Bullous Variant of Central Serous Chorioretinopathy in Goodpasture's Disease - A Case Report and Review of Literature.

PURPOSE: To describe a case of bullous variant of central serous chorioretinopathy (CSR) in Goodpasture's disease (GD) compared to an identical twin without GD, and summarize the literature on ocular manifestations of GD. METHODS: Interventional/observational case report and literature review. RESULTS: A 46-year-old white female with a history of GD presented with decreased vision. She demonstrated bilateral multifocal pigment epithelial detachments and a large inferior exudative retinal detachment in the left eye consistent with bilateral CSR with bullous variant CSR (bvCSR) in the left eye. Despite treatment, her disease remained refractory, with final VA of 20/200 in the left eye. The patient's identical twin sister did not have GD and demonstrated milder CSR on presentation with a more typical, self-limited disease course. Her final VA was 20/20 bilaterally. CONCLUSION: GD is associated with severe manifestations of CSR (exudative RD). Additional studies focusing on the association between GD and CSR severity may be of interest.

The 0.19-mg Fluocinolone Acetonide Implant for the Treatment of Diabetic Macular Edema: An Expert Consensus.

BACKGROUND AND OBJECTIVE: To better understand the level of agreement among retina specialists on the role of inflammation in diabetic retinopathy (DR) and diabetic macular edema (DME), and the use of 0.19-mg fluocinolone acetonide (FAc) implant in DME treatment, a consensus survey was drafted and disseminated to retina specialists across the United States. MATERIALS AND METHODS: Using the modified Delphi method, a list of 12 consensus statements were generated by the coauthors based on short-answer responses to an initial survey. In total, 56 retina specialists completed the entire consensus survey. Except for two multiple-choice questions, there were 10 consensus statements that used a modified Likert scale to indicate their level of agreement to the statement: Agree = 3, Mostly Agree = 2, Mostly Disagree = 1, Disagree = 0. Percentage agreement and 95% confidence intervals (CIs) were calculated, and a consensus threshold was set at > 80% agreement for each statement. RESULTS: Seven of 10 consensus statements using the modified Likert scale reached consensus, including those on the role of inflammation in pathophysiology of DR/DME, injection burden and patient adherence, and efficacy and safety of the FAc implant. The remaining three statements displayed high agreement with average scores > 80%, but the 95% CIs were below threshold. These included the impact of the FAc implant on DR progression, FAc as baseline therapy for DME, and the effectiveness of the steroid challenge to mitigate intraocular pressure risk after FAc use. Two multiple-choice questions focused on clinical situations in which corticosteroids would be used as baseline therapy for DME (pseudophakic eye [73%], recent stroke/myocardial infarction [66%], and pregnancy/breastfeeding [66%]) and which delivery route satisfies the steroid challenge for the FAc implant (intravitreal [100%], sub-tenon/periocular [73%], and topical [57%]). CONCLUSIONS: Physicians highly agreed on the role of inflammation in pathophysiology of DR/DME, injection burden and patient adherence, and efficacy and safety of the FAc implant. However, full consensus was not found on the impact of the FAc implant on DR progression, FAc as baseline therapy for DME, and the effectiveness of the steroid challenge to mitigate intraocular pressure risk after FAc use. [Ophthalmic Surg Lasers Imaging Retina. 2023;54(3):166-173.].

COMBINED RHEGMATOGENOUS RETINAL AND CHOROIDAL DETACHMENT: A Systematic Review.

PURPOSE: To review the literature on eyes with concurrent rhegmatogenous retinal and choroidal detachment (RRD-CD). METHODS: Several databases were searched for "rhegmatogenous retinal detachment" and "choroidal detachment" through October 2022. All English language primary literature was reviewed. RESULTS: Studies demonstrated that eyes with RRD-CD were very uncommon and had diminished baseline visual acuity (VA) and intraocular pressure (IOP) compared with eyes with RRD only. Although no randomized trials have been performed, pars plana vitrectomy with or without scleral buckle (SB) have reported higher surgical success rates than SB alone. Reattachment rates were affected by age, IOP, adjuvant steroids, and grade of proliferative vitreoretinopathy. CONCLUSION: Low IOP and poor initial VA are salient features of eyes with RRD-CD. Steroids can be useful adjuvants administered safely using several routes including periocular and intravitreal injection. PPV ± SB may result in best surgical outcomes.

