SPONTANEOUS RESOLUTION OF OPTIC DISK PIT MACULOPATHY IN A CHILD AFTER A SIX-YEAR FOLLOW-UP.

PURPOSE: Optic disk pit (ODP) is a rare congenital abnormality of the optic nerve head that can lead to a maculopathy characterized mainly by serous retinal detachment. Optic disk pit maculopathy (ODP-M) in children is rare, and at present, the best management is still unknown. Long-lasting ODP-M can lead to organic amblyopia and photoreceptor damage, whereas surgical treatments are invasive and have an uncertain prognosis. We present a case of spontaneous resolution of ODP-M in a child who was monitored morphologically and functionally during a 6-year follow-up. METHODS: Between January 2010 and January 2016, we conducted follow-up examinations by fundus photography, optical coherence tomography, and microperimetry. RESULTS: At the first visit, a 12-year-old girl was asymptomatic with a visual acuity of 20/20 in both eyes. Optic disk pit maculopathy was observed, and the progression was monitored by follow-up optical coherence tomography. A progressive anatomical improvement with a spontaneous resolution of ODP-M occurred over a 2-year period. However, at the last follow-up visit, microperimetry showed a loss of threshold values of visual sensitivity. CONCLUSION: The combination of morphologic and functional evaluation over time can be useful to determine the best management of ODP-M, particularly in children for whom the conservative approach seems to be a valid alternative to surgery.

Long-term natural history of highly myopic eyes with a dome-shaped macula with or without untreated serous retinal detachment: a 4-year follow-up study.

PURPOSE: To evaluate the long-term functional and morphological changes occurring in myopic eyes with a dome-shaped macula (DSM), with or without untreated serous retinal detachment (SRD). METHODS: This prospective, single-centre study enrolled consecutive cases of highly myopic patients with DSM with or without a SRD. Patients underwent complete ophthalmological examinations, optical coherence tomography, axial length measurements and autofluorescence. Follow-up visits were performed with a maximum interval of 6 months for 4 years. Eyes with choroidal neovascularisation were excluded. RESULTS: Twenty-six eyes from 18 patients (mean age 61.2) were included. At baseline, 13 eyes had SRD and 13 did not. The DSMs were either horizontal (69%) or round (31%). There were no significant differences in best-corrected visual acuity (BCVA) between eyes with and without SRD during the 48-month follow-up period. Multivariate analysis showed that baseline BCVA was the only parameter among those analysed (age and SRD height) to have a significant effect on the final BCVA (p<0.0001). SRD fluctuated overtime and SRD height was significantly influenced by choroidal thickness (p=0.002). The scleral bulge thickness had no effect on SRD thickness. CONCLUSIONS: BCVA remained clinically stable over 4 years without treatment despite the fluctuations and persistence of the SRDs.

Objective Quantification of Anterior Chamber Inflammation: Measuring Cells and Flare by Anterior Segment Optical Coherence Tomography.

PURPOSE: To assess the ability of swept-source (SS) optical coherence tomography (OCT) of the anterior segment (AS) to measure anterior chamber (AC) inflammation (both flare and cells) objectively. To compare OCT-derived inflammatory indices with standard techniques. DESIGN: Prospective evaluation of a diagnostic test. PARTICIPANTS: Patients diagnosed with anterior uveitis (active or inactive) and controls. METHODS: Participants underwent an AC inflammation evaluation including: clinical cell and flare grading and laser flare photometry (LFP). Uveitis patients were divided into active or inactive uveitis status according to clinical grading. Anterior segment SS-OCT scans were obtained for each participant. Tomographic images were analyzed to count the AC cells, and to calculate to absolute measurements of aqueous signal intensity. The absolute values were compared with the signal measured by the scan outside the eye, generating an optical density ratio (aqueous-to-air relative intensity [ARI] index). Correlations between OCT-derived AC inflammatory indexes and LFP, clinical grading, participant category (active or inactive uveitis, control), age, gender, and central corneal thickness (CCT) were assessed. MAIN OUTCOME MEASURES: Correlation between OCT-derived AC inflammatory indexes (ARI index and AC cells on OCT) and standard clinical techniques (LFP, clinical cell grading). RESULTS: Two hundred thirty-seven eyes (70 active uveitis, 97 inactive uveitis, and 70 controls) were included. Anterior chamber cells count on OCT did not differ between inactive uveitis and controls, but was significantly higher in active uveitis compared to the other categories (both P < 0.0001). All groups had different LFP (all P < 0.0001). Active uveitis had significantly higher ARI index compared with inactive uveitis and controls (both P < 0.0001). Interobserver agreement (intraclass correlation coefficient) for ARI index was 0.78. The ARI index correlated positively with age (P = 0.043) and negatively with CCT (P = 0.006). The ARI index correlated with LFP in the active uveitis group (P < 0.0001), but not in the others. Anterior chamber cells on OCT increased among all cell clinical grades (P < 0.0001). The ARI index increased among all flare clinical grades (P < 0.005). CONCLUSIONS: Anterior segment SS-OCT could be used for a comprehensive assessment of AC inflammation, providing objective measurements of inflammatory cells and aqueous flare.

