Ocular manifestations of severe familial hypercholesterolemia.

Background: To study ocular manifestations of patients with severe familial hypercholesterolemia (FH). Methods: In this population-based case-control study, patients suffering from severe familial hypercholesterolemia from the Lebanese Familial Hypercholesterolemia Registry, along with age and gender-matched healthy controls were recruited. All participants underwent a comprehensive eye examination, and patients underwent fluorescein angiography as well. Logistic regression models were used to identify any association between patients with severe familial hypercholesterolemia and abnormal eye findings, while adjusting for hypertension and pack-year smoking. The main outcome measure of this study was the development of ocular vascular abnormalities. Results: 28 patients and 28 controls were recruited. Patients with severe familial hypercholesterolemia had significantly greater odds of developing corneal arcus and xanthelasmas than the control group (p < 0.001). Retinal vascular abnormalities (plaques) were exclusively and more significantly present in patients with familial hypercholesterolemia (18 %). Similarly, retinal arteriosclerosis was exclusively and significantly more prevalent in the familial hypercholesterolemia group (p < 0.001, adjusted odds ratio 6.8). Stratification by LDL levels and genotypes did not show any significant change in the prevalence of any ocular finding. Conclusion: In addition to the well-established increase in incidence of corneal arcus and xanthelasmas, severe familial hypercholesterolemia patients have more prevalent retinal vascular abnormalities that include vascular plaques and arteriosclerosis.

Bilateral Endophthalmitis in a Postpartum Immunocompetent Patient.

Purpose: The aim of this report is to present the onset of bilateral endophthalmitis negative to culture testing and vitreous tapping in a postpartum immunocompetent patient. Methods: A 33-year-old patient developed floaters and severe blurry vision in both eyes 3 weeks after childbirth. With no previous surgery, no comorbidities in her clinical history, and negative diagnostic reports, endogenous endophthalmitis was suspected. Two days later, a pars plana vitrectomy was performed in both eyes one week apart, and intravitreal antibiotics and antifungals were administered during the surgery. No infectious source was identified since the cultures from the aqueous and vitreous humor returned negative in both cases. Results: Residual fibrosis around the fovea in the right eye and on the optic disc in the left eye was described. Nevertheless, the best corrected visual acuity of the patient was 20/20 in both eyes 4 months after the onset of the presumed endogenous endophthalmitis. Conclusion: This is the first report presenting a bilateral case of postpartum endophthalmitis negative to culture testing and vitreous tapping in a healthy patient with no previous surgeries nor long-term treatment. Early pars plana vitrectomy was fundamental for the correct management of this condition.

Sclerectomy Reverses Nanophthalmic Optic Neuropathy.

Introduction: Nanophthalmos is characterized by a short axial length, a thick choroid, and a thick sclera. Unilateral symptomatic disc swelling in nanophthalmos presents both a diagnostic and a therapeutic challenge. Case Presentation: A healthy 59-year-old man reported a two-week-long abrupt vision reduction in his right eye. 20/100 best spectacle (+17.25 diopter) corrected visual acuity, unilateral widespread disc enlargement, central scotoma, and a slight color vision disruption without an afferent pupillary defect were among the positive findings in the right eye. Workup for neuro-ophthalmology was negative. Numerous consultations did not suggest any form of treatment for the patient. Review of the optical coherence tomography (OCT) indicated a small, crowded optic nerve head and substantial diffuse choroidal thickening with dome-shaped temporal peripapillary area with choroidal expansion. In addition to circumferential anterior four-quadrant 95%-deep sclerectomy from recti insertion to the vortices, radial nasal posterior sclerotomy reaching the optic nerve sheath was performed on the patient. After the procedure, 2 weeks later, the patient's vision returned, and it persisted until the 6-month follow-up. By OCT, the two eyes were comparable as far as disc contour and nerve fiber layer thickness. Conclusion: This form of sclerectomy, which aims at decompressing the oncotic choroidal pressure, is an effective treatment for compressive optic neuropathy in the context of nanophthalmos. Could sclerectomy assist in treating other optic neuropathies associated with peripapillary pachychoroid?

Impact of the economic crisis, COVID-19 and the Beirut explosion on ophthalmology training in Lebanon: an observational cohort survey-based study.