VISION-RELATED MALPRACTICE INVOLVING PRISONERS: Analysis of the Westlaw Database.

PURPOSE: To characterize vision-related malpractice litigation involving prisoners. METHODS: Retrospective legal database review using the Westlaw database was performed to identify vision-related malpractice lawsuits involving prisoners in the United States from 1914 to 2020. Main outcomes and measurements were allegations of malpractice, verdicts, and settlements. RESULTS: Sixty-four vision-related malpractice lawsuits involving prisoners were identified. Mean defendant age was 49 years (range, 28-74 years). Fifty-seven percent of the defendants were ophthalmologists and 43% were optometrists. The cases were most commonly from the South and Midwest (n = 19 [30%] for each). Allegations of malpractice included inadequate medical care or treatment refusal (n = 21 [33%]), failure to treat fully leading to continued suffering (n = 18 [28%]), and delay in treatment or referral (n = 17 [27%]). Retina-related diagnoses were most common (n = 18 [28%]). Forty-six (72%) cases were closed, 14 (22%) were open, and four (6.3%) were partially closed. Only two (3.1%) cases were decided in favor of the plaintiff. The most common reason for an unsuccessful suit was lack of deliberate indifference by the eye care professional (n = 21 [46%]). CONCLUSION: The most common reasons for vision-related malpractice brought forth by prisoners were inadequate or incomplete eye care and treatment refusal. Retina was the most commonly involved subspecialty. However, the success rate of these suits was extremely low.

Efficacy of Maribavir in Valganciclovir-Resistant Cytomegalovirus Retinitis.

PURPOSE: To determine whether maribavir is effective at treating ganciclovir-resistant cytomegalovirus retinitis. METHODS: Retrospective case report of a lung transplant patient with bilateral cytomegalovirus retinitis documented with serum and aqueous humor studies and color fundus photographs. RESULTS: A 72-year-old lung transplant patient with active ganciclovir-resistant cytomegalovirus was treated with intravitreal foscarnet therapy in one eye. Retinitis developed in the contralateral eye and was managed with systemic maribavir alone. Active retinitis regressed in both the eye treated with intravitreal foscarnet and the un-injected eye. CONCLUSIONS: This patient's results suggest that systemic maribavir is an effective treatment for treatment-resistant cytomegalovirus retinitis.

Rhegmatogenous Retinal Detachment with concurrent Choroidal Detachment and Macular Hole Formation after Uncomplicated Cataract Extraction and Intraocular Lens Implantation - A Case Report and Review of Literature.

PURPOSE: To describe a case of concurrent rhegmatogenous retinal detachment, choroidal detachment, and macular hole (RRD-CD-MH) formation in a patient after uncomplicated cataract extraction and intraocular lens implantation (CEIOL). In addition, we summarize the previously reported cases of RRD-CD-MH in the literature. METHODS: Interventional case report and literature review. RESULTS: A 71-year-old white man without relevant past medical or ocular history underwent an uncomplicated CEIOL. He presented three weeks later with counting fingers vision and intraocular pressure (IOP) of 5 mmHg. On dilated fundus examination, multiple superior tears and total RRD with a serous CD was evident. Intraoperatively, a full-thickness MH was identified. The patient underwent routine RRD and MH repair, with instillation of 1000 centistoke silicone oil (SiO). The retina successfully re-attached, the CD resolved, and the IOP normalized; however, the MH did not initially close. The SiO was removed ∼4 months later and, while the MH remained open, the patient's vision improved to 20/80. Approximately three years after the surgical repair, the MH closed spontaneously, and the vision remained at 20/80. CONCLUSION: Our patient developed an RRD-CD-MH post-uncomplicated CEIOL. The MH displayed delayed closure. The vision improved after surgical repair, and remained stable in the long-term.

