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Purpose: To describe the clinical presentation and imaging features of a patient presenting with bilateral central retinal vein occlusion (CRVO), who was subsequently diagnosed with hyperviscosity retinopathy due to B cell lymphoproliferative disease, and had a good response to systemic immunosuppressive therapy. Observations: A clinical case report of an 87-year-old woman who presented with bilateral CRVO. Visual acuity, clinical examination, spectral domain optical coherence tomography (SD-OCT), color fundus photography and systemic evaluation were obtained. Ocular examination at presentation revealed bilateral CRVO, and OCT examination revealed significant central macular edema bilaterally. Six months after the diagnosis of hyperviscosity retinopathy and administration of systemic cyclophosphamide immunosuppressive therapy for B cell lymphoproliferative disease, most of the retinal hemorrhages resolved and partial resolution of the macular edema in the left eye was observed. Conclusion and Importance: This case describes the association between bilateral simultaneous CRVO and hyperviscosity. Under unique circumstances our patient received systemic therapy alone, without plasmapheresis. Although only limited therapy was applied, she didn't have deterioration or recurrent events; she had a small improvement in her macular edema and a significant improvement in her systemic functional state as well as reduction in her monoclonal IGM level. Keywords: Bilateral, CRVO, Hyperviscosity, Retinopathy, Waldenstrom's macroglobulinemia

Purpose To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cranial nerve (CN) III palsy. Observations A 20-year-old woman developed bilateral anterior uveitis, which resolved with topical steroids. Three weeks later she exhibited posterior pole lesions in both eyes, corresponding with a diagnosis of APMPPE, as confirmed by multimodal imaging. Two days later the patient presented with right CN III palsy. The patient was started on oral prednisone, which was gradually tapered off. Signs and symptoms improved rapidly, with complete resolution within two months. Conclusion and importance Though rare, APMPPE may present with neurological involvement, as in this previously unreported association with CN III palsy. Unlike uncomplicated APMPPE cases, in patients with neurological manifestations systemic therapy is advocated.

To investigate the efficacy and safety of 1% rimexolone ophthalmic suspension in children with chronic anterior uveitis under real-life conditions in a tertiary center.This is a retrospective longitudinal study. Medical records were analyzed at baseline, 1, 3, 6 and 12 months before and after switching to rimexolone for best-corrected visual acuity (BCVA), oral steroid use, number of flares, IOP and anti-glaucoma management.Twenty-four patients (41 eyes) diagnosed with either anterior uveitis (n = 25, 60.0%) or panuveitis (n = 16, 40%) were enrolled. The mean age was 10.5 years (4-16 years). The number of patients requiring oral prednisolone reduced from 8 patients (32.0%) at baseline to 3 patients (20.0%) at 12 months (P 0.001). Following baseline, the median number of uveitis flares reduced from 2.0 (inter-quartile range (IQR) 1.0-2.75) to 1.0 (IQR 0.0-1.0) compared to the 12 months before baseline (P 0.001). The mean IOP reduced from baseline (22.0 ± 7.3 mmHg) to 1 month (18.8 ± 8.7 mmHg, P = 0.01) and remained stable up to 12 months (15.9 ± 5.0 mmHg, P 0.001). Average BCVA, dose of oral prednisolone and anti-glaucoma treatments did not change compared to the baseline. The development for IOP ≥ 30 mmHg was associated with a known corticosteroid response [odds ratio (OR) 6.8, P = 0.003] and a dose 7.5 mg/day oral prednisolone (OR 4.4, P = 0.033).Rimexolone 1% ophthalmic suspension is an effective and safe topical steroid for pediatric anterior uveitis.

Background: In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment practices differ widely, within and between nations. Objectives: To provide recommendations for the diagnosis and treatment of JIA-associated uveitis. Methods: Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was constituted, consisting of nine experienced paediatric rheumatologists and three experts in ophthalmology from Europe. Recommendations derived from a validated systematic literature review were evaluated by an Expert Committee and subsequently discussed at two consensus meetings using nominal group techniques. Recommendations were accepted if >80% agreement was reached (including all three ophthalmologists). Results: In total, 22 recommendations were accepted (with >80% agreement among experts): 3 on diagnosis, 5 on disease activity measurements, 12 on treatment and 2 on future recommendations. Conclusions: The SHARE initiative aims to identify best practices for treatment of patients suffering from JIA-associated uveitis. Within this remit, recommendations for the diagnosis and treatment of JIA-associated uveitis have been formulated by an evidence-informed consensus process to suggest a standard of care for JIA-associated uveitis patients throughout Europe. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved.

Purpose: To report the effect of preoperative use of Ozurdex implant, as a prophylactic treatment to cataract surgery in 5 patients with chronic uveitis. Methods: A retrospective case reports. Results: 5 patients with chronic uveitis were included in this study. All patients were treated with Ozurdex prior to cataract surgery. Patients showed significant improvement in visual acuity one month post-surgery and no recurrence of uveitis up to three months post-surgery. Only two patients showed recurrence of CME 3-4 months post Ozurdex injection, which is attributed to Ozurdex maximal effect as was shown in previous studies. Conclusions: Intravitreal Ozurdex may serve as a perioperative anti-inflammatory treatment for uveitic patients undergoing cataract surgery.

