Pediatric Non-Infectious Uveitis: Long-Term Outcomes and Complications.

PURPOSE: To describe the long-term prevalence of ocular complications and visual prognosis in patients with pediatric uveitis. METHODS: Demographics, etiology and location of uveitis, type of complications, treatment and visual outcomes were recorded in 296 children at first examination and at 1-, 2-, 3-, 5- and 10-year time points. RESULTS: Αnterior uveitis represented 53.4% of cases, followed by intermediate (28.0%), posterior uveitis (11.1%) and panuveitis (7.4%). The leading diagnoses were idiopathic uveitis (31.1%), juvenile idiopathic arthritis (27.0%) and pars planitis (22.6%). Posterior synechiae was the most frequent complication of anterior uveitis and panuveitis, cystoid macular edema and disc edema of intermediate and posterior uveitis respectively. Posterior uveitis and panuveitis had more severe final vision loss (23.1% and 20% respectively). CONCLUSIONS: This study provides clinical characteristics and main complications in a longitudinal long-term follow-up of a large non-infectious pediatric uveitis Greek population. Early diagnosis and close monitoring remain of fundamental importance.

Biologic Treatment Options for Retinal Neovascularization in Behçet's Disease.

PURPOSE: Relapsing ocular inflammation occurs in about 70% of patients with Behçet's disease (BD) and can lead to permanent loss of vision. Neovascularization of the optic disc (NVD) or elsewhere in the retina (NVE) is a relatively uncommon but severe complication that lacks standardized treatment. METHODS: We report on the therapeutic use of anti-TNF monoclonal antibodies for BD-associated NVD and NVE in one pediatric patient (subcutaneous adalimumab) and one young man (intravenous infliximab). Also, we review the previously published experience on biologic therapeutic options, namely anti-TNF agents and interferon-alpha in a total of three and eight patients, respectively. RESULTS: A fast-onset therapeutic effect was observed in both patients leading to complete regression of neovascularizations. CONCLUSIONS: Both options may lead to regression of neovascularization, thus preventing loss of vision, but comparative studies need to determine the optimal treatment for this sight-threatening complication of BD.

The role of fundus autofluorescence imaging in the study of the course of posterior uveitis disorders.

BACKGROUND: To evaluate the correlation of fundus autofluorescence (FAF) with indocyanine green angiography (ICGA) in patients with various posterior uveitis disorders. METHODS: Interventional case series including 23 eyes of 15 patients with diagnosis of a specific type of retinochoroiditis, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous-like choroiditis, multifocal choroiditis (MFC), Harada disease, and syphilitic retinochoroiditis. Also, some cases with undefined retinochoroiditis were included. FAF and ICGA were performed and correlated at baseline and during follow-up after treatment. RESULTS: In ICGA, early hypofluorescence was found to be the hallmark of acute choroidal inflammation, resolving in later stages and remaining in the late phase in areas with retinal pigment epithelium (RPE) damage. Poorly defined hyperautofluorescent areas correlated with acute choroidal lesions. Hypoautofluorescent delineation suggested the initiation of RPE healing processes, correlating well with the late phase of ICGA and delineating the RPE damage. Early hyperautofluorescence with late hypofluorescence in ICGA indicated the presence of primary RPE involvement. CONCLUSION: FAF contributes to the interpretation of RPE disease and may be a useful tool for the follow-up of progressive inflammatory disorders. Comparative evaluation of FAF and ICGA allows a characterization of the sequence of inflammatory events and the level of tissue affected.

Evaluation of single-dose azithromycin versus standard azithromycin/doxycycline treatment and clinical assessment of regression course in patients with adult inclusion conjunctivitis.

BACKGROUND: Single-dose azithromycin (AZT) has been proved efficient in treating various human Chlamydia infections. However, it has not been thoroughly tested in patients with adult inclusion conjunctivitis (AIC). It is the aim of this study to perform a comparative evaluation of efficacy and safety of one-day AZT with long-term AZT and doxycycline (DOX) regimens in AIC and to present a clinical profile of regression course of the disease. MATERIALS: Eighty-three consecutive adults, with symptoms and signs of chronic conjunctivitis and positive Polymerase Chain Reaction (PCR) for chlamydia, were randomly assigned in four treatment groups; AZT 1-day 1000 mg orally, AZT 500 mg daily 9 and 14 days and DOX 200 mg 21 days orally. Follow-up visits were scheduled 1 and 2 weeks, 1, 3 and 6 months after treatment completion. PCR was repeated at the 2nd post-treatment week to confirm elimination of infectious agent. Detailed record of subjective symptoms and objective signs was performed at all visits. Retreatment rate among groups was evaluated as primary outcome. Regression rate of symptoms/signs among groups was recorded as secondary outcomes. RESULTS: All treatment groups provided statistically equivalent results of retreatment rate. Statistically significant regression of symptoms/signs was documented, initially from the 1st post-treatment week in general, but 1 month was required for complete patients' relief. Follicles were the most common clinical sign with the earliest regression after successful treatment. CONCLUSION: Single-dose azithromycin should be considered as equally reliable treatment option, comparing to long-term alternative regimens for AIC. Patients should wait for one week, until first signs of significant regression become obvious and should consider approximately one month to total relief. Follicles could be reasonably used as a key sign for clinical assessment of treatment success.

