Purpose: Refractory secondary glaucoma following pars plana vitrectomy with heavy silicone oil (Densiron) tamponade represents a significant surgical challenge due to gravity-dependent oil accumulation in the inferior angle. The PreserFlo® MicroShunt is a minimally invasive subconjunctival drainage device composed of highly biocompatible poly-styrene-block-isobutylene-block-styrene (SIBS), featuring a 70 µm lumen designed to regulate aqueous humor outflow with reduced surgical trauma compared to traditional drainage valves. Methods: We report the case of an 80-year-old male with refractory ocular hypertension peaking at 36 mmHg in the left eye following vitrectomy for retinal detachment managed with Densiron, where gonioscopy confirmed significant oil microemulsions predominantly saturating the inferior angle. Surgical intervention was performed via an ab externo approach with superonasal implantation of the MicroShunt and intraoperative application of mitomycin C. Results: At the six-month follow-up, the intraocular pressure stabilized at 18 mmHg with a functional filtering bleb and stable visual field parameters. Conclusion: This case underscores the strategic importance of superior quadrant placement in eyes with heavy oil tamponade, which establishes a drainage pathway physically distant from the primary site of oil accumulation to prevent potential obstruction of the 70 µm device lumen. Our findings support the PreserFlo® MicroShunt as a viable, less invasive alternative for achieving stable intraocular pressure control in complex vitrectomized eyes.
PURPOSE:To describe a case of refractory unilateral PEVAC macular edema initially treated with intravitreal faricimab injections. METHODS:Medical and imaging records were evaluated. Tracked optical coherence tomography (OCT) B scans were reviewed to assess intra-retinal fluid and exudation. RESULTS:A 79-year-old man with unilateral chronic macular edema secondary to PEVAC lesion underwent three monthly intravitreal faricimab injections with completely resolution of the fluid and visual recovery. A presumed thrombotic mechanism of the exudative vascular complex might have occurred in this case. CONCLUSION:After conducting a literature review on June, 2025, utilizing PubMed and Google Scholar, using the key words PEVAC, LRCA and faricimab therapy, there wasn't any prior reports of PEVAC case treated by a bi-specific antibody directed against Ang2 and VEGF (faricimab). Though, limitted by a short follow-up time (9 months), this unique clinical experience should be shared to inform that faricimab may lead to early remarkable anatomical and functional improvement in eyes with no responsive PEVAC lesions.
PURPOSE:Inherited retinal dystrophies (IRDs) may present as isolated entities or as multisystem syndromes. Yunis-Varon syndrome (YVS), seen with homozygous FIG4 mutations, is a rare autosomal recessive disorder. Herein, we report a case of YVS associated with early-onset rod-cone dystrophy. METHODS:Ultrawide-field fundus photography was documented, along with a comprehensive genetic evaluation and a brain neurosonogram. RESULTS:An 11-month-old child born out of a consanguineous marriage presented with absent eye contact, global developmental delay and several dysmorphic features suggestive of cleidocranial dysplasia. Fundus examination showed pigmentary retinopathy, pale disc and macular atrophic changes along with attenuated arterioles. Whole genome sequencing revealed a homozygous FIG4 variant of uncertain significance and a heterozygous likely pathogenic PANK2 variant. Basal ganglia abnormalities were seen in the neurosonogram. A diagnosis of YVS with atypical early onset rod-cone dystrophy was made. CONCLUSION:This case presents a rare ocular finding of YVS and broadens its phenotype. This helps to highlight the importance of comprehensive ocular examination in suspected syndromic patients, which would aid in diagnosis, genetic correlation and prognostication.