Drusen-like Deposits in Young Adults Diagnosed With Systemic Lupus Erythematosus.

PURPOSE: To determine the prevalence of drusen-like deposits (DLDs) and choroidal changes in patients with systemic lupus erythematosus (SLE), with or without glomerulonephritis; and to correlate ocular findings with systemic features. DESIGN: Case-control study. METHODS: Sixty patients with SLE (age, 18-55 years; 30 with and 30 without SLE-related glomerulonephritis) and 60 age- and sex-matched healthy controls were enrolled. All patients underwent noninvasive multimodal imaging that included fundus photography, near-infrared reflectance, blue autofluorescence, blue reflectance, and spectral-domain optical coherence tomography (SDOCT). Images were analyzed for the prevalence of DLDs. Distribution, size, and number of DLDs were measured. Correlations between ocular findings and systemic features were analyzed. Subfoveal choroidal thickness (SCT) was measured using the SDOCT. RESULTS: Drusen-like deposits were detected in 40% of SLE subjects and 3.33% of controls (P < .0001). Compared with other techniques, SDOCT detected the largest number of affected subjects. In eyes with DLDs, small, medium, and large lesions were found in 75%, 50%, and 42% of cases, respectively. Drusen-like deposits were located in the nasal, temporal, inferior, superior, and central regions of the posterior pole in 83%, 75%, 67%, 54%, and 25% of eyes, respectively. The prevalence of DLDs in patients with SLE was similar regardless of renal involvement, but patients with glomerulonephritis had more DLDs per eye, larger deposits, and DLDs in >3 quadrants (P < .001, P = .03, P = .009, respectively). Subfoveal choroidal thickness was greater in patients with SLE (P = .002). CONCLUSIONS: Drusen-like deposits in patients with SLE were independent of renal disease and were best detected with SDOCT. Lupus-related glomerulonephritis was associated with more fundus abnormalities and a screening SDOCT should be considered in all patients with SLE. Drusen-like deposits in the absence of glomerulonephritis may support the recent proposal that complement alteration is the primary cause of these lesions.

Corneal Confocal Microscopy in Dry Eye Treated with Corticosteroids.

PURPOSE: To evaluate, by in vivo laser scanning confocal microscopy (LSCM), the corneal findings in moderate-to-severe dry eye patients before and after treatment with topical corticosteroid and to associate the confocal findings to the clinical response. METHODS: Fifty eyes of 50 patients with moderate-to-severe dry eye were included in this open-label, masked study. Exclusion criteria were any systemic or ocular condition (other than dry eye) and any systemic or topical treatment (except artificial tears), ongoing or performed in the previous 3 months, with known effect on the ocular surface. All patients were treated with loteprednol etabonate ophthalmic suspension 0.5% qid for 4 weeks. Baseline and follow-up (day 30 ± 2) visits included Ocular Surface Disease Index (OSDI) questionnaire, full eye examination, and central cornea LSCM. We compared data obtained before and after treatment and looked for associations between baseline data and steroid-induced changes. Based on the previously validated OSDI Minimal Clinically Important Difference, we reanalyzed the baseline findings comparing those patients clinically improved after steroids to patients not clinically improved after steroids. RESULTS: Ocular Surface Disease Index score and LSCM dendritic cell density (DCD) significantly decreased after treatment. Baseline DCD correlated with both OSDI and DCD steroid-related changes (r = -0.44, p < 0.05 and r = -0.70, p < 0.01, respectively; Spearman) and was significantly higher in patients clinically improved after steroids than in patients not clinically improved after steroids (164.1 ± 109.2 vs. 72.4 ± 45.5 cells/mm2, p < 0.01; independent samples t test). CONCLUSIONS: Laser scanning confocal microscopy examination of DCD allows detection of treatment-related inflammation changes and shows previously unknown associations between confocal finding and symptoms improvement after treatment. These promising preliminary data suggest the need for future studies testing the predictive value of DCD for a clinical response to topical corticosteroids.

