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Background: The tumor invasion of the skull base structures is very frequent in spheno-orbital meningiomas. The aim of the present study is to evaluate the invasion rate of skull base structures and the best surgical approach and management. Methods: The surgical series of 80 spheno-orbital meningiomas was reviewed. The tumors were classified according to the intraorbital location with respect to the optic nerve axes into three types: I-lateral: II-medial; III-diffuse. The invasion of the orbital apex, optic canal, superior orbital fissure, anterior clinoid, ethmoid-sphenoid sinuses, and infratemporal fossa was evaluated. The rate and extension of involvement of these structures was correlated with the intraorbital location and the surgical approach. The preoperative ophtalmological symptoms and signs and their outcome were also evaluated. Results: Proptosis was found in 79 patients (97%), variable decrease of the visual function in 47 patients (59%), and deficits of the eye movements in 28(35%). The invasion of the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%) was more frequently found, whereas the tumor extension into the ethmoid-sphenoid sinuses (4%) and infratemporal fossa (4%) was rare. Types II and III meningiomas showed significantly higher involvement of the skull base structures than type I ones, which only had 15% invasion of the optic canal. Remission or significant improvement of the visual function occurred postoperatively in 24 among 47 cases (51%), with a higher rate for type I meningiomas vs. other types (p = 0.021, p = 0.019) and worsening in 7 (15%). Conclusions: Spheno-orbital meningiomas growing in the lateral orbital compartment show no involvement of the skull base structures excepting the optic canal as compared to those growing medially or diffusely. The surgical resection of tumor invading the skull base structures should be more extensive as possible, but the risk of optic and oculomotor deficits must be avoided.

Background: To investigate the effects of a fixed combination of Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) on vascular parameters in mild to moderate diabetic macular edema (DME). Design: Prospective, case-control study. Methods: Fifty-six patients affected by diabetes mellitus type II were enrolled in the study. Twenty-eight patients with DME received 2 tablets/day, before meals of a dietary complementary supplement containing in fixed combination Curcumin (200 mg), Artemisia (80 mg), Bromelain (80 mg), and Black pepper (2 mg) (Intravit®, OFFHEALTH Spa, Firenze, Italy) for 6 months. Twenty-eight age-matched subjects affected by diabetes mellitus type II were given placebo and served as control group. Patients underwent best correct visual acuity (BCVA), swept optical coherence tomography (OCT), and OCT-Angiography (OCTA). OCTA images of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were obtained for each eye. By the end of the follow-up patients were defined responder to the therapy when a decrease of more than 30 μm was registered in central retinal thickness (CRT) measurement, while a poor responder was determined by the absence of reduction or an increase in central retinal thickness at 6 months. We assessed the foveal avascular zone (FAZ) area, vessel density and quantified the number of microaneurysms in each layer. Results: A significant improvement of BCVA and CRT reduction was recorded at 6 months follow-up in the dietary complementary supplementation group compared to control (respectively p = 0.028 and p = 0.0003). VD of the total capillary plexus, microaneurysms count, glycaemia and HbA1c did not vary over the follow-up period between groups. Within the Intravit® group, poor responders tended to show a larger FAZ area, more microaneurysms, and a lower VD in the DCP compared to the good responders group (p < 0.0001). Conclusions: A fixed combination of Curcumin, Artemisia, Bromelain, and Black pepper oral administration may have a positive impact on central retinal thickness, visual acuity, and VD of the DCP in compensated type 2 diabetic patients with mild DME.

BACKGROUND Tumors involving the sellar area often cause visual disorders due to optic nerves/chiasm compression, so that surgery is required. However the likelihood of visual improvement/restoration is variable. Optical coherence tomography (OCT) is a valid diagnostic tool for defining pathological conditions of the optic apparatus. We herein investigated the role of the SD-OCT and the best corrected visual acuity in predicting postoperative visual recovery, in patients complaining of chiasm compression due to sellar-suprasellar lesions. MATERIALS AND METHODS We retrospectively analyzed 20 patients (7 females and 13 males; mean age 50,8 ± 17,87 years, range 11-83), with optic chiasm/nerve compression that underwent endoscopic endonasal approach at the Division of Neurosurgery of the Universita degli Studi di Napoli "Federico II", Naples - Italy, between June 2014 and February 2015. Pre-operative ophthalmological assessment focused on BCVA Snellen charts, Standard Automated Perimetry test and SD-OCT. Test were repeated at two weeks, one and three months after surgery. RESULTS A significant thickness reduction in all the patients, as compared to the control group was noted: GCC thickness reduction remained mostly unchanged postoperatively. 42.9% of the patients had altered visual acuity (VA), while the Perimetry revealed 90% of altered preoperative MD values (MD> -2.00): perimetry improvement was noted in 93% of cases and visual acuity in 91.3% of cases. A direct correlation between preoperative retinal status and functional recovery was identified considering the values of pRNFL and GCC. CONCLUSIONS OCT provides valuable information in regards to the entity of compression also in patients without any visual field defects. Although several factors should be taken into consideration, we retain that preoperative RNFL and GCC thickness values can be claimed as a reliable predictors of visual recovery in those patients presenting chiasm compression.

