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Intermediate uveitis (IU) in an intraocular inflammation that predominantly affects the vitreous. It typically manifests as chronic low-grade inflammation, mainly affecting children and young adults. Uncontrolled chronic intraocular inflammation carries a high risk of visual loss. IU, despite being a low-grade inflammation, is among the sight-threatening conditions owing to its intrinsically-associated vision-robbing ocular complications. It can cause early and late-onset ocular complications affecting the anterior and posterior segments of the eye. 40–60% of the patients who suffer from IU will develop at least one ocular complication during the disease course. Prompt management and long-term monitoring are vital for the early detection of ocular complications and timely intervention. Proactive screening programs are essential in children because of the occult nature of IU in the pediatric age group and the high prevalence of complications at the initial visit. This review provides a comprehensive panorama of anterior segment complications, namely, band keratopathy, corneal endotheliopathy, posterior synechiae, and cataract as well as of posterior segment complications including macular edema, retinoschisis, retinal detachment, vitreous hemorrhage, vasoproliferative tumor, optic neuritis, and papillitis, in addition to glaucoma and hypotony. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionPapillophlebitis is a rare condition, manifesting as CRVO in the young adults. We aim to present our experience in managing patients with papillophlebitis.Retrospective review of patients’ medical files.Included were seven patients with a mean presenting age of 24.86 ± 4.4 years and mean follow-up of 40.4 ± 50.5 months. No pre-existing systemic illness was reported by any patient. One patient was subsequently diagnosed to have Behçet disease and another patient was diagnosed with homozygous mutation to MTHFR C6771. On presentation, fluorescein angiograms showed diffuse vascular and optic disc leakage. Four patients presented with papillophlebitis-associated CME, for which they were treated with systemic steroids and intravitreal anti-VEGF injections. One patient showed full recovery. In 3 patients, due to the protracted course of papillophlebitis and refractory CME, adalimumab was added. All 3 patients eventually showed complete resolution of CME. Two of them eventually developed extensive peripheral capillary non-perfusion that was treated with panretinal photocoagulation. Three patients did not develop CME: In two patients, papillophlebitis resolved after a short course of prednisone while in the third patient, papillophlebitis resolved spontaneously. Mean ± SD presenting log MAR VA was 0.2 ± 0.32 and it was 0.057 ± 0.11 at the last follow-up.To the best of our knowledge, this is the first description that suggests a role for TNF-ɑ blockers in the management of patients with recalcitrant papillophlebitis and non-responsive CME. Further studies are needed in order to thoroughly investigate the molecular background of papillophlebitis and clinical outcomes associated with this class of medications. [ABSTRACT FROM AUTHOR]

To report two cases masquerading as TORCH but eventually diagnosed with Enhanced S-cone Syndrome (ESCS). Descriptive case report Case 1: A ten-month-old boy presented with high hypermetropia, strabismus and bilateral chorioretinal pigmented scars with a history of cat scratch of his mother during pregnancy. He was treated for suspected toxoplasma retinitis. Choroidal neovascular membranes (CNV) were diagnosed bilaterally and treated with intravitreal bevacizumab. Genetic testing showed homozygote mutation in NR2E3 gene. Case 2: A two-year old girl presented with bilateral high hypermetropia and strabismus. Funduscopy revealed extrafoveal chorioretinal lesions and surrounding subretinal fibrosis. An elevated titer of anti-toxocara IgG antibodies was detected and managed accordingly. LE CNV was diagnosed and treated with intravitreal bevacizumab. Genetic testing disclosed homozygote mutation in NR2E3. Ocular manifestations in ESCS can be reminiscent to TORCH. CNV may develop with an incidence of 15%. We report the youngest patient with ESCS-associated CNV. [ABSTRACT FROM AUTHOR]

