Short-wave Autofluorescence Research Articles

Rod-sparing in a bardet-biedl syndrome patient with mutations in the ARL6 gene.

Bardet-Biedl Syndrome (BBS) is an autosomal recessive disorder characterized by pleiotropism that affects multiple organ systems. The primary features of BBS include rod-cone dystrophy, renal anomalies, post axial polydactyly, and neurologic deficits. The clinical picture of BBS is extensively heterogenous, with inter and intra familial patients varying in levels of syndromic manifestations and severity of symptoms. In this study we examined a monocular BBS patient who was compound heterozygous for mutations in the ARL6 (BBS3) gene. The patient reported visual complaints consistent with a clinical picture of cone or cone-rod dystrophy. Fundus imaging showed retinal mottling on color photos and a parafoveal hyperfluorescent ring on short wave autofluorescence (SW-AF). Full field electroretinogram (ffERG) revealed normal scotopic step tracings and diminished amplitudes in the photopic steps. This rod-sparing result was consistent with cone-dystrophy and is the first known case of a rod-sparing ffERG phenotype in a BBS patient with mutations in the ARL6 gene. This contributes to the existing phenotype and may potentially contribute to furthering our understanding of BBS pathophysiology.

Physical modelling to assess the effect of micropulse modes of laser radiation at the chorioretinal complex of the human eye based on the theory of activated complex

Purpose: to construct a physical model based on the theory of activated complex (TAC) with indicators ΔH and ΔS mentioned in works of G.I. Zheltov and his colleagues so as to assess the impact of laser radiation micropulse modes at the chorioretinal complex (CRC), as well as to compare it with models based on the Arrhenius integral and with results of micropulse mode testing using autofluorescence method.Material and methods. Modeling of the degree of damage to CRC and adjacent structures was carried out using Eyring equation. Values of ΔH = 360 000 J/mol and ΔS = 890 J/mol·deg for complex of proteins and other RPE organic structures were determined by G.I. Zheltov and co-authors in vivo in a series of laser experiments on primates. For modeling, absorption coefficient of 460 cm–1 was used, for wavelength 577 nm – 10-micron-thick RPE layer and transmission 37 %. Micropulse mode testing (50 μs, 2.4 %, 10 ms, 100 mkm, 0.4–1.9 W) was performed using Navilas 577s navigation laser in patients aged 35–46 with appearance of types 2–3 by the Fitzpatrick scale; results of this testing were assessed using short-wave autofluorescence (488 nm) method.Results. A physical model based on TAC and Eyring integral was constructed. Results of modeling of the degree of RPE injury depending on power coincide with the results of testing of micropulse mode in patients according to autofluorescence method. The average RPE absorption coefficient equal to 440 cm–1 for patients aged 35–46 years and with appearance of type 2–3 by the Fitzpatrick scale was clarified.Conclusion. The computer modeling based on TAC using the Eyring equation with ΔH and ΔS for a complex of proteins and other RPE organic structures, which are described in works of G.I. Zheltov and his co-authors, has shown a high degree of compliance with the results of micro-pulse mode testing in real patients. Modeling does not require adjustment of any free parameters unlike approaches based on the Arrhenius equation; it also allows to find absorption coefficients for a small sample of patients and to assess the level of laser radiation damage to RPE and adjacent structures.

Diagnostic of small choroidal metastasis

Choroidal metastasis in 23–30 % of cases have multifocal growth. In 10–20 % of patients do not have subjective complaints, due to eccentrical localization of choroidal metastases. In these cases, they are detected by chance during examination. In the literature there is no information on the possibility of early diagnosis of “small” foci of metastatic lesions of the choroid using optical coherence tomography (OCT).The aim. To identify diagnostic signs of “small” choroidal metastases.Materials and methods. The study included 4 patients in whom “small” metastases (15 foci) were not visualized by ultrasound.Results. Choroidal metastases, which were not detected by ultrasound, mainly characterized by a multifocal growth pattern, and located mainly outside the central regions of the fundus. The OCT picture in these cases was characterized by the expansion of the choroidal complex up to 1300 μm with an asymmetric shape of the slopes, irregularities of its anterior surface with a moderate hyporeflective structure. At the same time, Bruch’s membrane was preserved, but a violation of the architectonics of the choriocapillary layer is characteristic with a flat profile of the anterior surface of the sclera. Secondary changes in the proper retina are represented by thickening of the retinal pigment epithelium, edema of the photoreceptor layer and of the nuclear layers, while neuroepithelium detachment (NED) appears mainly when the focus is more than 400 μm thick. Initially, choroidal metastasis can be detected by the fundus examination in MultiColor and OCT, as changes appear in the retina – edema of the photoreceptor layer with hyperreflective inclusions and NED, changes appear in the blue-peak autofluorescence (BAF) – hyperBAF with point hypoBAF inclusions, which leads to a change fundus pictures in MultiColor mode.Conclusion. For all patients with a burdened oncological history, even in the absence of visual complaints, in addition to an examination of the fundus of both eyes, it is necessary to provide an examination of the fundus in MultiColor and short-wave autofluorescence modes, followed by OCT in the zones of changes.

