The Silent Masquerade: Clinical and Imaging Features of Asymptomatic Vitreoretinal Lymphoma.

PURPOSE: To report and characterize ocular features of asymptomatic vitreoretinal lymphoma (VRL) associated with primary central nervous system lymphoma (PCNSL), by examining clinical and multimodal imaging characteristics and comparing with symptomatic VRL. DESIGN: Retrospective cross-sectional study. METHODS: Patients with cytologically or molecularly confirmed VRL were included. Patients were classified into three groups: primary VRL (PVRL), symptomatic VRL associated with PCNSL (PCNSL-S), or asymptomatic VRL associated with PCNSL (PCNSL-AS). Data encompassing demographics, visual symptoms, visual acuity (VA), and imaging characteristics were collected. Cross-sectional analyses of quantitative and categorical variables among groups were performed with one-way ANOVA and multinomial linear regression analyses. RESULTS: The study included 104 eyes from 56 patients with VRL. Twenty-nine patients (52%) were diagnosed with PVRL, and 27 patients (48%) were diagnosed with VRL associated with PCNSL. Among these, 17 (63%) reported visual symptoms (PCNSL-S), whereas 10 (37%) were asymptomatic (PCNSL-AS). PCNSL-AS patients exhibited better VA than PVRL patients (0.11 vs 0.76 LogMAR, P = 0.04) and distinct clinical features, with lower rates of anterior segment involvement (odds ratio [OR] = 0.02; 95% confidence interval [CI] 0.12-0.84; P < .01) and vitritis (OR = 0.32; 95% CI 0.11-0.91; P = .03). Subretinal infiltration was less common in PCNSL-AS cases compared to PVRL (OR = 0.14; 95% CI 0.02-1.11; P = 0.06) and PCNSL-S (OR: 0.08; 95% CI 0.01-0.69 P = 0.05) and was associated with worse VA (estimate = 0.55 LogMAR; 95% CI 0.29-0.8; P < .01). CONCLUSIONS: This study describes distinctive clinical and imaging features of asymptomatic VRL associated with PCNSL, characterized by better VA and less severe ocular involvement. The findings highlight the pivotal role of multimodal imaging in facilitating early detection of VRL in the staging of PCNSL. Future guidelines for PCNSL management should consider the necessity of diagnosing patients with asymptomatic VRL.

Unraveling the Spectrum of Uveitis: Insights from an Epidemiological Study in a National Referral Center in Northern Italy.

PURPOSE: Uveitis embraces a heterogeneous group of vision-threatening inflammatory conditions. Understanding uveitis epidemiology, etiology, and clinical findings is fundamental for a prompt diagnosis and optimal patient management. The aim of the study is to report the epidemiology of uveitis in a national referral center in Northern Italy and investigate the visual prognosis. METHODS: This retrospective study was conducted at Uveitis Service (Ospedale San Raffaele) between June 2016 and May 2023. Demographic data, clinical characteristics, and etiological diagnoses of uveitis patients were collected, and visual prognosis was longitudinally explored. RESULTS: 1105 patients with uveitis were included in the study, while 47 patients presented neoplastic masquerade syndrome and have been excluded. The population had a slight majority of females (M/F ratio = 0.76), mean age was 47 years. 25% presented infectious uveitis, primarily due to herpetic etiology, toxoplasmosis, and tuberculosis. Non-infectious uveitis was the most prevalent diagnosis (38%), with sarcoidosis, HLA-B27-associated uveitis, and Fuch's uveitis as prominent causes. Anatomically, anterior segment was most frequently involved (41%). Significant improvement in visual acuity was observed at follow-up, particularly in patients with infectious uveitis. CONCLUSIONS: Our study sheds light into the epidemiological landscape of uveitis in Northern Italy, reflecting changing patterns due to factors such as migration and changing sexual habits. In particular, higher percentages of syphilis have been observed, compared to other European reports. The distribution of non-infectious uveitis reflects other epidemiological European series. Higher percentages of neoplastic masquerade syndromes support the need of early recognition. Our findings offer precious insights for uveitis epidemiology and daily clinical practice.

Ocular complications of SAVI: A unique case of bilateral uveitis and retinal vasculitis.