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

To report on a patient with probable catastrophicantiphospholipid syndrome (CAPS) with eye, brain and skin involvement. Descriptive presentation of the case and of the relevant clinical photographs. A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, lower limb skin ulcers and livedo reticularis. Workup showed anemia, elevated ESR, CRP, and positive anti-phospholipid antibodies. Right eye funduscopy showed cotton-wool spots along the inferotemporal arcade with arteriolar occlusion, perivascular retinal hemorrhages, and diffuse retinal ischemia in the temporal peripheral retina. Fluorescein angiogram confirmed the widespread retinal ischemia. Brain MRI revealed several white matter lacunar infarcts, minute cortical/subcortical hemorrhages and subarachnoidal insular hemorrhage. The patient was treated with immunomodulatory therapy, Enoxaparin and retinal laser photocoagulation. Within 2 months of treatment, the facial livedo reticularis resolved and the leg ulcers markedly improved. CAPS causes multiple organ thrombosis and is associated with high rate of mortality. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo provide a comprehensive overview of the diagnostic and therapeutic journey of a pediatric patient with persistent sarcoid-associated panuveitis over a 10-year period, who ultimately developed bilateral macular subretinal fibrosis and visual loss.Retrospective case reportThe patient was diagnosed with sarcoidosis after undergoing a transbronchial biopsy. She was followed up because of granulomatous panuveitis, multifocal choroiditis, and papillitis bilaterally. She maintained a stable condition, and visual acuity was 0.3 RE and 0.5 LE. Immunomodulatory therapy included prednisone, methotrexate, and adalimumab. The patient was lost to follow-up for 20 months because of the COVID-19 pandemic. She was represented with active uveitis and was not responding to TNF-ɑ inhibitors (adalimumab and infliximab). Ultimately, the patient's intraocular inflammation was successfully controlled by using intravitreal steroids (Triamcinolone and Fluocinolone acetonide implant). However, the visual outcome was guarded because of bilateral subretinal fibrosis.10% of patients with sarcoidosis-associated uveitis risk blindness in one eye. The index case progressed to sight-robbing bilateral subretinal fibrosis, a rare complication of ocular sarcoidosis despite a combination of conventional and biologic anti-inflammatory therapies. There is a pressing need to develop new treatment agents for refractory non-infectious uveitis. [ABSTRACT FROM AUTHOR]

Purpose: To report the epidemiology, etiology, ocular characteristics, treatment and visual outcome of pediatric uveitis in Israel.Methods: Retrospective study from two tertiary uveitis centers.Results: Included were 107 patients (182 eyes), 55% females. Mean age at diagnosis 8.8 years. Uveitis was predominantly anterior, idiopathic, bilateral, and chronic. Systemic associations were seen in 36% of patients of which the most common disease was juvenile idiopathic arthritis. Infectious uveitis accounted for 37% of posterior uveitis cases of which toxoplasmosis was the most common cause. Anterior segment complications were commonly observed at presentation (41%); the most predominant were posterior synechiae, cataract, and band keratopathy. The most common posterior segment complications were papillitis, epiretinal membrane, and macular atrophy/scar. Ninety-three percent of eyes had visual acuity >20/40 at last follow-up.Conclusion: The pattern of pediatric uveitis in Israel is similar to that in the western world. Visual outcome was good in most eyes. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionSyphilis is a “Great Masquerader” because of its versatile clinical manifestations. We aim to report a patient whose first presentation was with presumed autoimmune hepatitis. Fulminant retinitis subsequently ensued, thus enabling correct diagnosis and treatment.Case description.A 62-year-old male presented with bilateral drop in vision. One month earlier, right eye (RE) arteritic ischemic optic neuropathy was suspected because of severe headache, sudden drop in vision, relative afferent pupillary defect, and elevated inflammatory markers. Systemic steroids were instituted. Brain imaging and temporal artery biopsy were unyielding. Four months earlier, liver biopsy performed because of elevated cholestatic liver enzymes, revealed granulomatous hepatitis. After ruling out viral hepatitis, autoimmune etiology was presumed, and prednisone was started. On presentation, visual acuity (VA) was counting fingers in RE and 6/20 in the left eye (LE). Bilateral panuveitis with punctate inner retinitis, placoid chorioretinitis was diagnosed. Serological tests were strongly positive for syphilis. Lumbar puncture confirmed the existence of neurosyphilis. Systemic penicillin was initiated. One month later, VA improved to RE 6/10, LE 6/7.5, with marked resolution of posterior uveitis. No recurrence was observed over 27-month-period.Acquired syphilitic hepatitis is rarely reported. Administering steroids potentially aggravated the infection. The characteristic features of placoid chorioretinitis and punctate inner retinitis connected the pieces of the puzzle together to the diagnosis of ocular and neurosyphilis. Intrahepatic cholestasis with negative serological panel of hepatotropic pathogens should raise the suspicion of non-hepatotropic pathogens especially syphilis. [ABSTRACT FROM AUTHOR]

