Infectious Keratitis: Characterization of Microbial Diversity through Species Richness and Shannon Diversity Index.

Purpose: To characterize microbial keratitis diversity utilizing species richness and Shannon Diversity Index. Methods: Corneal impression membrane was used to collect samples. All swabs were processed and analyzed by Biolab Laboratory (level V-SSN Excellence: ISO 9001:2015), Biolab Srl (Ascoli Piceno, Italy). DNA extraction, library preparation, and sequencing were performed in all samples. After sequencing, low-quality and polyclonal sequences were filtered out by the Ion software. At this point, we employed Kraken2 for microbial community analysis in keratitis samples. Nuclease-free water and all the reagents included in the experiment were used as a negative control. The primary outcome was the reduction in bacterial DNA (microbial load) at T1, expressed as a percentage of the baseline value (T0). Richness and Shannon alpha diversity metrics, along with Bray-Curtis beta diversity values, were calculated using the phyloseq package in R. Principal coordinate analysis was also conducted to interpret these metrics. Results: 19 samples were included in the study. The results exhibited a motley species richness, with the highest recorded value surpassing 800 species. Most of the samples displayed richness values ranging broadly from under 200 to around 600, indicating considerable variability in species count among the keratitis samples. Conclusions: A significant presence of both typical and atypical bacterial phyla in keratitis infections, underlining the complexity of the disease's microbial etiology.

Clinical Features of Infectious Uveitis in Children Referred to a Hospital-Based Eye Clinic in Italy.

Background and Objectives: To investigate the etiology, clinical features, ocular complications, and visual outcomes in children with infectious uveitis referred to a tertiary uveitis hospital-based service. Materials and Methods: Children with infectious uveitis were included in a retrospective cohort study. The data set was obtained after reviewing the medical records of pediatric patients with uveitis of different causes referred to our center during the period from 2009 to 2019. Clinical evaluations were performed at the time of diagnosis and the end of follow-up. Results: Uveitis of infectious origin was present in 57 (72 eyes) of 314 (18.1%) patients examined. The median age at presentation was 10.9 years (6.1-15.8), 52.6% of patients were female, and 47.4% were male. The main cause of infectious uveitis was viral (56.1% of cases), followed by Toxoplasma gondii infection (24.5%). The anatomical location of uveitis was posterior in 40.3%, anterior in 36.8%, panuveitis in 15.7%, and intermediate in 7% of cases. Ocular involvement was unilateral in 42 children (73.7%) and bilateral in 15 (26.3%) cases. The main causes of reduced visual acuity were cataract and maculopathy in 57.1% and 28.5% of cases, respectively. During the follow-up period, 75% of patients showed significant improvements in visual acuity. Conclusions: Specialist management in a tertiary referral eye care center facilitates early diagnosis and effective treatment of this serious cause of morbidity and vision loss in children.

A Novel Algorithm for the Evaluation of Corneal Nerve Beadings by in vivo Confocal Microscopy in Patients With Type 1 Diabetes Mellitus.

Purpose: Peripheral neuropathy could complicate diabetes mellitus (DM). In vivo confocal microscopy (IVCM) is an ocular examination for the diagnosis of small fiber neuropathies and the detection of the earliest corneal sub-basal nerve plexus (SBP) alterations. Corneal SBP characteristics include focal enlargement along with the nerve fiber, called corneal beadings. These dilatations represent a mitochondrial accumulation induced by the reactive oxygen stress, as a consequence of hyperglycemia. For this reason, corneal beadings are considered indicative of metabolic activity. This study aimed to describe the corneal characteristics of a population of type 1 diabetes mellitus (T1DM) well metabolically controlled, using a new algorithm for the analysis of corneal beading size (BS). Methods: Patients aged ≥18 years affected by T1DM were compared with healthy subjects who underwent IVCM (Confoscan 4; Nidek Technologies Padova, Italy). Starting from the coordinates of the beadings detected by the IVCM, we implemented a new algorithm for automatically measuring BS in corneal SBP images. Results: We compared 20 eyes of T1DM patients with 26 healthy controls. The corneal nerves' fiber length (p = 0.008), corneal nerves' fiber length density (p = 0.008), and the number of fibers (p = 0.017) were significantly lower in the diabetic group compared with controls. There was no difference between diabetic and healthy eyes in the mean number of corneal beadings both in the frame of analysis (p = 0.606) and for 0.1 mm of SBP nerve (p = 0.145). Regarding the BS, patients with T1DM had corneal beadings larger than controls (p = 0.036). Conclusions: We found that the corneal beadings parameters are similar in healthy and T1DM individuals. Nevertheless, measuring the BS with our algorithm, we showed that corneal beadings are enlarged in patients affected by T1DM when compared with healthy controls. Identifying beading expansion in corneal nerve fiber using IVCM should become a useful tool to predict peripheral neuropathy at an early stage.