PurposeA hypercoagulable state has been reported to cause potential sight-threatening ischemia in patients suffering from Coronavirus disease 2019 (COVID-19). This study aimed to determine whether vessel density (VD), as measured by optical coherence tomography angiography (OCT-A), has insights into retinal and choriocapillaris vascular changes in patients affected by SARS-CoV-2 infection.MethodsHundred and fifty two patients positive for SARS-CoV-2 infection were enrolled in this observational, retrospective, controlled study. A control group of 60 healthy subjects was selected for statistical comparisons. Raw OCT and OCT-A data were exported and 3D datasets were analyzed to determine VD.ResultsHundred and forty eyes (92.1%) were included for final analysis. The VD of the superficial capillary plexus (SCP) did not differ between the two groups. The mean VD of the deep capillary plexus (DCP) and the choriocapillaris (CC) was significantly lower in the foveal sector of the COVID-19 group compared to healthy controls. Within the post-COVID-19 group, the lowest DCP and CC foveal VD values were recorded in patients treated with antiviral therapy; no differences were observed among COVID-19 patients with other comorbidities (hypertension, diabetes, thyroid disease) or taking antiplatelet therapy. DCP and CC foveal VD were significantly lower in patients hospitalized in an intensive care unit (ICU) than asymptomatic patients.ConclusionFoveal vessel density at the level of DCP and CC was reduced in post-COVID-19 patients. Further studies evaluating these changes over time will be needed to corroborate the hypothesis of a microvascular retinal impairment in individuals who have recently recovered from SARS-CoV-2 infection.

Purpose Bietti crystalline dystrophy (BCD) is a rare autosomal recessive retinal dystrophy caused by pathogenic variants of CYP4V2 gene and characterized by shiny yellow deposits in the retina and progressive atrophy of the retinal pigment epithelium (RPE) and choriocapillaris. The main aim of the present study is to describe the optical coherence tomography angiography (OCTA) characteristics of a patient affected by BCD. Methods A 59-years-old female with genetically confirmed BCD underwent an ophthalmological examination complete of OCTA performed in the atrophic retina, the junctional zone and the apparently normal retina. The area of choriocapillaris (CC) atrophy was compared to the area of RPE atrophy on fundus autofluorescence (FAF) imaging. Results A severe vessel density (VD) deficit at the level of superficial and deep capillary plexa as well as CC was registered in atrophic areas, which resulted deeper with respect to the junctional area, whereas the apparently preserved retina revealed VD values similar to that of control eyes. The area of RPE atrophy on FAF was larger (55.90 mm2 in right eye and 48.76 mm2 in left eye) than the area of CC atrophy on OCTA imaging (51.86 mm2 and 42.44 mm2 respectively in right and left eye). Conclusions Our findings suggest that VD impairment of retinal plexa and CC follows the degeneration of RPE as demonstrated by the greater size of the area of RPE atrophy compared to CC atrophy. Further investigations based on OCTA imaging are necessary to enhance our knowledge of this rare disease.

Prostaglandin analogues (PGAs), including bimatoprost (BIM), are generally the first-line therapy for glaucoma due to their greater efficacy, safety, and convenience of use. Commercial solutions of preservative-free BIM (BIM 0.03% and 0.01%) are already available, although their topical application may result in ocular discomfort. This study aimed to evaluate the in vitro effects of preservative-free BIM 0.03% vs. 0.01% in the human conjunctival epithelial (HCE) cell line. Our results showed that long-term exposure to BIM 0.03% ensues a significant decrease in cell proliferation and viability. Furthermore, these events were associated with cell cycle arrest, apoptosis, and alterations of ΔΨm. BIM 0.01% does not exhibit cytotoxicity, and no negative influence on conjunctival cell growth and viability or mitochondrial activity has been observed. Short-time exposure also demonstrates the ability of BIM 0.03% to trigger reactive oxygen species (ROS) production and mitochondrial hyperpolarisation. An in silico drug network interaction was also performed to explore known and predicted interactions of BIM with proteins potentially involved in mitochondrial membrane potential dissipation. Our findings overall strongly reveal better cellular tolerability of BIM 0.01% vs. BIM 0.03% in HCE cells.

PURPOSE: The aim of this study was to report management and outcomes of patients in which conjunctival carcinomas arose in the anophthalmic socket. METHODS: The authors retrospectively analyzed the data of patients which presented the anophthalmic socket after surgery. The clinical records of 4 patients with histological diagnosis of conjunctival carcinoma in the anophthalmic socket, referred to our Department, between January 2014 and December 2019 was collected. RESULTS: The study included 4 men (median age 58 years). A previous enucleation surgery had been performed in all patients due to previous ocular trauma for three patients (Case 1, 3, and 4) and due to a retinoblastoma for 1 patient (Case 2). All patients underwent surgical excision of the lesion and only 1 patient (case 3) underwent orbital exenteration due to the high-grade of the cancer. The most common tumor was the squamous cell carcinoma (2 cases) followed by 1 case of high-grade mucoepidermoid carcinoma and 1 case of carcinoma in situ. Systemic work-up was performed for all the patients. Conjunctival TNM, according to the American Joint Committee on Cancer TNM staging system was used to stage the lesions. The median follow-up time was 15 months. CONCLUSION: Considering the severity and rarity of these tumors, a careful ophthalmological examination of the cavity and a correct revision of the prosthesis should be mandatory in order to ensure the elimination of any macro and microscopic irregularities that can then in the long-term cause chronic inflammation of the tissues and consequently promote the growth of carcinomas.

