Post-fever retinitis (PFR) is an infectious or para-infectious uveitic entity caused by bacterial or viral agents and seen mainly in tropical countries. Systemic symptoms such as joint pain, skin rash are common during the febrile stage. On the basis of only clinical presentation, it is difficult to pin-point the exact etiology for PFR. Serological investigations, polymerase chain reaction, and knowledge of concurrent epidemics in the community may help to identify the etiological organism. Bacterial causes of PFR such as rickettsia and typhoid are treated with systemic antibiotics, with or without systemic steroid therapy, whereas PFR of viral causes such as chikungunya, dengue, West Nile virus, and Zika virus have no specific treatment and are managed with steroids. Nevertheless, many authors have advocated mere observation and the uveitis resolved with its natural course of the disease. In this article, we have discussed the clinical features, pathogenesis, investigations, and management of PFR.

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Viral anterior uveitis (VAU) needs to be suspected in anterior uveitis (AU) associated with elevated intraocular pressure, corneal involvement, and iris atrophic changes. Common etiologies of VAU include herpes simplex, varicella-zoster, cytomegalovirus, and rubella virus. Clinical presentations can vary from granulomatous AU with corneal involvement, Posner-Schlossman syndrome, Fuchs uveitis syndrome, and endothelitis. Due to overlapping clinical manifestations between the different viruses, diagnostic tests like polymerase chain reaction and Goldmann-Witmer coefficient analysis on the aqueous humor may help in identifying etiology to plan and monitor treatment.

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Diagnosis and management of ocular tuberculosis (OTB) poses a significant challenge. Mixed ocular tissue involvement and lack of agreement on best practice diagnostic tests together with the global variations in therapeutic management contributed to the existing uncertainties regarding the outcome of the disease. The current review aims to update recent progress on OTB. In particular, the Collaborative Ocular Tuberculosis Study (COTS) group recently standardized a nomenclature system for defining clinical phenotypes, and also proposed consensus guidelines and an algorithmic approach for management of different clinical phenotypes of OTB. Recent developments in experimental research and innovations in molecular diagnostics and imaging technology have provided a new understanding in the pathogenesis and natural history of the disease.

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Scleritis is a rare painful ocular disorder, associated with severe ocular pain and tissue destruction. Although a majority of these cases are immune mediated and at least half of these are associated with systemic immune-mediated diseases, a smaller minority are due to infections of the sclera. The two conditions closely mimic each other, and a thorough knowledge of the subtle differences is necessary in order to reach a timely diagnosis. Diagnostic delay can lead to a poor outcome both due to the destruction caused by the uncontrolled infection and also due to propagation of the infection with the use of corticosteroids which may have been started for presumed immune mediated scleritis. In this review, we present the clinical features, etiological agents, and the differentiating features between immune and infectious scleritis. We also present diagnostic and management guidelines for managing scleral infection.

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Uveitis maybe induced by the use of various medications known as drug-induced uveitis (DIU), though rare it is an important cause of uveitis which one needs to be aware of. The drugs may be administered through any route including systemic, topical, and intravitreal. Ocular inflammation can be in the form of anterior, intermediate, posterior or pan uveitis, and rarely may present as episcleritis and scleritis. Identification of drug as the offending agent of uveitis is important as many a times stopping the drug may help recover the uveitis or the concomitant use of corticosteroids. An extensive literature review was done using the Pubmed. An overview of DIU is provided as it is important for us to be aware of this clinical entity.

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Autoimmune retinopathy (AIR) refers to a group of rare autoimmune retinal degenerative diseases presumably caused by cross-reactivity of serum autoantibodies against retinal antigens. The pathogenesis of AIR remains largely presumptive and there are a significant number of antiretinal antibodies that have been detected in association with AIR. The diagnosis of AIR is largely based on the demonstration of antiretinal antibodies in the serum along with suggestive clinical features and ancillary investigations. A high index of suspicion along with early diagnosis and treatment may play a critical role to lower the risk of irreversible immunological damage to the retinal cells in these patients. A multi-disciplinary approach for complete management and evaluation is helpful in such conditions. Various therapeutic options have been described for the treatment of AIR, though there is no consensus on standard treatment protocol.

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Interleukins and cytokines are involved in the pathogenesis of uveitis of heterogeneous origin. Understanding the basics of the ocular immune privilege is a fulcrum to discern their specific role in diverse uveitis to potentially translate as therapeutic targets. This review attempts to cover these elements in uveitis of infectious, noninfectious and masquerade origin. Insights of the molecular targets in novel therapy along with the vision of future research are intriguing.

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There are multiple approaches to inhibit inflammatory molecules and pathways in noninfectious uveitis. The cornerstone of local and systemic anti-inflammatory treatment is corticosteroid therapy. Corticosteroids remain the most potent and efficacious drugs for treating intraocular inflammation. However, their long-term use is limited by their medium- and long-term side effects, which are a major concern. The approach taken to limit corticosteroid side effects is to introduce steroid-sparing agents that suppress the inflammatory pathways and immune response differently than corticosteroids. There are several classes of such drugs that are affordable, effective, and generally well-tolerated. Relatively recently, an increasing range of biologic agents has become available to treat intraocular inflammation. However, the relatively expensive cost of these therapies limits their use in the developing world. This systemic review aimst to discuss the use of corticosteroids and different immunosuppressive regimens in the management of various uveitides.

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Uveitis is a complex disorder including both infectious and non-infectious etiologies. Clinical diagnosis is a challenge because many diseases share common clinical signs. Laboratory support is crucial for confirming the clinical diagnosis. Laboratory diagnosis includes direct tests and indirect tests. For example smear, culture, and molecular diagnostics demonstrate the pathogens, hence they are direct tests. Immunologic tests employ an antigen to detect presence of antibodies to a pathogen, or an antibody to detect the presence of an antigen, of the pathogen in the specimens. The immunological tests used in laboratories are made by producing artificial antibodies that exactly “match” the pathogen in question. When these antibodies come into contact with a sample they bind to the matching pathogen if found in the sample. Hence they are grouped under indirect evidence. There are several investigations in uveitis to reach the confirmed diagnosis including microbiological, immunological, imaging and molecular diagnostic testing. In this section we will discuss immunological investigations of infectious and non-infectious uveitis.

