Idiopathic Polypoidal Choroidal Vasculopathy

1. Idiopathic Polypoidal Choroidal Vasculopathy
Presenter –Dr.Ritesh Kotecha
Moderator –Dr.Devendra Venkatramani

2. Introduction
 Polypoidal choroidal vasculopathy (PCV) is a disease
of the choroidal vasculature.
 Serosanguineous detachments of the pigmented
epithelium and exudative changes leading to
subretinal fibrosis

3.  A 78 years old female patient housewife by occupation
residing at New Panvel visited to our institute with
c/o LE blurring of vision since one month.

4. Past ocular history
 h/o of using glasses since 40 years PGP 6months
 BE cataract surgery with iol implantation done 12
years back.
 h/o LE injection avastin 5 times and RE 3 times

6. Past medical history
 k/c/o hypertension under treatment since 10 years .

7. Systemic examination
 Patient was calm , quiet and conscious and well
oriented with time,place and person .

8. Local examination
 RE BCVA 6/12 N6 and LE 6/18N8
 On slit lamp examination ,

9. RE LE
Lid N N
Conjunctiva Quiet Quiet
Cornea Clear Clear
AC Deep and quiet Deep and quiet
Iris CPN CPN
Pupil 7mm 7mm
Lens PCIOL PCIOL
Fundus Cup disc ratio
0.5 :1 HNRR active
CNVM
Cup disc ratio
0.5 :1 submacular
haemorrhage

11. Investigation
 Fundus fluorescein angiography which reveals –RE
RPE atrophic patches and LE occult CNVM
 OCT
 RE-
 LE-

12. Treatment advised
 LE Intravitreal Anti VEGF injection

13.  During follow up period 3 intravitreal anti VEGF
injections administered .

14. One year later
 BE BCVA 6/9N6
 Slit lamp examination reveals BE PCIOL in situ
,fundus examination reveals BE CNVM

16. Investigation
 Repeat OCT BE

17. Treatment
 RE intravitreal AntiVEGF injection,

18. During Follow up
 Repeat RE intravitreal AntiVEGF injection

19. One month later
 BCVA BE 6/12NIG N6
 Fundus Examination reveals RE geographic atrophy
and LE PED approximation
 OCT BE reveals no SRF or oedema

20. 3month later
 BCVA BE 6/9N8
 Fundus examination BE reveals PED with oedema

21. Treatment advised
 BE intravitreal AntiVEGF injection

22. One month later
 BCVA BE 6/9N6
 Fundus examination reveals RE PED
 LE PED with minimal pockets of SRF

23. 3month later
 BCVA BE 6/9N6
 Fundus examination reveals –active CNVM with
oedema

24. Treatment advised
 LE intravitreal AntiVEGF injection

25. 15 days later
 BCVA RE 6/18 N6 LE 6/12N6
 Fundus examintion BE reveals PED with resolving
SRF.
 OCT
 RE –resolving SRF
 LE -resolved SRF

26. One month later
 BCVA RE 6/18 N6 LE 6/12N6
 Fundus examintion reveals –FVPED
 Adviced intravitreal AntiVEGF injection but deferred
because of lack of compliance of the patient

27. Two months later
 BCVA 6/12N6
 On examination BE scleral thinning noted
 Fundus examination reveals BE CNVEM with PED
with SRF

28. 3months Later
 BCVA RE 6/12N8,LE6/12N10
 Fundus examination reveals RE rnfl hemorrhage .
 LE reveals submacular haemorrhage .

30. Investigation
 RE perimetry
 BE OCT

31. Treatment advised
 LE intarvitreal Anti VEGF

32. One month later
 BCVA BE 6/9N6
 Fundus examination reveals RE active CNVM LE
submacular haemorrhage

34. Investigation advised
 ICGA reveals IPCV.

35. Treatment advised
 BE PDT and intarvitreal Anti VEGF

36. History
 Was first described by Dr. L Yannuzzi in 1982.

37. Prevalence and Incidence:
 Most commonly diagnosed in patients between the
ages of 50 and 65 years
 In Asians , Japan have higher prevalence rates are
present of approximately 23% to 54% in patients with
presumed age-related macular degeneration (AMD)

38. Etiology
 The etiology is not clearly understood.
 It has been proposed that there is a choroidal
vasculature propensity for dilation and aneurysmal
formation

39. Investigation of choice
 ICGA - The intravascular retention of the ICG
molecule allows better resolution of the choroidal
vasculature.
 Fluorescein angiography (FA) -is not as useful because
it lacks the same resolution of the choroidal
vasculature as ICGA. However, it is able to show large
polypoidal changes.