Twelve months of follow-up after intravitreal injection of ranibizumab for the treatment of idiopathic parafoveal telangiectasia.

AIMS: To report the anatomic and functional outcomes of intravitreal ranibizumab in idiopathic parafoveal telangiectasia (IPT). MATERIAL AND METHODS: Four eyes of three patients were included in this interventional case series. One patient (two eyes) had bilateral IPT (type 2) and two patients (two eyes) had unilateral (type 1) IPT. Retreatment was scheduled in case of leakage persistence in combination with visual acuity (VA) deterioration. Fluorescein angiography and optical coherence tomography were performed together with a full ophthalmic examination at baseline, 1, 3, 6, 9, and 12 months after injection. RESULTS: One intravitreal injection of ranibizumab was performed in all four eyes. Complete cessation of leakage was documented postintervention in three eyes and partial cessation in one eye, followed by improvement of best corrected VA in one of them. In all eyes, structural changes of the photoreceptor layer were detected in tomography and were responsible for visual loss, which was in most cases, refractory to the applied therapy. CONCLUSION: Use of ranibizumab might be efficient in eliminating leakage activity in the macular region in patients with IPT. Nevertheless, improvement in VA was infrequent. Preexisting early photoreceptor alteration in IPT might render such patients unable to improve VA.

Evaluation of direct immunofluorescence assay and cytological examination in comparison to polymerase chain reaction of conjunctival swabs in patients with adult inclusion conjunctivitis.

PURPOSE: To evaluate PCR, direct immunofluorescence assay (DIA) and cytological test of conjunctival swabs for the diagnosis of adult follicular conjunctivitis (AFC). METHODS: Eighty-three adult patients with chronic conjunctivitis and sixteen healthy individuals were included. Conjunctival scrapings underwent PCR, DIA and cytological analysis. Exams were repeated two weeks after treatment application. Sensitivity, specificity and agreement rate with PCR of DIA and Cytology were evaluated and correlated with clinical symptoms/signs. RESULTS: Cytology test was more sensitive than DIA and presented an acceptable agreement with PCR (K=0.44) in treatment-naïve patients, concerning especially the combination of both conventional exams (K = 0.77). Inferior diagnostic performance of was detected post-treatment, considering the combination as well (K=0.40). Negative post-treatment PCR correlated well with significant relief of symptoms/signs. CONCLUSION: Combination of Cytology and DIA seems to be a useful diagnostic option for treatment naïve AFC patients. However, PCR remains the most reliable test for post-treatment evaluation.

Human herpes virus-6 as a cause of recurrent posterior uveitis in a HIV-positive patient.

PURPOSE: To report a case of bilateral recurrent posterior uveitis caused by human herpes virus-6 (HHV-6) in a human immunodeficiency virus-positive individual. METHODS: Comprehensive ophthalmic examination, including imaging with optical coherence tomography, fluorescein and indocyanine green angiography, and adequate laboratory tests were performed. A human immunodeficiency virus-positive patient without any AIDS defining condition, with a history of recurrent bilateral posterior uveitis referred to us with the diagnosis of retinal detachment. RESULTS: Vitreous polymerase chain reaction detected an aberrant band for herpes viruses, which proved to be human herpes virus-6 by repeated polymerase chain reactions. Serum antibodies titer was positive for human herpes virus-6. The patient responded well to antiviral therapy with valacyclovir. CONCLUSION: This is the first case of human herpes virus-6-related bilateral posterior uveitis in a human immunodeficiency virus-positive individual without clinical manifestations of AIDS.

Structural and functional outcomes after treatment of uveitic macular oedema: an optical coherence tomography and multifocal electroretinogram study.