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Intraocular inflammation in patients with human immunodeficiency virus (HIV) infection is commonly due to infectious uveitis. Ocular lesions due to opportunistic infections (OI) are the most common and have been described extensively in the pre highly active antiretroviral therapy (HAART) era. Many eye lesions were classified as acquired immunodeficiency syndrome (AIDS) defining illnesses. HAART-associated improvement in immunity of the individual has changed the pattern of incidence of these hitherto reported known lesions leading to a marked reduction in the occurrence of ocular OI. Newer ocular lesions and newer ocular manifestations of known agents have been noted. Immune recovery uveitis (IRU), the new menace, which occurs as part of immune recovery inflammatory syndrome (IRIS) in the eye, can present with significant ocular inflammation and can pose a diagnostic and therapeutic challenge. Balancing the treatment of inflammation with the risk of reactivation of OI is a task by itself. Ocular involvement in the HAART era can be due to the adverse effects of some systemic drugs used in the management of HIV/AIDS. Drug-associated retinal toxicity and other ocular side effects are being increasingly reported. In this review, we discuss the ocular manifestations in HIV patients and its varied presentations following the introduction of HAART, drug-associated lesions, and the current treatment guidelines.

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Purpose: To report the clinical profile of a series of anterior nodular scleritis in Indian population. Methods: We conducted a retrospective review of medical records of 140 eyes of 123 consecutive patients with nodular scleritis who presented to a tertiary eye care institute between 2007 and 2018. Results: The mean age at presentation was 46.8 ± 13.1 years and 70.7% of the patients were female. Bilateral involvement was observed in 14% patients. The most common presenting symptom was redness (92.6%) and ocular pain (69.1%). Twenty-seven patients (22%) had some systemic association and rheumatoid arthritis (5%) was the most common autoimmune disease. Presumed ocular tuberculosis was diagnosed in 13% patients. Methotrexate was the most common immunosuppressive used in these patients and an additional immunosuppressive was required in 6.5% patients. Recurrence of inflammation was observed in 74.8% patients. Deterioration of vision noted in 2.8% eyes. Conclusion: Tuberculosis remains an important cause of nodular scleritis in India. Recurrence of scleritis is common in nodular scleritis and cases with non infectious nodular scleritis often require treatment with immune suppressives.

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Purpose: To describe disease manifestations and outcomes of ocular syphilis in Asian Indian population. Methods: Retrospective analysis of patients diagnosed with ocular syphilis at a tertiary referral center in India. Demographics, history, extraocular and ocular manifestations, ocular and systemic investigations, treatment and visual acuity outcomes were noted. All patients were diagnosed after necessary laboratory investigations including HIV ELISA (Human immunodeficiency virus, enzyme-linked immunosorbent assay), VDRL (venereal disease research laboratory), and TPHA (treponema pallidum hemagglutination). Results: Totally, 20 patients with mean age at presentation 38.25 ± 9.76 were analyzed. 9/20 patients had bilateral involvement. 8/20 had concurrent HIV at presentation with an average CD4 counts of 592.25 ± 411.34 cells/microliter. The mean duration of symptoms at time of presentation was 15.45 ± 35.15 weeks. VDRL test was reactive in 45% (9/20) patients whereas, all patients had a reactive TPHA test. Clinical manifestations included outer retinal placoid chorioretinitis lesions (8/20, 40%), followed by retinitis mimicking acute retinal necrosis as the second most common phenotype (4/20, 20%). Other presenting manifestations noted were panuveitis, miliary retinitis lesions, retinal vasculitis, intermediate uveitis, and anterior uveitis. The clinical phenotypes in immunocompromised included panuveitis, acute retinal necrosis and isolated anterior uveitis. Mean follow up duration was 6.32 ± 6.15 months. An improvement in mean best corrected visual acuity (BCVA) of (0.63 LogMAR, approximately 6 Snellen lines, P < 0.02) was noted at last follow-up. Conclusion: Phenotypic manifestations of ocular syphilis are varied. Non-treponemal tests like VDRL may be unreliable when compared with treponemal tests in diagnosing ocular syphilis. Syphilitic uveitis is considered equivalent to neurosyphilis and is treated similar to neurosyphilis.

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A 19-year-old girl presented with severe pain and redness in her right eye and also with nasal congestion and epistaxis since the past 5 months. The patient was systemically investigated and tested positive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR-3 ANCA) with raised erythrocyte sedimentation rate (ESR). The provisional diagnosis of granulomatosis with polyangiitis (GPA) was made, and she was started on systemic steroids and azathioprine after consultation with the rheumatologist. However on steroid taper, she developed severe reactivation of the scleritis and the corneal involvement was noted in the form of peripheral ulcerative keratitis. Instead of starting another course of high dose oral cortico-steroids or pulse cyclophosphomide, she was started on rituximab infusion (two doses), and oral methotrexate was added, leading to the successful remission of the disease.

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Purpose: To study seasonal variation and systemic associations in HLA-B27 related uveitis (HBU). Methods: This was a retrospective, observational chart review conducted in a single, tertiary eye care hospital, from January 2015 to December 2019. New cases presented from January 2017 to December 2019 were studied for incidence patterns (Group X). Cases with ≥1-year follow-up were studied for systemic association (Group Y) and cases with ≥4 episodes of active uveitis were studied for recurrence pattern (Group Z). A year was divided into 3 segments of the year (SoY): 1. November–February, 2. March–June, 3. July–October. Results: We found 157 cases of HBU from January 2015 to December 2019. The incidence in Group X (n = 105) was found to be least in SoY 2 in all 3 years (2017–2019). In Group Y (n = 85), 39 cases (45.88%) were diagnosed as spondyloarthropathies, among which 20 were of ankylosing spondylitis (23.52%). More than 1/2 the total number of episodes occurring in the same SoY (SoY Max), in Group Z (n = 25), was seen in 14 (56%) cases. SoY Max was the first segment (SoY 1) for most of these cases (n = 7). In Group Z, 8 (32%) patients were on immunomodulatory therapy at presentation. Conclusion: Our study confirms seasonal variation in HBU patients by documenting the least incidence from March to June and identifies 56% of patients that can have a maximum number of recurrences in a specific season of the year. Outcomes of surgical interventions planned accordingly, and responsible environmental factors for HBU should be studied further.