40. Symptom
 Blurred vision
 Central or paracentral scotoma
 Diminision of vision in the affected eye.

41. Investigation
 FFA-
 PCV lesions on FFA resemble occult CNVM lesions and
when submacular, they can be mistaken for AMD
 OCT-
 Helpful in identification of subretinal or sub-RPE fluid,
it can also delineate polypoidal lesions
 These lesions resemble dome-like elevations of RPE
with moderate internal reflectivity.
 In most cases, there is also a highly reflective line just
below these lesions consistent with location of vascular
branching network.
 The dual reflective layers are also called “double-layer
sign,” and are seen in 59% of eyes with PCV

42. ICGA
 The polyps present as focal hyperfluorescent spots. In
later stages, reversal pattern of dye is seen with the
center of the lesion becoming hypofluorescent and
surrounding becoming hyperfluorescent. Finally, in
the very last stage, there is “wash-out” of the lesion
that is seen in non-leaking PCV lesions

43.  Spectral-domain optical coherence tomography (SD-
OCT) is currently one of the best available
noninvasive imaging tools for the management of
PCV.

44. Differential diagnosis
 Age-related macular degeneration
 Central serous chorioretinopathy,
 Pathological myopia with neovascularization,
 Choroidal tumors
 Metastases.

45. Treatment
 Medical therapy
 Observation,
 Photodynamic therapy,
 Intravitreal injection of anti-VEGF therapy
 Combination therapy

46. EVEREST trial
 Multi-center, double-masked trial
 Compared three treatment regimens: verteporfin
photodynamic therapy (PDT) plus the anti-VEGF
agent ranibizumab (Lucentis), ranibizumab
monotherapy, and PDT monotherapy.

47. Cont...
 The patient population was sixty-one Asian patients
with symptomatic PCV.
 The primary end point was complete polyp
regression as assessed by ICG.
 PDT in combination with ranibizumab and PDT
monotherapy showed a significantly higher
proportion of patients with complete polyp regression
at month 6 than the ranibizumab monotherapy group

48.  Medical follow up
 Regardless of choice of treatment, patients should be
followed on regular intervals to detect and prevent
subretinal/subRPE fluid and hemorrhage.

49. Surgery
 There is no current surgical management for PCV.
 If surgical management is needed, it would be tailored
to complications and sequelae of PCV such as break-
through vitreous hemorrhage.

50. Complications
 Serosanguineous detachments of the pigmented
epithelium and exudative changes
 Subretinal fibrosis
 Pigment epithelial hyperplasia,
 Atrophic degeneration

51. Prognosis
 Depending on the extent of area involved prognosis is
generally good
 Symptomatic patients with PCV can have complete
regression without severe vision loss with PDT and
anti-VEGF treatment

52. General Principles of ICG Angiography
1. Binding
• 98% bound to proteins
2. Fluorescence
• Much less than fluorescein
• Less leakage from choriocapillaris
• Excitation peak 800 nm
• Emission at 835 nm
3. Filters
• Infrared barrier and excitation
4. Safer than fluorescein

53. Phases of normal ICG angiogram (1)
Early (20 sec)
• Disc hypofluorescence
• Poor perfusion of vertical
(watershed) zone near disc
• Prominent filling of choroidal arteries
• Early filling of choroidal veins
• Filling of retinal arteries but not veins
Early middle (3 min)
• Filling of watershed zone
• Fading of choroidal arterial filling
• Prominent filling of choroidal veins
• Filling of retinal arteries and veins

54. Phases of normal ICG angiogram
Late (21 min)
• Large choroidal and retinal vessels are
empty
Late middle (6 min)
• Reduced filling of choroidal vessels
• Diffuse hyperfluorescence due to
diffusion of dye from choriocapillaris
• Persistent filling of retinal vessels
• Diffuse background hyperfluorescence