Fundus Autofluorescence Findings Research Articles

Whole-Exome Sequencing Identifies Biallelic IDH3A Variants as a Cause of Retinitis Pigmentosa Accompanied by Pseudocoloboma

To identify the genetic cause of and describe the phenotype in 4 families with autosomal recessive retinitis pigmentosa (arRP) that can be associated with pseudocoloboma. Case series. Seven patients from 4 unrelated families with arRP, among whom 3 patients had bilateral early-onset macular pseudocoloboma. We performed homozygosity mapping and whole-exome sequencing in 5 probands and 2 unaffected family members from 4 unrelated families. Subsequently, Sanger sequencing and segregation analysis were performed in additional family members. We reviewed the medical history of individuals carrying IDH3A variants and performed additional ophthalmic examinations, including full-field electroretinography, fundus photography, fundus autofluorescence imaging, and optical coherence tomography. IDH3A variants, age at diagnosis, visual acuity, fundus appearance, visual field, and full-field electroretinography, fundus autofluorescence, and optical coherence tomography findings. We identified 7 different variants in IDH3A in 4 unrelated families, that is, 5 missense, 1 nonsense, and 1 frameshift variant. All participants showed symptoms early in life, ranging from night blindness to decreased visual acuity, and were diagnosed between the ages of 1 and 11 years. Four participants with biallelic IDH3A variants displayed a typical arRP phenotype and 3 participants were diagnosed with arRP and pseudocoloboma of the macula. IDH3A variants were identified as a novel cause of typical arRP in some individuals associated with macular pseudocoloboma. We observed both phenotypes in 2 siblings carrying the same compound heterozygous variants, which could be explained by variable disease expression and warrants caution when making assertions about genotype-phenotype correlations.

Fundus Autofluorescence Imaging in the Assessment of Acute Zonal Occult Outer Retinopathy

Purpose: To evaluate the fundus autofluorescence (FAF) findings in eyes with acute zonal occult outer retinopathy (AZOOR) and the relationships between the FAF, spectral-domain optical coherence tomography (SD-OCT), and Humphrey visual field (HVF) findings. Methods: We performed a retrospective review of the clinical features and FAF, SD-OCT, and HVF findings of 23 eyes of 22 consecutive patients with AZOOR. The mean deviation (MD) values of HVF were compared between areas of normal and abnormal FAF. Results: FAF abnormalities were detected in 21 (91.3%) eyes, with diffuse hyperautofluorescence in 19 (82.6%) eyes and mottling autofluorescence in 2 (8.7%) eyes. In all eyes with FAF abnormality, a clear boundary between the affected and unaffected retina was observed. SD-OCT revealed loss of ellipsoid zone in retinal regions corresponding to the dysfunctional areas demonstrated by HVF. The FAF abnormality also corresponded with the boundaries demonstrated by SD-OCT and/or HVF. The differences in HVF MD values between areas with normal and abnormal FAF in AZOOR eyes were significantly higher than in normal unaffected eyes (p < 0.05). Conclusions: FAF abnormalities were detected in most AZOOR eyes, and the former corresponded to the abnormal retinal regions revealed by SD-OCT and HVF. The findings suggested that FAF is a useful investigation for the assessment of AZOOR.

Multimodal imaging findings in ‘hyper-early’ stage MEWDS

ObjectiveTo describe a new stage of multiple evanescent white dot syndrome (MEWDS), occurring at a very early phase of the disease.MethodsRetrospective analysis of clinical, angiographic and tomographic findings in four...

Optical coherence tomography and fundus autofluorescence findings in areas of geographic atrophy owing to age-related macular degeneration

Optical coherence tomography and fundus autofluorescence findings in areas of geographic atrophy owing to age-related macular degeneration

ULTRA-WIDE-FIELD FUNDUS AUTOFLUORESCENCE FINDINGS IN PATIENTS WITH ACUTE ZONAL OCCULT OUTER RETINOPATHY.