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Purpose: To evaluate the anti-inflammatory effect of cortistatin (CST) in endotoxin-induced uveitis (EIU) model and to compare the results with corticosteroid treatment. Methods: A total of 35 healthy Wistar albino rats were randomly divided into five groups. EIU was induced by a single subcutaneous injection of lipopolysaccharide (LPS). Group I received intraperitoneal (ip) normal saline (NS), Group II received ip 150 μg LPS plus NS, Group III received ip 150 μg LPS plus 250 μg/kg CST, Group IV received ip 150 μg LPS plus 1mg/kg dexamethasone, and Group V received ip 250 μg/kg CST only. The aqueous humor was collected 24 h after injection and the infiltrating cells were determined. Moreover, histopathological and immunohistochemical examinations were also performed. Results: The clinical score and infiltrated cell count were reduced in Groups III and IV compared with Group II (P < 0.001). The pathological findings of Groups III and IV were significantly reduced compared with Group II (P < 0.001). These findings were similar between Groups III and IV (P = 1.000). Tumor necrosis factor-alpha (TNF-α) and interleukin 1 beta (IL-1β) immunoreactivity in the ciliary body of Group III and Group IV were significantly reduced compared with Group II (P < 0.001). TNF-α and IL-1β immunoreactivity in the ciliary body of Group III and Group IV were similar compared with Group I and Group V (range of P values was 0.539–0.958). Conclusion: CST administration as a therapeutic agent might ameliorate the severity of intraocular inflammation in uveitis patients. In conclusion, effect of CST and dexamethasone in EIU model was comparable.

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A 30-year-old female patient presented with bilateral anterior nodular sclerokeratitis and multiple erythematous skin lesions involving the face, trunk, arms, and legs. The patient had a history of temporary relief with steroids, however the lesions recurred. A dermatology consultation was sought and the patient was diagnosed to have syphilis, consequent to which she was started on benzathine penicillin and showed a dramatic improvement in both skin and ocular lesions. A high index of suspicion for syphilis should be kept in mind for patients presenting with nodular scleritis to initiate timely and appropriate management with penicillin.

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Purpose: To evaluate the effectiveness of biologic therapy in a cohort of patients with various types of refractory non-infectious uveitis and scleritis. Methods: A retrospective observational study on patients with non-infectious uveitis and scleritis who were not responding or had a high recurrence rate with the conventional treatment and had received biologic therapy. Results: We studied 18 patients (33 eyes) who received biological therapy between January 2017 and November 2019. The mean age was 30 ± 17 years and mean duration of uveitis was 36.8 months (range 1–120 months). Anterior uveitis (27.7%) was most commonly observed followed by scleritis, panuveitis, posterior, and intermediate uveitis. The most common etiology was Behçet's disease (4 patients, 22.2%) followed by juvenile idiopathic arthritis (3 patients, 16.6%), granulamotosis polyangitis, and idiopathic (2 patients each, 11.1%). Majority had trialled one or more immunosuppressive and were refractory in nature. Maximum patients had received adalimumab (61%) followed by infliximab (22%), rituximab (12%), and golimumab (6%). The median prednisolone dose was reduced from 30 mg (range 7.5–60 mg) to 5 mg (range 0–10 mg) after biological therapy (P = 0.002). Significant visual improvement was observed post biologic therapy (mean log mar VA 0.41 ± 0.62 improved to 0.23 ± 0.48 at the final visit, P = 0.008). Maximum number of patients (16 patients, 89%) responded well with biological therapy. Three patients developed recurrence and systemic complications were observed in two patients. Conclusion: Biologic therapy is effective in non-infectious refractory uveitis who were resistant to conventional therapy and may prolong disease recurrence.

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Sympathetic ophthalmia (SO) is often diagnosed when an inflammatory process appears to be advanced. Herein, the authors present the prospective optical coherence tomography (OCT) study of the onset of SO in the sympathizing eye. Prior to any signs of uveitis, we noted the mild disintegration of the retinal pigment epithelium (RPE) layer, the interdigitation zone (IZ), and the ellipsoid zone (EZ). The complete disruption of IZ and EZ was seen 12 weeks later. After 14 weeks, the uveal inflammation was present, and OCT imaging disclosed the formation of nodule-like lesions between the Bruch's membrane and the RPE layer. The histopathological evaluation of the enucleated exciting eye confirmed the diagnosis of SO.

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Purpose: To analyze the outcome of cataract surgery in patients with ocular tuberculosis (OTB). Methods: Medical records of patients with OTB who underwent cataract surgery between 2005 and 2018 were retrospectively reviewed. The primary outcome measure was corrected distant visual acuity (CDVA), and a good outcome was defined as CDVA 20/40 or better. Results: There were 35 patients (41 eyes), of whom 13 (37.1%) were males and 22 (62.9%) were females. The mean age was 41.6 ± 13.8 (range 18–65) years. Anterior uveitis was present in 24 (58.5%) eyes, intermediate uveitis in 5 (12.2%) eyes, posterior uveitis in 6 (14.6%) eyes, and panuveitis in 6 (14.6%) eyes. Posterior sub-capsular cataract (51.2%) was the most common type of cataract. Total cataract was present in 9 (22%) eyes. The two most common ocular comorbidities were small pupil (85.4%) and glaucoma (17.1%). Phacoemulsification was performed in 36 (87.8%) eyes and ECCE was performed in 5 (12.2%) eyes. Intraoperatively the pupil was mechanically dilated in 36 (87.8%) eyes. Overall 26 (63.4%) and 23 (56.1%) eyes achieved CDVA 20/40 or better at 1- and 6-month follow-up visit, respectively. The mean preoperative LogMAR CDVA significantly improved from 1.28 ± 0.57 to 0.38 ± 0.36 at 1-month (P < 0.0001). One eye had fibrinous anterior chamber reaction and 24 (58.5%) eyes had 1 or more episodes of reactivation of uveitis in the follow-up period. The three commonest complications were glaucoma (26.8%), posterior capsule opacification (19.5%), and epiretinal membrane (17.1%). Postoperative posterior segment complications like epiretinal membrane formation, vitreous inflammation and cystoid macular edema affected final visual acuity (P < 0.002). Conclusion: The visual outcome after cataract surgery in OTB was satisfactory as significant number of patients achieved a good final visual acuity.

