Polypoidal choroidal vasculopathy

1. POLYPOIDAL CHOROIDAL
VASCULOPATHY
Sujay Chauhan

2. Yannuzzi et al, 1982
Idiopathic polypoidal choroidal vasculopathy (IPCV)
Polypoidal subretinal vascular lesions associated with serous and
hemorrhagic detachments of the retinal pigment epithelium
Yannuzzi LA: Idiopathic polypoidal choroidal vasculopathy. Presented at the Annual Macular Society
Meeting, Miami, FL, 1982

3. Kleiner et al, 1984
Posterior Uveal Bleeding Syndrome (PUBS)
Peculiar hemorrhagic disorder of the macula, characterized by
recurrent subretinal and subretinal pigment epithelium bleeding
Kleiner RC, Brucker AJ, Johnson RL: Posterior uveal bleeding syndrome. Ophthalmology 1984; 91(Suppl
9):110.

4. Stern et al, 1985
Multiple recurrent retinal pigment epithelial (RPE) detachments in
black women
Stern RM, Zakov N, Zegarra H, et al: Multiple recurrent serous sanguineous retinal pigment epithelial
detachments in black women. Am J Ophthalmol 1985; 100:560–569.

5. Angiographic definition
• Presence of single or multiple focal nodular areas of
hyperfluorescence arising from the choroidal circulation within
the first 6 minutes after injection of indocyanine green, with or
without an associated choroidal interconnecting vascular network
• Presence of orange-red subretinal nodules with corresponding
indocyanine green hyperfluorescence is pathognomonic of PCV
PCV Roundtable Meeting Panel of Experts, Singapore, 2010

6. PATHOGENESIS
• Not clearly understood
• Propensity of choroidal vasculature for dilation and aneurysmal
formation

7. HISTOPATHOLOGY
Lafaut et al, 2000
Sub-RPE, intra-Bruch’s fibrovascular membrane containing dilated
thin-walled vesselsIslands of lymphocytic infiltration
Lafaut BA, Aisenbrey S, van den Broecke C, et al: Polypoidal choroidal vasculopathy pattern in age-
related macular degeneration. A clinicopathologic correlation. Retina 2000; 20:650–654.

8. Kuroiwa, 2004
Arteriosclerosis of choroidal vessels
Kuroiwa et al. Pathological features of surgically excised polypoidal choroidal vasculopathy
membranes. Clin Exp Ophthalmol 2004;32:297-302.

9. Nakashizuka et al, 2008
• Massive exudative change and leaking
• Hyalinization of vessels
• Disappearance of choriocapillaries
• Little granulation tissue
Nakashizuka et al. Clinicopathologic findings in polypoidal choroidal vasculopathy. Invest Ophthalmol
Vis Sci 2008;49:4729-4737.

10. DEMOGRAPHICS
• Blacks, Hispanics and Asians > whites
(AMD – Whites > blacks)
• Middle aged, 50 - 65 years (range 20-80 years)
• F:M = 4.7:1 (M>F in Asians)*
*Uyama M, Wada M, Nagai Y, et al: Polypoidal choroidal vasculopathy: natural history. Am J
Ophthalmol 2002; 133:639–648.

11. CLINICAL FEATURES
• Usually bilateral
• Dilated, choroidal vascular channels ending in orange, bulging,
polyp-like dilations in the peripapillary and macular area
• Vitreous hemorrhage, minimal fibrous scarring, absence of
drusen
• Serous exudation and hemorrhage - detachment of RPE and
sometimes NSR

12. CLASSIFICATION
Japanese Study Group of Polypoidal Choroidal Vasculopathy. [Criteria for diagnosis of polypoidal
choroidal vasculopathy.] Nippon Ganka Gakkai Zasshi 2005;109:417–27.
Japanese Study Group of Polypoidal Choroidal Vasculopathy
• Quiescent
• Exudative
• Hemorrhagic

13. QUIESCENT PCV
Polyps in absence of subretinal or intraretinal fluid or hemorrhage

14. EXUDATIVE PCV
Exudation without hemorrhage
Retinal thickening, NSD, PED, subretinal lipid exudation

15. HEMORRHAGIC PCV
Hemorrhage with or without other exudative characteristics

16. Depends on the affected vascular channels:
• Involvement of outer choroidal vessels – larger lesions – easily
diagnosed on biomicroscopy
• Involvement of middle choroidal vessels – smaller lesions –
angiography
SIZE

