Occlusive Retinal Vasculitis Research Articles

Neuro-Behcet Disease and Ocular Inflammation: A Case Report and Literature Review

ABSTRACT Purpose: To describe a case of panuveitis with occlusive vasculitis leading to the diagnosis of neuro-Behcet disease (NBD) and discuss the relationship between uveitis and NBD. Methods: Case report with a literature review of ocular inflammation in NBD. Results: A 26-year-old woman with a seven-month history of recurrent cerebral venous sinus thromboses (CVST) and right-sided hemiparalysis secondary to rhombencephalitis presented with bilateral panuveitis and occlusive retinal vasculitis. Systemic evaluations were negative for hypercoagulability and infection. Although HLA-51 negative, the diagnosis was consistent with NBD. Conclusion: NBD is a rare subset of BD with a limited number of studies and patients. However, uveitis is more common in adults with parenchymal disease; may predate the development of neurological symptoms. The most common locations of ocular inflammation were posterior and panuveitis. MRI/V of the brain can identify enhancing lesions in the rhombencephalon or CVST in patients with uveitis with neurological findings.

Acute retinal necrosis following dexamethasone intravitreal implant (Ozurdex\xae) administration in an immunocompetent adult with a history of HSV encephalitis: a case report

BackgroundDexamethasone intravitreal implants (0.7 mg) (Ozurdex®, Allergan Inc., Madison, NJ) are FDA approved for managing macular oedema (ME) of retinal vein occlusion (RVO). The major complications associated with intravitreal Ozurdex® implant include increased intraocular pressure and cataract progression. In regard to the occurrence of retinal complications, we report an unusual intravitreal Ozurdex® implantation-related acute retinal necrosis (ARN).Case presentationA 45-year-old immunocompetent woman with a history of encephalitis presented with photophobia, redness, floaters, and rapidly decreased vision in her left eye. Three and six months ago, she received two doses of intravitreal Ozurdex® implant for ME of RVO. Clinical evaluation, including slit-lamp biomicroscopy, retinal photography, and fluorescein angiography, revealed anterior chamber cells, granulomatous keratic precipitates, cells in the vitreous, optic disc oedema, occlusive retinal vasculitis, scattered retinal haemorrhages, one quadrant of peripheral white areas with retinal necrosis, optic disc and vessels fluorescein staining, and retinal nonperfusion zones. All the above clinical manifestations showed an ARN. Herpes simplex virus was detected in the aqueous and vitreous humour by quantitative polymerase chain reaction testing. Intravenous acyclovir 500 mg tid for 7 days followed by oral valcyclovir was immediately performed for ARN. At 4 months, the patient’s condition improved without retinal detachment, and the best-corrected visual acuity remained stable at 0.3.ConclusionsARN might represent a risk of Ozurdex® administration.

Behçet's Uveitis: Current Diagnostic and Therapeutic Approach

Behçet’s disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet’s uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis.

Uveitis in Behçet's Syndrome in Germany - Prognosis During Current Therapy

Behçet's syndrome (BS) is an inflammatory systemic disease associated with multiple organ manifestations. Major symptoms include oral and genital ulcerations, skin lesions and uveitis. Diagnosis of Behçet's syndrome is based on clinical findings. A typical for ocular Behçet's syndrome is recurrent non-granulomatous panuveitis with occlusive retinal vasculitis. Treatment used to be based only on the use of systemic corticosteroids and conventional immunosuppressives, but most often this treatment did not appear to be sufficient. In consequence, prognosis of ocular BS was very poor over a long period of time - especially if occlusive retinal vasculitis was present. In recent years, the introduction of biologicals has revolutionised the therapeutic regime and consecutively visual prognosis of ocular Behçet's syndrome has dramatically improved.

An Algorithm for the Diagnosis of Behçet Disease Uveitis in Adults

ABSTRACT Purpose: To develop an algorithm for the diagnosis of Behçet’s disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies.