Purpose: To study the prevalence and time of onset of ocular complications in intermediate uveitis (IU) and to assess their effects on visual outcome in short-term and long-term follow-up. Methods: A retrospective cohort study of patients with IU who had a minimal follow-up of one year. Results: 96 patients (174 eyes, 70% females) were included with a mean age at presentation of 30 years. Children constituted 38% of all patients. Mean follow- up was 64.9 months. Pars planitis was the most common form followed by sarcoidosis and multiple sclerosis. Cystoid macular edema (CME), cataract, epiretinal membrane and posterior synechiae were the most prevalent complications. Posterior synechiae, band keratopathy, cataract and papillitis at presentation were associated with worse presenting visual acuity (VA). Of the late-onset complications, glaucoma/ocular hypertension (OHT) was the most significantly associated with worse long-term VA. Most sight-threatening complications (namely CME and glaucoma) were diagnosed at presentation while late complications predominantly affected the posterior segment and included among others peripheral retinal elevations, vasoproliferative tumors, and vitreous hemorrhage. Median LogMAR VA improved at short-term and long-term follow-up, from 0.2 to 0.1 (p<0.001). Subgroup analysis revealed significant VA improvement for eyes with all the different complications except for eyes with glaucoma/OHT. Conclusion: IU is a chronic low-grade uveitis that may be associated with protean early and late complications of the anterior or posterior segments or both. Optimal treatment regimens are imperative for the strict control of inflammation and proper management of complications thus allowing a favorable long-term prognosis. [ABSTRACT FROM AUTHOR]

Purpose: To describe the demographics and clinical characteristics of patients with herpetic anterior uveitis (HAU), and compare characteristics by pathogen, recurrence, and association to iris atrophy.Methods: Multicenter, retrospective study of AU patients diagnosed clinically and by polymerase chain reaction (PCR).Results: The study included 112 eyes in 109 patients: 54 (48.2%) HSV, 34 (30.4%) VZV, 2 (1.8%) CMV, and 22 (19.6%) unspecified diagnosis. HSV eyes, compared to VZV, had a higher recurrence rate, corneal involvement, KPs, iris atrophy, elevated IOP and posterior synechia (p < 0.05). VZV patients had more frequent immunomodulatory treatments and history of systemic herpetic disease (p < 0.05). Fifty-nine (52.7%) eyes had recurrent disease. Iris atrophy was associated with a higher prevalence of posterior synechia, dilated distorted pupil, and high IOP (p < 0.05).Conclusion: Different HAU-causing Herpesviridae produce common clinical findings; therefore, PCR should be used more often to confirm specific diagnosis. Iris atrophy was associated with more severe disease. [ABSTRACT FROM AUTHOR]