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Leptospirosis is a bacterial cause for nongranulomatous uveitis in tropical countries. It is known to cause rapid maturation of cataract in young adults. Here we present six patients who had presenting sign as fully matured pearly white unilateral cataract without history of any trauma in their first visit. All except one patient were positive for leptospirosis by ELISA testing. Histopathological analyses of those lenses showed globular degeneration.

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Purpose: Retinal detachments (RD) secondary to retinal vasculitis are highly complex. We report the clinical profile and outcome of vitrectomy in RDs secondary to retinal vasculitis in terms of intraoperative findings, final anatomical, and functional outcome. Methods: In a retrospective review of 68 patients (6 with bilateral RD; 74 eyes) undergoing pars plana vitrectomy (PPV) between 2000 and 2015 for vasculitic RD, tractional RD was present in 50 (67.57%) eyes and combined RD in 24 (32.43%) eyes. Results: The mean age was 31.54 ± 9.95 years (62 males, 6 females). Fibrovascular proliferations (FVPs) involved major vascular arcades (22.98%), optic disc (10.81%), both arcades and disc (20.27%), peripheral retina (32.43%), and arcades with peripheral retina (13.51%). A total of 14 (18.92%) eyes had retinal folds, of which 9 had macular drag. Of 24 eyes with combined RD, 3 (12.5%) eyes had macular hole, 15 (62.5%) eyes had the primary retinal break anterior to equator, and 6 (25%) eyes had the primary break posterior to equator. Twenty-one (28.38%) eyes had iatrogenic retinal breaks. Thirty-eight (51.35%) eyes required an internal tamponade [gas in 31 (81.57%) eyes and silicon oil in seven (18.42%) eyes]. A scleral buckle was additionally required in 26 (35.14%) eyes. Postoperative complications included vitreous hemorrhage (27.03%), re-RD (12.16%), and iris neovascularization (9.46%). The median follow-up was 18 months (range 6-122 months). Sixty-eight (91.9%) eyes achieved final anatomical success. Fifty-two (70.27%) eyes had ≥2 lines visual improvement. Conclusion: Vasculitic RDs are complicated by tractional/combined RDs, peripherally located FVPs, retinal folds and iatrogenic retinal breaks, and carry a moderate prognosis.

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A 16-year-old girl was referred as retinitis and vasculitis post-typhoid fever. Fundus examination revealed bilateral pseudo-sheathing, scattered hemorrhages with retinal infiltrates. Wide-field fundus fluorescein angiography (FFA) showed peripheral vascular hyperfluorescence; 55° FFA images showed a “firecracker-like” peripheral vasculature, but a closer look revealed miliary microaneurysms (MAs). Peripheral smear examination clinched the diagnosis of chronic myeloid leukemia. MAs on FFA in leukemic retinopathy have been frequently described in the literature. Our report emphasizes its presence, miliary nature, and need for closer inspection of FFA images. We believe that this angiographic sign has potential to become one of the imaging biomarkers of leukemic retinopathy.

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Purpose: To compare the long-term results of the patients with branch retinal vein occlusion (BRVO) secondary to Behçet's syndrome (BS) with the patients with unknown etiology. Methods: Medical records and optical coherence tomography (OCT) imaging results of the patients with BRVO secondary to BS and with unknown etiology were reviewed retrospectively between 2016 and 2018 at a single center. The anatomical location of the BRVO, involvement of the macula, application of laser photocoagulation, and intravitreal injection were evaluated. Results: Twenty-eight eyes of 23 patients with BRVO secondary to BS as the study group and 22 eyes of 19 idiopathic BRVO patients as the control group were included in the study. The mean duration of follow-up after the development of BRVO was 74.6 ± 57.4 months in the study group and 63.6 ± 59 months in the control group. The rate of bilaterality, macular involvement, and application of laser photocoagulation was not statistically significantly different between the groups. However, the frequency of injection requirement was significantly lower in the patients with BRVO secondary to BS in comparison to the control group (P= 0.009). Conclusion: Although the treatment of BRVO is laser photocoagulation and intravitreal injection of anti-VEGF agents or dexamethasone implant, the patients with BS might respond very well to systemic immunomodulatory agents in case of BRVO. Thus, rearrangement of the immunomodulatory treatment before starting intravitreal injections should be considered in the patients with BRVO secondary to BS.

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Purpose: Re-emergent ocular syphilis in patients with Human immunodeficiency virus (HIV) co-infection has both diagnostic and management difficulties because of the overlapping risk factors. The clinical manifestations described in non-HIV may not be the same in patients with HIV coinfection. Immune recovery uveitis (IRU) may also alter the course of the disease causing recurrences. We studied the clinical features in correlation with CD4 counts, systemic immune status, sexual preferences and management outcomes in HIV/AIDS patients with ocular syphilis in the highly active antiretroviral treatment (HAART) era from a high endemic HIV population like India. Methods: Retrospective analysis of all patients with ocular syphilis and HIV/AIDS seen between 2016 and 2019 was done. Results: A total of 33 patients (56 eyes) with a CD4 count range of 42–612 cells/cu.mm were included. Ocular syphilis was found to be higher in individuals with high risk behavior such as men who have sex with men (MSMs) (45%). Panuveitis was the commonest manifestation (53.57%) and was even the presenting feature of HIV and syphilis in many patients. Significant vitritis, usually uncommon in HIV/AIDS immunocompromised patients was noted even with low CD4 counts in patients with ocular syphilis. Significant correlation was noted between ocular presentation and CD4 counts (P < 0.05). Conclusion: Ocular syphilis presents differently in patients with HIV/AIDS. Diffuse retinitis is seen commonly in low counts (<100 cells/cu.mm). Classical placoid chorioretinitis lesions usually described in non-HIV individuals is uncommon in HIV patients and is seen in higher CD4 counts ( >400 cells/cu.mm). Ocular manifestations can be an indicator of the immune status of the patient. Not all patients with ocular manifestations have associated features of systemic syphilis. Ocular manifestations can be the first presentation of HIV/AIDS. Although, there is good response to systemic penicillin and HAART, recurrences and immune recovery uveitis (IRU) can also occur.