Purpose: To describe a case of bilateral panuveitis in a patient with Stimulator of Interferon Genes (STING)-Associated Vasculitis with Onset in Infancy (SAVI). Observations: A 45-year-old patient diagnosed with SAVI presented bilateral panuveitis and uncontrolled secondary intraocular hypertension due to structural complications from uveitis. Multimodal imaging revealed the presence of intraretinal fluid and bilateral vasculitis. The patient was started with systemic methotrexate. Conclusions and importance: This case is essential to characterize ocular involvement in patients with SAVI. Awareness of these ocular manifestations is crucial for timely management and improvement of visual prognosis.

Anterior segment involvement in vitreoretinal lymphoma: clinical manifestations, molecular findings and in vivo confocal microscopy.

BACKGROUND: Intermediate and posterior manifestations of vitreoretinal lymphoma (VRL) are well characterised. However, there is limited information on anterior segment involvement in VRL. This study aimed to describe the anterior manifestations of VRL, and their association with molecular testing. METHODS: Retrospective analysis of patients with biopsy-proven VRL. Study variables included anterior segment manifestations, findings from slit-lamp photos and in vivo confocal microscopy (IVCM) when available. MYD88 L265P mutation and cytology in the aqueous humour, retinal and systemic findings were also analysed. RESULTS: The analysis included 108 eyes of 55 VRL patients. Anterior segment involvement was present in at least one visit in 55 eyes (51%) of 33 patients (60%); it included keratic precipitates (dendritiform with branching and irregular margins in 33 eyes, dust-like in 16 eyes and large granulomatous in 12 eyes), cells in the anterior chamber (51 eyes) and posterior synechiae (2 eyes). IVCM was available for 41 eyes and showed different morphologies of keratic precipitates, including floral, spikes and mulberry patterns (66%, 56% and 20%, respectively). MYD88 L265P mutation in the aqueous humour was detected in 10/21 (48%) eyes with no anterior segment involvement and 24/37 (65%) eyes with anterior segment involvement. CONCLUSIONS: Anterior segment manifestations are often present in VRL and include dendritiform and dust-like keratic precipitates. IVCM in VRL can identify different patterns associated with keratic precipitates. MYD88 L265P mutation in the aqueous humour of VRL patients can also be found in eyes without significant anterior segment involvement.

A comprehensive overview of diagnosis, imaging and treatment of vitreoretinal lymphoma.

Vitreoretinal lymphoma (VRL) is a rare B-cell intraocular neoplasia characterized by poor long-term prognosis and lack of effective therapies. It mainly involves the vitreous humor, the retina, and the retinal pigment epithelium (RPE), although anterior segment involvement can occur. VRL is classified as a lymphoma of immune privileged sites, along with testis lymphoma and primary central nervous system lymphoma (PCNSL). VRL and PCNSL are strictly connected indeed: 80% of VRL develop PCNSL, while 20% of patients with PCNSL present VRL during natural history of lymphoma. Due to the lack of worldwide consensus about diagnosis, therapy, and follow-up timing, VRL represents one of the most challenging ocular affections.VRL commonly masquerades as a posterior uveitis, and misdiagnosis often occurs because of partial response to topical steroids. Gold standard for diagnosis is cytological analysis of vitreous humor. However, this technique lacks sensitivity and supplemental molecular analyses can improve the diagnostic process. Multimodal imaging allows ophthalmologists to empower their clinical suspicion and a comprehensive examination can highlight typical features of VRL and justify further invasive procedures.There is no consensus about VRL therapy, and none of the therapeutical scheme has demonstrated to prevent cerebral involvement and improve patient's overall survival. Intravitreal injections of chemotherapeutics drugs, ocular radiation therapy and systemic chemotherapy can be considered in the treatment of VRL. Once cerebral involvement occurs, systemic chemotherapy must be included in the treatment as a life-saving therapy. Further multicentric studies are required to find out the best treatment of patients with VRL.

Acute and Chronic Manifestations of Sympathetic Ophthalmia on Multimodal Imaging.

