Related Articles

Purpose

To describe the retinal imaging findings in the index patient with Heimler syndrome (OMIM #234580).

Design

Non-interventional case report.

Methods

A 29-year-old woman with Heimler syndrome developed bilateral vision loss. Fluorescein angiography (FA), fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT) and electroretinography (ERG) were performed to assess the retinal anatomy and function.

Results

FA showed mottling of the retinal pigment epithelium (RPE) in the posterior pole and periphery of the retina. FAF revealed hyper and hypoautofluorescent dots corresponding to the RPE mottling observed on FA. SD-OCT documented loss of the inner/outer segments boundary, and RPE thinning. ERG testing excluded generalized rod-cone dysfunction.

Conclusion

We report an adult-onset macular dystrophy in one of the previously reported patients with Heimler syndrome and hypothesize that this syndrome is probably an expression of a ciliopathy.

PURPOSE

To describe the features of intraretinal retinal pigment epithelium (RPE) migration documented on a prototype spectral domain high speed, ultrahigh resolution optical coherence tomography (OCT) device in a group of patients with early to intermediate dry age-related macular degeneration (AMD). To correlate intraretinal RPE migration on OCT to RPE pigment clumping on fundus photographs.

DESIGN

Retrospective, non-comparative, non-interventional case series.

PARTICIPANTS

Fifty-five eyes of 44 patients seen at the New England Eye Center between December 2007 and June 2008 with early to intermediate dry AMD.

METHODS

3D OCT scan sets from all patients were analyzed for presence of intraretinal RPE migration, defined as small discreet hyper-reflective and highly-backscattering lesions within the neurosensory retina. Fundus photographs were also analyzed to determine the presence of RPE pigment clumping, defined as black-colored, often spiculated areas of pigment clumping within the macula. OCT en face images were correlated with fundus photographs to demonstrate correspondence of intraretinal RPE migration on OCT and RPE clumping on fundus photo.

MAIN OUTCOME MEASURES

Drusen, Dry AMD, intraretinal RPE migration, RPE pigment clumping.

RESULTS

54.5% of eyes (61.4% of patients) demonstrated intraretinal RPE migration on OCT scans. 56.4% of the fundus photographs demonstrated RPE pigment clumping. All eyes with intraretinal RPE migration on OCT had corresponding RPE pigment clumping on fundus photographs. RPE pigment migrated most frequently into the outer nuclear layer (66.7% of eyes) and less frequently into more anterior retinal layers. Intraretinal RPE migration mainly occurred above areas of drusen (73.3% of eyes).

CONCLUSIONS

The appearance of intraretinal RPE migration on OCT is a common occurrence in early to intermediate dry AMD, occurring in 54.5% of eyes or 61.4% of patients. The area of intraretinal RPE migration on OCT always correlated to areas of pigment clumping on fundus photography. Conversely, all but one eye with RPE pigment clumping on fundus photography also had areas of intraretinal RPE migration on OCT. The high incidence of intraretinal RPE migration observed above areas of drusen suggests that drusen may play physical and catalytic roles in facilitating intraretinal RPE migration in dry AMD patients.

Purpose:

To establish the characteristics of secondary retinal and retinal pigment epithelial (RPE) changes associated with the presence of choroidal melanoma and choroidal nevus as documented by optical coherence tomography (OCT) and fundus autofluorescence (FAF).

Materials and Methods:

PubMed review of major English publications examining the correlation between clinical characteristics of choroidal melanoma and nevus with OCT and FAF findings.

Results:

The intrinsic properties of choroidal melanoma, as well as overlying RPE changes, drusen, and lipofuscin are best characterized by FAF, while OCT is more sensitive for the identification of subretinal and intraretinal fluid as well as atrophy, degeneration, and photoreceptor loss in the neurosensory retina.

Conclusions:

Secondary retinal changes associated with choroidal melanocytic lesions can be documented by OCT and FAF. OCT-evident changes are observed more often with choroidal melanoma than choroidal nevus. OCT is better suited to identify the overlying retinal detachment and edema, even before these findings are clinically apparent. FAF is most useful in documenting the presence of lipofuscin, a finding that represents one of the important criteria in differentiating small choroidal melanoma from benign choroidal nevus.

Purpose

To correlate the degree of functional loss with structural changes in patients with Stargardt disease.