Vernal keratoconjunctivitis activity induces decrease of ocular surface CD14, TLR-4 and TLR-9 expression.

PURPOSE: CD14 is involved in the modulation of immune reaction via toll-like receptors (TLR) and may influence the development of allergic diseases. The role of CD14 in vernal keratoconjunctivitis (VKC) has not yet been investigated. The aim of this study is to evaluate changes of tear soluble sCD14 and conjunctival CD14, TLR-4 and 9 expression in patients with VKC in the active and quiescent phases. METHODS: Eighteen patients with VKC during active inflammation (group A, N = 9), in the quiescent phase (group Q, N = 5) and after recovery (group R, N = 4) and 10 healthy subjects were included. Expression of sCD14 in tears and of CD14, TLR-4, and TLR-9 by conjunctival epithelium were evaluated by Western Blot in all groups. RESULTS: Expression of tear sCD14 and of conjunctival CD14, TLR-4, and TLR-9 was significantly decreased in group A when compared with healthy subjects and with VKC group Q and R. Lower expression of sCD14, CD14, TLR-4, and TLR-9 were significantly correlated with the severity of papillary reaction, while the lower sCD14 was correlated with severity of conjunctival hyperemia. CONCLUSIONS: Tear sCD14, and conjunctival CD14, TLR4, and TLR-9 decreased during ocular surface inflammatory reaction in patients with VKC. CD14 and TLRs ocular surface evaluation may represent biomarkers of VKC activity and novel therapeutic target.

Corneal Subbasal Plexus in Eyes with Fuchs' Endothelial Corneal Dystrophy after Two Different Endothelial Surgeries.

PURPOSE: To evaluate the morphological features and density of corneal subbasal plexus (SBP) using in vivo corneal confocal microscopy (IVCCM) in patients affected by Fuchs' endothelial corneal dystrophy (FECD) six months after Descemet membrane endothelial keratoplasty (DMEK) and Descemet-stripping automated endothelial keratoplasty (DSAEK). METHODS: We included patients affected by FECD, requiring corneal endothelial surgery due to corneal oedema occurred from 3 to 6 months. 7 eyes underwent DMEK and 7 eyes DSAEK. All patients performed IVCCM preoperative and in six months postoperative. We analyzed SBP parameters, using CS4 Nerves Tracking Tool, and we studied the differences between the two endothelial keratoplasties. RESULTS: Comparing the eyes treated with DMEK with those treated with DSAEK, preoperative corneal thickness, corrected distance visual acuity (CDVA), and age were similar in both groups. SBP was not detectable at preoperative IVCCM in any eye. Postoperatively, the nerve fibers length, the nerve fibers density, the tortuosity, and the number of fibers and of branching did not differ in the eyes that underwent DMEK compared to DSAEK. The corneal beadings density was higher after DMEK than DSAEK, and this difference was statistically significant (P = 0.004). The type of endothelial keratoplasty was not associated with the presence or absence of postoperative corneal SBP (Pearson' chi-square, 0.755). CONCLUSIONS: Postoperative corneal reinnervation should be easily and noninvasively studied using IVCCM. Morphological postoperative features of SBP did not differ between two different types of endothelial keratoplasty, DMEK and DSAEK, despite the different sizes of the corneal incision. The lower beading density in the DSAEK group should be the consequence of a different distribution of mitochondria along the nerve fibers, as expression of a supposed higher metabolic distress in the DSAEK group.

To compare corneal confocal microscopy of eyes with Fuchs' endothelial corneal dystrophy after two different endothelial surgeries.

