Choroidal neovascular membrane in macular heme

Choroidal neovascular membrane

1. Case presentation Carlos J. Vives Alvarado MD August 2, 2018 PGY-4 Department of Ophthalmology, School of Medicine, University of Puerto Rico

2. History of present illness 6/19/208 CC:” No veo por mi ojo izquiero , y es el unico que me queda ” 75 y/o male with pmhx of DM, COPD, CHF, Hyperlipidemia, HTN and NKDA, hx of right eye trauma many years ago, comes due to 4 day history of painless, sudden left eye loss of vision. Denies recent trauma, illness, headache, jaw claudication, temporal pain, loss of weight, photopsias , curtain drop, floaters. Refers seeing trough a red veil.

3. Past medical history ROS Left eye loss of vision Social Ex-smoker, 25 pack years Ocular hx Right eye old trauma, s/p RD repair (more than 50 years ago), hx of injections to right eye out of va , 5-6 years ago Surgeries Right eye RD repair, aortic valve replacement Meds Albuterol Carvedilol 12.5mg Gabapentin 300mg Losaratan 25mg Simvastatin 40mg Metformin 1000mg AREDS 2 BID

4. Physical exam OD OS CF 1FT VA CF 2FT X (contact lens) IOP 13 Full and ortho EOM Full and ortho CL, diffuse k scar, Aphakia , correctopic pupil, +APD SLE RRTL, Ns+2 C/d 0.4, macular atrophy, fine drusen, fibrotic scar, 360 laser marks, buckle effect, retina grossly attached DFE C/d 0.4, macula preretinal , intraretinal and subretinal hemorrhage, inferotemporal retinoschisis, retina attached, no tear

5. Fluorescein angiography

6. OCT

7. Differential diagnosis Exudative AMD

8. Plan Most probable diagnosis: Hemorrhagic PED, suspected occult CNVM Intravitreal anti-VEGF x3 Cdw and seen with retinologist

9. Choroidal neovascularization Pathophysiology: Alterations in the normal transport of metabolites, ions and water through Bruch's membrane alter the nutrition and stability of retinal pigment epithelium (RPE) from choriocapillaris and the transport of waste out from the neurosensory retina. VEGF is then released by RPE as a stress signal and hypoxia that initiates a cascade of angiogenic responses at the choroidal endothelium level. Bruch´s membrane damage is required to allow neovascular passage through it from the choroidal vasculature to the retina. Any cause of damage or loss of integrity of RPE/Bruch’s membrane complex can induce CNV AMD, Myopia, Trauma, Inflammatory, Coagulopathies, Tumor, Iatrogenic

10. Exudative macular degeneration Age-related macular degeneration (AMD) is the leading cause of new blindness in the United States. Divided in two main types:

11. Exudative macular degeneration Characterized by choroidal neovascularization (CNV), or growth of abnormal blood vessels under the retina and macula These new blood vessels may leak fluid, break, and bleed, causing elevation of the retina and causing vision loss. Symptoms include acute/subacute painless blurring of vision and/or scotoma Signs include: grey-green/pink-yellowish lesion, associated medium-large drusen, localized subretinal fluid, intra and subretinal lipid deposition, intr /subretinal/pre-retinal and vitreous hemorrhage, Pigment epithelial detachment, disciform scar.

12. Classification Histologically, neovascular membranes are classified into: Type 1, sub-RPE neovascular membrane Type 2, sub-retinal neovascular membrane Type 3, RAP (Retinal Angiomatous Proliferation), which corresponds to neovascularization developed within the neurosensory retina. FA classification following Macular Photocoagulation Study Classic CNV (20%), well demarcated areas of intense hyperfluoresnce appearing early and showing progressive leakage Occult CNV (80%), limits can not be defined in the FA Fibrovascular PED (FV-PED) Late leakage of undetermined source (LLUS) Gass JD . Biomicroscopic and histopathologic considerations regarding the feasibility of surgical excision of subfoveal neovascular membranes. Am J Ophthalmol . 1994;118:285–298. Fine, SL. Macular Photocoagulation Study, Arch Ophthalmol . 1980;98(5):832.

