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Purpose: To review the clinical features, diagnosis, and treatment of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) and to report a case with the use of ultra-widefield fluorescein angiography (UWFA) for confirming the precise staging of IRVAN and aid in early treatment. The patient improved after being treated with intravitreal aflibercept injection., Results: A 26-year-old female complained of progressive blurred vision OD for one week. Her BCVA was 0.6 OD and 1.0 OS. Fundus examination showed vitritis, retinal hemorrhage, and vasculitis over bilateral eyes. Fluorescein angiography (FA) with a 55 degree of view revealed aneurysmal dilations of the peripapillary arteriole, peripapillary focal leakage, venous leakage, and capillary nonperfusion area. Stage 2 IRVAN was impressed OU. Oral prednisolone was administered. After four months, she experienced decreased visual acuity OS. Optical coherence tomography (OCT) revealed subretinal and intraretinal fluid with hyperreflective material. One posterior subtenon triamcinolone and one intravitreal aflibercept injection were performed OS, and macular edema subsided. A 105-degree ultra-widefield fluorescein angiography (UWFA) showed multiple peripheral background hypofluorescence areas corresponding to capillary nonperfusion. Retinal neovascularization (NV) was found OS, which had not been revealed by the previous 55-degree FA. Stage 3 IRVAN was made OS and panretinal laser photocoagulation (PRP) was performed. Oral prednisone and cyclosporine were prescribed. Her vision improved to 1.0 OU., Conclusion: UWFA provides visualization of peripheral retinal pathology and for precise staging. It also had direct implications in the follow-up and treatment strategy.

Post fever retinitis is a heterogenous entity that is seen 2-4 weeks after a systemic febrile illness in an immunocompetent individual. It may occur following bacterial, viruses, or protozoal infection. Optical coherence angiography (OCTA) is a newer non-invasive modality that is an alternative to fundus fluorescein angiography to image the retinal microvasculature. We hereby describe the vascular changes during the acute phase of post fever retinitis on OCTA. Imaging on OCTA was done for all patients with post fever retinitis at presentation with 3 × 3 mm and 8 × 8 mm scans centred on the macula and corresponding enface optical coherence tomography (OCT) scans obtained. A qualitative and quantitative analysis was done for all images. 46 eyes of 33 patients were included in the study. Salient features noted were changes in the superficial (SCP) and deep capillary plexus (DCP) with capillary rarefaction and irregularity of larger vessels in the SCP. The DCP had more capillary rarefaction when compared to the SCP. The foveal avascular zone (FAZ) was altered with an irregular perifoveal network. Our series of post fever retinitis describes the salient vascular features on OCTA. Although the presumed aetiology was different in all our patients, they developed similar changes on OCTA. While OCTA is not useful if there is gross macular oedema, the altered FAZ can be indicative of macular ischemia., (© 2021. The Author(s).)

Neuroretinitis is a rare clinical entity, characterized by optic nerve edema and star-shape hard exudate around fovea. The clinical features include acute unilateral visual loss, dyschromatopsia, relative afferent pupillary defect and visual field abnormalities. Increased vascular permeability of the optic disc is the main pathophysiology. As it is a not fully known clinical entity, diagnosis is challenging. In this case, we use multimodal imaging to reveal pathophysiology and anatomical change of early mild neuroretinitis. Case presentation : A 28-year-old healthy woman presented to the clinic with mild blurred vision in her left eye. After complete ophthalmic examination, outer retinal thickening of the temporal peripapillary area and optic disc edema were observed. Two days after diagnosis, the retinal edema and visual symptoms were aggravated. A hard exudate, maybe a part of macular star, was observed. Multimodal imaging including optical coherence tomography (OCT), swept-source OCT angiography (SS-OCTA), fluorescein angiography, and indocyanine green angiography visualized choroidal thinning and insufficient circulation beneath the outer retinal edema. Following steroid pulse therapy, the retinal edema and blurred vision were completely resolved. Conclusions : Multimodal imaging suggested that unilateral optic disc edema and early macular star help the diagnosis of neuroretinitis. In SS-OCTA, we found focal choroidal insufficiency. The focal insufficient choroidal circulation might be a contribution factor for idiopathic neuroretinitis. Multimodal imaging including SS-OCTA may be a valuable tool for detecting and monitoring disease progression.

Competing Interests: Competing interests: None declared.

