Purpose. To report clinical aspects, tomographic, angiographic, and autofluorescence patterns of two cases of isolated foveal hypoplasia. Methods. Foveal hypoplasia was found in a 23-year-old male patient and in a 64-year-old woman with impaired visual acuity of unknown etiology that remained unchanged for years. Results. In the first case, spectral-domain optical coherence tomography (SD-OCT) showed reduced foveal pit and continuity of inner retinal layers in the fovea. Photoreceptor layer had a normal thickness centrally. The foveal avascular zone (FAZ) was absent in the flourescein angiogram (FA). Fundus autofluorescence showed reduced foveal attenuation of autofluorescence. In the second patient, there was the same pattern in SD-OCT, with normal aspect in FA and only a slightly reduced foveal attenuation of autofluorescence. Conclusion. OCT, as a noninvasive and quick method, is helpful in the diagnosis of foveal hypoplasia. FA and fundus autofluorescence were less sensitive.
This is a report of a case of choroidal excavation accompanied by polypoidal choroidal vasculopathy (PCV) and retinal pigment epithelium detachment (PED).
A 57-year-old Japanese woman who had begun complaining of metamorphopsia in her left eye 7 months earlier underwent spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and indocyanine green angiography (IA), as well as a routine ophthalmological examination.
The patient’s intraocular pressure, visual acuity, and visual field were within normal range. Ophthalmoscopy revealed a serous macular detachment, soft drusen, exudates, and a reddish-orange elevated lesion in the macula of the left eye. The right eye was normal. SD-OCT revealed two lesions in the left eye. One was a PED accompanied by a notch sign, and the other was a choroidal excavation. Additionally, FA revealed a window defect in the PED, and IA showed typical PCV. Three monthly injections of antivascular endothelial growth factor preserved visual acuity, but failed to have any visible effect on the lesion during the 6-month follow up period.
This is the first report of choroidal excavation accompanied by PED and PCV. The data suggest that choroidal excavation may be associated with various changes that have not been previously reported. Careful observation of such cases may therefore be necessary.
choroidal excavation; polypoidal choroidal vasculopathy; anti-vascular endothelial growth factor treatment
Dome-shaped macula (DSM) was described by Gaucher et al as a convex protrusion of macula within a staphyloma in highly myopic eyes that causes visual impairment associated with serous foveal detachment (SFD). We describe a patient with spontaneous resolution of SFD in DSM documented by serial spectral domain optical coherence tomography (SD-OCT). A 28-year-old female Japanese patient presented with blurred vision in both eyes. Upon examination, her best-corrected visual acuity was 1.2 with −8.0 sphere and 0.5 with −8.0 sphere in the right and left eyes, respectively. SD-OCT in both eyes showed an anterior bulge of the macula with SFD. The diagnosis of DSM with SFD was made on the basis of characteristic findings. Three months later, the patient achieved spontaneous resolution of SFD without treatment. One month later, there was an occurrence of SFD in the right eye, but the SFD had disappeared in 1 month. In our case, her visual impairment was proven to be a consequence of SFD in relation to DSM. SD-OCT was useful for the detection and follow-up of SFD in DSM.
dome-shaped macula; serial spectral domain optical coherence tomography; resolution
An 82-year-old female presented with sudden painless decrease in vision in the right eye after awakening. She could see the “superior half” of her vision from the right eye only. On examination, best-corrected vision was 20/300 in the right eye and 20/30 in the left eye. The fundus in the right eye revealed recent superotemporal branch retinal artery occlusion (BRAO) with calcified plaque at the disc. Spectral domain optical coherence tomography (OCT) (OTI Ophthalmic Technologies, Inc.), revealed hyperreflectivity and increased thickness of the inner retinal layers of the superior compared to the inferior retina. Imaging at the optic disc revealed the blocked artery containing a highly reflective material. The high reflectivity of the material and underlying optical shadowing could be characterized as calcific emboli.
Artery; optical coherence tomography; retina; retinal artery occlusion; spectral domain
To investigate spectral domain optical coherence tomography (SD-OCT) findings and compare them with time domain (TD)-OCT imaging of macula and retinochoroiditis lesions of patients with toxoplasmosis.
Prospective cross-sectional study.
Ten eyes of 10 patients with active toxoplasmic retinochoroiditis were included. Morphologic features from the macula and retinochoroiditis lesions were obtained at baseline and at 6-week follow up. Scan acquisition protocols for TD-OCT included raster and radial lines through the retinochoroiditis lesion, fast macular, and a linear scan from the lesion to the fovea, whereas the acquisition protocols for SD-OCT also included horizontal volume scans at the lesion site and at the macula. Thickness measurements obtained by SD-OCT were analyzed.