PERSISTENT PLACOID MACULOPATHY IN A PATIENT WITH HIGH MYOPIA.

PURPOSE: To describe a case of persistent placoid maculopathy in a patient with high myopia. METHODS: Interventional case report. RESULTS: A 59-year-old white man with high myopia presented with blurriness in the left eye. Vision was 20/20 bilaterally. Optical coherence tomography showed subretinal thickening and fluid without definitive leakage on fluorescein angiography after which anti-vascular endothelial growth factor (anti-VEGF) therapy was initiated. The patient was diagnosed with persistent placoid maculopathy based on multimodal imaging and clinical response. His disease course was characterized by multiple bilateral recurrences despite active treatment. At final visit (23 months after presentation), the vision was 20/20 in the right eye and 20/25 in the left eye, and the disease remained quiescent on immunomodulating therapy. CONCLUSION: Prompt initiation of anti-VEGF injections and immunosuppression resulted in disease control and preservation of vision in both eyes for almost 2 years.

A 15-YEAR-OLD BOY WITH PROTRACTED VISION LOSS FROM ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY.

PURPOSE: To present an atypical case of acute posterior multifocal placoid pigment epitheliopathy in a 15 year old treated with immunosuppressive therapy. METHODS: Interventional case report. RESULTS: On initial presentation, the vision was 20/200 in the right eye and 20/300 in the left eye. The posterior poles of both eyes showed numerous creamy-white placoid lesions. Fundus autofluorescence demonstrated hypoautofluorescence lesions with hyperautofluorescence rims, while fluorescein angiography showed early blockage followed by late staining. These findings were consistent with a diagnosis of acute posterior multifocal placoid pigment epitheliopathy. Optical coherence tomography demonstrated outer retinal disruptions and thinning. Due to the severity of his disease, the patient was treated first with oral prednisone and later transitioned to mycophenolate mofetil by 2 months. His poor vision persisted beyond a 5-month follow-up visit despite fading of the lesions and reconstitution of the outer retinal layers and thickness on optical coherence tomography. By 8 months of follow-up, the visual acuity returned to 20/20 bilaterally without any further recurrences. CONCLUSION: Although most patients with acute posterior multifocal placoid pigment epitheliopathy have a relatively short course and recover vision quickly, the use of steroids and immunosuppression may be of benefit for those patients with severe and prolonged visual loss.

BETA-PERIPAPILLARY ATROPHY AND GEOGRAPHIC ATROPHY IN THE COMPARISON OF AGE-RELATED MACULAR DEGENERATION TREATMENTS TRIALS.

PURPOSE: To determine associations between beta-peripapillary atrophy (B-PPA) and incidence and growth of geographic atrophy (GA) in eyes treated with anti-vascular endothelial growth factor agents in the Comparison of Age-Related Macular Degeneration Treatments Trials (CATT). METHODS: We included 245 cases with incident GA and 245 controls matched by baseline demographics and characteristics associated with development of GA in the CATT. Baseline color images were graded for the type of B-PPA, defined as presence of hypopigmentation with visible choroidal vessels and sclera that is adjacent to the optic disk. Beta-peripapillary atrophy was further classified as scleral ring, sclera, sclera/choroidal blood vessels, or combination. Areas of each type of B-PPA and the circumferential extent of B-PPA were measured. RESULTS: Beta-peripapillary atrophy was present in 58% of eyes developing GA and in 52% without GA (P = 0.17). The greater circumferential extent of sclera/choroidal blood vessels B-PPA in relation to the optic disk was associated with incident GA (P = 0.02) and the GA size at first observation (P = 0.047). Beta-peripapillary atrophy was not associated with GA growth rates (P>0.05). Patients without B-PPA had a higher number of GA-associated risk alleles of ARMS2 (P = 0.0003) and HTRA1 (P = 0.001). CONCLUSION: The extent of sclera/choroidal blood vessel B-PPA was associated with the GA incidence and size but not with the growth rate in eyes treated for neovascular age-related macular degeneration. Beta-peripapillary atrophy and GA may share some common pathophysiologic pathways unrelated to the GA-associated risk alleles evaluated.

