To characterize perception of Haidinger’s brushes (HB) in individuals with albinism, in whom reduced macular pigment and abnormal foveal/Henle-fiber morphology may alter polarization sensitivity. Three participants with oculocutaneous albinism (aged 19–29 years; two with nystagmus) viewed 468-nm polarized/unpolarized LED stimuli in a custom polarimetry device. Six randomized conditions varied polarization (0
The photopic negative response (PhNR) of the full-field electroretinogram reflects retinal ganglion cell (RGC) function and is reduced in glaucoma, yet no quantitative synthesis of PhNR amplitude data across glaucoma studies exists. PubMed, Embase, Web of Science, Scopus, and Cochrane Central were searched from inception to February 2025 for studies reporting full-field PhNR amplitude in glaucoma patients versus healthy controls. Random-effects meta-analysis (DerSimonian-Laird) was performed. Subgroup analyses examined glaucoma subtype (POAG, NTG, OHT/suspect), disease severity, and recording methodology. Meta-regression assessed intraocular pressure (IOP) as a moderator. Publication bias was evaluated by Egger and Begg tests. Quality was assessed using the Newcastle–Ottawa Scale. Twenty-eight studies (2847 eyes: 1624 glaucoma, 1223 controls) met inclusion criteria. Glaucoma patients demonstrated significantly reduced PhNR amplitude versus controls (pooled standardized mean difference [SMD] = − 37.82 μV, 95
To study the interaction between responses to a sinusoidal modulation and a flash in the mouse ERG. We recorded responses to pulses on a 1 Hz sinewave background (Pulse on Sine Stimuli: PoS) in mice. Flashes were presented at 8 different temporal phases relative to the sinewave background. Recordings were performed for three different luminance levels with equal ratio between flash intensity and sinewave mean luminance. Control recordings with flashes on steady background, 1 Hz sinewave without flashes, and noise without modulation were recorded with the same mean luminances. The flash responses to the PoS stimuli depended strongly on the phase of the flash relative to the sinewave background. The flash response was small, when the instantaneous luminance of the sinewave was large. The flash response was large, when the sinewave luminance was small. The effect decreased with increasing mean luminance. The response to the sinewave background also depended on the phase of flash presentation. The sinewave response was small when the flash response was large for all luminances. The responses to the PoS stimuli could be described with a linear model that assumes that the responses to the combined stimuli are a scaled addition of the flash responses on a steady background and of the responses to the sinewave without a flash. The obtained scaling factors for both flash and sinewave responses depended on their relative phase. The responses to flashes and modulating background influence each other in a complex manner. This is in contrast to responses in human observers, where only the flash response is influenced by the flash phase relative to the sinewave background. We propose that mouse responses are more sluggish and are integrated over extended periods, resulting in complex mutual interactions between responses to flashes and backgrounds.
To report the irreversible vision loss caused by optic neuropathy in a course of a vitamin A deficiency. Electroretinography (ERG) and pattern visual evoked potentials (PVEP) were used to establish a diagnosis, and to monitor the treatment in a child suffering from poor vision likely as a result of vitamin A deficiency. A 13-year-old boy presented with eye pain, dryness, itching, photophobia, and decreased vision for the past 4 weeks. His medical history was significant for autism spectrum disorder, anxiety-depressive disorder, and eating disorder—neophobia. His visual acuity was severely impaired, measuring 1.2 LogMAR in the right eye and 1.0 LogMAR in the left eye. Slit lamp examination revealed keratinization of the conjunctiva and multiple punctate epithelial defects of the cornea. Optical coherence tomography (OCT) confirmed keratomalacia. The patient was referred to the Pediatric Gastroenterology Unit for further investigation and found to have severe malnutrition including hypovitaminosis A (< 0.05 μmol/L). Treatment included vitamin A supplementation, 200 000 IU, bandage contact lenses, moxifloxacin, and lubricant eye drops. Although it was impossible to perform ERG on the initial visit because of patients’ poor general and ocular condition, full field ERGs showed generalized retinal dysfunction after 12 days of treatment. After 11 months, full field ERGs revealed retinal function within normal limits, but the patient’s vision remained poor. Pattern visual evoked potentials (PVEPs) suggested persistent optic nerve dysfunction. Electrophysiological assessments, including ERG and (PVEP) were central to establishing an accurate diagnosis in cases where resolution of anterior segment lesions due to keratomalacia did not result in visual improvement. These tests are critical for the identification and monitoring of nutritional optic neuropathy.
