Learn Connect Succeed. JCAHPO Regional Meetings 2017

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1 Learn Connect Succeed JCAHPO Regional Meetings 2017

2 How Retinal Imaging Guides Treatment Odette Margit Houghton MD Question 1 Which OCT has the highest resolution? A: Swept source OCT B: Spectral domain OCT C: Swept source OCT 1 st JCAHPO Meeting 29 th July, MFMER slide MFMER slide-2 Question 2 True or False? OCT Angiography can identify leakage from blood vessels? Question 3 What is EDI-OCT most useful for? A: Identifying small macular holes B: Identifying vitreomacular traction C: Evaluating choroidal thickness D: Evaluating retinal detachments MFMER slide MFMER slide-4 Question 4 Which imaging modality is best at providing information regarding the health of the retinal pigment epithelium (RPE)? A: Swept source OCT B: Widefield fundus photography C: Fundus autofluorescence (FAF) D: OCT angiography Why is Retinal Imaging Important? Early diagnosis Precise diagnosis Monitoring of disease progression Appropriate treatment Monitoring of treatment response Improved understanding of disease mechanisms and manifestations 2017 MFMER slide MFMER slide-6 1

3 Retinal Imaging Modalities Optical Coherence Tomography (OCT) Fundus autofluorescence (FAF) Fundus photography & angiography Widefield Standard OCT-Angiography (OCT-A) OCT OCTMD.org 2017 MFMER slide MFMER slide-8 OCT OCT Clinical Uses Measuring retinal thickness RVO DME AMD Detecting: VMT RDs Advantages Noninvasive Reproducible Easy to interpret Disadvantages Does not identify blood well Unable to evaluate retinal perfusion Time domain (TD-OCT): m resolution Slower speed A-scan / sec Spectral domain (SD-OCT): 1-3 m resolution 3-D imaging Higher speed - 50K A-scans / sec Fewer artifacts 2017 MFMER slide MFMER slide-10 OCT Swept Source (SS-OCT) Highest imaging speeds (100K vs 50K Ascans / sec) 5 m resolution Simultaneous high-quality visualization of vitreous, retina & choroid Deeper imaging choroidal structure Wide-field scans (12 mm vs 6-9 mm) Overcomes lens opacities OCT Angiography (OCT-A) Noninvasive Visualize blood vessels to capillary level oct.eu 2017 MFMER slide MFMER slide-12 2

4 OCT Angiography (OCT-A) OCT Angiography (OCT-A) Retinal Physician Retinal Physician 2017 MFMER slide MFMER slide-14 OCT Macular Thickness and Volume Macular Thickness and Volume Average CSF varies based on OCT: Spectralis (SD-OCT): 270 m Cirrus (SD-OCT): 260 m Stratus (TD-OCT): 202 m Average macula thickness and total macular volume: males>females ETDRS plot of macular topography 2017 MFMER slide MFMER slide-16 Macular Thickness and Volume Macular Pucker Pre Op Post Op 2017 MFMER slide MFMER slide-18 3

5 Vitreomacular Traction Vitreomacular Traction 20/50 20/ MFMER slide MFMER slide-20 Diabetic Macular Edema Diabetic Macular Edema 2017 MFMER slide MFMER slide-22 Branch Retinal Vein Occlusion Recent Central Retinal Vein Occlusion 2017 MFMER slide MFMER slide-24 4

6 Normal Retinal OCT Anatomy: Outer Retina Post Macular Hole Repair A: BCVA: 20/100 B: Restoration of ELM C: BCVA 20/70 EZ recovering MFMER slide MFMER slide-26 Vitreous Subretinal Perfluoron Liquid Asteroid Hyalosis PVD Post surgery VMT 2017 MFMER slide MFMER slide horizontally oriented raster B- scans (8.2mm length) 125 mm interscan spacing. 24 radial B-scans (5.5 mm length) 7.5 degrees interscan spacing Conclusion Radial Scan patterns (high density Bscans) superior to standard raster volume scans Raster volume scan associated with missed holes Radial scans can also more accurately reflect hole dimensions American Journal of Ophthalmology MFMER slide-29 American Journal of Ophthalmology MFMER slide-30 5