Optic Disc Melanocytoma: A Case Report and Review

Introduction: Melanocytoma is a rare benign stationary tumor that usually appears as a pigmented lesion on the optic disk. Optic Disc Melanocytoma (ODM) can compress the optic nerve or undergo necrosis, leading to ischemic axonal loss and visual field defect, similar to those caused by glaucoma. Also, ODM often displays a clinical diagnostic dilemma due to its similarities with melanoma. Some patients have undergone enucleation because of uncertainty between both pathologies. Progressive growth and malignant transformation can be documented by close monitoring of the patient’s eyes. Fundus examination and ancillary imaging procedures such as fundus photo, autofluorescence, B-scan ultrasonography, fluorescein angiography, and spectral-domain optic coherence tomography are powerful tools for ODM diagnosis and management. Case Presentation: A 19-year-old female presented with a decrease in vision in the left eye for about 3 months. Her visual acuity was 20/20 and 20/80 in her right and left eyes, respectively. Funduscopic examination of the left eye showed a well-defined deeply pigmented brownish-black, dome-shaped nodular mass covered the entire optic disc with the normal-appearing overlying vitreous, macula, and surrounding retina. Short-wave autofluorescence revealed hypo-autofluorescence on the pigmented mass lesion. The patient’s condition did not change significantly over 2 years of follow-up. The diagnosis was made as ODM. Conclusions: Melanocytomas grow very slowly over several years or remains stable, in contrast to malignant melanoma. Although ODM tends to have benign behavior, it may adversely affect visual function. Yearly fundus examination is necessary for monitoring growth and detecting malignant transformation. Visual loss can result from optic neuropathy or retinal vascular obstruction. In suspicious cases, close follow-up with serial fundus photographs is essential, even though the malignant transformation is exceptional.

Correlation of multicolor images and conventional color fundus photographs with foveal autofluorescence patterns in diabetic macular edema.

Purpose:The aim of this study is to assess the ability of multicolour imaging (MCI) to detect foveal cysts in diabetic macular edema (DME) and compare it with conventional color fundus photography (CFP) and foveal autofluorescence (FAF) pattern.Methods:It was a retrospective review of 112 eyes of 84 DME patients with central foveal thickness ≥250 μ who underwent MCI, CFP and shortwave autofluorescence imaging. MCI was performed with Sepctralis spectral domain optical coherence tomography (SDOCT) (Heidelberg Engineering, Germany).Results:97 (86.6%) eyes had cystoid increased autofluorescence (cystoid iFAF), 9 (8%) had spot iFAF and 6 (5.35%) had irregular decreased FAF (dFAF). Among eyes with cystoid iFAF, OCT detected DME cysts in 93 (95.6%) eyes, MCI in 75 (77.3%) and CFP in 5 (5.15%) eyes. In all these eyes, the location of cysts on OCT and MCI corresponded with the location of cystoid iFAF, whereas none of the eyes with cyst seen on CFP correlated with the location of cystoid iFAF.Conclusion:MCI was superior to CFP in detecting DME cysts at fovea. It also correlated with hyperautofluorescence pattern in these eyes. MCI may have a potential role in diabetic retinopathy screening by segregating eyes with DME which would require treatment. Our findings need to be further validated in a larger and prospective study design.

Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy.

