Blepharospasm as a tardive manifestation of COVID-19 disease: A case report.

We report three cases of blepharospasms developed after a symptomatic COVID-19 infection, in order to describe a possible association between COVID-19 infection and essential blepharospasm. Blepharospasm could represent a late sign of COVID-19 infection (more than four weeks after the contagion) and may be triggered by the neurotropism of the coronavirus.

Atypical presentation of juvenile multiple sclerosis in a patient with COVID-19.

PURPOSE: To report our experience with a case of a very atypical clinical onset of multiple sclerosis in a young boy during a COVID-19 infection. CASE REPORT: A 16-year-old boy was referred to our ophthalmology clinic with a complete isolated bilateral horizontal gaze palsy. The condition had onset suddenly 2 weeks prior and he had no associated symptoms, as well as no significant medical history. His corrected visual acuity was 0.0 logMAR in both eyes. While hospitalized, he was found infected with COVID-19. Subsequent brain MRI showed multiple lesions typical of a yet undiagnosed MS, as well as an active pontine plaque which was highly probable the cause of the horizontal gaze palsy. High-dose steroid treatment was initiated 1 week later, after the patient exhibited negative COVID-19 test results. CONCLUSION: Clinical manifestations of MS are rarely seen in male teenagers and only a few cases of isolated bilateral horizontal gaze palsy have been reported as the initial manifestation, but never during concomitant COVID-19 infection. We presume that the presence of COVID-19 may have been a neuroinflammatory trigger of underlying MS.

X-linked retinoschisis: OCT-angiography in two brothers from a four-generation family with a p.Arg197Cys pathogenic variant in the RS1 gene.

INTRODUCTION: X-linked juvenile retinoschisis (XLRS) is a rare genetic disease causing retinal splitting. The aim of this work is to describe the optical coherence tomography angiography (OCTA) features in two brothers affected by an hemizygous c.589C>T (p.Arg197Cys) pathogenic variant in exon 6 of the RS1 gene. CASE DECRIPTION: Each patient underwent a complete ophthalmological examination, including measurement of best corrected visual acuity, slit-lamp biomicroscopy, fundus color photographs, fundus autofluorescence and infrared imaging, fluorescein angiography, spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA). En Face SD-OCT and OCTA revealed the presence of two different pattern of cystic lesions, fusiform and oval, disposed on a petaloid or irregular manner in the perifoveolar area. A widening of the foveal avascular zone with interruption of the vascular arcades was clearly evident. Furthermore, a capillary drop-out was observed in the superficial plexus of the central retina, other than capillary ectasia in the deep capillary plexus. Straight gray lines were visible among the cysts. CONCLUSIONS: OCTA data herein described allow a detailed morphological evaluation of XLRS other than a quantitative assessment of retinal capillary flow in this disease. The retinal alterations that we have reported may be helpful to better understand this rare condition with OCTA being a sensitive technique to monitor the evolution of the disease and the response to potential future therapeutic approaches aimed to restore vision.

Compass Fundus-Guided Perimetry in Geographic Atrophy.