Methods

Eighteen eyes of 10 Stargardt patients were studied. Scanning laser ophthalmoscope (SLO) infrared images were compared to corresponding spectral domain optical coherence tomography (SD-OCT) scans. Additionally, SLO microperimetry was performed and results were superimposed on SLO infrared images and in selected cases on fundus autofluorescence (FAF) images.

Results

Seventeen of 18 eyes showed a distinct hypo-reflective foveal and/or perifoveal area with distinct borders on SLO-infrared images which was less evident on funduscopy and incompletely depicted in FAF images. This hypo-reflective zone corresponded to areas of significantly elevated psychophysical thresholds on microperimetry testing, in addition to thinning of the retinal pigment epithelium (RPE), disorganization or loss of the photoreceptor cell inner-outer segment (IS-OS) junction and external limiting membrane (ELM) on SD-OCT.

Conclusion

SLO-infrared fundus images are useful for depicting retinal structural changes in Stargardt patients. An SD-OCT/SLO microperimetry device allows for a direct correlation of structural abnormalities with functional defects that will likely be applicable for the determination of retinal areas for potential improvement of retinal function in these patients during future clinical trials and for the monitoring of the diseases' natural history.

Background. To describe the standard autofluorescence (FAF), the near infrared autofluorescence (NIA) and optical coherence tomography (OCT) patterns in central serous chorioretinopathy, correlating them with fluorescein angiography. Methods. Cross-sectional observational study, in which patients with at least seven months of CSC underwent ophthalmologic examination, fundus photography, FAF, NIA, fluorescein angiography (FA), and spectral-domain OCT. Results. Seventeen eyes of thirteen patients were included. The presentation features were a mottled hyperFAF in the detached area and areas with pigment mottling. NIA images showed areas of hyperNIA similar to FAF and localized areas of hypoNIA, which correlated with the points of leakage in the FA. OCT showed pigment epithelium detachment at the location of these hypoNIA spots. Discussion. FAF showed increased presence of fluorophores in the area of retinal detachment, which is believed to appear secondary to lipofuscin accumulation in the RPE or the presence of debris in the subretinal fluid. NIA has been related to the choroidal melanin content and there were areas of both increased and decreased NIA, which could be explained by damage ahead the retina, basically RPE and choroid. These findings, along with the PEDs found in the areas of hypoNIA, support the notion of a primary choroidal disease in CSC.

Relative structural and functional peripapillary sparing is present with more widespread STGD1 disease. Photoreceptor degeneration may precede RPE atrophy in STGD1 pathogenesis.

Purpose.

To quantify and compare structure and function across the macula and peripapillary area in Stargardt disease (STGD1).

Methods.

Twenty-seven patients (27 eyes) and 12 age-similar controls (12 eyes) were studied. Patients were classified on the basis of full-field electroretinogram (ERG) results. Fundus autofluorescence (FAF) and spectral domain-optical coherence tomography (SD-OCT) horizontal line scans were obtained through the fovea and peripapillary area. The thicknesses of the outer nuclear layer plus outer plexiform layer (ONL+), outer segment (OS), and retinal pigment epithelium (RPE) were measured through the fovea, and peripapillary areas from 1° to 4° temporal to the optic disc edge using a computer-aided, manual segmentation technique. Visual sensitivities in the central 10° were assessed using microperimetry and related to retinal layer thicknesses.

Results.

Compared to the central macula, the differences between controls and patients in ONL+, OS, and RPE layer thicknesses were less in the nasal and temporal macula. Relative sparing of the ONL+ and/or OS layers was detected in the nasal (i.e., peripapillary) macula in 8 of 13 patients with extramacular disease on FAF; relative functional sparing was also detected in this subgroup. All 14 patients with disease confined to the central macula, as detected on FAF, showed ONL+ and OS layer thinning in regions of normal RPE thickness.

Conclusions.

Relative peripapillary sparing was detected in STGD1 patients with extramacular disease on FAF. Photoreceptor thinning may precede RPE degeneration in STGD1.

Purpose

To determine whether fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT) imaging allows better assessment of RPE and outer retina (OR) in subjects with chronic VKH compared to examination and angiography alone.

Methods

Cross-sectional analysis of a series of seven consecutive patients with chronic VKH undergoing FAF and SD-OCT. Chronic VKH was defined as during >3 months. Color fundus photographs were correlated to FAF and SD-OCT images. The images were later correlated to fluorescein angiography (FA) and indocyanine green angiography (ICG-A).