To describe the ultra-wide field imaging features of pigmented para-venous retino-choroidal atrophy. Retrospective review at a tertiary care centre. Eight eyes of five patients with pigmented para-venous retino-choroidal atrophy who presented to our retina clinic over last 2 years. Retrospective review of ultra-wide field pseudo-colour and short wave autofluorescence imaging was performed. In vivo histology of the macula and areas of retino-choroidal atrophy was studied with swept source optical coherence tomography (SS-OCT). The median age was 40 years (range: 22-67 years). Best corrected visual acuity ranged from perception of light to 20/20. The para-venous retino-choroidal atrophy and pigment clumping not only involved the major arcade vessels but also extended into the peripapillary area and retinal periphery. The affected areas demonstrated hypoautofluorescence with sharp hyperautofluorescent borders. Macular atrophy, epiretinal membrane and optic disc pallor were noted in two eyes each. In all cases, the affected pigmentary area had disorganization of inner retinal layers, disruption of outer retinal layers and retinal pigment epithelium and markedly thinned out choroid on swept source optical coherence tomography. Concurrent involvement with retinitis pigmentosa in the fellow eye was noted in two patients. Ultra-wide field imaging of pigmented para-venous retino-choroidal atrophy sheds light onto the widespread retino-choroidal abnormalities. Concurrent disc and macular involvement may jeopardize the visual function. Pigmented para-venous retino-choroidal atrophy may be considered as a self-limited form of retinitis pigmentosa.

Fibrotic pillar leads to focal choroidal excavation in Best vitelliform dystrophy.

To study focal choroidal excavations in patients with Best vitelliform dystrophy using optical coherence tomography and their topographical relation with fibrotic pillars. This is a retrospective cross-sectional study of consecutive patients diagnosed with Best vitelliform dystrophy at a tertiary eye care center. Records of patients with Best vitelliform dystrophy were reviewed for best-corrected visual acuity, color fundus photographs, shortwave autofluorescence, optical coherence tomography, and electrooculogram with special emphasis on the presence of focal choroidal excavation (FCE) and fibrotic pillar. Main outcome measure was to study the fibrotic pillar in relation to the FCE. Thirty-eight eyes of 19 patients with mean age of 34.6years were enrolled in the study. FCE was seen in eight eyes of six patients. Two patients had bilateral FCE and all the FCEs were located in the area of vitelliform lesion. Six out of eight eyes with FCE were in vitelliruptive stage of disease; one was in pseudohypopyon stage and one in atrophic stage. A fibrotic pillar was seen lying directly above the FCE in seven eyes. In one eye, hyper-reflective material not amounting to fibrotic pillar was seen lying above the FCE. A focal choroidal excavation in the setting of Best vitelliform dystrophy is seen predominantly in the vitelliruptive stage of the disease. Fibrotic pillars appear to play a role in the formation of these FCEs.

Near-infrared and short-wave autofluorescence in ocular specimens

To determine histopathologic characteristics of near-infrared autofluorescence (NIR-AF) and short-wave autofluorescence (SW-AF) in ocular tissue. Retrospective study. Unstained specimens from four enucleated eyes with uveal melanoma were prepared for evaluation by fluorescence microscopy. The filter settings for SW-AF were 450-490 nm for excitation, 500-550 nm for emission and for NIR-AF 672.5-747.5 nm and 765-855 nm respectively. Hyper-SW-AF was detected in the cornea, crystalline lens, anterior border layer of the iris, basement membrane of the iris posterior epithelium, retinal pigment epithelium (RPE), Bruch's membrane, and sclera. Hyper-NIR-AF was detected in pigmented tissues, i.e., iris anterior border layer, iris posterior epithelium, ciliary pigmented epithelium, RPE, pigmented cells in the choroid and pigmented cells in the melanoma tumoral masses. The iris anterior border layer had hyper-SW-AF and hyper-NIR-AF with low magnification. The cells on the iris surface were with hyper-SW-AF; under the iris surface cells with hyper-NIR-AF were detected with high magnification. Both hyper-SW-AF and hyper-NIR-AF were in RPE cells. Pigmented cells with hyper-NIR-AF in other uveal tissues did not have hyper-SW-AF. The pigmented cells in the melanoma tumoral masses had very weak NIR-AF. NIR-AF was seen in the ocular pigmented tissues. The only pigmented tissue with both hyper-SW-AF and hyper-NIR-AF was RPE, the combination of which might help interpret the cellular components of fundus lesions.

