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A middle-aged man had been diagnosed as retinitis and treated with steroids previously. The patient had developed macular infarction in the course of illness. We diagnosed him to have rickettsial retinitis on the basis of clinical features and positive Weil-Felix test. The patient’s condition improved after treatment with oral antibiotics. The vasculature of the infarcted macula showed partial reperfusion late in the course of follow-up.

To present a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome that was successfully managed with serial intravitreal aflibercept injections.Ophthalmic imaging and visual acuity were used to monitor disease state and track treatment methods to determine the most valuable combination of treatment medication and treatment interval.A 28-year-old woman with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome status after panretinal photocoagulation of both eyes presented with bilateral cystoid macular edema. We demonstrate successful management of retinal cystoid macular edema associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome using serial intravitreal aflibercept injections.Intravitreal aflibercept has a useful role in managing the potential retinal complications associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome and provides further insights into treatment of the later stages of this rare disease.

Purpose To describe a patient with recurrent acute retinal necrosis (ARN), her treatment, and propose a possible pathophysiologic mechanism. Method Case report. Results A four-year-old girl presented elsewhere with bilateral ARN, was treated, but developed a retinal detachment in the left eye that failed vitrectomy surgery. She was referred 10 years later with recurrent ARN. The infection was difficult to get under control, but eventually responded to intravenous acyclovir and foscarnet. She was given laser photocoagulation. She was placed on oral valacyclovir prophylaxis and was disease free for 10 years at which point she decided to go to South America on vacation and stop her valacyclovir. Within a few days she developed a recurrence of ARN and flew back for treatment. She had discrete areas of retinal necrosis, vasculitis, and the laser photocoagulation lesions appeared to be ringed by a retinal change suggestive of retinitis. She responded to antiviral treatment, but developed a retinal detachment that was successfully treated. Her visual acuity was 20/20 six years later, and she was using antiviral prophylaxis. Conclusions Recurrent ARN can respond to aggressive treatment. Chorioretinal scars, such as from photocoagulation, may be potential sites of viral invasion during recurrences. Anti-viral prophylaxis may be indicated for at risk patients.

Purpose To report two cases of cytomegalovirus (CMV) retinitis in renal transplant patients after conversion to belatacept immunosuppression. Methods Case report. Results Case A was a 41-year-old African American man with no ocular history, chronic hepatitis B infection on entecavir, status-post living relative renal transplant for end-stage renal disease secondary to IgA nephropathy presented with a chief complaint of "blurry vision" in his right eye. Fundoscopic examination of the right eye revealed perivascular retinitis of the superior arcade extending temporally and a lesion nasal to the optic disk. The patient was diagnosed with CMV retinitis with macular involvement. Case B was a 52-year-old woman with an ocular history significant for bilateral vein occlusions treated with laser, and a medical history of hypertension, hyperlipidemia, and end-stage renal disease secondary to hypoplastic kidney status-post deceased donor renal transplant presented with one week of blurred vision in the right eye. Fundoscopic examination of the right eye revealed macular star configuration of exudates, as well as subretinal fibrosis temporal to the macula. Fundoscopic examination of the left eye showed Grade 4 vitreous haze and multiple areas of retinal whitening and hemorrhage, consistent with CMV retinitis infection. The patient was diagnosed with bilateral CMV retinitis, with macular involvement in the right eye. Conclusion Renal transplant patients converted to belatacept immunosuppression may be at an increased risk for herpes virus infection, and thus herpes virus retinitis. Although the degree of risk remains uncharacterized, patients on belatacept therapy who are at the high risk of CMV infection (i.e., donor-positive/recipient-negative patients) should be counseled on the presenting signs and symptoms of CMV retinitis. In these cases, clinicians should also consider regular monitoring of serum CMV titers or continuation of antiviral prophylaxis.

PURPOSE To report two cases of cat-scratch fever with atypical posterior segment manifestations. METHODS Two cases were retrospectively reviewed. RESULTS A 27-year-old woman presented with painless blurring of central vision in her left eye. Clinical examination revealed a small focal area of retinitis within the macula associated with a subtle macular star. Spectral-domain optical coherence tomography showed a hyper-reflective inner retinal lesion in addition to subretinal and intraretinal fluid as well as hyperreflective foci within the outer plexiform layer. Serology was positive for anti-B. henselae IgM (titer 1:32). A 34-year-old woman presented with painless loss of vision in both eyes associated with headaches and pain with extraocular movement. Spectral-domain optical coherence tomography depicted subretinal fluid, intraretinal fluid, and hyperreflective deposits within the outer plexiform layer. A focal collection of vitreous cell was observed overlying the optic nerve in the left eye. Bilateral disk leakage was identified on fluorescein angiography. Serology revealed high-titer anti-B. henselae antibodies (IgM titers 1:32, IgG titers 1:256). CONCLUSION Our cases highlight the necessity of recognizing more unusual posterior segment presentations of ocular bartonellosis. Multimodal retinal imaging including spectral-domain optical coherence tomography may help better characterize lesions.

Background. Although the prevalence of HIV is low in Bangladesh, there is a potential for an increased number of cases. This is because of high cross-border mobility (India and Myanmar) of people and increased injection drug abusers amongst youth in the cities and rural areas, HIV can present in many ways, from asymptomatic to advanced disease, including various atypical (generalized itching) and advanced (loss of vision) manifestations. A high degree of suspicion is required to diagnose HIV in a country like Bangladesh. Early diagnosis and prompt treatment are essential to have a better outcome. Methods. Here, we report two thought-provoking cases where patients were suffering from generalized itchy lesions (pruritic papular eruption) throughout the body for a long time and gradual loss of vision in another case. Results. Due to lack of suspicion, initially, HIV screening was not done. Both patients visited several health centres, but no diagnosis was made. Moreover, COVID-19 pandemic worsens the situation. Finally, they were diagnosed with HIV; unfortunately, one of them lost her vision due to CMV retinitis and another patient died of other complications. Conclusion. Ongoing COVID-19 pandemic put many challenges to ensure optimum care, especially for patients with long-sufferings like HIV. Clinicians have to have a very high degree of suspicion while dealing with patients presented with rare manifestations, particularly in a low endemic clinical setting.

Purpose The purpose of this report is to describe a patient who has fluorescein angiographic evidence and retinal changes suspicious for intrauterine abusive ocular trauma. Methods A case report of a premature ward of the state with no prenatal care and a presentation suspicious for intrauterine abusive ocular trauma. We performed serial ophthalmologic examinations, reviewed available prenatal history with the infant's social worker, and all relevant hospital notes, laboratory results, and imaging results. Results Following initial empiric treatment for possible viral retinitis in the setting of a positive urine CMV, repeat examinations demonstrated fluorescein angiographic evidence and clinical findings suspicious for abusive ocular trauma including neovascularization and no evidence of retinitis; therefore, the child was treated with laser photocoagulation. Conclusion At-risk newborns will benefit from an examination of the retinal periphery and wide field fluorescein angiography. Intrauterine abusive ocular trauma should be included in the differential of retinal hemorrhage and avascular retinal periphery. It is imperative for clinicians to recognize this presentation to prevent progression of associated visual morbidity.