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An intraocular biopsy is performed for diagnostic, prognostic and investigational purposes. Biopsies help to confirm or exclude malignancies and differentiate inflammatory from infectious processes. Histopathological analysis is the final verdict in unresponsive uveitis, atypical inflammation, metastases and masquerade syndromes. Advances and refinement of techniques in cytopathology, immunohistochemistry, microbiological and molecular biologic study offer much more than just diagnosis. They provide prognosis based on cell characteristics and are helpful in planning treatment and intervention. Many biopsy procedures have evolved to provide more safety and minimise complications thus improving the quality of specimens or samples available for analysis. The type of biopsy and technique adopted varies based on the clinical suspicion, size and location of lesions. In uveitis, a working diagnosis of intraocular inflammation is made on clinical examination and laboratory investigations and ancillary tests. Malignancy and uveitis is interlinked and masquerade syndromes are among the commonest indications for biopsy and analysis of specimen. The various types of intraocular biopsies include aqueous tap, fine needle aspiration biopsy, vitreous biopsy, iris and ciliary body, and retinochoroidal biopsy. They will be reviewed in this article with respect to current perspective

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Zoledronic acid is recommended for patients with osteoporosis. To report a case of unilateral anterior uveitis after zoledronate infusion. An osteoporotic patient presented with pain, visual loss, hyperemia, photophobia, and watering from the left eye after zoledronate infusion. Circumcorneal injection, keratic precipitates, cells, and flare suggested anterior uveitis. Her symptoms resolved completely after 20 days of prednisolone acetate with atropine eye drops. Uveitis is a rare complication of zoledronic acid with an unclear mechanism. Proinflammatory cytokines may play a role in pathogenesis. Zoledronic acid may be associated with rare but serious inflammatory ocular adverse drug reactions.

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Pars plana vitrectomy (PPV) in uveitis is indicated for various diagnostic and therapeutic indications. With the advent of microincision vitreous surgery (MIVS), the use of PPV in uveitis has increased with a wider spectrum of indications due to shorter surgical time, less patient discomfort, less conjunctival scarring, and a decreased rate of complications as compared to standard 20G vitrectomy. Because of faster post-operative recovery in terms of visual improvement and reduction of inflammation, and reduced duration of systemic corticosteroids, MIVS has gained popularity in uveitis as an adjunctive therapy to the standard of care medical therapy. The safety and efficacy of MIVS is related to the emerging vitrectomy techniques with better and newer cutters, illuminating probes, and accessory instruments. Because of the instrumentation and fluidics of MIVS, PPV is emerging as a safe and useful alternative for diagnostic challenges in uveitis, aiding in earlier diagnosis and better outcome of inflammatory disease, even in the presence of severe and active inflammation, which was once considered a relative contraindication for performing vitreous surgery. However, for surgical interventions for therapeutic indications and complications of uveitis, it is advisable to achieve an optimum control of inflammation for best results. The increasing reports of the use of MIVS in uveitis have led to its wider acceptance among clinicians practicing uveitis.

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Purpose: The objective of this study was to correlate seasonal variation of epidemic retinitis (ER) with concurrent community outbreaks. Methods: This is a retrospective, observational, comparative study conducted in a tertiary care eye hospital in south India. Monthly variation in number of ER cases in comparison with reported community outbreaks by Integrated Disease Surveillance Program (IDSP) from 2009 to 2020 in the same region were studied. Month-wise graphs against number of patients were plotted for ER and for each community outbreak. Results: ER was diagnosed in 163 patients. Diagnosis of presumed rickettsial ER was made in 48 cases (29.44%), chikungunya in 5, dengue in 3 and typhoid in 6 cases, while in other cases the etiological diagnosis remained uncertain (n = 101). Multiple positive serological tests were seen in 6 patients (Weil Felix Test (WFT) with WIDAL in 4 and chikungunya IgM with WFT in 2 patients). Relevant reported outbreaks by IDSP were: Pyrexia of unknown origin (PUO) (n = 5148), Chikungunya (n = 6577), Dengue (n = 7350), Measles (n = 1422), Mumps (n = 881), Rubella (n = 288), Malaria (n = 2262), Chicken Pox (n = 2385), Typhoid (n = 597), Kyasanur Forest Disease (n = 381), Scrub Typhus (n = 13), Typhus fever (n = 4), Japanese Encephalitis (n = 15). None of the outbreak graphs pattern was identical or similar to the graph of ER. Inverse relation of the graph of dengue outbreak with ER was observed. Conclusion: Inverse correlation between dengue and ER should be further studied for causation, which we believe may prove dengue as least common cause. Reporting of rickettsial outbreaks should be enhanced by undertaking statewide awareness and procurement of gold standard tests.

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Leprosy is a chronic multisystem disease caused by Mycobacterium leprae with frequent ocular manifestations such as madarosis, lagophthalmos, lid abnormalities, impaired corneal sensations, chronic uveitis, and cataract. Granulomas are characteristic lesion of lepromatous leprosy commonly seen as conjunctival and iris granulomas. We report a case of leprosy with a rare ocular manifestation in the form of angle mass lesion or suspected granuloma which has not been reported before and its successful management following treatment with favorable outcome. The index case also highlights the anterior segment fluorescein angiography features of the lesion and study of its vascular pattern.

