A case of choroidal neovascularization as a first manifestaion of systemic lupus erythematosus.

BACKGROUND: To report a rare occurrence of pigment epitheliopathy associated with choroidal neovasculization as a first manifestation of systemic lupus erythematosus. CASE PRESENTATION: A 54-year-old female, with no prior medical history, sought a second opinion due to sudden drop in vision in her right eye to 20/80. Slit lamp examination was normal. Fundus examination revealed the presence of a subretinal hemorrhage in the macular area. Fundus imaging including optical coherence tomography and fluorescein angiography showed multifocal retinal pigment epitheliopathy associated with choroidal neovascularization (CNV). The patient had received an intravitreal injection of Bevacizumab 2 weeks ago. It was decided to complete the loading dose regimen with two additional Bevacizumab injections, and the first injection was done 2 weeks after her presentation. Two weeks later, the patient reported a rash on her cheeks, painful joints, and purpura. Systemic workup revealed positive ANA, anti-cardiolipin antibodies, and decreased complement levels, with negative anti-histone antibodies. This led to the diagnosis of systemic lupus erythematosus (SLE) based on the "Systemic Lupus International Collaborating Clinics" criteria. The patient was treated with 50 mg of prednisolone which was then tapered. 1 month after the third injection, an showed a total resolution of the sub-retinal fluid with an improvement of vision to 20/20. No recurrence was observed during follow-up. CONCLUSION: Based on the findings from the fundus exam and imaging, systemic symptoms and the blood work-up, we postulate that the pigment epitheliopathy associated with choroidal neovascularization was related to the vaso-occlusive disease at the level of the choroid that can be part of SLE vasculopathy. To our knowledge, this represents the first case in which pigment epitheliopathy and CNV were the primary manifestations of SLE.

A novel RLBP1 gene mutation associated to a flecked retina syndrome.

PURPOSE: To present an unusual fleck retina condition associated to a novel RLBP1 gene mutation.Methods/Results: A 25-year old male presented flecks on fundoscopic examination. Clinical presentation, multimodal imaging and ERG were compatible with the diagnosis of benign familial fleck retina. Genetic analysis detected an RLBP1 gene, a gene commonly associated with more severe retinal disease. CONCLUSION: Flecked retina syndromes and other genetic retinal diseases have a complex genotype-phenotype relation and need further research for their pathophysiology to be fully understood.

Retromode imaging in retinal diseases: A systematic review of the literature.

Retromode scanning laser ophthalmoscopy imaging captures a pseudo-3-dimensional image of the ocular fundus. Retromode scanning laser ophthalmoscopy imaging was introduced first in 2008 using the Nidek F-10 scanning laser ophthalmoscope (F-10; Nidek Co., Gamagori, Japan). At that time, no major role was described for this imaging modality. The interest in retromode scanning laser ophthalmoscopy imaging is reemerging with the recent advent of the Mirante that combines scanning laser ophthalmoscopy and optical coherence tomography (Nidek Co., Gamagori, Japan) that can capture retromode images of the fundus. We summarize the findings and clinical implications of retromode imaging using the Nidek F-10 and the Mirante in retinal diseases with the aim of helping researchers direct their future studies.

Outcome of Patients Treated by Intravitreal Injections During the COVID-19 Lockdown: An Institutional Experience.

PURPOSE: To determine the loss of follow-up ratio and reasons during the COVID-19 lockdown in patients with retinal diseases treated by anti-vascular endothelial growth factor intravitreal injections and to report the visual outcome and rate of complications of these patients 1 year after the end of the lockdown. METHODS: This is a prospective descriptive cohort study (NCT04395859) conducted at the Rothschild Foundation Hospital - Paris between April 2020 and May 2021. Patients with retinal diseases treated by repeated intravitreal anti-VEGF injections (IVI) since before October 2019 were included. They filled-out a questionnaire and were followed up during a period of 1 year. RESULTS: During the COVID-19 lockdown 198 eyes (82.5%) of 157 patients (82.6%) received their injections in a timely manner (group 1) while 42 eyes (17.5%) of 33 patients (17.4%) had their injections delayed or missed (group 2). No statistically significant difference was found between group 1 and group 2 when comparing the change of mean best corrected distance visual acuity (BCVA) between month 12 and inclusion (p = 0.6) and the rate of ocular complications. The most frequent reasons for missing scheduled injections are appointments cancellation by the hospital (12 patients, 36%), fear of virus exposure during transportation (7 patients, 21%) or at the hospital (5 patients, 15%). Eighty-four percent (130/157 patients) of patients who attended their appointment were satisfied by the protective measures used in the hospital. CONCLUSION: COVID-19 lockdown did not seem to negatively affect the 1-year outcome of patients with retinal diseases treated by anti-VEGF IVIs who missed their scheduled injections. The BCVA and rate of complications at 1 year did not differ whether patients missed their scheduled injections or not. Maintaining IVIs during lockdown periods and educating patients about the risks of missing injections are pivotal in improving prognosis of retinal diseases.

