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Dr. K. Vasantha M.S., F.R.C.S.
Director RIO Chennai (Rtd)
 More common in pigmented races
 Age – 60 to 70. Presents earlier than age related macular
degeneration
 Choroidal vessels are primarily involved with an
aneurysmal bulge, visible clinically as sub retinal reddish
orange polyp like structure
 Recurrent serous and hemorrhagic pigmentary epithelial
detachment due to leakage from the affected choroidal
vessels
 Compared to AMD vision may remain good as the lesion
is extra foveal
 Presents with defective vision, floaters, metamorphopsia
and central scotoma
 Reddish orange sub retinal raised lesions which are
more common in peripapillary area. May involve macula
also
 Serous or sero sanguinous PED
 Sub retinal hemorrhage
 Lipid deposits due to protein leakage from polyps
 Neuro sensory retinal detachment in the peripapillary or
macular retina
 The vascular abnormality may proliferate
 Polyp may become a vascular channel
 Cluster of vascular malformation can form a reddish
orange sub retinal mass
 Flattening of this mass
 RPE atrophy
 Quiescent – polyp present but no hemorrhage
 Active – exudates and hemorrhages present.
Peripheral exudative hemorrhagic chorio retinopathy
may break through and produce vitreous hemorrhage
also
ICGA EVEREST study:
 Nodular hyper fluorescence
 Hypo fluorescent halo around the nodule. As polyps leak
the halo will disappear
 Later the core will become hypo fluorescent
 Pulsation during dynamic ICGA
 Focal hyper fluorescence is an important sign.
 Stereo photography can be used to find the exact depth
 Abnormal irregular lattice network of blood vessels that
does not follow the lobular pattern
 Later hyper fluorescent isolated or cluster of polyps will
be seen
 Choroidal hyper permeability can be seen
 Late onset hyper fluorescence is due to staining of
unhealthy pigment epithelial cells or window defects
after 6 minutes
 After 10 minutes geographic hyper fluorescence with
demarcated margin and rosette pattern are seen
 Unless there is active leakage there will be a uniform
wash out of the dye
 Late ICG staining seen in classic or occult CNV is not
seen in idiopathic polypoidal choroidal vasculopathy
 This is done to differentiate sub retinal and intra retinal
fluid.
 Thumb like raised polyp with sharp peaked PED will be
seen
 Wavy elevated RPE with or without sharp notches
 Tall peaked PED
 Notch in the pigment epithelial detachment
 Hypo reflective lumen surrounded by hyper reflective
ring attached to the under surface of the PED
 Double layer sign- shallow irregular PED with elevation
of Bruch’s membrane
 In AMD choroid will be thin. Here it will be normal or even
thicker than normal
 Deep layer of vessels in the retina shows enlargement of
the foveal avascular zone, with loss of regular round
configuration
 Outer retina shows delicate neovascular structure
 Choroid may show defect in the vasculature due to polyp
base with finely branched new vessels at the edge of the
defect
 Central serous chorio retinopathy – younger age group
 Choroidal neovascularization – fibrous proliferation is
rare in PCV
 Choroidal hemangioma, osteoma
 Carcinoid metastasis
Serous detachment of RPE is seen in other conditions like
 CNV complicating CSR
 Dome shaped macula with sub foveal detachment in
myopia
 Pachy choroid neovasculopathy
 Optic disc pit
 Lupus erythematosus
Choroidal ischemia caused by
 Accelerated hyper tension
 Preeclampsia
 Systemic corticosteroid use
 Para proteinemia
 Choroidal tumors
 Vogt Koyanagi Harada disease
 Posterior scleritis
 AMD is seen more in whites, whereas PCV is seen in
pigmented races
 AMD is associated with soft drusen
 Local pigmentation is seen in AMD
 In AMD small caliber vessels are involved
 Greyish discoloration is often seen when CNV is present
 With ICG vessel wall staining is seen
 Choroid is thinner in age related macular degeneration
 Scar with fibrosis in AMD
 Standard fluence photo dynamic therapy (PDT) with or
without Ranibizumab or Ranibizumab alone
 PDT will cause thrombosis
 Polyps will resolve completely
 Thermal laser can be used for lesions beyond 100
microns from the fovea
 Photocoagulation to the leaking vessels with ICG
guidance has also been tried

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Idiopathic polypoidal choroidal vasculopathy