Quantitative Comparison of Near-infrared Versus Short-wave Autofluorescence Imaging in Monitoring Progression of Retinitis Pigmentosa

To quantitatively compare near-infrared autofluorescence (NIR-AF) and short-wave autofluorescence (SW-AF) as imaging modalities used to monitor retinitis pigmentosa (RP) disease progression, measured as a function of hyperautofluorescent ring constriction over time. Retrospective cohort study. NIR-AF and SW-AF images were acquired from 22 participants (44 eyes) at 2 clinic visits separated by an average of 2 years. On the images from each modality, the horizontal and vertical diameters and area of the hyperautofluorescent rings were measured twice, 2weeks apart. A progression rate for each parameter was obtained. Descriptive and comparative statistics were calculated to analyze these parameters and their respective progression rates. At both visits, the hyperautofluorescent ring exhibited a larger horizontal diameter (both visits: P < .001), vertical diameter (visit 1: P < .001, visit 2: P= .040), and ring area (visit 1: P= .001, visit 2: P= .011) in SW-AF vs NIR-AF images. In SW-AF, the horizontal diameter, vertical diameter, and ring area decreased yearly by 168 ± 204μm, 131 ± 159μm, and 0.7 ± 1.1mm2, respectively, while in NIR-AF, they decreased by 151 ± 156μm, 135 ± 190μm, and 0.7 ± 1.0mm2. No difference was observed in these rates between SW-AF and NIR-AF. Similar results were observed in the left eye. In SW-AF and NIR-AF images, similar rates of RP disease progression are observed. As such, NIR-AF may confer more advantages as the primary tool for tracking disease progression over the commonly used SW-AF, given the increased patient comfort and cooperation during imaging.

Parapapillary Optic Nerve Head Drusen in Leber Congenital Amaurosis

A 20-year-old male who was known to have Leber congenital amaurosis was assessed. Best-corrected visual acuity was counting fingers in both eyes (OU). Anterior segment OU revealed poorly reacting pupils and nystagmoid movements. Dilated fundus examination revealed widespread bone spicule pigmentation with "macular coloboma" OU (Figure a and b). Short-wave autofluorescence showed hyperautofluorescent buried optic nerve head drusen and generalized hypoautofluorescence OU (Figure c and d). Additionally, the left eye showed parapapillary drusen resulting from calcification of swollen and disrupted axons at a remote location in the parapapillary area (blue arrows; Figure b and d). The presence of buried optic nerve head drusen OU gives a clue to the diagnosis of parapapillary drusen, which can be easily mistaken for retinal astrocytoma. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:554.].

Reticular Pseudodrusen and Thin Choroid Are Associated With Angioid Streaks.

To report the association of angioid streaks in patients with Pseudoxanthoma elasticum (PXE) with reticular pseudodrusen (RPD), thin choroid, and retinal pigment epithelium (RPE) atrophy using swept-source optical coherence tomography (SS-OCT) and short-wave autofluorescence (SWAF). Retrospective cross-sectional study. Records of consecutive patients with angioid streaks due to PXE, who presented with a decrease of vision due to choroidal neovascularization (CNV), were reviewed for best-corrected visual acuity, color fundus photographs, SS-OCT, SWAF, and red-free images with special emphasis on presence or absence of RPD, subfoveal choroidal thickness (SFCT), and RPE atrophy. Sixteen eyes of eight patients with a mean age of 45.5 years ± 9.4 years were enrolled in the study. RPD were seen in 10 of the 16 eyes and were seen commonly along the superotemporal quadrant. Mean subfoveal thickness in study eyes (175.7 μm ± 37.2 μm) was significantly reduced when compared to controls (286.4 μm ± 40.8 μm). The mean SFCT was similar between the eyes with and without CNV. Four eyes had RPE atrophy in the macular area, whereas four eyes had peripapillary RPE atrophy. Angioid streaks in PXE are associated with RPD, thin choroid, and RPE atrophy. These features occur at a younger age as compared to age-related macular degeneration and appear to be interrelated because of single pathophysiological mechanism. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:402-408.].

Infrared autofluorescence, short-wave autofluorescence and spectral-domain optical coherence tomography of optic disk melanocytomas.