At baseline, macular serous retinal detachment was observed in five patients; two of them only seen by SD-OCT. Retinochoroidal lesions were 4260 μm distant from the fovea on average (R = 681–7130) and this distance had an indirect correlation to the presence of macular detachment. Epiretinal membrane and vitreo-macular traction were also observed. The posterior hyaloid was not identified in four patients by TD-OCT and only in one by SD-OCT at baseline. Perilesional subretinal fluid was observed in two patients. The median retinal thickness significantly decreased at the retinochoroiditis lesion (P = 0.0004), and all the patients remained with disorganized retinal layers reflectivity at follow up.
SD-OCT is a useful tool in the diagnosis of macular changes related with toxoplasmic retinochoroiditis. SD-OCT is superior in evaluating retinal changes associated with ocular toxoplasmosis.
toxoplasmosis; retinochoroiditis; optical coherence tomography
Central serous chorioretinopathy (CSCR) manifests as neurosensory detachment of the macula and can be attributed to focal or multifocal leakage in the retinal pigment epithelium (RPE). Fibrin accumulation in the subretinal space is an unusual and heretofore unreported visually damaging manifestation of severe CSCR.
The patient was followed up with the use of biomicroscopy, fluorescein angiography, and optical coherence tomography (OCT).
A 32-year-old woman was referred to our department complaining of metamorphopsia and decreased visual acuity in the right eye. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/20 in the left eye. Biomicroscopy revealed an irregularly shaped foveal elevation and wrinkling in the right eye. OCT showed a steep neurosensory retina elevation with a highly reflective material accumulation in the subretinal space, presumably fibrin. Our diagnosis was CSCR complicated by subretinal fibrin accumulation. Since most of these cases resolve spontaneously, the patient was kept under observation; 1 month later, the fibrin accumulation had expanded subfoveally (BCVA 20/200). The patient was offered 3 intravitreal ranibizumab injections. After the initial injection, BCVA improved to 20/50 and, after the 3 injections, to 20/30. Two months later (BCVA 20/30), fresh leakage was observed at the margin of the original lesion, and an additional intravitreal ranibizumab injection was performed. After another 2 months, BCVA stabilized at 20/25 and remained stable throughout the 12 months after the initial injection.
Prompt recognition of CSCR complicated by subretinal fibrin and immediate intervention may result in recovery from this potentially devastating complication. Ranibizumab may be an alternative treatment option in the management of refractory CSCR complicated by subretinal fibrin accumulation.
Central serous chorioretinopathy; Fibrin; Ranibizumab; Optical coherence tomography
We report a case of cat-scratch disease with unusual posterior segment manifestations. A 12-year-old healthy male presented with three weeks history of decreased visual acuity in the right eye. A significant history of cat exposure and elevated Bartonella titers were present. A large white-gray vascularized mass extending off the optic disk, an early stellate maculopathy, a plaque of choroiditis, an inferior serous retinal detachment involving the macula were present in the right eye. Sector papillitis and a focal area of chorioretinitis along the superotemporal arcade with associated retinal artery to vein anastomosis were present in the left eye. Bilateral optic nerve head involvement including peripapillary angiomatosis, retinal–retinal anastomosis and plaque choroiditis as ocular complications of cat-scratch disease have not been previously described to our knowledge and make this case noteworthy.
Cat-Scratch Disease; Choroiditis; Inflammatory Optic Nerve Head Mass; Retinal-Retinal Anastomosis
A 32-year-old Afro-Caribbean male presented with a 4 month history of blurred vision and distortion in his right eye. Fundus examination showed multiple pigment epithelial detachments which progressed over 2 months of observation to a large serous detachment of the macula. Fundus fluorescein angiography (FFA) showed multi-focal hyperfluorescence in the early phase which increased in the later stages. A diagnosis of multi-focal central serous retinopathy (CSR) was made. Due to the size of CSR and atypical findings on FFA further investigations including a T-spot test were performed. The T-spot test was reported as strongly positive and following discussion with the respiratory physicians he was started on empiric antituberculous therapy. Over the next 6 weeks, the patient had a significant visual improvement to 6/9 with resolution of the serous detachment.