Purpose: To evaluate compass (CMP), a recently introduced device that combines scanning ophthalmoscopy, automated perimetry, and eye tracking, for fundus-guided perimetry (microperimetry) with the purpose of correlating perimetric retinal sensitivity (PRS) and retinal geographic atrophy (GA) features. Materials and Methods: A retrospective, cross-sectional study was performed in 56 eyes of 43 patients affected by GA. All patients underwent compass 10-2 perimetry, consisting of a full-threshold visual field on fundus photography and an infrared (IR) image of the central 30° of the retina. Data were exported to an Excel sheet. Binarization with black/white (B/W) variables was applied on the compass photo fundus and matched with visual field scores. Patients underwent autofluorescence (AF) and IR images (Heidelberg, Germany): CMP and Heidelberg IR images were homologated by using GIMP software (https://www.gimp.org), and then atrophic areas were manually measured with the ImageJ program. CMP perimetric grid was overlapped with AF and IR pictures by using GIMP, obtaining composite TIFF images, which were then analyzed with the ImageJ greyscale score (GSS) tool. A hyperautofluorescent halo was identified on the GA edges of some patients. Pearson's correlation between GA size on IR compass and IR Heidelberg and between GSS and PRS values has been calculated; the independent t-test was realized to calculate the correlation between GSS and B/W variables identified on the CMP photo fundus. The Spearman correlation between total deviation and pattern deviation was calculated. Results: The AUC-ROC score between CMP scores and B/W variables was 93,4%. The Spearman correlation between total deviation and pattern deviation was highly significant (p = 0,00). The correlation between AF GSS values and PRS was significant (p value = 0,00), the correlation between GSS of hyperautofluorescent points and PRS was significant (p value = 0,00), and the correlation between IR GSS and PRS was significant (p value = 0,00). The correlation between AF GSS and B/W variables was significant (p value = 0,002), the correlation between hyperautofluorescent points and B/W was not significant (p value = 0,40), and the correlation between IR GSS and B/W was significant (p = 0,00). Conclusions: Based on our preliminary results, compass seems to be a reliable, quick, and safe device for the anatomical and functional study of GA. The direct visualization of the visual field on the fundus photography as a background allows a precise assessment and clinical monitoring of this disease.

Inter-Eye Comparison of the Ocular Surface of Glaucoma Patients Receiving Surgical and Medical Treatments.

Background: Ocular surface frequently affects glaucoma patients. In this paper we aimed at evaluating the impact of glaucoma surgery on the ocular surface of patients who received unilateral trabeculectomy. Methods: 26 consecutive patients successfully treated with trabeculectomy on one eye (Trab Eye) and under control with topical treatments on the fellow eye (Med Eye) were included in this observational study. They received IDEEL and OSDI questionnaires, Tear Film Osmolarity (TFO), grading of conjunctival hyperemia, fluorescein tear break-up time (tBUT), grading of corneal staining and Schirmer test. Results: IDEEL and OSDI scores were 48 ± 38 and 11 ± 12, respectively, with moderate correlation (r = 0.50, p = 0.03). Compared with Med eyes, Trab Eyes had higher tBUT (6.5 ± 3.5 vs. 5.1 ± 2.7 s, p = 0.004), lower conjunctival hyperemia (0.8 ± 0.9 and 1.7 ± 1.1 respectively, p < 0.001) and lower corneal staining (0.3 ± 0.5 and 0.6 ± 0.5, respectively, p = 0.03). Correlation between corneal staining and conjunctival hyperemia was 0.55 in Trab Eyes (p = 0.01) and 0.44 in Med Eyes (p > 0.05). Patients with bilateral corneal staining had had threefold worse questionnaire scores (p < 0.05). The duration of treatment and the daily exposure to preservatives did not directly affect OS parameters in this cohort of patients. Conclusions: Patients receiving successful trabeculectomy showed better OS homeostasis (higher TBUT, lower grading of conjunctival hyperemia and corneal staining) than fellow medically treated eyes. Presence of corneal epithelial damage in both eyes is the factor more consistently affecting questionnaire scores.

Yield Response and Physiological Adaptation of Green Bean to Photovoltaic Greenhouses.

The cultivation of the horticultural crops inside photovoltaic greenhouses (PVG) should be studied in relation to the shading cast by the photovoltaic (PV) panels on the roof. This work evaluated the green bean cultivation inside PVGs with a percentage of the greenhouse area covered with PV panels (PV cover ratio, PV R ) ranging from 25 to 100%. Three dwarf green bean cycles (Phaseolus vulgaris L., cv. Valentino) were conducted inside an iron-plastic PVG with a PV R of 50%. The average yield was 31% lower than a conventional greenhouse. Adverse effects on quality were noticed under the PV roof, including a reduction of pod weight, size, and caliber. Negative net photosynthetic assimilation rates were observed on the plants under the PV roof, which adapted by relocating more resources to the stems, increasing the specific leaf area (SLA), leaf area ratio (LAR), and the radiation use efficiency (RUE). The fresh yield increased by 0.44% for each additional 1% of cumulated PAR. Based on the linear regressions between measured yield and cumulated PAR, a limited yield reduction of 16% was calculated inside a PVG with maximum PV R of 25%, whereas an average yield loss of 52% can occur with a PV R of 100%. The economic trade-off between energy and green bean yield can be achieved with a PV R of 10%. The same experimental approach can be used as a decision support tool to identify other crops suitable for cultivation inside PVGs and assess the agricultural sustainability of the mixed system.