PURPOSE: To report the clinical and multimodal imaging features of sympathetic ophthalmia in the acute and chronic phases. METHODS: Retrospective cohort study of consecutive patients with sympathetic ophthalmia seen at a tertiary referral center. Charts, imaging studies, and histopathological specimens were reviewed. The clinical features and multimodal imaging in the sympathizing eye were analyzed by sorting features into those seen in the acute and chronic phase. RESULTS: Ten patients were included in the analysis and all of them had previous ocular trauma or complicated retinal detachment. In the acute phase, 70% had anterior uveitis, 70% had vitritis, and 100% had active posterior uveitis; posterior uveitis included multifocal choroiditis (80%), optic disc swelling (40%), multiple serous retinal detachments (20%), MEWDS-like findings (10%), and retinal vasculitis with chorioretinitis (10%). In the chronic phase, posterior manifestations included widespread patches of chorioretinal atrophy in the mid- and far-periphery (80%), peripapillary subretinal fibrosis (50%), and nummular perivascular atrophy (50%). CONCLUSIONS: Sympathetic ophthalmia shows different posterior segment manifestations in the acute and chronic phase. Active sympathetic ophthalmia should be ruled out in eyes with a MEWDS-like presentation or rapidly progressing chorioretinitis, and history of trauma in the fellow eye. Peripapillary subretinal fibrosis and perivascular nummular atrophy may be useful features to suspect SO once acute inflammation has resolved.

UNUSUAL CASE OF CLADOSPORIUM SPHAEROSPERMUM ENDOGENOUS ENDOPHTHALMITIS DURING GOLIMUMAB THERAPY: CASE REPORT AND LITERATURE REVIEW.

PURPOSE: To report a case of fungal endogenous endophthalmitis from Cladosporium sphaerospermum in a patient with juvenile idiopathic arthritis receiving chronic immunosuppressive therapy with golimumab (tumor necrosis factor-α blocker). METHODS: Case report and review of the literature. RESULTS: A 34-year-old woman receiving chronic immunosuppressive therapy with golimumab for juvenile idiopathic arthritis was referred for unilateral visual loss and ocular pain. Worsening conditions after corticosteroid therapy and raised serum beta-D-glucan levels pointed to an infectious fungal etiology. Panfungal polymerase chain reaction-based genetic sequencing on vitreous specimens obtained during vitrectomy detected C. sphaerospermum. The patient management combined surgical treatment and systemic and intravitreal voriconazole. CONCLUSION: Endogenous fungal endophthalmitis can be a rare complication in patients undergoing chronic immunosuppressive therapy (including golimumab) without other predisposing factors. Prompt diagnosis and appropriate treatment are the keys to preserve vision.

Diagnostic and therapeutic results of aqueous real-time polymerase chain reaction in infectious uveitis.

OBJECTIVE: This study was aimed primarily at describing the results of aqueous real-time polymerase chain reaction (RT-PCR) and reporting the rate of therapeutic modifications directly attributable to this procedure (profitability). Our secondary outcome was to compare demographic and clinical characteristics between patients with RT-PCR positivity and those with RT-PCR negative results. DESIGN: Retrospective observational study conducted at the Uveitis Service of San Raffaele Hospital (Milan, Italy) between November 2016 and July 2022. PARTICIPANTS: Patients with infectious uveitis suspect (anterior, intermediate, posterior uveitis, or panuveitis). METHODS: Patients with suspected infectious uveitis underwent aqueous RT-PCR for detection of herpes simplex 1 (HSV-1), herpes simplex 2 (HSV-2), varicella zoster virus (VZV), cytomegalovirus (CMV), and Toxoplasma gondii. RESULTS: Sixty-five eyes of 61 patients (60 ±16 years of age; 54% males) were included. Aqueous RT-PCR tested positive in 58% and negative in 42% of patients. CMV and HSV-1 were the most frequently detected pathogens. RT-PCR confirmed clinical suspicion in 38% of patients and altered the presumed etiologic diagnosis and treatment in 20% of patients. Profitability was associated with CMV positivity. HSV-1 positivity was related to iris atrophy. CMV positivity was correlated with keratic precipitates. Vitritis and retinitis were related to VZV, CMV, and T. gondii detection. Synechiae, retinitis, and neuritis were related to positive tests regardless of the pathogen investigated. Early complications related to paracentesis were rarely reported. CONCLUSION: Aqueous RT-PCR was a safe semi-invasive tool to confirm a presumptive diagnosis and to change initial suspicion in ambiguous cases of herpetic uveitis. Thus aqueous RT-PCR may alter therapeutic management.

Panuveitis of undetermined origin after diagnostic pars plana vitrectomy: clinical characterization and long-term outcome from a tertiary referral center.

PURPOSE: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy. METHODS: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported. RESULTS: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival. CONCLUSIONS: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function.