A 54-year-old man presented with blurred central vision in the right eye of two weeks' duration. On presentation, visual acuity was 40 / 50 in the right eye and fundus examination showed a whitish-yellow inflammatory lesion near an atrophic, pigmented retinochoroidal scar located in the superotemporal quadrant. Serologic assessment was negative for IgM, but serum IgG to toxoplasma was elevated. Spectral domain optical coherence tomography (SD-OCT) revealed increased reflectivity from the inner retinal layer, retinal thickening, and choroidal shadowing while focal posterior hyaloid thickening and detachment were observed in the new lesion. He was treated with trimethoprim/sulfamethoxazole, clindamycin, and prednisone. SD-OCT is helpful for definitively differentiating ocular toxoplasmosis from other retinal diseases.
Ocular toxoplasmosis; Retinochoroiditis; Spectral domain optical coherence tomography
To investigate changes in cone photoreceptors with adaptive optics (AO) fundus imaging and spectral domain optical coherence tomography (SD-OCT) in a case of occult macular dystrophy (OMD).
Patient and methods
Both eyes of a 42-year-old woman diagnosed with OMD were examined. We used an AO fundus camera to obtain images of cone photoreceptors in the macula of the OMD subject and five healthy control subjects. Correlations between the AO images and the SD-OCT images were examined. Cone photoreceptors in eight areas in the macula of OMD and healthy control subjects were analyzed and compared.
SD-OCT showed a loss of the cone outer-segment tips line outside of the fovea in both eyes of the subject with OMD. The left eye with decreased visual acuity showed a discontinuous photoreceptor inner-segment and outer-segment line and cone outer-segment tips line at the fovea in SD-OCT and loss of cone mosaics as a dark spot in the AO image. In panoramic AO images and cone-density maps, less cone density was observed in a ring-like region outside the fovea than in the peripheral retina. In most of the areas examined, the cone densities were lower in the OMD eyes than in the healthy control eyes.
Cone densities in the macula of the OMD patient were greatly decreased. AO images were found to be useful to evaluate morphologic changes in cone photoreceptors in patients with OMD.
occult macular dystrophy; adaptive optics; cone photoreceptor; cone analysis; optical coherence tomography
To characterize functional and anatomic sequelae of a bleb induced by subretinal injection.
Subretinal injections (100 μl) of balance salt solution (BSS) were placed in the superotemporal macula of one eye in 3 cynomolgus macaques. Fellow eyes received intravitreal injections (100 μl) of BSS. Fundus photography, ocular coherence tomography (OCT) and multifocal electroretinography (mfERG) were obtained before and immediately after injection and again at intervals up to 3 months post injection. Histopathologic analyses included transmission electron microscopy (TEM) and immunohistochemistry (IHC) for glial fibrillary acidic protein (GFAP), rhodopsin, M/L-cone opsin and S-cone opsin.
Retinas were re-attached by 2 days post-injection (by OCT). mfERG was suppressed post-subretinal injection within the subretinal injection bleb and surprisingly, also in regions far peripheral to this region. mfERG amplitudes were nearly completely recovered by 90 days. The spectral domain (SD)-OCT inner segment/outer segment (IS/OS) line had decreased reflectivity at 92 days. GFAP and S-cone staining were unaffected. Rhodopsin and M/L-cone opsins were partially displaced into the inner segments. TEM revealed disorganization of the outer segment rod (but not cone) disks. At all post-injection intervals, eyes with intravitreal injection were similar to baseline.
Subretinal injection is a promising route for drug delivery to the eye. Three months post subretinal injection, retinal function was nearly recovered, although reorganization of the outer segment rod disk remained disrupted. Understanding the functional and anatomic effects of subretinal injection per se is important for interpretation of the effects of compounds delivered to the subretinal space.
Subretinal injection is a new potential route for drug delivery to the eye. Separating drug effects from the procedural effects per se is critical.
This study aims to compare the topographic distribution of retinal pigment epithelium detachment (PED) in central serous chorioretinopathy (CSC) on spectral domain optical coherence tomography (SD-OCT) single-layer retinal pigment epithelium (SL-RPE) map and fluorescein angiography for the first time.
Twenty-seven eyes of CSC with PEDs were studied retrospectively. Topographic distribution of PEDs was documented on 50° fluorescein angiography and SD-OCT SL-RPE map on macular cube 512 × 128.
Quantity of PEDs ranged from 1 to 5 on SD-OCT SL-RPE map macular cube 512 × 128 and corresponding macular cube area on 50° fluorescein angiography. Topographic distribution of PEDs on SL-RPE map matched with PEDs on fluorescein angiography in the corresponding macular cube area, in all the cases (interobserver correlation = 0.9).
