ACUTE IDIOPATHIC MACULOPATHY: A Proposed Disease Staging Based on Multimodal Imaging.

PURPOSE: To describe the clinical course and the multimodal imaging of acute idiopathic maculopathy. METHODS: Medical records and multimodal imaging including color fundus photography, optical coherence tomography, and fundus autofluorescence were retrospectively reviewed. Recognition of the fundus autofluorescence patterns and their relationship with the disease duration, best-corrected visual acuity, and optical coherence tomography features represented the main outcome measures. RESULTS: Seventeen eyes of 16 patients (7 women; mean age 29.9 years) with a mean follow-up of 23.9 months were included. The mean best-corrected visual acuity at presentation was 0.63 ± 0.54 logarithm of the minimum angle of resolution (Snellen equivalent, 20/85). All but one patient had the best-corrected visual acuity recovery to 20/20. Four sequential patterns of fundus autofluorescence corresponding to 4 proposed stages of disease were observed. Patterns 1 (central hypoautofluorescence with surrounding hyperautofluorescence) and 2 (stippled hyperautofluorescence and hypoautofluorescence) were found at presentation. Patterns 3 (central hyperautofluorescence surrounded by hypoautofluorescence) and 4 (hypoautofluorescence) were observed during the disease course and/or at the last follow-up visit. Duration of the disease was significantly different between patterns at baseline and last visit. Pattern 1 significantly related to the presence of subretinal detachment (Fisher's exact test; P =0.003) on optical coherence tomography in comparison with Pattern 2. Pattern 4 showed unique homogeneously decreased autofluorescence with corresponding attenuation of retinal pigment epithelium and restored outer retinal layers on optical coherence tomography. CONCLUSION: A sequential disease staging based on multimodal imaging for acute idiopathic maculopathy is proposed. The recognition of the observed imaging patterns may help clinicians in the correct diagnosis and patient counseling.

NODULAR POSTERIOR SCLERITIS: Clinico-Sonographic Characteristics and Proposed Diagnostic Criteria.

PURPOSE: To report the clinical and ultrasound features and outcomes of a series of nodular posterior scleritis. METHODS: Retrospective medical record review of 11 consecutive patients with nodular posterior scleritis. Patient demographics, ocular and systemic findings, ultrasound features, and final anatomical and visual outcomes were recorded. RESULTS: There were 9 females and 2 males (11 eyes) with mean age at presentation of 57 years (range, 30-84 years). Underlying systemic inflammatory disease was present in 73%. Symptoms included pain in 73% and blurred vision in 45%. A solitary amelanotic mass without the presence of lipofuscin was found in all cases. Associated ocular features included retinal pigment epithelial changes (67%), intraocular inflammation (55%), subretinal fluid (50%), macular edema (50%), and choroidal folds (30%). B-mode ultrasound showed a sclerochoroidal mass with high internal reflectivity (100%) of mean elevation of 4.1 mm. There was nodular thickening of the sclera (100%) and fluid in Tenon space or "T" sign (36%). A complete regression of the nodule after the treatment was observed only in 1 patient (11%) and partial regression in 4 patients (44%). CONCLUSION: Nodular posterior scleritis should be considered in the differential diagnosis of a single amelanotic choroidal mass showing high internal reflectivity on ultrasound B-scan. It can produce intraocular inflammation in 50% of the cases and may be painless in 25%. It has a high association with a systemic underlying disease.

Do blue-light filtering intraocular lenses affect visual function?

PURPOSE: To study different aspects of visual function, macular changes, and subjective differences between the eye with an ultraviolet (UV) and blue-light filtering intraocular lens (IOL) and the fellow eye with a UV-light filtering IOL. METHODS: Thirty patients (60 eyes) with senile cataract had both cataracts extracted, and an IOL was implanted at least 2 years before clinical evaluation. In one eye, AcrySof SA60AT (a UV-light filtering IOL) was implanted, whereas in the contralateral eye, AcrySof IQ SN60WF (a blue-light filtering IOL) was implanted. Each patient underwent visual acuity testing, color vision testing (Ishihara and Farnsworth-Munsell 100-hue tests), and contrast sensitivity (CS) testing. The macula was evaluated with optical coherence tomography and with clinical examination. Patients were asked if they noted any difference between the implanted IOLs concerning visual impression. Subjective visual quality was evaluated using the National Eye Institute Visual Functioning Questionnaire. RESULTS: There was a borderline statistically significant difference in the mean best-corrected visual acuity (p = 0.05). As regards color vision, no significant changes in Ishihara and Farnsworth-Munsell 100-hue error scores were detected between both eyes (p = 0.48 and p = 0.59, respectively). Analysis of CS showed no significant difference between the groups at any spatial frequency. There were also no statistically significant differences in central macular thickness and total macular volume between the two IOL groups (p = 0.72 and p = 0.61, respectively). In both IOL groups, three eyes developed an epiretinal membrane, and six eyes developed early signs of age-related macular degeneration. CONCLUSIONS: This study showed no significant effects of a blue-light filtering IOL on visual acuity and no influence on color perception and CS. After more than 2 years, there were no significant differences in macular changes between the IOL groups. Clinical evidence of the effect of a blue-light filtering IOL on macular protection is still lacking.