Results

All patients had sunset glow fundus, which resulted in no apparent corresponding abnormality on FAF or SD-OCT. Lesions with decreased autofluorescence signal were observed in 11 eyes (85%), being associated with loss of the RPE and involvement of OR on SD-OCT. In 5 eyes (38%) some of these lesions were very subtle on clinical examination but easily detected by FAF. Lesions with increased autofluorescence signal were seen in 8 eyes (61.5%), showing variable involvement of the OR on SD-OCT and corresponding clinically to areas of RPE proliferation and cystoid macular edema.

Conclusion

Combined use of FAF and SD-OCT imaging allowed noninvasive delineation of RPE/OR changes in patients with chronic VKH, which were consistent with previous histopathological reports. Some of these changes were not apparent on clinical examination.

The aim of this study is to investigate fundus autofluorescence (FAF) as well as fluorescein angiography (FA), indocyanine green angiography (IA), and optical coherence tomography (OCT) in a patient with pigmented paravenous chorioretinal atrophy (PPCRA). A funduscopic examination revealed chorioretinal atrophy along the paravenous area in both eyes. A marked bone spicule pigment clumping together with the atrophy was noted left eye. FA and IA showed a window defect and hypofluorescence, respectively, which exclusively corresponds to the atrophic area along the retinal vein area and the optic disc both eyes. FAF revealed geographic hypofluorescence along the paravenous and supranasal retinal areas. Hyperfluorescence was noted, which comparatively surrounded the hypofluorescence in the peripheral paravenous distribution. Hypofluorescence detected by FAF corresponded to the areas of retinal thinning and atrophy detected by OCT and FA. FAF is a useful examination in PPCRA, which can noninvasively demonstrate the distribution of deficit and dysfunction of retinal pigment epithelium.

Objective

This study aims to describe changes in high-resolution spectral domain optical coherence tomography (SD-OCT) scans with simultaneous fundus autoflorescence (FAF) signals in tubercular serpiginouslike choroiditis (SLC).

Methods

Simultaneous SD-OCT and FAF imaging of eyes affected with SLC from acute stage until resolution of lesions was obtained using Spectralis HRA+OCT system (Heidelberg Engineering, Heidelberg, Germany).

Patients

Four eyes (three patients) with SLC were prospectively followed.

Results

Acute lesions of SLC (diffusely hyperautofluorescent) corresponded to hyperreflective areas on SD-OCT involving the retinal pigment epithelium (RPE), photoreceptor outer segment tips (POST), inner segment–outer segment (IS/OS) junction, external limiting membrane (ELM), and outer nuclear layer (ONL) with a minimal distortion of inner retinal layers. There was no backscattering from inner choroid. During healing, lesions became discrete with a hypoautofluorescent border and predominant hyperautofluorescence centrally. The hyperreflective fuzzy areas on SD-OCT scans disappeared, and irregular, knobbly elevations of outer retinal layers appeared. The RPE, POST, IS/OS junction, and ELM could not be distinguished. The ONL appeared normal. The choroid showed an increased reflectance. As the lesions healed further over the next 3–6 months, they became predominantly hypoautofluorescent with loss of RPE, POST, IS/OS junction, and ELM in SD-OCT scan.

Conclusion

The SD-OCT provided an insight into the ultrastructural changes in the outer retina during the course of acute SLC lesions. The changes on OCT correlated with abnormal FAF findings.

Objective

This study aims to describe changes in high-resolution spectral domain optical coherence tomography (SD-OCT) scans with simultaneous fundus autoflorescence (FAF) signals in tubercular serpiginouslike choroiditis (SLC).

Methods

Simultaneous SD-OCT and FAF imaging of eyes affected with SLC from acute stage until resolution of lesions was obtained using Spectralis HRA+OCT system (Heidelberg Engineering, Heidelberg, Germany).

Patients

Four eyes (three patients) with SLC were prospectively followed.