OBJECTIVES: The objective of the study is to investigate the effects of the COVID-19 pandemic, the economic crisis and the Beirut explosion on the training and work of ophthalmology residents and faculty in Lebanon. DESIGN: This is an observational cohort survey-based research conducted between January and December 2022. SETTING: The study targeted all ophthalmology residents and core faculty in Lebanon. PARTICIPANTS: A total of 52 participants, including 27 residents and 25 core faculty members, completed the survey. PRIMARY OUTCOME MEASURE: Primary outcomes comprised the subjectively reported effect of the three major external stressors on the training and well-being of ophthalmology trainees and educators in Lebanon. RESULTS: The study found that the majority of ophthalmology residents and core faculty members were significantly affected by the COVID-19 pandemic, Beirut explosion and the economic crisis in Lebanon. Significant percentage reported financial burden, decrease in outpatient and surgical load and educational activities. Furthermore, most participants reported higher levels of stress, anxiety and depression during the time of crises. CONCLUSIONS: This study emphasises the need to support healthcare professionals during times of crisis, as they are on the frontlines and can experience high levels of stress, anxiety and depression. By providing support and resources to healthcare professionals, they can better cope with the challenges they face and continue to provide essential care to their patients.

Impact of adalimumab in patients with active non-infectious intermediate, posterior, and panuveitis in real-life clinical practice: HOPE study.

BACKGROUND/AIM: This study evaluated real-life adalimumab impact in patients with active non-infectious intermediate, posterior, or panuveitis (NIIPPU). METHODS: Adults with active NIIPPU received adalimumab in this prospective, observational study (06/2017-04/2020). Patients were evaluated at baseline (V0) and four follow-up visits over 12 months (V1-V4). PRIMARY ENDPOINT: proportion of patients achieving quiescence (anterior chamber (AC) cells grade and vitreous haze (VH) grade≤0.5+ in both eyes, no new active chorioretinal lesions) at any follow-up visit. Secondary endpoints: proportion of patients achieving quiescence at each visit; proportion of patients maintaining response; and proportion of patients with flares. Workability, visual function, healthcare resource utilisation, and safety were evaluated. RESULTS: Full analysis set included 149 patients. Quiescence at any follow-up visit was achieved by 129/141 (91%) patients. Quiescence at individual visits was achieved by 99/145 (68%), 110/142 (77%), 102/131 (78%), and 99/128 (77%) patients at V1-V4, respectively. Number of patients in corticosteroid-free quiescence increased from 51/147 (35%; V1) to 67/128 (52%; V4; p<0.05). Proportion of patients with maintained response increased from 89/141 (63%; V2) to 92/121 (76%; V4; p<0.05) and proportion of patients with flare decreased from 25/145 (17%; V1) to 13/128 (10%; V4; p=0.092). Workability and visual function improved throughout the study. Proportion of patients with medical visits for uveitis decreased from 132/149 (89%; V0) to 27/127 (21%; V4). No new safety signals were observed. CONCLUSION: These results demonstrated adalimumab effectiveness in improving quality of life while reducing economic burden of active NIIPPU.

Bromfenac 0.09% for the Treatment of Macular Edema Secondary to Noninfectious Uveitis.

PURPOSE: The topical nonsteroidal anti-inflammatory drug bromfenac 0.09% has a potential benefit in uveitic macular edema (UME) with a safe side effect profile. The aim of the study is to assess the efficacy of bromfenac sodium solution in the treatment of UME. METHODS: The charts of 10 patients with macular edema due to noninfectious uveitis treated with bromfenac 0.09% were reviewed retrospectively. The main outcomes studied were the best-corrected visual acuity (BCVA) and the central retinal thickness (CRT) compared 4 months before bromfenac initiation, at the time of its initiation, and 4 months later. RESULTS: Twelve eyes of 10 patients were included. BCVA and CRT were unchanged 4 months befoew bromfenac compared to the time of bromfenac initiation (P = 1.0 and P = 0.2, respectively). There were a significant improvement in BCVA after 4 months of bromfenac treatment (P = 0.043) and a significant decrease in CRT (P = 0.002). Subretinal fluid resolved completely in 8/9 eyes, and 4/9 eyes had a complete resolution of cystoid macular edema at 4 months. CONCLUSION: Bromfenac may be a useful addition to the treatment of UME.