PURPOSE: To study the features of corneal confocal microscopy of eyes with Fuchs' endothelial corneal dystrophy (FECD) after successful Descemet stripping automated endothelial keratoplasty (DSAEK) versus Descemet membrane endothelial keratoplasty (DMEK). METHODS: Thirty-two eyes affected by FECD with corneal oedema requiring a corneal graft were treated with DSAEK (15 eyes) or DMEK (17 eyes). All patients underwent in vivo corneal confocal microscopy (IVCCM) at 6 months postoperatively. We evaluated preoperative and postoperative corrected distance visual acuity (CDVA) and the correlation with IVCCM characteristics. RESULTS: Using IVCCM, Z-scan curve analysis showed similar subepithelial reflectivity peaks between the two groups (DSAEK 1256 SU ± 514 vs DMEK 1118 SU ± 408, p = 0.411), while the interface reflectivity was significantly higher in the DMEK group (1511 SU ± 357) than in the DSAEK group (1029 SU ± 413, p = 0.002). CONCLUSION: Comparing the corneal confocal microscopic characteristics after DMEK with those after DSAEK and their correlation with visual outcome at 6 months, we hypothesized that the presence of a third reflectivity peak in the Z-scan curves of DSAEK patients could justify the poorer visual outcome with this endothelial surgery than with DMEK.

Repeatability of automated measurements by a new anterior segment optical coherence tomographer and biometer and agreement with standard devices.

We assess repeatability of automatic measurements of a new anterior segment optical coherence tomographer and biometer (ANTERION) and their agreement with those provided by an anterior segment-optical coherence tomography device combined with Placido-disk corneal topography (MS-39) and a validated optical biometer (IOLMaster 500). A consecutive series of patients underwent three measurements with ANTERION and one with MS-39. A subgroup of patients underwent biometry also with IOLMaster 500. Repeatability was assessed by means of within-subject standard deviation, coefficient of variation (COV), and intraclass correlation coefficient (ICC). Agreement was investigated with the 95% limits of agreement. Paired t-test and Wilcoxon matched-pairs test were performed to compare the measurements of the different devices. Repeatability of ANTERION measurements was high, with ICC > 0.98 for all parameters except astigmatism (0.963); all parameters apart from those related to astigmatism revealed a COV < 1%. Repeatability of astigmatism improved when only eyes whose keratometric astigmatism was higher than 1.0 D were investigated. Most measurements by ANTERION and MS-39 showed good agreement. No significant differences were found between measurements by ANTERION and IOLMaster, but for corneal diameter. ANTERION revealed high repeatability of automatic measurements and good agreement with both MS-39 and IOLMaster for most parameters.

An Unusual Retinal Vessel Modification in Patients Affected by JIA-Uveitis with a Follow-Up Longer Than 16 Years.

PURPOSE: To report unusual and rare clinical changes of retinal vessel pattern in a series of patients affected by Juvenile Idiopathic Arthritis (JIA) uveitis with a follow-up longer than 16 years. METHODS: A series of three patients with JIA-uveitis followed at the University of Rome "Sapienza" from 1998 to 2014 were reported. The retinal vessels were analyzed with fluorescein angiography using Heidelberg Retinal Angiogram-2 (HRA-2; Heidelberg Engineering GmBH, Dossenheim, Germany) and the Topcon TRC-50LX retinal camera (Topcon Europe, The Netherlands). A Spectralis Domain OCT (SD-OCT) (Spectralis Family Heidelberg, Germany) was performed to evaluate vessel anatomy. RESULTS: Fundus photography showed sheathed vessels localized around the optic disc in every case. Angiography revealed a normal physiology of vessel walls and flow; no sheathing or leakage of dye was observed. SD-OCT demonstrated reflective vessel walls. Vessel lumen appeared patent, and the normal "hourglass configuration" was blurred, but identifiable. CONCLUSIONS: Vessel modifications observed in long-standing JIA-uveitis are not signs of vascular inflammation and are not associated to hypoperfusion. In these cases, ophthalmologists should avoid further invasive investigation and should consider introducing SD-OCT as a routine method to evaluate the vessel changes during the follow-up.

Repeatability of anterior segment measurements by optical coherence tomography combined with Placido disk corneal topography in eyes with keratoconus.