Biallelic FLVCR1 variants have been linked to a phenotypic spectrum ranging from isolated autosomal recessive retinitis pigmentosa (RP) to syndromic posterior column ataxia with retinitis pigmentosa (PCARP), yet detailed phenotypic characterization remains limited, particularly in Chinese patients. To delineate the ocular and extraocular phenotype of FLVCR1-associated disease and to expand its mutational spectrum. We retrospectively reviewed five affected individuals with biallelic FLVCR1 variants confirmed by whole-exome sequencing (WES) and Sanger segregation analysis, and performed comprehensive ophthalmic assessments, including colour fundus photography, fundus autofluorescence (FAF), optical coherence tomography (OCT), perimetry, and full-field electroretinography (ffERG). FLVCR1-associated disease presented as early-onset severe retinal degeneration with heterogeneous neurological involvement. OCT consistently demonstrated outer retinal thinning with ellipsoid-zone attenuation, and FAF revealed characteristic abnormalities. Cone and rod function on ffERG could be severely impaired within the first decade of life, and high myopia was a recurrent accompanying feature. Neurological manifestations could be subtle or absent at initial presentation, with gait instability and neuropathic features emerging during longitudinal follow-up. We identified two previously unreported FLVCR1 variants, c.734A > G (p.Asn245Ser) and c.1024 + 1G > T. FLVCR1-associated disease can present as early-onset severe retinal degeneration (EOSRD), together with neurological involvement that may evolve over time. These findings refine phenotypic delineation, support multidisciplinary surveillance involving ophthalmology and neurology, and expand the known mutational spectrum of this rare disorder.
The aim of this study is to evaluate the results of the Full-Field Stimulus test (FST) in patients with Stargardt disease (STGD), and to compare these results with findings obtained from other ophthalmologicalassesments. Twenty-six eyes with STGD and 26 eyes from healthy controls were included in the study. Following a routine ophthalmologic examination, all participants underwent optical coherence tomography, full-field and multifocal electroretinography, and full-field stimulus testing. The mean age was 29.3 years in the STGD group and 27.1 years in the control group. All participants successfully completed the FST procedure. Visual acuity and central macular thickness were significantly lower in the STGD group compared to the control group (p < 0,01). Multifocal electroretinography (mfERG) analysis revealed significantly decreased mean P1-wave amplitudes and prolonged mean P1-wave implicittimes across all rings in the STGD group. Additionally, FST threshols for white, blue and red stimuli were significantly lower in the STGD group than in the control group (p < 0,01). Correlation analysis revealed a positive relationship between FST results and visual acuity, mfERG amplitudes and central macular thickness. FST is a simple, reliable and rapid test that can be applied in patients with low visual acuity. It effectively measures retinal sensitivity in patients with STGD. Given its practicality FST can be safely used to monitor the disease progression and treatment response in inherited retinal diseases.
To describe electrophysiologic detection of melanoma-associated retinopathy (MAR) in a patient initially presumed to have isolated trametinib-associated retinopathy. Case report incorporating spectral-domain optical coherence tomography (OCT), fundus photography and fundus autofluorescence, serial full-field electroretinography (ffERG), and serum anti-transient receptor potential melastatin 1 (TRPM1) autoantibody testing by Western blot analysis. A 68-year-old woman receiving trametinib for metastatic vulvar melanoma developed bilateral multifocal serous retinal detachments consistent with MEK inhibitor-associated retinopathy (MEKAR). The subretinal fluid resolved after discontinuation of trametinib, but nyctalopia persisted. ffERG performed according to the International Society for Clinical Electrophysiology of Vision (ISCEV) Standard demonstrated bilateral electronegative dark-adapted (DA) 3.0 responses with relatively preserved a-waves and markedly reduced b-waves, consistent with predominant post-photoreceptoral dysfunction. Serum testing demonstrated anti-TRPM1 autoantibody positivity. At approximately 6 months after the initial ffERG, visual acuity had improved, whereas nyctalopia persisted and ffERG findings remained essentially unchanged, with persistent bilateral electronegative DA 3.0 responses. These findings supported concomitant MAR in addition to MEKAR. In patients with melanoma who develop MEKAR during trametinib therapy, persistent nyctalopia or other visual symptoms despite anatomical resolution should prompt evaluation for concomitant paraneoplastic retinopathy. An electronegative ffERG together with anti-TRPM1 autoantibody positivity can provide important supportive evidence for MAR.