Streptococcus salivarius endogenous endophthalmitis.

We describe a case of endogenous endophthalmitis in an elderly man caused by Streptococcus salivarius An 88-year-old male patient with diabetes with iron deficiency anaemia and history of transcatheter aortic valve implantation presented with an insidious clinical picture of atraumatic endophthalmitis. No internal or external source could be identified. Diagnostic and therapeutic vitrectomy revealed papillomacular abscess and vitreous fluids grew S. salivarius Despite lack of an identifiable source of infection, a high index of suspicion for atypical presentations is required in patients with multiple comorbidities that could weaken their immune system towards opportunistic infections. Early detection, microbiological evaluation and prompt treatment are critical to avoid disastrous outcomes. While S. salivarius has been implicated in cases of exogenous endophthalmitis, this is the first reported case of endogenous endophthalmitis due to S. salivarius.

Patterns of uveitis in children according to age: comparison of visual outcomes and complications in a tertiary center.

BACKGROUND: Uveitis in the pediatric population is uncommon, accounting for 2 to 14% of all uveitis cases, yet resulting in significant ocular morbidity. A number of studies have focused on patterns and complications of uveitis in the pediatric age group (≤ 16 years). In this report, we studied children with uveitis syndromes focusing on demographics, anatomic distribution, etiologies, treatment, and complications. We additionally divided subjects into two age groups to look into any differential characteristics pertaining to the younger age group and the role of amblyopia as a cause of visual loss. METHODS: Retrospective chart review of 80 eyes of 49 uveitis patients aged ≤16 years. Subjects were categorized by age of onset into visually immature (≤8 years) and visually mature group (> 8 years). Data compared between the two age groups included demographics, disease characteristics, visual outcomes and complications. RESULTS: Idiopathic uveitis was the most common diagnosis (51%). Anterior uveitis complications (posterior synechiae and band keratopathy) were more common in the younger group (p = 0.002 and p = 0.03 respectively) while posterior uveitis manifestations (vitreous haze and vasculitis) were more common in the older age group (p = 0.04 and p < 0.001 respectively). Amblyopia was the most common cause of vision loss in the visually immature versus cataract in the visually mature. CONCLUSION: Anterior uveitis and its complications were more common in visually immature group in our cohort. Amblyopia was identified as the main cause of visual loss in the younger population.

Extensive circumferential partial-thickness sclerectomy in eyes with extreme nanophthalmos and spontaneous uveal effusion.

AIM: To describe an extensive scleral excision technique to treat uveal effusion in nanophthalmic eyes. METHODS: This prospective, interventional series of eight eyes of five consecutive patients with nanophthalmos underwent scleral window surgeries. Ninety per cent of the scleral thickness, extending from immediately behind the extraocular muscle insertions to the vortex veins for 3 and 1/4 quadrants, was removed. The main outcome measure was resolution of the uveal effusions. RESULTS: Eight eyes of five patients (one female and four male) with a mean age of 46 years were studied. The mean (range) axial length was 16.1 mm (14.6-17.6 mm), and the mean refractive error was +13.6 dioptres (+10.75 to +16.00 dioptres). Following scleral excision surgery, all uveal effusions resolved within an average (±SD) of 13.9 (±8.7) days. The uveal effusion recurred in only one eye that had a vasoproliferative retinal tumour. The mean best corrected visual acuity improved from 0.69 logarithm of the minimum angle of resolution (logMAR) (Snellen equivalent: 20/97) at baseline to 0.51 logMAR (Snellen equivalent: 20/64; Wilcoxon paired t-test: p=0.016) after a mean follow-up of 35.6 months. CONCLUSION: The circumferential scleral window technique produces rapid resolution of uveal effusion in nanophthalmic eyes. No adverse effects were noted after surgery and the clinical effect was durable through 1 year. TRIAL REGISTRATION NUMBER: NCT03748732.

Unmeasurable small size superficial and deep foveal avascular zone in nanophthalmos: the Collaborative Nanophthalmos OCTA Study.