To determine whether ultra-wide-field fundus autofluorescence (UWFFAF) findings in acute zonal occult outer retinopathy correlated well with perimetry, optical coherence tomography, and electroretinography findings. Retrospective observational study on 16 eyes of 10 subjects with AZOOR seen at a single referral center from October 2012 to March 2015 who had UWFFAF performed. Chi-square analysis was performed to compare categorical variables, and Mann-Whitney U test used for comparisons of nonparametric continuous variables. All eyes examined within 3 months of symptom onset (five of the five eyes) had diffusely hyperautofluorescent areas on UWFFAF. The remaining eyes contained hypoautofluorescent lesions with hyperautofluorescent borders. In 11/16 (68.8%) eyes, UWFFAF showed the full extent of lesions that would not have been possible with standard fundus autofluorescence centered on the fovea. There were 3 patterns of spread: centrifugal spread (7/16, 43.8%), centripetal spread (5/16, 31.3%), and centrifugal + centripetal spread (4/16, 25.0%). The UWFFAF lesions corresponded well with perimetric, optical coherence tomography, and electroretinography abnormalities. The UWFFAF along with optical coherence tomography can be useful in the evaluation and monitoring of acute zonal occult outer retinopathy patients.

Fundus Autofluorescence Patterns of Submacular Fluid Resolution Following Repair of Macula-Involving Rhegmatogenous Retinal Detachments.

Submacular fluid (SMF) can persist for months to years following rhegmatogenous retinal detachment (RD) repair. The authors' objective was to describe fundus autofluorescence (FAF) and optical coherence tomography (OCT) findings associated with the absorption of persistent submacular fluid (SMF) following RD repair. Retrospective review of clinical data and FAF and OCT imaging from sequential postoperative visits in a cohort of patients with persistent SMF following RD repair. In 11 of 13 eyes with persistent SMF, patches of decreased FAF signal corresponded to SMF on OCT. In eight eyes, there was a hypo- to hyperautofluorescence transition at the time of SMF resolution. These areas of increased FAF signal correlated with inner segment/outer segment (IS/OS) junction loss on OCT. FAF imaging can be informative when following SMF after RD repair; a hypo- to hyper-FAF signal transition correlates with SMF resolution and photoreceptor loss. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1020-1029.].

Clinical, optical coherence tomography, and fundus autofluorescence findings in patients with intraocular tumors.

PurposeTo describe clinical, optical coherence tomography (OCT) and fundus autofluorescence (FAF) findings in patients with intraocular tumors and determine if OCT and FAF could be helpful in the differential diagnosis and management of different choroidal tumors.MethodsForty-nine patients with untreated, macular, midperipheral, and extrapapillary intraocular tumors were included. All patients underwent ophthalmic examination: best-corrected visual acuity, slit-lamp biomicroscopy, funduscopy, and standardized B mode, and if possible A mode, ultrasonography, and OCT and FAF imaging of the surface of the intraocular tumors.ResultsOf the 49 patients studied, 19 had choroidal nevi, ten had indeterminate choroidal melanocytic lesions (IMLs), ten had malignant melanomas, and ten had other choroidal tumors. The choroidal nevi revealed subretinal fluid (SRF) on OCT in only 11%. FAF detected isoauto-fluorescence in 42%, hypoautofluorescence in 37%, patchy FAF pattern in 16%, and a diffuse FAF pattern in 5%. Seventy percent of patients with IML showed SRF on OCT and 20% showed tumor growth on follow-up, detected only by OCT and FAF imaging. FAF revealed a patchy pattern in 50% and a diffuse pattern in 40% of cases with IML. Ninety percent of the patients with choroidal melanoma had SRF on OCT and FAF revealed a patchy pattern in 60% and a diffuse pattern in 40%. Patients with other choroidal tumors had SRF on OCT in 30% of cases and no characteristic pattern on FAF.ConclusionBoth OCT and FAF were helpful in the differential diagnosis of choroidal nevi versus IMLs, choroidal melanomas, and other choroidal tumors. Also, detailed and periodical clinical evaluation of patients with intraocular tumors using OCT and FAF imaging for the detection of both SRF and FAF patterns overlying the tumor can be useful for detection of tumor growth.