To describe a patient with electronegative ERGs in association with probable TRPM1-related cancer associated retinopathy. A 67-year-old female presented with progressive visual disturbances, including night blindness, cloudy vision, color vison problems, and scotomas. Initial ophthalmic examination revealed mild retinal vasculitis and an epiretinal membrane in one eye. Visual acuity was bilaterally normal. Electroretinography (ERG) showed electronegative waveforms. Subsequent systemic investigation revealed a lung mass, later confirmed to be a small cell carcinoma, with metastases. Western blot analysis and immunohistochemistry were performed. The patient received cisplatin and etoposide chemotherapy, and sub-Tenon triamcinolone injections (STTA). ERGs showed an electronegative waveform with features suggesting pan-retinal loss of On-bipolar cell function. Serum Western blot analysis and immunohistochemistry identified anti-retinal autoantibodies to TRPM1 with binding specificity to the C-terminal region. Further systemic evaluation detected a lung mass, which was later confirmed as small cell lung cancer with metastases. Although initial visual field deterioration was noted, subsequent follow-up showed almost total improvement in visual function, including ERG recovery. Best-corrected visual acuity remained stable at 20/20. Despite the ocular improvements, the patient succumbed to her systemic illness 29 months after the initial visit. Electronegative ERGs are unusual in CAR, which usually affects photoreceptor function. This case, associated with anti-retinal autoantibodies to TRPM1, highlights the need for full systems review in a patient with possible paraneoplastic disease, even in the absence of systemic symptoms.
Bipolar cell activity plays a crucial role in myopia development. The light-adapted electroretinogram has its photoreceptoral (a-wave) and post-receptoral (mainly b-wave) origins with a later i-wave component primarily representing the activation of the OFF-bipolar cells. It has been proposed that retinal OFF-pathway alteration may contribute to the development of myopia. Therefore, elucidating specific electroretinography (ERG) changes in individuals with unilateral high myopia (UHM) could contribute to understand the underlying pathomechanisms of myopia development and devise targeted treatment options. This study aimed to compare light-adapted ERG waveforms between low-myopic/emmetropic and highly myopic eyes in patients with UHM eyes sharing an identical genetic blueprint. The subsequent comparison of the responses between highly myopic and healthy fellow eyes aimed to investigate possible photoreceptoral and/or post-receptoral pathway alterations that may play a role in myopic refractive errors. Ophthalmological and electrophysiological examinations were carried out in four individuals with UHM [mean interocular difference in spherical equivalent : 10.12 ± 2.7 (7 -13.5) D]. ISCEV standard light-adapted electroretinograms were recorded from both eyes using LKC RETeval® portable ERG unit and DTL electrodes under pupil dilation. The results showed comparable a- and b-wave amplitudes with consistently reduced i-wave amplitudes in the highly myopic eyes compared to the lower- or non-myopic fellow eyes. These findings suggest that, beyond genetic susceptibility and in the absence of overt unilateral ocular pathology, additional modulatory influences may contribute to the localized retinal regulatory mechanisms. Moreover, the i-wave appears to be a promising electrophysiological biomarker capable of detecting subtle alterations in retinal signaling, with potential implications for elucidating myopia pathogenesis and guiding targeted therapeutic development.