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Ocular manifestations of Sweet syndrome, or acute febrile neutrophilic dermatosis, are usually limited to the anterior segment. We report the case of a patient with bilateral panuveitis and retinal vasculitis associated with Sweet syndrome. A 45-year-old Asian female with an undiagnosed febrile illness with rash presented with bilateral panuveitis with haemorrhagic occlusive retinal vasculitis. Skin biopsy confirmed Sweet Syndrome. Intraocular inflammation resolved with a combination of topical and systemic corticosteroids as well as intravenous cyclophosphamide, with resulting permanent severe right visual impairment. Although an uncommon condition, Sweet syndrome should be considered in any febrile patient with skin lesions and uveitis.

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We report a case of exacerbation of posterior scleritis and orbital pseudotumor in a patient with granulomatosis polyangiitis, with initial rituximab infusion. Modifications in rituximab protocols including reduction in maintenance dose for subsequent infusions with increase in premedication dose of intravenous methyl prednisolone may be useful to prevent periinfusional flares. In this case report, we highlight a rare occurrence and modification in rituximab protocol to prevent post-infusional flare of inflammation.

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Purpose: To study efficacy of anti-vascular endothelial growth factor (anti-VEGF) in resolution of macular edema in epidemic retinitis (ER). Methods: In this retrospective, comparative study, patients diagnosed as ER with central macular thickness (CMT) ≥ 600 μm on SD-OCT at presentation were studied. Eyes which did not receive intravitreal anti-VEGF formed group A and eyes receiving additional anti-VEGF formed group B. Eyes receiving anti-VEGF monotherapy were studied separately. Cases with subsequent OCT scans with interval of more than 20 days and cases without OCT scan at the resolution were excluded. Treatment details, visual outcome, and days to resolution of macular edema were studied. Results: Mean CMT in group A (n = 8) was 820.1 μm (range 607-1004 μm) and in Group B (n = 4) was 756.0 μm (range 603-1000 μm). Macular edema resolved in 34.8 days (range: 16-65) and 39.0 days (range: 21–45) in group A and B, respectively. Two eyes with anti-VEGF monotherapy recovered in 45 and 18 days, respectively. Mean corrected distance visual acuity (CDVA) at presentation in group A was 19.1 (range: 0–61) ETDRS letters and in group B was 14.3 (range: 0–35) ETDRS letters. Mean CDVA improved to 65.7 (range: 0–85) and 50.8 (range: 20–76) ETDRS letters in group A and B, respectively. Anti-VEGF monotherapy eyes improved from 35 and 46 ETDRS letters to 70 and 85 ETDRS letters, respectively. Conclusion: Additional anti-VEGF therapy has no added advantage in speed of resolution of macular edema due to ER. A randomized controlled trial with steroids sparing “anti-VEGF monotherapy” may verify our observations.

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In this report we describe nongranulomatous uveitis followed by bilateral retinal vasculitis and much later by the loss of accommodation as initial presentations of demyelinating disease in a 42-year-old female with no other neurologic manifestations. The absence of demyelinating plaques in the initial magneric resonance imaging (MRI) (orbit and cranium) and its occurrence 2 years later, have been described as “lesions appearing with time”. Extensive laboratory investigations ruled out infections, systemic vasculitis, and connective tissue disorders. Due to the presence of oligoclonal bands in both cerebrospinal fluid (CSF) and serum, absence of antiaquaporin-4, antimyelin-oligodendrocyte glycoprotein immunoglobulin G (IgG) antibodies, and negative vasculitis profile, the exact cause of demyelination (multiple sclerosis/vasculitis related) could not be ascertained. She has currently received 2 cycles of rituximab and at the last follow-up did not show any recurrences.

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Mass lesions arising from the anterior segment in children involving the iris and ciliary body can be of myogenic, neurogenic, or hematogenic/vascular origin. These include nevi, melanomas, adenoma, adenocarcinoma, cysts, metastatic tumours among others. Multiple iris mass lesions due to tuberculosis in children are rare. We present an uncommon atypical presentation of multiple anterior segment mass lesions referred to us as neoplasia. Although excision biopsy can be diagnostic, it was deferred and anterior chamber tap was done. Aqueous cytology was suspicious of juvenile xanthogranuloma (JXG) but polymerase chain reaction (PCR) confirmed tuberculous etiology. Treatment with antituberculous therapy (ATT) and steroids lead to complete resolution of the lesions.

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A 65-year-old male presented with redness and pain associated with active necrotizing scleritis in the left eye. He was started on mycophenolate mofetil and oral corticosteroids, to which there was no response detected after 4 weeks. A rheumatology opinion was sought and he was started on tofacitinib, after which there was dramatic clinical improvement. Patients refractory to conventional immunosuppressive therapy can benefit from the new class of immunosuppressive agents, JAK/STAT kinase inhibitors.

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Post fever retinitis (PFR), characterized by multiple cotton wool spot like lesions in the posterior pole, is commonly reported following viral and bacterial infections. Retinal perfusion defects have been documented with the help of optical coherence tomography angiography (OCTA) in cases of PFR. But long term changes in such cases have not been reported earlier. In the following report, we have described the swept-source OCTA findings of two PFR patients at initial presentation and three years follow-up. Initial OCTA scans may not provide accurate information regarding the perfusion status due to associated retinal edema and inflammation. However, persisting perfusion defects at the superficial and deep retinal capillary plexus were noted on long term follow-up in both the cases.

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Behcet's disease (BD) is a multisystem disorder with a classical triad of recurrent oral ulcers, genital ulcers, and uveitis. It involves both arterial and venous system which can lead to life-threatening complications. Both superficial and deep venous systems can be involved leading to peripheral skin lesions and devastating complications like cerebral venous thrombosis and Budd–Chiari syndrome (BCS). This report describes a case of an HLA B-52 positive BD in a 22-year-old woman who presented with retinal vasculitis and venous ulcer on the foot and later on developed ascites due to obstruction of supra-hepatic inferior vena cava (BCS). This report highlights the fact that BCS can develop in patients of BD and every ophthalmologist should be aware of this life-threatening complication while they are treating these patients, as timely diagnosis and intervention can prevent mortality.