Fundus autofluorescence and SD‐OCT in progressive cone dystrohy

PurposeTo describe fundus autofluorescence (FAF) and Spectral domain optic coherence tomography (SD‐OCT) findings in progressive cone dystrophy (PCD) and evaluate the benefits of these methods for diagnosis.MethodsAll patients with the diagnosis of PCD based on color sense, visual field and electrophysiological tests were included. They underwent full ophthalmic examination with best‐corrected Snellen visual acuity (BCVA), fundus photography, SD‐OCT and FAF.ResultsSixteen eyes of 8 patients were included. Mean age was 36.5 years and 19 years at the beginning of signs. Mean BCVA was 0.18 (range 0.05–0.4). On biomicroscopy, we found changes in the retinal pigment epithelium ranging from barely detectable changes (6 eyes) up to the typical bull's eye appearance (8 eyes). We found central macular atrophy in 2 eyes. OCT showed statistically significant reduction in the thickness and structural changes in the macular retina, predominating at the perifoveolar zone in 8 cases. Atrophy was evident especially in the outer nuclear layer and the ellipsoid zone. Visual acuity was mainly dependent on the degree to which the continuity of the ellipsoid was maintained in subfoveolar. FAF was normal in 2 eyes, showed heterogeneous hyper‐FAF in 4 eyes, hypo‐FAF in 2 eyes, small area of mild hypo‐FAF in the perifoveal region surrounded by a ring of hyper‐FAF in 8 eyes. The junction between normal and abnormal outer retina on OCT corresponded to the hyper‐autofluorescent ring.ConclusionsOCT and FAF specifies the quantitative and qualitative changes in the macula and may contribute significantly to the diagnosis of the progressive cone dystrophy, particularly in the early stages of the disease which is difficult to diagnose.

Characteristics of Fundus Autofluorescence in Active Polypoidal Choroidal Vasculopathy.

Objectives:To define characteristic fundus autofluorescence (FAF) findings in eyes with active polypoidal choroidal vasculopathy (PCV).Materials and Methods:Thirty-five eyes of 29 patients with active PCV who were diagnosed at Ege University Faculty of Medicine, Department of Ophthalmology, Retina Division between January 2012 and November 2014 were included in the study. All the patients underwent a complete ophthalmological examination including fundus photography, spectral-domain optical coherence tomography, fluorescein angiography, FAF photography, and indocyanine green angiography (ICGA). ICGA was used to diagnose active PCV and identify lesion components. FAF findings were described at the retinal site of the corresponding lesions identified and diagnosed using ICGA.Results:The mean age of the 29 study patients (15 men, 14 women) was 64.6±7.5 years (range, 54-82 years). ICGA revealed active PCV in 35 eyes, consisting of polypoid lesions in 11 eyes (31.4%), branching vascular networks (BVN) in 10 eyes (28.6%), and a combination of polypoid lesions and BVNs in 14 eyes (40%). On FAF images, 4 different patterns were detected at the corresponding retinal sites of 25 polypoid lesions detected by ICGA: confluent hypoautofluorescence with a hyperautofluorescent ring in 18 eyes (72%), hyperautofluorescence with hypoautofluorescent ring in 2 eyes (8%), confluent hypoautofluorescence in 1 eye (4%), and granular hypoautofluorescence in 1 eye (4%). The remaining 3 eyes (12%) demonstrated blocked hypoautofluorescence because of the excessive hemorrhaging in the macula. The FAF images showed the granular hypoautofluorescent FAF pattern in all 24 BVNs (100%) consistent with the location of the lesions on ICGA.Conclusion:The typical PCV lesions, polypoid lesions and BVNs had characteristic autofluorescent findings on FAF imaging. Non-invasive, quick, and repeatable FAF imaging can be considered a reliable and helpful diagnostic technique for the diagnosis of active PCV.