AIM: To study the macular structure and vasculature in consecutive nanophthalmic eyes using optical coherence tomography angiography. METHODS: This is a prospective, multicentre, cross-sectional study of patients with nanophthalmos (one or both eyes). The superficial and deep foveal avascular zones (FAZ) were measured both manually and with the machine's built-in automated measurement tool. Correlations between best corrected visual acuity (BCVA), central macular thickness (CMT) and subfoveolar choroidal thickness (SFCT) were calculated. RESULTS: Sixty-five eyes of 35 subjects (16 men and 19 women) with a mean age of 37.4 years were analysed. The mean±SD of refractive error was 14.3±3.2 dioptres, axial length was 16.4±1.6 mm, CMT was 410.2±128.3 µm and SFCT was 450.1±108.3 µm. FAZ was unmeasurable small size in both the superficial and deep capillary plexus in all eyes, along with tortuosity of the superficial foveal capillaries and large vessels. Foveal folds were present in 29 eyes. Disc drusen was detected in 27 eyes and was absent in 31 eyes, while fundus autofluorescence was positive in 17 and negative in 24 eyes. BCVA varied from 20/20 to 20/800, with a mean of 20/76. Using Spearman's correlation, logarithm of the minimum angle of resolution BCVA correlated negatively with axial length (r=-0.30; p=0.015). CONCLUSIONS: FAZ attenuation, capillary tortuosity, foveal folds and thickened subfoveal choroid characterise the nanophthalmic macula. These findings may result from a redundant retina and the absence of apoptotic foveolar retraction because of developmental arrest of the optic vesicle after closure of the embryonic fissure.

Neutrophils contribute to vasculitis by increased release of neutrophil extracellular traps in Behçet's disease.

BACKGROUND AND OBJECTIVES: Behçet's disease (BD) is a multi-system inflammatory disorder that can cause vasculitis. Here we questioned whether Neutrophils in BD cause vasculitis via releasing Neutrophil Extracellular Traps (NETs), a process called NETosis. METHODS: Circulating neutrophils were isolated from a cohort of Middle Eastern BD patients with an active disease and healthy volunteers. The percentage of NETs release was monitored in neutrophils stimulated or not with BD serum, and treated or not with Colchicine, Dexamethasone, Cl-amidine or N-Acetyl Cysteine (NAC). The mRNA expression levels of PAD4 (a key enzyme in NETosis) was also assessed. The effect of NETs on the proliferation and cell death of endothelial cells was investigated using an in vitro co-culture model. The presence of NETs in skin tissues of BD patients was examined using immunolabeling of NETs associated proteins. RESULTS: Circulating Neutrophils from BD patients were more prone to release NETs in vitro and expressed higher levels of PAD4 compared to healthy volunteers. Spontaneous NETs formation in BD neutrophils was inhibited by Colchicine and Dexamethasone, two drugs used to treat BD. NETs formation was also inhibited by Cl-amidine, a specific PAD4 inhibitor, and by NAC, a ROS inhibitor. Interestingly, serum from BD patients stimulated circulating neutrophils from healthy volunteers to release more NETs and increased their mRNA PAD4 expression. Moreover, endothelial cells cultured in the presence of NETs from BD patients showed a decrease in proliferation and an increase in apoptosis and cell death. Finally, NETosis was predominantly identified around affected blood vessels in biopsies of vasculitis from BD patients. CONCLUSION: Our results provide evidence on the implication of NETosis in the pathophysiology of BD especially in inducing vasculitis.

INTRAVITREAL DEXAMETHASONE IMPLANT IN RETINITIS PIGMENTOSA-RELATED CYSTOID MACULAR EDEMA.

PURPOSE: To report the clinical outcome after intravitreal dexamethasone implant in patients with retinitis pigmentosa and cystoid macular edema. METHODS: Multicenter retrospective case series of eyes with retinitis pigmentosa and cystoid macular edema that underwent intravitreal dexamethasone implant. Primary outcome measures were best-corrected visual acuity in LogMAR and central macular thickness. Statistical analyses used two-tailed comparison with Wilcoxon signed-rank test. RESULTS: There were a total of 45 eyes from 34 patients with a mean age of 32.7 years (range 16-57) and mean follow-up of 15.5 ± 13.0 months. At Month 3 after the first injection, mean initial best-corrected visual acuity improved from 0.61 ± 0.38 (20/81) to 0.37 ± 0.16 (20/47) (P = 0.012), whereas mean central macular thickness (µm) decreased from 506 ± 288 µm to 311.7 ± 71.6 µm (P < 0.001) and mean intraocular pressure increased from 15.7 ± 2.3 mmHg to 19.8 ± 11.0 mmHg (P = 0.01). Fourteen eyes had multiple injections (1-7 reinjections) at a mean interval of 6 months. Treatment effect was durable with multiple injections, but with seven eyes developing visually significant cataracts. CONCLUSION: Best-corrected visual acuity improved up to 4 months in around half of the eyes. Eyes that benefited the most were pseudophakic, steroid nonresponsive, with large initial central macular thickness, and profuse fluorescein dye leakage.