Purpose: to report three cases of Silicone Oil-related Vision Loss (SOVRL) with an unreported Optical Coherence Tomography sign before and after removal. Methods: Three patients with SOVRL underwent multimodal retinal imaging, including spectral-domain OCT. Results: Three male patients underwent pars plana vitrectomy for rhegmatogenous retinal detachment (RRD). SO was used as intraocular tamponade in two cases with giant retinal tear (GRT) retinal detachment, and one case of macula-on retinal detachment and epiretinal membrane (ERM). In all cases, a foveal columnar hyperreflectivity (FOCOH) was identified before silicone oil removal (ROSO). Following ROSO, all the patients experienced reduction in visual acuity after an initial recovery. Two patients demonstrated regression of the FOCOH sign—one with concurrent visual recovery, while the other experienced persistent visual impairment despite disappearance of the sign (final VA 20/20 and 20/200). In the third case the hyperreflectivity persisted, accompanied by inner retinal cystoid spaces and poor final visual acuity (final VA 20/200). In the first case FOCOH disappeared after three months, in the second case the sign disappeared after one month from ROSO, and in the third case the FOCOH persisted for more than 2 months. Conclusions: The early presence of FOCOH under SO suggests its potential role as a predictive biomarker of visual decline. Recognizing FOCOH may provide additional clues for the early diagnosis of SOVLR and guide therapeutic interventions.
Purpose: To describe a case of presumed paraneoplastic acute exudative polymorphous vitelliform maculopathy (AEPVM) associated with early gastric carcinoma with subsequent slow regression of vitelliform lesions followed by localized outer retinal atrophy. Methods: a 58-year-old woman with a remote history of central serous chorioretinopathy presented with new metamorphopsia and multifocal subretinal yellowish deposits. Multimodal imaging—including spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography—was performed at baseline. Genetic and serologic testing as well as systemic evaluation with total-body MRI and endoscopy were conducted to exclude inherited or inflammatory causes and to identify a possible paraneoplastic association. Results: At presentation OCT revealed shallow serous detachments with hyperreflective subretinal material consistent with multifocal vitelliform lesions. At seven months, partial regression of lesions was noted, leading to systemic evaluation that uncovered an early intramucosal gastric adenocarcinoma, successfully treated by endoscopic submucosal dissection alone. Over seven years multimodal imaging demonstrated near-complete resorption of vitelliform material and the development of a localized zone of retinal pigment epithelium and outer retinal atrophy along the superior arcade. Best-corrected visual acuity remained 20/20 throughout. No ocular or systemic immunosuppressive therapy was administered. Conclusion: AEPVM may be associated with early gastric carcinoma and spontaneous regression of vitelliform lesions evolving into localized atrophy without visual decline. Long-term follow-up is essential given the late progression to atrophy.
Purpose: To report a case of nivolumab induced hypoalbuminemia causing a serous retinal detachment. Methods: Multimodal imaging including optical coherence tomography, fundus photography, and fluorescein angiography was performed. Results: A 59-year-old female with a history of metastatic renal cell carcinoma on nivolumab/ipilimumab immunotherapy with a recent hospitalization for anasarca, ascites, pleural effusion, and hypotension developed bilateral chemosis and a serous retinal detachment in the right eye. There were no pachychoroid vessels, intraocular inflammation, or leakage on fluorescein angiography to suggest central serous chorioretinopathy or a Vogt-Koyanagi-Harada-like reaction to immunotherapy. The third spacing and hypoalbuminemia was thought to be due to nivolumab-induced capillary leak syndrome, and the offending medication was stopped. The conjunctival chemosis and subretinal fluid improved with correction of underlying systemic hypoalbuminemia. Conclusion: This case highlights a case of nivolumab-induced hypoalbuminemia causing a serous retinal detachment. This serves as an excellent teaching case as the authors discuss pertinent clinical exam and multimodal imaging features that allow differentiation of serous retinal detachments due to medications, central serous chorioretinopathy, and Vogt-Koyanagi-Harada syndrome.
Purpose: To report a case of a 30-year-old male with bilateral cuticular drusen with bilateral pachychoroid phenotype and left eye pachychoroid driven central serous chorioretinopathy (CSC). Methods: Clinical fundus examination and multimodal imaging which included optical coherence tomography (OCT), fundus autofluorescence (FAF) and fundus fluorescein angiography (FFA) was done. Results: A patient presented to us with subretinal fluid at the macula in the left eye. FFA was done and the presence of ink blot pattern leakage confirmed the diagnosis of CSC in left eye, along with multiple, small, round, hyperfluorescent spots, creating a classic “starry-sky” pattern along the vascular arcades characteristic of cuticular drusen in both eyes. On OCT, we noted characteristic features of pachychoroid in both eyes. Conclusion: The role of multimodal imaging in diagnosing a rare case of cuticular drusen with pachychoroid CSC in a young adult is important.