Corneal tomography is an important tool to identify and follow up eyes with keratoconus. Our study evaluate the repeatability of the automatic measurements provided in keratoconic eyes by a new anterior-segment optical coherence tomographer (AS-OCT) combined with Placido-disk topography (MS-39, CSO) and assess their agreement with the corresponding measurements taken with a rotating Scheimpflug camera combined with Placido-disk topography (Sirius, CSO). Mean simulated keratometry, posterior and total corneal power, total corneal astigmatism, corneal asphericity, thinnest corneal thickness, epithelial thickness, corneal diameter, and aqueous depth were evaluated. Repeatability was assessed using test-retest variability, the coefficient of variation, and the intraclass correlation coefficient; agreement was assessed by the 95% limits of agreement. Good repeatability was achieved for most parameters. Moderate repeatability was found for total corneal astigmatism measurements. The repeatability of mean simulated keratometry and total corneal power measurements worsened with more severe stages of keratoconus with a statistically significant relationship between the individual coefficient of variation and corneal power values. Agreement with the Scheimpflug camera was moderate for aqueous depth and thinnest corneal thickness and poor for most other measured parameters. The good repeatability of automatic measurements suggests the new AS-OCT device to be a viable option in clinical practice of eyes with keratoconus.

Early Alterations of Corneal Subbasal Plexus in Uncomplicated Type 1 Diabetes Patients.

PURPOSE: The purpose of our study is to describe the in vivo corneal confocal microscopy characteristics of subbasal nerve plexus in a highly selected population of patients affected by type 1 diabetes mellitus (T1DM) without any microvascular diabetes complications. METHODS: We included 19 T1DM patients without diabetic peripheral neuropathy, diabetic autonomic neuropathy, diabetic retinopathy, and microalbuminuria. All patients underwent in vivo corneal confocal microscopy and blood analysis to determine subbasal nerve plexus parameters and their correlation with clinical data. We compared the results with 19 healthy controls. RESULTS: The T1DM group showed a significant decrease of the nerve fiber length (P=0.032), the nerve fiber length density (P=0.034), the number of fibers (P=0.005), and the number of branchings (P=0.028), compared to healthy subjects. The nerve fiber length, nerve fiber length density, and number of fibers were directly related to the age at onset of diabetes and inversely to the duration of DM. BMI (body mass index) was highly related to the nerve fiber length (r = -0.6, P=0.007), to the nerve fiber length density (r = -0.6, P=0.007), and to the number of fibers (r = -0.587, P=0.008). No significant correlations were found between the corneal parameters and HbA1c. CONCLUSIONS: Early subclinical fiber corneal variation could be easily detected using in vivo corneal confocal microscopy, even in type 1 diabetes without any microvascular diabetes complications, including diabetic peripheral neuropathy, diabetic autonomic neuropathy, diabetic retinopathy, and microalbuminuria.

Standardization of red flags for referral to rheumatologists and ophthalmologists in patients with rheumatic diseases and ocular involvement: a consensus statement.

Ocular involvement is a common manifestation of inflammatory rheumatic diseases, often requiring a multidisciplinary collaboration between rheumatologists and ophthalmologists. The aim of this study was to standardize "red flags" for referral for rheumatologists and ophthalmologists using a Delphi consensus for the management of rheumatic diseases with ocular involvement. The scientific board comprised 11 Italian hospital-based rheumatologists (N = 6) and ophthalmologists (N = 5). A systematic review identified potential red flags for referral. The panel developed 19 statements consisting of (a) referral from ophthalmologist to rheumatologist (b) referral from rheumatologist to ophthalmologist and (c) overarching principles relating to multidisciplinary roles/goals and management. Voting was performed anonymously using an online Delphi method. Each participant expressed a level of agreement on each statement using a 5-point scale (1="strongly disagree"; 5="strongly agree"). Total cumulative agreement was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"), consensus defined as ≥ 80% cumulative agreement for each statement. Positive consensus among 11 participants was reached for 15/19 (78.9%) statements. Statements not reaching consensus were discussed in a face-to-face meeting prior to the second vote (10 participants). Positive consensus was reached for all 19 statements, with final total cumulative agreement of 90-100%. This is the first Delphi consensus undertaken to standardize red flags for referral to rheumatologists and ophthalmologists for patients with rheumatic diseases and ocular involvement.

Psoriasis and Psoriatic Arthritis-Related Uveitis: Different Ophthalmological Manifestations and Ocular Inflammation Features.