To investigate the findings of infrared fundus autofluorescence (IR-AF) and spectral-domain optical coherence tomography (SD-OCT) in eyes with optic disc melanocytoma (ODM). IR-AF findings and those of other ophthalmologic imaging examinations, including short-wave autofluorescence (SW-AF), fluorescein angiography (FA), fundus color photography, and SD-OCT of 8 eyes of 8 consecutive cases with ODM were assessed. The ODMs in all cases (100%) presented similar IR-AF, SW-AF, and FA findings. On IR-AF images, ODMs showed outstanding hyper-AF with well-defined outline. On SW-AF images, the area of ODMs presented as hypo-AF. FA images revealed the leaking retinal telangiectasia on the surface of the ODMs. On SD-OCT images in 8 cases (100%), the ODMs were sloped with highly reflective surface, which were disorganized retina and optic nerve layers. In 7 cases (87.5%), peripapillary choroids were involved. The melanocytomas of 8 cases (100%) presented as optically empty spaces. Vitreous seeds were found in one case (12.5%). IR-AF imaging may provide a new modality to evaluate the pathologic features of ODMs, and together with SW-AF imaging, offers a new tool to study biological characteristics associated with ODMs. SD-OCT is a valuable tool in delimitating the tumor extension and providing morphological information about the adjacent retinal tissue.

Long-term efficacy of half-dose photodynamic therapy on chronic central serous chorioretinopathy

BackgroundTo evaluate the long-term efficacy and complications of half-dose photodynamic therapy (PDT) for treating chronic central serous chorioretinopathy (CSC).MethodsA retrospective, interventional case series of patients with chronic CSC (symptoms ≧3...

Diagnosis value of shortwave autofluorescence for cystoid macular edema

Background Cystoid macular edema (CME) is a manifestation secondary to multiple fundus diseases and often leads to the decline of visual acuity.Optical coherence tomography (OCT) is a common diagnostic method for CME, but it can not reflect the metabolism information of retinal pigment epithelium (RPE).Shortwave autofluorescence (SW-AF)may find the abnormality of macula, but its clinical value remains to be confirmed. Objective This study was to investigate the value of SW-AF in the diagnosis of CME. Methods One hundred and eighty-nine eyes of 140 patients who have the relative diseases to CME or received intraocular surgery were included from May 2010 to August 2011 in Lihuili Hospital of Ningbo city.SW-AF, infrared autofluorescence (IR-AF), fundus fluorescein angiography (FFA) and OCT were performed in all the eyes under the informed consent of each patient.The sensitivity, specificity and other indicators in diagnostic test of SW-AF for CME were evaluated and compared with OCT. Results SW-AF had certain accuracy in diagnosis of CME with the sensitivity 78.29%, specificity 96.67%, Youden index 0.75, positive predictive value 98.06%, negative predictive value 67.44%.The area under curve (AUC) of SW-AF for the diagnosis of CME was 0.875 with the 95% confidence interval (CI) 0.823-0.926 (P<0.001).Compared with OCT, the differences of SW-AF and IR-AF in the diagnosis for CME was statistically significant(χ2=22.53, 91.35, both at P<0.001), and agreement coefficients of diagnosis result between SW-AF、IR-AF or FFA and OCT were 0.67, 0.12 and 0.85. Conclusions SW-AF can be used as a new rapid, non-invasive, ancillary technique in diagnosis of CME with certain accuracy, and the diagnostic agreement of SW-AF with OCT is lower than FFA but is higher than IR-AF. Key words: Macular edema/diagnosis; Fundus autofluorescence; Tomography, optical coherence; Fundus fluorescein angiography; Lipofuscin/metabolism; Diagnostic test

Novel optical coherence tomography classification of torpedo maculopathy.

