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Aims: To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function., Methods: SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. "Improvement," "unchanged," or "worsening" in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought., Results: Absence of systemic treatment or optic disc hyperfluorescence was associated with improved general health (SF-36). Improvement in binocular contrast sensitivity resulted in better ocular pain score; absence of anterior uveitis relapse, stable fundus findings, no use of cyclosporine or no intravitreal injections resulted in unchanged/better dependency score; no intravitreal injections resulted in unchanged/better mental health score (VFQ-25)., Conclusion: Stability/improvement in QoL scores was associated with controlled inflammation, better visual function, and no need for treatment. Subclinical inflammatory signs did not impact QoL scores.

Background: The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease., Methods: Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface., Results: All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published "fingerprint sign". Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation., Conclusions: Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the "fingerprint sign" on en face reconstructions. These changes persisted for many years despite disease quiescence., (© 2022. The Author(s).)

Purpose: To evaluate associations between functional and structural measurements in patients with non-acute VKHD., Methods: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment. Age- and gender-matched controls were included. Main outcomes were functional and structural abnormalities and their correlation; secondary outcome was correlation of these findings with clinical characteristics, including fundus abnormalities., Results: SAP and mfERG parameters were significantly worse in patients than in controls. Fourteen eyes (43.7%) had disrupted ellipsoid zone (EZ); visual acuity (VA) was similar between eyes with intact or disrupted EZ. Eyes with intact and disrupted EZ differed significantly concerning N1 and P1 amplitudes and N1 peak time values on mfERG and mean sensitivity (MS), central sensitivity (CS), foveal threshold, visual field index, mean deviation (MD) and pattern standard deviation values on SAP. The area under the curve on receiver operating curves for P1 amplitude was 0.81 (cut-off value = 34.7 nV/deg 2 ) and for MD value was 0.84 (cut-off value = -5.2 dB). Central retinal thickness (CRT) significantly correlated with N1 and P1 amplitudes and P1 peak time values on mfERG (r = 0.354, r = 0.442 and r = -0.405, respectively) and MD, MS, CS and fovea threshold (log values) on SAP (r = 0.372, r = 0.406, r = 0.431 and r = 0.414, respectively). Statistically significant associations were found with the presence of peripapillary atrophy and recurrent anterior uveitis with a worse MD value (p = 0.004 and p < 0.001, respectively)., Conclusion: In non-acute VKHD, disrupted EZ and reduced CRT were correlated with impaired mfERG and SAP parameters, even in patients with good VA., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose : To assess the effectiveness of intravitreal (IV) bevacizumab and the need for systemic immunosuppressive therapy (IMT) in choroidal neovascularization (CNV) in Vogt-Koyanagi-Harada disease (VKHD). Methods : CNV treatment consisted of three monthly IV bevacizumab injections as a loading dose; if intra/subretinal fluid (IRF) persisted, further injections were proceeded besides increment in systemic IMT. Outcome analyses at 3, 6, and 12 months were visual acuity, central foveal thickness, macular volume, IRF, and addition of IMT. Results : Seven eyes of six patients were included. Five patients (five eyes) completed a 12-month follow-up and received 12 IV bevacizumab injections. At the 12-month follow-up, visual acuity improved in four out of five eyes ( p = .0568); all eyes had decreased macular volume ( p = .0431) but they still had persistent IRF; and all cases needed IMT introduction/increment. Conclusion : Intravitreal bevacizumab in association with systemic IMT was effective for CNV in VKHD. Active CNV may indicate disease of inadequate clinical control.