17. NUMBER AND ARRANGEMENT
• Solitary (arbitrarily defined as one or two polyps)
• Multiple – Arranged in:
– Ring (or whorl) pattern, or
– Cluster (or bunch of grapes)

18. • Peripapillary - >1/2 of the lesion (polyp and BVN) located within
1500 µm of disc margin
• Macular - >1/2 of the lesion within 6000 µm of centre of foveal
avascular zone excluding the peripapillary zone
• Extra-macular - >1/2 of the lesion located outside the above-
mentioned two zones
• Multi-location - lesion straddles over all three regions with none
of them containing >1/2 of the lesion, or if there is >1 non-
contiguous lesion
LOCATION

19. Macula
(most common)
Under temporal
retinal vascular
arcade
Peripapillary
Mid-periphery

20. Clinical, OCT or FA evidence of any 1 of the following:
• Vision loss of ≥5 letters (ETDRS chart)
• Subretinal fluid with or without intraretinal fluid
• Pigment epithelial detachment
• Subretinal hemorrhage
• Fluorescence leakage
CHARACTERISTICS OF ACTIVE PCV

21. • Chronic atrophy and cystic degeneration of fovea
• Vitreous hemorrhage
• Secondary CNV with disciform scarring
• Massive spontaneous choroidal hemorrhage – rare – poor visual
outcome despite immediate drainage
CAUSES OF DECREASED VISION

22. DIFFERENTIAL DIAGNOSIS
Reddish-orange, subretinal mass-like lesion
• Neovascular age-related macular degeneration (AMD)
• Central serous chorioretinopathy (CSCR)
• Pathological myopia with neovascularization
• Choroidal hemangioma, osteoma
• Metastases
• Posterior scleritis

23. TYPICAL AMD PCV
Grayish membrane Reddish mass, nodular elevation
Solitary, central macula Multiple centers, paramacular
Drusen in involved and fellow eye No drusen
Older age group Younger age group
More aggressive growth
Rapid drop in vision
Slow growth, wax & wane
Months to years
End stage - Fibrosis, disciform scar Minimal scarring
FFA: Classic or occult Mostly occult
ICGA: Smaller vessel involvement
Hot spot or plaque
Diffuse late staining
Aneurysm-like dilation
Interconnecting BVN
OCT: Diffuse retinal edema
Intraretinal cyst
Diffuse sub-RPE thickening
Nodular RPE detachment
Sub-retinal fluid

24. INVESTIGATIONS
• Indocyanine green angiography (ICGA)
• Fundus fluorescein angiography (FFA)
• Optical coherence tomography (OCT)

25. INVESTIGATIONS
• Indocyanine green angiography (ICGA)
• Fundus fluorescein angiography (FFA)
• Optical coherence tomography (OCT)

26. INDOCYANINE GREEN ANGIOGRAPHY (ICGA)
• Gold standard
• ICG absorbs and emits near-IR light – readily penetrates RPE
• High binding affinity to plasma proteins – does not leak from
choriocapillaries – better resolution of choroidal vasculature

27. Initial phase
• Nodular hyperfluorescence (90%)
• Branching vascular network of inner choroidal vessels (75%)
• Hypofluorescent halo in first 6 minutes (45-50%)

28. Mid phase
Lesions appear to leak slowly and surrounding hypofluorescent area
becomes increasingly hyperfluorescent

29. Late phase
Reversal of pattern of fluorescence
Area surrounding lesion becomes hyperfluorescent and center of
lesion demonstrates hypofluorescence (60-63%)
Hyperfluorescent plaque/geographic hyperfluorescence (55-60%)

30. Very late phase
Disappearance of fluorescence from lesions - ‘washout’
(seen in non-leaking lesions; leaking lesions remain hyperfluorescent)

31. • Cluster of hyperfluorescent dots (25%)
• Pulsation in polyps (4-21%) - seen on video ICGA
OTHER CHARACTERISTIC FEATURES OF PCV ON ICGA

32. GUIDELINES FOR DIAGNOSIS
Japanese Study Group, 2005
Definite cases - At least one of the following:
• Protruding elevated orange-red lesions on fundus examination
• Characteristic polypoidal lesions seen on ICGA
Probable cases - At least one of the following:
• Only an abnormal vascular network seen on ICGA
• Recurrent hemorrhagic and/or serous detachments of RPE

33. Everest Criteria
Subretinal focal ICGA hyperfluorescence (within 5 minutes)
plus
At least one of the following angiographic or clinical criteria:
• BVN - Abnormal vascular channel(s) supplying the polyps
• Pulsatile polyp
• Nodular appearance when viewed stereoscopically
• Presence of hypofluorescent halo
• Orange subretinal nodule on colour photograph
• Massive submacular haemorrhage