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Tubercular granulomas are a common manifestation of intraocular tuberculosis. These are said to be hypoxic granulomas with increased expression of vascular endothelial growth factor (VEGF). Management of these granulomas includes a combination of antitubercular therapy (ATT) and oral corticosteroids. We report a case of tubercular granuloma with exudative retinal detachment which was treated with weekly intravitreal anti-VEGF and antibiotic injections along with ATT and corticosteroids. The VEGF levels measured paralleled with the clinical regression of the granuloma.

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We report a case of a young male from Udupi, Karnataka, presenting with features of bilateral non-granulomatous anterior uveitis with mild vitritis. There was a history of fever and myalgia a month before ocular features, which was partially treated. He also gave a history of being actively involved in the flood relief work in the district, before the onset of fever. This prompted an evaluation of leptospirosis, which came positive. The possibility of leptospira uveitis needs to be considered, especially in cases of non-granulomatous uveitis after floods in areas endemic for leptospirosis, which may otherwise be wrongly labeled idiopathic.

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A 31-year-old male patient presented with sudden onset loss of vision in the left eye. Ocular examination revealed significant vitritis with chorioretinitis lesion in the posterior pole. Subsequent investigations revealed positive human immunodeficiency virus (HIV) and syphilis serology; chest imaging revealed active pulmonary tuberculosis. Polymerase chain reaction from aqueous aspirate was positive for Mycobacterium tuberculosis. There was complete resolution of the lesions following antisyphilitic medications, antitubercular therapy along with highly active antiretroviral therapy. Syphilis and tuberculosis coinfection in a previously unknown HIV patient is rare but can occur. It is worthwhile to look for multiple coinfections in HIV patients.

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Purpose: The aim of our study was to investigate the change in the subfoveal choroidal thickness in unilateral acute anterior uveitis with treatment. Choroidal inflammation is uncommon but can occur in the acute stage of anterior uveitis. Methods: All diagnosed patients with the first episode of unilateral acute anterior uveitis were included in the study conducted for a period of July 2017–July 2018. Choroidal thickness at subfoveal region was measured with spectralis spectral domain optical coherence tomography using enhanced depth-imaging scans. Standardized, masked manual measurement of the choroidal thickness was performed in the center of the ETDRS fields. The unaffected fellow eye of the same patient was taken as a control group and their subfoveal choroidal thickness was measured. Follow up of the patients was done at 2 weeks posttreatment to reevaluate the choroidal thickness. Results: A total of 61 eyes of 61 patients with unilateral acute anterior uveitis were included in the study. The mean central subfoveal choroidal thickness was significantly higher (304.82 ± 73.17 μm) as compared to that of controls (251.28 ± 66.38 μm) at presentation (P < 0.001). Following treatment and at two weeks follow up, the subfoveal choroidal thickness significantly reduced from 304.82 ± 73.17 to 274.46 ± 63.82 μm (P < 0.001). Also the subfoveal choroidal thickness was positively correlated with visual acuity (r = 0.22, P < 0.251) before treatment. Conclusion: Acute anterior uveitis is associated with an increase in the subfoveal choroidal thickness followed by the significant decrease in choroidal thickness with treatment, suggesting that choroid is also inflamed along with anterior segment inflammation.

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Ocular neoplasia can masquerade as an inflammatory condition and vice-versa, which if untreated, can lead to loss of vision or even life. We present a young immunocompetent adult male referred to us as choroidal melanoma. He had a large atypical choroidal mass and exudative retinal detachment. He had no systemic complaints and ocular lesions were the first manifestation of disseminated disease. Aqueous polymerase chain reaction for Mycobacterium tuberculosis (MTB) was negative, but Mantoux, chest radiology, acid-fast bacilli, and caseation necrosis on pleural nodule histopathology confirmed tuberculosis (TB). While on antitubercular therapy, a rare “Coats'-like response” was noted which eventually resolved completely with treatment.

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Episcleritis, scleritis, and anterior uveitis are common clinical manifestations of ocular leprosy. Erythema nodosum leprosum (ENL) is an acute, exaggerated systemic immunological reaction that complicates the course of this chronic indolent disease. We present an ocular immunohistochemical study of severe form of ENL involving even the ciliary body and choroid resulting in the perforation of the globe on the initiation of anti leprosy therapy. We used CD-3, CD-68, S-100, and CD-20 for immunohistochemistry. It revealed plenty of CD-3-positive T-cells and CD-68-positive macrophages and a few S-100 and CD-20-positive cells. The inflammatory exudates stained positive for IgG and IgM. The diagnosis was ocular ENL.

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Pediatric intermediate uveitis (IU), usually idiopathic, can also be associated with tuberculosis (TB) and sarcoidosis. A 14-year-old girl was diagnosed with idiopathic IU after ruling out TB and sarcoid. She was treated with oral steroids and methotrexate (MTX) with good inflammation control. One year later, she presented with subretinal (SR) abscess. Lab tests were still negative but aqueous polymerase chain reaction confirmed TB. With antituberculosis treatment, complete resolution of the lesion was noted. The likelihood of a change in phenotype of ocular TB, from an IU to TB SR abscess or a possible reactivation of latent TB due to MTX are discussed.

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A 33-year-old patient was diagnosed with acute Vogt-Koyanagi-Harada (VKH) disease and was prescribed prednisolone (1 mg/kg/day) and azathioprine (2.5 mg/kg/day). She mistakenly took an excessively high dose (4 mg/kg/day) of prednisolone for 14 days. The erroneous dose of corticosteroids was progressively corrected. Several weeks after initial presentation, the patient developed a polymerase chain reaction-proven bilateral cytomegalovirus retinitis, with extensive occlusive arteritis in the right eye. Systemic immunosuppressive therapy was temporarily discontinued and viral retinitis was successfully managed with systemic and intravitreal ganciclovir. Corticosteroids were reintroduced to control recurrent VKH disease. Final visual acuity was 20/1000 in the right eye and 20/50 in the left eye.