Cytomegalovirus (CMV) infection remains an important cause of morbidity and mortality post cord blood transplantation (CBT). It has been suggested that the graft-versus-host disease (GVHD) and immunosuppressants have an impact on CMV infection. This study evaluated the incidence, outcomes, and risk factors of CMV infection, while focusing on GVHD and the use of immunosuppressants, in 103 children who had received CBT. Among the patients, 92.2% were positive for CMV serology, while CMV antigenemia was observed in 68.9% and CMV disease developed in 26.2%. CMV enterocolitis was the most common, followed by retinitis and pneumonia. Patients with positive CMV serology and grade II to IV GVHD were independently associated with CMV antigenemia. Recurrent CMV antigenemia was observed significantly more frequently in patients with extensive chronic GVHD. Patients with CMV disease showed significantly worse overall survival, relapse-free survival, and non-relapse mortality than those without CMV disease. In conclusion, CMV infection is common post-CBT in countries with a high rate of CMV seropositivity in the general population and is related to worse outcomes. GVHD severity is associated with the development and recurrence of CMV infection. Thus, efforts need to be made to prevent CMV infection in children post-CBT.

In a tertiary government hospital where cost is an invariable concern, this report validates monotherapy of intravitreal ganciclovir as an effective option for patients due to affordability and ease of administration compared to systemic therapy. The case also exhibits the potential of intravitreal therapy to improve visual acuity in advance bilateral retinal disease

Background Cytomegalovirus (CMV) retinitis may occur in non-HIV individuals following systemic immunosuppressive treatment or periocular corticosteroid administration. However, simultaneous multiple viral retinitis is rare in HIV-negative individuals. We report a case of dual viral retinitis in a non-HIV female on systemic immunosuppressive for pemphigus vulgaris who was administered a periocular corticosteroid injection. Method A 32-year-old female on double immunosuppressive therapy (prednisolone and cyclophosphamide) for pemphigus vulgaris, presented with gradual painless diminution of vision in the right eye for one month. She was initially diagnosed to have possible autoimmune neuroretinitis by the referring ophthalmologist and received a single injection of posterior subtenon triamcinolone acetonide for the same. Her vision however deteriorated further and she received an intravitreal ganciclovir injection with a revised diagnosis of CMV retinitis. Due to suboptimal response she was referred to us. Aqueous Polymerase chain reaction (PCR) revealed dual positivity for CMV and Herpes simplex virus. She was successfully managed with intravitreal ganciclovir injections, systemic acyclovir and tapering of systemic immunosuppressive drugs. Result The retinitis lesions resolved gradually leaving behind a pale optic disc and foveal atrophy at 12 weeks follow-up. Conclusion Infective etiology must be ruled out in immunosuppressed patients before considering periocular corticosteroids. Dual viral involvement, although rare, may cause fulminant retinitis in predisposed individuals. High index of suspicion and PCR from ocular fluids should be performed at the earliest in patients with atypical or poorly responding retinitis lesions.

In recent years, syphilis (Treponema pallidum) has become increasingly prevalent in Canada, and as a result, rates of ocular syphilis are also rising. Classically, syphilis was seen primarily in men who have sex with men; now, it is increasingly seen in people of all age groups, sexes, and sexual orientations. We present a series of 26 cases of ocular syphilis from London, Ontario, 5 of which are discussed in detail to illustrate the varied presentations and diagnostic challenges of ocular syphilis. The presentations include uveitis, iris granuloma (gumma), retinitis (acute syphilitic posterior placoid chorioretinitis), vasculitis, optic neuritis, and serous retinal detachment. The 5 cases are mostly middle-aged heterosexual men and women without the typical risk factors that would alert the examiner to suspect syphilis. We emphasize the importance of testing for syphilis when assessing and treating inflammatory eye disease, regardless of demographics and known risk factors, given the increasing prevalence of this disease. Diagnosis of syphilis relies on serologic testing, which is complex and has undergone significant changes from historical reliance on the Venereal Disease Research Laboratory test. We provide an overview of the strategy and rationale for modern serologic testing. The mainstay of treatment remains intravenous penicillin G, with alternative antibiotics (e.g., ceftriaxone) being less effective.

Background Intraocular lymphoma accounts for fewer than 1% of intraocular tumors. When the posterior segment is involved, it can be further classified as vitreoretinal or choroidal lymphoma. Vitreoretinal lymphoma (VRL) can rarely masquerade as an infectious retinitis making diagnosis and management challenging. Results A 73-year-old woman with a history of non-central nervous system (CNS) involving diffuse large B-cell lymphoma (DLBCL) was referred for worsening blurry vision—visual acuity of count figures at 2 ft—in her right eye for 8 months. Dilated fundus examination of the right eye was significant for retinal whitening and dot-blot hemorrhages, which was concerning for a viral retinitis and guided initial management. Secondary intraocular lymphoma was also considered. The retinal disease continued to progress despite intravitreal and systemic antiviral therapy, and a diagnostic vitrectomy was inconclusive. A retinal biopsy was then performed, which showed DLBCL, confirming a diagnosis of secondary VRL. Three subsequent treatments with intravitreal methotrexate led to regression of the VRL. Conclusions Our case highlights the utility of a retinal biopsy after an inconclusive diagnostic vitrectomy in a challenging scenario of VRL to establish a diagnosis and initiate successful treatment. A multidisciplinary team of providers was essential for diagnosis, comprehensive workup, medical and surgical management of the patient.

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.

Purpose: To report a case of unilateral progressive atypical acute zonal outer retinitis in a healthy young female.Case summary: A 32-year-old healthy woman presented with visual disturbance in the left eye for 1 week. Best corrected visual acuity was 0.4 in the left eye. No abnormal findings were detected on brain magnetic resonance imaging and a laboratory work-up. Zonal retinal opacity around the optic nerve disc was noticed in a fundus photo which was consistent with the outer retinal signal defect on optical coherence tomography and a defect in the central visual field from perimetry. Multiple evanescent white dot syndrome was suspected based on fluorescein angiography and indocyanine green angiography. Prednisolone was prescribed. Visual acuity was reduced to hand-motion after 10 days with extended zonal retinal opacity. No virus was detected by multiplex polymerase chain reaction of the aqueous humor. The lesion did not improve despite high-dose intravenous steroid and antiviral treatment.Conclusions: Visual function was lost with the progression of outer retinitis. This case differed from previous cases as the outer retinitis progressed rapidly from the posterior pole to the peripheral retina. It was named fulminant progressive atypical acute zonal outer retinitis.