34. Total lesion area for PCV
Area of all polyps and the BVN as imaged by ICGA

35. INVESTIGATIONS
• Indocyanine green angiography (ICGA)
• Fundus fluorescein angiography (FFA)
• Optical coherence tomography (OCT)

36. FUNDUS FLUORESCEIN ANGIOGRAPHY (FFA)
• Lesser resolution of choroidal vasculature
• Able to show large polypoidal changes
• PCV lesions resemble occult CNVM lesions and when submacular,
they can be mistaken for AMD

37. Vascular lesion in peripapillary area
Serosanguineous PED in inferior and temporal macular area

38. INVESTIGATIONS
• Indocyanine green angiography (ICGA)
• Fundus fluorescein angiography (FFA)
• Optical coherence tomography (OCT)

39. Thumb-like polyps
Sharp PED peaks

40. Double-layer sign
RPE & another highly reflective layer underneath (correlates with BVN)

41. Triple-layer sign
Hyporeflective space between sub-RPE neovascular tissue (type I
CNVM) and underlying choroid

42. String of pearls appearance
Multiple PCV structures adherent to under-surface of a detached RPE

43. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

44. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

45. OBSERVATION
Unless there is persistent or progressive exudative change
threatening central vision

46. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

47. THERMAL LASER PHOTOCOAGULATION
• Extrafoveal PCV
• Stable or improved vision: 55-100 %
• Vision loss: 13-45 %
• Entire PCV lesion (polyps + BVN) should be treated
• Kwok et al. Polypoidal choroidal vasculopathy in Chinese patients. Br J Ophthalmol 2002;86:892–7.
• Lee et al. Argon laser photocoagulation for the treatment of polypoidal choroidal vasculopathy. Eye
(Lond) 2009;23:145–8.
• Yuzawa et al. A study of laser photocoagulation for polypoidal choroidal vasculopathy. Jpn J
Ophthalmol 2003;47:379–84.
• Nishijima et al. Laser photocoagulation of Indocyanine green angiographically identified feeder vessels
to idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol 2004;137:770–3.

48. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

49. PHOTODYNAMIC THERAPY
• Angio-occlusion - Regression or resolution of polyps
• Suitable for subfoveal PCV
• Entire PCV lesion should be treated

50. • Improvement in visual acuity - 56% patients
• Stable vision - 31% patients
• Decrease in visual acuity - 12% patients
• Improved vision in 80-100% of patients after 1 year
• Spaide et al. Treatment of polypoidal choroidal vasculopathy with photodynamic therapy. Retina
2002; 22:529–535.
• Chan et al. Photodynamic therapy with verteporfin for symptomatic polypoidal choroidal
vasculopathy: one-year results of a prospective case series. Ophthalmology 2004;111:1576–84.

51. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

52. ANTI-VEGF THERAPY
• Ranibizumab, Bevacizumab, Aflibercept
• Resolution of macular edema
• Decreased polypoidal complexes
• Kokame et al. Continuous anti-VEGF treatment with Ranibizumab for polypoidal choroidal
vasculopathy: 6-month results. Br J Ophthalmol 2010;94:297–301.
• Gomi et al. One-year outcomes of photodynamic therapy in age-related macular degeneration and
polypoidal choroidal vasculopathy in Japanese patients. Ophthalmology 2008;115:141–6.
• Hara C et al. One-year results of intravitreal aflibercept for polypoidal choroidal vasculopathy. Retina
2015;0:1-9.

53. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

54. COMBINATION THERAPY
EVEREST TRIAL
• Verteporfin PDT monotherapy
• 0.5 mg ranibizumab monotherapy
• Combination therapy

55. • Combination therapy and verteporfin PDT monotherapy -
superior to ranibizumab monotherapy
• Combination therapy - improvements in BCVA and central retinal
thickness

56. TREATMENT
• Observation
• Thermal laser photocoagulation
• Photodynamic therapy
• Anti-VEGF therapy
• Combination therapy
MEDICAL SURGICAL

57. SURGERY
• Vitrectomy and submacular removal of polypoidal vessels and
subretinal blood
• Macular translocation
• Shiraga et al. Surgical treatment of submacular hemorrhage associated with idiopathic polypoidal
choroidal vasculopathy. Am J Ophthalmol 1999; 128:147–152.
• Fujii et al. Complications associated with limited macular translocation. Am J Ophthalmol 2000;
130:751–762.