IntroductionPCV is an exudative maculopathy with

features similar to neovascular AMD with hemorrhage, pigment epithelium detachment and neurosensory detachment.

Pathogenesis mostly unknownIt remains controverial as to weather PCV

represents a sub type of neovascular AMD

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.

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

PathogenesisIt 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.

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.

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.

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

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%

Demography (contd)Varies with age Predominantly middle aged ,one or two

decade earlier than classical AMDCommonly 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.

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.

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.

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

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. PED4. Subretinal hemorrhage or

fluorescence leakage.

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.

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

Classification

Differential DiagnosisAMDCSCR

TreatmentThermal 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.

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.

Anti VEGF therapy: Intravitreal Ranibizumab resolving the

macular oedema, polypoidal complexes decreased in 4/12 (33%) eyes.

Intravitreal Bevacizumab 1/11 (9.09%) eyes

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.