PCV - University of Louisville Department of Ophthalmology and

Report
Grand Rounds
Shivani V. Reddy, M.D.
7/18/14
University of Louisville
Department of Ophthalmology and Visual Sciences
Patient Presentation

CC: Blurry Vision OD

HPI: 80 y/o AAF presents for yearly Plaquenil check
She states that her vision had been stable until 1 month
ago when she noticed some blurriness and dimming of
vision OD. She denies experiencing metamorphopsia,
flashes, floaters or scotomas.
History
POHx: refractive error
PMHx: Rheumatoid Arthritis, Hypertension, Hypothyroidism
FAMHx: uknown
ROS: joint pain
MEDS: Plaquenil ( 3.04 mg/kg/day (65.7kg weight), 200mg daily x 5 years)
Toprol, MTV, triamterone, synthroid
ALLERGIES: NKDA
Exam
8
20/50-2 (+0.75 +0.50 x 005)
VA
TP
20/20-2 (-0.25 +0.50 x 175)
4→3
P
12
4→3
no RAPD
EOM: full OU
CVF: full OU
Exam
OD
OS
LIDS/LASHES
WNL
WNL
CONJ
WNL
WNL
CORNEA
WNL
WNL
IRIS
WNL
WNL
LENS
2+NS, 1+CC
2+NS, 1+CC
Fundus Photos
OD
OS
OD photo demonstrates orangish-red faintly circumscribed lesions in the
peripapillary region
FAF Photos
OD
OS
1.OD
2.OD
3.OS
1. Nasal pigment epithelial detachment with associated neurosensory detachement
with sub-neurosensory fluid accumulation, some loss of foveal contour
2. Nasal pigment epithelial detachment with overlying heme and fluid
FA/ICG
Early AV Phase
00:19:45
Peripapillary staining, supero-nasal
hypofluorescence of dye
Superonasal hypofluorescence, network of
dilated peripapillary vasculature with
terminal bulb
FA/ICG
AV Phase
00:29:45
Increasing peripapillary hyperfluorescence
circular of hyperfluorescence at end of
vascular network
FA/ICG
Recirculation Phase
1:21:45
continual demonstration of peripapillary
dye leakage, increasing over area of polyp
Hyperfluorescence of polypoid lesion and
demonstration of surrounding vascular
network
FA/ICG
Late Phase
2:00:45
Persistent peripapillary dye pooling
representing leakage
Decreased hyperfluorescence of polyp
with surrounding area of hypofluorescence
Summary
80 y/o AAF presents for routine plaquenil check with 1 month history
of blurriness and dimming of vision OD. BCVA OD is 20/50-2. ICG
angiography demonstrates choroidal polyps with surrounding vascular
network and corresponding leakage on FA. OCT demonstrates PED’s
OD with overlying neurosensory retinal detachment
DDx:
 Polypoidal Choroidal Vasculopathy
 Other entities causing subretinal neovascularization
 AMD
 CSCR (central serous chorioretinopathy)
 Pathologic myopia
 Choroidal tumors or Mets
 Infection
Treatment
First Visit
20/50-2
1 month post Avastin #1
VA 20/50
1 month post Avastin #2
VA 20/40-2
Polypoidal Choroidal Vasculopathy
 Also known as posterior uveal bleeding syndrome
First described by Dr.Yannuzzi in 1982
Characterized by sub-retinal vascular lesions
associated with serous and hemorrhagic detachments
of the RPE
 Named for its network of branching, choroidal
vessels with terminal polyp-like aneurysmal dilations
Polypoidal Choroidal Vasculopathy
First described in middle -aged African
American woman
Most commonly seen in 50-65 age group
Occurs 23-55% in Asian patients presenting with
neovascular AMD
4% to 9.8% prevalence reports in Caucasian
patients with presumed AMD
Polypoidal Choroidal Vasculopathy
 Diagnosis based on ICG angiography high definition OCT and
fundus photography
 FA not helpful as it demonstrates too much dye leakage