Aims : To evaluate associations between vision-related (VR-) and health-related (HR-) QoL metrics and inflammation and treatment in non-acute VKHD patients. Methods : Cross-sectional study in a tertiary center in Sao Paulo, Brazil with 22 patients with non-acute VKHD followed prospectively for ≥12 months since acute disease onset, with systematic evaluation and predefined treatment protocols. VR- and HR-QoL aspects were assessed by VFQ-25 and SF-36 questionnaires, respectively. Associations between the questionnaire's subscale item scores with inflammation and systemic medical therapies were assessed. Results : After generalized linear model analysis, worse VA, severe fundus changes, fluctuation of VA and fluctuation of anterior chamber cells impacted negatively on VR-QoL items. Higher cumulative total dose of corticosteroids and use of immunosuppressive therapy impacted negatively on both questionnaires. Conclusion : Worse VA, clinical inflammation and systemic treatment have a significant impact on VR- and HR-QoL questionnaires. Subclinical choroidal inflammation did not seem to impact QoL.

Purpose : To assess health-related (HR-) and vision-related (VR-) quality of life (QoL) in patients with long-standing Vogt-Koyanagi-Harada disease (VKHD). Methods : Cross-sectional study of 49 patients with disease duration ≥12 months followed at Uveitis Service, Universidade de São Paulo, BR, for at least 12 months. HR- and VR-QoL were evaluated using SF-36 and NEI VFQ-25 questionnaires, respectively. Demographic, clinical and visual function data were compared with questionnaire scores. Results : After generalized linear models, lower mensal household income was associated with lower scores in both questionnaires while unemployment was associated with SF-36 questionnaire only. Treatment with peri-/intraocular medications and ocular surgery were associated with higher scores on SF-36 questionnaire. Worse visual acuity (VA), ocular complications and no ocular surgery were related to lower scores on NEI VFQ-25 questionnaire. Conclusions : On HR- and VR-QoL questionnaires difficulties perceived by patients with long-standing VKHD were mainly associated with socio-economic aspects, VA, local treatment and ocular complications.

Purpose: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset., Methods: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol., Results: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02)., Conclusions: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches.

Hemoglobinopathies are a group of inherited disorders characterized by quantitative or qualitative malformations of hemoglobin (Hb). Some of these diseases present vaso-occlusive phenomena that are responsible for high morbidity in clinical and/or ophthalmologic terms. Diagnosis of hemoglobinopathies is performed exclusively through hemoglobin electrophoresis. From the ophthalmologic perspective, the most important representative of this group of diseases is sickle cell retinopathy, which presents a wide spectrum of fundus manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated. The aim of this review is to present the classification of sickle cell retinopathy and to describe current management and future perspectives for its treatment, taking into consideration the clinical management of these patients.

To report a case of a patient with Valsalva retinopathy that developed sub-internal limiting membrane hemorrhage, underwent pars plana vitrectomy and had visual acuity improvement after that. A 35-year-old healthy patient presented with sudden and painless vision loss of her right eye, after coughing. During the ophthalmologic evaluation, she had a pre-macular hemorrhage and no other abnormalities. Initially, we chose for expectant management, but the hemorrhage did not clear totally. Thus, pars plana vitrectomy associated with internal limiting membrane peeling was indicated, with considerable improvement of her visual acuity, without perioperative complications or significant findings in the optical coherence tomography, autofluorescence and multifocal electroretinogram. In this case, sub-internal limiting membrane hemorrhage treatment with vitreoretinal surgery was relatively useful, with visual acuity improvement and resolution of sub-internal limiting membrane hemorrhage.

This case report describes the presence of bilateral macular atrophy in a patient with Alport syndrome and compares this finding with literature. At fundoscopy, there was a discrete circumscribed macular thinning showing intense retinal pigment epithelium color and the presence of whitish circular retinal lesions ("dots" and "flecks") at nasal mid periphery of both eyes. Optical coherence tomography showed bilateral partial atrophy of the neurosensory retina in the macula, with a greater extent in the temporal region. This case describes a rare ophthalmological finding in Alport syndrome and important to be recognized for a precise diagnosis as well as for determining visual prognosis.