Distance-Thresholded Intercapillary Area Analysis Versus Vessel-Based Approaches to Quantify Retinal Ischemia in OCTA

PurposeTo develop and compare different analytic approaches for quantifying ischemia in OCT-angiography (OCTA), including vessel-based approaches and intercapillary area analysis.MethodsEn face OCTA (6 × 6) images of the superficial plexus of 20 healthy eyes and 20 eyes with different ischemic retinal diseases were analyzed retrospectively. Included retinal diseases were diabetic retinopathy (n = 9), central (n = 5) and branch retinal vein occlusion (n = 4), hypertensive retinopathy (n = 1), and occlusive retinal vasculitis in sarcoidosis (n = 1). Vessel-based approaches consisted of the mean gray scale, perfusion density, and vessel density. Intercapillary areas (ICAs) were analyzed measuring the distance of each intercapillary pixel from the surrounding vessels. In particular, we applied a vector method to measure the shortest, the mean, and the longest distance in eight predefined directions. Size of ICAs was determined applying different global or local distance thresholds.ResultsAll approaches revealed significant differences between ischemic and healthy retinae (P < 0.001, with Bonferroni-Holm correction P = 0.001–0.025; Wilcoxon-Mann-Whitney test). Discrimination between the healthy and ischemic retinae based on ROC curves was best in the ICA analysis using a locally set threshold of the shortest distance.ConclusionsIn the present study, ICA analysis was superior to vessel-based approaches in the quantification of retinal ischemia when defining a local or global distance threshold.Translational RelevanceIn order to establish OCTA imaging in everyday clinical and scientific practice, standardized, device-independent image analysis methods are necessary.

Successful Clinical Outcome of Vancomycin-induced Hemorrhagic Occlusive Retinal Vasculitis

ABSTRACT We present a case of a patient that experienced severe hemorrhagic occlusive retinal vasculitis secondary to injection of 1.0 mg/0.1 ml of intracameral vancomycin for endophthalmitis prophylaxis after an uneventful cataract surgery. The case is especially unique in that our patient ended up maintaining 20/25 vision with an ocular disease that is typically visually threatening. This may be due to the aggressive administration of periocular and oral steroids combined with scheduled anti-VEGF injections that were later transitioned into a treat and extend regimen.

Toxicities of and inflammatory responses to moxifloxacin, cefuroxime, and vancomycin on retinal vascular cells

Prophylactic intracameral injection of antibiotics is commonly used to prevent endophthalmitis after cataract surgery. However, devastating visual complications have been reported including hemorrhagic occlusive retinal vasculitis (HORV).To determine the toxic and inflammatory effects of moxifloxacin, cefuroxime, and vancomycin on human retinal vascular cells, human retinal vascular endothelial cells (RVEC) and pericytes were exposed to three antibiotics, and the adverse effects were assessed by membrane damage, loss of intrinsic esterase activity, kinetic cell viability, and inflammatory cytokine secretion. Their retinal toxicity was examined by live/dead assays after an intravitreal injection of the three antibiotics into mice eyes. In vascular cells in culture, membrane damage and loss of esterase activity were induced after exposure to the three antibiotics. The toxic effects were most obvious after moxifloxacin (RVEC, ≥125 μg/mL; pericytes, ≥1000 μg/mL) at 24 h. Cefuroxime also reduced esterase activity and the membrane integrity of vascular cells but were less toxic than moxifloxacin. Kinetic cell viability testing showed that 500 μg/mL of moxifloxacin exposure induced significant decrease (29%) in the viability as early as 1 h. When the inflammatory effects of the antibiotics were examined, a significant induction of IL-8 was observed especially by RVECs after exposure to cefuroxime or vancomycin which was exacerbated by L-alanyl-γ-D-glutamyl-meso-diaminopimelic acid (Tri-DAP), a NOD1 ligand. Intravitreal injections in mice showed that cefuroxime and vancomycin caused retinal and vascular toxicity extending to the inner nuclear layers. Collectively, moxifloxacin causes immediate damage to retinal vascular cells in vitro, while cefuroxime and vancomycin induced significant inflammatory effects on vascular endothelial cells and caused retinal toxicity. Surgeons need to be cautious of the toxicity when antibiotics are used prophylactically especially by intravitreal administration.

Choroidal and retinal occlusive vasculitis

Choroidal and retinal occlusive vasculitis

Intracameral antibiotics for prophylaxis of postoperative endophthalmitis in Australia: a review.

Postoperative endophthalmitis is a severe complication which may result in loss of vision. Intracameral antibiotics have been shown to reduce the risk of this complication and are extensively used worldwide, with vancomycin, cefuroxime and moxifloxacin most commonly used. Choices in Australia are currently limited to vancomycin and cefazolin, since cefuroxime and moxifloxacin are not available. Vancomycin has been safely and effectively used in our department for more than two decades. Recent publications have risen concerns regarding the association of haemorrhagic occlusive retinal vasculitis a potentially blinding complication with intracameral vancomycin. This concern has driven us to review the safety and efficacy of currently available antibiotics in the context of Australian practice.

