PURPOSE:To describe a case of sympathetic ophthalmia following vitreoretinal surgery for myopic traction maculopathy, highlighting novel clinical findings that provide in vivo support for a melanophage-mediated immune mechanism. METHODS:Observational case report. RESULTS:A 26-year-old high-myopic female developed bilateral granulomatous panuveitis four weeks after pars plana vitrectomy and fluid-gas exchange for myopic traction maculopathy OD. Clinical examination revealed bilateral mutton-fat keratic precipitates and vitreous cells, more severe OD. Prominent pigment within granulomatous keratic precipitates and pigment-laden cells posterior to the crystalline lens were also observed OU. View of the posterior segment in OD was poor. Multifocal yellowish choroid lesions (Dalen Fuchs nodules) were present in OS. Optical coherence tomography only possible in OS demonstrated bacillary layer detachment, subretinal fluid, and marked choroidal thickening. Cerebrospinal fluid analysis revealed marked leukocytic pleocytosis. Sympathetic ophthalmia (SO) was diagnosed. Following prompt initiation of high-dose systemic corticosteroids and steroid-sparing immunosuppression, bilateral ocular inflammation resolved with normalization of choroidal thickness. CONCLUSIONS:Dense hyperpigmented clumps within keratic precipitates and pigment-laden cells in the posterior segment consistent with melanophage activity, support the histopathological hallmark of sympathetic ophthalmia, in which macrophages phagocytose liberated uveal melanocytes.
PURPOSE:To describe an ab externo surgical technique for submacular band removal in pediatric patients with rhegmatogenous retinal detachment (RRD) complicated by proliferative vitreoretinopathy (PVR). METHODS:Ten eyes of 10 pediatric patients diagnosed with RRD and PVR grade C associated with submacular bands. All eyes underwent submacular band removal using an ab externo transscleral approach combined with scleral buckling and 23-gauge pars plana vitrectomy. Preoperative localization of submacular bands was performed using optical coherence tomography and/or B-scan ultrasonography, with intraoperative targeting guided by Resight viewing. RESULTS:Submacular band removal via the ab externo approach was successfully performed in all 10 eyes. The mean patient age was 10.8 ± 3.2 years (range, 5-16 years). Preoperative hypotony (intraocular pressure <6 mmHg) was present in three eyes. Postoperatively, visual acuity improved in four patients, remained stable in four, and worsened in two. No cases of postoperative epiretinal membrane formation or significant subretinal hemorrhage were observed. Retinal reattachment was achieved in 9 of 10 eyes (90%), while one eye progressed to pre-phthisis. CONCLUSIONS:Ab externo submacular band removal appears to be a useful adjunct to pars plana vitrectomy in selected pediatric RRD cases with PVR, allowing relief of submacular traction without the need for retinotomy.
PURPOSE:To evaluate the anatomical and functional outcomes of an epiretinal cryopreserved human amniotic membrane (hAM) overlay technique for the management of recurrent retinal detachment (RD) associated with peripapillary breaks in highly myopic eyes. METHODS:Retrospective interventional case series including five eyes with recurrent RD (mean axial length 30.70 ± 2.03 mm). Two eyes presented with recurrence under silicone oil (SO) tamponade, and three after prior SO removal. All eyes underwent 25-gauge pars plana vitrectomy with SO removal when present. A customized 8-9 mm hAM patch was positioned epiretinally and unrolled bimanually under perfluorocarbon liquid (PFCL). Final tamponade was achieved with SO 1000 cSt, followed by scheduled SO removal. RESULTS:Complete retinal reattachment was achieved in all cases (100%) after a single procedure and remained stable throughout follow-up. Mean best-corrected visual acuity (BCVA) improved from 1.00 ± 0.60 to 0.68 ± 0.58 logMAR at 2 months after SO removal (p = 0.068). Optical coherence tomography demonstrated stable integration of the hAM as a hyperreflective layer over the peripapillary defects. No cases of graft dislocation, proliferative vitreoretinopathy, or significant inflammatory complications were observed. CONCLUSIONS:The epiretinal hAM overlay technique appears to be a safe and effective approach for sealing complex peripapillary breaks in highly myopic eyes with chorioretinal atrophy. By providing both mechanical support and a biological scaffold for tissue repair, this technique may represent a valuable surgical option in cases where conventional retinopexy is not feasible or has failed.