PURPOSE:Using retrospective data, we demonstrate a technique of grid laser retinopexy together with vitrectomy and gas tamponade (pneumatic maculopexy) to permanently resolve branch retinal vein occlusion (BRVO)-related cystoid macular edema (CME). METHODS:After laser ablation to ischemic peripheral retina, and six months of unsuccessful medical therapy with intravitreal bevacizumab injections, we performed vitrectomy with grid laser treatment to superotemporal macular edema, and C3F8 15% gas tamponade (pneumatic maculopexy). RESULTS:Immediately after resolution of gas tamponade all CME had resolved, and visual acuity had improved from 20/40 to 20/30. Twenty-four months after vitrectomy and without further therapy, visual acuity had improved to 20/25 and the macula remained dry. CONCLUSION:When medical therapy is insufficiently effective, maculopexy by grid laser combined with intravitreal drying agents and/or gas tamponade can achieve long-term resolution of cystoid macular edema in selected cases. Parafoveal laser treatment must be precisely performed.
Purpose: To report a novel case of kratom ( Mitragyna speciosa )-associated ocular toxicity presenting with visual snow and ellipsoid zone disruption, expanding the known ophthalmic complications of chronic kratom use. Methods: A case report and literature review were performed. Results: A 70-year-old man with 4 years of daily kratom use (6.8 g daily) presented with worsening nyctalopia and a visual snow-like phenomenon. OCT showed bilateral ellipsoid zone disruption and peripheral RPE changes. Fundus autofluorescence showed macular hypo-autofluorescence. Indocyanine Green and Fluorescein Angiographies were unremarkable. ERG was also unremarkable. His MRI showed chronic, nonspecific age-related changes. Cutaneous hyperpigmentation was also noted. Urine mitragynine was positive. Conclusions: This case raises the possibility that chronic kratom use may be associated with visual snow-like symptoms, ellipsoid zone disruption, and RPE changes. Consider multimodal retinal imaging in chronic users, especially with dermatologic signs. More studies are needed to define kratom's ocular toxicity profile.
Purpose: To describe the in vivo hourly resolution of a bacillary layer detachment (BALAD) with an intact anterior wall in a patient with fovea-off rhegmatogenous retinal detachment (RRD). Methods: Case report. Results: A 58-year-old female patient presented with a fovea-off RRD and foveal BALAD with an intact anterior wall, with visual acuity of counting fingers at 3 feet. She underwent pneumatic retinopexy with an anterior chamber paracentesis and intravitreal injection of 0.6 cc of pure sulfur hexafluoride gas and was instructed to maintain face-down positioning for 6 hours. Hourly swept-source optical coherence tomography (SS-OCT) imaging with the PLEX Elite 9000 (Carl Zeiss, Dublin, California, USA) with high-definition horizontal spotlight scans and 12×12 mm raster scans demonstrated excellent structural recovery of the fovea. At the one-month follow-up, the retina remained attached with visual acuity improving to 20/100. Two months later, visual acuity improved to 20/30. The OCT showed significant recovery of the outer retinal bands, although a small sub foveal defect in the interdigitation zone (IZ) remained. Conclusion: This case highlights the importance of recognizing fovea-off RRD associated with BALAD, particularly when the anterior wall is intact. Urgent intervention can allow for foveal structural recovery potentially preventing the development of a BALAD-lamellar hole and full-thickness macular hole.
