Papilledema in childhood for diagnosis of pseudotumor cerebri or late hydrocephalus: a case report

A case of hydrocephalus is presented in a 13-year-old female with transient loss of vision from 1 week and papilledema, previous ophtalmological history was negative. Visual field was performed, and neurogical examination proved to be hydrocephalus. In literature few cases of Papilledema in adolescent children with hydrocephalus has been reported. The aim of this case report is to decode the signs, symptoms and factors associated with papilledema in children with hydrocephalus at an early stage in order to prevent a poor visual-fun- ctional residual (permanent low vision)

Effects of repeated intravitreal injections of dexamethasone implants on intraocular pressure: a 4-year study

Purpose: Dexamethasone and other corticosteroids are administered intravitreally to treat a variety of retinal diseases. As a side effect, they can alter intraocular pressure (IOP). The purpose of this study is to describe the incidence, severity, and management of ocular hypertension following the administration of multiple intravitreal injections of dexamethasone implants. Materials and Methods: A total of 78 eyes of 78 subjects (males 62%; females 38%; mean age 67 ± 13 years SD) received a total of 152 intravitreal injections of 0.7 mg dexamethasone implants over 4 years. Indications included retinal vein occlusion (87%), diabetic macular edema (9%), wet-type age-related macular degeneration (4%). Ocular hypertension was defined as intraocular pressure above 23 mmHg or any pressure increase of 10 mmHg or more from baseline values. IOP was measured by applanation tonometry before the injection (T0), as well as one week (T1), one month (T2), and three months (T3) afterwards. Results: Five percent (4/78) of subjects developed ocular hypertension after the 1st injection. On the second and third rounds, additional 7.2% (3/42) and 4.2% (1/24) of subjects developed the same side effect. Among the 8 subjects who received a fourth injection, none was found with OHT. Pressure elevations were detected at T2 and T3. In all patients, topical medical therapy was sufficient to lower the IOP below threshold. Mean pressure variations following the first injection as compared to previous recorded values were +0.97 mmHg (T1), +0.92 mmHg (T2), and −0.41 mmHg (T3) (p < 0.05). Mean pressure variations following the second injection were +0.54 mmHg (T1), +0.23 mmHg (T2) and −0.66 mmHg (T3) (p < 0.05). Conclusion: Ocular hypertension is a recognized side effect of intravitreal dexamethasone. Some patients develop it right after the first injection, while others develop it subsequently, on the 2nd or 3rd round. This side effect becomes most apparent 30–90 days following the implantation procedure and responds well to topical pressure-lowering medications

Utility of ocular motility tests in orbital floor fractures with muscle entrapment that is not detected on computed tomography

Purpose: Determine the usefulness of ocular motility testing to detect the presence of muscle entrapment. Materials and Methods: Cross-sectional study of patients with symptoms of diplopia secondary to facial trauma. Inclusion criteria: age between 20 and 80 years; symptoms of diplopia following facial trauma; presence of orbital floor fracture confirmed radiologically; presence of muscle entrapment confirmed at the time of surgery; best-corrected visual acuity of 0.6 or more. Exclusion criteria: muscle entrapment visible on computed tomography; candidate for immediate surgical correction; prior history of strabismus surgery. Outcome measures: Abnormal Head Position (AHP), Hirschberg Corneal Reflexes (CR), Cover/Uncover and Alternating Cover Test, Hertel exophthalmometry, Near Point of Convergence (NPC), Kestenbaum Limbus test, Red Filter test, and Hess screen test. Results: Forty-six subjects (38 males, 8 females, mean age 27 ± 3.3 SD years). Pre-operative assessment: forty-six (100%) reported diplopia on the Red Filter test and showed some degree of abnormality on the Hess Screen test. Forty-two (91%) showed AHP. Forty-one (89%) had exophthalmometry values that differed 2 mm or more between the two eyes and insufficient NPC. Thirty-two (69.6%) showed deficits of 3 mm or more on the Kestenbaum Limbus test. Sixteen (35%) had abnormal Hirschberg corneal reflexes. Eleven (24%) demonstrated constant or intermittent strabismus. Conclusion: Ocular motility testing can differentiate non-invasively, pre-operatively, and cost-effectively the presence of muscle entrapment even when this is not visible on computed tomography

