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.

Clinical Experience in a Large Cohort of Patients with Vitreoretinal Lymphoma in a Single Center.

Background/aims: To report our five-year experience on vitreoretinal lymphoma (VRL) as a single-center tertiary hospital. Methods: The ophthalmic, cytopathology, and onco-hematologic records of patients with VRL consecutively seen from 2014 to 2019 were reviewed. Results: Fifty-nine eyes of 31 patients with large B-cell VRL were included. Eighty-one percent has developed central nervous system lymphoma at the end of follow-up. Several different imaging findings were noted, including vitritis, leopard spot appearance, Bruch's membrane/RPE infiltrations, and ellipsoid zone disruption. A variable combination of MYD88-L265P mutation in the aqueous and/or in the vitreous and positive cytology/histology allowed to reach a definite diagnosis in all the patients. Therapies included intravitreal injections of methotrexate and rituximab, systemic chemotherapy, pan-encephalic radiotherapy, and hematopoietic stem cell transplantation. Conclusion: No definite guidelines exist for VRL management. It is crucial to collect as much data as possible from tertiary referral hospitals, which suitably manage a conspicuous number of VRL patients.

Management of Juvenile idiopathic arthritis-associated uveitis duringthe COVID-19 pandemic in a pediatric referral center in Lombardy.

Italy was the first European country to be affected by the SARS-CoV-2 pandemic. In this scenario, we had to face a new clinical approach in our Pediatric Rheumatology Unit for the management of patients affected by juvenile idiopathic arthritis (JIA)-associated uveitis. During the lockdown (phase 1), the weekly outpatient clinic was discontinued and telephone consultations were set up. A toll-free telephone number was instituted for emergencies. None of our children with JIA-associated uveitis was advised to stop the ongoing immunosuppressant systemic therapy. We had no cases of COVID-19 infection and uveitis activity was under control in all but two out of 125 patients, which was comparable with the pre-COVID-19 situation. During phase 2 of the pandemic, hospital and ambulatory rearrangements were made to minimize the risk of SARS-CoV-2 infection. Overall, during the first 4 weeks of phase 2, we did not notice an increased number of patients with uveitis activity.

Widefield OCT angiography and ultra-widefield multimodal imaging of Susac syndrome.

The aim is to present the changes in ultra-widefield and widefield multimodal imaging, including optical coherence tomography angiography of a 33-year-old woman diagnosed with Susac syndrome, over 1 year of follow-up. Fundus examination and multimodal imaging revealed bilateral arterial occlusion of multiple vascular branches with retinal ischemia. Over 1 year follow-up, best-corrected visual acuity improved while retinal ischemia gradually resolved. Widefield optical coherence tomography angiography showed reperfusion of macular large vessels, but not of the small capillaries. Despite anatomical improvement, functional defects of the visual field persisted. In conclusion, widefield and ultra-widefield imaging provided high-resolution details of the central and peripheral damages in Susac syndrome.

MYD88 L265P MUTATION DETECTION IN THE AQUEOUS HUMOR OF PATIENTS WITH VITREORETINAL LYMPHOMA.

PURPOSE: To detect the presence of MYD88 L265P mutation in the aqueous humor of patients with cytologically proven vitreoretinal lymphoma. METHODS: Eight consecutive patients with bilateral vitreoretinal lymphoma (16 eyes) were prospectively evaluated. Genomic DNA was extracted from aqueous samples after paracentesis and vitreous humor samples after diagnostic vitrectomy. MYD88 codon 265 mutation was investigated by both amplification-refractory mutation system polymerase chain reaction approach and pyrosequencing assay in the aqueous humor of all patients and in the vitreous of 6 patients. A control group of 8 age-matched patients with established diagnosis of noninfectious uveitis was also tested for the presence of MYD88 L265P mutation in the aqueous humor. RESULTS: Eight patients (three men, five women) with mean age of 69.5 years (range 50-85 years) were considered. All the patients tested for MYD88 L265P in the vitreous (six) were positive, and this result was consistent with cytological examination in all samples but one. The MYD88 L265P mutation was found in the aqueous of 6 patients (75%), and in 3 of them, the mutation was present in both eyes. Results of MYD88 L265P mutation in aqueous and vitreous sample were consistent in 7 of the 8 eyes with available samples. The aqueous humor of the noninfectious uveitis control group was negative for the detection of MYD88 L265P mutation. CONCLUSION: MYD88 mutation was detected in the aqueous humor of 75% of patients with cytologically proven vitreoretinal lymphoma. This technique may be considered as an additional diagnostic tool in the detection of the disease.