The role of near-infrared reflectance imaging in retinal disease: A systematic review.

Near-infrared reflectance (NIR) retinal imaging aids in a better visualization of structures at the level of outer retina, retinal pigment epithelium, and choroid. It has multiple advantages, including easy acquisition in association with structural spectral domain optical coherence tomography, more comfort for patients, and enhanced contrast and spatial resolution. It helps in the diagnosis of chorioretinal diseases that present with minimal funduscopic findings and can be used to follow up many chorioretinal conditions. We describe the chorioretinal NIR imaging appearance and the clinical role of NIR imaging in ocular inflammatory disease, vascular and acquired disease, degenerative disease, tumors, associated systemic condition, toxic and traumatic disease, optic nerve head conditions, and physiological findings.

"En Face" Spectral-Domain Optical Coherence Tomography versus Multifocal Electroretinogram in Hydroxychloroquine Retinopathy Screening.

INTRODUCTION: The performance of "en face" optical coherence tomography (OCT) in screening for chloroquine (CQ) or hydroxychloroquine (HCQ) retinopathy has not been largely explored. The aim of this study was to determine the concordance of "en face" OCT with multifocal electroretinography (mfERG) in screening for CQ/HCQ retinopathy. METHODS: This is a prospective cohort study conducted at the Rothschild Foundation Hospital, Paris, between August 2016 and February 2021. Patients taking HCQ were followed up over 2 consecutive years and received an "en face" OCT and a mfERG on each visit. RESULTS: A total of 91 patients (182 eyes) were analyzed. mfERG and "en face" OCT were concordant in 147 eyes (86.3%). Cohen's kappa coefficient for concordance between mfERG and "en face" OCT was considered weak with a value 0.61 (95% CI: 0.50-0.72). The sensitivity and specificity of "en face" OCT were 70% (95% CI: 59-79%) and 91% (95% CI: 83-96%), respectively, relatively to mfERG. Proportion of abnormal R2/R5 and R3/R5 ratios did not differ between patients with normal and abnormal "en face" OCT (p = 0.2). DISCUSSION: "En face" OCT and mfERG have low concordance and cannot be used interchangeably as each investigation evaluates a different facet of CQ/HCQ retinopathy. "En face" OCT could be used as a complement in screening for CQ/HCQ retinal toxicity if the anomalies detected on "en face" OCT are confirmed by B-scan OCT sections.

Intraocular lens power calculation after excimer laser corneal refractive surgery: A retrospective study to compare the predictability and the efficacy of commonly used and modified formulas.

PURPOSE: Our article aims to assess the accuracy of modified and commonly used formulas of intraocular lens (IOL) power calculation after excimer laser corneal refractive surgery. METHODS: This is a retrospective study, with data retrieved for 50 eyes of 32 patients who underwent uncomplicated cataract surgery after excimer laser corneal refractive surgery. The expected spherical equivalent was calculated using the American Society of Cataract and Refractive Surgeons (ASCRS) IOL power calculator for Shammas and Barrett True-K, using three-fourth generation formulas (Haigis-L, Barrett True-K no history, and Holladay 2), and using three-third generation formulas (SRKT, Holladay 1, and Hoffer Q) with single k, as a reference, and adjusting these formulas by calculating the keratometry readings by two methods (Jarade's index and formula). The mean refractive error and mean absolute refractive error (MARE) were calculated at the 1 postoperative month. RESULTS: When all data was available (eight eyes), 13 formulas were compared. Holladay 1 as modified by Jarade's index and formula, and Hoffer Q as modified by Jarade's formula resulted in MARE <0.75D (P < 0.05). In the group of 25 eyes with only ablation available, the formulas with MARE <0.75D were Haigis L, Barrett TK (from ASCRS), Hoffer Q, and the three conventional formulas in Jarade's index (P < 0.001). In the group of 17 eyes with no available prerefractive data, only Haigis-L and Barret TK (no history) had a MARE <0.75 D. CONCLUSION: The use of Hoffer Q or Holladay 1, when prerefractive data are available, gives reliable results with Jarade's index.