Results

Acute lesions of SLC (diffusely hyperautofluorescent) corresponded to hyperreflective areas on SD-OCT involving the retinal pigment epithelium (RPE), photoreceptor outer segment tips (POST), inner segment–outer segment (IS/OS) junction, external limiting membrane (ELM), and outer nuclear layer (ONL) with a minimal distortion of inner retinal layers. There was no backscattering from inner choroid. During healing, lesions became discrete with a hypoautofluorescent border and predominant hyperautofluorescence centrally. The hyperreflective fuzzy areas on SD-OCT scans disappeared, and irregular, knobbly elevations of outer retinal layers appeared. The RPE, POST, IS/OS junction, and ELM could not be distinguished. The ONL appeared normal. The choroid showed an increased reflectance. As the lesions healed further over the next 3–6 months, they became predominantly hypoautofluorescent with loss of RPE, POST, IS/OS junction, and ELM in SD-OCT scan.

Conclusion

The SD-OCT provided an insight into the ultrastructural changes in the outer retina during the course of acute SLC lesions. The changes on OCT correlated with abnormal FAF findings.

Background

The purpose of this study was to evaluate the role of spectral domain optical coherence tomography (SD-OCT), MP-1 microperimetry, and fundus autofluorescence imaging for planning surgical procedures in combined hamartomas of the retina and retinal pigment epithelium (CHR-RPE) and following epiretinal membrane removal.

Methods

In an interventional retrospective case series, six consecutive subjects with CHR-RPE underwent vitrectomy and epiretinal membrane peeling, with 4 years of follow-up. Each underwent complete ophthalmic examination, including best corrected visual acuity, fundus examination, fundus fluorescein angiography, SD-OCT, MP-1, and fundus autofluorescence at one, 6, 12, and 48 months.

Results

Six eyes from six subjects with CHR-RPE were studied (mean age 31 ± 14 years). All patients were phakic and five were male (83.3%). Lesions were unilateral, ie, three macular, two juxtapapillary and macular, and one pericentral. Preoperative best corrected visual acuity was 0.3 ± 0.08 Snellen, with significant improvement to 0.9 ± 0.17 Snellen (P = 0.001) at 4 years of follow-up. Mean retinal sensitivity within the central 20° field improved from 16.6 ± 1.84 dB to 18.8 ± 0.96 dB (P = 0.07). There was also a statistically significant reduction in the visual defect (P = 0.04). SD-OCT demonstrated that the epiretinal membranes were completely removed in all but one patient, with significantly decreased macular edema on follow-up at one, 6, 12, and 48 months (P = 0.001). A positive correlation was shown between preoperative macular sensitivity and postoperative best corrected visual acuity. Fundus autofluorescence demonstrated a block in background autofluorescence at the site of the lesion, and hyperautofluorescence at the edematous retina overlain by the epiretinal membrane.

Conclusion

Surgery is an effective treatment for CHR-RPE. SD-OCT, fundus autofluorescence, and MP-1 are valuable and noninvasive tools to guide surgical procedures for CHR-RPE. To the best of our knowledge, this study represents the first use of MP-1 in CHR-RPE in conjunction with SD-OCT and fundus autofluorescence imaging for better guided surgery as well as anatomical and functional prognosis.

Purpose

To correlate the clinical and histopathologic features of Best vitelliform macular dystrophy (BVMD).

Methods

Two eyes were obtained postmortem from a patient with BVMD. The patient’s clinical information was reviewed. Series sections of the globes were performed and sequentially stained with hematoxylin-eosin, periodic acid-Schiff or Masson trichrome. A section of the left macula was submitted for electron microscopic processing. Histopathologic findings were reconstructed in a scaled two-dimensional map and compared with fundus photography, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA) and optical coherence tomography (OCT) images.

Results

The macular lesion of the right eye was identified as a well-demarcated region with pigment, elevated submacular yellow material and subretinal fluid. This corresponded histopathologically to a well-circumscribed area of RPE hyperplasia, accumulation of lipofuscin in the RPE, deposition of granular material in the photoreceptors, macrophages and drusen. The left eye displayed a 1 disc diameter chorioretinal scar with surrounding shallow fluid and submacular pigment. This corresponded to RPE changes and a fibrocellular proliferation in the choriocapillaris.

Conclusion

Histopathologic mapping revealed retinal edema, RPE abnormalities, drusen and a chorioretinal scar in BVMD that correlated with the fundus, FFA, FAF and OCT findings.

Purpose

To describe fundus autofluorescence (FAF) and optical coherence tomography (OCT) in a series of patients with congenital grouped albinotic spots (CGAS).

Methods

Three eyes of three patients with CGAS were evaluated with FAF and OCT imaging to evaluate the nature of the albinotic spots.