Photoreceptor Progenitor mRNA Analysis Reveals Exon Skipping Resulting from the ABCA4 c.5461-10T→C Mutation in Stargardt Disease

To elucidate the functional effect of the ABCA4 variant c.5461-10T→C, one of the most frequent variants associated with Stargardt disease (STGD1). Case series. Seventeen persons with STGD1 carrying ABCA4 variants and 1 control participant. Haplotype analysis of 4 homozygotes and 11 heterozygotes for c.5461-10T→C and sequence analysis of the ABCA4 gene for a homozygous proband. Fibroblasts were reprogrammed from 3 persons with STGD1 into induced pluripotent stem cells, which were differentiated into photoreceptor progenitor cells (PPCs). The effect of the c.5461-10T→C variant on RNA splicing by reverse-transcription polymerase chain reaction was analyzed using PPC mRNA. Invitro assays were performed with minigene constructs containing ABCA4 exon 39. We analyzed the natural history and ophthalmologic characteristics of 4 persons homozygous for c.5461-10T→C. Haplotype and rare variant data for ABCA4, RNA splice defects, age at diagnosis, visual acuity, fundus appearance, visual field, electroretinography (ERG) results, fluorescein angiography results, and fundus autofluorescence findings. The frequent ABCA4 variant c.5461-10T→C has a subtle effect on splicing based on prediction programs. A founder haplotype containing c.5461-10T→C was found to span approximately 96 kb of ABCA4 and did not contain other rare sequence variants. Patient-derived PPCs showed skipping of exon 39 or exons 39 and 40 in the mRNA. HEK293T cell transduction with minigenes carrying exon 39 showed that the splice defects were the result of the c.5461-10T→C variant. All 4 subjects carrying the c.5461-10T→C variant in a homozygous state showed a young age of STGD1 onset, with low visual acuity at presentation and abnormal cone ERG results. All 4 demonstrated severe cone-rod dystrophy before 20 years of age and were legally blind by 25 years of age. The ABCA4 variant c.5461-10T→C is located on a founder haplotype lacking other disease-causing rare sequence variants. Invitro studies revealed that it leads to mRNA exon skipping and ABCA4 protein truncation. Given the severe phenotype in persons homozygous for this variant, we conclude that this variant results in the absence of ABCA4 activity.

Multifocal Electroretinogram and Fundus Autofluorescence Findings in Acute Zonal Occult Outer Retinopathy during a Three- Year Follow-Up

Acute zonal occult outer retinopathy is a disease difficult to diagnose. Within this pathology, many clinical and para-clinical tests appear normal regardless the great advances in technology. However, the findings we discovered with electrophysiology when paired with a thorough clinical anamnesis are the more reliable parameters for giving the right diagnosis.We present a clinical case of a 26 year old patient with diagnosis of acute zonal occult outer retinopathy whose optical coherence tomography and fluorescein angiography results were normal while the results of the wide field electroretinogram and multifocal electroretinogram showed alterations in the outermost layers of the retina which correlated with inferior arcuate defect in the visual field and peri-macular areas of hyperautofluorescence in the auotofluorescence. The evolution of the case is shown during 3 years appreciating the disappearance of the autofluorescence as well as progressive improvement in the visual field.

Outer Retinal Layers Alterations in Chronic Central Serous Chorioretinopathy: Spectral Domain-OCT and Fundus Autofluorescence Findings