7 Normal Choroidal OCT Anatomy Enhanced-depth Imaging EDI OCT Average subfoveal choroidal thickness 287 μm - thins with age Diurnal variation: Thickest in AM / thinnest in PM Normal Choroidal OCT Anatomy Conditions with thick choroid: CSR Polypoidal choroidal vasculopathy (IPCV) Conditions with thin choroid: Myopia Age-related choroidal atrophy AMD 2017 MFMER slide MFMER slide-32 Choroidal OCT Anatomy Normal Choroidal OCT Anatomy Normal Age-related choroidal atrophy Central Serous Retinopathy 2017 MFMER slide-33 Invest. Ophthalmol. Vis. Sci ;54(7): MFMER slide-34 Fundus Autofluorescence (FAF) Commonly used FAF imaging systems: Fundus camera cslo Widefield cslo FAF What causes fundus autofluorescence? Lipofuscin: Liposomes that are storing photoreceptor waste products Lipofuscin fluoresces at nm 2017 MFMER slide MFMER slide-36 6

8 FAF What is hyper- and hypo- autofluorescence? Hyper: Excess lipofuscin accumulation Hypo: Lack of lipofuscin (dead or absent RPE cells) Yung International Journal of Retina and Vitreous MFMER slide MFMER slide-38 FAF: AMD Geographic AMD Yung International Journal of Retina and Vitreous MFMER slide MFMER slide-40 FAF: Stargardt Disease FAF: Acute CSR PED and serous RD Macular detachment Yung International Journal of Retina and Vitreous MFMER slide-41 Yung International Journal of Retina and Vitreous MFMER slide-42 7

9 FAF: Chronic CSR Gravitational tract from chronic inactive CSR Retinitis Pigmentosa Yung International Journal of Retina and Vitreous MFMER slide MFMER slide-44 Plaquenil Toxicity Plaquenil Toxicity 2017 MFMER slide MFMER slide yo M: Referral for Treatment of Wet AMD OD 20/60 20/50 82 yo M: Referral for Treatment of Wet AMD OD OD OS 2017 MFMER slide MFMER slide-48 8

10 82 yo M: Referral for Treatment of Wet AMD OD 82 yo M: Referral for Treatment of Wet AMD OD 20/60 Sp 3x bevacizumab OD Sp 1x ranibizumab OD 2017 MFMER slide MFMER slide yo M: Referral for Treatment of Wet AMD OD 20/60 82 yo M: Referral for Treatment of Wet AMD OD 20/60 20/60 Sp 3x bevacizumab OD Sp 1x ranibizumab OD 20/60 Observation OD 1 year later 2017 MFMER slide MFMER slide yo M: Referral for Treatment of Wet AMD OD 82 yo M: Referral for Treatment of Wet AMD OD 2017 MFMER slide MFMER slide-54 9

11 Pattern Dystrophy Photography & Angiography: What does wide field have to offer? Images courtesy of Sunil K. Srivastava, MD, Cole Eye Institute, Cleveland Clinic 2017 MFMER slide MFMER slide-56 Photography & Angiography: What does wide field have to offer? 200 o view of retina Single noncontact digital capture Case Presentation: Uveitis 16 year old girl presents with decreased vision OD >OS for 12 months Diagnosed with uveitis Treated with low dose methotrexate CME is gone, OCT is normal Few cells in back of eye Why can t she see? Images courtesy of Sunil K. Srivastava, MD, Cole Eye Institute, Cleveland Clinic 2017 MFMER slide MFMER slide-58 Case Presentation: Uveitis Case Presentation: Uveitis 2017 MFMER slide MFMER slide-60 10

12 Case Presentation: Uveitis Wide field angiography Identification of leakage sign of activity Case Presentation: Vision loss unknown etiology 29 yo lady SLE Presents with complaints of recurrent vision changes OS Loses vision then it returns VA: 20/20 OU 2017 MFMER slide MFMER slide-62 Case Presentation: Vision loss unknown etiology Case Presentation: Vision loss unknown etiology 2017 MFMER slide MFMER slide-64 Case Presentation: Vision loss unknown etiology Case Presentation: Vision loss unknown etiology 2017 MFMER slide MFMER slide-66 11

13 Case Presentation: Vision loss unknown etiology Case Presentation: Vision loss unknown etiology 2017 MFMER slide MFMER slide-68 Case Presentation: Vision loss unknown etiology FA quiet But since symptomatic immunosuppression was increased Case Presentation: Vision loss unknown etiology Over next few months few episodes of vision loss OS Quiet exams Negative FA Then MFMER slide MFMER slide-70 Case Presentation: Vision loss unknown etiology Case Presentation: Vision loss unknown etiology 2017 MFMER slide MFMER slide-72 12