We report the case of a seven year-old male patient, born at term without any perinatal complications, referred to the Retina/Vitreous Service for diagnostic elucidation. He had a history of progressive visual acuity loss on his left eye that started four years ago. On examination, he had decreased corneal diameter and corectopia of the right eye (OD), without any noteworthy findings on the biomicroscopy of the left eye (OS). The fundus of the OD revealed total retinal detachment, and the OS initially showed peripheral retinal vascular abnormalities and retinal exudation, associated with retinal vitreous traction on the temporal sector. The CT and MRI of the brain/orbits showed no abnormalities, except for findings suggestive of an old retinal detachment on the OD, confirmed by ultrasonography, which also showed microphthalmia of the OD. The diagnosis of familial exudative vitreoretinopathy, a rare disease of autosomal dominant inheritance and related to consanguineous marriages, that can initially simulate Coats disease, was proposed. The patient was treated with diode laser photocoagulation in the temporal periphery of the OS, with improvement in the areas of vitreoretinal traction.

A case of a perfluoro-n-octane leakage into the orbital cavity after corneoscleral suture, scleral buckling and pars plana vitrectomy in an eye with perforating injury after trauma is reported for the first time. A previously healthy 39-year-old man was sent for ophthalmic evaluation one day after suffering a penetrating ocular trauma in his right eye while hammering a nail. On the initial evaluation, the patient presented sudden reduction of visual acuity on his right eye with a perforating corneoscleral injury, intraocular foreign body and retinal detachment. The patient was submitted to corneoscleral suture, pars plana vitrectomy with perfluoro-n-octane administration to flatten the retina and scleral buckling, when it was found transfixation of the globe by the intraocular foreign body. Postoperatively, computed tomography scans of the orbit were ordered due to proptosis, which showed the presence of hyperdense images, suggesting leakage of perfluoro-n-octane into the orbital cavity. Thus, in cases of perforating eye injury, one should be suspicious about the possibility of intraocular foreign body, as well as possible occurrence of transfixation of the globe when scheduling the surgery.

Purpose: To assess the prevalence of trachoma in schoolchildren of Botucatu/ SP-Brazil and its spatial distribution., Methods: Cross-sectional study in children aged from 7 to 14 years, who attended elementary schools in Botucatu/SP in November/2005. The sample size was estimated in 2,092 children, considering the 11.2% historic prevalence of trachoma, accepting an estimation error of 10% and confidence level of 95%. The sample was random, weighted and increased by 20%, because of the possible occurrence of losses. The total number of children examined was 2,692. The diagnosis was clinical, based on WHO guidelines. For the evaluation of spatial data, the CartaLinx program (v1.2) was used, and the school demand sectors digitized according to the planning divisions of the Department of Education. The data were statistically analyzed, and the analysis of the spatial structure of events calculated using the Geode program., Results: The prevalence of trachoma in schoolchildren of Botucatu was 2.9% and there were cases of follicular trachoma. The exploratory spatial analysis failed to reject the null hypothesis of randomness (R= -0.45, p>0.05), with no significant demand sectors. The analysis for the Thiessen polygons also showed that the overall pattern was random (I= -0.07, p=0.49). However, local indicators pointed to a group of low-low type for a polygon to the north of the urban area., Conclusion: The prevalence of trachoma in schoolchildren in Botucatu was 2.9%. The analysis of the spatial distribution did not reveal areas of greater clustering of cases. Although the overall pattern of the disease does not reproduce the socio-economic conditions of the population, the lower prevalence of trachoma was found in areas of lower social vulnerability.

Purpose: To report a case of radiation retinopathy treatment with intravitreal injection of bevacizumab (Avastin®) in a patient undergoing radiotherapy for lymphoma in the right orbit. Patient of 55 years-old male, diabetic, diagnosed with an orbital MALT lymphoma three years ago, treated with local radiotherapy (dose: 35Gy) two years ago, complaining of reduced visual acuity of the right eye for about four months. During the ophthalmologic evaluation, he had an exam suggestive of radiation retinopathy. Macular thickness at the optical coherence tomography was 480 µm. Patient was referred to intravitreal injection (0.05 ml) of bevacizumab (Avastin®) in the right eye, showing reduction of macular edema and mild improvement of visual acuity. In this case, the treatment of radiation retinopathy with intravitreal injection of bevacizumab (Avastin®) was relatively useful, with mild improvement of visual acuity due to the regression of macular edema.