CLINICAL CHARACTERISTICS AND MULTIMODAL IMAGING FINDINGS IN UNILATERAL FROSTED BRANCH ANGIITIS: A CASE REPORT.

PURPOSE: Our aim is to report a comprehensive multimodal imaging case of unilateral frosted branch angiitis in a 40-years-old Caucasian female . METHODS: Case report involving clinical examination, ultra- wide field fundus photograph, ultra-wide field fluorescein angiography (UWFA), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). RESULTS: A 40 years old patients presented with unilateral acute vision loss. On fundus examination, extensive retinal veins sheathing, macular edema and vascular congestion were observed while UWFA revealed an hyperfluorescent "hot" optic disc and blood retinal barrier disruption. OCTA displayed foveal avascular zone (FAZ) enlargement and excluded papillary neovascularization. Extensive laboratory work-up for infectious, autoimmune and inflammatory disorders were negative, thus, a diagnose of acute idiopathic unilateral frosted branch angiitis was made. Intravitreal injection of dexamethasone implant was administered with a good clinical response. CONCLUSIONS: Multimodal imaging is crucial to correctly diagnose and treat FBA. Up to our knowledge, the use of OCTA as a complementary tool to the diagnostic process in FBA has been described in literature just once as a photo essay of cytomegalovirus-related FBA and it might be of great value for better characterizing clinical features of this disorder and for following disease activity in a non-invasive fashion.

Imaging biomarkers of leukaemic choroidopathy.

PURPOSE: To longitudinally investigate choroidal and choriocapillaris perfusion metrics and the number of choroidal hyperreflective foci (HRF) in patients with acute leukaemia (AL) before and after disease remission and to correlate these metrics with systemic parameters during active disease. METHODS: Prospective, longitudinal study of 26 eyes of 14 AL patients. All patients underwent optical coherence tomography (OCT) and OCT-angiography (OCTA) in the acute phase. Subfoveal choroidal thickness (CT), total, luminal and stromal choroidal area (TCA, LCA, SCA), choroidal vascularity index (CVI), choriocapillaris flow deficits (cFD) density, and choroidal HRF number were computed. In a subset, the measurements were repeated after AL remission. Age- and gender-matched 26 healthy controls were recruited for cross-sectional comparisons. RESULTS: Patient's mean age was 59 ± 12 years. The TCA, LCA, SCA and choroidal HRF number were significantly higher in patients than controls (p = 0.028, p = 0.044, p = 0.024 and p = 0.001, respectively). Lower haemoglobin levels were associated with lower CT (r = 0.58, p = 0.008). Higher D-dimer values were associated with lower TCA (r = -0.52, p = 0.008), lower LCA (r = -0.50, p = 0.006), higher cFD density (r = 0.41, p = 0.044) and higher choroidal HRF number (r = 0.47, p = 0.008). The CT, TCA, SCA and choroidal HRF number significantly reduced after AL remission (p = 0.001, p = 0.047, p = 0.007 and p = 0.002 respectively). The CVI increased significantly compared to the active phase (p = 0.013). CONCLUSION: The study demonstrates a subclinical choroidal involvement in patients with AL, with relative stromal thickening in the acute phase, and normalization after disease remission. Choroidal HRF were identified as a biomarker of leukaemic choroidopathy. Choriocapillaris and choroidal vascularity were inversely correlated with a systemic pro-coagulant state.

Case report: Intravitreal methotrexate in intraocular acute lymphoblastic leukemia.

Direct leukemic infiltration of the eye is most frequently associated with acute lymphoblastic leukemia (ALL), probably due to its well-known central nervous system (CNS) tropism. Systemic treatment alone may not be sufficient for intraocular leukemia. Data on local treatment are scarce. Here, we present two cases of intraocular ALL treated with intravitreal methotrexate (MTX). Initially, anatomical improvement and visual stability were observed. The first patient experienced anatomical and visual worsening after a year of treatment. Treatment was withheld after 2 months for the second patient due to poor systemic conditions. Corneal toxicity and intraocular pressure elevation were observed in the first case. In both cases, eye involvement was associated with CNS or systemic relapse. This highlights the importance of incorporating ocular disease management in a comprehensive approach to therapy. Our experience corroborates previous findings on MTX injections as an effective and safe therapeutic option for intraocular leukemia. Further evidence is needed to consolidate the use of intravitreal MTX to treat such a debilitating localization of leukemia.