14 Case Presentation: Vision loss unknown etiology Case Presentation: Vision loss unknown etiology 2017 MFMER slide MFMER slide-74 Asteroid Hyalosis Asteroid Hyalosis 2017 MFMER slide MFMER slide-76 Retinal Detachment or Schisis? Retinal Detachment! 2017 MFMER slide MFMER slide-78 13

15 Retinal Detachment or Schisis? Retinal Detachment! 2017 MFMER slide MFMER slide-80 Retinal Detachment or Schisis? Schisis! 2017 MFMER slide MFMER slide-82 Sickle Cell Familial Exudative Vitreoretinopathy (FEVR) 2017 MFMER slide MFMER slide-84 14

16 Familial Exudative Vitreoretinopathy (FEVR) Retinal Hemorrhage 2017 MFMER slide MFMER slide-86 Retinal Hemorrhage. Peripheral Melanotic Lesion 2017 MFMER slide MFMER slide-88 Multimodality Imaging Cases 70 yo M: Referred with Wet AMD OU No Response to Multiple Aflibercept Injections VA 20/60 VA 20/ MFMER slide MFMER slide-90 15

17 70 yo M: Referred with Wet AMD OU No Response to Multiple Aflibercept Injections 70 yo M: Referred with Wet AMD OU No Response to Multiple Aflibercept Injections OD 20/60 OS 20/200 FAF 2017 MFMER slide MFMER slide-92 Diagnosis = Chronic Central Serous Chorioretinopathy Diagnosis = Chronic Central Serous Chorioretinopathy 2017 MFMER slide MFMER slide yo M: Chronic CSR OU No Response to Multiple Aflibercept Injections 70 yo M: Chronic CSR OU No Response to Multiple Aflibercept Injections FA FA ICG ICG 2017 MFMER slide MFMER slide-96 16

18 Treatment: ½ fluence PDT Reduction in prednisone dose 70 yo M: Chronic CSR OU sp Reduced Fluence PDT & Reduction in Prednisone Dose: 2 Months Later baseline FA ICG 2 months post PDT FA ICG VA 20/ MFMER slide-97 VA 20/ MFMER slide yo M: Chronic CSR OU sp Reduced Fluence PDT & Reduction in Prednisone Dose: 2 Months Later 70 yo M: Chronic CSR OU sp Reduced Fluence PDT & Reduction in Prednisone Dose: 2 Months Later baseline FA ICG Additional ½ fluence PDT to hot spot OS 2 months post PDT FA ICG VA 20/200 VA 20/ MFMER slide MFMER slide-100 VA 20/30 70 yo M: Chronic CSR OU Sp PDT OD x 1 & PDT OS x 2: 2 Months Later VA 20/30 70 yo M: Chronic CSR OU Sp PDT OD x 1 & PDT OS x 2: 2 Months Later OD OD VA 20/200 VA 20/200 OS OS 2017 MFMER slide MFMER slide

19 70 yo M: Chronic CSR OU EDI OCT 70 yo M: Chronic CSR OU EDI OCT CSR AMD CSR 2017 MFMER slide MFMER slide-104 Melanoma Melanoma 3 months later 2017 MFMER slide MFMER slide-106 Melanoma & Nevi SRF SRF 2017 MFMER slide MFMER slide

20 75 yo M Referred for Treatment of Wet AMD With Cystoid Macular Edema 75 yo M Referred for Treatment of Wet AMD VA 20/60 VA 20/30 OD OS 1+ NS OU 2017 MFMER slide MFMER slide yo M Referred for Treatment of Wet AMD Red free FA FA 75 yo M Referred for Treatment of Wet AMD Red free FA FA 2017 MFMER slide MFMER slide yo M Referred for Treatment of Wet AMD AMD? Red free FA FA ILM Draping 2017 MFMER slide MFMER slide

21 Macular Telangiectasia ILM Draping Case Presentation: Atypical Uveitis 64 yo lady 2 months blurred vision, photophobia and photopsias 2017 MFMER slide MFMER slide-116 VA 20/ MFMER slide MFMER slide-118 VA 20/ MFMER slide MFMER slide

