Retina Conference. Janelle Fassbender, MD, PhD University of Louisville Department of Ophthalmology and Visual Sciences 09/04/2014

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1 Retina Conference Janelle Fassbender, MD, PhD University of Louisville Department of Ophthalmology and Visual Sciences 09/04/2014

2 Subjective CC/HPI: 64 year old Caucasian female referred by outside ophthalmologist for large macular scar and decreased vision OS.

3 History POH: Presumed ocular histoplasmosis for 35 years Strabismus surgery (1966) Cataract surgery OU (2001) PMH: Hypertension Meds: Triamterene Trazodone Multivitamins FOH: Cataracts (Parents)

4 Objective OD OS Va(sc): 20/20 20/200 (eccentric) Pupils: No RAPD IOP: EOM: Full Full CVF: Full Full

5 Anterior and Posterior Segments OD OS Anterior segment: PCIOL OU ON: c/d 0.2 pink/sharp c/d 0.2 pink/sharp large peripapillary atrophy OU Macula: Histo spot along inferior arcade; epiretinal membrane Central scar with heme Vessels: WNL WNL Periphery: Histo spots Histo spots

6 OCT OD: Lamellar hole secondary to epiretinal membrane

7 OCT OS: Subretinal fibrosis, disruption of outer retinal layers

8 Infrared OD Peripapillary hyper- and hypo-reflectance and punctate hyper-reflectance surrounding fovea representing epiretinal membrane.

9 Infrared OS Peripapillary hyper-reflectance with kidney-shaped area of hyper-reflectance and interspersed areas of hypo-reflectance.

10 Autofluorescence OD Peripapillary hypo-autofluorescence and hypo-autofluorescence corresponding to histo spots along superior and inferior arcade. Also, slight hyperautofluorescence in the fovea.

11 Autofluorescence OS Large, kidney-shaped macular hypoautofluorescence in addition to peripapillary hypoautofluorescence.

12 Fluorescein angiogram OD Arterial-venous (A-V) phase with hyperfluorescence along margin of peripapillary hypofluorescence (1m 23s) Central hypo- with ring of hyperfluorescence in histo spots along arcades and peripapillary area, consistent with staining.

13 Fluorescein angiogram OS Large choroidal vessels due to capillary filling defect. Increasing hyperfluorescence in pit of kidney bean shaped hypofluorescent scar indicates leakage. Staining along rim of macular and peripapillary scar. 37s 1m 59s 54s 4m 18s

14 Impression Presumed ocular histoplasmosis (POHS) with new sub-retinal neovascular membrane

15 Differential Diagnosis Exudative age-related macular degeneration Choroidal neovascular membrane secondary to macular scar

16 Assessment 64 yr/o female with 1. Macular scar and active subretinal neovascular membrane OS with long-history of POHS OU, and 2. Asymptomatic lamellar hole OD secondary to epiretinal membrane.

17 Diagnosis POHS with active subretinal neovascular membrane OS.

18 Intravitreal Avastin OS Plan

19 Follow up 4 week follow up: Visual acuity stable: 20/200 Persistent but decreased subretinal fluid on OCT Second intravitreal Avastin administered

20 4-Week Follow up OCT Persistent sub-retinal fluid

21 Presumed ocular histoplasmosis Histoplasmosis capsulatum found in soil and carried in bird or bat droppings Endemic to Ohio and Mississippi river valleys 60% of residents are skin test positive for Histo antigen Only 1.5% manifest chorioretinal findings 3.8% develop choroidal neovascular membranes Bilateral findings in 60% of patients

22 Diagnosis Clinical signs: 1. Peripapillary atrophy/pigmentary change 2. Punched out chorioretinal scars 3. Absence of vitritis 4. Choroidal neovascular membranes Fluorescein angiography

23 Treatment Macular Photocoagulation Study Group Demonstrated effectiveness of focal laser for extrafoveal CNVM secondary to POHS (1991). Eyes with POHS had 3.6 times the risk of severe vision loss (loss of >6 lines) in untreated group at 5 years. Untreated eyes had lost a mean of 4.4 lines of visual acuity after 5 years, compared with only 0.9 lines lost by laser-treated eyes. 26% of treated eyes had recurrent neovascularization by 5 years. Photodynamic therapy (PDT) with verteporfin Verteporfin in Ocular Histoplasmosis study (VOH) Eliminated active CNVM in 85% of patients after mean treatment of 4 sessions (2004). Surgical excision Showed benefit in eyes with less than 20/100 vision (Submacular Surgery Trial) Retrospective showed similar data (Almony, 2008)

24 Treatment continued Anti-VEGF therapy Retrospective review showed PDT plus bevacizumab was not superior to bevacizumab alone Addition of PDT does not decrease the number of injections required (Cionni, 2012). Prospective study ranibizumab alone or quarterly PDT (Ramaiya, 2013) PDT group (n = 2) required ranibizumab rescue therapy (2-5 injections) Patients in ranibizumab group (n = 5) had on average 7.7 injections (range 1-11)

25 References Ramaiya, et al Ophthalmic Surgery, Lasers & Imaging Retina, 44(1): Rosenfeld, et al Verteporfin in Ocular Histoplasmosis (VOH) study group. Ophthalmology, 111(9): Macular Photocoagulation Study Gorup Argon laser photocoagulation for neovascular maculopathy: 5-year results from randomized clinical trials. Arch Ophthalmol, 109(8): Cionni, et al Ophthalmology, 119(2): Verteporfin in Photodynamic Therapy (VIP) Study group Ophthalmology, 110: Almony, et al Ophthalmology, 115:

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