PURPOSE: To compare clinical features of uveitis in patients affected by psoriasis and psoriatic arthritis (PsA). METHODS: A retrospective case-control study. RESULTS: 117 patients were affected by uveitis and psoriasis or PsA (92 and 25 patients, respectively) from 2003 to 2013. Axial PsA was associated with unilateral uveitis onset compared to the peripheral pattern (p < 0.03). The prevalence of eyes with at least one complication involving anterior segment was significantly more represented in the PsA group than the psoriasis one (p = 0.024). Anterior segment complications were more frequently associated with posterior ones in PsA patients than in psoriasis patients (p = 0.005). Most common complications in total sample at baseline examination were cataract (29.7%), ocular hypertension (17%), macular edema (7%), and pupillary seclusion (4.4%). CONCLUSION: Uveitis in patients with psoriasis and PsA may have distinguishing clinical features. PsA patients have more ocular complications than those with psoriasis. Both groups need an ophthalmological examination to promptly detect ocular co-morbidity.

Ocular Behçet's Disease: Changing Patterns Over Time, Complications and Long-Term Visual Prognosis.

PURPOSE: To investigate demographic, clinical features and complications of Italian patients with ocular Behçet's disease (BD) over time. METHODS: Retrospective study of 385 patients examined from 1968 to 2011, 265 of whom had follow-up ≥ 12 months. RESULTS: A significant increase in the proportion of females (p = 0.03) and anterior uveitis (p < 0.001), and a decrease in the complete type of BD in all patients (p = 0.003) and in males (p = 0.002), and in hypopyon (p = 0.024) were observed over time. Optic neuropathy (p < 0.0001), maculopathy (p = 0.002), retinal detachment, and retinal neovascularization (p = 0.02) decreased over time, with no difference between genders, concomitant with an increase in the use of immunosuppressive drugs (p = 0.003). Visual acuity ≤1/10 was detected more often in males than females (p = 0.003). CONCLUSIONS: A significant shift in clinical manifestations of BD patients was observed over 44 years. Immunosuppressive therapy succeeded in lowering ocular complications. The incidence of ocular BD is increasing in females, but visual prognosis is still worse in males.

Aspergillus flavus endophthalmitis after penetrating keratoplasty combined with cataract phacoemulsification and IOL implantation.

To report the clinical findings and management of a case of Aspergillus flavus endophthalmitis following penetrating keratoplasty (PKP) and combined cataract extraction. Clinical cornea appearance was evaluated by slit-lamp examination. Ocular ultrasonography was performed to evaluate the anterior chamber and vitreous cavity. The cornea was scraped. The corneal-scleral donor rim and media were cultured. The diagnosis of A. flavus infection was made. The patient received fortified antifungal drops (voriconazole 1 % solution) plus systemic voriconazole 400 mg/die. A second corneal transplant was performed, and the anterior chamber was cleaned and washed with a solution of voriconazole 1 %. At the end of follow-up, CDVA was 20/20 and slit-lamp examination showed a clear cornea graft. This case illustrates a severe A. flavus endophthalmitis after PKP and demonstrates the possibilities of visual function restoration. Furthermore, this case describes the different sources of fungal infection after PKP and the different clinical appearances.

Juvenile idiopathic arthritis-associated uveitis at an Italian tertiary referral center: clinical features and complications.

PURPOSE: To describe the frequencies and risk factors of ocular complications and poor visual outcomes in children with juvenile idiopathic arthritis (JIA). METHODS: Retrospective cohort study, including 69 consecutive children (116 eyes) affected by JIA-associated uveitis managed at a tertiary uveitis clinic. RESULTS: The incidence of visual loss to the 20/50 or worse threshold was 0.04/eye-year (EY) and to the 20/200 or worse threshold was 0.02/EY. The most common complications at baseline were posterior synechiae (52%), band keratopathy (38%), and cataract (12%). Risk factor for a visual acuity threshold of 20/50 or worse included hypotony (p = 0.01; hazard ratio [HR] 3.7; 95% CI 1.3-10.4); anterior chamber flare >1 (p = 0.04; HR 1.3; 95% CI 0.5-3.4); a positive antinuclear antibody (ANA) (p = 0.02; HR1.4; 95% CI 0.8-2.4). Hypotony and positive ANA are also associated to the 20/200 or worse threshold (p = 0.03; HR 5.1; 95% CI 1.1-23.9 and p = 0.04; HR 1.0; 95% CI 0.4-2.3; respectively). Use of immunosuppressive drugs was associated with a reduced risk of visual loss of 20/200 or worse (odds ratio 0.14, 95% CI, 0.02-1.29; p = 0.04). CONCLUSIONS: Loss of vision and ocular complications still occur among children with JIA-related uveitis. Prompt diagnosis and a strict follow up associated to immunosuppressive therapy may decrease the poor visual outcome.