Assessment of Firework-Related Ocular Injury in the US.

Importance: Fireworks are popularly used for recreation but can lead to potentially blinding injuries. Studies quantifying the trend and national prevalence of firework-related ocular injuries are limited. Objective: To characterize firework-related ocular injuries treated in emergency departments (EDs) in the US from 1999 to 2017. Design, Setting, and Participants: This cross-sectional study used the National Electronic Injury Surveillance System, a stratified probability sample of more than 100 hospital-affiliated US EDs representing more than 5300 nationwide hospitals. Deidentified individuals in the National Electronic Injury Surveillance System database who experienced eye injury due to fireworks between January 1, 1999, and December 31, 2017, were included. Analysis began February 2019. Main Outcomes and Measures: Annual prevalence of firework-related ocular injury, firework type stratified by demographic information, diagnosis, event location/date, and patient disposition. Results: A total of 34 548 firework-related ocular injuries were seen in US EDs during the 19-year study period (estimated from 1007 individuals in the National Electronic Injury Surveillance System database). Overall, 664 individuals (65.9%) were 18 years or younger, 724 (71.9%) were male, and 512 (50.8%) were white. Twenty-eight patients (2.8%) sustained a ruptured globe, while 633 (62.9%) sustained ocular burn injuries, 118 (11.7%) had ocular foreign bodies, 97 (9.6%) had conjunctival irritation, and 46 (4.6%) experienced other severe eye trauma. Of 1007 individuals, 911 (90.5%) were treated and released without transfer, while 87 (8.7%) required admission or transfer to another hospital. The most common specified firework types included firecrackers (193 [19.2%]), bottle rockets (177 [17.6%]), sparklers (88 [8.7%]), roman candles (66 [6.6%]), and novelty devices (65 [6.5%]) (eg, poppers and snappers). Bottle rockets disproportionately caused the most severe injuries, including ruptured globe (odds ratio, 5.82; 95% CI, 2.72-12.46; P < .001). A total of 411 cases (74.9%) occurred at home. Injuries most commonly occurred near the time of Independence Day and New Year's Day: 707 patients (70.2%) presented in July, 74 (7.4%) in June, 101 (10.0%) in January, and 47 (4.7%) in December. Conclusions and Relevance: These findings support that firework-related ocular injuries range from mild irritation to ruptured globe, and most occur frequently in those who are young, male, and white. Focused preventive methods and regulations may be imperative in decreasing fireworks-related ocular morbidity, namely from bottle rockets and especially near national holidays.

RECURRENT BRANCH RETINAL ARTERY OCCLUSION FROM SUSAC SYNDROME: CASE REPORT AND REVIEW OF LITERATURE.

PURPOSE: We report a patient with branch retinal artery occlusion predominant Susac syndrome and review the literature on current diagnostic modalities and therapeutic approaches for this rare condition. METHODS: Case report. RESULTS: A 23-year-old white male presented with a 5-month history of a scotoma in his right eye vision, headaches, and intermittent paresthesias of the lower extremities. Ophthalmic examination was normal with the exception of a hypopigmented small retinal area inferonasal to the fovea in the right eye. Optical coherence tomography demonstrated inner retinal atrophy and optical coherence tomography angiography showed deep capillary layer vessel dropout in both eyes. Ultra-wide-field fluorescein angiography revealed bilateral arteriolar wall hyperfluorescence. Corpus callosal lesions were present on magnetic resonance imaging. Lumbar puncture demonstrated elevated protein. Audiogram was normal. A diagnosis of Susac syndrome was made. The patient initially received oral steroids followed by intravenous steroids and mycophenolate mofetil because of new branch retinal artery occlusions. Thirteen months after initial presentation, the vision was stable at 20/20 in both eyes. CONCLUSION: It is a diagnostic challenge when recurrent branch retinal artery occlusion secondary to Susac syndrome presents without the classic symptoms. Optical coherence tomography angiography and subtle magnetic resonance imaging findings aided in early recognition of the diagnosis in our patient. Ultra-wide-field fluorescein angiography was an important tool in disease monitoring. Aggressive management with intravenous corticosteroids and initiation of immunosuppression resulted in long-term preservation of vision and improvement in visual field.