Purpose: The purpose of this study is to evaluate the anatomical and functional outcomes of 25-gauge (G) pars plana vitrectomy (PPV) in patients with cytomegalovirus retinitis (CMVR)-related rhegmatogenous retinal detachment (RRD). Methods: Single-center retrospective consecutive case series of patients who underwent 25-G PPV for CMVR-related RRD repair with a minimum follow-up of 3 months. Complete anatomic success was defined as the complete attachment of retina including the periphery. Best-corrected visual acuity (BCVA) of ≥20/400 was defined as functional success. Results: Sixteen eyes of 15 patients were included in the study. Eleven patients were human immunodeficiency virus positive, three patients had hematological malignancies, and one patient suffered from dyskeratosis congenita. The mean follow-up was 20.5 ± 17.4 months (range 3–60 months). Complete anatomical success was seen in 15 eyes (93.75%). One eye had a residual inferior detachment with attached macula. Silicone oil was used as tamponade in 15 eyes and C3F8 gas in one eye. The mean change in BCVA was statistically significant, preoperative LogMAR BCVA was 2.05 ± 0.94 while the final follow-up postoperative LogMAR BCVA was 1.03 ± 0.61 (P < 0.001). Thirteen eyes (81.25%) had final BCVA ≥20/400. Conclusion: Microincision vitrectomy surgery can achieve excellent retinal reattachment rates in post-CMVR RRDs without significant intraoperative and postoperative complications. The visual outcome varies depending on the status of the optic disc and macula. Majority of the patients maintained functional vision.

To determine classification criteria for acute retinal necrosis (ARN).Machine learning of cases with ARN and 4 other infectious posterior uveitides / panuveitides.Cases of infectious posterior uveitides / panuveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the infectious posterior uveitides / panuveitides. The resulting criteria were evaluated on the validation set.Eight hundred three cases of infectious posterior uveitides / panuveitides, including 186 cases of ARN, were evaluated by machine learning. Key criteria for ARN included (1) peripheral necrotizing retinitis and either (2) polymerase chain reaction assay of an intraocular fluid specimen positive for either herpes simplex virus or varicella zoster virus or (3) a characteristic clinical appearance with circumferential or confluent retinitis, retinal vascular sheathing and/or occlusion, and more than minimal vitritis. Overall accuracy for infectious posterior uveitides / panuveitides was 92.1% in the training set and 93.3% (95% confidence interval 88.2, 96.3) in the validation set. The misclassification rates for ARN were 15% in the training set and 11.5% in the validation set.The criteria for ARN had a reasonably low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.

Retinitis following typhoid fever is an immune-mediated reaction that is rarely reported in literature. We present a case of a 20-year-old pregnant female who presented with progressive loss of vision in both eyes for three weeks. She had completed treatment for enteric fever 4 weeks before. Presenting vision was 6/36 in right eye and 3/60 in left eye. Fundus examination showed retinitis with retinal haemorrhages and macular stars in both eyes. After approval from gynaecologist, oral steroid was given. Vision improved significantly to 6/6 in right eye and 6/12 in left eye after 1.5 months of treatment. There was resolution of haemorrhages, exudates and stellate maculopathy. Hence, post-typhoid immune-mediated retinitis is rare sequelae of typhoid fever which can be treated with good visual outcome using oral steroids.

To describe composite multicolour (MC) imaging features along with the monocoloured fundus reflectance images in active and resolving stages of post-fever retinitis (PFR). Retrospective image analysis of cases of PFR who underwent dilated retinal clinical examination followed by optical coherence tomography and MC imaging. Twenty-five eyes of 18 patients diagnosed with PFR were included. There were 11 males and 7 females. Mean age of patients was 30.63 years. The retinitis lesion appeared bright white on MC image and white mainly on blue and green reflectance images during the active stages of PFR. The lesion appeared dull-grey to greyish white during the resolving stages and as dull-green in resolved cases. The active stages showed the presence of intraretinal/subretinal fluid which appeared as green colour on MC images and less green to normal during resolving stages. Hard exudates were seen as bright yellow- or orange-coloured spots on MC image during the resolving stages of the disease. The different stages of PFR show different colour on multicolour image and different reflectance patterns on individual colour reflectance channels. Hence, multimodal fundus imaging with different wavelength can be helpful for differentiation of activity in PFR.

To evaluate the clinical characteristics and treatment outcomes of idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) in a cohort of Chinese patients. The clinical history, imaging evaluation, treatment and outcomes of 42 eyes in 21 patients diagnosed with IRVAN in a 15-year period were reviewed. Most patients were females (90%) ranged from 15 to 58 years old. The initial decimal best corrected visual acuity (BCVA) of the patients ranged from light perception (LP) to 1.5 (0.55 ± 0.38). Eighteen eyes were in stage 2; 21 eyes in stage 3; and 1 in stage 5 at the initial visits according to the present staging system. Two eyes had vitreoretinal fibrovascular proliferation (FVP) and tractional retinal detachment (RD) at the initial visit. Intra-retinal microvascular abnormality (IRMA) was found in 7 eyes. Thirty-four eyes received retinal photocoagulation, 27 of which were pan-retinal photocoagulation (PRP). Total of 8 PPV were performed for VH, vitreoretinal FVP and RD, and macular epimembrane. Aneurysms on the head of optic nerve and artery bifurcations disappeared in 8 eyes and decreased in number in 2 eyes 1 year after photocoagulation. However, the BCVA of the patients did not have significant difference from that at the initial visits (P = 0.534). Seven eyes suffered severe visual impairment (BCVA ≤ 0.1) due to vitreoretinal FVP and tractional RD (3), exudative maculopathy (2), paracentral acute middle maculopathy (PAMM)(1), and neovascular glaucoma (NVG) (1). We found that IRVAN have a predilection to female gender. Vitreoretinal FVP and tractional RD and exudative maculopathy are major causes of severe visual impairment in IRVAN patients. We propose to revise the present staging system to include vitreoretinal FVP and RD in the staging of IRVAN patients.