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A 16-year-old boy presented with uveitis in both eyes with recurrent febrile illness and renal ailments. The patient was referred to a nephrologist. Subsequent investigations revealed acute tubulointerstitial nephritis in a renal biopsy and raised urinary beta-2 microglobulin (B2M). Based on his clinical findings and laboratory investigations, a diagnosis of tubulointerstitial nephritis and uveitis (TINU) syndrome was made. Since, the literature on TINU through India is sparse, the two available case reports were published through nephrology setup. Our case illustrates how ophthalmologist can aid in the diagnosis of such a rare clinical entity using interdisciplinary approach.

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An immunocompetent, 25-year-old gentleman with bilateral chronic uveitis presented to various uveitis clinics at different points of time with documented typical clinical features of Toxoplasma Chorioretinitis (Headlight in fog appearance), Behcet's Disease (Hypopyon with peripheral retinal vasculitis), and Presumed Ocular Tuberculosis (Granulomatous Intermediate Uveitis with positive Interferon-gamma release assay) and had been treated with anti-toxoplasma drugs, oral prednisolone, and immunomodulation with oral Mycophenolate/oral Azathioprine to no avail. After cytological examination of vitreous aspirate, he was found to have non-Langerhans cell Histiocytosis which responded to chemotherapy with Vinblastine and Cyclophosphamide.

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Ocular tuberculosis (TB) is a form of extra-pulmonary TB, which can involve almost any intraocular structure or ocular adnexa. Posterior uveitis, the commonest form of intraocular TB manifests as choroidal tubercles, choroidal tuberculoma, subretinal abscess, neuroretinitis, or serpiginous-like choroiditis. These forms of posterior tubercular lesions can be broadly classified into two groups based on their pathophysiology and morphology. One group of lesions is related to the direct invasion and reactivation of the bacilli in the choroidal tissue, whereas the other is a result of hypersensitivity reaction to the bacilli. Simultaneous bilateral active posterior uveitis with such varying morphology and pathophysiology in either eye of the same patient is an extremely rare presentation. We report a case with pulmonary TB on Anti-tubercular therapy (ATT), who presented to us with two mutually exclusive and distinctly different forms of tubercular posterior uveitis in either eye simultaneously. Both lesions were active at the time of presentation.

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We report a case of a patient who presented with symptoms of acute onset scleritis in the left eye but was found to have an underlying necrotic choroidal melanoma. Asymptomatic choroidal melanoma can undergo spontaneous necrosis and manifest as severe scleritis and should be considered as an important differential.

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Purpose: Serpiginous choroiditis (SC) is primarily an inflammation of choriocapillaris leading to nonperfusion. A quantitative assessment of choriocapillaris perfusion can be done by measuring the flow-density by OCT-Angiography (OCTA). This study measures a change in the flow-density of choriocapillaris with the resolution of inflammation. Methods: The OCTA images of a choriocapillaris slab of 30 eyes with active SC were subjected to binarization and vessel density was measured at baseline and final visits and compared. Results: Upon comparing the vessel density of the affected areas by OCTA of choriocapillaris-slab at baseline and final visits, there was statistically significant (P < 0.0001) improvement after the resolution of inflammation. The vessel density of a demarcated normal area was significantly higher when compared to that of lesions at baseline (P < 0.0001) and final visit (P < 0.0001). Conclusion: OCTA is a useful tool to assess reduction in the inflammatory activity on treatment in SC. This study shows that with treatment the perfusion of choriocapillaris improves; however, it remains lower than normal.

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A premature newborn with systemic sepsis due to Candida albicans and parapsilosis developed skin, eye, and mouth herpetic infection. Ocular disease presented atypically with vitritis and pre and subretinal hemorrhage due to herpes simplex virus-1 confirmed fulminant bilateral acute retinal necrosis. Pars plana vitrectomy revealed necrotizing retinitis with poor visual prognosis. The baby has survived suffering from multiple morbidities which include post-hemorrhagic hydrocephalus, chronic lung disease, patent ductus arteriosus, and developmental delay.

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Ocular tuberculosis (OTB) in patients with human immunodeficiency virus (HIV) commonly presents as choroidal tubercles or granuloma. We report a rare presentation of OTB with hypopyon granulomatous uveitis in a patient with HIV on highly active antiretroviral therapy (HAART). Aqueous humor polymerase chain reaction (PCR) was positive for Mycobacterium tuberculosis (MTB). Antitubercular therapy (ATT) was initiated despite which it progressed to scleral abscess and panophthalmitis. Enucleation with ball implantation was done. Histopathology revealed caseating granuloma with numerous acid-fast bacilli (AFB) and real-time PCR showed increased copies of MTB genome. After a full course of ATT, he had stable ocular and systemic condition at final follow-up.

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Ocular manifestations due to cysticercosis may involve the vitreous cavity or subretinal space in the posterior segment of the eye. Management of subretinal cysticercosis is challenging, especially if it involves the submacular region. Removal of submacular cysticercosis (SMC) requires utmost care and competent expertise. In recent times, modern vitreo-retinal equipment has improved the outcome of SMC. On the contrary, untreated SMC run a high risk of spontaneous rupture, liberation of toxin from dying worm, and intense intraocular inflammation threatening vision eventually. In the present report, two cases of SMC were discussed who presented to us within a span of 1 week. We described the course of disease and final outcome in these two patients (one underwent surgical removal while other denied surgery) highlighting the urgent need of cyst removal in such scenarios.

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Rosai- Dorfman disease (RDD) is a rare systemic pseudo-lymphomatous disorder with unknown etiology. No guidelines exist regarding its management and treatment when the disease is progressing. Choroidal involvement in RDD has rarely been reported and has often been misdiagnosed. We describe a case of a 64-year-old male diagnosed with RDD by means of choroidal biopsy, successfully treated with a MEK inhibitor, namely Cobimetinib, and its follow-up over 5 years, with good final anatomical and functional results. This is the first reported case of RDD diagnosed with an intraocular biopsy performed on a non-enucleated globe, thus preserving the integrity and function of the eye. This case emphasizes the need for a choroidal biopsy when the diagnosis is not straightforward and the starting of targeted therapy to retain a good visual function.

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