Uveal effusion in Waldenstrom's macroglobulinemia.

PURPOSE: To reastport a case of Waldenstrom macroglobulinemia-related choroidal detachments. METHOD: Case report. RESULTS: A 80-year-old woman was referred for bilateral visual loss for few months. She was hospitalized for a Waldenstrom's disease. Both anterior chambers were deep and quiet. Fundus revealed bilateral choroidal detachment without serous retinal detachments. No vitritis, retinal tear or pigmented lesion were observed. After eliminating all other causes of uveal effusion, the patient was treated for her hemopathy with chemotherapy associated with corticosteroids and plasmapheresis. One month later, fundus showed a complete disappearance of choroidal detachments and vision improved. CONCLUSION: Uveal effusion is an extremely rare ocular damage of Waldenström disease. As choroidal vessels are porous, they may allow immunoglobulins, over produced, to leak toward supra-choroidal space triggering choroidal detachments.

Ophthalmic vascular manifestations in eosinophil-associated diseases: a comprehensive analysis of 57 patients from the CEREO and EESG networks and a literature review.

Introduction: Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia. Methods: We conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (≥0.5 x109/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations. Results: Fifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher's retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x109/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x109/L in all cases (n=4). Discussion: This study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented.

Endogenous endophthalmitis: New insights from a 12-year cohort study.

OBJECTIVES: To describe the clinical and microbiological characteristics of patients with endogenous endophthalmitis (EE), determine factors associated with outcome and propose a management plan for EE. METHODS: Retrospective case series in two tertiary referral centers from 2010 to 2022. RESULTS: Sixty-four eyes of 53 patients were included. Bilateral involvement occurred for 11/53 patients (21%). Ocular symptoms were the only first manifestation of the disease in 36/53 (68%) of cases; signs of sepsis were evident in 17/53 (32%). Imaging tests detected at least one extraocular focus of infection in 34/53 patients (64%), with contrast-enhanced thoraco-abdominopelvic computed tomography showing relevant findings in 28/50 (56%) of cases. EE was microbiologically confirmed in 43/53 patients (81%); the organisms involved were: Gram-positive bacteria (19/53, 36%), Gram-negative bacteria (13/53, 25%) and Candida sp. (11/53, 21%). Klebsiella pneumoniae was the most common bacteria (10/32, 31%). Blood cultures were positive in 28/53 patients (53%) and eye samples in 11/41 eyes (27%). All patients were treated with systemic antimicrobial therapy, 39/64 eyes (61%) received anti-infective intravitreal injection(s) and 17/64 eyes (27%) underwent vitrectomy. Four patients (8%) died due to uncontrolled systemic infection. Final visual acuity (VA) was < 20/400 in 28/57 eyes (49%) and ocular structural loss (bulbar phthisis or enucleation/evisceration) was reported in 18/64 eyes (28%). In multivariate analysis, initial VA was the only parameter associated with visual and/or structural loss of the eye (OR = 24.44 (4.33-228.09) and 5.44 (1.33-26.18) respectively). CONCLUSIONS: EE remains a severe infection with a poor ocular outcome. We propose a standard protocol to improve diagnosis and medical management.

Visual acuity and visual field as a function of disease duration in patients with birdshot chorioretinitis.

BACKGROUND: Birdshot chorioretinitis (BSCR) is a chronic bilateral posterior uveitis, which can affect central as well as peripheral vision. The aim of this study was to assess how visual acuity and visual field evolved over time in patients with BSCR. METHODS: This was a prospective, observational, single-centre study based on data from the CO-BIRD cohort. Patient visits were categorised based on the time elapsed since the first symptoms, and groups of patients with different disease duration were defined. The main outcome measures were the best corrected visual acuity (BCVA), the mean deviation (MD) and the standard pattern deviation (PSD). RESULTS: The study included 447 Caucasian patients (181 males and 266 females), all of whom HLA-A29 carriers. From onset to 30 years of disease duration, the number of patients in each consecutive 5-year period was 237, 250, 196, 147, 78 and 32, respectively. Overall, the range of visual acuity and visual field results increased with disease duration. BCVA gradually decreased and showed a significant decline after 11-15 years after the first symptoms. Among the visual field indices, PSD significantly increased after 16-20 years, while MD showed a significant decline after 21-25 years. No major gender differences were found in visual outcomes, indicating comparable severity. The intereye correlations of MD and PSD were stronger than those of BCVA. CONCLUSIONS: BSCR resulted in a large heterogeneity of visual outcomes, which increased with time. Our data provide an overview of the visual consequences of BSCR as a function of disease duration.

MACULAR ATROPHY AFTER MACULAR HOLE SURGERY ASSISTED BY MEMBRANE BLUE DUAL: A CASE SERIES.

PURPOSE: The aim of this study is to describe the clinical and multimodal imaging findings in patients with macular atrophy after macular hole surgery assisted by Membrane Blue Dual. METHOD: This study is a monocenter, retrospective, observational case series that included patients who presented with macular atrophy following macular hole surgery. RESULTS: Among the patients included in this study, four were operated for idiopathic macular hole and one for total retinal detachment associated with macular hole. In all patients, the internal limiting membrane was brittle and adherent, and multiple stains were required. One month postoperatively, all patients showed a reduced visual acuity except the patient with total retinal detachment. At fundus examination all patients showed patchy atrophy with a mottled hypopigmented and hyperpigmented appearance in the macular region. Optical coherence tomography scans demonstrated a closed macular hole with retinal thinning, disruption of the external retinal layers, and irregular retinal pigment epithelium thickening. Fundus autofluorescence showed a well-defined area of both hypoautofluorescence and hyperautofluorescence involving the macular area. CONCLUSION: Macular atrophy after Membrane Blue Dual-assisted internal limiting membrane peeling represents a severe complication that vitreoretinal surgeons should be aware of and that should be taken into account in preoperative evaluation and surgical procedure planning. To reduce the risk of this complication, we recommend to ensure the best conditions of visibility, to reduce as much as possible the intensity and the distance of the endoillumination from the retina, and to use as little dye as possible.