A 45-year-old woman presented with diminished vision in the left eye. Visual acuity was 6/9 and fundus showed a very large live nematode near the macula along with inflammatory outer retinal lesions in the periphery. We diagnosed the case as diffuse unilateral subacute neuroretinitis and treated with immediate focal photocoagulation of the worm along with oral antihelminthic drugs and corticosteroids. The report highlights the importance of prompt laser and steroids in achieving good structural as well as the functional outcome, and the added significance of advanced imaging techniques in prognosticating such patients., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Three cases of patients with decreased visual acuity and papillitis at onset with subsequent macular star development after a few weeks are presented. Complementary tests were unremarkable in all included patients. Based on this clinical context, they were diagnosed with Leber's idiopathic stellate neuroretinitis, were treated with corticosteroids, as well as with antibiotics in 2cases. All patients showed favourable outcomes, although signs of papillary atrophy were observed in the affected eyes. Leber's idiopathic stellate neuroretinitis diagnosis can be challenging due to the wide spectrum of conditions that have to be ruled out. In addition, macular star may appear later on, which should not exclude its diagnosis. Use of antibiotics and/or corticosteroids is controversial considering its benign nature, but should be considered in selected severe cases. Moreover, the routine use of complementary tests should be carefully evaluated, mainly those that can be aggressive and/or expensive, which should be rationally used., (Copyright © 2019 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Branch retinal artery occlusion (BRAO) is rare in children. Bartonella is a known cause of branch retinal artery occlusion in adults, but it is typically not considered in the differential diagnosis for pediatric BRAO. We present the case of a 12-year old boy with a BRAO caused by a Bartonella henselae infection. This is the youngest such case reported in the literature. Although rare, Bartonella infection may be an important and underrecognized cause of pediatric BRAO., (Copyright © 2019 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Rationale: An idiopathic macular hole that causes substantial reduction in central visual acuity is believed to involve no obvious underlying diseases; thus, it is suspected to form due to the presence of idiopathic tractional forces at the vitreoretinal interface. Importantly, it is effectively treated with pars plana vitrectomy (PPV), which removes the mechanical forces. However, while it is exceedingly rare, a macular hole can develop in eyes after PPV; fresh or postoperative macular holes can close spontaneously without surgical removal of traction. Thus, another mechanism might be involved, although it remains obscure., Patients Concerns: A 67-year-old woman experienced 4 episodes of distorted and/or blurred vision., Diagnosis: She was diagnosed with recurrent macular hole formation., Interventions: For each episode, she either underwent surgery or was placed under observation., Outcomes: The macular hole was twice closed with PPV and twice without. The 2nd PPV procedure, which was performed at the time of 2nd recurrence, confirmed the absence of the epiretinal membrane and internal limiting membrane that cause tractional forces at the vitreoretinal interface in the macular area. At the time of the 3rd recurrence, fluorescein angiographies (FAs) revealed the presence of mild and diffuse inflammation throughout the peripheral retina, although there were no other findings indicative of ocular inflammation during the general eye examination conducted for every episode of macular hole formation. After the initiation of topical steroid treatment, inflammation (as recorded on FA) was reduced, and the macular hole subsequently closed. Development and resolution of perifoveal cystoid change and retinal protrusion were observed in every episode in optical coherence tomography (OCT) images. A bridging element in an OCT image was observed during the 4th closure of the macular hole., Lessons: Dynamic changes in FA and OCT images unraveled the pathogenesis of a macular hole that was originally diagnosed as idiopathic; mild inflammation was involved. The FA is typically not used for the diagnosis and management of macular hole formation; however, its use in this case helped determine a new mechanism in an otherwise idiopathic disease.

Aims: To compare optical coherence tomography (OCT) features of active necrotising infectious retinitis (NIR) due to toxoplasmosis or herpesviruses and to determine distinctive OCT signs for these two causes of infectious retinitis., Methods: OCT scans from eyes with active NIR due to varicella zoster virus (VZV), herpes simplex virus (HSV), cytomegalovirus (CMV), and toxoplasmosis (TOXO) were reviewed. All images were evaluated for the presence of previously described OCT findings in TOXO-NIR and compared with the viral group. New OCT findings were recorded and compared. Retinal and choroidal thickness were measured at the site of NIR and compared., Results: 10 eyes diagnosed with TOXO-NIR and 13 eyes affected by viral-NIR (9 CMV and 4 VZV) were analysed. All eyes showed full thickness hyper-reflectivity, disruption of the retina and a variable degree of vitritis. Among previously described OCT signs, hyper-reflective oval deposits and hypo-reflectivity of the choroid had a higher prevalence in TOXO (p=0.018 and p<0.0001, respectively). Among the new signs, hyper-reflective round deposits along the posterior hyaloid, retrohyaloid hyper-reflective spots and a disruption of the choroidal architecture were more frequent in TOXO eyes (all p<0.01). Intra-retinal oedema and hyper-reflective vertical strips within the outer nuclear layer were suggestive of a viral aetiology (p=0.045). Retinal thickness at the site of NIR did not differ between the two groups. Choroidal thickness was significantly higher in TOXO eyes (p=0.01)., Conclusions: The diagnosis of NIR is largely based on clinical and laboratory findings. OCT changes may be useful in differentiating different causes of NIR., Competing Interests: Competing interests: GS has financial relations with OCT manufacturers including: Heidelberg Engineering, Zeiss, Nidek, Canon, Optovue., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA)., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Severe macular oedema causing marked loss of vision is seen in cases of retinitis developing postviral fever. The use of antivascular endothelial growth factor agents for macular oedema and submacular fluid secondary to viral retinitis has not been studied or well established in the past. We report a case series of two patients of postviral retinitis with severe macular oedema resistant to steroid therapy, treated with intravitreal bevacizumab. The patients showed significant symptomatic improvement in the visual acuity. The retinitis lesions resolved slowly and macular oedema regressed. Bevacizumab appears to be a safe and useful agent to manage macular oedema subsequent to postviral retinitis. An early resolution of macular oedema helps in the preservation of visual acuity which left untreated can cause severe visual loss., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Competing Interests: Competing interests: None declared.