To examine the relationship between multifocal ERG (mfERG) topography and peak cone density measured with adaptive optics scanning light ophthalmoscopy (AOSLO) in persons with albinism (PWA). We obtained best corrected visual acuity (BCVA), mfERG, retinal imaging with AOSLO, and optical coherence tomography (OCT) data in seven PWA and one control. The relationship between central peak cone density, mfERG amplitude topography, and foveal hypoplasia (FH) grade was calculated using Spearman rank correlation. Parafoveal cone densities among a subset of PWA were compared to a previously reported control group. All PWA had variably below average peak cone density, decreased BCVA, flattened mfERG topography, and FH. Higher peak cone density significantly correlated with higher peripheral mfERG amplitudes (r2 = 0.73, p = 0.0049), but not central (r2 = 0.25), and did not correlate with steeper (more normal) mfERG topography (r2 = 0.031). Parafoveal cone densities did not greatly differ outside of the central 2° (within Ring 1) between PWA and those of previously reported controls. The association of increased foveal cone packing with elevated peripheral, but not central mfERG amplitudes, suggests aberrant post-receptoral circuitry in the macula. The presence of excess connections between cone photoreceptors and bipolar cells, similar to the unrefined circuitry seen in fetal retinae, may underly the electrical dysfunction seen in these PWA, and explain why, even with milder FH and denser cone packing, normal visual acuity is often not achieved.
We describe a case of a 4-year-old Chinese girl who presented with elevated intraocular pressure (IOP), and foveal retinoschisis (FRS) in both eyes, which was later diagnosed as CRB1-associated retinopathy. Ultrasound biomicroscopy demonstrated shallow anterior chamber, angle closure, anterior insertion of iris and anteriorly positioned ciliary body. The patient received laser peripheral iridotomy and topical medications for ocular hypertension and macular oedema. Longitudinal follow-up over 2 years revealed improved visual acuity, and effective IOP control. However, the FRS continued to progress. Genetic screening confirmed a novel copy number loss and a pseudo-homozygous c.4207G > C (p.Glu1403Gln) variant of the CRB1 gene. This case underscores the importance of comprehensive ophthalmologic examination and genetic testing in young patients with primary angle closure. In addition, the patient's hemizygous state for the CRB1 gene provides a unique opportunity to establish a genotype–phenotype association between the c.4207G > C (p.Glu1403Gln) variant and maculopathy as well as elevated IOP.
Transient flickering light stimulation (FLS) enhances the electrical activity in the middle retinal layer (MRL) of wild-type mice. This study investigates how short-term moderate and high intraocular pressure (IOP) elevation influences flicker-induced enhancement in the retinal activity using full-field electroretinograms (ffERG). Baseline blood pressure (BP), IOP, mean ocular perfusion pressure (OPP), and ffERG were measured before and after FLS in eighteen C57BL/6 J mice. The mice were subsequently divided into two groups: ocular hypertension (OHT, n = 9) and control (n = 9). In the OHT group, IOP was firstly transiently elevated to 35 mm Hg for 5 min (termed as Loop on Phase-1 (LOP-1)) using an adjustable vascular loop in one randomly chosen eye, while the control group had the loop placed without IOP elevation. IOP was further elevated to 65 mm Hg in the same eye for another 5 min (termed as Loop on Phase-2 (LOP-2)) in the OHT group, while the control group had the loop placed in the same eye without IOP increase. The BP, IOP, mean OPP and ffERG measurements were repeated before and after FLS in each condition. While BP showed no significant differences, mean OPP was significantly reduced at LOP-1 and LOP-2 in the OHT group compared to both baseline (p < 0.001) and to the control group (p < 0.001). The b-wave amplitudes recorded after FLS were significantly higher than those before FLS at baseline and LOP-1 conditions in both control (p < 0.01) and OHT groups (p < 0.001). In the LOP-2 condition, the OHT group showed no significant difference (p > 0.05) between pre- and post-FLS b-wave amplitudes, while the control group exhibited a significant increase (p < 0.001). The percentage change in b-wave amplitude was significantly reduced in the OHT group at LOP-2 condition (Pre-Loop vs LOP-2: p < 0.01; LOP-1 versus LOP-2: p < 0.001), while the control group maintained a consistent percentage increase in b-wave amplitudes. No such significant changes were found in other parameters of ffERG response after FLS. Short-term high IOP elevation ( 65 mm Hg), but not moderate ( 35 mm Hg), disrupted flicker-induced enhancement of MRL electrical activity. This implies that the retina can adapt to a short period of moderate IOP, sustaining a normal increase in the retinal electrical activity in response to FLS. However, even a short period of high IOP would cause certain physiological damage to the retina.