The epidemiology of Moebius syndrome in Italy.

BACKGROUND: The epidemiology of Moebius syndrome (MBS) is difficult to assess. In the present study, we investigated the epidemiology of MBS in a well-defined population within a precise geographical area. MATERIALS AND METHODS: Our university hospital is the only national referral center for the diagnosis and treatment of MBS. Participants in this cross-sectional study were patients affected by MBS who had been periodically followed by our medical staff since 1998. Most of the patients were referred to our hospital by the Italian Association of Moebius Syndrome (AISMO). Demographic data necessary for study purposes were made available in the AISMO database, updated to April 2018. Subjects were assigned to geographical macroareas that are conventionally used in surveys and epidemiological investigations by the Italian National Institute of Statistics. The rates and prevalence of MBS cases were calculated on the basis of the last available survey of the Italian population. Each study parameter was then calculated with reference to the whole country and macroarea partition. The sex rate and the corresponding prevalence were calculated with respect to the weighted whole population and to the respective sex population. Chi-square analysis was adopted to investigate possible differences among geographical regions and/or sexes. A p value < 0.05 was considered statistically significant. RESULTS: One hundred and sixty-four out of 212 MBS patients fulfilled our inclusion criteria. All cases occurred in Caucasian patients and were sporadic. The median age at diagnosis was 3.6 years, ranging from 0 to 55 years; this range was significantly reduced to 0-5 years (median age at diagnosis: 2.2 years) in patients included after 2007. The calculated prevalence at birth was 0.06 cases per 10,000 live births, with an overall prevalence of 0.27/100,000, without any sex or geographical predominance. CONCLUSIONS: The prevalence of MBS observed herein, rounded for possible underestimation, was 0.3/100,000 people, without any regional difference in the distribution of cases. Our data confirm the rarity of the disease on a national level.

Optical coherence tomography angiography in multiple sclerosis: A cross-sectional study.

OBJECTIVES: To evaluate retinal axonal density and retinal capillary flow density (CFD) variations in patients affected by multiple sclerosis (MS) as documented by Optical Coherence Tomography Angiography (OCT-A). MATERIAL AND METHODS: A cross-sectional study was performed in a tertiary university eye hospital on 94 eyes from 48 MS patients compared to 37 eyes from 23 matched controls. MS patients were divided in two groups: those with previous episodes of optic neuritis (MS ON+, 71.4%) and those without any previous visual complaint (no optic neuritis group, MS ON, 28.6%). Patients underwent macular and optic nerve head OCT-A with Optovue XR Avanti (Optovue, Freemont, California) after that preliminary evaluation of the ganglion cell complex (GCC) and of the retinal nerve fiber layer (RNFL) was achieved for each single eye by SD-OCT. CFD was evaluated in three different retinal layers of MS patients and controls: superficial capillary plexus (SCP), deep capillary plexus (DCP) and the choriocapillaris layer (CL). Each layer was analyzed in 18 preset subregions automatically detected by the system. CFD values were then correlated to the RNFL thickness and GCC thickness in the groups: p values were computed by t-tests between each group of MS patients and controls. A p-value of <0.05 was considered significant. RESULTS: A significant difference in the overall CFD values was found between ON+ and ON- patients when compared to controls in 18 subregions of SCP. Furthermore, a significant difference was found between MS patients and controls in 16 subregions analyzed corresponding to the CL layer without difference between the two MS subgroups (ON+ and ON-). CONCLUSIONS: OCT-A when performed at the optic nerve head level and at the macular region is characterized by a reduction of retinal perfusion in a significant portion of MS patients independently if they had a previous history of optic nerve inflammation or not.