RETINAL MICROVASCULAR CHANGES IN PATIENTS WITH ACUTE LEUKEMIA.

PURPOSE: To evaluate the retinal circulation in patients with active acute leukemia, to correlate the perfusion metrics with systemic laboratory values, and to assess the vascular perfusion after leukemia remission. METHODS: Longitudinal study of 22 eyes from 12 patients with acute leukemia; healthy eyes were recruited as control subjects. All patients underwent optical coherence tomography angiography at baseline. Optical coherence tomography angiography was repeated in case of morphologic leukemia remission. RESULTS: Patients' age ranged from 37 to 74 years. All participants had a 20/20 vision. In all leukemic eyes, optical coherence tomography angiography detected vascular alterations in the macula and the peripapillary region. Vessel density values in the superficial capillary plexus were lower in patients with leukemia than control subjects (46.8 ± 3.6 vs. 49.2 ± 2%, P = 0.08), irrespective of the presence of leukemic retinopathy (7 eyes, 32%). Lower vessel density was associated with lower white blood cells ( P = 0.09) and lower platelets ( P = 0.001). Reappearance of small capillaries, increase in vessel density, reduction in vessel diameter, and increase in fractal dimension were seen after remission. CONCLUSION: Subclinical, reversible reduction in vessel density and complexity on optical coherence tomography angiography occurs in patients with active acute leukemia and is presumably associated with bone marrow function failure. Further studies are warranted to explore its functional and prognostic significance.

Hemorrhagic Mass-Like Presentation of Vitreoretinal Lymphoma.

Purpose: The aim of this study was to report the presentation, treatment, and outcomes of vitreoretinal lymphoma (VRL) associated with hemorrhagic mass-like lesions (HMLs) in the retina. Methods: This study was a retrospective analysis of patients with HMLs associated with VRL seen at a single tertiary referral center. For each patient, the clinical charts, the fundus imaging, and the treatment outcomes were reviewed. Results: Three eyes of 2 patients had VRL with HMLs. In all study eyes, HMLs were preceded by an area of retinitis-like retinal infiltration and evolved into elevated hemorrhagic masses. Two eyes had multiple relapses with HMLs. All HMLs regressed with treatment and were replaced by extensive chorioretinal atrophy. Conclusion: VRL can present with HMLs. HMLs seem to correspond to massive intraretinal infiltration by VRL, mimicking a solid mass. Despite response to therapy, HMLs are associated with poor anatomical and functional outcomes.

CHORIORETINAL ATROPHY IN VITREORETINAL LYMPHOMA: Risk Factors and Visual Outcomes.

PURPOSE: To investigate the frequency, risk factors, and functional prognosis of chorioretinal atrophy (CRA) in vitreoretinal lymphoma (VRL). METHODS: This was a retrospective cohort study of consecutive patients with vitreoretinal lymphoma. The demographic, clinical, and retinal features and the treatment modalities of each patient were collected. The charts and the multimodal imaging at each visit were reviewed. The risk factors associated with CRA were investigated with a mixed-model Cox regression. RESULTS: Of the 79 eyes of 40 patients included, 41 eyes (52%) had CRA; 27 and 14 eyes had focal and diffuse CRA, respectively. The rate of vitreoretinal lymphoma lesions in the macula was similar between focal and diffuse CRA (96% vs. 93%). The eyes with CRA had worse best-corrected visual acuity (P = 0.006) than eyes with no CRA; diffuse atrophy had the worst best-corrected visual acuity (P < 0.001). The presence of retinal infiltrates (hazard ratio = 3.75, 95% confidence interval [CI] 1.46-9.59, P = 0.006) and vertical hyperreflective lesions (hazard ratio= 4.13 95% CI 1.14-14.93, P = 0.03) on optical coherence tomography and macular involvement (hazard ratio = 6.59, 95% CI 1.41-30.53, P = 0.02) were associated with a higher risk of CRA. CONCLUSION: Vitreoretinal lymphoma presenting with retinal infiltrates and macular involvement carried a higher risk of CRA. Risk factors for CRA should be identified for the potential of severe visual loss. Prompt diagnosis of vitreoretinal lymphoma may allow better control of the disease.

Efficacy of 0.19 mg Fluocinolone Acetonide Implant in Non-infectious Posterior Uveitis Evaluated as Area Under the Curve.