Gene Therapy in Hereditary Retinal Dystrophies: The Usefulness of Diagnostic Tools in Candidate Patient Selections

Purpose: Gene therapy actually seems to have promising results in the treatment of Leber Congenital Amaurosis and some different inherited retinal diseases (IRDs); the primary goal of this strategy is to change gene defects with a wild-type gene without defects in a DNA sequence to achieve partial recovery of the photoreceptor function and, consequently, partially restore lost retinal functions. This approach led to the introduction of a new drug (voretigene neparvovec-rzyl) for replacement of the RPE65 gene in patients affected by Leber Congenital Amaurosis (LCA); however, the treatment results are inconstant and with variable long-lasting effects due to a lack of correctly evaluating the anatomical and functional conditions of residual photoreceptors. These variabilities may also be related to host immunoreactive reactions towards the Adenovirus-associated vector. A broad spectrum of retinal dystrophies frequently generates doubt as to whether the disease or the patient is a good candidate for a successful gene treatment, because, very often, different diseases share similar genetic characteristics, causing an inconstant genotype/phenotype correlation between clinical characteristics also within the same family. For example, mutations on the RPE65 gene cause Leber Congenital Amaurosis (LCA) but also some forms of Retinitis Pigmentosa (RP), Bardet Biedl Syndrome (BBS), Congenital Stationary Night Blindness (CSNB) and Usher syndrome (USH), with a very wide spectrum of clinical manifestations. These confusing elements are due to the different pathways in which the product protein (retinoid isomer-hydrolase) is involved and, consequently, the overlapping metabolism in retinal function. Considering this point and the cost of the drug (over USD one hundred thousand), it would be mandatory to follow guidelines or algorithms to assess the best-fitting disease and candidate patients to maximize the output. Unfortunately, at the moment, there are no suggestions regarding who to treat with gene therapy. Moreover, gene therapy might be helpful in other forms of inherited retinal dystrophies, with more frequent incidence of the disease and better functional conditions (actually, gene therapy is proposed only for patients with poor vision, considering possible side effects due to the treatment procedures), in which this approach leads to better function and, hopefully, visual restoration. But, in this view, who might be a disease candidate or patient to undergo gene therapy, in relationship to the onset of clinical trials for several different forms of IRD? Further, what is the gold standard for tests able to correctly select the patient? Our work aims to evaluate clinical considerations on instrumental morphofunctional tests to assess candidate subjects for treatment and correlate them with clinical and genetic defect analysis that, often, is not correspondent. We try to define which parameters are an essential and indispensable part of the clinical rationale to select patients with IRDs for gene therapy. This review will describe a series of models used to characterize retinal morphology and function from tests, such as optical coherence tomography (OCT) and electrophysiological evaluation (ERG), and its evaluation as a primary outcome in clinical trials. A secondary aim is to propose an ancillary clinical classification of IRDs and their accessibility based on gene therapy’s current state of the art. Material and Methods: OCT, ERG, and visual field examinations were performed in different forms of IRDs, classified based on clinical and retinal conditions; compared to the gene defect classification, we utilized a diagnostic algorithm for the clinical classification based on morphofunctional information of the retina of patients, which could significantly improve diagnostic accuracy and, consequently, help the ophthalmologist to make a correct diagnosis to achieve optimal clinical results. These considerations are very helpful in selecting IRD patients who might respond to gene therapy with possible therapeutic success and filter out those in which treatment has a lower chance or no chance of positive results due to bad retinal conditions, avoiding time-consuming patient management with unsatisfactory results

Effect of COVID-19 quarantine on refractive errors in children aged 5 – 12 years: a retrospective study