Intermediate uveitis: comparison between childhood-onset and adult-onset disease.

PURPOSE: To compare demographic and clinical data, systemic disease association, visual prognosis, and complications found in childhood and adult onset of intermediate uveitis (IU). METHODS: Retrospective cohort study of 287 patients with IU: 122 (42.5%) children at onset (<16 years), 165 (57.5%) adults. The data were entered on a computer-based standardized data entry form for statistical analysis. Student t test was used regarding differences in means. To assess significance of differences in proportions, we used the χ2 test, but when the population in the subgroup was ≤ 5 patients, we used the Fisher exact test (p<0.05 were deemed to be statistically significant and as reported were not corrected for multiple testing and so should be viewed as nominal). RESULTS: A total of 61% of childhood-onset cases occurred in boys (44% in the adult group), while adult onset was more common in women (56% vs 39%) (p = 0.004, odds ratio [OR] 2.06). Regardless of the onset age, IU was frequently bilateral and idiopathic. The most frequent complication was cystoid macular edema (27.5%, p = 0.469, OR 0.85), which was also the most frequent cause of visual loss. Optic disc edema was more prevalent in children (15.5% vs 9.2%; p = 0.027, OR 1.81), ocular hypertension in adults (15.6% vs 7.5%, p = 0.007, OR 0.44). A total of 25 patients had non-idiopathic IU. A total of 14 patients had multiple sclerosis. CONCLUSIONS: Intermediate uveitis was mostly idiopathic and bilateral in both groups. Visual prognosis was good and it was not age-related. Childhood onset was more frequent in boys, adult onset in women. Endocrinal factors could be involved.

Severe macular edema in patients with juvenile idiopathic arthritis-related uveitis.

Purpose. To report the onset of severe macular edema in adolescent female patients affected by juvenile idiopathic arthritis (JIA). Methods. Four female patients affected by JIA-related chronic anterior uveitis (CAU), complicated by severe macular edema, were retrospectively analyzed. Macular area was evaluated by fluorescein angiography and optical coherence tomography (OCT). Results. CAU was bilateral in three patients. Mean age of uveitis and arthritis onset was, respectively, 4.5 ± 1.7 years and 6.0 ± 3.9 years. All patients underwent cataract extraction surgery. Despite ocular inflammation being controlled by topical/systemic therapy, during adolescence (mean age of appearance/diagnosis: 12.7 ± 3.9 years) patients developed severe unilateral macular edema. OCT revealed massive macular thickening (range from 550 µ m to 1214 µ m). Conclusions. Macular edema appeared in female adolescent patients in eyes with long-dating CAU submitted to cataract surgery. In such patients, in presence of age-related microvascular changes due to the enhancer effect of sex hormones, cataract extraction should be a factor triggering the retinal complication.

Uveitis with retinal occlusive vasculitis and sensorineural hypoacusia as first symptoms of relapsing polychondritis.

We herein describe an atypical case of relapsing polychondritis, presenting initially with isolated ocular signs characterised by uveitis and retinal occlusive vasculitis preceded by 10 years of auricular and laringothracheal chondritis. This case highlights the importance of considering connective tissue inflammatory conditions in any retinal vasculitis. A systemic enquiry is invaluable in order to avoid a delayed diagnosis and the subsequent associated complications and mortality.

Intermediate uveitis in a pediatric Italian population.

PURPOSE: To investigate clinical data, outcome, and treatment of intermediate uveitis in children. METHODS: Retrospective cohort study, including 116 children affected by intermediate uveitis. RESULTS: Sixty-two percent of patients were males. Ocular involvement was bilateral in 84%. Mean age at uveitis onset was 10.3 ± 3.6 years and 12.2 ± 7 years at first visit. Ninety-seven percent were classified as having idiopathic uveitis. The incidence of ocular complications during follow-up was 0.131/eye-year. Macular edema or a marked vitreous haze at presentation may be risks factors for incident visual loss. The most frequent causes of visual loss were macular edema (64%) and cataract (21%). CONCLUSIONS: Most cases are idiopathic (pars planitis); only few children presented associated systemic disease. Macular edema and severe vitritis at presentation are associated with increased risk of visual impairment. Cystoid macular edema is the main cause of visual impairment.