Dimple in vascularized serous pigment epithelial detachment secondary to neovascular age-related macular degeneration.

BACKGROUND: To describe the "dimple," a previously unreported structural optical coherence tomography (OCT) finding in vascularized serous pigment epithelial detachment (PED) secondary to neovascular age-related macular degeneration (AMD). METHODS: Retrospective, longitudinal, case series study. Clinical charts and multimodal imaging including OCT (structural and angiography) and dye-based angiography (fluorescein and indocyanine green) examinations of patients with dimple-defined as a localized invagination of the vascularized serous PED-were analyzed in 2 high-volume referral centers. RESULTS: Nineteen eyes of 18 patients were included. Mean follow-up was at 64.1 ± 35.8 months. The greater basal and height diameters of the vascularized serous PED were respectively 3425.8 ± 1049.6 µm and 667.1 ± 279.9 µm at baseline and 3076.2 ± 1649.9 µm (p = 0.8) and 368.3 ± 295.1 at last follow-up (p = 0.0006). OCT analysis identified 2 phenotypes of dimple: type 1 or ("top denting") (9 eyes) and type 2 (or "side denting") (10 eyes). Both phenotypes are associated with hyper-reflective holding sub-retinal pigment epithelium (RPE) band encompassing the posterior face of the RPE and extending to the Bruch's membrane. Hyper-reflective holding band is not correlated with angiographic signs of neovascular tissue in all cases. During follow-up, no case of RPE tear was observed. CONCLUSIONS: We describe the characteristics of the dimple and its association with hyper-reflective holding sub-RPE bands in the context of large vascularized serous PED in neovascular AMD. The presence of a dimple does not seem to be an additional risk factor for the development of RPE tearing in high-risk PED secondary to neovascular AMD.

Multimodal OCT Reflectivity Analysis of the Cystoid Spaces in Cystoid Macular Edema.

PURPOSE: To compare and evaluate images of macular cysts with different degrees of reflectivity (from gray to black signal) as observed in B scan spectral domain OCT (SDOCT) and EnFace OCT with decorrelation signal obtained with OCT-angiography (OCTA) in eyes with cystoid macular edema (CME) secondary to diabetic retinopathy (DR) and retinal vein occlusion (RVO). METHODS: Images from 3033 patients affected by CME secondary to diabetes or RVO examined OCTA (Optovue XR Avanti, Optovue, USA) at the University Eye Clinic of Créteil, Hôpital Intercommunal, France, and at the University Eye Clinic of Cagliari, "San Giovanni di Dio" Hospital, Italy, were retrospectively examined. The deep capillary plexus OCTA images and the corresponding EnFace OCT images, both acquired with the same automatic segmentation, had been overlapped to compose RGB color images as red and green channels, respectively, using ImageJ software (National Institutes of Health, Bethesda, MD). Afterward, linear regions of interest were traced on the color images to obtain the profiles of OCTA and EnFace gray values. Number of pixels, mean gray value and standard deviation of the area traced in OCT-A, and EnFace image were analyzed and statistically correlated. Data were exported to Excel to create the plots. RESULTS: 94 patients with DME and 27 patients with RVO showed intraretinal macular cystoid spaces with similar homogeneous, gray-looking content; 73 patients with DME and 113 patients with RVO showed macular cystoid spaces with homogeneous, black-looking content, as observed at SD-OCT, EnFace and OCTA scans. Interestingly, the limits of macular cystoid spaces were clearly detectable with OCTA. The analysis of red and green profiles demonstrated a clearly visible overlap between average OCTA and EnFace signal observed around cystoid spaces that could be attributed to a relationship between the dynamic vascularization and the structural density of the tissue. CONCLUSIONS: This is the first investigation that characterizes and correlates OCTA and EnFace signals on images of macular cystoid spaces in DR and RVO. The low intensity OCTA signals observed inside cystoid spaces raise a relevant question about their nature, as to whether they are due to the presence of corpusculated material pouring out from bloodocular-barrier or they should be considered OCTA artifacts.