Purpose: the COVID-19 quarantine led children to spend significantly more time indoors on near-work activities and digital devices. This study aims to explore whether these lifestyle changes had any effect on the prevalence of myopia among children aged 5 – 12 years1-3 . Subjects, Materials and Methods: retrospective study conducted in Italy. Population: children aged 5 – 12. Selection: random. Inclusion criteria: healthy children presenting for a routine eye exam since 2016. Exclusion criteria: presence of ocular comorbidities other than refractive error, spherical equivalent less than -4D or greater than +4D, BCVA less than 20/20, blepharoptosis, media opacities, corneal or retinal dystrophies, strabismus, amblyopia, nystagmus, or concurrent therapy with atropine 0.01%. Outcome measure: age measured in months, spherical equivalent (SE) of the right eye (RE) measured in diopters (D) under cycloplegia (cyclopentolate 1%). Statistical analysis: ANOVA, Chi-square, Tukey’s test. Significance: p < .05. Results: a total of 803 children. In the years prior to COVID-19, the mean SE ± SD diopters in the RE: 0.54 ± 1.49 D in 2016 (n = 160), 0.43 ± 1.84 D in 2017 (n = 145), 0.34 ± 1.41 D in 2018 (n = 152), 0.35 ± 1.75 D in 2019 (n = 166) (ANOVA, p = .659). In 2021 (n = 180), the mean SE was -0.08 ± 1.44 D (ANOVA, p = .005). Using the Tukey’s test, the mean SE of 2021 changed by -0.619 D [-1.091, -0.147] 95% C.I. and -0.501 D [-0.986, -0.016] 95% C.I. as compared to the SE of 2016 and 2017, respectively. Mean age was comparable in all groups (ANOVA, p = .307). The decrease of the mean SE of the 2021 group corresponds to an increase in the percentage of myopes (≤-0.5D) and a decrease in the percentage of hyperopes (≥ 2D). Myopes represent the 24.10% of children aged 60 – 96 months, and 63.86% of children aged 97 – 144 months. Hyperopes represent 9.64% of children aged 60 – 96 months, and 6.02% of children aged 97 – 144 months. This represents a statistically-significant increase in the number of myopes (Chi- square, p = .016) and decrease in the number of hyperopes (Chi-square, p = .001), as compared to the previous years (2016-2019). Conclusions: this retrospective study shows a statistically- significant decrease in the mean SE in children aged 5-12 in the year following the COVID-19 quarantine (2021). The percentage of myopes has increased significantly, while the percentage of hyperopes has decreased. Children aged 97 – 144 months showed the greatest refractive change. The lifestyle changes imposed by the quarantine led children to spend more time on near-work activities and digital devices, which are both known risk factors for the onset and progression of myopia. These lifestyle changes were likely responsible for the increased prevalence of myopia observed in the post-quarantine yea

Risk factors in central retinal vein occlusion: a multi-center case-control study conducted on the Italian population: demographic, environmental, systemic, and ocular factors that increase the risk for major thrombotic events in the retinal venous system