Purpose: To document alteration of the outer retinal layers and fundus autofluorescence (FAF) change in eyes with chronic central serous chorioretinopathy (CSCR). Methods: Seventeen eyes of fifteen patients diagnosed with chronic CSCR from January 2014 to March 2015 with at least 6 months of follow-up and no history of surgery were enrolled in this study. Morphologic alterations in the retinal pigment epithelium (RPE), the photoreceptor inner segment/outer segment (IS/OS) junction layer, and the outer retina were evaluated by Spectral domain optical coherence tomography (SD-OCT). The FAF images were obtained via Heidelberg Retina Angiogram using a view mode of 30° and were described using distribution patterns of retinal autofluorescence compared to background autofluorescence. After tomographic alignment of the FAF intensities with the OCT findings, we analyzed and assessed the alteration of the outer retinal layers and the characteristics of retinal autofluorescence. Results: RPE detachment lesions in SD-OCT showed little or no change in autofluorescence pattern. Five of seven eyes with RPE humps in SD-OCT showed hyper-fluorescence in FAF. All of the eyes with defects or changes of the reflective line representing the IS/OS junction in SD-OCT correlated with hyper-fluorescence in FAF. Seven of nine eyes with persistent subretinal detachment (SRD) showed specific ring-shaped hyper-fluorescence in FAF matching up with the border of the SRD, and five eyes with photoreceptor OS elongation within SRD showed a granular type of hyper-fluorescence within the ring-shaped hyper-fluorescence in FAF. Conclusions: In chronic CSCR, we found that RPE humps, the lesion IS/OS junction defect, the border of SRD, photoreceptor OS elongation in SD-OCT correlate with hyper-fluorescence lesions in FAF. The areas of the autofluorescence changes in the FAF images are associated with the extent of change in the RPE and outer retina. J Korean Ophthalmol Soc 2016;57(5):763-771

Fundus Autofluorescence and Enhanced Depth Imaging Spectral-Domain Optical Coherence Tomography in Hunter Syndrome-New Insights

Introduction: Hunter syndrome or mucopolysaccharidosis type II is a rare progressive multi-systemic disorder, caused by an abnormal storage of glycosaminoglycans (GAGs) in almost every cell type, including most ocular tissues [1,2]. Patients have a short life expectancy and ocular manifestations can be present early in the course of disease [1,2]. Purpose: To report the fundus autofluorescence and tomographic ocular findings in Hunter syndrome. Methods: A 18-year-old male patient with Hunter syndrome with progressive nyctalopia was submitted to color fundus photography, blue fundus autofluorescence (FAF), fluorescein angiography (FA) and spectral domain optical coherence tomography with enhanced-depth imaging (EDI-SD OCT). Results and discussion: Fundus examination and wide-field fluorescein angiogram revealed normal optic discs and bilateral pigmentary atrophic changes at the mid periphery with macular sparing. SD OCT revealed a retinal thinning due to external retinal atrophy affecting the photoreceptor layer beyond the parafoveal area. Although a prominent central external limiting membrane (ELM) was present, both the ellipsoid zone band and ELM could not be tracked beyond the central 2-mm and 2. 5 mm diameter ring, respectively. EDI-SD OCT revealed a highly irregular choroid, especially in its outer boundary, probably due to GAG scleral deposition. Blue FAF presented a symmetric hyperautofluorescent parafoveal ring that corresponded to the area where the ELM was present in the absence of the ellipsoid band. A mottled hyper/hypofluorescent pattern was present at the mid-peripheral retina. Scarce hyperautofluorescent dots were found in the left optic disc resulting from disc drusen which could result from axoplasmic flow disturbances due to GAGs scleral deposition. Conclusions: To our knowledge this is the first report of fundus auto-fluorescence imaging in Hunter syndrome. We also report, for the first time, optic disc drusen in this disease. New imaging techniques can provide new insights for the understanding of this disease, and could potentially provide valuable biomarkers for treatment guidance.

Fundus autofluorescence findings in central serous chorioretinopathy using two different confocal scanning laser ophthalmoscopes: correlation with functional and structural status.