Iris Ring Melanoma Presenting as Scleral Pigmentation.

To report a patient with a very rare variant of iris melanoma that grows in the shape of a ring (ring melanoma). A 65-year-old patient was examined because of a pigmented lesion on the sclera. After a complete ophthalmic and ultrasound examination, a ring melanoma was diagnosed. Enucleation of the affected eye was performed, and histology report confirmed iris ring melanoma. This type of malignancy represents an exceedingly rare variant of uveal melanoma, and because of atypical clinical picture, it can be easily overlooked or misdiagnosed, which often delays adequate treatment. Gonioscopy, transillumination, and ultrasound help us to recognize and diagnose ring melanoma. Suspicion should be raised with a clinical picture that shows unilateral pigmentary glaucoma. The objective of this presentation is to describe and outline the challenging diagnosis and management of this rare disease entity.

Perinatal risk factors associated with central retinal changes in former preterm children on optical coherence tomography angiography.

PURPOSE: To assess the association between perinatal risk factors for retinopathy of prematurity (ROP) and central retinal structures of former preterm children seen on optical coherence tomography angiography (OCTA). METHODS: This prospective cohort study included 40 children with a history of preterm birth and 33 healthy full-term children. We documented their birth weight, gestational age, other significant risk factors for ROP development and presence of ROP. Imaging was performed using swept-source OCTA, and quantitative evaluation was performed. Analytic parameters included the area of foveal avascular zone (FAZ), foveal depth (FD), central subfoveal retinal thickness (CSFT) and capillary density index (CDI) of the deep and superficial capillary plexus. RESULTS: Preterm children had significantly smaller FAZ, lower FD and higher CSFT compared to controls (all p < 0.001). Both groups exhibited no differences in total CDI at the superficial (p = 0.969) and deep capillary plexus (p = 0.370). The duration of mechanical ventilation correlated negatively with FAZ and FD but positively with CSFT. The duration of supplemental oxygen treatment correlated negatively with FD. The presence of intraventricular haemorrhage correlated negatively with FAZ and FD but positively with CSFT. Regression analysis found that the duration of mechanical ventilation and the presence of bronchopulmonary dysplasia were associated with lower FD (p = 0.002 and 0.01, respectively) and higher CSFT (p = 0.002 and 0.028, respectively). CONCLUSION: Central retinal anomalies were identified in former preterm children using OCTA. Macular changes were associated with several risk factors for ROP development.

Anti-VEGF Treatment of Diabetic Macular Edema: Two-Year Visual Outcomes in Routine Clinical Practice.

PURPOSE: The purpose of this study was to evaluate 2-year visual outcomes in patients with diabetic macular edema (DME) treated with anti-VEGF agents in a routine clinical setting. METHODS: The medical records of patients treated with ranibizumab or aflibercept due to DME at the Eye Hospital, University Medical Centre Ljubljana, Slovenia, between January 2016 and March 2019 were retrospectively reviewed. After applying inclusion and exclusion criteria, 123 patients (123 eyes) were included in the study. RESULTS: Baseline visual acuity (VA) was 60.9 ± 15.2 letters (median 63; range 7-85). Baseline central retinal subfield thickness (CRT) was 440.7 ± 132.5 µm (median 430; range 114-1000). No significant change in VA over 2 years was found (mean change +2.1 ± 16.8 letters (median 2; range -53-52)). However, there was a significant change in VA in the subgroup with baseline VA <70 letters (mean change +5.7 ± 17.9 letters (median 5; range -52-52)). VA gains of ≥15 letters were achieved in 25 eyes (20.3%). Changes in CRT were significant over 2 years. Patients received 4.5 ± 2.1 (median 5, range 1-9) and 2.6 ± 2.3 (median 2, range 0-8) injections in the first and second years, respectively. CONCLUSIONS: The two-year visual outcomes in this retrospective analysis appear to be comparable to previously reported outcomes in routine clinical practice. Our analysis provides some information about the effectiveness of anti-VEGF treatment in routine clinical practice in Slovenia. More intensive treatment should be implemented in the management of patients in order to achieve better visual outcomes.