To clarify the cut off value of blood CMV load to indicate CMV retinitis and its relationships with ocular features.Patients were divided into non-CMV and CMV retinitis groups. A logistic regression model was applied to estimate the association of each variable with CMV retinitis. Spearman correlation was used to estimate the correlation between the blood and aqueous CMV load.Blood CMV load higher than 4logBlood CMV load higher than 4log

Diffuse unilateral subacute neuroretinitis (DUSN) is a rare form of chorioretinitis caused by retinal infestation by nematode larvae. Definitive diagnosis requires visualization of a motile nematode, but this can be challenging because the nematode is small, translucent, and usually motionless with overlying media opacity from cellular inflammation. The authors describe a case of DUSN in which widefield swept-source optical coherence tomography angiography was used to localize a midperipheral intraretinal nematode facilitating subsequent clinical observation of movement. Inflammation resolved after focal laser photocoagulation of the nematode with recovery of visual acuity to 20/20. [ Ophthalmic Surg Lasers Imaging Retina . 2021;52:345–349.]

Background Cytomegalovirus (CMV) retinitis is a common opportunistic infection in patients with acquired immunodeficiency syndrome. The common funduscopic manifestations are haemorrhagic necrotising variety and granular variety. Frosted branch angiitis (FBA), as a special form, when it occurred after antiretroviral therapy(ART), could possibly be associated with immune reconstitution. We report a case of FBA secondary to CMV infection-associated unmasking immune reconstitution inflammatory syndrome (IRIS). Case presentation A 27-year-old man with human immunodeficiency virus infection developed FBA after 35 days of ART. The left Aqueous humour (AqH) tested positive for CMV DNA, and the patient was diagnosed with CMV retinitis. The degree of intraocular inflammation was reflected by increased levels of interleukin (IL)-6 and IL-8 in AqH. After anti-CMV treatment and continuous ART for several months, his FBA and vision significantly improved. CMV DNA became undetectable in the left AqH, and the IL-6 and IL-8 levels in AqH decreased. Conclusion FBA could be a sign of CMV-associated unmasking IRIS. Anti-CMV treatment alone or combination with steroid treatment may be administered, depending on the changes in CMV DNA load and immunologic profile of AqH.

We experienced an atypical endophthalmitis occurring post consecutively performed in-office procedures; an intravitreal injection (IVI) of ranibizumab followed by an anterior chamber (AC) paracentesis performed twice in an eye with neovascular glaucoma (NVG). A 52-year-old diabetic male who was asymptomatic developed signs of endophthalmitis and decreased vision without pain in his left eye a few days post-IVI and AC paracentesis. The condition worsened after an initial vitreous tap and injection of antibiotics. Cultures of vitreous and aqueous samples were negative. Complete resolution occurred after a pars plana vitrectomy with IVI of antibiotics and steroid with removal of a dense “yellowish-brown” fibrinous plaque. The absence of pain, presence of a peculiar colored fibrin, mild-to-moderate vitritis without retinitis, negative cultures, and complete recovery despite the fulminant presentation; favor a diagnosis of inflammation over infection. We hypothesize that a micro-leak from a 26-gauge AC tap tract might have served as an entry port for 5% povidone-iodine from the ocular surface thus inciting inflammation. However, an exuberant inflammatory response that can be typically seen in NVG eyes after intraocular procedures cannot be excluded. Various causes of inflammation post-procedures, both toxic and nontoxic should be considered in atypical culture-negative fulminant endophthalmitis cases with good outcome posttreatment. Any minor ocular procedure may carry a risk of such complication. Patient counseling and care must be exercised in performing these procedures.

Contains fulltext : 237970.pdf (Publisher’s version ) (Open Access) The CEP83 protein is an essential part in the first steps of ciliogenesis, causing a ciliopathy if deficient. As a core component of the distal appendages of the centriole, CEP83 is located in almost all cell types and is involved in the primary cilium assembly. Previously reported CEP83 deficient patients all presented with nephronophthisis and kidney dysfunction. Despite retinal degeneration being a common feature in ciliopathies, only one patient also had retinitis. Here, we present two unrelated patients, who both presented with retinitis pigmentosa, without nephronophthisis or any form of kidney dysfunction. Both patients harbor bi-allelic variants in CEP83. This report expands the current clinical spectrum of CEP83 deficiency. For timely diagnosis of CEP83 deficiency, we advocate that CEP83 should be included in gene panels for inherited retinal diseases.

Purpose To evaluate the effect of prophylactic anti-malarial chloroquine treatment, and its cessation, on electroretinographic (ERG) responses of captive African penguins. Methods A brief ERG protocol ("QuickRetCheck") was recorded under mesopic conditions with manual restraint and no sedation or pupil dilation. Birds were recorded on two separate occasions, first while being treated with a daily chloroquine dose of 10 mg/kg for 12 days (n = 15, treatment group) and second after 4 months without chloroquine treatment (n = 6, off-treatment group). Three birds were recorded on both occasions. Three other birds from the flock that died were studied histopathologically. Results Scotopic responses were unmeasurable in either recording and therefore were not analyzed. Mean a- and b-wave amplitudes of the mixed rod-cone responses to standard (3 cd·s/m2 ) and high (10 cd·s/m2 ) intensity flashes were higher in the off-treatment group. No difference in implicit times was observed. Sex, age, and number of previous chloroquine treatments did not affect ERG responses. Histopathology revealed Plasmodium spp.in the lungs, liver, and brain, but not in the eyes, of the necropsied birds, and there were no signs of retinitis or retinopathy. Conclusions Daily chloroquine treatment was associated with attenuated ERG responses in penguins, which improve following cessation of treatment. Further work is warranted to establish a chloroquine dose that is efficacious yet has minimal adverse effects. Our results suggest that ERG responses of captive penguins undergoing ERG for any indication (such as prior to cataract surgery), must be evaluated in light of the birds' anti-malaria treatment status.

Purpose: To compare the efficacy of adjuvant therapy of topical steroids eye drops with oral antibiotics and monotherapy of oral antibiotics in a typhoid fever associated retinitis. Materials and Methods: The study was conducted on a 30yr old male who presented to our OPD with decreased vision in left eye and was followed up for 1 month. Results: A 30yr old male patient presented to our OPD with decreased vision with left eye with history of typhoid fever for a duration of 4 weeks and was treated successfully. His BCVA was 6/6 in Right Eye and 6/36 in Left Eye. Fundus examination revealed multiple whitish ill defined lesions suggestive of retinitis lesions which was noted superiorly to the disc which was associated with macular neuro-sensory detachment and blurring of disc margins in the left eye. Significant improvement in BCVA was observed with adjuvant therapy of topical steroids with antibiotics over a period of 1 month. Diagnosis of post typhoid immune mediated retinitis was made with good resolution following treatment. Keywords: Post typhoid retinitis, Immune mediated retinitis, Post fever retinitis.

