Panoramic Fundus Autofluorescence: Clinical Applications And

Transcript

8/6/2012 Panoramic Fundus Autofluorescence: Clinical Applications and Limitations By Jerome Sherman, OD, FAAO • Fundus Autofluorescence ( FAF or AF) is a novel, non-invasive imaging procedure that often yields abnormalities that are invisible to ophthalmoscopy and standard color fundus photography. It allows mapping of lipofuscin distribution in the outer retina which are crucial findings in complex retinal diseases. FAF, however, is limited to outer retinal disorders. • FAF is likely due to lipofuscin, the “wear and tear” pigment found in retinal cells, especially RPE cells. • The normal retinal pigment epithelium (RPE) yields a slightly granular AF glow in contrast to the optic disc and retinal blood vessels which appear black. • The accumulation of lipofuscin, often due to lysosomal dysfunction, results in increased AF and suggests RPE dysfunction or stress. • Decreased FAF suggests loss of RPE cells (as well as possibly photoreceptors) and correlates to reduced levels of lipofuscin. • As revealed in a series of cases to follow, Panoramic FAF is now possible and appears to reveal abnormalities throughout the entire retina, often invisible to other imaging modalities Case 1: Optomap® Color Fundus Image OD A 25 year old moderate myope exhibits an essentially normal color and AF panoramic images in each eye. Case 1: Optomap® Color Fundus Image OS Case 1: Optomap® Fundus Auto Fluorescence Image OD A typical AF image from a normal fundus reveals a slightly granular glow to the RPE. The disc and blood vessels appear as black in distinct contrast to the RPE glow. Case 1: Optomap® Fundus Auto Fluorescence Image OS 1 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 1: Optomap® OD KEY:: Color Optomap Red Separation Green Separation Auto Fluorescence Case 1: Optomap® OS KEY:: Color Optomap Red Separation Green Separation Auto Fluorescence Case 1: Optomap® OD and OS Case 2: Optomap® Color Fundus Image OD KEY:: Color Optomap Auto Fluorescence A 56 year old, 5 diopter myopic Hispanic female presented for examination as a glaucoma suspect. BCVA is 20/20-2 in the right eye and 20/400 with eccentric viewing in the left eye. In the color fundus image, note the visibility of the choroidal vessels, most likely due to myopic stretching and reduction in RPE. Note the nasal, large arcuate pattern of increased visibility of choroidal vessels, most likely due to a posterior staphyloma. Case 2: Optomap® FAF Image OD AF image is essentially within normal limits. Case 2: Optomap® Color Fundus Image OS In addition to similar findings in the fundus of the right eye, the left eye exhibits some subtle areas of pigmentary migration most marked in the mid temporal region. With standard fundus photography of 40-50 degrees of the central fundus, none of these pigmentary changes are imaged. 2 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 2: Optomap® Fundus Image OS Case 2: Optomap® FAF Image OS KEY:: Color Optomap Red Separation Green Separation Auto Fluorescence Note the dramatic FAF in the left eye. Large black (hypo AF) circular and oval zones represent loss of the RPE and lipofuscin. A similar hypo AF zone is surrounding the disc. However, the central part of the image exhibits hyper AF which is suggestive of metabolically stressed RPE. Case 2: Optomap® Color Fundus and FAF Comparison OS Case 2: Optomap® FAF Comparison OD and OS Patient reported sudden loss of vision in her left eye about 3 decades earlier. Evaluation at that time failed to reveal any specific etiology. When questioned she does not recall any trauma prior to vision loss and has no family history of eye problems. Differential diagnosis includes the very rare unilateral RP, DUSN, and trauma such as uniltaral ophthalmic occlusion. Patient is presently being evaluated. Although ophthalmoscopy, standard fundus photography reveal very little difference between the two eyes, Panoramic AF reveals a profound contrast between the normal right eye and the affected left eye. Case 2: Optomap® Color Fundus and FAF Comparison OD and OS Case 3: Optomap® Color Fundus Image OD KEY:: Color Optomap Auto Fluorescence Note that the color fundus images exhibit only a minor difference between the two eyes but the PAF differences are dramatic and unmistakable. A 63 yo BF patient presented for follow up of diabetic retinopathy previously treated with focal laser OU. The patient denied having any difficulty seeing at night. BCVA 20/20 -2 OD and 20/30+2 OS. 3 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 3: Optomap® FAF Image OD Case 3: Optomap® Color Fundus and FAF Comparison OD There is also a peri-macular ring of hyper AF. Note the large hypo AF zone around the disc with an inferior arcuate extension into the temporal retina. Case 3: Optomap® Color Fundus Image OS Case 3: Optomap® FAF Image OS Note very similar findings in the left eye. Case 3: Optomap® Color Fundus and FAF Comparison OS In the color fundus image, note the hard exudates which are typically in the outer plexiform layer. Hard exudates in Henle’s Fiber Layer tend to form a macular star. As expected, hard exudates are virtually invisible with FAF, since FAF is essentially a RPE phenomena. Case 3: Optomap® FAF Comparison OD and OS Note the remarkable symmetry in the PAF images. 4 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 3: Optomap® OD and OS Case 4: Optomap® Color Fundus Image OD Such symmetry suggests a retinal degeneration. This is most likely a case of peri-central RP which often has an autosomal dominant inheritance pattern and typically progresses slowly. We have recommended ERGs and genetic testing. A 60 yo HM who is being followed for primary open angle glaucoma and RP. He notes severe visual field constriction and on occasion reports that he walks into walls. BCVA is 20/30 OU. The flash ERG was extinguished (flat) in both eyes. The fundus appearance is typical of RP and the flat ERG confirms the diagnosis. KEY:: Color Optomap Auto Fluorescence Case 4: Optomap® FAF Image OD Case 4: Optomap® Color Fundus and FAF Comparison OD The hypo AF zones are not predictable from either ophthalmoscopy or Optos color fundus images PAF reveals large, mid-peripheral round and oval dark zones of various sizes. Case 4: Optomap® Color Fundus Image OS Case 4: Optomap® FAF Image OS Similar PAF findings are revealed in the left eye. Note also the hyper AF areas (both eyes) surrounding the macula which suggest that this large zone is under metabolic stress. Such zones of hyper AF most often progress to zones of hypo AF. Initially, it appears as if the RPE is stressed before cell death. 5 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 4: Optomap® Color Fundus and FAF Comparison OS Similar, comparative findings between color fundus and FAF are revealed in the left eye. Case 4: Optomap® OD and OU Case 4: Optomap® FAF Comparison OD and OS Note the symmetry of the PAF images which is quite typical of retina degenerations. Case 5 : Topcon 3D OCT Fundus Image OD KEY:: Color Optomap Auto Fluorescence A 25 yo WM presented with blurred vision and difficulty reading in each eye for the past month although his BCVA is 20/20 –OD and 20/20 OS. The patient reported that he began using Accutane 2 months prior to vision loss. Accutane has recently been discontinued. Case 5 : Optomap® Color Fundus OD Ophthalmoscopy, standard fundus photography and optos color, red separation and green separation images were all normal. Case 5: Optomap® FAF OD In contrast, the PAF images reveal a peri-foveal ring of hypo AF in each eye. 6 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 5 : Topcon 3D OCT Horizontal Raster Image OD Normal PIL Present ELM Present Patient PIL Present ELM Present Cirrus High Resolution Horizontal Scan Image of Normal Control OD External Limiting Membrane PIL OS/RPE PIL Missing The top SD OCT from a control subject reveals a normal photoreceptor integrity line (PIL) that extends uniformly across the 6 mm scan. Below is a horizontal scan through the fovea of the affected right eye. Note that the PIL is present under the fovea, is absent perifoveally and reappears beyond the perifoveal area. RPE/BM RPE Complex Case 5: Topcon 3D OCT Fundus Image OS Although the retinal pigment epithelium (RPE) is only a single layer of cells, it appears as two reflective layers with a dark zone in between on high definition OCT scans. The “inner reflection” has been referred to by some authors as the outer segment-RPE interdigitation (OS/RPE) or Verhoeff’s membrane. The outer reflection is the RPE/Bruch’s membrane complex (RPE/BM). Note the two reflections from the RPE complex are only visible on high definition scans. On lower resolution OCT scans the RPE generally appears as one solid thick band. Case 5 : Optomap® Color Fundus OD Similarly, ophthalmoscopy, standard fundus photography, Optos color, green separation and red separation images were all normal in the left. Case 5: Optomap® FAF OS Case 5 : Topcon 3D OCT Radial Raster Image OS Normal PIL Present ELM Present Patient PIL Present ELM Present PIL Missing The PAF image in the left eye reveals a peri-foveal ring of hypo AF as well. A horizontal section through the fovea of the left eye reveals similar findings as displayed previously in the right eye. A small, but intact, PIL is present under the fovea and a perifoveal absence of the PIL is documented. With loss of the PIL, the intact external limiting membrane (ELM) appears to drape over the missing tissue. 