Purpose The purpose of this study was to investigate the changes in structural spectral-domain optical coherence tomography (SD-OCT), OCT Angiography (OCTA) parameters, and visual acuity, 1 year after endoscopic endonasal approach for the removal of an intra-suprasellar pituitary adenoma compressing optic chiasm and compare outcomes with 48 hours postoperative data. Methods Sixteen eyes of eight patients (4 males, 4 females, mean age 52 ± 11 years) were enrolled in this prospective study. The primary outcome was to evaluate the changes over time before and after surgery, analyzing the Best Corrected Visual Acuity (BCVA), Ganglion Cell Complex (GCC), Retinal Nerve Fiber Layer (RNFL) thicknesses, the retinal vessel density (VD) of Superficial Capillary Plexus (SCP), Deep Capillary Plexus (DCP), Radial Peripapillary Capillary (RPC) and the Foveal Avascular Zone (FAZ). The secondary outcome was to identify potential biomarkers that could predict visual acuity changes after 1-year follow-up. Results When comparing SD-OCT and OCTA measurements obtained after 1 year with those observed 48 hours after surgery, GCC and RNFL were significantly improved. After a significant reduction at 48 hours, GCC thickness showed a significant increase at 1 year after surgery (p = 0.007), while a significant restoration of RNFL thickness was found at 1 year (p = 0.005), as well as the VD of SCP, DCP, and RPC values. FAZ area did not change over time. BCVA significantly improved at each time after surgery (p = 0.037, p = 0.013). A statistically significant correlation was found between the preoperative BCVA, VD of SCP, DCP, RPC, and the postoperative BCVA at 1 year (p = 0.017, p = 0.029, p = 0.031, p = 0.023). Conclusion SD-OCT and OCTA provide helpful information to identify the retinal structural and vascular improvements 1 year after surgery. OCTA parameters could serve as potential predictive markers for visual acuity recovery at long-term follow-up.

In this cross-sectional study we assessed the vascular alterations in retinal and choriocapillaris perfusion in patients affected by β-thalassemia, by means of optical coherence tomography angiography (OCTA). A total of 124 eyes of 62 patients (mean age 44.74 ± 5.79 years old) affected by β-thalassemia (transfusion dependent thalassemia (TDT), non-transfusion dependent thalassemia (NTDT) and minor) were compared to 40 eyes of twenty healthy subjects. We evaluated the vessel density (VD) in superficial capillary plexus, deep capillary plexus, radial peripapillary capillary, choriocapillaris and the foveal avascular zone area. The TDT group showed a statistically significant reduction in retinal and choriocapillaris VD respect to controls and the other groups (p &lt, 0.05). No statistically significant difference was found in OCTA parameters between β-thalassemia minor and controls. The NTDT group showed a significant reduction in VD in deep capillary plexus respect to controls and β-thalassemia minor. Significant negative correlations were shown in TDT group between foveal avascular zone and hemoglobin (r = −0.437, p = 0.044) and between ferritin levels and VD of choriocapillaris (r = −0.431, p = 0.038). The OCTA parameters provided a deeper understanding on retinal and choriocapillaris vascular impairment affected by tissue hypoxia levels and the oxidative stress in different clinical phenotypes of the β-thalassemia.

Purpose Kaposi's sarcoma (KS) is a rare multi-centric vascular neoplasm, first described by Moritz Kaposi in 1872. It can appear in four different forms: classic, endemic, HIV-related and post-transplant form. We present an uncommon case of seronegative HIV infection patient with skin KS involving conjunctiva of both eyes. Observations Firstly, right eye lesions were surgically removed. Subsequently, the patient was administered with systemic chemotherapy (doxorubicin) to treat both skin and left eye lesions. No signs of recurrence were observed at 20 months’ follow-up. Conclusions Both eyes involvement in KS is rarely described in scientific literature. Both surgery and chemotherapy could be considered valid treatment options for conjunctival KS.

AIMS: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions. METHODS: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy. The results of orbital ultrasonography were compared with the final histological diagnosis. RESULTS: Orbital lesions were localized by ultrasonography in 133/138 cases (96.4%); the false-negative rate of orbital echography was 3.6% (5/138). The nature of the orbital lesions was correctly determined by ultrasonography in 54.3% of the cases (75/138) preoperatively (true positives). In 58/138 (42%) patients, there was no correspondence between the ultrasound diagnosis and the histological diagnosis (false positives). The sensitivity of orbital ultrasonography for the detection of an orbital mass was 93.75% (CI 87.87–99.63%), while the specificity yielded no meaningful result (CI 0.00–60.24%). Moreover, the specificity of orbital ultrasonography to identify a malignant tumor falls within a CI of 0–62.72%. CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive in displaying an orbital mass; however, the specificity in the differential diagnosis of orbital lesions is not meaningful, particularly if malignancy is suspected. The assessment of orbital diseases requires multiple diagnostic approaches to balance the strengths and weaknesses of each method.