Borrelia burgdorferi is a known infective cause of neuroretinitis. We present a case of B burgdorferi neuroretinitis complicated by macular hole in a 22-year-old man. The neuroretinitis was managed with early high-dose intravenous corticosteroid and oral antibiotic. The macular hole was managed with macular hole surgery after intraocular inflammation had resolved., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

This article reports the case of a 32-year-old female patient who presented with severe spasmodically occurring retrobulbar pain, headaches and light sensitivity. The neurological examination confirmed cephalalgia but no evidence of other neurological symptoms was found. Ophthalmoscopy revealed yellowish patchy lightened spots in the middle periphery. Fluorescein angiography (FLA) and indocyanine green angiography (ICGA) revealed findings typical for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Hypofluorescent areas visible using ICGA in the early and late phases showed corresponding hypoperfused areas by optical coherence tomography angiography (OCT-A). To the best of our knowledge this is the first description of these findings in OCT-A.

Purpose: To report the retinal findings and evolution of a visually symptomatic case of West Nile virus meningoencephalitis., Methods: Case report. Main outcome measures include serologic testing for West Nile virus as well as longitudinal funduscopic examination, fluorescein angiography, and spectral domain optical coherence tomography., Results: A 47-year-old diabetic man was referred for ophthalmic evaluation after hospitalization and treatment for West Nile meningoencephalitis. The patient presented with decreased vision and black spots in the right eye. Baseline visual acuity was 20/100 in the right eye and 20/40 in the left. Funduscopic examination and fluorescein angiography revealed multiple outer-retinal, punctate white spots in the macula and midperiphery of the right eye with no irregularities noted in the left eye. Spectral domain optical coherence tomography revealed granular hyperreflective specks casting variably dense shadows scattered throughout multiple retinal layers, most prominently within the outer and inner nuclear layers of the right eye. The patient was observed over the course of 14 weeks, and final visual acuity was 20/50 in the right eye. Longitudinally, the number of specks progressively decreased., Conclusion: During West Nile virus infection, granular hyperreflective specks located predominantly within the outer and inner nuclear layers were visualized by spectral domain optical coherence tomography and may be a sign of West Nile virus infection.

Visual loss in infectious posterior uveitis or panuveitis can occur if proper therapy is delayed because of diagnostic uncertainty. Some disorders, such as acute retinal necrosis and bacterial endophthalmitis, can be rapidly progressive, and therefore require prompt and accurate diagnosis to guide initial therapy. Other more slowly evolving infections, such as toxoplasmic chorioretinitis or fungal endophthalmitis, can be worsened by empiric use of corticosteroids without specific antimicrobial coverage. Key ocular diagnostic features are helpful but highly variable with overlap with both non-infectious uveitis and neoplastic masquerades, even for key signs such as hypopyon. Close examination of the fundus with attention to color, location, size, border, and opacity of lesions and associated arteriolitis or frosted branch angiitis is helpful in the diagnosis of chorioretinitis. Ultrasonography is an important tool in the evaluation of eyes with suspected endophthalmitis, especially those with intracapsular infection or focal infected deposits. Testing of intraocular fluid can be extremely useful but suffers from inaccessibility, poor sensitivity, and test selections dependent on a presumptive diagnosis, which may be wrong. The dilemma for clinician is to make the correct diagnosis of a rare, blinding, variegated disease quickly enough to intercede with specific therapy or to apply empiric therapy in a sufficiently skilled manner to avert disaster and confirm the diagnosis by response to treatment. When non-infectious uveitis is in the differential, empiric corticosteroids must sometimes be used, at great risk, if clinical examination, ancillary testing, and any available intraocular diagnostic tests have failed to confirm a diagnosis.

