Retina Imaging Conference Brett Mueller, D.O., Ph.D. 2/11/2016 University of Louisville Department of Ophthalmology and Visual Sciences

Patient Presentation CC: Decreased Vision Left Eye HPI: 59 yo WM presents w/ gradual, painless blurry vision OS that has been getting worse for the past several months. Pt denies any history of trauma, HAs, or jaw claudication.

History POHx: none PMHx: Hypertension and controlled on medical management FAMHx: none ROS: none MEDS: Metoprolol, aspirin, and a statin ALLERGIES: none

Exam VA TP P 18 20 3→2 EOM: full OU 20/20 (+1.25 sph) 20/CF 1 ft (+1.75 sph) 18 20 3→2 NO RAPD EOM: full OU

Exam OD OS LIDS/LASHES WNL WNL CONJ WNL WNL CORNEA WNL WNL IRIS WNL WNL LENS 1+NS 1+NS

Fundus Photos OD OS OS photo demonstrates a 1-2 DD choroidal nevus with a CNVM juxtaposition to it extending into the fovea Greenish fibrotic lesion,

IR Photos OD OS OS photo demonstrates a 1-2 DD hyper-reflective lesion in the choroid Hypointense area, non specific chronic changes under the area of retinal swelling -speculed areas

FAF Photos OD OS Hypoautofluoercent lesion 2 DD in the macula. And hyperautofluoercent signal surround.

OCT OD Macula OS Macula Distorted contour of the fovea. Hyper-reflective materal subfoveal with CME above w/ subretinal fluid consistent with CNVM OS photo demonstrates a subretinal fluid, w/ a CNVM, retinal thickening, and CME

OCT OS Below the Fovea OS Through the Nevus IR photo: hyperintense on IR, and OCT shows hyper-reflective signal. Hypo-reflective on IR OCT. It is optically clear and why there is enhancement of the OCT signal

FA Late Phase Early Venous Phase 00:12:00 02:00:00 Middle see a NVM and a blocked flurescetn area with stiplling with hyperfluorescent stippling dots. After 2 mins you see a leak in the membrane area that is consistent area of window defect. Blocked flurescent (blood or fluid) and window defect (stippling) --hyeperflurescent lesion..late you see leakage in the angio.classic CNVM 00:12:00 02:00:00

FA/ICG Blockage, leakage, staining, and window defect (transmission defects)….Hypoflurescent..blocked fluorescent ICG, and blocked bc of fluid. Nevus also blocking the fluresecent. Cant see vasc connection between the 2 lesions Demonstrates a well defined CNVM in the same location as the retinal leakage

Summary DDx: Choroidal neovascular membrane Idiopathic ? Nevus ? 59 y/o WM with decreased vision OS. Examination and FA reveal a choroidal neovascular membrane w/ a concomitant choroidal nevus DDx: Choroidal neovascular membrane Idiopathic ? Nevus ? PLAN: Avastin injection with 1 month f/u

Exam s/p 2 Avastin Injections VA TP P 20/20 (+1.25 sph) 20/300 (+1.75 sph) 16 3→2 no RAPD EOM: full OU

OCT OS Resolution of subretinal fluid and CME, but integrity of the outer retinal layer is diminished

OCT OS Below the Fovea OS Through the Nevus Shadow and pigmentary changes over the prolonged edema Resolution of subretinal fluid and CME, but integrity of the outer retinal layer is diminished, and below the fovea there is a PED

Cause of the CNVM Is it due to the choroidal nevus? Or is it an idiopathic CNVM?

