Five-Year Follow-Up of Repeated Intravitreal Bevacizumab for Macular Edema in a Pediatric Patient with Retinal Arteriovenous Malformation and Excellent Vision.

We report a case of a 9-year-old girl presenting with unilateral retinal arteriovenous malformation (AVM) with symptomatic macular edema. Over 5 years of follow-up includes optical coherence tomography (OCT), fundus photographs, and fluorescein angiography at baseline and at follow-up. Systemic and neurologic workup was completed and negative for intracranial AVM. Vision has correlated with macular edema, ranging from 20/20 to 20/80. The patient has received nine injections of intravitreal bevacizumab and has not required an injection for the last couple of years. Follow-up is ongoing.

Ocular syphilis on the rise: a 10-year analysis from 2010 to 2020.

PURPOSE: The purpose of the study was to evaluate the incidence of ocular syphilis as well as diagnostic parameters, comorbidities, and visual outcomes over a 10-year time period in West Virginia. METHODS: A retrospective chart review included 25 eyes of 17 patients with ocular syphilis between 2010 and 2020. RESULTS: The incidence of systemic syphilis at a large tertiary referral center has increased from 27 cases in 2010 to 105 cases in 2020. Seventeen patients were identified with ocular syphilis. Bilaterality was present in 47.1% of cases. In this study, 70.6% of patients were male and 29.4% were female. The median age of presentation was 40.2 years (range 21-63). Panuveitis was the most common (60.0%) followed by isolated anterior uveitis (16.0%), chorioretinitis (12.0%), inner retinitis (4.0%), and papillitis (8.0%). Forty percent of patients had visual acuity worse than 20/400 on presentation. Post-treatment visual acuity improved in all patients. Rapid plasma reagin (RPR) and Treponema pallidum particle agglutination (TP-PA) tests were positive in 84.6% and 100% of cases, respectively. CSF venereal disease research laboratory (VDRL) was positive in 36.4%, CSF pleocytosis was present in 72.7%, and elevated CSF protein was observed in 81.8%. Human immunodeficiency virus (HIV) co-infection was present in 31.3%. A majority of patients experienced maculopapular rash and/or history of genital chancre. The anatomic classification of presenting uveitis (anterior, intermediate, posterior, and panuveitis) did not correlate with clinical variables including age, gender, HIV status, serologic test, presence of rash, or year of diagnosis (p > 0.05). CONCLUSION: Ocular syphilis is becoming increasingly prevalent and can present with a variety of ocular findings; therefore, it should be considered in the differential diagnosis for patients with ocular inflammation. Visual prognosis is excellent with timely diagnosis and treatment.

Incidence of Epiretinal Membrane Formation After Pars Plana Vitrectomy for Giant Retinal Tear-Associated Retinal Detachment.

Objective: To report the incidence of postoperative epiretinal membrane (ERM) formation after primary pars plana vitrectomy (PPV) for giant retinal tear associated retinal detachment (GRT-RD) repair as well as its clinical characteristics and visual outcomes at a level one trauma and tertiary referral academic center. Patients and Methods: Patients with primary RD repair for GRT-RD at West Virginia University from September 2010 to July 2021 were identified using the ICD-10 codes (H33.031, H33.032, H33.033 and H33.039). Imaging studies including optical coherence tomography (OCT) were manually reviewed pre- and post-operatively for ERM formation after PPV for GRT-RD repair in patients who underwent PPV or combined PPV and scleral buckle (SB). Univariate analysis was performed to analyze clinical factors for ERM formation. Results: The study included 17 eyes of 16 patients who underwent PPV for GRT-RD. Postoperative ERM was observed in 70.6% (13 of 17 eyes) of the patients. Anatomic success was achieved in all patients. The mean (range) preoperative and final best corrected visual acuity (BCVA) in logMAR units by macula status was 0.19 (0-0.5) and 0.28 (0-0.5) for macula-on and 1.7 (0.5-2.3) and 0.7 (0.2-1.9) for macular-off GRT-RDs. Clinical variables including use of medium-term tamponade with perfluorocarbon liquid (PFCL), cryopexy, endodiathermy, number of tears or total clock hours of tears did not correlate with an increased risk of ERM formation. Conclusion: Post-vitrectomized eyes for GRT-RD repair have a significantly higher incidence of ERM formation, nearing 70% in our study. Surgeons may consider prophylactic ILM peel at the time of removal of tamponade agents or weigh in ILM peel at the time of primary repair, a more challenging surgical technique in our opinion.

Clinical Characterization of Autosomal Dominant and Autosomal Recessive PROM1 Mutation With a Report of Novel Mutation.