SD-OCT SL-RPE map is a noninvasive, three-dimensional advanced tool for documentation of topographic assessment of PEDs and obviates the need of fluorescein angiography for monitoring CSC.
Central serous chorioretinopathy; Spectral domain optical coherence tomography; Fluorescein angiography; Single-layer retinal pigment epithelium map; Pigment epithelium detachment
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).
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.
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.
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.
To describe a case of microcystoid maculopathy diagnosed by high-resolution Fourier-domain optical coherence tomography (Fd-OCT) in a patient treated with tamoxifen who had previously unexplained vision loss.
Report of a case of a 66-year-old woman treated with tamoxifen for 4 years who had progressive unexplained vision loss in the left eye for 2 years.
Visual acuity was 20/25 in the right eye and 20/70 in the left eye. Findings of funduscopy and fluorescein angiography were unremarkable except for minimal pigment alteration in the macula in the left eye. Stratus OCT showed possible microcystoid changes in the fovea in both eyes. Fd-OCT revealed scattered intraretinal microcystoid changes and patches of focal loss of photoreceptors in the macula in both eyes with foveal involvement in the left eye. Full-field electroretinogram was normal. Multifocal electroretinogram showed some decreased response in the peripheral macula and borderline low foveal response in both eyes.
Microcystoid maculopathy associated with tamoxifen use can be diagnosed by Fd-OCT in a normal appearing fundus. It can present as unexplained vision loss.
To assess photoreceptor (PR) layer morphology in patients with Stargardt’s disease (STGD) and fundus flavimaculatus (FFM) using high resolution spectral domain optical coherence tomography (HD-OCT; OCT 4000 Cirrus, Humphrey-Zeiss, San Leandro, CA).
This was a prospective observational case series. Sixteen consecutive patients with STGD and FFM underwent a complete ophthalmologic examination. Optical coherence tomography examination was performed with HD-OCT, a high-speed (27,000 axial scans per second) OCT system using spectral/Fourier domain detection, with an axial image resolution of 5 μm.
A total of 31 eyes were included in the study. Transverse loss of the PR layer in the foveal region was shown by HD-OCT. Twenty eyes with clinically evident central atrophy had a disruption of either the Verhoeff‘s membrane (VM) or the layer corresponding to the interface of inner segment (IS) and outer segment (OS) of PR in the foveal region. Among these eyes, 12/20 eyes had a loss of the PR layer (loss of both VM and IS-OS interface) in the foveal region. Eleven eyes (11/31) without clinically evident central atrophy had an intact interface of IS and OS of PR centrally. Moreover, we observed hyperreflective deposits: type 1 lesions located within the retinal pigment epithelium (RPE) layer and at the level of the outer segments of PR, and type 2 lesions located at the level of the outer nuclear layer and clearly separated from the RPE layer. Type 1 lesions alone were associated with absence of loss of the PR layer in the foveal region in all eyes; type 2 lesions were always associated with presence of type 1 lesions, and often (8/12 eyes) associated with loss of the PR layer within the foveal region. Mean best-corrected visual acuity (BCVA) was significantly correlated with loss of the PR layer in the foveal region (P < 0.001), as well as to presence of type 2 flecks (P = 0.03).
Type 2 deposits in STGD/FFM patients seem to represent a marker of the possible evolution towards foveal atrophy.
fundus flavimaculatus; high definition optical coherence tomography; retinal dystrophy; stargardt’s disease
Circumscribed choroidal hemangiomas are vascular tumors associated with secondary changes in the overlying retinal pigment epithelium and neuro-sensory retina. Spectral-domain optical coherence tomography, a recent advancement in fundus imaging techniques provides high resolution images of the retina. We describe spectral domain Optical coherence tomography findings in a case of circumscribed choroidal hemangioma which was successfully treated with photodynamic therapy.
A 41-year-old white male presented with decreased vision in his right eye. Fundus evaluation showed findings consistent with circumscribed choroidal hemangioma. Spectral-domain optical coherence tomography revealed a large serous retinal detachment overlying the tumor with an intact photoreceptor layer. The patient underwent photodynamic therapy and a repeat tomography scan confirmed the resolution of serous detachment with return of normal foveal contour.