PURPOSE:To describe the first reported case of non-proliferative Duchenne muscular dystrophy-associated retinopathy manifested as bilateral perifoveal ischemia. METHODS:This observational case report details a 21-year-old male with genetically confirmed Duchenne muscular dystrophy (DMD) who presented with bilateral visual decline. A comprehensive ophthalmic evaluation was performed including best-correct visual acuity (BCVA) assessment, slit-lamp biomicroscopy, dilated fundus examination, full-field and multifocal electroretinography (ERG) in accordance with ISCEV standards and ERGs to sawtooth modulation, structural spectral-domain optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) in both eyes. RESULTS:BCVA was 20/40 in both eyes. Anterior segment examination revealed bilateral posterior subcapsular cataracts, while dilated fundoscopic examination was unremarkable. Multifocal ERG demonstrated reduced amplitudes in the central and parafoveal rings, indicating localized retinal dysfunction. OCTA disclosed bilateral, irregular enlargement of the foveal avascular zone consistent with perifoveal ischemia. These vascular abnormalities corresponded to the areas of inner retinal thinning with secondary outer nuclear layer expansion in structural OCT. CONCLUSION:DMD-associated retinopathy may present with retinal ischemia in the absence of overt fundoscopic abnormalities. Multimodal structural and functional modalities including multifocal ERG, OCT and OCTA may be critical to the early detection of subclinical ischemic changes and for identifying patients at risk of progression to proliferative retinopathy.
PURPOSE:To evaluate the association between dynamic pupillometry parameters and in vivo confocal microscopy (IVCM)-derived corneal sub-basal nerve morphology in patients with immune-related dry eye disease. METHODS:A cross-sectional study was conducted on 28 patients with immune-related dry eye disease (DED). Corneal sub-basal nerve morphology, including corneal nerve fiber length density (CNFL), intensity, and tortuosity, was quantified using IVCM. Dynamic pupillary light reflexes, including pupil size diameter and constriction/dilation velocities, were measured via automated pupillometry. Correlation and multivariate regression analysis were employed to determine the independent associations between structural nerve metrics and functional pupillary parameters. RESULTS:Significant positive correlations were observed between CNFL and baseline pupil size, recovery pupil size, and total contraction and dilation time. Multivariate regression confirmed that CNFL remained an independent predictor of baseline pupil diameter after adjusting for age and gender. CONCLUSION:Structural rarefaction of the corneal sub-basal nerves is closely mirrored by functional impairments in pupillary dynamics. Dynamic pupillometry may provide complementary functional information associated with corneal nerve alterations in patients with immune-related DED.