7 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 5: Fundus Comparison OD KEY:: Topcon 3D OCT Fundus Photo Color Optomap Green Separation Auto Fluorescence Case 5: Fundus Comparison OS KEY:: Topcon 3D OCT Fundus Photo Color Optomap Green Separation Auto Fluorescence As in the right eye, only the AF image reveals an abnormality in the left eye. Only the AF image reveals an abnormality in the right eye. Case 5: Optomap® plus with Resmax FAF OD and OS Case 6: Optomap® Fundus Autofluorescence Image of Left Eye The AF Bull’s Eye Maculopathy above is quite symmetric and could represent a retinal toxicity, a cone degeneration or an early cone- rod degeneration. Flash ERGs were performed and were normal under all conditions, effectively ruling out a cone or a cone-rod degeneration. The findings are rather typical of plaquenil toxicity but the patient has never been on plaquenil. Optomap® image of the left eye of a patient with geographic atrophy of the RPE several disc diameters in size. Note the choroidal vessels which are obvious within the lesion because of the dropout of the overlying RPE and choriocapillaris. In addition to drusen nasal to the disc, note widespread peripheral drusen. *Courtesy of the Reykjavik Eye Study Case 6: Optomap® plus with Resmax OS Case 6: Optomap® plus with Resmax Color Fundus Image of Left Eye Note that the hypo AF lesions nasally do not correspond to the drusen and hence the AF images are yielding additional information. At higher magnification the underlying choroidal vessels are more easily visualized within the macular lesion. *Courtesy of the Reykjavik Eye Study *Courtesy of the Reykjavik Eye Study 8 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 7: Optomap® Color Fundus Image OD An 89 yo WF presented for f/u for choroidal neovasculairzation in the OS which was treated with laser 6 years previously. The BCVA remains 20/30 in each eye. Although the macula has no drusen or pigmentary changes, the temporal retina displays drusen and the nasal retina reveals pigmentary clumping. Case 7: Optomap® plus with Resmax™ FAF OD The dark spots below the disc are in different positions in each image and represent remnants of a PVD. Case 7: Optomap® FAF OS The AF image demonstrates hypo AF in the treated lesion, confirming the destruction of the RPE. Peripheral findings are similar to those in the right eye. Case 7: Optomap® FAF OD The PAF image in the right eye has some correspondence with the color image. Case 7: Optomap® Color Fundus Image OS In the left eye which was treated years earlier with laser, the retina, RPE and some of the choroid has been destroyed. The CNV membrane was successfully eliminated and the fovea was spared. See RR #32 for microperimety and SD OCT findings confirming this. Case 7: Optomap® plus with Resmax™ FAF OS The preserved central vision in the left eye is due to the skill of the retinal surgeon combined with a little bit of good fortune. The CNVM has not recurred as well. 9 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 7: Optomap® plus with Resmax™ FAF OD and OS Although the visual acuity is the same in each eye, the appearance of the central retina is dramatically different. This is a case of similar peripheral abnormalities but of different macula involvement. Does peripheral drusen and pigmentary changes increase the risk of macula degeneration? Case 8: Optomap® plus with Resmax™ FAF OD Note that the FAF images accentuate the abnormalities, especially the arcuate perifoveal black zone which signifies death of RPE cells. Case 8: Optomap® Color Fundus Image OS Similar findings are displayed in the left eye. In addition to the retinal findings, note the tilted discs with very large cups. Pressures have remained normal and the peripapillary RNFL is normal and has not changed in over a decade. The patient is still being monitored as a glaucoma suspect. Case 8: Optomap® Color Fundus Image OD 35 yo HM with a history of dots and spots in his fundus over the past decade. BCVA has remained 20/20 OU throughout this period. The appearance of the fundus is rather typical of Stargardt Macular Degeneration with Fundus Flavimaculatus. Note that the spots are not round as the vast majority of drusen are, but are fish tail