Torpedo maculopathy is a rare condition with a twofold clinical significance. Firstly, it is a differential of atypical congenital hypertrophy of the retinal pigment epithelium. Secondly, visual field loss has been reported. We demonstrate the spectrum of structural abnormality of torpedo maculopathy as seen on optical coherence tomography, and correlate this with age of presentation, fundus autofluorescence, retinal sensitivity loss and visual field abnormality. A retrospective, observational case series. Five Australian patients seen between 2008 and 2013. Fundus photography, optical coherence tomography, fundus autofluorescence and visual field analysis. One patient underwent fluorescein angiography. Lesion appearance on each imaging modality, and visual field analysis. We consistently observed a flat, hypopigmented lesion located in the temporal macula, with a distinctive tip pointing toward the fovea. Optical coherence tomography demonstrated variable retinochoroidal features ranging from mild outer retinal disturbance (type 1) to outer retinal cavitation (type 2). Lesion appearance on short-wave autofluorescence showed varying degrees of hypo-autofluorescence. Near-infrared autofluorescence was performed in two patients and showed a well-defined region of hypo-autofluorescence. Microperimetry showed normal sensitivity over the lesion in one patient and a dense paracentral scotoma over the temporal portion of the lesion in another. On Humphrey field analysis, only one of two patients tested had a paracentral scotoma. Two types of torpedo maculopathy lesions are described here with unique optical coherence tomography, demographic, fundus autofluorescence and retinal sensitivity features. These may represent different stages of the same disease that evolve over several decades.

Infrared Fundus Autofluorescence and Central Serous Chorioretinopathy

To investigate the findings of infrared fundus autofluorescence in eyes with central serous chorioretinopathy (CSC). This study was an observational follow-up of 83 eyes of 80 consecutive patients with CSC recruited from a hospital referral practice. Infrared autofluorescence (IR-AF) findings and those of other clinical studies, including short-wave autofluorescence (SW-AF), fundus color photography, and optical coherence tomography were assessed. The IR-AF changes that appeared during the follow-up period were recorded. The relationship between IR- and SW-AF was analyzed by comparing the categories of focal autofluorescence (granular hyper-AF, granular hypo-AF, and mixed AF). The influence of final clinical findings on final best corrected visual acuity (BCVA) was analyzed. Twenty-three of 83 (27%) eyes showed granular hyper-IR-AF, whereas 53 (64%) eyes showed granular hyper-SW-AF. Most of the eyes with granular hyper-IR-AF (92%) showed granular hyper-SW-AF. On the contrary, the eyes with granular hyper-SW-AF showed various patterns of IR-AF. The deposits with hyper-IR-AF corresponding to hyper-SW-AF turned into hypo-IR-AF with hyper-SW-AF in four eyes. Final BCVA was significantly worse in eyes with granular hypo-IR-AF compared with the eyes without the findings (P = 0.035). Granular hyper-IR-AF from the deposits in CSC appeared concurrently with hyper-SW-AF. Granular hyper-IR-AF changed from hyperautofluorescence to hypoautofluorescence during the follow-up period. This change of IR-AF characteristics was different from that of SW-AF. The changes are attributable to the modification of melanin in the RPE. The authors speculate that the lipofuscin-like materials contribute to the characteristic changes of IR-AF through the modification of melanin in the RPE.

Autofluorescence findings in Vogt-Koyanagi-Harada disease

To report shortwave autofluorescence (SW-AF) and near infrared autofluorescence (NIR-AF) findings in a case of Vogt-Koyanagi-Harada (VKH) disease. A 22-year-old man presented with a 2-week history of headache, moderate hearing loss, discolored hair, and bilateral gradually decreased vision (20/800 bilaterally). Ophthalmologic examinations revealed bilateral granulomatous anterior uveitis and multiple patchy bullous retinal detachments. Fundus fluorescein angiography (FFA), SW-AF, and NIR-AF imaging were performed with a confocal scanning laser ophthalmoscope at the beginning and after resolution. The patient responded well to systemic prednisolone treatment. Visual acuity and hearing loss improved quickly (visual acuity 16/20 bilaterally). Multilobular dye pooling with a dark rim was observed in serous detached retinal areas on late-phase FFA. These areas were observed as hypoAF in SW-AF and NIR-AF mode due to the blockage. After resolution of serous detachment, numerous hypoAF granular dots were observed scattered over the previously serous detached areas in SW-AF and NIR-AF mode. These hypoAF granular dots were seen as window defect lesion in FFA and interpreted as retinal pigment epithelial damage or atrophy. SW-AF and NIR-AF imaging confirmed granular retinal pigment epithelium atrophy which corresponds to FFA findings. SW-AF and NIR-AF imaging methods are noninvasive and useful techniques for documentation of fundus changes in VKH disease.