To compare autofluorescence (AF) findings using wide-field (Optomap) and conventional (HRA-AF) confocal scanning laser ophthalmoscopy (cSLO) systems in patients with central serous chorioretinopathy (CSC), and to investigate the correlations between AF findings and functional and anatomical status. Optical coherence tomography (OCT) and AF images were compared in 73 eyes with serous retinal detachment (SRD) (group A) and 30 eyes without SRD (group B). We evaluated AF findings from the SRD region, atrophic area, and foveola. Correlations between AF findings and outer retinal abnormalities in OCT and visual acuity (VA) were analyzed. Optomap-AF was more effective than HRA-AF in identifying the margins of a detached area (P = 0.001) in group A, and for monitoring mild outer retinal damage (P = 0.041) in group B. The foveolar AF grades in both instruments were significantly correlated with VA and central foveal thickness (CFT) in both group A (Optomap, VA r s = 0.33, P = 0.012; CFT r s = -0.38, P = 0.002; HRA, VA r s = 0.62, P < 0.001; CFT r s = -0.70, P < 0.001) and group B (Optomap, VA r s = 0.71, P < 0.001, CFT r s = -0.78, P < 0.001; HRA, VA r s = 0.40, P = 0.026, CFT r s = -0.40, P = 0.030). Optomap-AF was found to be advantageous for monitoring subretinal status in eyes with SRD, and more accurately reflected mild outer retinal changes in eyes without SRD. Foveolar AF grades of both imaging modalities were significantly correlated with functional and anatomical status.

Optical Coherence Tomography and Fundus Autofluorescence evaluation in an animal model of Retinal Degeneration

PurposeTo evaluate fundus autofluorescence (FAF) and OCT changes comparing with immunohistochemical (ICC) analysis in long term degeneration of P23H rat and to investigate retinal and choroidal vascularization using fluorescein and indocianin green angiography.MethodsTwenty‐albino homozygous P23H line 1 rats aging from 18 postnatal days (P18) to 27 months and wild‐type albino Sprague‐Dawley (SD rats) (2 and 15 months old) were used for this study. Normal pigmented Long Evans (LE) 2 months old rats were used to compare FAF findings. ICC was performed to correlate with the findings of OCT and AF changes.ResultsFAF pattern varied from not findings at P18 to a mosaic of hyperfluorescent dots in the rats of 6 months or older. Retinal thicknesses diminished during the time: 205.2–183.18 μm in SD rats and 189.88–58.15 μm in P23H rats. In long term degeneration, OCT showed severe changes at the retinal layers; ICC helped to identify the cell loss and remodeling changes.ConclusionsAutofluorescent ophthalmoscopy is a non‐invasive method that can detect changes in metabolic activity at the RPE in animal models of retinal degeneration in vivo. Retinal vascular plexus changed with aging. OCT showed a diminution of retinal thickness and retinal layer changes with the degeneration. ICC shows a good correlation.

Choroidal neovascularization, outer retinal tubulation and fundus autofluorescence findings in a patient with enhanced S‐cone syndrome

Choroidal neovascularization, outer retinal tubulation and fundus autofluorescence findings in a patient with enhanced <scp>S</scp>‐cone syndrome

Progression of Retinal Pigment Epithelial Atrophy in Antiangiogenic Therapy of Neovascular Age-Related Macular Degeneration

PurposeTo monitor retinal pigment epithelial (RPE) atrophy progression during antiangiogenic therapy of neovascular age-related macular degeneration (AMD) over 2 years using polarization-sensitive optical coherence tomography (OCT).DesignProspective interventional case series.Methodssetting: Clinical practice. study population: Thirty patients (31 eyes) with treatment-naïve neovascular AMD. observation procedures: Standard intravitreal therapy (0.5 mg ranibizumab) was administered monthly during the first year and pro re nata (PRN; as-needed) during the second year. Spectral-domain (SD) OCT and polarization-sensitive OCT (selectively imaging the RPE) examinations were performed at baseline and at 1, 3, 6, 12, and 24 months using a standardized protocol. RPE-related changes were evaluated using a semi-automated polarization-sensitive OCT segmentation algorithm and correlated with SD OCT and fundus autofluorescence (FAF) findings. main outcome measures: RPE response, geographic atrophy (GA) progression.ResultsAtrophic RPE changes included RPE thinning, RPE porosity, focal RPE atrophy, and development of GA. Early RPE loss (ie, RPE porosity, focal atrophy) increased progressively during initial monthly treatment and remained stable during subsequent PRN-based therapy. GA developed in 61% of eyes at month 24. Mean GA area increased from 0.77 mm2 at 12 months to 1.10 mm2 (standard deviation = 1.09 mm2) at 24 months. Reactive accumulation of RPE-related material at the lesion borders increased until month 3 and subsequently decreased.ConclusionsProgressive RPE atrophy and GA developed in the majority of eyes. RPE migration signifies certain RPE plasticity. Polarization-sensitive OCT specifically images RPE-related changes in neovascular AMD, contrary to conventional imaging methods. Polarization-sensitive OCT allows for precisely monitoring the sequence of RPE-related morphologic changes.