or pisciform in shape. Without a beaten bronze fovea and normal VA , the clinical diagnosis is Fundus Flavimaculatus. Case 8: Optomap® plus with Resmax™ OD The hyper AF spots suggest that the RPE cells in these areas are stressed. These sick cells may go on to die and the corresponding spots will change from white to black. Case 8: Optomap® plus with Resmax™ FAF OS Equally dramatic AF images are exhibited in the left eye as well. 10 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 8: Optomap® plus with Resmax™ OS As in the right eye, the AF images are far more dramatic than the color images. Case 9: Optomap® Color Fundus Image OD Case 9: A 56 year old, 5 diopter myopic Hispanic female presented for examination as a glaucoma suspect. BCVA is 20/20-2 in the right eye and 20/400 with eccentric viewing in the left eye. In the color fundus image, note the visibility of the choroidal vessels, most likely due to myopic stretching . Note the nasal, large arcuate pattern of increased visibility of choroidal vessels, most likely due to a posterior staphyloma. Case 9: Optomap® Fundus Image OD Case 8: Optomap® plus with Resmax™ FAF OD and OS Note the relative symmetry between the two eyes suggestive of a retinal degeneration. Genetic testing did not reveal an abnormality in the ABCA4 gene and hence only a clinical diagnosis of Stargardt Disease (or really Fundus Flavimaculatus) can be made at present. Another case of Stargardt Disease can be found in and includes detailed SD-OCT images. Case 9: Optomap® FAF Image OD AF image is essentially within normal limits. Case 9: 1: Optomap® Natural Color Fundus Fundus Image Image OSOD KEY: Natural Optomap Color Optomap Auto Fluorescence In addition to similar findings in the fundus of the right eye, the left eye exhibits some areas of pigmentary migration most marked in the mid temporal region. With standard fundus photography of 40-50 degrees of the central fundus, none of these pigmentary changes are imaged. 11 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 9: Optomap® Fundus Image OS Case 9: Optomap® FAF Image OS KEY: Natural Optomap Color Optomap Auto Fluorescence Note the dramatic FAF in the left eye. Large black (hypo AF) circular and oval zones represent loss of the RPE and lipofuscin. A similar hypo A F zone is surrounding the disc. However, the central part of the image exhibits hyper AF which is suggestive of metabolically stressed RPE. Case 9: Optomap® Color Fundus and Natural Fundus OD and OS KEY: Color Optomap Natural Optomap Case 9: Optomap® Color Fundus and FAF Comparison OD and OS KEY: Color Optomap Auto Fluorescence Note that the color fundus images exhibit a modest difference between the two eyes but the PAF differences are dramatic and unmistakable. Recently obtained review of old records suggest that a unilateral ophthalmic artery occlusion secondary to facemask anesthesia as a traumatic event was the etiology . Some clinicians prefer the Natural image to the traditional Color image. Case 10: Optomap® Color Fundus Image OD Case 10: A 45 year old Hispanic female presented with a recent vision blur in her right eye. She also reported that her vision reduction in her left eye dated back about 3 years and was due to plaquenil toxicity. The patient was taking a long list of drugs for lupus (SLE) but she discontinued the plaquenil after 2.5 years because of the vision reduction. The diagnosis of lupus was first made 19 years earlier and oral steroids have been used for virtually the entire 2 decades. She also reported kidney failure, systemic hypertension and dialysis several times a week. Case 10: Optomap® FAF Image OD Best corrected VA was 20/80 in the right eye. The FAF image reveals 3 zones of hypo AF (red arrows) and hyper AF zones within the vascular arcade. In addition, a large stippled zone of hyper AF is also visible. (green border) 12 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 10: Optomap® Color Fundus Image OS The BC VA in the left eye was counting fingers are 6ft. Case 10: Optomap® FAF Image OS The Panoramic AF image reveals that approximately 3 quarters of the fundus exhibits hypo AF suggesting non viable or dead RPE. There is a zone of hyper AF suggesting that the RPE here is stressed. Near normal AF is present superiorly. Plaquenil toxicity, as reported by the patient, is an unlikely etiology of this retinopathy. Case 10: Optomap and Topcon 3D OCT Images OD Case 10: Optomap® OD and OS KEY: Color Optomap Auto Fluorescence Normal The SD OCT in the right eye reveals a large serous detachment of the neurosensory retina which includes