Purpose: To study the long-term clinical outcomes of uveitic glaucoma and to identify risk factors for progression.Methods: Retrospective study of uveitic glaucoma patients in two tertiary medical centers in 2003-2015. Patient- and disease-related data was retrieved. Clinical parameters and visual fields measured at predetermined time points were recorded. Outcome measures included maintaining intraocular pressure ≤21 mmHg and preserving visual fields.Results: Included were 34 patients (53 eyes), with a mean follow-up of 7 years. Idiopathic anterior uveitis and open-angle glaucoma were most common. In total, 62% of eyes were steroid responders. Higher IOP was associated with posterior synechiae, peripheral-anterior synechiae, steroidal, and immunomodulatory therapy (p<0.05). Glaucomatous field defects developed in 49%, with most showing no progression, despite elevation of cup-to-disc ratio (p<0.05).Conclusions: Chronic severe uveitis, expressed by structural complications and immunomodulatory therapy, was associated with high IOP and the need for more IOP lowering medications, but was unrelated to glaucomatous damage. [ABSTRACT FROM AUTHOR]

Purpose: To report on the clinical data of seven patients with T-cell intraocular lymphoma (IOL).Methods: Retrospective case series.Results: Seven immunocompetent patients, 12 eyes, 6 women, with T-cell-IOL were included from five countries. Mean age was 53.5 years (range: 25-82). Four patients had systemic-ocular lymphoma, two had CNS-ocular lymphoma, and one had systemic-CNS- ocular lymphoma. Vitritis was the most frequent clinical sign, followed by anterior uveitis and serous retinal detachment. Cytopathologic examination was performed on all ocular specimens (vitreous in six and iris mass biopsy in one patient). Adjunctive diagnostic procedures included immunohistochemistry, molecular tests, and cytokine profiling of vitreous samples. Treatment modalities included systemic chemotherapy (five patients), intravitreal methotrexate (three patients), globe radiotherapy, and intrathecal chemotherapy. Mean survival from diagnosis was 21.7 months (range: 2-69). Two patients are still alive.Conclusions: T-cell IOL has variable clinical manifestations and prognosis. Systemic involvement, SRD, and vitreoretinal involvement were frequently observed. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 45-year-old jeweler with eye dilation. Topics discussed include results of various medical tests; complications of eye dilation and uveitis; and the usage of computed tomography (CT) scan, visual field testing and spectraldomain optical coherence tomography in the treatment.

Purpose Methods Results Conclusions To study the efficacy and long-term effects of infliximab and adalimumab in patients with active refractory non-infectious intermediate, posterior, or panuveitis (NIPPU).Retrospective, longitudinal study.Included were 61 patients (104 eyes) of whom 34 were males (55.74%). Mean age at diagnosis of uveitis was 26.5 ± 16.14 years. All patients had active uveitis at baseline (time of initiation of biological therapy). Median interval between the start of conventional immunomodulatory therapy (IMT) to the introduction of biological therapy was 13.0 (IQR 26.0) months. Ocular inflammation was effectively controlled in 92 eyes (88.46%). The most commonly used TNF-α inhibitor was adalimumab in 47 patients (77%). Mean follow-up time after baseline was 40 ± 34.08 months. In the year preceding the institution of TNF-α inhibitors, the average number of flares was 1.5 ± 1.1/year and it decreased to 0.08 ± 0.29/year in the first year after baseline (p < .0005). Forty-four eyes (42.30%) experienced flare over the entire follow-up period. Mean time to first flare was 14.5 ± 9.26 months. At baseline, the mean dose of prednisone was 25.5-±20.8 mg/day. A marked decrease to a mean prednisone dose of 7.85 ± 9.7 mg/day was observed at 6 months (p = .03). In patients treated with adalimumab, the mean time to prednisone dose ≤7.5 mg/day was 4.02 ± 4.89 months compared to 15.64 ± 21.34 months in patients treated with infliximab (p = .03). 64.3% of patients treated by infliximab had Behçet uveitis compared to 27.7% of patients treated by adalimumab. Eyes treated with adalimumab experienced first flare at a mean time of 14.11 ± 6.29 months, whereas eyes treated with infliximab experienced first flare at 18.29 ± 14.24 months after baseline (p < .0005). The risk for moderate and severe visual loss was lower with shorter duration of uveitis before initiating anti-TNF-α treatment (odds ratio, 0.003; 95% CI, 0.000–0.005; p = .023), better presenting logMAR VA (odds ratio, 0.266; 95% CI, 0.172–0.361; p < .0005) and when adalimumab was used (odds ratio, 0.354; 95% CI, 0.190–0.519, p < .0005).Anti-TNF-α therapy was successful in controlling refractory NIPPU in the majority of cases. It significantly reduced flare rate, exerted steroid-sparing effects, and preserved visual potential. Adalimumab use, better initial visual acuity, and earlier introduction of anti-TNF- α therapy were associated with a lower risk of visual loss. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the ultrasonographic tissue characteristics of mushroom-shaped versus dome-shaped uveal melanoma and to compare them with the tumor vasculature. Methods We examined clinically and ultrasonically 18 dome-shaped and 11 mushroom-shaped uveal melanomas (29 consecutive patients; mean age 57 years) and compared their normalized A-scan patterns. Using histological sections from a different set of patients (12 dome- and 17 mushroom-shaped tumors), we calculated the percentage of area covered by blood vessels. Results: A-scans of dome-shaped tumors presented low homogeneous reflectivity, whereas the heads of mushroom-shaped tumors had high and irregular reflectivity (p [ABSTRACT FROM AUTHOR]