Crohn’s disease and ulcerative colitis are chronic inflammatory bowel diseases, which are often accompanied by inflammation of other organs. This article presents modern data on etiology, pathogenesis and clinical course of inflammatory bowel diseases, as well as information on extraintestinal eye manifestations of nonspecific ulcerative colitis and Crohn’s disease. The role of microbiota, genetic factors, immune system defects in pathogenesis of intestinal inflammation and extraintestinal eye manifestations is considered. The possibility the development of ophthalmopathology not only against the background of intestinal inflammation, but also as a consequence of therapeutic and surgical methods of treatment of ulcerative colitis and Crohn’s disease is noted. The peculiarities of the course of episcleritis/scleritis, keratitis, uveitis, chorioretinitis, optical neuritis for patients with inflammatory bowel diseases are considered. The presence of these complications may reflect the activity of the underlying disease, which in some cases requires correction of therapy. Anterior uveitis and episcleritis/scleritis are the most common extraintestinal manifestations of inflammatory bowel disease. Inflammation of tissues of the posterior segment of the eye and optic nerve against the background of ulcerative colitis and Crohn’s disease are less common, but are of clinical importance, as they can catastrophically damage the structures of the eye and, as a consequence, lead to complete blindness. Considering the possibility of mild clinical symptoms and asymptomatic course of inflammation in the eye envelopes, the importance of ophthalmological examination of all patients with ulcerative colitis and Crohn’s disease is emphasized. Aspects of modern therapy of ophthalmopathology and background intestinal inflammation are highlighted. Biological preparations — antagonists of pro-inflammatory cytokines — have been identified as the most promising in the treatment of inflammatory intestinal diseases and extraintestinal manifestations. The important role of proper nutrition and biologically active supplements containing omega-3 fatty acids, vitamin D, microelements, was noted as auxiliary therapy of both intestinal and extraintestinal inflammation.

PURPOSE To evaluate microstructural changes in cystoid macular edema in retinitis pigmentosa after intravitreal dexamethasone implant injection. METHODS In an extended cohort of a randomized trial of intravitreal dexamethasone implant for the management of retinitis pigmentosa-associated cystoid macular edema, microstructural changes during six months after the treatment were evaluated using spectral-domain optical coherence tomography. RESULTS Forty-two eyes were included, and all had cystoid space in the inner nuclear layer (INL) at baseline. No eyes showed subretinal fluid, and 28.6% showed hyperreflective foci. Among 38 eyes with cystoid space both in the INL and outer nuclear layer/Henle's layer, 13 (34.2%) showed complete resolution and 12 (31.6%) showed cystoid space only in the INL at 2 months after injection, whereas others showed persistent cystoid space in both layers. After complete resolution, cystoid space recurrence was earlier in the INL than in the outer nuclear layer/Henle's layer. Multivariable analysis showed that greater cystoid space area in the INL and outer nuclear layer/Henle's layer, presence of macular leakage, and longer intact external limiting membrane at baseline were associated with greater cystoid space area decrease after the treatment. CONCLUSION Resolution and recurrence pattern of retinitis pigmentosa-associated cystoid macular edema after dexamethasone treatment showed that the INL is the primary layer of cystic change, and this suggests its pathogenesis is most likely caused by Muller cell dysfunction.

We report a finding of a pigmented chorioretinal scar with acute retinal necrosis (ARN) caused by herpes simplex virus 2 (HSV-2) infection rather than toxoplasma, creating an initial diagnostic dilemma. A 53-year-old functionally monocular male presented with painless floaters and blurry vision in his seeing eye over a period of 4 days. An exam demonstrated anterior chamber (AC) reaction, vitritis, multifocal patches of whitening, and an occlusive retinal vasculitis. A superior pigmented chorioretinal scar with overlying contracted vitreous was noted in the periphery with no adjacent retinal whitening. The patient was treated for both ARN and toxoplasma chorioretinitis until PCR study of the vitreous and AC returned positive for HSV-2 and negative for toxoplasmosis. Management consisted of a dual therapy regimen of both oral and intravitreal antiviral agents as well as oral corticosteroids. The patient’s clinical course was complicated by rhegmatogenous retinal detachment within 2 weeks after symptom onset, requiring pars plana vitrectomy with silicone oil and intraoperative intraocular incubation with foscarnet. We review emerging evidence for pigmented chorioretinal scars in ARN specifically caused by HSV-2, as well as diagnostic and treatment dilemmas in the management of ARN and ARN detachments.

Behçet's disease is a multi-organ inflammatory disorder with systemic vasculitis of unknown etiology. Ocular lesions occur in about 70% of patients with Behçet's disease, and it is more frequent and severe in men. The frequency of ocular inflammatory attacks has been used as a main outcome measure to assess the efficacy of therapy on uveoretinitis in patients with Behçet's disease. The ocular Behçet's disease research group of Japan have recently proposed a new scoring system, Behçet's disease ocular attack score 24 (BOS24), to assess the disease activity of ocular Behçet's disease. This review highlights the efficacy and application of the BOS24 scoring system in clinical practice for patients with ocular Behçet's disease. In addition, a new semi-quantitative scoring system to evaluate the degree of retinal vascular leakage on fluorescein angiography reported by our group is described.

Purpose Determine the efficacy of combination intravitreal and systemic antiviral therapy for the treatment of acute retinal necrosis (ARN) and risk factors impacting visual acuity (VA) and retinal detachment (RD) outcomes. Design Single-center retrospective case series. Participants Patients with an ARN diagnosis based on clinical features and polymerase chain reaction confirmation who were treated at a tertiary referral, university-based academic practice. Methods Patient records were reviewed for demographic information including age and gender. Snellen VA, disease findings including RD outcomes, optic nerve involvement, and treatments were recorded. Incidence rates of major VA and RD outcomes were calculated based on the number of events and exposure times. Cox proportional hazards regression modeling and survival analyses were used to identify factors related to VA and RD outcomes over time. Main Outcome Measures Logarithm of the minimal angle of resolution VA, 2-line or more VA gain, severe vision loss (SVL) of 20/200 or worse, RD development, and fellow eye involvement. Results Twenty-three eyes of 21 patients (11 male, 10 female) were reviewed. Thirteen patients (62%) had herpes simplex virus and 8 patients (38%) had varicella zoster virus. The event rate for 2-line or more VA gain was 0.49 events/eye-year (95% confidence interval [CI], 0.26–0.86 events/eye-year), whereas the rate of SVL was 0.61 events/eye-year (95% CI, 0.34–1.02 events/eye-year). Retinal detachment development was observed at a rate of 0.59 events/eye-year (95% CI, 0.33–1.00 events/eye-year). Thirteen of 23 eyes (57%) demonstrated RD with a mean time of 120 days after ARN diagnosis. With each additional quadrant of retina involved, a greater risk of RD development over time was observed (hazard ratio, 2.21; 95% CI, 1.12–4.35). Nine percent of eyes progressed with additional quadrantic involvement, despite combination systemic and intravitreal antiviral therapy; however, none of the 19 patients demonstrating unilateral ARN showed fellow-eye involvement after initiation of therapy. Conclusions Combination intravitreal and systemic antiviral therapy for ARN can be effective in improving VA and limiting retinitis progression. Each additional quadrant of retina involved was associated with a 2.2-fold greater risk of RD, which may impact monitoring, timing of intervention, and patient counseling.