INTRODUCTION: This study reports the outcomes of fluocinolone acetonide intravitreal implant (FAc, Iluvien®, SIFI, Italy) in patients affected by macular edema secondary to chronic non-infectious uveitis of the posterior segment (NIU-PS). METHODS: This was a retrospective study of patients with NIU-PS and macular thickening undergoing FAc implant at San Raffaele Hospital (Milan, Italy). Clinical data, including best-corrected visual acuity (BCVA), intraocular pressure (IOP), and central macular thickness (CMT), were collected at the time of FAc administration (baseline) and at 1, 6, and 12 months. The area under the curve (AUC) of the BCVA (AUCBCVA) and CMT (AUCCMT) was correlated with baseline factors; ß estimates and 95% confidence interval (CI) are provided. RESULTS: Ten eyes of seven patients (60 ± 12 years; 4 male, 57%) were included. The BCVA significantly improved from month 6 (p = 0.03). The CMT improved from month 1 and was persistently lower than baseline until month 12 (p < 0.001). The AUCBCVA correlated with baseline BCVA (ß = 2.5 logMAR; 95% CI 1.59-3.41; p < 0.001), while the mean AUCCMT positively correlated with the baseline CMT (ß = 2.1 µm; 95% CI 0.41-3.80; p = 0.02). No adverse events were recorded over 1 year. CONCLUSIONS: Better visual acuity at the time of FAc administration was associated with better visual function after FAc. Less severe macular edema correlated with better anatomic response. The FAc implant was a safe option for resolving macular edema secondary to NIU-PS.

MULTIMODAL IMAGING AND TREATMENT OF SYPHILITIC CHOROIDAL NEOVASCULARIZATION.

PURPOSE: To report a rare case of choroidal neovascularization (CNV) developed 2 years after successful treatment of ocular syphilis, identified by optical coherence tomography angiography. METHODS: Case report. RESULTS: A 31-year-old man with a history of syphilitic chorioretinitis developed a CNV 2 years after clinical remission of the infection. Structural optical coherence tomography (OCT) and optical coherence tomography angiography were helpful in providing detailed evidence of an extrafoveal CNV in an easy and noninvasive way. In comparison, the identification of CNV on fluorescein angiography was difficult because of the retinal blood barrier breakdown and intense choroidal background fluorescence for diffuse chorioretinal scarring of syphilitic chorioretinitis. The patient underwent 3 intravitreal injections of anti-vascular endothelial growth factor in addition to 25 mg/day of oral prednisone, with the restoration of previous visual acuity. CONCLUSION: Choroidal neovascularization is a rare, but sight-threatening complication of syphilitic chorioretinitis. The combination of different imaging modalities, and in particular optical coherence tomography angiography, allowed reaching a definite diagnosis of CNV. Combined treatment of systemic steroid and intravitreal anti-vascular endothelial growth factor was effective in controlling the CNV and improving the visual outcome.

MULTIPLE RETINAL PIGMENT EPITHELIUM APERTURES ASSOCIATED WITH PSEUDO-VITELLIFORM LYMPHOMATOUS MACULOPATHY.

PURPOSE: To describe the case of a 66-year-old man with bilateral cytologically proven vitreoretinal lymphoma who presented with a pseudo-vitelliform macular lesion and multiple retinal pigment epithelium apertures. METHODS: The patient underwent comprehensive ophthalmologic evaluation, including best-corrected visual acuity, intraocular pressure, anterior segment and fundus examination, and optical coherence tomography, at baseline and during follow-up. RESULTS: A new-onset foveal yellow, ill-defined lesion was noticed in the right eye during the follow-up; best-corrected visual acuity was 20/20, and the patient was asymptomatic. The lesion was isoautofluorescent with the surrounding retina. The optical coherence tomography revealed hyperreflective subretinal material, an irregularly thickened retinal pigment epithelium, and a shallow, hyperreflective retinal pigment epithelium detachment with multiple retinal pigment epithelium apertures. After 15 days, the lesion was completely reabsorbed and replaced by outer retinal atrophy and cystoid macular edema; best-corrected visual acuity dropped to 20/100. CONCLUSION: The macular lesion may be a sign of macular infiltration, a case of paraneoplastic cloudy vitelliform submaculopathy, or coexistence of both. Retinal pigment epithelium apertures are not disease-specific and are described in vitreoretinal lymphoma for the first time.