PurposeTo evaluate the retinal vessel density (VD) in the macular region and the foveal avascular zone (FAZ) area using optical coherence tomography angiography (OCTA) in patients with and without disorganization of retinal inner layers (DRILs) after resolution of diabetic macular edema.MethodsThirty-seven eyes of 37 DRIL patients (mean age 63 ± 13.97 years), 30 eyes of 30 no DRIL patients and 35 eyes of 35 controls were enrolled in this study. We evaluated the VD in the macular region of superficial capillary plexus (SCP), deep capillary plexus (DCP) and FAZ area.ResultsDRIL and no DRIL groups showed decreased VD in SCP and DCP (pConclusionOCTA highlights the role of retinal vascular ischemia in the pathogenesis of DRILs. This parameter could represent an important functional predictive factor in diabetic patients.

Purpose: Two new cases of primary cutaneous CD30+ anaplastic large-cell lymphoma (cALCL) of the eyelid are reported; these are analysed alongside existing cases to identify challenges relating to the diagnosis and management of such rare lesions. Material and Methods: A review of existing literature on the PubMed database is conducted using the keywords: ‘eyelid lymphoid proliferations’, ‘lymphoma of the eyelid’, and ‘primary cutaneous CD30+, ALK-anaplastic large-cell lymphoma of the eyelid’. Two new cases of cALCL are reported. Cases where patients present solely with a nodular periocular lesion are analysed for recurrence and survival rate. Results: Two new patients with a painless ulcerated nodule on the upper eyelid receive a confirmed diagnosis of cALCL after undergoing an excisional biopsy. The first, elderly patient has spontaneous remission; the second patient, with a concomitant chronic infection of hepatitis C virus (HCV), presents a more diffuse disease at the onset and requires radiotherapy. Together with 13 patients a primary cALCL identified from 11 previous studies, this constitutes a cohort of 15 patients. Of these, 10 present with an exclusively nodular lesion of the eyelid and four experience disease recurrence; no deaths from cALCL are reported. Conclusion: Differential diagnosis between primary cALCL and lymphomatoid papulosis is essential and requires careful consideration of clinical and pathologic features. Radiologic staging examination is crucial in order to exclude systemic ALCL, particularly for patients with comorbidity. Though cALCL has the pathological features of a malignant lesion, the prognosis seems favourable for patients; a relatively high percentage even experience spontaneous resolution.

Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. Results: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8&ndash, 32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6&ndash, 83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 (p = 0.0112) and 9.239 (p = 0.0094), respectively. Conclusions: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

Purpose: To report a case of quiescent choroidal neovascularization (CNV) associated with choroidal nevus using optical coherence tomography angiography (OCTA) during 5 years follow-up. Methods: Observational case report. Results: A 53-year old woman was referred our department with diagnosis of choroidal nevus. At 1 year follow-up, fundus autofluorescence revealed hyper/hypoautofluorescent area, infrared image showed hyperreflective area and enhanced depth imaging OCT presented a shallow pigment epithelium detachment without exudation or hemorrhage. Fluorescein angiography and indocyanine green angiography suspected the presence of CNV. Finally, OCTA confirmed a quiescent CNV with an increased flow area of the vascular lesion without any changes in best-corrected visual acuity. At 20 months and 5 years follow-up, the CNV remained quiescent showing no clinical activity on structural SD-OCT and no changes in CNV morphology on OCTA. Conclusion: This case shows the features of quiescent CNV secondary to choroidal nevus after long-term follow-up. OCTA represents a valid imaging technique that allows to identify the CNV and to monitor its progression over time.

Background: Multi-parametric MRI used for preoperative assessment of orbital lesions does not routinely include DCE-MRI, since its accuracy in differential diagnosis of orbital mass is still under debate. Aim of this study is to characterize orbital lesions by multi-parametric MRI, analysing the incremental predictive value of DCE-MRI in differential diagnosis of orbital lesions. Methods: In this prospective triple-blind study, 43 consecutive patients with unilateral orbital lesion underwent conventional multimodal MRI and DCE-MRI before biopsy in a tertiary referral centre. Pre-operative MRI examination including conventional unenhanced MRI protocol, DWI with ADC maps, static CE 3D-T1 w and dynamic CE T1 w sequences, was performed within 1 week from surgery (anterior/lateral orbitotomy depending on location of the lesion, to carry out incisional/excisional biopsy). Results: Comparison between conventional T1 w/T2 w, DWI, CE 3D-T1 w and DCE-MRI groups showed a statistically significant difference in scores distribution (p

Objective To assess the presence of retinal vascular network abnormalities in amnestic mild cognitive impairment (aMCI) patients and healthy subjects (HS) through optical coherence tomography angiography (OCTA). Methods OCTA and SD-OCT were performed in aMCI patients and cognitive normal HS. A complete neuropsychological evaluation was performed. Differences in vessel density (VD) in each retinal vascular plexus and in foveal avascular zone (FAZ) were evaluated with linear mixed model after correction for age, sex and disease duration. Results Twenty-seven aMCI patients (10 Single domain aMCI, 17 Multidomain aMCI) and 29 HS were enrolled. aMCI patients showed a statistically significant reduced VD in superficial capillary plexus (SCP), deep capillary plexus (DCP) and an increased FAZ compared to controls. When aMCI patients were divided in single domain (SD) and multiple domains (MD) aMCI, SD aMCI showed no VD differences in SCP, DCP and Radial Peripapillary Capillary, while the FAZ area was significantly larger compared to controls. In MD aMCI, VD values were lower and FAZ was increased compared to controls. Comparing both aMCI groups, MD aMCI showed a significant reduction in VD values of SCP. No correlation was found between mini mental state examination (MMSE) scores and OCTA parameters. Conclusions OCTA is able to detect changes in retinal microvascular network in early cognitive deficits and, the most sensitive alteration seems to be the enlargement of the FAZ. This non-invasive tool provides useful information on retinal involvement patterns in MCI diagnosis and follow up. Vascular network impairment seems to be related to the number of domains affected and not to MMSE.