Purpose. Proliferative index of uveal melanoma cells serves as a prognostic factor. However, different methods are being used to determine proliferative index using immunostaining for proliferative markers. The major differences among assessment methods are whether the mean proliferative activity of all tumor cells in a section or for areas of rapidly proliferating cells is determined, and whether 10 or 20 fields are being evaluated. We aimed to assess the correlation among proliferative indexes obtained by different immunostaining evalutaion methods. Methods. Sections from 60 formalin-fixed paraffin-embedded uveal melanomas were immunostained with MIB-1 antibody. Immunostaining was assessed by counting immunoreactive cells in semi-randomly selected fields (nonselective method) and in areas with maximal immunoreactivity (selective method). Proliferative actvity indexes according to the two methods in 10 and 20 high power fields (one high power filed = 0.785mm[SUP2]) were calculated and compared. Results. The mean positive cell counts per mm[SUP2]( MPCC/mm[SUP2]) according to the selective and the non-Selective methods were 29.8 ± 8.1 and 10.7 ± 2.4 respectively (p = 0.004, paired t-test). Despite these different values, there was a good correlation between the MPCC/mm[SUP2] obtained by the nonselective and the selective methods for each tumor (r = 0.737, p < 0,0001, Pearson correlation). In addition, according to both methods, the readings of the first 10 fields and those of fields 1-20 correlated well. Conclusions. Mean proliferative activity of uveal melanoma cells correlates with the proliferative activity in localized areas of the tumor with rapidly proliferating cells, Therefore, uveal melanomas are classified similarly by the selective and non-selective methods of immunostaining evaluation. However, the two methods yield different proliferative index values for the same tumors, a fact that should be taken into account when results of... [ABSTRACT FROM AUTHOR]

Purpose Methods Results Conclusions To examine whether patients with non-infectious uveitis (NIU) are at increased risk for celiac disease (CeD).Celiac antibody testing was completed in 112 patients. The control group included patients who had undergone upper endoscopy for suspicion of CeD.2/112 (1.79%) of patients with NIU had positive anti-tTG serology and CeD was confirmed in both patients. When compared to the expected risk of CeD in the general Israeli population of 0.31%, this corresponded to an odds ratio of 5.77 (95% CI 1.4118 to 23.4737, P = 0.049). Three additional patients had positive serology for CeD but the diagnosis was not confirmed.An increased risk of CeD was detected in patients with NIU. We therefore recommend screening for CeD in NIU patients. Larger prospective studies are required to further validate these results. [ABSTRACT FROM AUTHOR]