  • 1. Dr. K. Vasantha M.S., F.R.C.S. Director RIO Chennai (Rtd)
  • 2.  More common in pigmented races  Age – 60 to 70. Presents earlier than age related macular degeneration  Choroidal vessels are primarily involved with an aneurysmal bulge, visible clinically as sub retinal reddish orange polyp like structure
  • 3.  Recurrent serous and hemorrhagic pigmentary epithelial detachment due to leakage from the affected choroidal vessels  Compared to AMD vision may remain good as the lesion is extra foveal  Presents with defective vision, floaters, metamorphopsia and central scotoma
  • 4.  Reddish orange sub retinal raised lesions which are more common in peripapillary area. May involve macula also  Serous or sero sanguinous PED  Sub retinal hemorrhage  Lipid deposits due to protein leakage from polyps  Neuro sensory retinal detachment in the peripapillary or macular retina
  • 5.  The vascular abnormality may proliferate  Polyp may become a vascular channel  Cluster of vascular malformation can form a reddish orange sub retinal mass  Flattening of this mass  RPE atrophy
  • 6.  Quiescent – polyp present but no hemorrhage  Active – exudates and hemorrhages present. Peripheral exudative hemorrhagic chorio retinopathy may break through and produce vitreous hemorrhage also
  • 7. ICGA EVEREST study:  Nodular hyper fluorescence  Hypo fluorescent halo around the nodule. As polyps leak the halo will disappear  Later the core will become hypo fluorescent  Pulsation during dynamic ICGA  Focal hyper fluorescence is an important sign.  Stereo photography can be used to find the exact depth
  • 8.  Abnormal irregular lattice network of blood vessels that does not follow the lobular pattern  Later hyper fluorescent isolated or cluster of polyps will be seen  Choroidal hyper permeability can be seen  Late onset hyper fluorescence is due to staining of unhealthy pigment epithelial cells or window defects after 6 minutes  After 10 minutes geographic hyper fluorescence with demarcated margin and rosette pattern are seen
  • 9.  Unless there is active leakage there will be a uniform wash out of the dye  Late ICG staining seen in classic or occult CNV is not seen in idiopathic polypoidal choroidal vasculopathy
  • 10.  This is done to differentiate sub retinal and intra retinal fluid.  Thumb like raised polyp with sharp peaked PED will be seen  Wavy elevated RPE with or without sharp notches  Tall peaked PED  Notch in the pigment epithelial detachment
  • 11.  Hypo reflective lumen surrounded by hyper reflective ring attached to the under surface of the PED  Double layer sign- shallow irregular PED with elevation of Bruch’s membrane  In AMD choroid will be thin. Here it will be normal or even thicker than normal
  • 12.  Deep layer of vessels in the retina shows enlargement of the foveal avascular zone, with loss of regular round configuration  Outer retina shows delicate neovascular structure  Choroid may show defect in the vasculature due to polyp base with finely branched new vessels at the edge of the defect
  • 13.  Central serous chorio retinopathy – younger age group  Choroidal neovascularization – fibrous proliferation is rare in PCV  Choroidal hemangioma, osteoma  Carcinoid metastasis
  • 14. Serous detachment of RPE is seen in other conditions like  CNV complicating CSR  Dome shaped macula with sub foveal detachment in myopia  Pachy choroid neovasculopathy  Optic disc pit  Lupus erythematosus
  • 15. Choroidal ischemia caused by  Accelerated hyper tension  Preeclampsia  Systemic corticosteroid use  Para proteinemia  Choroidal tumors  Vogt Koyanagi Harada disease  Posterior scleritis
  • 16.  AMD is seen more in whites, whereas PCV is seen in pigmented races  AMD is associated with soft drusen  Local pigmentation is seen in AMD  In AMD small caliber vessels are involved  Greyish discoloration is often seen when CNV is present  With ICG vessel wall staining is seen  Choroid is thinner in age related macular degeneration  Scar with fibrosis in AMD
  • 17.  Standard fluence photo dynamic therapy (PDT) with or without Ranibizumab or Ranibizumab alone  PDT will cause thrombosis  Polyps will resolve completely  Thermal laser can be used for lesions beyond 100 microns from the fovea  Photocoagulation to the leaking vessels with ICG guidance has also been tried