In response to COVID-19 pandemic the European Society of Ophthalmic Plastic and Reconstructive Surgery (ESOPRS) recommends to avoid any treatment unless urgent or emergent care, in order to reduce ...

Objective Hepatitis C virus (HCV) has been proposed as a possible etiologic factor in ocular adnexal marginal zone lymphoma (OAML). We aimed to assess the prevalence of HCV infection in patients with OAML through a systematic review and meta-analysis. Methods Electronic databases were searched from their inception to August 2019 for studies assessing HCV seroprevalence in patients with OAML. Pooled prevalence of HCV infection was calculated with 95 % confidence interval (CI). Statistical heterogeneity among studies was quantified via the inconsistency index (I2). Funnel plot symmetry was used to assess the risk of bias across studies. Results Nine studies with 360 patients were included. Overall pooled prevalence of HCV in OAML was 12.7 %, with low statistical heterogeneity (I2 = 17.4 %) and with asymmetrical funnel plot. The studies clustered into two groups: 5 studies (3 from Italy and 2 multicenter with a major Italian contribution) showed a higher HCV prevalence in OAML (15.6 %), while the other 4 (from countries other than Italy) showed a lower prevalence (4.7 %); in both subgroups, statistical heterogeneity was null (I2 = 0%) and funnel plot was symmetrical. Conclusion HCV might be a significant etiologic factor of OAML in Italy.

PurposeThe aim of the present study is to analyze the changes in retinal vessel density (VD), using Optical Coherence Tomography Angiography (OCT-A), in patients that received endoscopic endonasal approach for the removal of an intra-suprasellar pituitary adenoma compressing optic chiasm.MethodsWe evaluated the VD in Superficial Capillary Plexus (SCP), Deep Capillary Plexus (DCP), Radial Peripapillary Capillary (RPC) and the Foveal Avascular Zone (FAZ) area in a series of fourteen patients (7 males, 7 females, mean age 56 ± 13 years), as compared to healthy controls. We also detected the structural Spectral Domain (SD)-OCT parameters: Ganglion Cell Complex (GCC), Retinal Nerve Fiber Layer (RNFL), visual field parameters (Mean Deviation, Pattern Standard Deviation) and Best Corrected Visual Acuity (BCVA). These measurements were performed prior than surgery and 48 hours after.ResultsThe patients showed a significant decrease in VD of the macular and papillary regions, a significant increase in FAZ area, a significant impairment in SD-OCT, VF parameters and BCVA respect to 14 eyes of 14 healthy controls (pConclusionOCT-A allows to detect the early changes occurring within 48 hours after surgery showing that the improvement in retinal vessel density could occur before the recovery of the structural OCT parameters and can be a positive predictive factor for the functional recovery.

BACKGROUND This study examined whether eye-sparing surgery is associated with better or worse outcomes than exenteration for the treatment of lacrimal gland carcinomas. METHODS Forty-six patients treated for lacrimal gland carcinoma were retrospectively reviewed and compared. A statistical analysis was performed using Kaplan-Meier plots. RESULTS The overall survival rates for eye-sparing surgery were 52% and 37% at 5 and 10 years, and those for exenteration were 37% and 25% at 5 and 10 years, respectively (P = .73). The proportion of patients with local regional control at both 5 and 10 years after eye-sparing surgery was 0.75, and that for exenteration was 0.47 (P = .30). For eye-sparing surgery, the proportions of distant metastasis-free survival at 5 and 10 years were 0.51 and 0.39 for eye-sparing surgery and 0.29 and 0.14 for exenteration (P = .50). CONCLUSION Because the outcomes were not significantly different, the authors suggest that eye-sparing surgery can be proposed as a reasonable approach for lacrimal gland carcinomas in appropriately selected patients.

Background: Corneal collagen cross-linking (CXL) has become the gold standard for mild and moderate stages to stop the progression of keratoconus. We analyzed some corneal topography indices to compare iontophoresis epi-on and iontophoresis epi-off techniques throughout a two-year follow-up. Methods: A total of 64 eyes of 49 patients who underwent CXL were recruited. In 30 eyes the epi-off technique was performed, whereas the remaining 34 eyes were treated with the epi-on technique. All patients underwent a complete ophthalmologic examination that included CDVA, central and thinnest corneal thickness, Schirmer test I, TBUT test, and the Ocular Surface Disease Index. Results: In both groups, a significant improvement in visual function was recorded. No statistically significant differences between Kmax, Mean K, Flat K, Steep K values were found. Statistically significant differences (p < 0.05) between the epi-on and epi-off groups’ pachymetry before and after 24 months follow-up as well as between the epi-on and epi-off groups’ topographically thinnest point in the immediate post-surgery and 24 months after surgery were recorded. Conclusion: Our study highlighted that both techniques are valid in mid-term corneal stabilization. The advantage of the new iontophoresis epi-off cross-linking technique could be found in a faster imbibing time of the cornea, therefore reducing surgical times, with a lower risk of complications.