Introduction Bartonella henselae causes cat scratch disease (CSD), spread by a cat scratch or bite. Cats are its main reservoir. This sometimes results in optic neuritis or neuroretinitis. Objective To review these conditions in Gipuzkoa (Spain), 2014−2019. Methods A retrospective review of serology registries and clinical registries, selecting those with consistent clinical signs, contact with cats and positive serology for B. henselae (IgG-IFA ≥1/256). Results Sixty-four patients had CSD. Of these, one had optic neuritis and 3 had neuroretinitis (4/64; 6.3%). In 3 patients, flu-like symptoms preceded eye symptoms. Two suffered from loss of visual acuity at discharge, despite prolonged treatment with antibiotics and corticosteroids. Conclusion Optic neuritis and neuroretinitis caused by B. henselae are severe complications with a non-negligible incidence among patients with CSD in Gipuzkoa. We recommend ruling out CSD in patients with symptoms of optic neuritis or neuroretinitis (sudden vision loss, etc.) and contact with cats.

Purpose Review octopus ocular anatomy and describe the histopathologic findings in three octopuses diagnosed with phakitis and retinitis. Animals Two common octopuses (Octopus vulgaris) and one giant Pacific octopus (Enteroctopus dofleini) with a history of ophthalmic disease. Methods A literature search was performed for the ocular anatomy section. Both eyes from all three octopuses, and two control eyes, were submitted for histopathologic evaluation. Hematoxylin and eosin stain was used for standard histopathologic evaluation; GMS stain was used to screen for fungi, gram stain for bacteria; and Fite's acid fast stain for acid fast bacteria. Results Anatomically, the anterior chamber of the octopus has direct contact with ambient water due to an opening in the dorsal aspect of a pseudocornea. The octopus lens is divided into anterior and posterior segments. The anterior half is exposed to the environment through the opening into the anterior chamber. Neither part of the lens has a lens capsule. The retina is everted, unlike the inverted vertebrate retina, and consists of just two layers. Histopathology revealed inflammatory phakitis and retinitis of varying severity in all six eyes of the study animals. No intraocular infectious organisms were recognized but one common octopus eye had clusters of coccidian parasites, identified as Aggregata sp., in extraocular tissues and blood vessels. Conclusion We describe inflammatory phakitis and retinitis in two species of octopuses. The underlying cause for the severe intraocular response may be direct intraocular infection, water quality, an ocular manifestation of a systemic disease, or natural senescence.

To describe a case of outer retinitis with frosted branch angiitis associated with mumps infection treated with hyperbaric oxygen (HBO) therapy.Observational case report.A four-year-old boy with bilateral blindness was diagnosed with necrotizing outer retinitis with frosted branch angiitis associated with serologically confirmed mumps virus infection. He was treated with HBO therapy. Visual acuity subsequently improved to 20/40 in the right eye and to 20/320 in the left eye. Sequential follow-up optical coherence tomography examinations showed progressive recovery of the outer retinal layers in the right eye.HBO therapy appears to be a feasible and safe treatment that might improve the anatomical and functional outcome in patients with mumps retinitis.

Purpose: The aim of this study was to describe the clinical features, course, and clinical outcomes of eyes with cytomegalovirus (CMV) retinitis in immunosuppressed patients of different etiologies. Methods: This was a retrospective observational study from a single ophthalmic tertiary care center. The patients included referrals from the nodal cancer center and the local human immunodeficiency virus (HIV) treatment clinic. Demographics, history, visual acuity, ocular features, treatment protocol, and final visual outcome of patients who were diagnosed with CMV retinitis in the period of five years from 2014 to 2019 were studied. Results: CMV retinitis was diagnosed in 25 eyes of 14 patients. Age of the patients ranged from 11–54 years. Ten (71.43%) patients were male and four (29.57%) were female. Eight of them had acute lymphoblastic leukemia (ALL), four were suffering from HIV infection and one patient each had lymphoma and history of a kidney transplant. The treatment for CMV retinitis ranged from two to sixty weeks depending on disease activity and systemic condition. Three of the patients were on maintenance therapy for ALL at the time of reactivation. Conclusion: Duration of treatment for CMV retinitis in patients of ALL was longer as compared to the other etiologies, and in recurrences, it needed to be continued till the completion of maintenance therapy for ALL. It is prudent to advise regular ophthalmic screening of all immunocompromised patients, as they are at a high risk of developing CMV retinitis. Patients of ALL, especially while on maintenance therapy, should be monitored for possible development or reactivation of CMV retinitis.

Cytomegalovirus retinitis is a rare complication in kidney transplant patients with only one other reported incidence. A 70-year-old female with a previous history of kidney transplant began experiencing a decreased vision of her right eye and was diagnosed with CMV retinitis and started on ganciclovir treatment. After completing the treatment, the patient has had no signs of recurrence or any other complaints. There is only one other published case report of CMV retinitis in a kidney transplant patient, therefore appropriate treatment and predictability of recurrence are unknown.

Aim The current world has changed in all shapes since the emergence of the novel coronavirus (nCoV-2) also known as COVID-19. Among the extra-pulmonary manifestations of nCoV-2, ophthalmic symptoms have less been systematically studied. The so far existing body of evidence indicates that nCoV-2 has the potential to affect both anterior and posterior chambers of the eye. Albeit, the exact mechanisms which underlie ophthalmic manifestations of nCoV-2 are yet to be elucidated. Methods The present brief review is an attempt to put together and highlight the significant yet limited number of studies which have spotlighted ophthalmic issues in nCoV-2 patients using a systematic literature search strategy. Results All case series or reports (including both published and preprint articles) which described ocular manifestations of patients with COVID-19 and/or documented testing of SARS-COV-2 in ocular secretions via various sampling or detection methods were sought to be included. Conclusion The ophthalmic presentations in SARS-COV-2 are often found to be salient. Raising awareness in this respect may help defining evidencebased protective measures in today’s practice of ophthalmology and allied disciplines.