Purpose: To explore the risk factors for central retinal vein occlusion (CRVO) by comparing a large sample of patients with healthy controls. Materials and Methods: Multi-center case-control study. The study group includes patients affected by central retinal vein occlusion, confirmed angiographically, aged 50 years old or above (Group A). The control group includes healthy subjects without an history of retinal vein occlusion (Group B). Outcome measures: age, gender, active smoking, presence of uncontrolled arterial hypertension (uHTN), presence of the following comorbidities: diabetes mellitus type II (DMII), chronic liver disease (CLD), chronic kidney disease (CKD), thyroid disease (TD), systemic lupus erythematosus (SLE), hyperhomocystenemia (HHcy), dyslipidemia (DLip), carotid artery disease (CAD), glaucoma, atrial fibrillation (AF), migraine headache (MH), chronic obstructive pulmonary disease (COPD), obstructive sleep apnea syndrome (OSAS), history of myocardial infarction (MI). Odds-ratios were calculated with logistic regression analysis. Results: A total of 203 patients (Group A) and 339 controls (Group B). Statistically-significant differences were found for the following variables: age (OR: 1.109 [1.081–1.138], p <.001), active smoking (OR: 2.048 [1.210- 3.466], p <.008), DMII (OR: 4.533 [2.097–9.803], p <.001), HHcy (OR: 4.507 [2.477–10.001], p <.001), DLip (OR: 2.255 [1.352–3.762], p =.002), CAD (OR: 6.632 [2.944- 14.942], p <.001), glaucoma (OR: 4.656 [2.031–10.673], <.001), OSAS (OR: 1.744 [1.023–2.975], <.041), uHTN (OR: 3.656 [2.247–5.949], <.001). No statistically-significant differences were found for the other variables. Conclusions: Older age, active smoking, as well as presence of DMII, HHcy, DLip, CAD, glaucoma, OSAS, and uHTN, all increase the risk for CRVO. A comprehensive assessment of patients with CRVO is paramount. Adequate control of all the aforementioned risk factors is likely of great significance in reducing the incidence of CRVO among the general population, and it likely plays an important role in improving the prognosis following the occlusive event

Oral citicoline: influence of long-term therapy on perimetric glaucoma defects

Primary Open Angle Glaucoma (POAG) is characterized by a gradual loss of retinal ganglion cells (RGCs). Visual impairment ranges from visual field defects to blindness. Intraocular pressure (IOP) is the primary risk factor for POAG progression. However, recent studies suggest that therapy cannot be limited to IOP lowering alone. In fact, despite adequate IOP control, the disease still progresses in a percentage of patients [3]. In these, vascular or mechanical neurodegenerative processes are likely responsible. Therefore, the current trend is to prescribe molecules capable of inhibiting RGCs apoptosis. Citicoline, also known as choline CDP, is an endogenous organic neuroprotective molecule and acts as an intermediary in phosphatidylcholine synthesis. Growing evidence suggests that it stimulates neural cell metabolism, inhibits phospholipids degradation, and possibly even apoptosis. For this purpose, authors performed a retrospective study to explore the effect of citicoline supplementation on visual field defects progression

Efficacy and safety of intravitreal fluocinolone acetonide microimplant (ILUVIEN®) in patients with chronic diabetic macular edema: 1 year follow-up

Purpose: Evaluate the efficacy and safety of intravitreal 0.19 mg fluocinolone acetonide (FAc) micro implant in patients with chronic diabetic macular edema (cDME). Methods: Prospective study recruiting subjects with cDME. Inclusion criteria: cDME for at least 2 years documented with OCT imaging; pseudophakia; previous treatments with laser photocoagulation and intravitreal injections of anti-VEGF and/or dexamethasone. Exclusion criteria: phakia; ocular hypertension; tractional component visible on OCT; glaucoma; previous vitrectomy. Outcome measures included best-corrected visual acuity (BVCA), intraocular pressure (IOP), and central macular thickness (CMT), measured 1, 3, 6, and 12 months post-injection. Data were compared with the Friedman test and significance was set at p < 0.05. Results: A total of 18 eyes with a median duration of cDME of 45 months (25-118 months). The 77% of subjects either maintained or improved their BVCA. About 17% and 33% of subjects showed an improvement of 15 ETDRS letters or more at 3 and 12 months respectively. The 17% and 28% of subjects showed a CMT <250 microns at 3 and 12 months, respectively. The median change in CMT thickness was of -370 and -373.5 microns at 3 and 12 months post-injection respectively (p-value is 0.025). Changes in median IOP at 3 and 12 months post-injection were not statistically significant (p-value is 0.210). Ocular hypertension (OHT) was detected in two eyes (11%). Conclusion: The FAc micro implant has proved efficacy in improving and/or maintaining BVCA in 77% of patients with cDME up to 12 months post-injection. Ocular hypertension is the most common side effect but responds well to topical therapy

Effect of COVID-19 lockdown on refractive errors in Italian children aged 5–12 years: a multi-center retrospective study