Intravitreal Adalimumab for the Control of Breakthrough Intraocular Inflammation.

PURPOSE: Investigate the efficacy of intravitreal adalimumab in breakthrough panuveitis in patients on systemic adalimumab for more than 3 months. METHODS: Retrospective study of patients on systemic adalimumab with breakthrough panuveitis requiring intravitreal adalimumab therapy. RESULTS: Seven eyes of four patients with Adamantiades-Behçet disease panuveitis were included and all were maintained on systemic adalimumab for 7.3 months (range 3-11) with inflammation controlled for 4.1 months (range 2-10) before breakthrough uveitis. The total number of attacks was 13 over 24.5 months (range 12-30). Resolution of attack was defined as return to baseline visual acuity with resolution of inflammatory markers. Three attacks resolved after only one injection and 10 attacks required an average of 2.4 injections (range 2-3). No systemic or ocular complications were noted. CONCLUSIONS: Intravitreal adalimumab warrants further investigation as a potentially effective, practical and safe adjunctive therapy for the control of breakthrough inflammation in select patients maintained on systemic adalimumab.

Operating room central serous chorioretinopathy.

OBJECTIVES: The operating room is a place of surgical intervention with its accompanying bodily and cognitive strain on the performers. Stress in the operating room may lead to the onset of central serous chorioretinopathy as reported hereby in a retina surgeon and is labeled as operating room central serous chorioretinopathy. METHODS: The same operator performed the optical coherence tomography scans on one retina surgeon. A masked observer estimated the maximal height of the subretinal fluid. RESULTS: Central serous chorioretinopathy recurred four times over a 1-year period 1 -2 days after a stressful day in the operating room, especially when cases were done under topical or subtenon anesthesia for cataract surgery, vitreous surgery or combined surgeries with complex ocular and medical problems and inability for anesthesia team to intervene. Stress management allowed resolution of subretinal fluid between 3 and 4 weeks. Adopting this strategy, no further attacks were documented by optical coherence tomography for 5 years. CONCLUSIONS: (1) This is one of a few optical coherence tomography documentation of resolution of central serous chorioretinopathy within 3-4 weeks of its occurrence and its recurrence induced by stress in the operating room; (2) Unassisted topical anesthesia required in patients with complex medical and ocular problems causes more cognitive stress than when surgery is carried under assisted local or general anesthesia (partly due to unexpected ocular or bodily movements); and (3) the available evidence suggests that those overcommitted surgeons (type A personality) may very well be most susceptible to burnout and central serous chorioretinopathy.

Intraocular Inflammation in Diabetic Populations.

PURPOSE OF REVIEW: The purpose of this review is to determine the prevalence of uveitis in the diabetic population, the clinical features of the uveitis and diabetes when coexisting and pathophysiology of a possible correlation. We also aim to review the cases of diabetes and uveitis in the literature. RECENT FINDINGS: The basis of an association between uveitis and diabetes mellitus (DM) is the common pathophysiology of inflammation. There are several reports on a DM-related uveitis, defined as idiopathic anterior uveitis in the presence of poorly controlled DM, but causation has not been established. There are conflicting results in the literature regarding an association between uveitis and DM. More studies are needed to determine if an association truly exists.

Intravitreal Adalimumab in Active Noninfectious Uveitis: A Pilot Study.