Purpose/method: To report a clinical case of a 26-year old woman with Multiple Evanescent White-dot Syndrome (MEWDS) in left eye with recurrence in the fellow eye four year later, therefore bilateral and asynchronous. We review the clinical findings and the results of ancillary diagnostic techniques in both eyes., Clinical Case/conclusions: MEWDS is an uncommon inflammatory disorder with a benign course. The recurrences and the bilaterality are not usual. The affectation pattern in the fellow eye is similar to initial lesions seen four years earlier.

A new method for quantitative evaluation of ultrasonic scanograms of the eye, that permits differentiation between retinoblastoma and Coats' retinitis, was employed in echographic examinations of the eyes in 21 children with suspected intraocular volumic processes. Image analysis by plotting and processing of histogram was employed. High-confidence differences in the 'excess' and 'skewness' meanings of gradient histograms of patients with these two conditions have been detected.

The clinical, angiographic, ultrastructural and immunohistochemical features of a bilateral and asymmetrical case of Coats' disease in a three year old girl are described. The left eye showed advanced disease and was enucleated. Pathological examination revealed an exudative vasculopathy with ultrastructural evidence of interendothelial cell separation and formation of fenestrations. An isolated vascular malformation was discovered in the right eye. This was successfully treated with Argon laser under general anaesthesia.

The 595 angiographic lesions observed in 233 eyes of 169 patients affected with central serous retinopathy have been classified upon their localization. 498 angiographic lesions (83.7 percent) are located within a three optic disc diameter area centered by the foveola. Among them 20 are located right behind the foveola, 113 are located in the upper temporal quadrant, 166 in the upper nasal quadrant and 85 in the lower temporal quadrant. The relative density of the angiographic lesions decreases from the center of the macula to the periphery. 97 angiographic lesions (16.3 percent) are located beyond the three optic disc diameter central area. 41.1 percent of the lesions are situated behind the macula or/and behind the papillo-macular bundle. Therefore only 58.9 percent of the angiographic lesions observed in central serous retinopathy can be treated without any risk by means of photocoagulation.

The authors reported a Cytomegalovirus retinitis in an acute leukemia. It appears differently in each eye: localised seats of retinitis in one eye, and central venous occlusion associated with central artery occlusion in the other eye. The clinical polymorphism of herpes group virus is discussed.

Coats' disease represents a disorder of the retinal vascular walls. In a series of 112 consecutive eyes with Coats' disease the involvement was unilateral in 90.2%. The majority (72%) of patients were male. 57.8% were under 20 years of age. There was, however, a second peak of the disease (30.4%) between the age of 41 and 60. Severe cases were found mainly among juvenile patients, whereas in the senile group milder cases prevailed. Fluorescein angiographic findings comprised vascular aneurysms in almost all cases, rarefaction and coarsening of the capillary bed in 71%, capillary occlusions in 70%, occlusions of arterioles or arteries in 27%, shunt formations in 27%, extensive widening of arteries or veins in 27% and leakage of dye mainly in advanced cases. The participation of the central retina in the disease process was variable. Evidence is presented that Leber's military aneurysm retinitis is not a separate entity but a special form of Coats' disease.

123I metaiodobenzylguanidine (MIBG) is a radiopharmaceutical used for imaging neural crest tumours. The possibility of using 123I MIBG for imaging retinoblastomas has been assessed in this pilot study. Ten patients were studied, nine with clinically and histologically proved retinoblastomas and one with Coats's disease. 123I MIBG scintigraphy correctly identified the neoplasm in eight patients but gave a negative result in two, one of whom had Coats's disease and the other a retinoblastoma which proved to be extensively necrotic on histological examination. These preliminary results suggest that 123I MIBG scintigraphy may have a role in differentiating retinoblastomas from lesions that simulate them.

Computed tomography (CT) scan is a valuable adjunct in the differential diagnosis and management of retinoblastoma. Thirty-one of 33 retinoblastoma patients studied with high resolution computed tomography had intraocular calcification demonstrable in at least one eye. Over 80% of tumors showed evidence of calcification on CT scan. The degree of calcification appeared to depend upon tumor size; only small tumors were devoid of calcification. The amount and distribution of calcification was similar on both histological and CT review. In patients under three years of age in whom a retinoblastoma is suspected, the presence of calcification on CT scan is virtually diagnostic of retinoblastoma; patients with leukocoria without calcification on CT probably have a simulating lesion.