BACKGROUND AND OBJECTIVE: This study aims to provide clinical characterization of PROM1 mutation with a report of novel mutation. PATIENTS AND METHODS: This study is a retrospective case series of six patients from a single institution with multimodal imaging, electroretinography, and genetic testing. RESULTS: Six patients aged 12 to 47 years were identified. Patients with autosomal recessive (AR) variants showed more severe panretinal dystrophy with symmetrical macular involvement and peripheral retinal pigment epithelium atrophy. The autosomal dominant (AD) variants, on the other hand, showed milder macular involvement with bull's eye maculopathy phenotype with minimal peripheral involvement. Among patients with AR variants, a younger patient with aberrant splicing showed a milder phenotype compared with patients with a nonsense mutation and an additional ABCA4 mutation. CONCLUSION: The authors describe patients with PROM1 retinopathy inherited AD and AR inherited patterns. Novel mutations of c.1909C>T and c.2050C>T were identified, leading to truncation of the protein at sequence p.Gln637* and p.Arg684*, respectively. [Ophthalmic Surg Lasers Imaging Retina 2022;53:422-428.].

Multiple organ dysfunction syndrome and disseminated intravascular coagulation causing extensive multiple Amalric triangular choroidal infarctions.

PURPOSE: To present a case of extensive bilateral choroidal infarctions from multiple organ dysfunction syndrome (MODS) and disseminated intravascular coagulation (DIC). METHODS: A retrospective case report. The medical and imaging records, including fundus photography, optical coherence tomography, fluorescein angiography and visual fields were reviewed. PATIENT: A 49-year-old female presented after a failed suicidal attempt from drug overdose. The patient was subsequently intubated for acute hypoxic respiratory failure with presumed septic shock from aspiration pneumonia while requiring exogenous adrenergic support. Her hospital course was further complicated by development of multiple organ dysfunction syndrome and disseminated intravascular coagulation. The patient complained of blurry vision in both eyes after regaining consciousness. RESULTS: Fundus photography showed bilateral dot-blot hemorrhages and cotton wool spots followed by multiple areas of triangular granular pigmentation, consistent with choroidal infarcts. Fluorescein angiography revealed delayed patchy filling, followed by late staining. Optical coherence tomography showed diffuse thinning of the choroid with overlying retina pigment epithelium atrophy. Goldmann visual field displayed discrete areas of visual field deficits. At the most recent visit, the best corrected final visual acuity remained 20/100 OD and 20/30 OS. CONCLUSION: DIC has been associated with choroidal infarcts. We present a case of extensive bilateral choroidal infarction in a patient with combined DIC and MODS consistent with Amalric choroidal triangular sign.

Chryseobacterium indologenes Keratitis: Successful Treatment of Multidrug-Resistant Strain.

A 72-year-old male with history of monocular vision with complete vision loss in his right eye from previous retinal detachment presented with 20/200 vision in the left eye with a corneal ulcer. Culture was obtained, and the patient was started on fortified tobramycin, fortified vancomycin, and amphotericin. Despite the antibiotics, the patient did not significantly improve, after which another culture was obtained before the patient was taken to the surgery for cryotherapy and a partial conjunctival flap. The culture identified Chryseobacterium indologenes. There have been fewer than a handful of cases reported in the last three decades with different antibiotic susceptibility profiles. Our patient was successfully treated with ciprofloxacin and ceftazidime with the final vision of 20/40.

Clinical Course and Characteristics of Eyes with Recurrent Episodes of Endophthalmitis.