Background Inflammatory myofibroblastic tumor (IMT) is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma, which typically occurs in children or young adults. The IMT is considered generally a benign lesion, although about 20% of cases may experience recurrence, and most rarely develop metastasis. Herein, we present the largest series of primary orbital IMT ever reported. Patients and methods The clinical records of 25 patients, collected between the 1995 and 2015, with biopsy-proven diagnosis of orbital IMT were retrospectively reviewed to determine demographic, clinical, radiologic and pathological features, management, and outcome. Results The study included 13 females and 12 male patients, age ranged from 5 to 76 years. Disease onset was in all cases unilateral (25/25), with posterior location (10/25) or extending anterior to posterior (7/25). The most common signs and symptoms were: proptosis (19/25), ptosis (18/25), diplopia (10/25), periocular swelling (9/25), pain (8/25), redness (7/25). All patients underwent to incisional biopsy which included total or subtotal tumor resection avoiding arming of the adjacent structure, followed by systemic steroid therapy (22/25) or radiotherapy (3/25). The disease recurred in 6 (24%) patients who responded to the subsequent therapy. No one developed metastasis or died because of the disease. Conclusion IMT is a distinct entity which may occur in the orbit primarily. It should be considered in differential diagnosis in all orbital masses, particularly with onset of acute or subcronic inflammation. Surgical biopsy associated to a partial debulcking of the tumor, avoiding to damage adjacent vital structure may contribute to improve the outcome. Steroid therapy, seems to be the suitable as first line medical therapy, although, as reported in literature, not all cases respond to this treatment regimen. Radiotherapy, may be considered as an alternative therapy. Recurrences occurred in 24% of patients and may be treated with additional surgical resection and a new course of steroid or radiotherapy. No specific pathological features which may correlate with the prognosis have been found in this series.

Background: Fabry disease (FD) is a X-linked recessive lysosomal storage disorder characterized by altered biodegradation of glycosphingolipids. It is a multisystem pathology, also involving ophthalmological systems that show modifications of the vessel wall due to glycosphingolipid deposits. Optical coherence tomography angiography (OCT-A) allows for an objective analysis of retinal microvasculature alterations, evaluating retinal vessel density in macular region. Methods: A total of 54 FD patients (34 females, 20 males, mean age 44.1 &plusmn, 15.6 years) and 70 controls (36 females, 34 males, mean age 42.3 &plusmn, 15.6 years) were included in this study. We evaluated vessel density in different macular areas (whole image, fovea, and parafovea) of both the superficial capillary plexus (SCP) and of the deep capillary plexus (DCP). Results: In the SCP there was a significantly lower vascular density in patients compared with controls in whole image (49.95 &plusmn, 5.17% vs. 51.99 &plusmn, 2.52%, p &lt, 0.001), parafovea (52.01 &plusmn, 6.69% vs. 54.30 &plusmn, 2.61%, p = 0.002), and fovea (22.38 &plusmn, 9.01% vs. 29.31 &plusmn, 5.84%, 0.0001). In the DCP the vessel density was statistically increased in each macular area in patients compared with controls (54.82 &plusmn, 8.07% vs. 50.93 &plusmn, 5.46%, p = 0.005, 57.76 &plusmn, 7.26% vs. 53.59 &plusmn, p = 0.0001, and 39.75 &plusmn, 8.59% vs. 34.43 &plusmn, 8.68%, 0.0001 for whole image, parafovea, and fovea, respectively). Conclusion: OCT-A analysis showed that the macular vessel density was significantly reduced in the SCP and increased in the DCP in FD patients compared with controls. These findings, which might be a consequence of the alteration of vascular wall occurring in FD, support the hypothesis that the evaluation of early retinal microvascular network changes could be a useful tool in the clinical evaluation of the disease.

Merkel cell carcinoma (MCC) is one of the rarest eyelid tumors, with high mortality rate due to lymphatic and metastatic spread. We hereby report six cases of patients with histological diag- nosis of MCC referred to our Orbit Unit between 2012 and 2018, focusing on diagnosis, treatment, and subsequent follow up. All patients underwent surgical excision and systemic work-up. Both MCC TNM and eyelid MCC TNM were used to stage lesions. MCC of the eyelid is usually misdiagnosed as benign or other malignant lesions. A prompt examination and a wide local excision are mandatory. A close follow-up of these patients is advised due to high recurrence rate and lymphatic spread

Blau syndrome (BS) is a rare granulomatous auto-inflammatory disease, characterized by the classic clinical triad of joints, skin and ocular involvements. Ocular manifestation usually consists in a bilateral insidious chronic anterior uveitis with a potential evolution to panuveitis. We describe the case of two siblings, an 8-years old female and a 5-years old male, with a diagnosis of BS, evaluated by Anterior Segment-Optical Coherence Tomography (AS-OCT). In the female patient, slit-lamp examination revealed bilateral anterior granulomatous uveitis and inflammatory sequelae. AS-OCT revealed high intensity reflective layers in the anterior cornea, hyperreflective dots both in the aqueous humor and in the posterior corneal surface. In the male, no signs of inflammation were detected both on slit-lamp examination and AS-OCT scans. AS-OCT is a valuable, non-invasive tool that could improve the diagnosis of ocular involvement, better characterize and follow-up corneal alterations and anterior segment features in pediatric patients with BS.