Fundus autofluorescence and optical coherence tomography findings in glutathione synthetase deficiency

We report the autofluorescence pattern and optical coherence tomography (OCT) characteristics in a 6-year-old boy with glutathione synthetase deficiency. The patient underwent complete ophthalmological examination, including full-field electroretinography, visual evoked potentials, fundus autofluorescence, and OCT imaging. Electrophysiological studies showed time-delay and subnormal responses. Fundus autofluorescence imaging revealed increased parafoveal autofluorescence compared to normal, and OCT showed alteration of photoreceptor and retinal pigment epithelium layers in the parafoveal region.

Bartonella Neuroretinitis: A Case Report with New Insights from Fundus Autofluorescence Imaging

Purpose: Fundus autofluorescence (FAF) is a modern functional imaging modality utilized to characterize abnormalities of the retinal pigment epithelium (RPE). There are no prior studies demonstrating FAF findings in both symptomatic and asymptomatic eyes of patients diagnosed with Bartonella neuroretinitis. Methods: Case report and review of the literature. Patients: Single patient case study. Results: In the symptomatic right eye, (FAF-Heidelberg Retina Angiograph; Heidelberg Engineering, Heidelberg, Germany) identified abnormality of the RPE which corresponded to pathology seen on fundus photography, fluorescein angiography (FA-Heidelberg Retina Angiograph; Heidelberg Engineering, Heidelberg, Germany) and spectral domain optical coherence tomography (SD-OCT-Spectralis®, Heidelberg Engineering, Heidelberg, Germany). Interestingly, FAF also demonstrated sub-clinical anatomic abnormalities not seen clinically nor detected as clearly by any other imaging modalities in the patient’s asymptomatic left eye. As the patient improved clinically, normalization of the FAF occurred in both eyes. Discussion: This report is the first study to describe novel subclinical and clinical FAF findings as part of the evaluation of a patient with infectious neuroretinitis. Compared to FA, SD-OCT and clinical examination, FAF provided a more detailed functional understanding of this condition in its early stages and was also used to follow the clinical course of this patient. This functional imaging modality confirmed normalization of anatomic abnormalities also demonstrated by the other techniques.

MULTIMODAL IMAGING OF THE RETINA AND CHOROID IN SYSTEMIC AMYLOIDOSIS.

To present the multimodal imaging findings of four patients with systemic amyloidosis, renal failure, and chorioretinopathy. Retrospective analysis of four patients presenting to four institutions with evidence of amyloid induced chorioretinopathy. Fundus photography, autofluorescence, and spectral domain optical coherence tomography findings were studied and are presented. Four patients with biopsy-proven systemic amyloidosis demonstrated progressive chorioretinal degeneration with color fundus photography and autofluorescent imaging. With spectral domain optical coherence tomography analysis, amyloidosis-induced chorioretinopathy was characterized by a widened choriocapillaris band, choroidal infiltration, diffuse photoreceptor dysfunction, and thinning of the outer nuclear layer. Multimodal imaging including spectral domain optical coherence tomography analysis in eyes of patients with systemic amyloidosis shows deposition in the choroid. The deposition may cause a secondary toxic and or barrier effect resulting in diffuse retinal pigment epithelium and photoreceptor dysfunction.