Choroidal structural changes in preterm children with and without retinopathy of prematurity.

PURPOSE: Evaluate choroidal structural changes in preterm children with and without retinopathy of prematurity (ROP) using image binarization technique on swept-source optical coherence tomography (SS-OCT) scans. METHODS: Prospective case-control study. Forty-one (79 eyes) children aged 5-15 years with a history of preterm birth and 33 (63 eyes) age-matched full-term children were recruited. Demographics including gestational age at birth, birth weight and history of ROP were documented. All subjects had undergone complete eye examinations, including best-corrected visual acuity and SS-OCT imaging. Subfoveal choroidal thickness (SFCT) was calculated, and images were binarized to obtain stromal and luminal areas (LA). The choroidal vascularity index (CVI) was derived from the proportion of LA to the total subfoveal choroidal area. RESULTS: There were no significant differences in SFCT between the preterm children with (286.63 ± 83.98 µm) or without (306.59 ± 77.29 µm) ROP and the full-term children (311.82 ± 42.87; p = 0.20 and 0.67, respectively). The CVI was significantly reduced in the preterm children with ROP (68.66 ± 3.24%; p = 0.005) compared with the CVI in the full-term control group (71.37 ± 3.63%); however, the CVI in the preterm children without ROP (71.68 ± 3.09%; p = 0.93) was not significantly affected. CONCLUSION: The reduced CVI in preterm children with ROP may indicate compromised choroidal vascularity. The CVI was found to be a more sensitive OCT biomarker than the SFCT and may be helpful in evaluating associated choroidal structural changes in preterm children, especially those with a history of ROP.

Solitary Iris Plasmacytoma With Anterior Chamber Crystalline Deposits.

PURPOSE: To report a case of solitary iris plasmacytoma successfully treated with ruthenium plaque radiotherapy. METHODS: A 44-year-old white woman presented with pain in the right eye and raised intraocular pressure. Her medical history included breast cancer treated 11 years earlier with lumpectomy, lymph node clearance, chemotherapy, and radiotherapy. On examination, the right iris had a tan-colored mass with crystalline deposits visible on the mass surface and on the corneal endothelium. The fundus and left eye were normal. An anterior segment ultrasound scan showed a mass with mixed internal echogenicity and internal blood flow. RESULTS: An iris biopsy showed an infiltrate of plasma cells. Immunohistochemistry for kappa and lambda light chains demonstrated lambda light chain restriction. Systemic hematologic investigations including complete blood count, serum paraproteins, bone marrow biopsy, and full-body magnetic resonance image were normal. The monoclonal plasma cell infiltrate was consistent with a solitary iris plasmacytoma. The mass was treated with ruthenium plaque radiotherapy. After 4 years of follow-up, the mass remained regressed, and no systemic myeloma has developed. CONCLUSIONS: Iris plasmacytoma is rare and should prompt systemic evaluation to rule out multiple myeloma. Solitary iris plasmacytoma can be successfully treated with plaque radiotherapy.

MESECTODERMAL LEIOMYOMA OF THE CILIARY BODY PRESENTING AS ANTERIOR STAPHYOMA.

PURPOSE: To report two cases of mesectodermal leiomyoma of the ciliary body presenting as anterior staphyloma. METHODS: Two case reports with cytopathologic correlation. RESULTS: First patient (15-year-old boy) presented with a nodular lesion in the sclera. Second patient (31-year-old woman) was found to have a brown ciliary body mass. Growth of the lesion and extrascleral extension was noticed after several years of follow-up. Ultrasonography, light microscopy, and immunohistochemistry of both cases are described confirming mesectodermal leiomyoma of the ciliary body. CONCLUSION: Mesectodermal leiomyoma, despite its rarity, should be considered in the differential diagnosis of uveal tract tumors. Clinically, the diagnosis is difficult and histopathological and immunohistochemical assesment is necessary to avoid inappropriate diagnosis and erroneous treatment.

Patients' Perspectives on Their Dry Eye Disease.