One-year outcome of neovascular age-related macular degeneration patients followed-up using different optical coherence tomography modalities.

PURPOSE: The aim of this study is to compare the one year outcome of neovascular age-related macular degeneration (nAMD) patients treated by a PRN regimen of Anti-vascular endothelial growth factor (VEGF) intravitreal injections (IVTs), using optical coherence tomography B-scan (OCT-B) or OCT Angiography (OCT-A) imaging modalities during follow-up. METHODS: Patients older than 50 years with nAMD currently treated by PRN regimen of Anti-VEGF IVTs were recruited from Rothschild Foundation Hospital - Paris and Centre Ophtalmologique Maison Rouge - Strasbourg and followed-up for a year. Patients were randomized in two groups: one group was followed by OCT-B while the other was followed by OCT-A. RESULTS: Thirty-three patients were followed by OCT-A and 31 patients were followed by OCT-B. Twenty-nine patients in the OCT-A group and 27 patients in the OCT-B group attended the last visit. No statistically significant difference was found between the two groups at 1 year concerning: improvement or stabilization of the best corrected distance visual acuity (BCVA) (p > 0.9), exudative signs (p > 0.9), number of injections (p = 0.8) and the delay until the first reinjection was performed (p = 0.5). CONCLUSION: The use of OCT-A or OCT-B as imaging modalities in nAMD treated by a PRN regimen of Anti-VEGF IVTs seem to be comparable at one year.

Displaced Orbital Fractures with Concurrent Orbital Compartment Syndrome: A Case-Based Systematic Review.

Orbital compartment syndrome (OCS) is an emergency that complicates intra-orbital, retrobulbar hemorrhage in most cases. Bony orbital decompression is an effective treatment for OCS, and displaced orbital fractures are protective. Nevertheless, in rare cases, OCS occurs despite a displaced orbital fracture. The aim of the current review is to present its pathophysiology and management based on what is published in the medical literature, and our center's experience. A systematic review of literature was conducted through PubMed, Medline, Embase, and Cochrane from inception through February 2021. The following search query was used: "orbital fracture" and "trauma" and "orbital compartment syndrome." Studies tackling the pathophysiology and management of concurrent displaced orbital fracture with OCS were included and a cohort of patients was constituted. A cohort of 18 cases reported in the literature were included (49.3 ± 30.6 years, 50% M). Given that OCS occurs with or without orbital fracture, pathophysiology of OCS is mostly explained by the division of the orbital fat into many compartments, due to the presence of Koorneef's fibrous septa. Management of OCS in such circumstances consisted of inferior-lateral canthotomy and cantholysis in 50% of cases, subperiosteal drainage with myringotomy in 22.2% of cases, subperiosteal drainage in 16.7% of cases, and complete lateral cantholysis in 16.7% of cases. Two cases who presented to our tertiary care center were also added to this review. Physicians should be aware that OCS can occur even with displaced fractures of the orbital cavity. When clinical suspicion is present, an emergent management is needed.

The role of funduscopy in severe thrombocytopenia: A prospective study.