BACKGROUND: The aim was to evaluate the correlation between the anatomical and functional outcomes before and after treatment of uveitic macular oedema. METHODS: Thirty-three eyes of 33 patients with uveitic macular oedema were included in the present study. Visual acuity (VA), optical coherence tomography (OCT) and multifocal electroretinogram (mfERG) were measured before and after treatment of the macular oedema. Correlation analyses between VA, OCT and mfERG parameters were performed. RESULTS: The VA and mfERG measurements showed statistically significant improvement after treatment of the macular oedema (p < 0.01) and OCT-measured central foveal thickness decreased significantly from 434 ± 135 µm before treatment to 267 ± 92 µm after treatment (p < 0.001). Correlation analyses showed that uveitic central foveal thickness before treatment was correlated with mfERG N1 response amplitude of area 1 (Spearman's r = -0.62, p < 0.001). VA (logMAR) after treatment had a negative correlation with the mfERG N1 response amplitude of area 1 (Spearman's r = -0.56, p = 0.001). Also, there was no correlation between the final VA and pre-treatment OCT and mfERG measurements. CONCLUSION: This study deals with cystoid macular oedema associated with recurrent uveitis. In cystoid macular oedema, the value of mfERG before treatment is related to the central foveal thickness and VA. In contrast, after treatment the decrease of macular thickness is not always followed by an improvement of mfERG and VA. This supports the view that in uveitic macular oedema, the decrease in macular thickness after treatment may not be used as a predictor of improvement of macular function.

Atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits.

PURPOSE: To report a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits. METHODS: Observational case report of one patient. RESULTS: A 52-year-old man presented with reduced vision in the left eye. Findings resembling acute exudative polymorphous vitelliform maculopathy were noted with ophthalmoscopy, fluorescein angiography, and optical coherence tomography. Funduscopic examination revealed an exudative macular detachment with yellowish subretinal deposits inferior to the fovea. On fluorescein angiography, the perifoveal lesions were minimally hyperfluorescent, with no abnormal fluorescence in the central macula. The subretinal deposits were found to be hyperautofluorescent on fundus autofluorescence imaging. Optical coherence tomography confirmed a serous detachment of the retina with intraretinal cystic spaces. The right eye did not show any abnormalities except for an epiretinal membrane. CONCLUSION: We describe a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits.

Efficacy and safety of latanoprost in eyes with uveitic glaucoma.

BACKGROUND: To compare the efficacy and safety of latanoprost against a fixed combination of dorzolamide and timolol in eyes with elevated intraocular pressure (IOP) or glaucoma and anterior or intermediate uveitis. METHODS: Fifty-eight patients with anterior or intermediate uveitis and elevated IOP or glaucoma presented or followed up in the Ocular Inflammation and Immunology Service of General Hospital of Athens were randomly assigned to receive treatment either with latanoprost (30) or with dorzolamide/timolol (28). The main outcome measures were inflammatory relapses and IOP response to treatment. RESULTS: Ten patients (34%) in the latanoprost group and sixteen patients (57%) in the dorzolamide/timolol group experienced relapses of anterior uveitis (p = 0.93). There was no statistical difference between the two groups in respect of inflammatory relapses (p = 0.21). Twenty-one patients were followed up before starting latanoprost. The number of recurrences of anterior uveitis per patient per year before treatment with latanoprost was 0.82 +/- 1.2. The rate of relapses per patient per year after starting latanoprost was 0.39 +/-0.7 for these patients (p = 0.038). After 1 year of treatment, intraocular pressure was dropped from 27.8 +/- 8.4 mmHg to 18.6 +/- 5.3 mmHg (p < 0.001) in the latanoprost group and from 28.2 +/-8.1 mmHg to 22.6 +/-10.1 mmHg (p < 0.001) in the dorzolamide/timolol group. Four patients during treatment with latanoprost and five patients during treatment with dorzolamide/timolol developed macular edema. CONCLUSION: Latanoprost is safe and equally effective to a fixed combination of dorzolamide and timolol in the treatment of uveitic glaucoma.

Course of macular edema in uveitis under medical treatment.