PURPOSE: Although it has been known that patients' perspectives on their disease can significantly affect their level of functional disability as well as disease outcome, limited data are available on patients' perceptions of their dry eye disease (DED). The aim of this questionnaire-based study was to evaluate patients' perspectives on their DED. METHODS: This cross-sectional study included 91 patients with DED. In addition to clinical evaluation, all patients completed a questionnaire to evaluate their perspectives on their DED. This included their satisfaction with understanding DED, their opinion on the easiness of following doctors' advice, their opinion on the effectiveness of the treatment, their satisfaction with the eye care, and their general outlook on DED. RESULTS: This study included 75 (82%) women and 16 men (18%) with a mean age of 57 ± 14 years who had been treated for DED for 5.2 ± 5.4 years. 93% of the patients were satisfied with their understanding of DED, and 76% found it easy to follow their doctors' advice for DED management. Furthermore, 95% thought that the DED treatment had been helpful and 95% were satisfied with their eye care for DED. Forty-eight percent expressed optimism regarding the long-term prospects of their DED. CONCLUSIONS: Although the majority of DED patients have positive perspectives on their disease, close to half report a lack of optimism regarding the long-term outlook for their condition.

Bilateral Posterior Scleritis: Analysis of 18 Cases from a Large Cohort of Posterior Scleritis.

PURPOSE: To describe the clinical and epidemiological characteristics of patients and analyze factors associated with bilateral posterior scleritis. METHODS: In this retrospective cohort study, records of patients with diagnosis of bilateral posterior scleritis at two tertiary-care centers in the UK and India were analyzed in comparison with the clinical profile of patients with unilateral scleritis. RESULTS: In total, 18 patients with bilateral posterior scleritis were identified and compared with 96 patients of unilateral posterior scleritis; 14 (77%) were women and the median age was 48 years. Headache (p = 0.04), optic nerve swelling (p = 0.01), and elevated antinuclear antibodies (ANA) titers (p = 0.03) were present more frequently in patients with bilateral than in unilateral posterior scleritis. Seven patients (38.88%) required immunosuppressive therapy to attain resolution of the inflammation and to prevent relapse. CONCLUSIONS: Bilateral posterior scleritis is an uncommon but severe inflammation of the posterior sclera. The majority of them are idiopathic, often requiring aggressive treatment to prevent visual loss.

Posterior Scleritis: Analysis of Epidemiology, Clinical Factors, and Risk of Recurrence in a Cohort of 114 Patients.

PURPOSE: To describe the clinical and epidemiological characteristics of patients with posterior scleritis, and to analyze the response to treatment and time to relapse. METHODS: Retrospective study of 114 cases of posterior scleritis from two tertiary care, university-affiliated, referral centers in the United Kingdom and India between 2004 and 2013. Data included sociodemographic factors, medical history, clinical, laboratory and ultrasound findings, therapies, and outcomes. LogMAR visual acuity at presentation and final visit and time to relapse were the main outcome measures. RESULTS: The mean age was 45.9 ± 16.8 years, 71.1% were women, and 18 (15.8%) patients had bilateral disease; 71 (62.3%) cases were idiopathic. Rheumatoid polyarthritis (12.28%), systemic lupus erythematous (4.38%) and pANCA(+) systemic vasculitis (5.26%) were the most frequent systemic associations. VA improved by 0.24 ± 0.36 LogMAR between presentation and last follow up (p < 0.001). The median time to remission was 210 days (95% CI: 184-256 days). Recurrences after remission were observed in 36.63%. The observed incidence rate of posterior scleritis relapse after remission was 15.81% per person-year (95% CI: 11.78-20.77%). Systemic disease was present significantly in patients more than 50 years of age (OR = 2.29; 95% CI: 1.01-5.17; p = 0.044). CONCLUSION: Posterior scleritis is an uncommon disease causing pain and visual loss. In around 40% of the cases, it can be associated with other systemic diseases. Median time to relapse was 210 days. Relapses may occur in around 1 in 3 patients, with an incidence rate of 15.81% per person/year.

Floating vitreous cyst: two clinical cases.

PURPOSE: To report two cases of solitary unilateral vitreous cyst. METHODS: A complete ocular examination, fundus photography, B-scan ultrasound and spectral-domain optical coherence tomography were performed in both patients. RESULTS: The first patient (a 39-year-old man) presented with transient blurred vision in the right eye. The second patient (a 78-year-old man) reported transient blurred vision in the right eye when changing head position. He was referred to the Eye Hospital because of vitreomacular traction in the other eye. After examination, a diagnosis of vitreous cyst was made in both cases. CONCLUSIONS: Vitreous cysts are rare clinical findings. They can occur in normal eyes or in eyes with certain ocular pathologies. When a cyst floats into the visual axis area, it can disturb visual function; therefore, patients usually report transient blurring of vision. A prompt clinical examination is necessary for differentiating this rare condition.