Results

In all three eyes with CGAS, FAF imaging revealed autofluorescent spots corresponding to the albinotic spots seen on stereo biomicroscopy. One eye also had additional spots detected on FAF imaging that were not visible on stereo biomicroscopy or color fundus photographs. FAF imaging of the spots demonstrated decreased general autofluorescence as well as decreased peripheral autofluorescence surrounding central areas of retained or increased autofluorescence. OCT revealed a disruption in signal from the hyper-reflective layer corresponding to the photoreceptor inner and outer segment junction as well as increased signal backscattering from the choroid in the area of the spots. Fluorescein angiography (FA) demonstrated early and stable hyperfluorescence of the spots without leakage.

Conclusion

In this case series, FAF demonstrated decreased autofluorescence of the spots consistent with focal RPE atrophy or abnormal material blocking normal autofluorescence as well as areas of increased autofluorescence suggesting RPE dysfunction. OCT and FA findings suggest photoreceptor and RPE layer abnormalities. FAF and OCT are useful noninvasive diagnostic adjuncts that can aid in the diagnosis of GCAS, help determine extent of disease, and contribute to our understanding of its pathophysiology.

Purpose

To report cases of spontaneous retinal pigment epithelial (RPE) tear complicating serous pigment epithelial detachment (PED).

Methods

The records of 3 Asian patients with spontaneous giant RPE tear were reviewed retrospectively by including clinical presentation, angiography, optical coherence tomography, fundus autofluorescence imaging, and visual outcome.

Results

Three patients (4 eyes) were included in this study, with a mean age of 48.3 (42–56) years, and a mean follow-up period of 7.75 (4–18) months. Fundus examination in all patients showed giant RPE tear associated with bullous PED. Two cases had a history of prior corticosteroid use, and 1 had no history of medication use. All 3 patients developed spontaneous resolution of subretinal fluid with no treatment. However, in patients who used corticosteroids, initial progression of the tear and subretinal fluid were observed despite ceasing medication. On subsequent follow-up, an incomplete RPE regeneration was demonstrated by fundus autofluorescence imaging, and choroidal neovascularization developed in 1 patient.

Conclusion

Large PED with RPE tear is a rare manifestation. When the fovea is spared, visual prognosis is favorable. No specific treatment is required, but careful choroidal neovascularization monitoring should be performed.

Purpose

To improve our understanding of Stargardt disease by comparing structural changes seen on spectral domain optical coherence tomography (SD-OCT) to those visible on fundus autofluorescence (FAF).

Methods

FAF and SD-OCT were obtained on 22 eyes of 11 patients with Stargardt disease. SD-OCT images were obtained at the fovea and at the eccentric preferred retinal locus (PRL). The diameters of “absent” (hypo-autofluorescent) and “abnormal” FAF areas were measured. The extent of the transverse defect of the junction between the inner and outer segments of the photoreceptors (IS-OS) was measured in the foveal area. The PRL was evaluated with fundus photography and microperimetry.

Results

Twenty-one of 22 eyes showed defective FAF. For 17 eyes, FAF was absent in the fovea and for 4 eyes the FAF was abnormal. All eyes showed disorganization and/or loss of the IS-OS junction in the foveal area on SD-OCT. The diameter of the absent FAF area was smaller than the measurement of the IS-OS junction loss; the latter was closer to the diameter of the abnormal FAF area. Seventeen eyes had an eccentric PRL associated with a retinal area with no defects on FAF.

Conclusions

For the majority of eyes changes on SD-OCT correlated well with changes on FAF. However for 3 patients, photoreceptor abnormalities were seen in the fovea on SD-OCT without an equivalent abnormality on FAF. This suggests that for these patients, the structural integrity of the photoreceptors may be affected earlier than changes in the RPE at least as detected by FAF.

Purpose

To evaluate the long-term outcome of autologous graft of retinal pigment epithelium (RPE) in patients with geographic atrophy.

Methods

Ten patients with progressive geographic atrophy underwent translocation of an autologous graft of RPE, Bruch membrane and choroid. The visual acuity (VA), reading performance, microperimetry, optical coherence tomography (OCT), fundus autofluorescence, fluorescein angiography and indocyanine green angiography were assessed.