Complications of Choroidal Nevi Naumann  GOHHellner  KNaumann  LR Pigmented nevi of the choroid: clinical study of secondary changes in the overlying tissues. Trans Am Acad Ophthalmol Otolaryngol.1971;75110- 123 Complications of Choroidal Nevi Numerous studies postulate that the prevalence of choroidal nevi in the general population is anywhere from 0.2% - 30% Most use 6.5% as the incidence of nevi in the general population A total of 3654 subjects aged 49 to 97 years participating in the Blue Mountains Eye Study had a detailed eye examination, including photography of 6 standard retinal fields. Nevi were graded from photographs. Results  Nevi were present in 6.5% of the population (n = 232),  Sumich P, Mitchell P, Wang JJ. Choroidal nevi in a white population: the Blue Mountains Eye Study. Arch Ophthalmol. 1998 May;116(5):645-50. PubMed PMID: 9596501

Complications of Choroidal Nevi Naumann  GOHHellner  KNaumann  LR Pigmented nevi of the choroid: clinical study of secondary changes in the overlying tissues. Trans Am Acad Ophthalmol Otolaryngol.1971;75110- 123 Complications of Choroidal Nevi Decreased visual acuity attributable to Serous foveal detachment Photoreceptor degeneration CNVM Blue Mountains Eye Study: None had complications associated with vision loss A total of 3654 subjects aged 49 to 97 years participating in the Blue Mountains Eye Study had a detailed eye examination, including photography of 6 standard retinal fields. Nevi were graded from photographs. Results  Nevi were present in 6.5% of the population (n = 232),  Gonder JR, Augsburger JJ, McCarthy EF, Shields JA. Visual loss associated with choroidal nevi. Ophthalmology. 1982 Aug;89(8):961-5. PubMed PMID: 6182515. Sumich P, Mitchell P, Wang JJ. Choroidal nevi in a white population: the Blue Mountains Eye Study. Arch Ophthalmol. 1998 May;116(5):645-50. PubMed PMID: 9596501

Complications of Choroidal Nevi 22 of 206 patients (11%) with choroidal lesions had decreased VA attributable to : Serous foveal detachment (50%) Photoreceptor degeneration (42%) CNVM (8%) 322 eyes with giant choroidal nevi, 2.2% had decreased vision 4100 choroidal nevus ID 322 (8%) of giant choroidal nevi > = 10mm Gonder JR, Augsburger JJ, McCarthy EF, Shields JA. Visual loss associated with choroidal nevi. Ophthalmology. 1982 Aug;89(8):961-5. PubMed PMID: 6182515. Li HK, Shields CL, Mashayekhi A, Randolph JD, Bailey T, Burnbaum J, Shields JA. Giant choroidal nevus clinical features and natural course in 322 cases. Ophthalmology. 2010 Feb;117(2):324-33. doi: 10.1016/j.ophtha.2009.07.006. Epub 2009 Dec 6. PubMed PMID: 19969359.

Complications of Choroidal Nevi Choroidal Nevus Risk Factors for Vision loss Nevus proximity to foveola (0 mm vs > 0 mm): RR = 36.46 Subretinal fluid (> 3 to 6 mm vs none): RR = 498.33 RPE detachment (present vs absent):RR = 33.01 CNVM (present vs absent): RR = 98.72 4100 choroidal nevus ID 322 (8%) of giant choroidal nevi > = 10mm Li HK, Shields CL, Mashayekhi A, Randolph JD, Bailey T, Burnbaum J, Shields JA. Giant choroidal nevus clinical features and natural course in 322 cases. Ophthalmology. 2010 Feb;117(2):324-33. doi: 10.1016/j.ophtha.2009.07.006. Epub 2009 Dec 6. PubMed PMID: 19969359. Shields CL, Furuta M, Mashayekhi A, Berman EL, Zahler JD, Hoberman DM, Dinh DH, Shields JA. Visual acuity in 3422 consecutive eyes with choroidal nevus. Arch Ophthalmol. 2007 Nov;125(11):1501-7. PubMed PMID: 17998511.