the macula. (A case series of central serous retinopathy complicating SLE has been reported)1 Case 2 CirrusTM HD-OCT Horizontal Scan Image of Normal Control OD Histological Section as Compared to the OCT Image Histological Section Normal OCT Image PIL Inner Segments External Limiting Membrane PIL OS/RPE OS/RPE RPE/BM RPE Complex Although the retinal pigment epithelium (RPE) is only a single layer of cells, it appears as two reflective layers with a dark zone in between on high definition OCT scans. The “inner reflection” has been referred to by some authors as the outer segment-RPE interdigitation (OS/RPE) or Verhoeff’s membrane. The outer reflection is the RPE/Bruch’s membrane complex (RPE/BM). Note the two reflections from the RPE complex are only visible on high definition scans. On lower resolution OCT scans the RPE generally appears as one solid thick band. Outer Segments RPE Retinal Pigment Epithelium Although the photoreceptor integrity line, or the PIL (defined as the junction between the inner and outer segments) is barely visible in most histological sections, it is highly prominent in normal SD OCTs. The PIL, as shown above, should be continuous throughout the entire scan in normal eyes. The PIL is considered by some as a mere artifact that is due to the difference in the index of refraction of the inner and outer segments but this artifact is remarkably useful in SD OCT interpretation. * The PIL as Revealed by SD OCT is available at: http://www.lulu.com 13 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 10: Optomap and Topcon 3D OCT Images OS Case 10: Optomap and Topcon 3D OCT Images OD Normal This horizontal OCT section in the right eye is below the fovea and reveals 2 well defined pigment epithelial detachments along with the larger serous detachment of the neurosensory retina and also edema residues. These findings confirm a diagnosis of central serous chorioretinopathy (CSCR) in the right eye. (Visual loss from CSCR in systemic lupus has been reported. ) Case 10 Edema Residues PED Case 10: Optomap and Topcon 3D OCT Images OS In contrast, the SD OCT of the left eye fails to reveal any large fluid elevations. In addition, the PIL is not present and hence explains the very poor visual acuity. Long standing fluid in central serous can lead to loss of the PIL but the photoreceptor loss persists even after the fluid is eventually reabsorbed. Note that lesions inferior to the macula are common in CSCR and are likely due to long term accumulation of fluid inferior to the initial leakage which then “drips” down because of gravity. Note that the last zone to be affected is the superior quadrant since the “drip” begins inferiorly and eventually spreads superiorly. Case 10 Case 10: Optomap® FAF Image OD and OS An OCT section inferior temporal to the macula through the lesion reveals it to be elevated and likely due to a fibro-vascular scar secondary to previous choroidal neovascularization (CNV). Chronic CSCR is known to sometimes result in CNV. Case 10 Although males get CSCR nearly ten times as often as females, females are far more likely to have an auto-immune disease such as SLE. Others have demonstrated that CSCR as a manifestation of SLE can be caused by various factors: systemic hypertension, renal disease, RPE dysfunction and glucocorticoid therapy.1 All are applicable in our patient. It is now well established that corticosteroids are a significant risk factor for CSC. 3 Other far less likely diagnoses are certainly still plausible. Case 11: Optomap® Color Fundus Image OD Case 11: A 47 year old black male presented on referral because of progressive difficulty seeing at night that began at most a decade earlier. He also reported some difficulty with reading in either eye. No family history of any significant eye problems was revealed. Best corrected VA was 20/30– with eccentric fixation in the right eye and 20/50- with eccentric fixation in the left eye. Case 11: Optomap® Fundus Auto Fluorescence Image OD Although pigmentary atrophy, hypertrophy and RPE migration was observable clinically and with the Optomap color, red separation and green separation images, the most dramatic images were obtained with FAF. The bull’s eye lesion in the macula was not at all apparent with the standard images but jumped out with FAF. 14 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 11: Optomap® Color Fundus Image OS Case 11: Optomap® Fundus Auto Fluorescence Image OS The findings are quite similar in the left eye as well. Case 11: Optomap® OD KEY: Color Optomap Red Separation Green Separation Auto Fluorescence