Postoperative Hemorrhagic Occlusive Retinal Vasculitis Associated with Intravitreal Injection of Vancomycin

PURPOSE: To present a case of hemorrhagic occlusive retinal vasculitis (HORV) associated with intravitreal injection of vancomycin after pars plana vitrectomy (PPV).METHODS: Single case report.RESULTS: A 67-year-old woman presented with best corrected visual acuity (BCVA) of 20/400 in left eye. For clinical suspicion of chronic endophthalmitis, empirical treatment was initiated with vancomycin and ceftazidime without clinical improvement. The patient was submitted to pars plana vitrectomy. During surgery rhegmatogenous retinal detachment was observed and treated. At the end of surgery, intravitreal vancomycin was injected. After 18 days BCVA was counting fingers and fundus examination showed diffuse retinal vascular sheathing, intraretinal hemorrhages and ischemic macular whitening. The clinical diagnosis was compatible with HORV secondary to retinal toxicity due to intraocular vancomycin.CONCLUSIONS: This case illustrates the importance of including intravitreal vancomycin during PPV surgery on the list of potential causes when investigating a case of suspected hemorrhagic occlusive retinal vasculitis.

Hemorrhagic occlusive retinal vasculitis leading to the diagnosis of ciliary body melanoma

Hemorrhagic occlusive retinal vasculitis (HORV) is a condition associated with intraocular vancomycin during surgical intervention, most frequently following bilateral sequential cataract surgery. Because of the high rate of ophthalmic vascular complications in this condition, gonioscopic evaluation for identification of neovascularization of the angle and iris is essential. Careful examination can reveal previously asymptomatic and unassociated lesions of the iris or ciliary body. We present the case of a 71-year-old female who was diagnosed with a ciliary body melanoma secondary to complete ophthalmic examination associated with HORV. She reported decreased vision to light perception in the left eye (OS) following sequential, bilateral cataract surgery. Fundus examination OS demonstrated diffuse retinal vasculitis, retinal ischemia, and extensive hemorrhage. Evaluation included inflammatory and coagulopathy laboratory evaluation, carotid ultrasonography and magnetic resonance imaging (MRI) of the brain and orbits, all of which proved unrevealing, except for an enhancing mass OS on MRI. Further evaluation revealed a pigmented mass with features of melanoma in the anterior chamber angle extending into the ciliary body. Fine needle aspiration biopsy revealed high risk cytogenetic characteristics, and plaque radiotherapy was successfully employed.

Vaso-occlusive retinopathy and stage IV Lupus Nephritis; Primary manifestation of a 30 year old female

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. Ocular involvement is not included in diagnostic criteria of SLE. Vaso-occlusive retinopathy, as the primary manifestation of systemic lupus erythematosus (SLE), is relatively rare. We report a 30-year-old female, who suddenly suffered bilateral visual loss. Fundus examination revealed bilateral occlusive retinal vasculitis. She fulfilled the American College of Rheumatology criteria for diagnosing SLE; she had arthralgia, photosensitivity, positive ANA, high titre of anti-dsDNA, and proteinuria. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with pulse steroid and immunosuppressant. Best corrected visual acuity of both eye improved to finger counting after treatment. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.Bangladesh J Medicine Jan 2018; 29(1) : 36-40

Intracameral antibiotics during cataract surgery: evidence and barriers.

There is continuing debate regarding the safety, efficacy, and necessity of intracameral antibiotics to prevent postoperative endophthalmitis. We summarize the most recent evidence-based publications that either support or caution against this practice. Several additional large, international retrospective clinical studies found a significant reduction in endophthalmitis rates with intracameral antibiotic prophylaxis. Many surgeons are looking for alternatives to vancomycin for intracameral prophylaxis because of its association with the rare but sight-threatening complication of hemorrhagic occlusive retinal vasculitis. Although most efficacy data has been reported for intracameral cefuroxime, one of the largest clinical studies to date shows convincing efficacy with intracameral moxifloxacin prophylaxis. This same study reported a significant benefit with intracameral moxifloxacin following posterior capsular rupture, and surgeons should consider intracameral prophylaxis in these high risk cases. Despite mounting evidence that routine prophylaxis with intracameral cefuroxime and moxifloxacin reduce the endophthalmitis rate following cataract surgery, many surgeons have not adopted this practice. Reasons include lack of level 1 evidence from randomized trials, and the potential risks from compounding or mixing antibiotic solutions when no approved formulations are commercially available. Countries with commercially approved intraocular antibiotic formulations have the highest adoption rates of routine intracameral prophylaxis.