Results

No recurrence of RPE atrophy was seen. All but one transplant were revascularised. Vascularisation persisted throughout the 3 years' follow-up. Spectral-domain OCT in some cases showed intact photoreceptors or intact outer nuclear and outer plexiform layer overlying the graft. In three cases, the grafts were positioned eccentrically; these patients did not benefit from surgery. The mean VA decreased from 20/80 (range: 20/800 to 20/40) at baseline to 20/200 (range: perception of hand movements to 20/32) at last follow-up. In two patients, VA were stable from 20/50 to 20/32 and 20/40 at the last follow-up, respectively. Postoperative complications included retinal detachment due to proliferative vitreoretinopathy, macular pucker, iritis, branch retinal vein occlusion and secondary ocular hypertension.

Conclusions

Some patients benefit for at least 3 years from a functioning RPE-choroid graft. Functional outcome in most patients, however, was limited due to complications and unfavourable patient selection.

A 41 year-old female patient with acquired immune deficiency syndrome (AIDS) presented with progressive nasal visual field loss in her right eye. Ophthalmic exam revealed widespread areas of retinal opacification with hemorrhage consistent with progressive outer retinal necrosis (PORN), which was confirmed by polymerase chain reaction (PCR) for varicella zoster virus (VZV) DNA. The patient was treated with intravenous and intravitreal foscarnet and ganciclovir with a resultant improvement clinically. Optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging revealed progressive changes indicative of widespread retinal pigment epithelial (RPE) and outer retinal dysfunction. OCT was useful in documenting progressive changes in macular architecture during therapy including neurosensory elevation, cystoid macular edema, and severe outer retinal necrosis, at initial exam, 1 week, and 1 month follow-up. Fundus autofluorescence revealed areas of stippled, hyperfluorescence within extensive zones of hypofluorescence, which progressed during the follow-up period. These areas appeared to represent lipofuscin or its photoreactive components within larger regions of RPE loss. The combination of OCT and FAF was useful in the characterization of the RPE and retinal anatomy in this patient with PORN.

Purpose. To describe the findings of fundus autofluorescence (FAF) and optical coherence tomography (OCT) in patients with branch retinal vein occlusion (BRVO). Methods. In this institutional, retrospective, observational case series, FAF was evaluated in 65 eyes with BRVO in 64 consecutive patients and compared with visual acuity, OCT findings, and other clinical observations. Results. Five types of autofluorescence appeared during the course of BRVO: (1) petaloid-shaped hyperautofluorescence in the area of macular edema and (2) hyperautofluorescence coincident with yellow subretinal deposits. (3) Diffuse hyperautofluorescence appeared within the area of serous retinal detachment (SRD) and OCT showed precipitates on the undersurface of the retina in 5/5 of these eyes (100%). (4) The area of vein occlusion showed diffuse hyperautofluorescence after resolution of the retinal bleeding. (5) Hard exudates exhibited hyper- or hypoautofluorescence. OCT indicated that most of the hard exudates with hyperautofluorescence were located on the retinal pigment epithelium. Conclusions. Hyperautofluorescence associated with subretinal fluid or hard exudate appeared in the subretinal space. This type of hyperautofluorescence may be attributed to blood cell or macrophages. FAF and OCT are noninvasive modalities that provide additional information regarding macular edema due to BRVO.

Purpose

To evaluate peripapillary acute zonal occult outer retinopathy in a 67-year-old man.

Methods

Images were obtained using fundus photography, fluorescein angiography (FA) and indocyanine green angiography (ICGA), fundus autofluorescence (FAF) imaging, and optical coherence tomography (OCT). Visual field testing and multifocal electroretinogram (mfERG) were also performed to evaluate retinal function.

Results

Fundus photographs showed subtle pigmentary changes in the peripapillary region, while FAF imaging showed clearly defined hypofluorescent areas in the peripapillary region. Intense hyperfluorescent lesions were also seen underneath hypofluorescent areas. A transmission defect with a granular hyperfluorescence was visible on FA, and ICGA showed hypofluorescence within the lesion. The outer border of the peripapillary zone appeared to block the underlying choroidal fluorescence. The photoreceptor inner segment/outer segment line was absent on OCT images from this area in both eyes, and hyperreflective punctate drusen-like materials were present at the outer border of the peripapillary zone. Visual field testing by Goldmann perimetry showed blind-spot enlargement, and mfERG showed corresponding amplitude reductions.