To determine whether photopic negative response (PhNR) parameters obtained using a handheld electroretinography (ERG) device reflect quantitative indicators of residual retinal ganglion cell (RGC)-driven inner retinal function and how these functional measures relate to OCT-derived structural metrics in chronic unilateral non-glaucomatous optic neuropathy (ON). In this retrospective observational study, 27 patients (54 eyes) with unilateral chronic ON were examined using handheld full-field photopic ERG (RETeval™, LKC Technologies) without pupil dilation or corneal electrodes. Several ERG parameters, including the PhNR72 amplitude, PhNR minimum amplitude, P-ratio, and W-ratio, were analyzed. Optical coherence tomography (OCT)—derived ganglion cell–inner plexiform layer (GCIPL) and retinal nerve fiber layer (RNFL) thicknesses, and Humphrey field analyzer (HFA) indices were acquired on the same day. Linear mixed-effects models accounting for intereye correlation and false discovery rate (FDR) correction were used to assess structure–function relationships. ON eyes showed significantly reduced PhNR72 and PhNR minimum amplitudes, and lower P-ratio (PhNR72/b-wave) and W-ratio (PhNR minimum/(b-wave minus a-wave) (all p < 0.01) compared with unaffected eyesof the patients. In multivariable models, the W-ratio was independently associated with GCIPL thickness in the inferotemporal sector (β = 0.45; 95
To describe the detailed clinical course and genetic findings of a Japanese patient with nanophthalmos (isolated microphthalmia) who developed bilateral uveal effusion syndrome (UES). A 50-year-old Japanese man presented with distorted and decreased vision in his right eye. Ophthalmoscopy revealed non-rhegmatogenous retinal detachment with shifting subretinal fluid. The horizontal corneal diameter was 11.5 mm in both eyes, while the axial lengths were 15.6 and 15.3 mm in the right and left eyes, respectively, confirming the diagnosis of nanophthalmos (microphthalmia)-associated UES. After steroid pulse therapy followed by sclerectomy with vortex vein decompression (S-VVD), the UES resolved 10 months postoperatively. The eyes remained stable for 15 years, after which the patient developed bilateral cataracts and elevated intraocular pressure. Cataract surgery was done uneventfully, but signs of UES appeared in the left eye five days postoperatively. This was controlled with multiple treatments, including steroid pulse therapy, intravitreal anti VEGF injection, and oral carbonic anhydrase inhibitor. However, UES recurred after five months. S-VVD and vitrectomy with silicone oil tamponade were performed, and the UES resolved after one year postoperatively. Ultimately, electroretinography (ERG) became non-recordable, and the visual fields were constricted in both eyes. Whole-exome sequencing identified a previously unreported homozygous nonsense variant in PRSS56 (c.202C>T, p.Arg68Ter), classified as pathogenic based on the ACMG criteria. This report describes a patient with an unreported pathogenic PRSS56 variant who exhibited a typical and severe course of nanophthalmos (isolated microphthalmia) complicated by bilateral UES. Although the effusions eventually resolved after multiple interventions, the prolonged UES was challenging to manage and resulted in irreversible retinal dysfunction with a non-recordable ERG.
To describe the clinical and multimodal imaging characteristics of congenital grouped albinotic spots (CGAS) and to explore their functional implications using full-field electroretinography (ERG). Two patients with incidentally detected CGAS underwent comprehensive ophthalmic examination, including best-corrected visual acuity, refraction, dilated fundus evaluation, swept-source optical coherence tomography (SSOCT), fundus autofluorescence (FAF) imaging, and full-field ERG. Longitudinal follow-up was available for one case over 7 years. Clinical and imaging findings were qualitatively compared with previously reported CGAS features and with differential diagnoses of flecked retina disorders. Both patients demonstrated numerous, bilaterally symmetrical, flat hypopigmented lesions that were small and round at the posterior pole, sparing the fovea and larger, linear, and radially oriented in the periphery. FAF showed striking hyperautofluorescence corresponding to the hypopigmented patches. SSOCT in both cases showed preserved foveal contour, normal retinal architecture, and intact RPE–photoreceptor complex. Visual acuity was 20/20 in both patients at last examination, after refractive correction. ERG responses were largely within normal reference limits, although each case exhibited minimally reduced photopic responses. CGAS represents a benign, nonprogressive RPE abnormality with characteristic peripheral hypopigmented spots that are paradoxically hyperautofluorescent on FAF and associated with preserved retinal structure and good visual function. FAF and OCT are valuable, non-invasive tools for confirming the diagnosis, delineating lesion extent, and distinguishing CGAS from inherited flecked-retina dystrophies. Additional case series and genetic studies are warranted to clarify underlying mechanisms and potential associations.