Purpose The aim of this work was to study the ocular manifestations and its management in spotted fever and typhus group of rickettsial disease. Methods A retrospective analysis of 50 patients with serologically confirmed Rickettsial disease. In all patients, relevant history, investigations and treatment details were collected and they underwent complete ophthalmic evaluation including measurement of best-corrected visual acuity, anterior segment examination and dilated fundus examination. Results Mean age was 12.5 ± 8.99 years. Of the 50 patients, 40 patients were ≤18 years of age and 27 (54%) had ocular involvement. Out of 27 patients, bilateral involvement was seen in 10 patients. Most of the patients had no ocular symptoms. Ocular findings included, Retinal vasculitis 6 (22.22%); macular edema 4 (14.81%); vasculitis with macular edema 1 (3.7%); Retinitis 7 (25.92%); Papilloedema 6 (22.22%); Papilloedema with 6th cranial nerve palsy 1 (3.7%); Isolated 6th cranial nerve palsy 1 (3.7%) and optic neuritis 1 (3.7%). Ocular involvement was more common in double antigen group (68%) than spotted fever group (50%) or Scrub typhus group (21%) (P = 0.01). Ocular involvement was seen in 94% of the patients with CNS involvement. Cases with bilateral involvement (P = 0.01), pediatric age group (P = 0.01) and CNS involvement (P = 0.02) had poor visual outcome. Conclusion Rickettsioses patients can have ocular manifestations with predominant posterior segment involvement during acute phase of illness. Ocular involvement was more common in the double antigen group. For any patient who presents with fever and rash living in endemic area, ophthalmic evaluation should be part of routine checkup during the acute phase of illness associated with less frequent ocular symptoms.

Objectives: The aim of present work was to assess cytomegalovirus (CMV) viremia in Iranian human immunodeficiency virus (HIV)-1-infected patients with a CD4+ count 3 and to explore whether CMV DNA loads correlate with CD4+ cell counts or associated retinitis. Methods: This study was conducted at the AIDS research center in Iran on HIV-1-infected patients with CD4+ count 3, antiretroviral therapy-naive, aged ≥18 years with no previous history of CMV end-organ disease (CMV-EOD). Results: Thirty-nine of 82 patients (47.56%) had detectable CMV viral load ranging from 66 to 485,500 IU/mL. CMV viral load in patients with retinitis ranges from 352 to 2,720 IU/mL, and it was undetectable in 2 patients. No significant associations between CMV viremia and CD4+ cell count was found (p value = 0.31), whereas significant association of CMV viremia in HIV-infected patients with retinitis was found (p < 0.02). Conclusions: We estimated the frequency of CMV viral load infection in Iranian HIV-1-infected patients with a CD4+ cell count 3/mL in the largest national referral center for HIV-1 infection in Iran. Further research is required on the relevance of CMV viral load in diagnostic and prognostic value of CMV-EOD.

Typhoid fever is an acute infectious disease capable of rarely producing ocular complications. These complications are usually seen after the third week, and is notoriously called, as the "Week of complications" by direct invasion or by allergic immune mediated reactions. It can present as uveitis, vasculitis, choroiditis, endophthalmitis or panophthalmitis. We here present an 18 year old boy presenting as retinitis with severe exudation and neurosensory detachment and which was managed successfully with steroids.

Objective To describe functional and structural features of presumed cancer‐associated retinopathy (CAR) mimicking sudden acquired retinal degeneration syndrome (SARDS) in dogs and describe treatment outcomes. Animals Subjects were 17 dogs from 8 eight US states and Canada diagnosed with SARDS or immune‐mediated retinitis (IMR) by 12 ophthalmologists. Nine eyes from seven deceased patients were used for microarray (MA), histology, or immunohistochemical (IHC) analysis. Procedures Dogs underwent complete ophthalmic examination, including retinal photography, optical coherence tomography (OCT), chromatic pupil light reflex testing (cPLR), and electroretinography (ERG), in addition to complete systemic examination. Histology, microarray, and IHC analysis were performed in CAR retinas to evaluate histological and molecular changes in retinal tissue. Results None of the patients evaluated satisfied previously established criteria for diagnosis of SARDS (flat ERG+ no red – good blue PLR), and all were diagnosed with IMR. All patients were diagnosed with a cancer: meningioma (24%), sarcoma (18%), pituitary tumor (12%), and squamous cell carcinoma (12%), other (34%). Median survival time was 6 months from diagnosis (range 1‐36 months). Most frequent systemic abnormalities were as follows: proteinuria (78%); elevated liver enzymes (47%); and metabolic changes (PU/PD, polyphagia – 24%). Immunosuppressive therapy resulted in the reversal of blindness in 44% of treated patients, with 61% of all treated patients recovering and/or maintaining vision. Median time for preservation of vision was 5 months (range 1‐35 months). Conclusions Observed changes are highly suggestive of immune‐mediated damage in IMR‐CAR eyes. A relatively high percentage of patients with CAR responded positively to immunosuppressive therapy.

Although cytomegalovirus (CMV) remains a leading cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT), the incidence of CMV retinitis is considered to be lower than the incidence of CMV infection in other organs following allogeneic HSCT. In this study, the incidence and characteristics of CMV retinitis were retrospectively evaluated in recipients of allogeneic HSCT. Ophthalmological screening was performed at the development of ocular symptoms or positive CMV infection using peripheral blood evaluated by pp65 antigenemia or polymerase chain reaction. Of the 514 patients, 13 patients developed CMV retinitis. The median onset of CMV retinitis was day 34 (range, 21-118) post transplant, and the cumulative incidence was 2.5% (95% CI, 1.6-4.2) at 6 months after transplantation. Five patients presented ocular symptoms at the onset. In the remaining eight asymptomatic patients, the diagnosis of CMV retinitis was made by the screening guided by positive CMV infection. All evaluable patients responded to antiviral treatment but three showed incomplete improvement with ocular sequela. Our results suggest that the incidence of CMV retinitis after allogeneic HSCT is not negligible and active ophthalmological screening based not only on symptoms but also positive CMV infection monitoring contributes to the early diagnosis of CMV retinitis.

Neovascular complications from cytomegalovirus necrotizing retinopathy developed in 5.1%/cases and 3.4%/eyes in patients after haploidentical stem cell transplantation. Immune-mediated cytomegalovirus vasculitis could be an isolated manifestation in patients with a minimal immune deviation, which could only be found on fluorescein angiography and warrants the needs for long-term follow-up., Purpose: To report the incidence and clinical features of neovascular complications from cytomegalovirus (CMV) necrotizing retinopathy in patients after haploidentical hematopoietic stem cell transplantation. Methods: Thirty-nine patients (58 eyes) of CMV necrotizing retinopathy after haploidentical hematopoietic stem cell transplantation in our institute between January 2018 and June 2020 were retrospectively reviewed, and cases that developed neovascular complications during follow-up were identified and described. Results: Two (2 eyes) cases that developed neovascular glaucoma from CMV necrotizing retinopathy were identified. Both of them manifested as granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which were consistent with chronic retinal necrosis. Insidious progression of isolated immune-mediated occlusive vasculitis that could only be observed on fundus fluorescein angiography without active retinitis or intraocular inflammation was recognized to be the cause in one of two cases. Conclusion: Neovascular glaucoma developed in 5.1%/cases and 3.4%/eyes complicated by CMV chronic retinal necrosis and vasculitis in patients after haploidentical hematopoietic stem cell transplantation, which warrants the needs for long-term follow-up. Immune-mediated CMV vasculitis could be an isolated manifestation in patients with a minimal immune deviation and may only be found on fundus fluorescein angiography, which emphasizes the importance of fundus fluorescein angiography on a regular basis during follow-up.