Conclusions

We speculate that the intense hyperautofluorescent material at the outer border of the peripapillary zone might be accumulated drusenoid lipofuscin.

Purpose

Punctate inner choroidopathy (PIC) is an ocular inflammatory disease. Spectral domain optical coherence tomography (SD-OCT) allows detailed visualization of retinal and choroidal structures. We aimed to describe the retinal changes on SD-OCT associated with PIC lesions localized in the macula.

Methods

Retrospective case series: PIC lesions not associated with choroidal neovascularization (CNV) and captured by macular SD-OCT scans were identified and characterized.

Results

Twenty-seven PIC lesions from seven patients (eight eyes) were identified and classified into four categories according to disease activity and temporal changes. Among clinically inactive patients, two main patterns were noted on OCT: (1) retinal pigment epithelium (RPE) elevation with sub-RPE hyper-reflective signals and (2) localized disruption of outer retinal layers with choroid and Bruch's membrane (BM) generally spared. Clinically active patients demonstrated lesions with intact BM with RPE elevation that fluctuated with disease activity and sub-RPE hyper-reflective signals. Photoreceptor-associated bands on SD-OCT (PRs) were not visible during active disease, but returned to normal visibility when lesions were clinically stable. Seven lesions in patients without clinically detected activity demonstrated alteration of RPE elevation.

Conclusion

SD-OCT can provide detailed structural characteristics of PIC lesions. RPE elevation is noted in many lesions while BM and choroid are spared. Photoreceptor-associated bands on SD-OCT appear compressed during clinically active stages and are visible during stabilization. OCT may provide information on activity not detected clinically.

Electronic supplementary material

The online version of this article (doi:10.1007/s12348-011-0054-6) contains supplementary material, which is available to authorized users.

AIM

To present retinal microstructure, metabolism and function abnormalities in the course of multiple evanescent white dot syndrome (MEWDS) by Heidelberg spectralis modality imaging platform and observe its outcome by EDI-SD-OCT and two wavelength autofluorescence.

METHODS

A case of multiple evanescent white dot syndrome in a 23-year-old female presented initially with a 15-day history of floaters and a central scotoma in the right eye. To establish the diagnosis, multimodality imaging was performed, namely, blue light-fundus autofluorescence (BL-FAF, excitation 488nm, emission >500nm), near-infrared fundus autofluorescence (NIR-FAF, excitation 787nm, emission >800nm) using a confocal scanning laser ophthalmoscope, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), spectrum-domain enhance depth imaging optical coherence tomography (SD-EDI-OCT), multifocal electroretinography (mf-ERG) and fundus photogragh were performed and followed up at the eighth month after initially visiting.

RESULTS

Optical coherence tomography (OCT) showed a transient disruption of the foveal photoreceptor outer segments in correspondence to foveal granularity. NIR-FAF showed hypoautofluorescent areas, ≤40µm in size, mostly concentrated around the posterior pole and its temporal side less than that in BL-FAF. Mf-ERG show pinnacle disappeared in fovea and macula and responses decreased markedly compared with the follow eye. At the eighth month follow up, hyperfluorescence in BL-FAF were disappear, while, NIR-FAF Hypofluorescent spots in early stage of such lesion were reduced. But OCT demonstrated the structure was recovered in residual Hypofluorescent area in NIR-FAF. The subfoveal choroidal thickness was decreased from 372µm to 307µm slightly and cost line was recovered.

CONCLUSION

MEWDS is a benign self-healing disease and there is no pathological evidence to investigate the natural course of such disease. SD-OCT allows highly detailed images approaching histopathology to certify the microstructural changes. Two-wave length FAF and mf-ERG provide more information about metabolism in outer retina especial RPE and photoreceptor. Spectralis OCT combined with two-wavelength FAF and mf-ERG provide a new way to analyze this disease and offer more details for therapy and follow-up.

AIM

To evaluate the role of central green-light fundus autofluorescence (FAF) in diabetic macular edema (DME).

METHODS

A consecutive series of 92 study eyes with diabetic retinopathy were included. Out of those, 51 diabetic eyes had DME and were compared to 41 diabetic eyes without DME. In all subjects, green-light FAF images were obtained, quantified and classified into various FAF patterns. Cross-sectional optical coherence tomography (OCT) scans were obtained for evaluation of Inner/Outer segment (IS/OS) layer integrity, measurements of central RPE-IS/OS layer thickness as well as classification of DME into various subtypes.