PURPOSE:This case describes congenital stationary night blindness (CSNB) with a fundus albipunctatus-like phenotype linked to compound heterozygous RPE65 variants, highlighting the diagnostic value of multimodal imaging and evidence-based variant interpretation. METHODS:A retrospective case review including best-corrected visual acuity (BCVA), color vision testing, fundus photography, spectral-domain optical coherence tomography (SD-OCT), full-field electroretinography (ERG), and next-generation sequencing with parental segregation analysis. Variant pathogenicity was assessed using in-silico prediction models (PolyPhen-2 HumDiv and HumVar), and findings were integrated with clinical and imaging data. RESULTS:A sixteen-year-old male reported lifelong nyctalopia and stable difficulty seeing in dim. ENVIRONMENTS:BCVA was 20/30 OD and 20/25 OS with normal color vision. Fundus examination revealed multiple white-yellow flecks along the arcades and mid-periphery with macular sparing. SD-OCT demonstrated preserved outer retinal architecture and an intact ellipsoid zone, and full-field ERG showed preserved rod and cone responses. Genetic testing identified two RPE65 missense variants: c.433G>A (p.Ala145Thr), likely pathogenic, and c.946A>G (p.Asn316Asp), reported as a VUS. PolyPhen-2 analysis classified p.Asn316Asp as damaging (HumDiv/HumVar score 1.000), and parental segregation demonstrated the variants in trans. Integration of genotype and phenotype supports p.Asn316Asp as likely pathogenic. CONCLUSION:Compound heterozygosity for hypomorphic RPE65 variants can produce a stationary fundus albipunctatus-like phenotype rather than progressive retinal degeneration. Careful integration of multimodal phenotyping and variant interpretation was essential for confirming disease causality.
PURPOSE:To describe macular ischemia following cosmetic penile filler injection due to presumed branch retinal artery occlusion from embolization through a patent foramen ovale (PFO). METHODS:Multimodal imaging, including fundus photography, spectral domain optical coherence tomography (OCT), OCT angiography (OCTA), and fluorescein angiography (FA) were performed. RESULTS:A 33-year-old healthy male presented with an acute paracentral scotoma of the left eye (OS) shortly after undergoing an elective cosmetic penile filler injection. Retinal examination and color fundus photography illustrated a cotton wool spot corresponding to the acute scotoma OS. Spectral domain OCT showed corresponding hyperreflectivity of the inner and middle retina OS and paracentral acute middle maculopathy (PAMM). OCTA of the superficial and deep capillary plexus showed corresponding flow deficit and fluorescein angiography illustrated corresponding segmental staining of the retinal arterioles in the macula suggestive of branch retinal artery occlusion (BRAO) OS. Extensive systemic evaluation was unremarkable except for transthoracic echocardiography, which revealed a PFO. Brain magnetic resonance imaging (MRI) was normal and infectious, inflammatory, and hypercoagulable workups were negative. CONCLUSION:and Importance: This report highlights a case of occlusive vascular maculopathy following distal cosmetic filler injection likely mediated by embolization through a PFO. Arterial occlusive events can occur after elective cosmetic procedures and patients should be counselled accordingly.
PURPOSE:Biallelic variants in the HGSNAT gene are associated with Sanfilippo syndrome, a rare lysosomal storage disorder caused by deficiency of heparan acetyl CoA glucosamine N-Acetyl-transferase enzyme(HGSNAT). The syndrome is characterized by multiple systemic findings, including progressive neurological and retinal degeneration. Retinitis pigmentosa due to HGSNAT gene variants are rarely reported without systemic manifestations. METHODS:Here we describe two patients with retinitis pigmentosa and reduced enzymatic activity. One of our patients also had hearing loss. We also present a review of the literature for cases of non-syndromic retinal dystrophy associated with HGSNAT variants in the discussion. RESULTS:The first patient with retinitis pigmentosa and hearing loss has two variants of uncertain significance in the HGSNAT gene(p.Thr522Met and p.Cys79Arg). HGSNAT enzyme activity was 0.43nmol/17hr/mg(normal:5.8-45nmol/17hr/mg protein). Our second patient with isolated retinitis pigmentosa has two heterozygous pathogenic variants in HGSNAT (p.Arg124Trp and p.Ala615Thr). HGSNAT enzyme activity was 1.13nmol/17hr/mg(normal:5.8-45nmol/17hr/mg protein). CONCLUSIONS:The retinal findings are consistent with HGSNAT-related disease. The first case is the second report of retinitis pigmentosa and hearing loss linked to HGSNAT-related disease. Both cases showed reduced HGSNAT enzyme activity in blood. Although more evidence is needed to definitively link adult-onset hearing loss to HGSNAT variants, it may be due to reduced enzyme activity.