OBJECTIVE: To describe the response of uveitic macular edema to various treatment methods using optical coherence tomography (OCT). METHODS: This is a prospective study of consecutive uveitis patients with macular edema in at least one eye. The patients received medical treatment. Best corrected Snellen Visual Acuity (BCVA) and tomographic features of the macula, including macular thickness measurement, were obtained at one, three, six, and 12 months after commencing treatment. RESULTS: Eighty-one eyes of 58 patients were analyzed. Complete resolution of macular edema occurred in 38 eyes (47%). The average BCVA was 20/34 logarithm of minimum angle of resolution (-logMAR, 0.2 +/- 0.3) upon study entry and 20/27 (-logMAR, 0.13 +/- 0.29) upon study completion. The difference was statistically significant (p = 0.04). The corresponding mean retinal thickness at the central fovea was 319 +/- 150 microm at the beginning of the study compared to 241 +/- 125 microm at 12 months (p < 0.001). A weak but statistically significant correlation between the reduction of macular thickness and the improvement of BCVA (r = 0.3, p = 0.01) was found. Thirteen of the 43 eyes (30%) with persistent macular edema had a more than 15% reduction of macular thickness compared to baseline, whereas 10 eyes (23, 3%) had a more than 15% increase in macular thickness. Statistical analysis indicated that the presence of an epiretinal membrane and an OCT pattern of diffuse macular edema was a significant factor associated with medical treatment failure. CONCLUSION: This study demonstrates the overall favorable visual prognosis of uveitic macular edema under medical treatment. The presence of an epiretinal membrane is an important factor associated with medical treatment failure.

Intravenous pulse methylprednisolone therapy for acute treatment of serpiginous choroiditis.

PURPOSE: To evaluate the safety and efficacy of high-dose intravenous steroid therapy (HDIST) for the acute treatment of vision-threatening serpiginous choroiditis. METHODS: Retrospective review of the records of five patients with serpiginous choroiditis who were treated with HDIST (1 g methylprednisolone for three days) in addition to their standard immunosuppressive treatment. The visual acuities and improvement of ocular signs after HDIST were evaluated. RESULTS: Twelve episodes of macula-threatening choroiditis in five patients with serpiginous choroiditis were treated during a seven-year period. All patients responded to HDIST with evidence of a decrease in intraocular inflammation immediately after and complete restoration of visual acuity within 10 days of commencing treatment. In one patient, medical intervention was required because of gastric distress. During the follow-up, three out of five patients experienced new attacks and two patients developed subretinal neovascularization. CONCLUSION: HDIST is effective in controlling severe vision-threatening serpiginous choroiditis and in improving visual function in a short period of time. However, the effect of this treatment in long-term disease control is uncertain.

Regression of neovascular age-related macular degeneration following infliximab therapy.

PURPOSE: To describe the effects of the antitumor necrosis factor (TNF) monoclonal antibody Infliximab systemic therapy on choroidal neovacularisation (CNV) secondary to age-related macular degeneration (AMD). DESIGN: Prospective, noncomparative series of three patients. METHODS: A subretinal membrane secondary to AMD was documented by fluoroangiography at baseline in three elderly patients scheduled to receive Infliximab therapy for inflammatory arthritis (infusions of 5 mg/kg at weeks 0, 2, 6, and every 8 weeks thereafter). Follow-up was performed at three months post-baseline, as well as during 18 months of continuing treatment in the first patient. RESULTS: CNV regressed partially at three months and resolved at six months in the first patient. Best-corrected visual acuity (BCVA) increased from 0.05 to 0.2; this effect was sustained at 18 months. Regression of subretinal membrane and increase of BCVA was also documented in the other patients. No ocular or extra-ocular side effects were noted. CONCLUSIONS: These findings suggest a plausible pathogenetic role of TNF in CNV secondary to AMD. Additional patients should be studied to confirm the promising clinical results.

Infliximab for chronic cystoid macular edema associated with uveitis.

PURPOSE: To assess the efficacy of the anti-TNF monoclonal antibody infliximab in uveitis patients without clinically evident ocular inflammation and impaired visual acuity because of chronic cystoid macular edema (CME). DESIGN: Prospective, noncomparative, interventional case series. METHODS: Patients with refractory CME (14 eyes, mean duration of 14 months), associated with intermediate uveitis (n = 6), Adamantiades-Behcet disease (n = 2), adult-type vascular pseudotumor (n = 1), and HLAB27+-related uveitis (n = 1) received an intravenous infliximab infusion (5 mg/kg); five patients were retreated after 1 month. RESULTS: Macular thickness, measured by ocular coherence tomography, was reduced from 428 +/- 138 microm to 219 +/- 51 microm at 2 months postbaseline (P = .0001), while visual acuity increased from 0.41 +/- 0.18 to 0.83 +/- 0.17 (P < .00001). Anatomic and functional improvement was sustained at 6 months in all. No ocular or extra-ocular side effects were noted. CONCLUSION: These promising results suggest that TNF may play an important pathogenetic role in chronic CME, thus, a controlled trial is warranted.

