black, Japanese and other Asian
than in white.
 Incidence is high in Asian.

12. Course
Natural course of disease is Variable:-
 May be relatively stable
 May repeated bleeding and leakage with vision loss and
chorioretinal atrophy
 With or without fibroitic scarring
 Reddish- orange nodules alone or nodule and small sub-
retinal hemorrhage and absence of hard exudates.
 May association with other condition like
thrombocytopenia and massive hemorrhage has been
reported
 Also association with sickle cell disease and irradiation.

13. Clinical feature Usually bilateral
 Protruding orange –red elevated lesion often with
nodular elevations of RPE.
 Also described as –
 Polypoidal lesion ,polyp like or grasp like
 Association serous exudation and hemorrhage
, that may lead to detachment of the RPE and
sometimes neurosensory retina
 Sometime associated features recurrent
hemorrhage and vitreous hemorrhage.

14. Clinical feature (contd)
Location of the lesion:
 In the macular area -69.5%
 Under the temporal retinal vascular arcade
15%
 Peripapillary area (within one disc
diameter of disc edge -4.5%.
 Also mid peripheral area

15. Clinical feature (contd)Lesions may active or inactive.
• Lesions considered as active on the evidence of
clinical, OCT, FFA any one of the following:
1. Vision loss of 5 or more letters (ETDRS
chart)
2. Subretinal fluid with or without
intraretinal fluid.
3. PED
4. Subretinal hemorrhage or fluorescence
leakage.

16. ICGA :-
• Accepted as the gold standared for diagnosis of PCV.
• Better visualization in ICGA than FFA
 ICG absorbs and
emit near infrared
light, which readily
penetrates the RPE
and enhancing veiwing
of choroidal lesions
 ICG binding affinity
with plasma
proteins, that means –
it does not leak from
chorio-capillaries in the
same way as
fluorescence, so
choroidal lesions are
less obscured.

17. Characteristics feature of PCV in ICGA:-
 A branching network of inner choroidal vessels.
 Nodular polypoidal aneurysm or dilations at the edge
of these abnormal vessels network, which correspond
to orange sub-retinal nodules.
 Presence of single or multiple focal nodular areas of
hyperfluorescence arising from choroidal circulation
within the first 6 minutes

20. Classification
According to Japanese group on PCV
Quiescent: Polyps in the absence of subretinal or intraretinal fluid
or hemorrhage.
Exudative : Exudation without hemorrhage, which may include
sensory retinal thickening, Neuro-sensory retinal
detachment, PED, subretinal lipid exudation.
Hemorrhagic: Any hemorrhage with or without other exudative
characteristics

21. Differential Diagnosis
 AMD
 CSCR

22. Treatment
Thermal laser Photocoagulation:
To be beneficial ,albeit it with short term follow up
and for extra foveal lesion.
In some studies
 Improve and stabilize vision- in 55-100%
 Vision loss – in 13-45%
 Whole lesion compared with polyps only appears to
be more efficacious.

24. Photodynamic therapy:
 Regression or resolution of the polyp by in angio
occlusive mechanism of action
 Restrict loss of letters on ETDRS chart to less than
15 letters or improved vision 80-100% patient after 1
year.

25. Anti VEGF therapy:
 Intravitreal Ranibizumab resolving the macular
oedema, polypoidal complexes decreased in 4/12 (33%)
eyes.
 Intravitreal Bevacizumab 1/11 (9.09%) eyes

26. Combination therapy:
 Both combination therapy and vertiporfin PDT
monotherapy superior to ranibizumab monotherapy
in achieving complete polyp regression at 6 months.
 Improvement in BCVA and central retinal thickness
also favored combination therapy.

27. THANK YOU