Published on March 21, 2014
Polypoidal Choroidal Vasculopathy Dr. Md Mominul Islam Fellow Vitreo - Retina
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
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
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.
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 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.
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. PED 4. 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 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
Differential Diagnosis AMD CSCR
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.
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.
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