Pathogenic variants in the RPE65 gene cause various forms of inherited retinal dystrophies (IRD), which include Leber congenital amaurosis (LCA), early childhood-onset retinal dystrophy (ECORD), and various forms of retinitis pigmentosa (RP). To date, no study has characterised the heterogeneity of RPE65 variants associated with IRD in the Egyptian population. This study aimed to identify RPE65 pathogenic variants in a cohort of Egyptian children with IRD, with implications for gene therapy eligibility. A total of 44 patients in the paediatric age group (from birth to 18 years old), from 27 unrelated Egyptian families with non-syndromic IRD, underwent detailed ophthalmic examination, which include electroretinogram (ERG), optical coherence tomography (OCT), and fundus autofluorescence (FAF), and their DNA samples were screened for RPE65 variants using Sanger sequencing. Among the 44 patients studied, 8 (18.2
BACKGROUND:Serous Maculopathy Due to Aspecific Choroidopathy (SMACH) is a rare chorioretinal disease characterized by polymorphic, non-pigmented choroidal lesions, with or without subretinal fluid (SRF). Its clinical manifestations overlap with common conditions such as central serous chorioretinopathy (CSC) and age-related macular degeneration (AMD), leading to a high risk of misdiagnosis, particularly in elderly patients. Multimodal imaging, including optical coherence tomography (OCT), OCT angiography (OCTA), fluorescein fundus angiography (FFA), and indocyanine green angiography (ICGA), is critical for accurate diagnosis. CASE PRESENTATION:A 76-year-old Asian female presented with a 6-month history of blurred vision in her left eye. Ophthalmic examination revealed multiple yellowish-white subretinal lesions with mild elevation in the posterior pole of the left eye. OCT and OCTA demonstrated hyperreflective fibrinous exudates between the retinal pigment epithelium (RPE) and Bruch's membrane, choroidal thickening, and outer retinal damage. Fluorescein fundus angiography (FFA) and indocyanine green angiography (ICGA) provided complementary findings consistent with choroidal dysfunction and blood-retinal barrier disruption. Systemic evaluations, including laboratory tests for rheumatological immune indicators, tuberculin test, syphilis serology, and imaging examinations, excluded other systemic diseases. Based on these multimodal imaging findings and exclusion of alternative diagnoses, the patient was diagnosed with SMACH. CONCLUSIONS:This case report describes a typical presentation of SMACH in an elderly patient, emphasizing that multimodal imaging, as demonstrated in this case, can help identify characteristic features of SMACH (choroidal thickening, hyperreflective exudates), though its diagnostic reliability in larger populations requires further validation.The exclusion of systemic diseases and differentiation from similar chorioretinal disorders (e.g., CSC, AMD) are crucial for accurate diagnosis.This case adds to the existing limited data on SMACH in elderly populations and further supports the role of multimodal imaging in improving diagnostic accuracy to reduce misdiagnosis.
Impaired pupillary dynamics are a well-recognized feature of pseudoexfoliation syndrome (PXF), yet little is known about how cataract surgery influences postoperative iris function in these eyes. This study aimed to determine the longitudinal effects of cataract surgery on static pupil diameters and dilation velocity in eyes with pseudoexfoliation syndrome compared with age-matched controls. This longitudinal study included 166 eyes of 166 patients undergoing cataract surgery, comprising 91 eyes with pseudoexfoliation syndrome and 75 control eyes without pseudoexfoliation. Pupillary parameters were measured preoperatively and at six months postoperatively using automated pupillometry. Static pupil diameters were assessed under scotopic (0.04 lx), mesopic (4 lx), and photopic (40 lx) illumination conditions. Dynamic pupillary function was evaluated by measuring dilation velocity (DVel, mm/s) following a standardized light stimulus. Postoperative changes (Δ) were calculated as the difference between preoperative and postoperative measurements. Static pupil diameters remained stable in the PXF group across all illumination conditions (p > 0.05). In contrast, the control group demonstrated a significant reduction in scotopic pupil diameter after surgery (p = 0.008), while mesopic and photopic diameters remained unchanged. The most notable finding was observed in pupillary kinetics: dilation velocity significantly increased in the PXF group from 0.13 ± 0.04 mm/s to 0.17 ± 0.05 mm/s (p < 0.001), whereas no significant change was detected in the control group. Between-group comparison showed a significantly greater improvement in dilation velocity in PXF eyes (p < 0.001). Cataract morphology was not associated with postoperative pupillary changes. These findings suggest that cataract surgery may be associated with measurable changes in dynamic pupillary behavior in PXF eyes, particularly in dilation velocity, while static pupil diameter remains largely unchanged.