Why the Difference Between the Studies In the Blue Mountains Eye Study, the average diameter for the nevus was between 1-2mm, with 1.5% of them being over 4mm Wills Eye 3,000+ patients with choroidal nevi have an average diameter of 5.1-5.7mm

Conclusion Is the CNVM secondary to the choroidal nevus? Or is it just an idiopathic CNVM, and the choroidal nevus is just an innocent bystander Choroidal nevi can cause decreased VA Proximity to the fovea Subretinal fluid RPE detachment CNVM

THANK YOU

References 5. Retina and Vitreous, BCSC Ophthalmic Pathology and Intraocular Tumors, BCSC American Academy of Ophthalmology eye wiki website on idiopathic CNVMs Krypton laser photocoaguluation for idiopathic neovascular lesions: results of a randomized clinical trial. Photocoagulation Study Group. Arch Ophthalmol. 1990; 108: 832-837 Persistent and recurrent NV after krypton laser photocoagulation for NV lesion of ocular histoplasmosis. Macular Photocoagulation Study Group. Arch Ophthalmol. 1989; 107:344-352 Kanski’s Clinical Ophthalmology A systemic Approach, Eighth Edition. Brad Bowling Gonder JR, Augsburger JJ, McCarthy EF, Shields JA. Visual loss associated with choroidal nevi. Ophthalmology. 1982 Aug;89(8):961-5. PubMed PMID: 6182515. D.G. Callanan, M.L. Lewis, S.F. Byrne, J.D.M. Gass Choroidal neovascularization associated with choroidal nevi Arch Ophthalmol, 111 (1993), pp. 789–794 Gass  JD Problems in the differential diagnosis of choroidal nevi and malignant melanoma: XXXIII Edward Jackson Memorial lecture. Trans Am Acad Ophthalmol Otolaryngol.1977;8319- 48 Ganley  JPComstock  GW Benign nevi and malignant melanomas of the choroid. Am J Ophthalmol. 1973;7619- 25 Naumann  G Pigmented nevi of the choroid and ciliary bodies: a clinical and histopathological study. Adv Ophthalmol. 1970;23187- 272 Hale  PNAllen  RAStraatsma  BR Benign melanomas (nevi) of the choroid and ciliary body. Arch Ophthalmol. 1965;74532- 538 Wilder  HC Intraocular tumors in soldiers: World War II. Mil Surg. 1946;99459- 490 Lang  GKDaumann  FJ Peripheral fundus changes in subjects with healthy eyes (pilots). Klin Monatsbl Augenheilkd. 1982;181493- 495 Link to Article Albers  EC Benign melanomas of the choroid and their malignant transformation. Am J Ophthalmol. 1940;23779- 783 Albert  DMRobinson  NLFulton  AB  et al.  Epidemiological investigation of increased incidence of choroidal melanoma in a single population of chemical workers. Int Ophthalmol Clin.1980;2071- 92 Albert  DMSearl  SSForget  BLavin  PTKirkwood  JNordlund  JJ Uveal findings in patients with cutaneous melanoma. Am J Ophthalmol. 1983;95474- 479 Rodriguez Sains  RS Ocular findings in patients with dysplastic nevus syndrome. Ophthalmology. 1986;93661- 665 Smith  REGanley  JP Ophthalmic survey of a community, I: abnormalities of the ocular fundus. Am J Ophthalmol. 1972;741126- 1130 Sumich P, Mitchell P, Wang JJ. Choroidal nevi in a white population: the Blue Mountains Eye Study. Arch Ophthalmol. 1998 May;116(5):645-50. PubMed PMID: 9596501 5.

Idiopathic CNVM Is a disease of exclusion that can be made after all other causes associated with CNV have been ruled out: Angioid streaks High myopia AMD Chorioretinal inflammatory conditions Tumors Trauma

Idiopathic CNVM Some speculate that it could represent POHS w/o the punched-out chorioretinal lesion Other speculate that it could be a variant of AMD w/out drusen or RPE abnormalities Krypton laser photocoaguluation for idiopathic neovascular lesions: results of a randomized clinical trial. Photocoagulation Study Group. Arch Ophthalmol. 1990; 108: 832-837 Persistent and recurrent NV after krypton laser photocoagulation for NV lesion of ocular histoplasmosis. Macular Photocoagulation Study Group. Arch Ophthalmol. 1989; 107:344-352