PURPOSE: To evaluate the short-term efficacy of intravitreal adalimumab (IVA) for the treatment of eyes with active noninfectious uveitis. METHODS: Consecutive eyes with active noninfectious uveitis were injected with IVA at 0, 2, then every 4 weeks for total of 26 weeks. RESULTS: Six out of 7 patients (12 of 13 eyes) completed 26 weeks of treatment. One patient (1 eye) failed treatment. Seven out of 12 eyes had improvement of ≥2 ETDRS lines. Three out of three eyes had resolution of anterior chamber cells. And 9 of 10 eyes with vitreous haze had zero haze at 26 weeks. Five out of 8 eyes with macular edema had complete resolution. Median fluorescein angiography score improved from 14 to 4 on last follow-up. CONCLUSIONS: IVA was effective in controlling the inflammation, decreasing the macular edema, and improving the best corrected visual acuity in the majority of eyes in this series.

Uveitis in the Aging Eye: Incidence, Patterns, and Differential Diagnosis.

Uveitis is a vision threatening inflammation of the eye that carries considerable morbidity. It is responsible for 10% of legal blindness in the United States and up to 25% in the developing world. Uveitis in patients more than 60 years of age is less common. The aging body has a changing response of the immune system, which might reflect a different pattern of uveitis in the elderly population. In this paper we review the incidence and patterns of uveitis in the elderly as reported in the literature and discuss changes with time. We also delineate a thorough differential diagnosis of de novo uveitis in the elderly.

Nontuberculous Mycobacterial Ocular Infections: A Systematic Review of the Literature.

Nontuberculous or atypical mycobacterial ocular infections have been increasing in prevalence over the past few decades. They are known to cause periocular, adnexal, ocular surface and intraocular infections and are often recalcitrant to medical therapy. These infections can potentially cause detrimental outcomes, in part due to a delay in diagnosis. We review 174 case reports and series on nontuberculous mycobacterial (NTM) ocular infections and discuss etiology, microbiology, risk factors, diagnosis, clinical presentation, and treatment of these infections. History of interventions, trauma, foreign bodies, implants, contact lenses, and steroids are linked to NTM ocular infections. Steroid use may prolong the duration of the infection and cause poorer visual outcomes. Early diagnosis and initiation of treatment with multiple antibiotics are necessary to achieve the best visual outcome.

Combined Nonmydriatic Spectral-Domain Optical Coherence Tomography and Nonmydriatic Fundus Photography for the Detection of Age-Related Macular Degeneration Changes.

BACKGROUND AND OBJECTIVE: Nonmydriatic fundus photography (FP) has been a suboptimal tool for detecting age-related macular degeneration (AMD) changes. This study sought to enhance the detection of AMD changes by combining nonmydriatic FP with nonmydriatic spectral-domain optical coherence tomography (SD-OCT). PATIENTS AND METHODS: The study population included 249 patients aged 65 years and older who were assessed for AMD changes using standard mydriatic biomicroscopic fundus examination. Each eye then underwent nonmydriatic FP in one session followed 1 week later with nonmydriatic FP coupled with nonmydriatic SD-OCT. Images were interpreted for detection of AMD changes, and findings were compared to the original mydriatic biomicroscopic examination. RESULTS: Nonmydriatic FP had 64% sensitivity, 97% specificity, and a kappa value of 0.67 in detecting AMD changes compared with the traditional mydriatic biomicroscopic examination. Combined nonmydriatic FP and nonmydriatic SD-OCT increased sensitivity to 91.5%, specificity to 98.6%, and kappa to 0.91. CONCLUSION: The addition of nonmydriatic SD-OCT to nonmydriatic FP enhances the detection of AMD changes.

Etiology and Clinical Features of Ocular Inflammatory Diseases in a Tertiary Center in Lebanon.

PURPOSE: To identify the pattern of uveitis in patients presenting to the American University of Beirut Medical Center (AUBMC) uveitis service in Lebanon. METHODS: The charts of patients seen between January 2009 and September 2011 were retrospectively reviewed. Data pertaining to patient demographics, eye examination on presentation, workup, and final diagnoses were collected. RESULTS: The total number of charts reviewed was 209. The most common noninfectious etiologies were Behçet disease, sarcoidosis, and HLA-B27-associated uveitis, while toxoplasmosis, herpes, and tuberculosis were the most common infectious etiologies. The mean duration of the disease before presentation to our center was 38 ± 56 months (range 0-284 months), with cataract formation in 90/234 (38%) and visual impairment in 134/291 (46%) eyes on presentation. CONCLUSIONS: This case series reveals a significant delay in referring uveitis cases to a specialized center, which may contribute to the high percentage of patients presenting with ocular complications.