RESULTS

Mean central green-light FAF intensity of eyes with DME (1.289±0.140)log did not significantly differ from diabetic patients without DME (1.317±0.137)log. Most classifiable FAF patterns were seen in patients with cystoid DME. Mean central retinal thickness (CRT) of all study eyes with DME was (501.9±112.4)µm compared to (328.2±27.0)µm in diabetic patients without DME. Patients with DME had significantly more disrupted photoreceptor IS/OS layers than diabetic patients without DME (28/51 vs 5/41, P<0.001). Mean RPE-IS/OS thickness of patients with DME (60.7±14.1)µm was significantly (P<0.001) lower than in diabetic eyes without DME (73.5±9.4)µm. Correlation analys1s revealed non-significant correlations of green-light FAF intensity and OCT parameters in all subtypes of DME.

CONCLUSION

Our results indicate a poor correlation of central green-light FAF intensity with CRT, IS/OS layer integrity or RPE-IS/OS layer thickness in diabetic patients with or without DME and its various subtypes. Thus, central green-light FAF is not suitable for detection of retinal thickening in DME.

Lipofuscin results from digestion of photoreceptor outer segments by the retinal pigment epithelium (RPE) and is the principal compound that causes RPE fluorescence during autofluorescence imaging. Absorption of the 488-nanometer blue light by macular pigments, especially by the carotenoids lutein and zeaxanthin, causes normal macular hypo-autofluorescence. Fundus autofluorescence imaging is being increasingly employed in ophthalmic practice to diagnose and monitor patients with a variety of retinal disorders. In macular edema for example, areas of hyper-autofluorescence are usually present which are postulated to be due to dispersion of macular pigments by pockets of intraretinal fluid. For this reason, the masking effect of macular pigments is reduced and the natural autofluorescence of lipofuscin can be observed without interference. In cystic types of macular edema, e.g. cystoid macular edema due to retinal vein occlusion, diabetic macular edema and post cataract surgery, hyper-autofluorescent regions corresponding to cystic spaces of fluid accumulation can be identified. In addition, the amount of hyper-autofluorescence seems to correspond to the severity of edema. Hence, autofluorescence imaging, as a noninvasive technique, can provide valuable information on cystoid macular edema in terms of diagnosis, follow-up and efficacy of treatment.

Aims: To report the optical coherence tomographic (OCT) findings in retinal pigment epithelium (RPE) detachments.

Methods: 24 eyes were analysed by OCT and by fluorescein (FAG) and/or indocyanine green angiography.

Results: RPE detachments were classified by the OCT images into those with a partial or total highly reflective line in the area of the detachment (nine eyes), and those that showed the same reflex in the detached area as in other parts (15 eyes). The nine eyes had irregular hyperfluorescence by FAG, significantly larger detachments, and lower visual acuity than the 15 eyes.

Conclusion: The OCT showed that two types of RPE detachments are associated with eyes with different characteristics.

Background

To describe autofluorescence patterns of choroidal melanocytic lesions using the Heidelberg Retinal Angiograph 2 system (HRA2).

Methods

20 patients with choroidal melanocytic lesions in the ocular fundus underwent ophthalmologic examination, fundus photography, autofluorescence and optical coherence tomography (OCT). Pathologic examination was performed on one enucleated eye with a large choroidal melanoma.

Results

15 patients had choroidal nevi and 5 had malignant choroidal melanoma (1 small, 1 medium and 3 large tumours). Choroidal nevi did not show any characteristic autofluorescence pattern, although secondary retinal pigment epithelium (RPE) changes, such as drusen and pigment epithelium detachment, appeared faintly hyperautofluorescent in 2 patients. Only the small malignant choroidal melanomas had prominent orange pigmentation, although all melanomas had an intense confluent hyperautofluorescent signal over the lesions. Pathology of one large malignant melanoma revealed lipofuscin underlying RPE.

Conclusion

Most nevi did not have characteristic hyperautofluorescent features, but choroidal melanomas seemed to have a pattern of confluent hyperautofluorescence. Therefore, autofluorescence may be a useful non‐invasive tool to assess lipofuscin in pigmented choroidal lesions, which may contribute to the diagnosis of malignancy. This hypothesis, however, remains to be confirmed in large prospective studies.