Purpose: To investigate the utility of intravitreous methotrexate and rituximab in the treatment of Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP). Methods: The observational cohort study of five eyes of three patients with BDUMP receiving combination injections of intravitreous methotrexate 400 mcg/0.05 mL and rituximab 1 g/0.1 mL as part of multimodal treatment of BDUMP. Change in central foveal thickness and subfoveal fluid was obtained by optical coherence tomography, and change in logMAR/Snellen vision was compared before and after each injection and over the follow-up duration. Results: Five eyes of three male patients with BDUMP received 39 combination injections of intravitreous methotrexate and rituximab. The median follow-up period was 48.2 months; all eyes exhibited a decrease in central foveal thickness and subfoveal fluid and an improvement in vision at some point from baseline. The median/mean vision increase was 5/3.4 Snellen lines, decrease in central foveal thickness was 243/235 μ m, and decrease in subfoveal fluid was 240/273 μ m. Over the follow-up period, one eye had a gradual decline in vision from 20/40 to 20/150 over 48 months Four of the 5 eyes had marked improvement and sustained vision: from vision ranging 20/50 to 20/150 at baseline to all 4 improving to 20/25 or 20/20. Conclusion: Intravitreous methotrexate and rituximab may be a useful, well-tolerated adjunctive treatment for BDUMP. Improvements in central foveal thickness and subfoveal fluid were noted following injections and over the follow-up period. Unlike the often, rapid decline in vision that is expected of this paraneoplastic disease, the eyes in this cohort appeared to have more favorable outcomes.
PURPOSE:The purpose of this study was to describe a patient with peripapillary hyperreflective ovoid mass-like structures (PHOMS) associated with macular type 2 choroidal neovascularization. METHODS:Case report. RESULTS:A healthy 36-year-old woman presented with vision loss in the right eye over the previous 3 days. An optical coherence tomography (OCT) B-scan of the optic nerve head showed PHOMS, and OCT-angiography of the macular region showed a type 2 choroidal neovascularization. The patient was treated with an intravitreal injection of anti-vascular endothelial growth factor with marked improvement. CONCLUSION:PHOMS is an OCT finding presently believed to result from axoplasmic stasis due to herniation of the retinal nerve fiber layer at the optic nerve head. Several systemic and ocular findings associated with PHOMS have been commonly described, but macular choroidal neovascularization remains a rare association.
PURPOSE:To report a case of scrub typhus retinopathy presenting with angular sign of Henle layer hyperreflectivity (ASHH) on optical coherence tomography (OCT). METHODS:This is a retrospective case report with multimodal imaging. RESULTS:A 43-year-old Indian man presented with sudden, painless diminution of vision in both eyes,(left more than right) following a febrile illness. He was diagnosed with scrub typhus based on positive IgM ELISA. Fundus examination revealed scattered intraretinal haemorrhages in both eyes and a yellowish foveal lesion in the left eye. Optical coherence tomography (OCT) demonstrated angular sign of Henle layer hyperreflectivity (ASHH) in both eyes, with a hard exudate clump at the fovea in the left eye and corresponding areas of blocked fluorescence on fluorescein angiography. A diagnosis of scrub typhus retinopathy was made. After treatment with oral corticosteroids, at one-month follow-up, visual acuity, with marked resolution of haemorrhages and regression of ASHH on OCT. CONCLUSION:Scrub typhus can present with retinopathy associated with ASHH, a marker of deep retinal ischemia. Multimodal imaging, particularly OCT, plays a crucial role in identifying ischemic retinal changes and may help in assessing disease severity and visual prognosis.