Case 11: Optomap® OS KEY: Color Optomap Red Separation Green Separation Auto Fluorescence Case 11: Optomap and Topcon 3D OCT Images OD Case 11: Optomap® OD and OS KEY: Color Optomap Auto Fluorescence In contrast to a horizontal SD OCT through the fovea in a normal patient above, the PIL is present under the fovea but thins and then collapses onto the RPE several degrees away from the fovea. The first change may be the progressive shortening of the outer segments. Normal Around the bull’s eye macula lesion, note that much of the posterior pole revealed hyper AF ovals and symmetric lesions OU. PIL Present PIL Absent PIL Absent 15 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 11: Optomap and Topcon 3D OCT Images OS Case 12: Optomap® Color Fundus Image OD Very similar findings are present in the left eye. The patient has a retinal degeneration which exhibits symmetry between the eyes and slow progression OU. Genetic analysis for known RP defective genes was recommended. Also note the attenuated arteries, a very common finding in retinal degenerations. PIL Present PIL Absent PIL Absent Case 12: A 73 year old white female presented on referral because of unusual photopsias that the patient described as looking like a golden PAC-MAN that moved across her visual field in her right eye. The symptom waxed and waned for nearly a decade. Best corrected VA was 20/25 in each eye. General health history was unremarkable. Visual fields revealed an enlarged blind spot in the right eye and a small scotoma below fixation in the left eye. Case 12: Optomap® Color Fundus Image OS Case 12: Optomap® Fundus Auto Fluorescence Image OD The FAF revealed a large hypo FAF zone around the disc and some small hyper AF spots surrounding the lesion. Much of the temporal and inferior retina revealed a large hypo AF zone. Case 12: Optomap® OD Case 12: Optomap® Fundus Auto Fluorescence Image OS KEY: Color Optomap Red Separation Green Separation Auto Fluorescence FAF revealed a one disc diameter hypo AF lesion within or near the superior arcade above the macula. 16 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 12: Optomap® OS Case 12: Optomap® OD and OS KEY: Color Optomap Red Separation Green Separation Auto Fluorescence KEY: Color Optomap Auto Fluorescence Lack of symmetry in the two eyes suggests that this is not an inherited retinal degeneration. Case 12: Topcon 3D OCT Scan OD Case 12: Optomap and Topcon 3D OCT Images OD Normal Although there is no perfect correspondance between Topcon and Optos images (because of a subtle increase in magnification outside the fovea with Optos), comparisons can be made but are not as precise as desired. The PIL appears quite attenuated where the FAF demonstrates hypo FAF as shown above. Although the PIL appears normal under the fovea in this horizontal section, note the zone of a poorly defined PIL and a zone with a fenestrated PIL between the fovea and the disc. Case 12 PIL Poorly Defined PIL Fenestrated Case 12 : maia™ Sensitivity Map and Zeiss VF OS Case 12: Optomap and Topcon 3D OCT Images OD 3/2011 Based upon the subjective photopsias and the large blind spot, a diagnosis of AZOOR (Acute Zonal Occult Outer retinopathy) is quite possible.5 Although AZOOR often begins invisible to ophthalmoscopy, it often progresses and changes in the RPE can be detected with FAF. The etiology of AZOOR is not known but some believe it to be an autoimmune disorder. Several treated cases suggest that treatment with a drug such as Imuran may be beneficial to prevent further progression. 11/2011 Based upon the maia microperimeter which appears to demonstrate progressive loss of sensitivity over a half year period (greater in the right eye than the left), the patient was informed and is now considering seeing a rheumatologist for a workup and Imuran therapy. 17 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 13: Optomap® Natural Fundus Image OD Case 13: A 64 year old Hispanic female presents for a routine follow-up for a retinal disorder in each eye. As previously, she denies having any visual difficulties. Both her day and her night vision are reported to be OK with no change. No family members are reported to having any visual problem. Best corrected visual acuity is 20/30+ in the right eye and 20/25- in the left eye. Case 13: Optomap® Fundus Auto Fluorescence Image OD FAF reveals hypo AF in zone corresponding to the vascular arcades superior temporal and inferior temporal and a large area of dense hypo AF nasally. In addition, there is a ring of hyper AF immediately within the arcades. The far periphery