Idiopathic Polypoidal Choroidal Vasculopathy

Presenter –Dr.Ritesh Kotecha Moderator –Dr.Devendra

Venkatramani

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

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.

Past ocular history h/o of using glasses since 40 years PGP

6monthsBE cataract surgery with iol implantation

done 12 years back.h/o LE injection avastin 5 times and RE 3

times

Past medical history k/c/o hypertension under treatment since 10

years .

Systemic examination Patient was calm , quiet and conscious and

well oriented with time,place and person .

Local examination RE BCVA 6/12 N6 and LE 6/18N8On slit lamp examination ,

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 ratio0.5 :1 HNRR active CNVM

Cup disc ratio0.5 :1 submacular haemorrhage

Investigation Fundus fluorescein angiography which

reveals –RE RPE atrophic patches and LE occult CNVM

OCT RE-LE-

Treatment advised LE Intravitreal Anti VEGF injection

During follow up period 3 intravitreal anti VEGF injections administered .

One year later BE BCVA 6/9N6Slit lamp examination reveals BE PCIOL in

situ ,fundus examination reveals BE CNVM

Investigation Repeat OCT BE

Treatment RE intravitreal AntiVEGF injection,

During Follow up Repeat RE intravitreal AntiVEGF injection

One month later BCVA BE 6/12NIG N6Fundus Examination reveals RE geographic

atrophy and LE PED approximation OCT BE reveals no SRF or oedema

3month later BCVA BE 6/9N8Fundus examination BE reveals PED with

oedema

Treatment advised BE intravitreal AntiVEGF injection

One month later BCVA BE 6/9N6Fundus examination reveals RE PED LE PED with minimal pockets of SRF

3month later BCVA BE 6/9N6Fundus examination reveals –active CNVM

with oedema

Treatment advised LE intravitreal AntiVEGF injection

15 days later BCVA RE 6/18 N6 LE 6/12N6Fundus examintion BE reveals PED with

resolving SRF.OCT RE –resolving SRF LE -resolved SRF

One month later BCVA RE 6/18 N6 LE 6/12N6Fundus examintion reveals –FVPEDAdviced intravitreal AntiVEGF injection but

deferred because of lack of compliance of the patient

Two months later BCVA 6/12N6On examination BE scleral thinning noted Fundus examination reveals BE CNVEM with

PED with SRF

3months Later BCVA RE 6/12N8,LE6/12N10Fundus examination reveals RE rnfl

hemorrhage .LE reveals submacular haemorrhage .

Investigation RE perimetryBE OCT

Treatment advised LE intarvitreal Anti VEGF

One month later BCVA BE 6/9N6 Fundus examination reveals RE active CNVM

LE submacular haemorrhage

Investigation advised ICGA reveals IPCV.

Treatment advised BE PDT and intarvitreal Anti VEGF

HistoryWas first described by Dr. L Yannuzzi in

1982.

Prevalence and Incidence:Most commonly diagnosed in patients

between the ages of 50 and 65 yearsIn Asians , Japan have higher prevalence

rates are present of approximately 23% to 54% in patients with presumed age-related macular degeneration (AMD)

EtiologyThe etiology is not clearly understood. It has been proposed that there is a choroidal

vasculature propensity for dilation and aneurysmal formation

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.

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

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

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

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

Differential diagnosisAge-related macular degeneration Central serous chorioretinopathy,Pathological myopia with neovascularization,Choroidal tumors  Metastases.

Treatment Medical therapy

Observation, Photodynamic therapy, Intravitreal injection of anti-VEGF therapy Combination therapy

EVEREST trialMulti-center, double-masked trial Compared three treatment regimens:

verteporfin photodynamic therapy (PDT) plus the anti-VEGF agent ranibizumab (Lucentis), ranibizumab monotherapy, and PDT monotherapy.

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

Medical follow upRegardless of choice of treatment, patients

should be followed on regular intervals to detect and prevent subretinal/subRPE fluid and hemorrhage.

SurgeryThere 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.

Complications Serosanguineous detachments of the

pigmented epithelium and exudative changesSubretinal fibrosisPigment epithelial hyperplasia, Atrophic degeneration

PrognosisDepending on the extent of area involved

prognosis is generally goodSymptomatic patients with PCV can have

complete regression without severe vision loss with PDT and anti-VEGF treatment

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

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

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