PURPOSE: Platelet transfusions for patients suffering from severe thrombocytopenia are regulated by clinical bleedings and platelet counts. The aim of this study was to assess the prevalence of retinal hemorrhage in patients with hematologic and oncologic malignancies and severe thrombocytopenia, and to determine the benefit of systematic funduscopic examination in this setting. MATERIALS AND METHODS: Hospitalized patients with hematologic and oncologic malignancies having a platelet count less than 25,000 per µL underwent indirect ophthalmoscopy. The prevalence of retinal hemorrhage and its correlation with different patients' characteristics were determined. The decision to transfuse platelets or not following bedside indirect ophthalmoscopy was left at the discretion of the treating physician. RESULTS: A total of 34 severe thrombocytopenic patients were included in the study. The prevalence of retinal hemorrhage was detected in 10 patients (29.4%). No significant correlation was found between the occurrence of retinal hemorrhage and age, platelet count or thrombocytopenia etiology (P>0.05). No significant difference was found concerning the rate of transfusion between those with and without retinal hemorrhage. CONCLUSION: According to our statistical results, retinal hemorrhage is a frequent finding in severely thrombocytopenic patients. Early detection may lead to an increase in the platelet transfusion threshold from to 30,000 per µL offering additional protection against spontaneous bleedings. Funduscopy is a safe and easy exam to perform systematically in patients with severe thrombocytopenia.

Characteristics of open eye injuries in the Beirut Port explosion.

INTRODUCTION: On the 4th of August 2020 an ammonium nitrate explosion took place at the Port of Beirut in Lebanon. The aim of this study was to present the open eye injuries caused by the Beirut Port blast, and to describe their characteristics and visual outcomes at 6 months. METHODS: Patients who suffered from open globe injuries during the Beirut port explosion were recruited. Characteristics of the ocular injuries and best corrected distance visual acuity (BCVA) were recorded at presentation and at 6 months. RESULTS: All included eyes (23 eyes of 19 patients) suffered from penetrating open globe injuries secondary to glass material and were operated by globe exploration + primary repair. Four of the 19 patients suffered from bilateral injuries none of whom suffered from legal blindness. BCVA at presentation was hand motion or worse in 21 of 23 eyes (91%). Seven of 23 eyes (30%) had expulsive loss of intraocular tissue, 4 of 23 eyes (17%) had retinal detachment and 4 of 23 eyes (17%) had traumatic cataracts. Ocular trama score (OTS) had a mean +/- standard deviation (SD) of 61 +/- 12. Eleven of 23 eyes (48%) had zone 2 ocular injuries and 10 of 23 eyes (43%) had zone 3 ocular injuries. At 6 months follow-up 15 of 23 eyes (65%) had a BCVA of less than 20/200. All patients with Grade 2 OTS, zone 3 injuries, expulsive loss of intraocular tissue and retinal detachment had BCVA of less than 20/200 at 6 months. CONCLUSION: Open eye injuries secondary to explosions occur most frequently due to glass fragments. Most of the victims were indoors, near windows and less than 500m away of the explosion site at the time of the blast. The majority of victims have very low BCVA, a low OTS and a high ocular ZOI at presentation which reflects the danger of open globe injuries secondary to blasts. A high ZOI, a low OTS, the expulsive loss of intraocular tissue and retinal detachment seem to be predictors of worse VA at 6 months.

Evaluation of corneal sensitivity in multiple sclerosis patients.

PURPOSE: To measure the corneal sensitivity in patients with multiple sclerosis (MS), to compare it with normal values and to study its correlation with different disease characteristics. METHODS: Corneal sensitivity of 28 MS patients was compared to corneal sensitivity of 28 age- and gender-matched normal controls. Corneal sensitivity was measured using the Cochet-Bonnet esthesiometer and was correlated to the duration, type and severity indexes of the disease. RESULTS: Corneal sensitivity was comparable between both groups (P = 0.79). No statistically significant correlation was found between corneal sensitivity and the duration of MS (P = 0.55) nor the severity indexes of MS (expanded disability status scale [EDSS] P = 0.52, global multiple sclerosis severity score [MSSS] P = 0.64). Following subgroup analysis, only the primary progressive (PPMS) form of MS had a reduced corneal sensitivity with P = 0.023, while remittent-recurrent (RRMS), secondary progressive (SPMS), and clinically isolated (CIS) forms of MS did not have any reduction in the corneal sensitivity. "ROC curve analysis" showed an area under the curve of 0.48. CONCLUSION: In the exception of PPMS subtype, MS patients have similar corneal sensitivity in comparison to controls. Cochet-Bonnet esthesiometer does not seem to be a good diagnostic tool or a disease severity marker for patients with MS.