 Classification based on imaging characteristics
 Branching vascular network vs. feeder vessel supplying
polyps
 Cluster of polyps vs. single polyp
 Juxtafoveal vs. extrafoveal polyps
Polypoidal Choroidal Vasculopathy
CNV or Not?
PCV is?
 More likely to occur in pigmented races, although
also demonstrated in Caucasians
 lesions more likely to be peripapillary
 ICG in late phases shows ‘wash-out’ of polyp in
contrast to late staining seen in occult CNV
Polypoidal Choroidal Vasculopathy
Treatment
 Anti- VEGF treatment alone not helpful
 EVEREST TRIAL
 Compared PDT monotherapy and PDT + ranibizumab to
ranibizumab alone
 Significantly higher proportion in the first 2 groups showed
regression at 6 months
 Surgical intervention based on complications such as breakthrough vitreous hemorrhage
Polypoidal Choroidal Vasculopathy
Prognosis
 Clinical outcomes in literature vary considerably
 35-68.2% of patient with reported poor outcomes
 Larger polyps, juxtafoveal location of polyps and clustering of
polyps are risk factors for massive subretinal and subsequent
vitreous hemorrhages
 In patients with large detachments, subretinal fibrosis can lead to
eventual vision loss
 PDT can lead to subretinal hemorrhage in upto 31% of eyes, VH
in 6-12.5% post treament, 8.9% of bleeding post anti-VEGF
Retrospective chart review
17 patients with massive subretinal hemorrhages (SRH)
secondary to PCV (16.7+/- 7.1 DD) and associated vitreous
hemorrhage (VH) enrolled
Patients underwent 20G Pars Plana Vitrectomy +/bevacizumab injection for new heme, PED or CME
Main outcome measure: BCVA at end of follow-up period
Mean post-op follow up of 25.2 months
Mean duration from SRH to VH was 3.5 +/- 1.2 weeks
Time from VH to PPV 1.3 +/- 0.7 months
BVCA (LogMar)
Pre VH : 0.95 +/- 0.60
Pre PPV: 2.65 +/- 0.57
3 months post-op: 1.62 +/- 0.67
Final Visit: 1.43 +/- 0.82
 VA > 20/400 in 16.7% of juxtafoveal polyp patients, 87.5% of
extraoveal polyp patients
THANK YOU
References
1.Retina and Vitreous, BSCS
2. Retina, Vitreous and Macula , David R. Guyer MD, Lawrence A. Yannuzzi MD, Stanley
Chang MD and Jerry A. Shields MD
3.Yannuzzi LA, Sorenson J, Spaide RF, Lipson B. Idiopathic polypoidal choroidal
vasculopathy (IPCV). Retina 1990; 10(1): 1–8.
4.Koh et al. EVEREST STUDY: Efficacy and Safety of Verteporfin Photodynamic Therapy
in combination with Ranibizumab or Alone Versus Ranibizumab Monotherapy in Patients
with Symptomatic Macular Polypoidal Choroidal Vasculopathy. Retina 2012; 32:1453-1464.
5. Uyama M, Wada M, Nagai Y, et al. Polypoidal choroidal vasculopathy: Natural history. Am J
Ophthalmol. 2002; 133(5):639-48.
6. Lim TH, Laude A, Tan CS. Polypoidal choroidal vasculopathy: An Angiographic discussion.
Eye (Lond) 2010; 24:483–490
7.Kamaeda T, Tsujikawa A, Otani A, et al. Polypoidal choroidal vasculopathy examined with
en face optical coherence tomography. Clin Experiment Ophthalmol. 2007; 35(7):596-601.
8.Sato T, Kishi S, Watanabe G, et al. Tomographic features of branching vascular networks in
polypoidal choroidal vasculopathy. Retina. 2007 27; 589-594.
9.Park DH, Shin JP, Kim IT. Association of plasma malondialdehyde with ARMS2 genetic
variants and phenotypes in polypoidal choroidal vasculopathy and age-related macular
degeneration. Retina 2013; 0:1–10.

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