Patterns of macular edema in patients with uveitis: qualitative and quantitative assessment using optical coherence tomography.

PURPOSE: To describe the morphologic characteristics of uveitic macular edema by the use of optical coherence tomography (OCT) and to investigate the correlation between tomographic features and visual acuity (VA). DESIGN: Cross-sectional study. PARTICIPANTS: Seventy consecutive patients with uveitis in a university-based practice with a clinical diagnosis of macular edema in at least one eye. Inclusion criteria were: (1) verification of macular edema by OCT, (2) adequate media clarity for fundus visualization, and (3) absence of coexisting ocular disease limiting visual potential. METHODS: Complete ophthalmic examination: best-corrected Snellen VA, slit-lamp examination, fundus biomicroscopy, indirect ophthalmoscopy, and OCT. Fluorescein angiography was performed in selected cases. MAIN OUTCOME MEASURES: Best-corrected Snellen VA and tomographic features of the macula, including macular thickness measurement and correlation of macular thickness with VA. RESULTS: Eighty-four eyes of 60 patients met the inclusion criteria. The mean VA (Snellen test) was 20/36. There were 3 patterns of macular edema: diffuse macular edema (DME), cystoid macular edema (CME), and serous retinal detachment (RD). Serous retinal detachment was detected in 17 eyes (20.2%). Patients were classified into the following groups: DME (46 eyes, 54.8%), CME (21 eyes, 25%), DME and RD (5 eyes, 5.9%), CME and RD (12 eyes, 14.3%). Epiretinal membrane was detected by OCT in 34 eyes (40.5%). Eight eyes (9.5%) demonstrated vitreomacular traction. The mean retinal thickness at the central fovea was 333+/-171 microm (mean +/- standard deviation). Macular edema was located mainly in the outer retinal layers. Eyes with CME had significantly greater retinal thickness measurements than eyes with DME (P<0.001). Multivariate analysis revealed that VA was negatively correlated with increased macular thickness, presence of CME, and RD (P<0.05). CONCLUSIONS: Optical coherence tomography demonstrated 3 patterns of macular edema in patients with uveitis: DME, CME, and RD. Epiretinal membrane coexisted in a significant percentage of patients. In patients with uveitis with clear media, the morphologic features of macular edema and macular thickness correlated with VA.

Macular hole closure after peribulbar steroid injection.

PURPOSE: To report the closure of a macular hole after a peribulbar corticosteroid injection in a patient with anterior uveitis associated with human leukocyte antigen B*27. DESIGN: Interventional case report.The patient was evaluated by comprehensive ophthalmic check-up and optical coherence tomography. RESULTS: A healthy 50-year-old woman presented to our service with active human leukocyte antigen B*27-associated anterior uveitis, macular hole in the right eye and macular edema in the left eye. Visual acuity was 20/200 in both eyes. Peribulbar injections of triamcinolone acetonide were administered in both eyes. Three months later, visual acuity improved to 20/50 in both eyes, and the uveitis was in complete remission. Optical coherence tomography verified that the macular hole in the right eye was closed. CONCLUSIONS: Inflammation and macular edema might be contributing factors in macular hole formation in patients with uveitis. This case indicates that treatment of inflammation and macular edema might lead to macular hole closure in similar cases.

Cytomegalovirus as a cause of anterior uveitis with sectoral iris atrophy.

OBJECTIVE: To report two cases of recurrent anterior uveitis with sectoral iris atrophy and ocular hypertension during attacks caused by cytomegalovirus (CMV). DESIGN: Two observational case reports. PARTICIPANTS: Two immunocompetent patients with a history of recurrent unilateral hypertensive anterior uveitis with sectoral iris atrophy were referred to us with the presumptive diagnosis of herpetic uveitis. MAIN OUTCOME MEASURES: Comprehensive ophthalmic examination, aqueous humor polymerase chain reaction (PCR), and peripheral blood serologic studies were performed on both patients. RESULTS: Examination of aqueous humor by PCR was positive for CMV and negative for herpesvirus. Serum IgG/IgM titers disclosed past CMV infection. Both patients responded well to antiviral therapy with ganciclovir. The final visual acuity level was 20/20 in both eyes of both patients. CONCLUSIONS: CMV infection can produce recurrent attacks of anterior uveitis with clinical characteristics indistinguishable from those previously considered highly suggestive or even pathognomonic for herpetic infection. This observation has implications for the therapeutic management of such patients.