PURPOSE:To report a case of unilateral endogenous Streptococcus anginosus endophthalmitis in order to improve timely recognition and treatment strategies for similar presentations. METHODS:A retrospective review of the electronic medical record of one patient was performed from September 2024 through December 2024. RESULTS:A 50-year-old healthy male presented with a unilateral hypopyon and dense vitreous opacities. The patient was found to have evidence of systemic infection and endophthalmitis secondary to S. anginosus; specifically, the patient was found to have a large subcarinal mass as well as abscesses in the left cerebrum, both of which were positive for S. anginosus. The patient underwent treatment with intravitreal and systemic antibiotics as well as pars plana vitrectomy. CONCLUSION:S. anginosus endophthalmitis in the setting of a confirmed primary mediastinal infection has not previously been reported. Our patient had a particularly virulent presentation of S. anginosus endophthalmitis, and the ophthalmic findings led to the diagnosis of a systemic infection. A thorough systemic evaluation of patients with suspected endogenous endophthalmitis should be pursued, and a seemingly noncontributory medical history can prove to be significant.
PURPOSE:To evaluate the feasibility of single-port illuminated endolaser photocoagulation using wide-angle visualization with BIOM as a nonvitrectomy approach for treating peripheral large retinal tears with localized retinal detachment. METHODS:A 60-year-old patient presented with a peripheral large retinal tear and localized retinal detachment (RD). Owing to media opacity and far peripheral location, barrier laser could not be completed with conventional laser delivery systems. Because of the potential risk of aggravating RD in the setting of a large retinal break with overlying vitreoretinal traction, external cryotherapy was not used. Instead, we used 25g single-port illuminated endolaser probe to complete the barrier laser photocoagulation around the retinal break and localized RD area with the assistance of scleral indentation using a 150° wide-angle BIOM system without vitrectomy. RESULTS:The single-port illuminated endolaser approach provided enhanced visualization including extreme peripheral location of the retina while depressing the sclera, allowing for comprehensive laser coverage of the lesion. This effectively prevented the progression of RD and facilitated fluid absorption, ultimately leading to the resolution of the localized RD. At 4 months postlaser treatment, the patient's retina is stable with well-formed chorioretinal adhesion. CONCLUSION:When retinal breaks are located in the far periphery with severe VR traction and media opacity is present, completion of conventional laser treatment may be challenging. A 25 g single-port illuminated endolaser photocoagulation without vitrectomy under BIOM may serve as an effective treatment option.
PURPOSE:Letrozole is a non-steroidal aromatase inhibitor commonly used for the treatment of postmenopausal estrogen receptor-positive breast cancer. We report a novel case of bilateral panuveitis associated with letrozole therapy. METHODS:Retrospective chart review. RESULTS:A 39-year-old female with a history of breast cancer presented with bilateral anterior chamber and vitreous inflammation, perivenular sheathing, retinal hemorrhages, and a focal area of retinal whitening in the left eye six weeks after starting letrozole therapy. Extensive infectious and systemic inflammatory work-up was negative. With letrozole cessation and corticosteroid therapy, the inflammation resolved, and the visual acuity improved to 20/20 bilaterally. DISCUSSION AND CONCLUSION:Letrozole-associated panuveitis has not previously been reported. This case highlights the importance of detailed medication history as well as the need for interdisciplinary collaboration when managing drug-induced uveitis. Early recognition and appropriate therapy can result in favorable visual outcomes.
PURPOSE:To report a pediatric case of exudative bullous retinal detachment and uveal effusion syndrome (UES) in Kenny-Caffey syndrome (KCS) complicated by nanophthalmos. METHODS:Multimodal imaging evaluation and clinical outcome of a pediatric patient presenting emergently with decreased visual acuity. RESULTS:A 13-year-old girl with KCS presented with 2-day blurred vision in the LE (20/100, +17 D; RE 20/20, +15 D). Fundus exam revealed exudative bullous retinal detachment involving the macula and temporal periphery. Ultrasound showed scleral and choroidal thickening with short axial length, consistent with nanophthalmos. The patient was treated with oral prednisone and acetazolamide. Complete resolution of subretinal fluid was observed at 6-week follow-up, with improved visual acuity. CONCLUSION:We describe a rare association of KCS, nanophthalmos, and UES. Conservative medical therapy may be an effective alternative to surgery mostly in pediatric cases.