Purpose: To describe a hybrid surgical sequence using multi-flap free internal limiting membrane (ILM) grafting with sub-perfluoro-n-octane (PFO) stabilization for repair of a persistent large macular hole (MH) after prior ILM peeling. Methods: A 70-year-old pseudophakic woman presented with a persistent large full-thickness MH in the right eye two months after pars plana vitrectomy with ILM peel and sulfur hexafluoride (SF6) tamponade (BCVA 20/200). Revision surgery was performed with ILM staining using indocyanine green, followed by the creation of three small, hinged ILM flaps from the edge of the prior peel border in balanced salt solution. PFO was then injected over the entire posterior pole. Under the PFO bubble, the hinged flaps were converted to multiple free ILM graft fragments, transferred sub-PFO, and layered within the MH crater to augment scaffold volume. Fluid-air exchange was completed followed by 15% perfluoropropane (C3F8) gas tamponade with intraoperative confirmation of graft retention. Postoperative positioning included upright positioning during the day and side sleeping at night. Results: Intraoperative visualization demonstrated stable retention of the ILM graft fragments within the MH after fluid-air exchange. At postoperative month 5, optical coherence tomography confirmed complete MH closure, and BCVA improved from 20/200 to 20/50 in the operative eye. Conclusion: Multi-flap free ILM grafting performed with sub-PFO transfer and stabilization offers enhanced fluidics control and mechanical graft retention during fluid-air exchange, providing a practical technique for refractory MH repair when juxtafoveal ILM is exhausted after prior peeling.
Purpose: To describe a case of acute, severe bilateral foveolysis following systemic ifosfamide therapy and the subsequent anatomical and functional recovery after discontinuation of the drug and administration of metabolic therapy targeting mitochondrial dysfunction. Methods: The patient had a comprehensive ophthalmological examination including high resolution optical coherence tomography. After cessation of ifosfamide, she was treated with nicotinamide adenine dinucleotide 850 mg, alpha lipoic acid 600 mg, and coenzyme Q10 100 mg daily. Results: A 34-year-old woman receiving ifosfamide for an inoperable skull-base chordoma developed rapid bilateral 20/60 visual acuity. Optical coherence tomography revealed complete dissolution of the central fovea in each eye with accumulation of necrotic debris centrally. Given the known pathway of ifosfamide neurotoxicity, mitochondrial complex I inhibition with depletion of nicotinamide adenine dinucleotide (NAD), the patient received high-dose oral NAD, coenzyme Q10, and alpha-lipoic acid. Over the next several weeks, both eyes showed symmetrical reconstitution of the fovea with centripetal migration of the outer nuclear layer and eventual restoration of outer retinal architecture. By 12 weeks, foveal structure was normal and visual acuity improved to 20/25 and 20/20. Conclusions: Systemic ifosfamide was associated with an acute symmetrical dissolution of the central fovea, termed foveolysis. Although spontaneous improvement after drug withdrawal cannot be excluded, the rapid and near-complete recovery after metabolic therapy supports a mechanistic link between ifosfamide-induced mitochondrial dysfunction, NAD depletion, and reversible foveal injury. The foveal architecture was restored through the defined sequence of foveation, demonstrating the capacity of the adult fovea for organized structural reconstitution.
PURPOSE:To report the management of traumatic submacular hemorrhage (SMH) in a highly myopic eye using early pars plana vitrectomy and low-dose subretinal recombinant tissue plasminogen activator (rtPA) delivered with a console-controlled micro-dosing system under microscope-integrated optical coherence tomography (iOCT) guidance. METHODS:A 36-year-old man with high myopia (-15.00 D) presented with acute traumatic SMH and severe visual loss. He underwent combined phacoemulsification and pars plana vitrectomy with internal limiting membrane peeling. Subretinal rtPA (25 μg/0.1 mL) was delivered through a 38-gauge cannula using a viscous fluid control-based injector assembled from standard sterile components. iOCT was used to confirm retinotomy position, subretinal placement, and bleb formation. Fluid-air exchange and 12% C3F8gas tamponade completed the procedure. RESULTS:The hemorrhage displaced progressively and resorbed over follow-up. Best-corrected visual acuity improved from counting fingers at presentation to 0.4 at 1 month and 0.7 at 3 months, remaining stable at 6 months. Optical coherence tomography showed restoration of foveal contour with persistent outer retinal disruption. No intraoperative or postoperative complications were observed. CONCLUSION:In this case, iOCT-guided console-controlled micro-dosing enabled controlled subretinal rtPA delivery and effective displacement of traumatic SMH using nonproprietary instrumentation. This workflow may be useful when dedicated commercial microinjectors are unavailable.
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.