22 2017 MFMER slide MFMER slide MFMER slide MFMER slide-124 Case Presentation: Atypical Uveitis No evidence for uveitis: No AC cell or vitreitis; no posterior synechiae. Uveitis labs obtained by referring ophthalmologist All negative! 2017 MFMER slide MFMER slide

23 I think we are missing something here!... I think we are missing something here!... Acute Exudative Polymorphous Vitelliform Maculopathy Best Disease (Vitelliform Macular Degeneration) Retina Image Bank 2017 MFMER slide MFMER slide-128 Additional Medical History? Sent to oncology for PET scan and metastatic surveillance Stage IIIA Cutaneous Melanoma (Pathologic T2a, N1a) Acral Lentiginous melanoma right index finger 10 years prior Sp Amputation of distal phalanx Sp Excision of right upper extremity lesion (metastatic melanoma) 7 years prior Declined adjuvant interferon options Missed previous 2 years metastatic surveillance PET scans 2017 MFMER slide MFMER slide-130 Sent to oncology for PET scan and metastatic surveillance Paraneoplastic Vitelliform Maculopathy Wide spread pulmonary & abdominal metastatic disease Osseous metastasis - L4 spine Brain metastases 2017 MFMER slide MFMER slide

24 Images from referring physician taken 1 yr prior to presentation Case Presentation: Referral for PDT Therapy 35 yo lady Referred for PDT therapy SRF OS for 1 year Diagnosed with CSR OS during pregnancy Fluctuating blurred vision OS 2017 MFMER slide-133 Jun MFMER slide-134 Case Presentation: Referral for PDT Therapy Autofluorescence 2017 MFMER slide MFMER slide-136 Case Presentation: Referral for PDT Therapy EDI -OCT 2017 MFMER slide MFMER slide

25 Case Presentation: Referral for PDT Therapy OS E a r l y IR FA Treatment options? ICG Case Presentatio M i d RF Elevation = 1.4 mm Basal dia. 5.8 mm lat. x 6.0 mm radially 2017 MFMER slide-139 L a t e FAFF MFMER slide-140 OSAugust January March F u n d u s U S 20/30 20/ /25-2 OSAugust January March U S B S C A N Max. elevation = 1.4mm Basal dia. = 5.8mm X 6.0mm Max. elevation = 2.1mm Basal dia. = 6.9mm X 6.5mm Max. elevation = 2.2mm Basal dia. = 6.9mm X 6.5mm S D O C T 2017 MFMER slide-141 A S C A N 2017 MFMER slide-142 Pathology Courtesy: Dr Proia (Duke Pathology) Case Presentation: Referral for PDT Therapy Treatment: Enucleation 2017 MFMER slide MFMER slide

26 Pathology Courtesy: Dr Proia (Duke Pathology) 87 yo F With Abnormal Macular OCT OU A. LEFT EYE (ENUCLEATION): UVEAL MELANOMA Q1: Does this patient have AMD? Q2: Is it safe to proceed with cataract surgery? HISTOPATHOLOGIC TYPE: SPINDLE CELL TYPE (>90% SPINDLE CELLS). HISTOLOGIC GRADE: pg1 (SPINDLE CELL MELANOMA) MFMER slide MFMER slide yo F With Abnormal Macular OCT OU 87 yo F With Abnormal Macular OCT OU Q: Does this patient have AMD? Is it safe to proceed with cataract surgery? VA 20/50 VA 20/70 VA 20/50 VA 20/ MFMER slide MFMER slide yo F With Abnormal Macular OCT OU Abnormal Macular OCT OU FA 2017 MFMER slide-149 Grob et al. Saudi Journal of Ophthalmology MFMER slide

27 Adult-onset Foveomacular Vitelliform Dystrophy (AOFVD) Adult-onset Foveomacular Vitelliform Dystrophy (AOFVD) AKA: Adult-onset vitelliform macular detachment Pseudovitelliform macular degeneration Adult-onset foveomaulcar pigment epithelial dystrophy (AOFPED) 2017 MFMER slide-151 Not a VEGF mediated condition OK to proceed with CE/IOL OU 2017 MFMER slide yo F: Referred for Treatment of AMD with Macular Hemorrhage OD VA 20/ yo F: Referred for Treatment of AMD with Macular Hemorrhage OD 2017 MFMER slide MFMER slide yo F: Referred for Treatment of AMD with Macular Hemorrhage OD Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) Anti-VEGF & PDT 2017 MFMER slide MFMER slide