The purpose of this case report is to describe the obstruction of cilioretinal artery in a patient with acute focal retinitis occurring due to Chikungunya virus (CHIKV) fever. A 45-year-old female ...

Background To report the first case of belatacept-associated multidrug-resistant Cytomegalovirus retinitis in a kidney transplant recipient. Case presentation A 76-year-old African male renal allograft recipient was admitted for acute visual loss of the right eye. Ophthalmological examination of the right eye showed anterior uveitis and vitritis associated with large paravascular haemorrhages and yellow necrotic borders, involving the posterior pole but not the fovea. Both Cytomegalovirus DNA in plasma and aqueous humor were positive. The patient had had several episodes of Cytomegalovirus reactivation subsequent to the introduction of belatacept. His cytomegalovirus was multi-drug resistant, and was treated with maribarir, intravitreal and systemic injections of foscarnet, and anti-Cytomegalovirus human immunoglobulin. In parallel, belatacept was stopped and switched to tacrolimus. Cytomegalovirus DNA became undetectable and there was partial improvement of visual acuity at the last ophthalmologic examination, 18 months after the initial diagnosis of Cytomegalovirus retinitis. Conclusion Cytomegalovirus retinitis is an uncommon opportunistic infection in kidney transplant recipients. Cytomegalovirus retinitis is a serious infection because of the risk of blindness and the occurrence of associated life-threatening opportunistic infections. In view of the recent literature, kidney transplant recipients treated by belatacept immunosuppression may be at increased risk for Cytomegalovirus disease, notably Cytomegalovirus retinitis. The occurrence of Cytomegalovirus retinitis may help improve the selection of patients converted to belatacept.

Background Ocular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of antiphospholipid syndrome (APS) that results in multiorgan failure and a high mortality rate, has rarely been reported. Case presentation A 15-year-old girl presented with sudden vision blurring in both eyes. She had marked optic disc swelling and macular exudates in the right eye and intra-arterial white plaques, a few retinal blot hemorrhages, and a white ischemic retina in the left eye. Systemic examination revealed she had acute kidney injury with thrombotic microangiopathy (TMA), multiple cerebral infarcts, valvular dysfunction, and a high titer of triple aPL. Thus, she was diagnosed with CAPS involving the brain, eyes, heart, and kidneys. Plasma exchange and the administration of glucocorticoids, immunoglobulin, warfarin, and rituximab brought a sustained recovery of the TMA, visual symptoms, and echocardiographic findings. Conclusions Ocular involvement of both vaso-occlusive retinopathy, an APS-related thrombotic microangiopathy, and neuroretinitis, a non-thrombotic microangiopathy, can occur as an initial presentation of CAPS.

Purpose. To present a rare clinical case of bilateral Coates retinitis in a 5-year-old girl. Material and methods. A clinical case of a 5-year-old girl with rare Coat's retinitis in both eyes is described. The disease occurred abruptly: the child's parents noted the appearance of leukocoria in the right eye. After the examination Coates ' retinitis was diagnosed. At the time of examination in the clinic, there was a retinal detachment in the right eye, the surgical treatment was performed: lensvitrshvartectomy, plastic surgery of the anterior chamber, with the melting of the retina with perfluoroorganic compounds, drainage of subretinalfluid, endolaser coagulation on the right eye. A month after the first symptoms appeared in the right eye, the patient complained of decreased vision in the left eye. Examination on the pediatric retinal camera showed an increase in vascular activity in the left eye, and signs of Coats' retinitis were revealed. Results. As a therapy for this disease, the patient was twice injected intravitreally inhibitor of vascular endothelial growth factor (VEGF inhibitor) into the left eye with positive dynamics noted as a decrease in the activity of retinal vessels and leveling of the exudative component, as well as with an increase in visual acuity in the left eye. Conclusion. This clinical case demonstrates that intravitreal administration of inhibitor VEGF with traditional methods of treatment can be used as a treatment for patients with Coates retinitis. Key words: Coat's retinitis, Coats' disease, vascular endothelial growth factor inhibitor, aflibercept, intravitreal injection, anti-VEGF, retinal detachment.

Purpose: Ocular syphilis is re-emerging globally especially in patients with human immunodeficiency virus (HIV). Atypical manifestations of ocular syphilis and/or other associated opportunistic infections often lead to a diagnostic dilemma. We evaluated the utility of aqueous humor (AH) Treponema pallidum hemagglutination assay (TPHA) titers in the diagnosis of ocular syphilis. Methods: Retrospective case series of five HIV positive patients with positive syphilis serology in whom AH sampling was performed. All patients had ocular manifestations suspicious of infectious etiology. Results: Panuveitis with/without retinitis was the commonest presentation. Along with blood investigations, polymerase chain reaction (PCR) testing from AH was done for Mycobacterium tuberculosis (MTB), Herpes Simplex Virus (HSV), Varicella Zoster Virus (VZV), Cytomegalovirus (CMV), and Toxoplasma gondii. In addition, serum antibody titers for Toxoplasma, rapid plasma reagin (RPR) and TPHA tests for syphilis were done. In patients with raised serum RPR/TPHA, aqueous TPHA titers were also assessed. Mean serum RPR titer was ≥ 1:32 and TPHA titer was ≥1:1280. Aqueous humor titers of TPHA was high in all patients (range ≥ 1:320 to ≥1:5120). Aqueous PCR was negative for all other infectious etiologies in four patients. In one patient, PCR-CMV was also positive, suggestive of a dual infection. Post-treatment with highly active antiretroviral therapy (HAART) and appropriate anti-syphilitic regime, complete resolution of lesions with corresponding fall in the serum RPR/TPHA titers were noted in all patients. Conclusions: Ocular syphilis with atypical presentations is usually diagnosed based on a positive syphilis serology and by excluding other infectious causes. In the present study, we have shown an excellent correlation between raised AH TPHA titers with serological values and the clinical presentation. Considering the ease of collection of AH in contrast to vitreous fluids, the AH-TPHA assay could potentially be a valuable tool in the diagnosis of ocular syphilis.