Background: To perform a quantitative analysis of retinal and choriocapillaris vessel density (VD) in patients with central serous chorioretinopathy (CSC) after low-fluence verteporfin photodynamic therapy (vPDT), using Optical Coherence Tomography Angiography (OCTA). Methods: A total of 28 eyes of 27 patients with CSC (21 females, 6 males, mean age 47 ± 11 years) were included in this retrospective study. At baseline and after 6 months after vPDT, we evaluated VD of the superficial capillary plexus (SCP), the deep capillary plexus (DCP) and the choriocapillaris (CC) in different macular areas (whole image, parafovea and fovea). We also analyzed the central foveal thickness (CFT) and subfoveal choroidal thickness (SFCT) with Enhanced Depth Imaging-Optical Coherence Tomography (EDI−OCT). Results: Eighteen eyes and ten eyes were responders and non responders to low-fluence vPDT, respectively. The responders group showed a significant increase in VD in DCP and CC after treatment (p < 0.05). In the non responders group the VD in SCP, DCP and CC did not differ before and after treatment. We also found a significant correlation in responders group between Best-Corrected Visual Acuity (BCVA) and CFT (r = 0.566; p = 0.014) and between BCVA and the increased VD of CC (r= -0.559; p = 0.016). In non responders group, the correlation between OCT, OCTA parameters and BCVA was not statistically significant. Conclusions: OCTA allowed us to enhance our knowledge regarding the pathophysiology of vascular changes in retinal and CC networks after low-fluence vPDT. OCTA may represent a new biomarker to evaluate the efficacy of low-fluence vPDT in the treatment of CSC.

Background: To evaluate retinal and choriocapillaris (CC) vessel density in macular region in patients affected by adult-onset foveomacular vitelliform dystrophy (AOFVD) using optical coherence tomography angiography (OCTA) Methods: A total forty-four right eyes of 44 AOFVD patients (20 females, 24 males, mean age 69.17 ± 11.57 years) divided in 3 stages (vitelliform, pseudohypopyon and vitelliruptive) and 60 normal right eyes of 60 controls (20 females, 40 males, mean age 66.04 ± 6.40 years) were included in this prospective study. We evaluated the vessel density of superficial capillary plexus (SCP), deep capillary plexus (DCP) and CC in different macular areas (whole image, parafovea and fovea). We also analyzed the subfoveal choroidal thickness (SFCT) with Enhanced Depth Image (EDI)-OCT. Results: The vessel density of SCP and of DCP did not differ between patients and controls in all macular sectors. The vessel density of CC was lower in patients compared to controls but the difference turned out to be statistically significant only in foveal region (p < 0.001). We found that the foveal vessel density of the CC was lower in vitelliform stage and significantly increased in vitelliruptive stage (p = 0.031). At EDI-OCT, the SFCT revealed a statistically significant increase in patients compared to controls (p = 0.002) whereas it was similar in the different stages of this dystrophy (p = 0.276). Conclusions: In vitelliform stage of AOFVD, OCTA and EDI-OCT can be useful to avoid mistakes of evaluation, due to the masking effect artifact. OCTA provides us a better understanding of the vascular role in the physiopathology of the macular diseases.

BACKGROUND AND PURPOSE Vascular pathology is increasingly acknowledged as a risk factor for multiple sclerosis (MS). Vascular density (VD) is reduced in the eyes of patients with MS on optical coherence tomography (OCT) angiography. We performed a 1-year prospective study to estimate VD variations over time and possible clinical correlates. METHODS A total of 50 patients with MS underwent spectral domain-OCT and OCT angiography at baseline and after 1-year follow-up. Mixed-effect linear regression models were used to assess variations of each OCT measure and its relation to treatment and clinical outcomes. RESULTS We observed an increase in parafovea VD (coefficient, 1.147; 95% confidence interval, 0.081-2.214; P = 0.035). Reduction in parafovea VD was associated with increase in Expanded Disability Status Scale score (coefficient, -0.969; 95% confidence interval, -1.732/-0.207; P = 0.013). CONCLUSIONS Retinal VD can improve over time in MS, particularly in patients experiencing disease stability. Longer follow-up, inclusion of early MS cases and combination with conventional markers of MS severity (i.e. brain atrophy) are needed to better define VD as a potential new biomarker.

Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, systemic, autoimmune subepidermal inflammatory disease that affects mucous membranes and the eye. In its most severe forms, this disease needs systemic therapy, usually based on steroids and immunosuppressant agents. In unresponsive cases or in the presence of contraindications or severe side effects due to conventional systemic corticosteroid and/or immunosuppressant therapy, a therapy shift to high-dose intravenous immunoglobulins (IVIg) has been recommended in other reports. This new therapy has proven to be effective in stopping ocular pemphigoid, but the data regarding the long-term effect on the disease activity or reactivation are extremely scarce, so the novel scientific aim of this study was to evaluate the clinical outcomes after a 9-year follow-up in 12 eyes (6 patients) affected by MMP with ocular involvement, successfully treated with IVIg therapy, as previously described in our report published in 2008. The evaluation of ocular and extraocular disease progression was performed at the end of IVIg therapy and at the end of the follow-up period. After 9 years, all the eyes enrolled showed a long-lasting remission of ocular and oral symptoms with a significant steroid-sparing effect. In conclusion, the IVIg has to be considered as a safe and successful alternative therapy in patients with severe ocular mucous membrane pemphigoid; furthermore, this kind of therapy seems to be effective in maintaining the clinical remission by the time.

Coexistence of orbital cavernous hemangioma and other vascular malformations is unusual and few cases have been reported. We describe the clinical and radiological features of three cases of orbital cavernous hemangiomas associated with other vascular malformations, selected reviewing a series of 181 cases of cavernous hemangiomas. All patients were males (age ranging from 43 to 67 years) without vascular systemic disorders and/or a clinical syndrome. They experienced slow progressive exophthalmos. One of them developed acute pulsatile proptosis (case 2), while another experienced slow progressive diplopia (case 3). In one case vascular lesions were bilateral (case 3) and in two patients two different lesions coexisted in the same orbit (cases 1 and 2). All patients underwent surgical excision, which was partial in two cases. Two patients had cavernous hemangiomas in association with a venous malformation (a varix in case 1 and a lymphangioma in case 2), while in the other ones (case 3) cavernous hemangioma was associated with a low-flow arteriovenous malformation. No patient denied visual impairment postoperatively. Few cases of orbital cavernous hemangiomas coexisting with other vascular malformations have been reported in the literature. This entity seems to be an association of different variants of orbital vascular malformations, presenting with a wide spectrum of clinical forms and probably with the same pathogenesis.

Purpose: To describe the case of a patient affected by orbital lymphoma mimicking pleomorphic adenoma of the lacrimal gland. Methods: This was a retrospective case report. Results: We present the case of a patient with 15-year history of slowly progressive left proptosis and inferomedial bulbar dislocation who had the presumptive diagnosis of lacrimal gland pleomorphic adenoma based on clinical and radiological features. The patient underwent lateral orbitotomy and lacrimal gland excision. Postoperative histological features were consistent with low-grade B-cell non-Hodgkin lymphoma. Conclusion: The accepted clinico-radiological criteria used for the diagnosis of lacrimal gland fossa lesions might have a certain false-positive rate, even in recent years. The initial surgical approach with the appropriate choice between fine-needle aspiration biopsies, intraoperative biopsies and lacrimal gland excisions might be a challenge.

Ocular adnexa B-cell lymphomas are a relatively rare group of extranodal lymphomas, marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphomas) being the most frequent type at this location. As with other nongastrointestinal MALT lymphomas, ocular adnexa MALT lymphomas have distinct characteristics from those of the gastric MALT model, implying specific pathogenic events, which could be of interest in the prediction of clinical behavior and the choice between therapeutic options. In a series of 39 cases of ocular adnexa MALT lymphomas, studied using a tissue microarray, we observed that the most frequent alteration was related to apoptosis regulation. Thus, caspase 3 activity was completely abolished, and phosphorylated IkappaBalpha, a marker of NF-kappaB activation, showed increased expression, while cases with an increased number of large cells displayed increased expression of survivin and other cell-cycle-related proteins, such as cyclin A, cyclin E and Ki67, and p16 expression was reduced. There were no occurrences of t(11;18)(q21,q21), while 5/37 cases exhibited t(14;18)(q32;q21). Aberrant nuclear expression of bcl10 was observed in 11 cases, independently of the presence of translocations, and was significantly associated with phosphorylated IkappaBalpha expression and a reduced TdT-mediated biotin-dUTP nicked-end labeling apoptotic index. Moreover, patients with tumoral bcl10 nuclear expression showed shorter failure-free survival.

Background/Aim. To evaluate the efficacy of methotrexate for the treatment of thyroid eye disease (TED). Methods. 36 consecutive patients with active TED, previously treated with corticosteroids but stopped due to the occurrence of side effects, were commenced on methotrexate therapy. Two different weekly doses were administered depending on the weight of the patient (7.5 mg or 10 mg). Clinical activity score (7-CAS), visual acuity (VA), ocular motility, exophthalmos, and eyelid position were retrospectively evaluated at 3, 6, and 12 months and compared with baseline data. Results. There was a statistically significant improvement in 7-CAS at 3, 6, and 12 months after treatment ( ). There was no significant change in visual acuity. Ocular motility disturbances improved at 6 and 12 months ( ). There was no significant change in exophthalmos (mean 24 mm, SD 3 mm) or eyelid position (marginal reflex distance mean 6 mm, SD 1.5 mm) during the follow-up period. No side effects were registered. Conclusions. Methotrexate therapy is effective in reducing CAS and ocular motility disturbances. No significant improvement in proptosis or eyelid retraction should be expected from this treatment. Eventually, it might be considered a suitable alternative treatment in TED for patients who cannot tolerate steroids.