Grand Rounds

Grand RoundsGrand Rounds

Shivani V. Reddy, M.D.Shivani V. Reddy, M.D.

7/18/147/18/14

University of LouisvilleUniversity of Louisville

Department of Ophthalmology and Department of Ophthalmology and Visual SciencesVisual Sciences

Patient PresentationPatient Presentation CCCC: : Blurry Vision ODBlurry Vision OD

HPIHPI: : 80 y/o AAF presents for yearly 80 y/o AAF presents for yearly Plaquenil check She states that her vision Plaquenil check She states that her vision had been stable until 1 month ago when had been stable until 1 month ago when she noticed some blurriness and dimming she noticed some blurriness and dimming of vision OD. She denies experiencing of vision OD. She denies experiencing metamorphopsia, flashes, floaters or metamorphopsia, flashes, floaters or scotomas. scotomas.

HistoryHistoryPOHx: POHx: refractive error refractive error

PMHx:PMHx: Rheumatoid Arthritis, Hypertension, Rheumatoid Arthritis, Hypertension, Hypothyroidism Hypothyroidism

FAMHx:FAMHx: uknown uknown

ROS: ROS: joint painjoint pain

MEDS:MEDS: Plaquenil ( 3.04 mg/kg/day (65.7kg weight), Plaquenil ( 3.04 mg/kg/day (65.7kg weight), 200mg daily x 5 years)200mg daily x 5 years)

Toprol, MTV, triamterone, synthroid Toprol, MTV, triamterone, synthroid

ALLERGIESALLERGIES: : NKDA NKDA

ExamExam

VA TP P

20/50-2 (+0.75 +0.50 x 005)

20/20-2 (-0.25 +0.50 x 175)

8

12 no RAPD

EOM: full OU CVF: full OU

4→3

4→3

Exam 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 Fundus Photos

OD photo demonstrates orangish-red faintly circumscribed lesions in the peripapillary region

OD OS

FAF Photos FAF Photos OD 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

1.OD

2.OD

3.OS

FA/ICG FA/ICG Early AV Phase

Peripapillary staining, supero-nasal hypofluorescence of dye

Superonasal hypofluorescence, network of dilated peripapillary vasculature with terminal bulb

00:19:45

FA/ICG FA/ICG AV Phase

Increasing peripapillary hyperfluorescence

circular of hyperfluorescence at end of vascular network

00:29:45

FA/ICG FA/ICG Recirculation Phase

continual demonstration of peripapillary dye leakage, increasing over area of polyp

Hyperfluorescence of polypoid lesion and demonstration of surrounding vascular network

1:21:45

FA/ICG FA/ICG Late Phase

Persistent peripapillary dye pooling representing leakage

2:00:45

Decreased hyperfluorescence of polyp with surrounding area of hypofluorescence

SummarySummary

DDx:Polypoidal Choroidal Vasculopathy Other entities causing subretinal neovascularization

AMD CSCR (central serous

chorioretinopathy) Pathologic myopia Choroidal tumors or Mets Infection

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

Treatment Treatment

First Visit20/50-2

1 month post Avastin #1VA 20/50

1 month post Avastin #2VA 20/40-2

Polypoidal Choroidal Polypoidal Choroidal VasculopathyVasculopathy

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

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



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


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


Treatment

Anti- VEGF treatment alone not helpful

EVEREST TRIALCompared 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 break-through vitreous hemorrhage


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 +/- 0.60Pre PPV: 2.65 +/- 0.57 3 months post-op: 1.62 +/- 0.67Final Visit: 1.43 +/- 0.82 VA > 20/400 in 16.7% of juxtafoveal polyp patients,

87.5% of extraoveal polyp patients

THANK YOU

References 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; 24:483–4907.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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