Purpose To report our experience with intra-silicone oil (SO) injection of antiviral agents for treatment of viral retinitis and to review the relevant literature. Design Two case reports and a literature review. Participants Two patients with viral retinitis and SO tamponade. Methods Two patients with viral retinitis were treated with intravitreal injections of low-dose ganciclovir (2 mg/0.05 ml), foscarnet (1.2 mg/0.05 ml), or both after retinal detachment repair with SO tamponade, in addition to systemic antiviral therapy from 2014 through 2015. The literature on the use of intraocular antiviral agents in the setting of SO vitreous substitute was reviewed. Main Outcome Measures Clinical outcomes after administration of intra-SO antiviral therapy. Results A patient with progressive outer retinal necrosis received 5 intra-SO injections of low-dose ganciclovir and foscarnet after surgery over 6 weeks. Another patient with acute retinal necrosis received weekly low-dose foscarnet injections into his SO-filled eye for 8 weeks after surgery. Significant retinitis regression with long-term retinitis control was achieved in both patients throughout follow-up. No articles reporting the administration of soluble antiviral agents into an SO-filled human eye were identified. Conclusions Our preliminary findings indicate that administration of low-dose ganciclovir and foscarnet into an SO-filled eye may be used as adjunctive treatment for viral retinitis. Further studies are needed to confirm these results.

Background The yield of imaging in Horner syndrome has been explored only in children. This study evaluates the yield of imaging in adults. Methods This was a retrospective cohort study of 52 patients with Horner syndrome examined in 2 neuro-ophthalmology hospital clinics. Patients were divided into 3 groups according to the ability to determine the etiology at the time of the first neuro-ophthalmology consultation: group I, etiology of Horner syndrome known at the initial neuro-ophthalmologic examination; group II, etiology of Horner syndrome not known at the initial neuro-ophthalmologic examination, but sufficient information obtained to allow targeted imaging; and group III, etiology of Horner syndrome not known at the initial neuro-ophthalmologic examination, and sufficient information not obtained to allow targeted imaging. The yield of investigation and the frequency of the different etiologies were evaluated. Results In 32 (62%) patients, the etiology was already known at the initial neuro-ophthalmologic examination (group I). The most prevalent etiology was surgical trauma. In 11 (21%) patients, a targeted imaging workup was possible, revealing an etiology in 7 patients (group II). Carotid dissection and cavernous sinus mass were the most common etiologies. In 9 (17%) patients, a nontargeted imaging evaluation was necessary, revealing an etiology in only 1 patient, who had a previously undetected thyroid malignancy (group III). Conclusions The etiology of Horner syndrome is usually known at the time of initial presentation to a neuro-ophthalmologist. When the etiology is not known and clinical information permits a targeted imaging evaluation, an etiology can usually be determined, most commonly a cervical carotid artery dissection or a cavernous sinus mass. When the etiology is not known and clinical information is insufficient to allow a targeted imaging evaluation, an etiology is rarely discovered. Even so, nontargeted imaging is warranted because life-threatening lesions, such as thyroid malignancies, may rarely be detected.

Red desaturation is a subjective, sensitive test for patients with optic nerve disease. There is no quantitative clinical test to measure the severity of color desaturation. The current study introduces a simple PowerPoint presentation for quantification of red desaturation in patients with optic neuritis. A Microsoft Office PowerPoint presentation was designed. A red square is shown on each half of the screen. The degree of desaturation on one side is increased in each successive slide. The patient holds a black cardboard divider between his/her eyes, such that each eye can see one-half of the screen. The patient uses the mouse to forward through the slides, thereby gradually increasing the desaturation of the red square presented to the normal eye until it matches the color of the red square seen with the tested eye. Desaturation is graded by the number of slide changes needed until subjective equal color between the eyes is perceived. The test was presented to normal volunteers and to patients with optic neuritis. The difference in saturation levels between the right and the left eyes in patients with optic neuritis vs controls was compared. A total of 83 patients were included. Patients with optic neuritis had significantly increased desaturation compared to controls (6.17 ± 3.3 vs 0.24 ± 2.8 respectively; p

The most common ophthalmologic manifestation of hypertension is hypertensive retinopathy. Our study was designed to evaluate the factors that determine retinal vessel caliber in hypertensive subjects.During a 6-month period, retinal photographs were taken of participants during a routine yearly check-up. The photographs were evaluated by an ophthalmologist and the diameters of the individual retinal vessels were measured and summarized by a computer program. Data including patient's medical history, physical examination and laboratory evaluations were collected. The findings of the ophthalmologic examinations were analyzed with respect to the relevant systemic data.We evaluated 285 subjects (43 women) with an average age of 62 ± 9 years (range 33-87). Hypertension was reported in 204 (71.6%) subjects and diabetes mellitus in 60 subjects (21%). The arteriolar calibers were inversely related to age (R = - 017; p = 0.01). Retinal arteriolar and venular calibers were wider in smokers and in subjects with dyslipidemia. History of hypertension was not associated with any vascular changes. However, retinal arteriolar caliber was inversely related to blood pressure levels. Diuretic treatment was associated with narrow retinal venular caliber.The association between different cardiovascular risk factors and hypertensive retinopathy is complex. Effective blood pressure control has a beneficial effect on the vascular changes in the retina.