Optical coherence tomography is a useful tool in the differentiation between true edema and pseudoedema of the optic disc.

PURPOSE: To assess the usefulness of spectral-domain optical coherence tomography (SD-OCT) peripapillary retinal nerve fiber layer (RNFL) thickness measurement in discriminating early phase optic disc edema (ODE) from pseudoedema (PODE). METHODS: Hospital-based, multicenter, cross-sectional study involving external patients referred for recent identification of "presumed ODE". Patients underwent SD-OCT optic nerve head (ONH) RNFL thickness measurement at their first evaluation. In 155 of these, the causative etiology was subsequently ascertained and the respective eyes (one per patient) were assigned to the ODE (95 eyes) or PODE (60 eyes) group. Admission SD-OCT data were retrieved and used for the analysis. ROC curve analysis was used to calculate specificity, sensitivity and predictive value (PV) of the RNFL values. RESULTS: The PODE group was significantly younger than the ODE group (p = 0.007). The average and any single-quadrant RNFL thickness was significantly higher in the ODE group compared with the PODE and control groups. The average and the inferior quadrant thicknesses tested the most powerful parameters to differentiate ODE from PODE. A cutoff value of ≥ 110 µm for the average area, or of ≥ 150 µm for the inferior quadrant was associated with maximal sensitivity and specificity with positive PV greater than 80%. CONCLUSIONS: The SD-OCT evaluation of the peripapillary RNFL achieved good specificity, sensitivity and positive PV in discriminating between ODE and PODE. Despite the correct differential diagnosis between these categories still relies on a careful medical history taking and other ancillary testing, we proved the usefulness of SD-OCT RNFL measurement in supporting the diagnostic process.

An uncommon case of intermittent Pourfour du Petit Syndrome associated with acute angle-closure glaucoma successfully treated by laser iridotomy.

PURPOSE: To describe a case of acute angle-closure glaucoma secondary to intermittent mydriasis related to Pourfour du Petit Syndrome caused by tracheal deviation. MATERIALS AND METHODS: A 70-year-old Caucasian woman visited the Emergency Room of the University Eye Clinic complaining of blurring of vision and difficulty to move superior eyelid in her right eye. Examination revealed reactive mydriasis, and upper lid retraction on the right side. The rest of the ophthalmological examination was normal, and a cranial computed tomography (CT) did not identify any abnormalities. A cervical CT showed the presence of an accentuated lateral right convex deviation of the trachea, attributable to a fibrothorax. A right Pourfour du Petit syndrome was suspected. Although the mydriasis had in the meantime vanished, the patient was admitted to the Neurological Clinic. RESULTS: Five days later she suffered acute pain in her right eye. Ophthalmological examination of the right eye revealed conjunctival hyperemia, marked corneal edema, reduced depth of anterior chamber, permanent mydriasis. As assessed by Goldmann applanation tonometry, intraocular pressure (IOP) was 48 mm Hg. Fundus examination was normal in both eyes. Gonioscopy revealed angle closure in all quadrants. Slit lamp examination of the contralateral eye was normal; IOP was 10 mm Hg. After hypotensive medical therapy, iridotomy with YAG laser was performed. Thereafter, IOP stabilized at 12 mm Hg. CONCLUSIONS: This is the first report in the literature of a case of acute angle-closure glaucoma secondary to mydriasis related to Pourfour du Petit Syndrome caused by tracheal deviation.

Centrifugal Extension of Retinal Atrophy in Retinal Pigment Epithelium Tears Secondary to Age-Related Macular Degeneration.