Early anti-VEGF treatment for hemorrhagic occlusive retinal vasculitis as a complication of cataract surgery

BackgroundWe report a case of hemorrhagic occlusive retinal vasculitis (HORV) after prophylactic intracameral vancomycin use during an uneventful cataract surgery treated with early anti-VEGF treatment.Case presentationA 51-year-old female underwent uneventful cataract surgery with prophylactic intracameral vancomycin during the procedure. On the seventh post-operative-day, she presented with sudden painful, visual loss. Fundus examination revealed peripheral hemorrhagic retinal vasculitis. She received anti-VEGF therapy to prevent further vision loss and retinal neovascularization due to extensive retinal ischemia. At the 6-month follow-up visit, visual acuity was 20/20 with no sign of neovascularization.ConclusionsPostoperative HORV is a devastating condition that can occur after otherwise uncomplicated cataract surgery. The nature of this rare condition remains unknown. Early anti-VEGF administration seems to demonstrate favorable results.

RETINAL OCCLUSIVE VASCULITIS SECONDARY TO TUBERCULOSIS – A CLINICAL REPORT

Purpose: To report the case of a patient with persistent right eye (RE) hemovitreous secondary to occlusive retinal vasculitis secondary to tuberculosis. Methods: Male patient, 35 years old, Indian descent, with no history of previous systemic illness. First presented to our outpatient clinic with decreased visual acuity RE with 8 months duration. On exam, BCVA RE: <5/200 and BCVA LE: 20/20; keratic precipitates were visible and anterior chamber flare was described as 1+; extensive RE hemovitreous were present precluding fundus observation. A fluorescein angiography (FA) performed on his LE was normal. He was started on topical steroids and submitted to pars plana vitrectomy + intra-operative retinal photocoagulation on his RE for peripheral retinal neovascularization. Results: Post-surgery exam, BCVA RE: 20/100 and BCVA LE: 20/20, no anterior chamber reaction bilaterally. RE macular edema with exuberant vascular turtuosity and “ghost vessels”. FA confirmed active occlusive vasculitis on RE. Diagnostic work-up was positive for a 28 mm induration tuberculin test, supporting the diagnosis of ocular tuberculosis. The patient was started on anti-tuberculous medication. Three months later, BCVA RE was 20/25 with no anterior chamber reaction OU. Eighteen months on, the patient maintains good bilateral visual acuity without any evidence of disease reactivation. Conclusions: In the clinical case described there was a good response after antituberculosis treatment instituted unassociated oral corticosteroid therapy, with improved visual acuity and remission of inflammatory angiographic signs, stressing the importance of tuberculous etiology research in cases of retinal vasculitis.

Optical coherence tomography angiography in occlusive retinal vasculitis

PurposeFluorescein angiography (FA) has been the gold standard for the evaluation and management of occlusive retinal vasculitis. Our purpose is to describe swept‐source optical coherence tomography angiography (OCTA) findings in eyes with occlusive retinal vasculitis.MethodsThis prospective study included 15 patients (25 eyes) diagnosed with occlusive retinal vasculitis involving the posterior pole or the periphery. All patients were evaluated using FA, spectral domain optical coherence tomography, and OCTA.ResultsThe causes of occlusive retinal vasculitis included Behcet disease in 12 patients (21 eyes), ocular tuberculosis in 1 patient (2 eyes), West Nile virus infection in 1 patient (1 eye) and rickettsiosis in one patient (1 eye). OCTA was superior to FA in evaluating perifoveal microvascular changes. It showed in twenty eyes (80%) areas of retinal capillary nonperfusion/hypoperfusion with or without associated rarefied, dilated, or shunting vessels. The deep retinal capillary plexus was more severely affected than the superficial capillary plexus.ConclusionsOCTA allowed better evaluation of macular ischemia than FA in eyes with occlusive retinal vasculitis. The deep capillary plexus appeared to be more severely involved than the superficial capillary plexus.

Tuberculosis

SummaryThe diagnosis of intraocular tuberculosis (TB) is often presumptive, made on the basis of suggestive ocular features, evidence of latent or manifest TB, exclusion of other causes of uveitis, and positive response to antitubercular treatment (ATT). The most typical features include serpiginous‐like choroiditis and occlusive retinal vasculitis. When the posterior segment is involved, treatment relies on ATT, usually in association with systemic or periocular corticosteroids. Patients should be monitored for response of inflammation to treatment, recurrences, adherence to ATT and treatment side‐effects. Clinical assessment of ocular TB at presentation and during follow‐up includes grading of vitreous haze, characterization of choroidal involvement, retinal vascular involvement and optic disc changes, and evaluation of anterior chamber cells and flare. Laser flare photometry may be used to monitor anterior chamber inflammation. Ocular imaging tests including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, SD and EDI optical coherence tomography (OCT), and OCT angiography may be very useful in analyzing and quantifying specific TB retinal and choroidal changes.