PURPOSE: Infectious endophthalmitis is a devastating, yet rare, complication after intraocular surgery, trauma, and systemic illness. Given its rare incidence, few patients would be expected to experience more than 1 episode of infectious endophthalmitis in their lifetime. We reviewed our patients who were diagnosed with and treated for at least 2 separate episodes of endophthalmitis. DESIGN: A retrospective, consecutive case series was conducted of patients managed at Associated Retinal Consultants PC (Royal Oak, Michigan) from January 2013 through December 2019. PARTICIPANTS: Patients were identified with the diagnosis of endophthalmitis by International Classification of Diseases, Ninth and Tenth Editions, codes. METHODS: Those diagnosed and then treated either with a vitreous tap and intravitreal injection of antibiotics or with pars plana vitrectomy at least twice were included. Those treated multiple times for the same episode of endophthalmitis were excluded. MAIN OUTCOME MEASURES: Cause and risk factors for recurrent endophthalmitis. RESULTS: Charts of 535 patients were reviewed, and 12 patients met inclusion criteria. The median age at initial presentation was 72.5 years, and 33.3% were men. Eight of the 12 patients (66%) experienced recurrent endophthalmitis in the same eye, and 4 of the 12 patients (33%) experienced separate episodes in different eyes. The average time between episodes was 604 days (range, 90-2366 days). The average follow-up from the second episode was 492 days (range, 119-1185 days). The most common cause for both the first and second episodes was recent intravitreal injection (50% and 58.3%, respectively) followed by surgery associated (41.6% and 33.3%, respectively). The cause was the same for the first and second episodes of 8 patients (75%). Of the 24 recorded episodes of endophthalmitis, culture results were positive in 41.6%, with coagulase-negative Staphylococcus being the most common bacteria identified. CONCLUSIONS: Recurrent endophthalmitis is rare and seen most commonly after intravitreal injections. Most patients in this series showed culture-negative results. Each successive episode of endophthalmitis was associated with a worse final visual outcome. The cumulative number of intravitreal injections may be an independent risk factor for recurrent postinjection endophthalmitis.

Characterization of Epiretinal Proliferation in Full-Thickness Macular Holes and Effects on Surgical Outcomes.

PURPOSE: Epiretinal proliferation is a distinct clinical entity from epiretinal membrane that classically is associated with lamellar macular holes, but its prevalence and association with full-thickness macular holes (FTMH) have not been well described. We characterized macular hole-associated epiretinal proliferation (MHEP) and its effects on long-term surgical outcomes. DESIGN: Multicenter, interventional, retrospective case-control study. PARTICIPANTS: Consecutive eyes that underwent surgery for FTMH with a minimum of 12 months follow-up. METHODS: All eyes underwent pars plana vitrectomy, removal of any epiretinal membranes, and gas tamponade, with or without internal limiting membrane (ILM) peeling. Spectral-domain OCT imaging was obtained before and after surgery. MAIN OUTCOME MEASURES: Improvement in visual acuity and single-surgery hole closure rates in eyes with, versus without, MHEP at 12 months. RESULTS: Seven hundred twenty-five charts were analyzed, and 113 patients met inclusion criteria. Of 113 eyes with FTMH, 30 (26.5%) showed MHEP. Patients with FTMH and MHEP were older (P < 0.002) and more often men (P = 0.001), and showed more advanced macular hole stages than those without MHEP (P = 0.010). A full posterior vitreous detachment was more common in eyes with MHEP (P < 0.004). Twelve months after surgery, FTMH with MHEP patients showed significantly less improvement in visual acuity (P = 0.019) with higher rates of ellipsoid and external limiting membrane defects (P < 0.05) and with a higher rate of failure to close with 1 surgery compared to FTMH without MHEP (26.7% vs. 4.8%; P = 0.002]). Peeling the ILM was associated with improved rates of hole closure in FTMH with MHEP (P < 0.001). Multivariate testing confirmed that the presence of MHEP was an independent risk factor for less visual improvement (P = 0.031) and for single-surgery nonclosure (P = 0.009) and that ILM peeling improved single-surgery closure rates (P = 0.026). CONCLUSIONS: We found that FTMH with MHEP showed poorer anatomic and visual outcomes after vitrectomy compared with FTMH without MHEP. Internal limiting membrane peeling was associated with improved closure rates and should be considered when MHEP is detected before surgery.

Predictors of Endophthalmitis after Intravitreal Injection: A Multivariable Analysis Based on Injection Protocol and Povidone Iodine Strength.

PURPOSE: To determine the incidence of endophthalmitis after anti-vascular endothelial growth factor (VEGF) therapy at our institution and to identify potential risk factors for endophthalmitis occurring after injection. DESIGN: Retrospective, single-center cohort study. PARTICIPANTS: All patients who received an intravitreal injection of an anti-VEGF medication between January 1, 2014, and March 31, 2017. METHODS: Current Procedural Terminology and International Classification of Diseases billing codes were used to identify instances of anti-VEGF administration and cases of endophthalmitis. Medical records and injection technique were reviewed carefully in each case. Multivariable logistic regression analysis was performed in a stepwise fashion to determine independent predictors of endophthalmitis based on injection protocol. MAIN OUTCOME MEASURES: Incidence of endophthalmitis after injection and odds of endophthalmitis by injection technique with 95% confidence intervals (CIs). RESULTS: A total of 154 198 anti-VEGF injections were performed during the period of interest, resulting in 58 cases of endophthalmitis (0.038% [1:2659]). After adjustment for confounders, both 2% lidocaine jelly (odds ratio [OR], 11.28; 95% CI, 3.39-37.46; P < 0.001) and 0.5% Tetravisc (Ocusoft, Richmond, TX; OR, 3.95; 95% CI, 1.15-13.50; P = 0.03) use were independent risk factors for endophthalmitis after injection. Lid speculum use, povidone iodine strength (5% vs. 10%), injection location (superior or inferior), conjunctival displacement, use of provider gloves, use of a strict no-talking policy, use of subconjunctival lidocaine, and topical antibiotic use were not statistically significant predictors of endophthalmitis after injection. There was no difference in endophthalmitis rate among the anti-VEGF agents (bevacizumab, ranibizumab 0.3 mg, ranibizumab 0.5 mg, and aflibercept). CONCLUSIONS: The incidence of endophthalmitis after anti-VEGF injections is low. Use of lidocaine jelly or Tetravisc may increase the risk of endophthalmitis after injection.