28 Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) Aneurysmal changes Branching vessels & hyperfluorescent bulbs cluster of grapes 73 yo F: Difficulty With Color Vision VA 20/60 VA 20/ MFMER slide MFMER slide yo F: Difficulty With Color Vision 73 yo F: Difficulty With Color Vision 2017 MFMER slide MFMER slide yo F: Difficulty With Color Vision 1 Year Prior VA 20/60 VA 20/50 73 yo F: Difficulty With Color Vision 1 Year Prior PMH: includes rheumatoid arthritis Meds: includes hydroxychloroquine 400 mg bid x 14 mo 2017 MFMER slide MFMER slide

29 73 yo F: Difficulty With Color Vision 1 Year Prior Hydroxychloroquine Toxicity Hydroxychloroquine Discontinued 1 year Later VA 20/70 VA 20/ MFMER slide MFMER slide-164 Hydroxychloroquine Discontinued 2 years later VA CFs VA CFs 58 yo F Refered For Hydroxychloroquine Screening PMH: Lupus Meds: Include hydroxychloroquine 400mg qd >10 yrs 20/20 20/ MFMER slide MFMER slide-166 y y VA: 20/20 OD; 20/25 OS y y VA: 20/20 OD; 20/25 OS 2017 MFMER slide MFMER slide

30 Radiation Retinopathy Radiation Retinopathy 2017 MFMER slide MFMER slide-170 Radiation Retinopathy Radiation Retinopathy: Macular Edema 2017 MFMER slide MFMER slide-172 Subretinal Fluid Subretinal Fluid 2017 MFMER slide MFMER slide

31 Subretinal Fluid Subretinal Fluid 2017 MFMER slide MFMER slide-176 Vogt-Koyanagi-Harada Disease (VKH) Vogt-Koyanagi-Harada Disease (VKH) 2017 MFMER slide MFMER slide-178 Vogt-Koyanagi-Harada Disease (VKH) 2017 MFMER slide MFMER slide

32 2017 MFMER slide MFMER slide-182 Conclusions Retinal imaging: Continues to revolutionize the field retinal diseases Is crucial for the accurate diagnosis & guiding of treatment Improves our understanding of retinal disease Is constantly evolving Questions & Discussion 2017 MFMER slide MFMER slide-184 Question 1 Which OCT has the highest resolution? A: Swept source OCT B: Spectral domain OCT C: Swept source OCT Question 2 True or False? OCT Angiography can identify leakage from blood vessels? 2017 MFMER slide MFMER slide

33 Question 3 What is EDI-OCT most useful for? A: Identifying small macular holes B: Identifying vitreomacular traction C: Evaluating choroidal thickness D: Evaluating retinal detachments. Question 4 Which imaging modality is best at providing information regarding the health of the retinal pigment epithelium (RPE)? A: Swept source OCT B: Widefield fundus photography C: Fundus autofluorescence (FAF) D: OCT angiography 2017 MFMER slide MFMER slide yo M: Gradual Central Vision Loss ~ 15 yrs VA 20/20 VA 20/60 49 yo M: Gradual Central Vision Loss ~ 15 yrs VA 20/20 VA 20/60 Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide-189 Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide yo M: Gradual Central Vision Loss ~ 15 yrs 49 yo M: Gradual Central Vision Loss ~ 15 yrs Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide-191 Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide

34 AMD? 49 yo M: Gradual Central Vision Loss ~ 15 yrs OD OS Masquerade Differential includes: Cone-rod dystrophy Central areolar choroidal dystrophy (CACD) North Carolina macular dystrophy Myopic Degeneration Drug toxicity Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide MFMER slide yo M: Gradual Central Vision Loss ~ 15 yrs (Late-onset) Cone-Rod Dystrophy fferg: Moderate decrease in photopic responses OU Mild decrease in scotopic responses OU Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide-195 Case courtesy of Dr. Seema Garg, UNC 2017 MFMER slide-196 Central Areolar Choroidal Dystrophy (CACD) 49 yo M: Gradual Central Vision Loss ~ 15 yrs 4 Years Later VA 20/25 VA CF Smailhodzic et al. IOVS, MFMER slide MFMER slide