Purpose: To evaluate whether the PhotoScreener (PS) is useful as a method of screening in the prevention of amblyopia in children., Methods: 227 preschool children were evaluated through the application of a questionnaire, refratometry measurements and photos using the PS. After this, all children were submitted to cycloplegic refraction, 30 minutes after one drop of cycloplegic agent. Refraction was performed by a mydriatic automatic refractor Shin Nippon. Children identified as suffering from eye problems were treated, according to the criteria: hyperopia greater than +1.50 D, myopia greater than -1.00 D and astigmatism greater than 1.00 D. Data were analyzed through Goodman test, descriptive statistics and study of the specificity and sensitivity of the use of PS, comparing its results with other ophthalmologic assessment methods., Results: There was a male predominance (54.6%), and the majority of children were four or five years old. PS sensitivity (S) compared to refraction under cycloplegia was 50.9%. The specificity (E) was 78.9%, positive predictive value (PPV) = 70%, negative predictive value (NPV) = 62.5% and accuracy (A) = 65.1%., Conclusions: PS, when compared to cycloplegic refraction spherical equivalent, is a reasonable method of screening, although the low sensitivity. A positive point to be emphasized is the considerable specificity.

Purpose: To investigate the dynamics of ocular eyelid movements in newborn infants and preschool-age children., Methods: Fifty newborn infants and 200 preschool children aged 4-6 years were examined. Images of each child, with his or her eyes in the primary eye position looking at an object placed at the child's height, were recorded with a digital videocamera for 3 mins. Complete and incomplete blink rates, opening, closing and complete blink times were calculated., Results: Newborn infants presented a lower number of incomplete movements than preschool children. The complete blink rate was lower in newborn infants (6.2 blinks/min) than in preschool children (8.0 blinks/minute). Eyelid closing, opening and compete blink times were longer in newborn infants than in preschool children at all observation times., Conclusions: Newborn infants had a different pattern of eyelid movement compared with preschool children. Specific characteristics that are found in this group of children particularly, such as immaturity of the neural system and more resistant tear film, may explain these findings in part.

Purpose: To evaluate spontaneous blink activity in the primary eye position in normal preschool children., Methods: Two hundred normal children aged 4 to 6 years were prospectively evaluated. They were digitally videotaped in a standard setting, taking the images on frontal and lateral plans, during the vigil state, in the primary eye position. The object of observation was located at the same level of the pupil. A Sony Lithium camera was used. The images were recorded on 8 mm tapes and transferred to a personal computer (MacIntosh G4) and processed with the iMovie software. Opening, closure and total blink times were measured using frame scale. The eyelid movement rhythm per minute was also evaluated, during three minutes. The data were submitted to statistical analysis., Results: The complete eye blink was more frequently observed than the incomplete eye blink. The blink rate increased with age. The incomplete blink rate was the same for all the evaluated ages. The eyelid opening and closing times and the complete blink time were similar for both sexes, during all evaluation moments. There were no differences between genders regarding eye blink. The closing eyelid time was slower than the opening eyelid time., Conclusions: The complete blink rate increases with age. The closing and the opening eyelid times and the complete blink were similar for both sexes in all evaluated ages and the opening time is faster than the closing time.

Purpose: To evaluate the eyebrow position in children and elderly using angle measurements., Methods: The eyebrow position was evaluated by digital images in 4 to 6-year-old children (children group) and in patients with 50 years or more (elderly group), separated by age range. The images were taken in the primary gaze position with a Sony Lithium camera and then transferred to a MacIntosh G4 computer and processed by the NIH 1.58 program. The eyebrow position was evaluated using angle measures. Comparisons were done according to age, sex and laterality and the data were submitted to statistical analysis., Results: The comparison of the angular measures showed that there was a significant difference in the position of eyebrow position between the studied groups with the same age. But comparing children and elderly there were differences in all performed measurements., Conclusion: The eyebrow external angle positions showed differences between children and elderly with a positive association between eyebrow position and age.