Impact of clinicopathologic factors on survival in patients with sebaceous carcinoma of the eyelid - a population-based analysis.

Purpose: To evaluate prognostic factors and survival of patients with sebaceous carcinoma of the eyelid through a population-based analysis. Methods: A total of 940 patients with primary sebaceous carcinoma of the eyelid were derived from the Surveillance, Epidemiology, and End Results (SEER) database in the United States from 1973 to 2013. Kaplan-Meier univariate analysis and Cox Regression multivariate analysis were performed to examine prognostic factors in overall survival (OS). 5- and 10-year survival rates, median survival, and prognostic variables with statistical significance were measured. Results: Kaplan-Meier analysis showed that OS is 66% and 44% at 5 years and 10 years respectively. Median OS is 9.4 years. Multivariate Cox regression analysis demonstrated that independent prognostic factors for OS are age at diagnosis (HR = 4.61 [95% CI 1.93-11.0], P = 0.001), surgical treatment (HR = 0.196 [95% CI 0.07-0.55], P = 0.002), combined surgical and radiation treatment (HR = 0.227 [95% CI 0.06-0.81], P = 0.023), and greater tumor size at diagnosis (HR = 3.381, [95% CI 1.77-6.45], P < 0.001). Conclusion: We report the largest population study to date to evaluate prognostic factors of patients with sebaceous carcinoma of the eyelid. Multivariate analysis shows that older age, and greater tumor size correlate with decreased overall survival, whereas surgical treatment or combined surgical and radiation treatment correlate with increased overall survival. Interestingly, tumor grade, lymph node involvement, and distant extent of tumor have not demonstrated to be independent prognostic factors for overall survival.

Prognostic Factors and Survival Outcome in Patients with Chordoma in the United States: A Population-Based Analysis.

OBJECTIVE: To evaluate prognostic factors of patients with chordoma through a population-based analysis. METHODS: Surveillance, Epidemiology, and End Results (SEER) database was queried for patients with chordoma from 1973 to 2013. Kaplan-Meier univariate analysis and Cox regression multivariate analysis were performed to examine prognostic factors in overall survival (OS) and disease-specific survival (DSS). RESULTS: One thousand five hundred ninety-eight patients with chordoma are identified. Kaplan-Meier analysis showed that OS and DSS were 61% and 71% at 5 years and 41% and 57% at 10 years. Multivariate Cox regression analysis demonstrated that independent predictors of OS and DSS are age at diagnosis (hazard ratio [HR]= 2.80 [95% confidence interval {CI}, 2.12-3.70], P < 0.001; HR = 1.60 [95% CI, 1.18-2.16], P = 0.002), surgical treatment (HR = 0.62 [95% CI, 0.52-0.73], P < 0.001; HR = 0.64 [95% CI, 0.52-0.79], P < 0.001), radiation therapy (HR = 1.23 [95% CI, 1.07-1.42], P = 0.004; HR = 1.29 [95% CI, 1.09-1.54], P = 0.004), tumor size (HR = 1.53 [95% CI, 1.32-1.78], P < 0.001; HR = 1.62 [95% CI, 1.35-1.94], P < 0.001) and distant metastasis (HR = 3.40 [95% CI, 2.45-4.71], P < 0.001; HR = 3.77 [95% CI, 2.61-5.45], P < 0.001). CONCLUSION: We report the largest study to date to evaluate prognostic factors of patients with chordoma. Multivariate analysis demonstrated that older age, greater tumor size, and distant metastasis were correlated with decreased survival, whereas surgical resection was correlated with increased survival. Patients receiving radiation therapy also showed decreased survival, likely an indication of the patients' advanced stage of disease, making them poor surgical candidates.