IMMUNOGLOBULIN G4-RELATED OPHTHALMIC DISEASE MIMICKING INTRAOCULAR LYMPHOMA: A CASE REPORT.

PURPOSE: To describe a case of immunoglobulin G4-related choroiditis mimicking intraocular lymphoma. METHODS: The patient underwent a complete ophthalmological evaluation including multimodal imaging, with structural optical coherence tomography, fluorescein angiography, indocyanine green angiography, ultra-widefield color, and autofluorescent fundus photographies to assess the ocular involvement. RESULTS: Patient's best-corrected visual acuity was of 20/25 in the right eye and 20/20 in the left eye. Fundus appearance showed abnormal yellowish choroidal lesions and moderate vitritis in both eyes. Fluorescein angiography was within normal limits, whereas indocyanine green angiography showed areas of choroiditis in both eyes, and structural optical coherence tomography scans disclosed retinal small roundish lesions in the corresponding regions. Laboratory examinations and lymph node biopsy led to the final diagnosis of immunoglobulin G4-related disease. CONCLUSION: We describe a case of immunoglobulin G4-related choroiditis mimicking intraocular lymphoma. The proper use of multimodal imaging associated with laboratory investigations was helpful to reach the correct diagnosis.

Bilateral acute retinal necrosis during treatment with alemtuzumab for multiple sclerosis.

INTRODUCTION: Alemtuzumab is a humanized monoclonal antibody used as a treatment of multiple sclerosis (MS) and chronic lymphocytic leukemia. It decreases T cell count leading to significant immunosuppression, with increased risk of systemic and ocular infections. Herein, we report a unique case of bilateral acute retinal necrosis (ARN) caused by varicella-zoster virus (VZV) in a patient affected by MS under treatment with alemtuzumab. CASE DESCRIPTION: A 36-year-old man with a relapsing-remitting MS under treatment with alemtuzumab developed bilateral visual loss. Anterior segment examination displayed granulomatous keratic precipitates and 3+ cells in the anterior chamber, while fundoscopy showed bilateral 1+ vitritis and peripheral retinal necrosis, complicated by retinal detachment in the left eye. The high viral load for VZV in aqueous humor samples had a univocal interpretation for viral reactivation. In addition to systemic therapy with acyclovir, the patient was treated with bilateral intravitreal injections of foscarnet and underwent pars-plana vitrectomy and silicone oil tamponade for retinal detachment in the left eye. CONCLUSION: This report shows a unique case of bilateral ARN caused by VZV associated with alemtuzumab. Any visual loss in MS patients under biologic therapy should not be underestimated, performing an accurate differential diagnosis with optic neuritis.

The importance of clinical presentation on long-term outcomes of external dacryocystorhinostomies: Our experience on 245 cases.

PURPOSE: External dacryocystorhinostomy (EXT-DCR) is a surgical approach commonly used to treat post-canalicular acquired lacrimal obstruction whose success rate has been described, equally with endoscopic DCR, to be superior to other available treatments. METHODS: At San Raffaele Hospital, Milan (Italy), from January 2008 to December 2020, 245 EXT-DCRs were performed. All patients underwent routine pre-operative work-up including Jones tests and probing and irrigation of the lacrimal pathway; when necessary, a multidisciplinary approach with nasal endoscopy was performed. The surgical approach was followed by positioning of a bicanalicular stent which was left in place for 6 months. Success was defined as resolution of clinical signs/symptoms and free lacrimal flow on functional test. RESULTS: Of the cases enrolled, 26.9% were treated for recurrent epiphora (group 1), and 73.1% for epiphora associated with chronic dacryocystitis (group 2). After a median follow-up of 71 months, group 1 had success at T0 (1 month) and T1 (long-term) of 81.8% and 60.6%, respectively, compared to 93.8% and 77.7% in group 2. Statistical analysis showed a significant association with surgery both at T0 (p = 0.018) and T1 (p = 0.012) with group 2 showing better outcomes. Additionally, the cosmetic outcome of the external scar was defined as invisible in 91.8% of cases and slightly visible in 8.2%. CONCLUSIONS: External dacryocystorhinostomy provides long-term reliable results particularly in case of chronic dacryocystitis. Moreover, the optimal esthetic outcome of the external scar should be no longer considered the only guiding principle of treatment modality, particularly in the elderly.

QUANTITATIVE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY PARAMETER VARIATIONS AFTER TREATMENT OF MACULAR NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Macular neovascularization (MNV) secondary to age-related macular degeneration can be characterized by quantitative optical coherence tomography angiography. The aim of the study was to assess the evolution of quantitative optical coherence tomography angiography parameters after 1 year of antivascular endothelial growth factor injections. METHODS: Naive age-related macular degeneration-related MNV eyes were prospectively recruited to analyze optical coherence tomography and optical coherence tomography angiography parameters, including MNV vessel tortuosity (VT) and reflectivity, at baseline and at the end of the follow-up. Macular neovascularization eyes were categorized by a MNV VT cutoff, and quantitative parameter variations were documented after 1 year of treatment. We divided MNV eyes into Group 1 (MNV VT < 8.40) and Group 2 (MNV VT > 8.40). RESULTS: Thrity naive age-related macular degeneration-related MNV eyes (30 patients) were included. Our cohort included 18 Type 1 MNV and 12 Type 2 MNV lesions. Baseline central macular thickness (411 ± 85 µm) improved to 323 ± 54 µm at 1 year (P < 0.01). Only Group 1 MNV displayed significant visual improvement. Macular neovascularization VT values remained stable over the follow-up in both subgroups. Group 2 MNV eyes showed increased MNV reflectivity and increased MNV area at the end of the follow-up. Quantitative retinal capillary plexa parameters were found to be worse in Group 2 MNV. Outer retinal atrophy occurred in 2 of the 18 eyes in MNV Group 1 (11%) and in 6 of the 12 eyes in MNV Group 2 (50%) after 1 year. Vessel density proved to be always worse in Group 2 than in Group 1. CONCLUSION: Macular neovascularization VT provides information on the blood flow and identifies two subgroups with different final anatomical and visual outcomes, regardless of the treatment effect.

Combination of Mustardè cheek advancement flap and paramedian forehead flap as a reconstructive option in orbital exenteration.

PURPOSE: To describe a combinatory technique made of Mustardè cheek advancement flap and paramedian forehead flap as a reconstructive option after orbital exenteration. METHODS: We retrospectively reviewed all patients who underwent orbital exenteration and subsequent reconstruction with this technique at our Head and Neck Department, Divisions of Ophthalmology and Otolaryngology, at San Raffaele Hospital, Milan, Italy. RESULTS: Three patients were treated with the aforementioned technique, following orbital exenteration due to malignancies. All of them were affected by recurrent diseases arising from the ocular components or periorbital structures: one basal cell carcinoma and two squamous cell carcinomas. Excellent result was achieved considering skin texture and colour match, aesthetic results and intra and post-operative complications (only minor, surgically controlled haemorrhage and minimal dehiscence subsequent to defective healing occurred). CONCLUSION: The proposed technique can be considered by the head and neck reconstructive surgeon as a good option in extended orbital exenteration.