13. Idocyanine green angiography (ICG) Demonstrate CNV as a focal hyperfluorescent hot spot or plaque and benefit FA findings Increase sensitivity in the detection of CNV (low density hemorrhage, fluid or pigment preclude adequate FA visualization) Distinction of CNV due to AMD from other conditions such as PCV and RAP Helps delineate occult CNV and serve as treatment guidance

14. Retinal pigment epithelial detachment PED is a disruption from the inner collagenous layer of Bruch’s membrane Thought to be caused by reduction in hydraulic conductivity in a thickened and dysfunctional Bruch’s membrane, impending movement from the RPE towards the choroid Different types of PED: Serous Fibrovascular Drusenoid Hemorrhagic

15. Polypoidal Choroidal Vasculopathy PCV is considered a variant of exudative AMD Branching vascular network of inner choroidal vessels with multiple terminal aneurysmal protuberances that leads to bleeding and exudates More common in African ad East Asian, 5:1 women to men ratio and in younger population, usually bilateral, but asymmetrical Recurrent serosanguineous retinal and RPE detachments ICG is diagnostic, with hyperfluorescent nodules and a network of large choroidal vessels in early phase, with poly-like swellings that begin to leak Yannuzzi , L.A., Sorenson, J., Spaide , R.F., and Lipson, B. Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina. 1990; 10: 1–8

16. Treatment Laser photocoagulation Occludes leaking blood vessels in CNV, only recommended for extrafoveal lesions and juxtafoveal CNV Photodynamic Therapy Injects Verteporfin, a light activated compound, intravenously and then is activated by diode laser to induce thrombosis, used in subfoveal CNV Anti-VEFG Inhibitors of VEGF block the interaction by the receptors on endothelial cells and thus stop and/or reverse abnormal blood vessel growth

17. Back to our patient Injected with Avastin 1/3 OS on 6/26/18, no complications F/u 7/5/18 VA 20/200 (from Cf 2ft), no RD, no tear, Scheduled for Avastin 2/3 on 8/7/18 and then 3/3 9/11/18 F/u after #3 injection with new OCT and FA

18. Thank you for your attention

19. References Grossniklaus HE, Green WR: Choroidal neovascularization. Am J Ophthalmol 2004;137:496-503. Gehrs KM, Anderson DH, Johnson LV, Hageman GS: Age-related macular degeneration - emerging pathogenetic and therapeutic concepts. Ann Med 2006;38:450-471. Cohen SY, Laroche A, Leguen Y, Soubrane G, Coscas GJ: Etiology of choroidal neovascularization in young patients. Ophthalmology 1996;103:1241-1244. Wormald R, Evans J, Smeeth L, Henshaw K: Photodynamic therapy for neovascular age-related macular degeneration. Cochrane Database Syst Rev 2007:CD002030. Krzystolik MG, Afshari MA, Adamis AP, Gaudreault J, Gragoudas ES, Michaud NA, Li W, Connolly E, O'Neill CA, Miller JW: Prevention of experimental choroidal neovascularization with intravitreal anti-vascular endothelial growth factor antibody fragment. Arch Ophthalmol 2002;120:338-346. Rosenfeld PJ, Brown DM, Heier JS, Boyer DS, Kaiser PK, Chung CY, Kim RY: Ranibizumab for neovascular age-related macular degeneration. N Engl J Med 2006;355:1419-1431. Cohen SY: Anti-VEGF drugs as the 2009 first-line therapy for choroidal neovascularization in pathologic myopia. Retina 2009;29:1062-1066. Vedula SS, Krzystolik MG: Antiangiogenic therapy with anti-vascular endothelial growth factor modalities for neovascular age-related macular degeneration. Cochrane Database Syst Rev 2008:CD005139. Lee JH, Canny MD, De Erkenez A, Krilleke D, Ng YS, Shima DT, Pardi A, Jucker F: A therapeutic aptamer inhibits angiogenesis by specifically targeting the heparin binding domain of VEGF 165 . Proc Natl Acad Sci USA 2005;102:18902-18907. Gragoudas ES, Adamis AP, Cunningham ET Jr, Feinsod M, Guyer DR: Pegaptanib for neovascular age-related macular degeneration. N Engl J Med 2004;351:2805-2816. BCSC AAO 2016, Retina and Vitreous, Section 12 EyeWiki Kanski Clinical Ophthalmology