appears to be within normal limits. Case 13: Optomap® OD Case 13: Optomap® Fundus Auto Fluorescence and Topcon 3D OCT OD KEY: Natural Optomap Color Optomap Auto Fluorescence The PIL is somewhat disorganized under the fovea, normal in the perifoveal area and appears to collapse away from the fovea. Case 13 The appearance of the fundus is dramatic under all three image conditions. Case 13: Optomap® Fundus Auto Fluorescence and Topcon 3D OCT OD Hyper AF appears to correspond to the zones where the PIL becomes attenuated and then collapses onto the RPE. This is best appreciated nasally. Case 13: Optomap® Fundus Auto Fluorescence and Topcon 3D OCT OD Pigment migration to blood vessels in the inner retina is characteristic of many retinal degenerations. Pigment Migration 18 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 13: Optomap® Natural Fundus Image OD Case 13: Optomap® Fundus Auto Fluorescence Image OS Very similar findings are revealed in the left eye. Case 13: Optomap® OS Case 13: Optomap® Fundus Auto Fluorescence and Topcon 3D OCT OS KEY: Natural Optomap Color Optomap Auto Fluorescence Similar findings here as well. The PIL is disrupted under the fovea and thins away from the macula. The diagnosis is pericentral RP, a mild form of RP which is most often autosomal dominant. Attempts to evaluate family members, perform ERGs and obtain genetics are presently underway. As in the right eye, this is most marked nasally here in the left eye as well. Case 14: Optomap® Color Fundus Image OD Case 14: Optomap® Color Fundus Image OD Case 14: A 50 year old Hispanic female presented on referral for an unusual, localized pigmentation in each eye. She had no family history of night blindness or any other serious eye problem. Best corrected VA was 20/20- in each eye. Note the bone spicule pigmentation localized to the inferior retina in the right eye. The crescent shaped bright zone is an artifact since it is not present in the next image. 19 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 14: Optomap® Fundus Auto Fluorescence Image OD Case 14: Optomap® Fundus Auto Fluorescence Image OD In the FAF images, there appears to be a zone of hyper AF between the pigmented area and the posterior pole. This hyper AF indicates that the condition is active and will likely progress more posteriorly. Case 14: Optomap® Fundus Auto Fluorescence Image OS Case 14: Optomap® Color Fundus Image OS Similar findings are revealed in the left eye. Case 14: Topcon 3D OCT Scan Images OD Case 14: Topcon 3D OCT Scan Images OD A horizontal SD OCT section through the fovea OD reveals a normal PIL under the fovea. However, the PIL appears to thin away from the fovea. Normal Pigment Migration Case 14 PIL RPE migration to the retinal vessels in the inner retina is revealed in this SD OCT section. 20 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 Case 14: Topcon 3D OCT Scan Images OS Case 14: Topcon 3D OCT Scan Images OS *PIL Missing RPE Present Similar findings are revealed in the left eye. Normal In the section above, slightly posterior to the band of hypo AF, the OCT reveals that the PIL is absent. Recommendations for a follow –up evaluation were made and include a flash ERG and genetic testing. Evaluating other family members was also suggested. Case 14 PIL Pigment Migration OD vs. OS AF OD vs. OS ResMax AF OD vs. OS AF ResMax OD vs. OS AF 21 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF OD vs. OS AF 22 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF OD vs. OS AF 23 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF 24 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF 25 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF ResMax OD vs. OS AF OD vs. OS ResMax AF OD vs. OS AF OD vs. OS AF ResMax OD vs. OS AF 26 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com) 8/6/2012 OD vs. OS AF ResMax OD vs. OS AF Comments and Conclusions • PAF allows 200 degrees of the fundus to be imaged without dilation in a single 250 msec flash. • PAF documents the integrity of the RPE (and overlying photoreceptors) throughout the entire image. • PAF abnormalities occur in myriad retinal disorders. • No other technology -including OCT and BIO-is capable of providing this level of assessment of the RPE. • PAF reveals abnormalities often invisible to ophthalmoscopy. • Correlation of these myriad FAF patterns with genetics is a future goal. • Additional studies are required to determine the clinical significance of these remarkable images. 27 Create PDF files without this message by purchasing novaPDF printer (http://www.novapdf.com)