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.

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.

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.

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.

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.

Isolated posterior capsular rupture following blunt head trauma.

Closed-globe traumatic cataract is not uncommon in males in the pediatric age group. However, there is a relative paucity of literature on isolated posterior lens capsule rupture associated with closed-globe traumatic cataract. We report a case of a 6-year-old boy who presented with white cataract 1 day after blunt trauma to the forehead associated with posterior capsular rupture that was detected by B-scan ultrasonography preoperatively. No stigmata of trauma outside the posterior capsule could be detected by slit-lamp exam, funduscopy, and optical coherence tomography. Phacoemulsification with posterior chamber intraocular lens implant was performed 24 hours after trauma, with the patient achieving 6/6 visual acuity 1 week and 6 months after surgery. Our case is unique, being the youngest (amblyogenic age) to be reported, with prompt surgical intervention, and with no signs of trauma outside the posterior capsule.

Adult ocular toxocariasis mimicking ciliary body malignancy.

Purpose. To discuss an unusual presentation of ocular toxocariasis. Methods. Case report. Results. A 40-year-old woman presented with decreased vision in the left eye with a long history of recurrent red eye from uveitis. Eosinophilia and positive ELISA titers for Toxocara canis favored the diagnosis of ocular toxocariasis. Over 3 months, an anterior scleral mass had a rapid growth raising the possibility of medulloepithelioma, which rarely can mimic uveitic syndromes. Surgical plan changed from local excision to enucleation. Histopathology demonstrated a large homogeneous mass of chronic inflammatory cells with inflammation of the overlying thinned out sclera, medial rectus insertion, and limbal cornea. The triad of peripheral granuloma, eosinophilia, and positive blood serology established the diagnosis of ocular toxocariasis. Conclusions. Ocular toxocariasis can mimic ocular malignancy such as medulloepithelioma in adults and rarely presents as an anterior scleral mass.

Visual outcomes from pars plana vitrectomy versus combined pars plana vitrectomy, phacoemulsification, and intraocular lens implantation in patients with diabetes.

PURPOSE: To compare visual acuity outcomes and diabetic retinopathy progression after pars plana vitrectomy (PPV) versus combined pars plana vitrectomy and phacoemulsification (PPVCE) in patients with diabetes. METHODS: Retrospective review of 222 consecutive diabetic patients undergoing PPV or PPVCE. RESULTS: A total of 251 eyes of 222 patients were evaluated (PPV = 122, PPVCE = 129). Four-year follow-up was 64% (161 eyes). Overall, patients undergoing PPVCE had better preoperative visual acuity (PPVCE = 20/80, PPV = 20/160, P = 0.03). At 4-year follow-up, visual acuity improved (PPV = +22, PPVCE = +11 letters) compared with baseline in both groups. After correcting for baseline differences in visual acuity, no statistically significant difference in final visual acuity was observed (PPVCE = 20/32, PPV = 20/50, P = 0.09). Results did not differ substantially by surgical indication (vitreous hemorrhage, traction retinal detachment, epiretinal membrane, and/or diabetic macular edema). Cataract progression occurred in 64%, and cataract surgery was performed in 39% of phakic eyes undergoing PPV. Rates of diabetic retinopathy progression, vitreous hemorrhage, and retinal detachment were not statistically different. Neovascular glaucoma developed in 2 patients (2%) after PPV and 6 patients (8%) after PPVCE (P = 0.07). CONCLUSION: In diabetic patients, equivalent visual acuity improvement over 4 years was observed after PPV or PPVCE. Visual outcomes and retinopathy progression rates were not significantly different after either intervention, suggesting that PPVCE may be appropriate when indicated in patients with diabetes.

Chronic postoperative endophthalmitis: a review of clinical characteristics, microbiology, treatment strategies, and outcomes.

Chronic postoperative endophthalmitis (CPE) is a delayed infectious intraocular inflammation process that occurs more than six weeks after ocular surgery and frequently masquerades as autoimmune uveitis. These cases are at risk of delayed diagnosis and erroneous long-term treatment with corticosteroids. This paper aims to review the epidemiology, microbiology, clinical characteristics, diagnosis, management strategies, and outcome of chronic postoperative endophthalmitis. The incidence of CPE is still uncommon, and multiple pathogens have been reported with varying frequencies. Review of the literature reveals that CPE cases have a high incidence of visual impairment and recurrence rate might be decreased with aggressive surgical approach.

Combined intravitreal bevacizumab with phacoemulsification in visually significant cataract and visually significant exudative maculopathy.

PURPOSE: We investigated the visual outcome of combined phacoemulsification with intravitreal bevacizumab, in eyes with dense cataract and visually significant exudative maculopathy. MATERIALS AND METHODS: Prospective longitudinal pilot study of consecutive patients treated by two surgeons in 2006, using intravitreal bevacizumab at the end of phacoemulsification. The historical control group consisted of consecutive subjects with exudative maculopathy and dense cataract treated by the same surgeons with the help of phacoemulsification without intravitreal bevacizumab prior to 2006. RESULTS: Thirty-one treated patients had the mean (SD) logMar best corrected visual acuity improving from - 1.48 (0.50) preoperatively to - 0.67 (0.38) in the first postoperative week (P < 0.001), to - 0.64 (0.40) in the first postoperative month (P < 0.001), and to - 0.62 (0.42) (P < 0.001) on the last follow-up (mean 4.2 months, range 1 - 9 months). Fourteen control patients had the mean (SD) logMar best corrected visual acuity improving from - 1.78 (0.79) preoperatively, to - 0.91 (0.53) in the first postoperative week (P < 0.001), to - 0.86 (0.45) in the first postoperative month (P < 0.001), and to - 0.90 (0.47) (P < 0.001) on the last follow- up (mean 19.6 months, range 1 - 49 months). Initial visual acuities, final visual acuities, and percentage of visual improvement at one month were all not significantly better in the intervention compared to the control group at one month. In the study group, the fovea was flattened at the one-month follow-up, by 90-diopter slit lamp examination and / or Optical coherence tomography. CONCLUSION: The combination of intravitreal bevacizumab and phacoemulsification is beneficial for maximal visual rehabilitation in the first postoperative month.