Epidemiology and clinical features of inflammatory retinal vascular occlusions: pooled data from two tertiary-referral institutions.

In a subset of patients with retinal vasculitis, there is occlusion of blood flow through the retinal vessels. These eyes are at high risk of sight-threatening complications. To characterize epidemiology, clinical course, treatment and outcomes of occlusive retinal vasculitis (ORV). Retrospective study PARTICIPANTS: Seventy-seven uveitis patients with ORV at two large tertiary-care institutions (the USA and India). Out of 2438 patients screened, 346 patients were diagnosed with retinal vasculitis of which 77 patients (96 eyes) were diagnosed with ORV. Patients with ORV (capillary, arteriolar and/or venular) were further analysed. Diagnostic criteria for occlusive vasculitis included (i) absence of blood flow in vessels (arterioles, venules and/or capillaries), (ii) capillary non-perfusion areas and/or arteriolar-venous anastomosis and (iii) intraretinal haemorrhages, cotton-wool spots or vitreous haemorrhage. Best-corrected visual acuity, treatment and complications. The mean age was 32.09±13.51years. Most common aetiologies were tuberculosis and Adamantiades-Behçet's disease in India and systemic lupus erythematosus in the USA. Best-corrected visual acuity improved from 0.38±0.30 logMAR (20/48 Snellen equivalent) (baseline) to 0.25±0.30 (20/35 Snellen equivalent) at final visit (P<0.0001). Vitreous haemorrhage was seen in 31.08% eyes. Pars plana vitrectomy was performed in 12.16% eyes. Therapy with systemic steroids was required in 78.48% patients. In addition, 46.75% patients required immunomodulators and/or biologics. Occlusive retinal vasculitis is caused by heterogeneous group of uveitides depending upon the geographic location. It is imperative to identify eyes with ORV as they are predisposed to complications requiring aggressive therapy.

Endophthalmitis Reduction with Intracameral Moxifloxacin Prophylaxis: Analysis of 600 000 Surgeries

To compare the postoperative endophthalmitis rate before and after initiation of intracameral (IC) moxifloxacin prophylaxis for both phacoemulsification and sutureless, manual small-incision cataract surgery (M-SICS), as well as in patients with posterior capsular rupture (PCR). Retrospective, clinical registry. All cataract surgeries (617 453) performed during the 29-month period from January 2014 to May 2016 at the 10 regional Aravind eye hospitals were included. The electronic health record data for all study eyes were analyzed. Endophthalmitis rates before and after moxifloxaxin were statistically compared for all eyes and separately for both phacoemulsification and M-SICS, and for the eyes complicated by PCR. The postoperative endophthalmitis rates before and after initiation of IC moxifloxacin prophylaxis. Overall, 302 815 eyes did not receive IC moxifloxacin and 314 638 eyes did, and there was a significant decline in the endophthalmitis rate, from 0.07% (214/302 815) to 0.02% (64/314 638) (P < 0.001), with moxifloxacin. For the 194 252 phacoemulsification eyes, the endophthalmitis rate was 0.07% (75/104 894) without IC moxifloxacin prophylaxis, compared with 0.01% (11/89 358) with moxifloxacin (P < 0.001). For the 414 657 M-SICS eyes, the endophthalmitis rate was 0.07% (135/192 149) without IC moxifloxacin prophylaxis, compared with 0.02% (52/222 508) with moxifloxacin (P < 0.001). Approximately half of the 8479 eyes that had PCR received IC moxifloxacin, and half did not. Without IC moxifloxacin, PCR increased the endophthalmitis rate nearly 7-fold to 0.48% (20/4186); IC moxifloxacin reduced the endophthalmitis rate with PCR to 0.21% (9/4293) (P= 0.034). No adverse events were due to IC moxifloxacin. Routine IC moxifloxacin prophylaxis reduced the overall endophthalmitis rate by 3.5-fold (3-fold for M-SICS and nearly 6-fold for phacoemulsification). There was also a statistical benefit for eyes complicated by PCR, and IC antibiotic prophylaxis should be strongly considered for this high-risk population. These conclusions are strengthened by the high volume of cases analyzed at a single hospital network over a comparatively short time frame. Considering the association of hemorrhagic occlusive retinal vasculitis with vancomycin and the commercial unavailability of IC cefuroxime in many countries, moxifloxacin appears to be an effective option for surgeons electing IC antibiotic prophylaxis.