Spectral domain optical coherence tomography is an emerging modality in imaging of the retina and reveals ultrastructural changes occurring in various retina pathologies. In this case report we illustrate the use of spectral domain optical coherence tomography for the first time to document retinal changes overlying a choroidal hemangioma and its role as a non-invasive tool in planning the treatment and prognosticating the final visual outcome following treatment for circumscribed subfoveal choroidal hemangiomas.
Purpose. Solar retinopathy is a rare clinical disturbance, for which spectral-domain optical coherence tomography (SD-OCT) findings are not always consistent. We report on two cases of solar retinopathy and discuss its differential diagnosis. Methods. This is an observational case study. Results. A 12-year-old female was referred to ophthalmology for bilateral scotoma. Visual acuity was 20/50 in both eyes. Fundus examination was unremarkable, except for slight yellowish material in the central macula, bilaterally. SD-OCT revealed juxtafoveal microcystic cavities in the outer retina, interruption of the external limiting membrane and the inner and outer segment junctions, with disorganized material in the vitelliform space. Fundus autofluorescence showed hypoautofluorescence surrounded by a relatively hyperautofluorescent ring, bilaterally. Similar clinical and morphological findings were detected in a 27-year-old male. Conclusions. Solar retinopathy has a subtle presentation and patients often deny sun-gazing. SD-OCT and fundus autofluorescence are noninvasive and useful tools for its diagnosis.
The aim of this study was to compare the agreement between spectral domain optical coherence tomography (SD OCT) and time domain stratus OCT (TD OCT) in evaluating macular morphology alterations in wet age-related macular degeneration (AMD).
This retrospective study was performed on 77 eyes of 77 patients with primary or recurring subfoveal choroidal neovascularization secondary to AMD. All patients underwent OCT examination using Zeiss Stratus OCT 3 (Carl Zeiss Meditec Inc, Dublin, CA) and Opko OTI Spectral SLO/OCT (Ophthalmic Technologies Inc, Toronto, Canada). In all radial line scans, the presence of intraretinal edema (IRE), serous pigment epithelium detachment (sPED), neurosensory serous retinal detachment (NSRD), epiretinal membrane (EM), inner limiting membrane thickening (ILMT), and hard exudates (HE) were evaluated. The degree of matching was quantified by Kappa measure of agreement.
The percentage distribution of TD OCT findings versus SD OCT findings was: IRE 36.3% versus 77.9%, sPED 57.1% versus 85.7%, NSRD 38.9% versus 53.2%, EM 10.5% versus 26.3%, ILMT 3.8% versus 32.4%, and HE 6.4% versus 54.5%. The agreement was as follows: sPED: kappa value 0.15; NSRD: kappa value 0.61; IRE: kappa value 0.18; EM: kappa value 0.41; ILMT: kappa value 0.02; HE: kappa value 0.06.
The agreement in the evaluation of macular lesions between the two techniques is poor and depends on the lesion considered. SD OCT allows better detection of the alterations typically related to choroidal neovascularization such as IRE, PED, ILM thickening, and HE. Consequently its use should be strongly considered in patients with wet AMD.
spectral domain; OCT; time domain; macular degeneration; AMD
A congenital anomaly, optic nerve pit is often associated with serous retinal detachment involving macula. Long standing serous detachment leads to outer retinal atrophy and decrease in visual sensitivity. Recently, spectral-domain optical coherence tomography (OCT) has been reported to demonstrate a communication between the optic nerve sheath and the subretinal space. Vitreous cavity is proposed as an alternate source of fluid for accumulation in the subretinal space. We imaged a patient with optic nerve pit with Spectralis OCT and report the findings seen including the presence of an area of peripapapillary retinal atrophy, due to the spontaneous resolution of associated long-standing retinal detachment.
optic nerve pit; SD-OCT; autoflourescence; microperimetry
We report a 38-year-old man with a complaint of blurred vision in his right eye for the previous 5 days. He had bilateral optic disc drusen. Fluorescein angiography revealed multiple hyperfluorescent foci within temporal optic discs and temporal inferior arcade in late phase. Optical coherence tomography showed bilateral peripapillary serous detachment as well as right macular detachment. This is the first reported case of a concurrent peripapillary and macular detachment in a patient with central serous papillopathy by optic disc drusen. Central serous papillopathy is an atypical form of central serous chorioretinopathy that should be considered as a potential cause of acute loss of vision in patients with optic nerve head drusen.