Purpose: to explore the potential consequences of the COVID-19 lockdown on the prevalence of myopia among Italian children aged 5-12 years. Materials and methods: retrospective multicenter study conducted in Italy. Population: children aged 5-12. Selection: random selection of children who received an eye exam between 2016 to 2021. Inclusion criteria: healthy children presenting for a routine eye exam. Exclusion criteria: presence of ocular comorbidities other than refractive error, such as blepharoptosis, media opacities, corneal or retinal dystrophies, strabismus, amblyopia, or concurrent therapy with atropine 0.01%. Outcome measure: age and spherical equivalent (SE) measured in diopters (D) in the right eye (RE) in cycloplegia. Statistical analysis: ANOVA test. Results: total of 803 children. In the years prior to COVID-19, the mean SE ± SD of healthy age-school children was: 0.54 ± 1.49 D in 2016; 0.43 ± 1.84 D in 2017; 0.34 ± 1.41 D in 2018; 0.35 ± 1.75 D in 2019 (ANOVA, p = .659). In 2021, the mean SE changed to -0.08 ± 1.44 D (ANOVA, p = .005). Mean age was comparable in all groups (ANOVA, p = .307). The prevalence of myopes (SE ≤-0.5D) and hyperopes (SE ≥ 2D) was respectively 24.10% and 9.64% among children aged 60-96 months, and 63.86% and 6.02% among children aged 97-144 months. These values represent a statistically-significant increase in the number of myopes (Chi-square, p = .016) and decrease in the number of hyperopes (Chi-square, p = .001), as compared to previous years (.06 and.48 respectively). Conclusion: this retrospective study shows a statistically-significant decrease in the mean SE in children aged 5-12 in the year following the COVID-19 lockdown. The percentage of myopes has increased significantly, while the percentage of hyperopes has decreased. The lifestyle changes caused by the lockdown led children to spend more time on near-work activities and digital devices, and less time outdoors. These are known risk factors for the development and progression of myopia. Studies in different countries are encouraged

Efficacy and safety of intravitreal Fluocinolone Acetonide micro implant (ILUVIEN®) in patients with chronic diabetic macular edema: one-year follow-up.

Purpose : evaluate the efficacy and safety of intravitreal 0.19 mg fluocinolone acetonide (FAc) micro implant (ILUVIEN®) in patients with chronic diabetic macular edema (cDME). This is defined as edema that persists or recurs despite treatment. Methods : observational prospective study recruiting subjects with cDME. Inclusion criteria: cDME for at least 2 years documented with OCT imaging, pseudophakia, previous treatments with laser photocoagulation, intravitreal injections of anti-VEGF and/or dexamethasone. Exclusion criteria: phakia, ocular hypertension, glaucoma, previous vitrectomy. Outcome measures included best-corrected visual acuity (BVCA), intraocular pressure (IOP), and central macular thickness (CMT), measured one, three (T3), six (T6), and twelve (T12) months post-injection. Data was compared with the Friedman test and significance was set at p < 0.05. Results : 18 eyes with a median duration of cDME of 45 months [25 - 118 months]. 77% of subjects either maintained or improved their BVCA (Fig. 1 and 2). 17% and 33% of subjects showed an improvement of 15 ETDRS letters or more at 3 and 12 months respectively (Fig. 3). 17% and 28% of subjects showed a CMT <250 microns at 3 and 12 months respectively (Fig. 4). The median change in CMT thickness was of -370 and -373.5 microns at 3- and 12-months post-injection respectively (p-value is .025). Changes in median IOP at 3- and 12- months post-injection were not statistically significant (p-value is .210). Ocular hypertension (OHT) was detected in 2 eyes (11%) one-week post-injection. Conclusions : the FAc micro implant has proved efficacy in improving and/or maintaining BVCA in 77% of patients with cDME up to 12 months post-injection. Ocular hypertension is the most common side effect in pseudophakic patients but generally responds well to topical pressure-lowering medications