Evaluation of a novel eyelid-warming device in meibomian gland dysfunction unresponsive to traditional warm compress treatment: an in vivo confocal study.

The purpose of the study was to evaluate the efficacy and safety of wet chamber warming goggles (Blephasteam(®)) in patients with meibomian gland dysfunction (MGD) unresponsive to warm compress treatment. We consecutively enrolled 50 adult patients with low-delivery, non-cicatricial, MGD, and we instructed them to apply warm compresses twice a day for 10 min for 3 weeks and to use Blephasteam(®) (Laboratoires Thea, Clermont-Ferrand, France) twice a day for 10 min for the following 3 weeks. We considered "not-responders" to warm compress treatment the patients who showed no clinically significant Ocular Surface Disease Index (OSDI) improvement after the first 3 weeks. Clinical and in vivo confocal outcome measures were assessed in the worst eye (lower BUT) at baseline, after 3 weeks, and after 6 weeks. Eighteen/50 patients were not-responders to warm compress treatment. These patients, after 3 weeks of treatment with Blephasteam(®), showed significant improvement of OSDI score (36.4 ± 15.8 vs 20.2 ± 12.4; P < 0.05, paired samples t test), increased BUT (3.4 ± 1.6 vs 7.6 ± 2.7; P < 0.05), and decreased acinar diameter and area (98.4 ± 18.6 vs 64.5 ± 14.4 and 8,037 ± 1,411 vs 5,532 ± 1,172, respectively; P < 0.05). Neither warm compresses nor Blephasteam(®) determined adverse responses. In conclusion, eyelid warming is the mainstay of the clinical treatment of MGD and its poor results may be often due to lack of compliance and standardization. Blephasteam(®) wet chamber warming goggles are a promising alternative to classical warm compress treatment, potentially able to improve the effectiveness of the "warming approach."

The cornea in classic type Ehlers-Danlos syndrome: macro- and microstructural changes.

PURPOSE: To analyze in vivo corneal morphology and ultrastructural features in patients with classic Ehlers-Danlos syndrome (EDS). METHODS: Fifty patients with classic EDS and 50 age- and sex-matched control subjects were studied. A clinical evaluation was made with the Ocular Surface Disease Index (OSDI) questionnaire and a complete ophthalmic examination, including assessment of the best-corrected visual acuity and refraction, slit-lamp biomicroscopy, tear break-up time, intraocular pressure, Schirmer test without topical anesthesia, and corneal diameter. Scheimpflug camera topography and in vivo confocal microscopy (IVCM) were used to investigate corneal morphology and corneal ultrastructural features respectively. RESULTS: Classic EDS patients, compared to controls, had thinner and steeper corneas (P < 0.001 and P < 0.05, respectively; independent samples t-test). IVCM showed thinner stromas, lower keratocyte densities (P < 0.001), increased applanation-related stromal folds (P < 0.001; Mann-Whitney U test), and increased endothelial hyperreflective dots (P < 0.05) in these patients. The study group also had increased symptoms (OSDI score: P < 0.01, independent samples t-test) and signs (tear break-up time and Schirmer test: P < 0.001 and P < 0.05, respectively) of tear film dysfunction. CONCLUSIONS: Patients with classic EDS had macro- and microstructural changes of the cornea, which is a target tissue of the disease. These findings should be considered to optimize clinical management of these patients and to evaluate the opportunity of adding ocular findings to the classic EDS diagnostic criteria.