central serous papillopathy; peripapillary central serous chorioretinopathy; optic nerve head drusen; peripapillary subretinal fluid
Aim. To report the optical coherence tomography (OCT) findings in three members of the same family with nanophthalmos associated with elevated papillomacular retinal fold. Methods. Complete ophthalmic examination as well as ultrasonography and OCT was performed in all patients. Results. Axial lengths ranged from 16.75 mm to 17.48 mm and refractive errors ranged from +17.50 D to +20.50 D. Main fundus findings were the hyperopic crowded, cupless optic disc, and retinal fold through papillomacular region. Macular OCT scans revealed retinal fold with normal retinal pigment epithelium and choriocapillaris. Interpretation. It is presumed that the retinal folds in nanophthalmos result from a redundancy of the retinal layer caused by retarded growth of the scleral, choroidal, and retinal pigment epithelial layers. The anatomic information provided by the current study is consistent with this thesis.
To report the effects of aflibercept on eyes with large retinal pigment epithelial detachment (PED) associted with polypoidal choroidal vasculopathy (PCV).
We reviewed the medical records of patients with PEDs associated with PCV that were treated with aflibercept after intravitreal ranibizumab had failed.
Three eyes of patients aged 72, 79, and 80 years were studied. Reflective material was seen in the PED along the outer surface of the retinal pigment epithelium (RPE) by spectral-domain optical coherence tomography (SD-OCT). A complete resolution of the serous PEDs was found after two aflibercept injections; however, all eyes had a fibrovascular PED. In addition, one eye developed a retinal hemorrhage and a recurrent PED just after the third injection of aflibercept. The visual acuity in this eye decreased from 10/20 to 2/20.
The reflective material below the outer surface of the RPE in serous PED suggests the presence of neovascularization. Intravitreal aflibercept could be considered for large PEDs in eyes with PCV but should be carefully applied.
age-related macular degeneration; retinal pigment epithelial detachment; polypoidal choroidal vasculopathy; aflibercept
To correlate the clinical and histopathologic features of Best vitelliform macular dystrophy (BVMD).
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.
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.
Histopathologic mapping revealed retinal edema, RPE abnormalities, drusen and a chorioretinal scar in BVMD that correlated with the fundus, FFA, FAF and OCT findings.
Best vitelliform macular dystrophy; Optical coherence tomography; Fundus autofluorescence; Fundus fluorescein angiography; Two-dimensional reconstruction; Clinicopathologic correlation
The aim of this study is to report a patient with multipe evanescent white dot syndrome (MEWDS) presenting with classic foveal granularity and pathology localized to the outer retina.
Case study methodology was used in the current study.
A 34-year-old Caucasian female presented with photopsias and blurry vision in her left eye. Examination, particularly the foveal granularity noted in her affected eye, was archetypal for the diagnosis of MEWDS. Fundus autofluorescence, fluorescein and indocyanine green angiography were also consistent with this diagnosis. Spectral-domain optical coherence tomography (SD-OCT) demonstrated increased retinal pigment epithelium granularity and disruption of the photoreceptor inner segment–outer segment junction subfoveally.
Foveal granularity may be the most specific feature of MEWDS with SD-OCT capable of localizing pathology to the outer retina—a historically controversial finding.
MEWDS; Inflammation; Spectral-domain OCT; White dot syndrome; Medicine & Public Health; Ophthalmology
To study tissue remodelling and wound healing after retinal pigment epithelium (RPE) tears due to age-related macular degeneration.
Retrospective longitudinal study of 36 eyes (33 patients) with RPE tears. Imaging was performed using fundus autofluorescence (FAF) (λ=488 nm) and spectral-domain optical coherence tomography (SD-OCT). Presence of intraretinal hyper-reflective dots in SD-OCT, which correlated with hyperfluorescent dots in FAF, indicating RPE migration was studied. Morphology of subretinal mass and RPE layer integrity in the RPE denuded area over time were examined.
7 of 36 eyes (19.4%) showed patchy or hazy hyperfluorescent areas in FAF, and the majority of eyes (83.3%) showed hyper-reflective dots, which possibly represent intraretinal RPE migration and hard exudates. Homogenous subretinal mass was encountered in about half of all cases. In one case (2.8%), the RPE layer proliferated and covered the defect.
SD-OCT and FAF showed a considerable amount of RPE proliferation, migration and repopulation. Intraretinal RPE migration did not form a functional RPE layer. A small defect might be repaired by cell proliferation. But this RPE proliferation is not sufficient to cover large defects.
Retinal pigment epithelium tear; pigment migration; spectral-domain optical coherence tomography; fundus autofluorescence; macula; pharmacology; neovascularisation; drugs; clinical trial; treatment surgery