BACKGROUND AND OBJECTIVE: To investigate the progression of retinal atrophy in patients with retinal pigment epithelium (RPE) tears secondary to neovascular age-related macular degeneration. PATIENTS AND METHODS: In this retrospective case series, patients were analyzed at two high-volume referral centers. The extension of the areas without RPE was analyzed yearly from baseline to last examination through fundus autofluorescence (FAF) imaging using Region Finder (Heidelberg Engineering, Heidelberg, Germany). RESULTS: Sixteen eyes of 14 patients were included in the study. Mean follow-up was 70.11 months ± 15.5 months. The average area of atrophy was 6.89 mm2 ± 5.4 mm2 at baseline and 9.21 mm2 ± 7.7 mm2 at the last visit (P < .0001). This accounts for a progression of 0.36 mm2 ± 0.46 mm2/year. In all cases, FAF revealed centrifugal extension of retinal atrophy. CONCLUSIONS: In this series, the area of retinal atrophy enlarged over time. Atrophy enlargement is characterized by centrifugal extension from the base of the tear. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:705-710.].

Rebound macular edema following oral acetazolamide therapy for juvenile X-linked retinoschisis in an Italian family.

BACKGROUND: Juvenile X-linked retinoschisis (RS1, OMIM: 312700) is a hereditary vitreoretinal dystrophy characterized by bilateral foveal schisis and, in half of the patients, splitting through the nerve fiber layer in the peripheral retina. In the first decade of life, patients usually develop a decrease in visual acuity. Long-term visual outcomes can be poor due to the limited number of known successful treatments. PURPOSE: The purposes of this study were to present, for the first time, a p.Arg197Cys missense mutation in the RS1 gene (OMIM: 300839) in a four-generation Italian family with RS1 and to examine the clinical response to the treatment with acetazolamide tablets alone or in combination with dorzolamide eye drops as assessed by spectral-domain optical coherence tomography (SD-OCT). METHODS: Eleven individuals, including two brothers with RS1 (patients 1 and 2), underwent a full medical history examination and a comprehensive ocular assessment that involved SD-OCT, fluorescein angiography, electroretinography and DNA analysis. Each RS1 patient received oral acetazolamide (375 mg daily) during the first three months. Thereafter, patient 1 continued only with dorzolamide eyedrops three times a day for a period of three months, while patient 2 spontaneously stopped both medications. RESULTS: Sequence analysis of the RS1 gene identified a hemizygous c.589C>T (p.Arg197Cys) missense mutation in exon 6, which has not been previously reported in an Italian family. A different response to the medical therapy was observed in the four eyes of the two affected brothers hemizygous for this abnormality. Of note, after acetazolamide interruption, a rebound effect on cystoid macular edema reduced the beneficial effects of the initial therapy for RS1 from p.Arg197Cys mutation. Indeed, a minimal rebound effect on cystoid macular edema, and an improvement in visual acuity, was observed in patient 1 during the six months of treatment. Conversely, in patient 2, an initial improvement in cystoid macular edema was not associated with visual acuity changes, followed by a marked rebound effect. CONCLUSION: This study showed that the sequential use of acetazolamide tablets and dorzolamide eye drops should be considered and studied further as a possible treatment for macular edema and visual impairment in patients with RS1 from a hemizygous p.Arg197Cys mutation.

Simultaneous occlusion of three cilioretinal arteries following scleral buckling surgery under local anesthesia.

BACKGROUND: Cilioretinal artery (CRA) occlusions are rare in young patients. In these cases, the most commonly associated causes are considered to be the same as those implicated in central retina artery occlusions, such as vasculitic processes, migraine, cardiac disorder, and coagulation abnormality. The aim of this article was to report for the first time the medical records and investigational results of an unusual case of simultaneous occlusion of three CRAs after scleral buckling surgery under local anesthesia. METHODS: A complete ophthalmic examination, including color fundus image, fundus fluorescein angiography, optical coherence tomography, visual field, as well as systemic and laboratory assessments, was performed. RESULTS: A case of contemporaneous blockage of three CRAs after ab externo surgery for retinal detachment in a 29-year-old Caucasian woman was reported. The interdisciplinary approach and the imaging results have allowed us the clinical definition of such a very rare case. CONCLUSION: Here, we reported that optical coherence tomography is an indispensable tool to better delineate the pathological process and follow atrophic changes in the macula, especially in cases in which fundus fluorescein angiography and systemic tests may be poorly informative.