Association of Opioids With Incisional Ocular Surgery.

IMPORTANCE: Opioid abuse has been declared a public health emergency. Currently, little is known about the association between opioids and ocular surgery. OBJECTIVE: To characterize rates of filled opioid prescriptions after incisional ocular surgeries. DESIGN, SETTING, AND PARTICIPANTS: This cohort study included patients with incisional ocular surgeries within a large national US insurer's administrative medical claims database. All incisional ocular surgeries from January 2000 through December 2016 were evaluated. An opioid prescription was eligible if it occurred from 1 day before to 7 days after a surgery. Any surgery on a patient who was younger than 18 years, had more than 30 consecutive days of an opioid prescription in the prior 6 months, or had less than 6 months of data in the database prior to surgery was excluded. Data analysis occurred from May 2018 through November 2018. MAIN OUTCOMES AND MEASURES: The rate of opioid prescriptions filled for all incisional ocular surgeries from 2000 through 2016. Primary analysis looked at the rate of filled opioid prescriptions for each ophthalmic subspecialty surgery over time. Secondary analysis assessed which patient or surgical characteristics (ie, age, sex, race/ethnicity, geographic locations, yearly income, educational level, and type of eye surgery) were associated with filling an opioid prescription. Multivariate logistic regression using generalized estimating equations was used to determine odds ratios (ORs) of filling an opioid prescription. RESULTS: A total of 2 407 962 incisional ocular surgeries were included, of which 45 776 (1.90%) were associated with an opioid prescription. The rate of filled opioid prescriptions varied considerably over time, with the lowest rate occurring in the 2000-2001 cohort year (671 of 45 776 [1.24%]) and the highest in 2014 (5559 of 45 776 [2.51%]). An increasing trend was seen over the course of the study (2000-2001: 671 of 45 776 [1.24%]; 2016: 5851 of 45 776 [2.07%]; P < .001). Multivariate logistic regression showed that year of surgery was significantly associated with filling an opioid prescription, with the highest odds in 2014 (OR, 3.71 [95% CI, 3.33-4.1]), 2015 (OR, 3.33 [95% CI, 2.99-3.70]), and 2016 (OR, 3.27 [95% CI, 2.94-3.63]) compared with 2000 to 2001 (P < .001). CONCLUSIONS AND RELEVANCE: These findings suggest the rate of filled opioid prescriptions are increasing for all types of incisional ocular surgery over time. Given the ongoing national opioid epidemic, understanding patterns of use can help in reversing the epidemic.

ACUTE EXUDATIVE PARANEOPLASTIC POLYMORPHOUS VITELLIFORM MACULOPATHY DURING VEMURAFENIB AND PEMBROLIZUMAB TREATMENT FOR METASTATIC MELANOMA.

PURPOSE: To describe a patient with BRAF mutation-positive cutaneous melanoma who developed acute exudative polymorphous vitelliform maculopathy during vemurafenib and pembrolizumab treatment for metastatic melanoma. METHODS: Retrospective case report documented with wide-field fundus imaging, spectral domain optical coherence tomography, and fundus autofluorescence imaging. RESULTS: A 55-year-old woman with bilateral ductal breast carcinoma and BRAF mutation-positive metastatic cutaneous melanoma complained of bilateral blurred vision within 5 days of starting vemurafenib (BRAF inhibitor). She had been on pembrolizumab (program death receptor antibody) and intermittently on dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor), and had a normal ophthalmologic examination. On presentation three weeks after the introduction of vemurafenib, her visual acuity had declined to 20/40 in both eyes. Her examination showed diffuse elevation of the fovea with multifocal yellow-white, crescent-shaped subretinal deposits within the macula of both eyes and bilateral neurosensory retinal detachments by spectral domain optical coherence tomography. Discontinuation of vemurafenib and introduction of difluprednate and dorzolamide led to a gradual resolution (over four months) of the neurosensory detachments with recovery of vision. CONCLUSION: This case report suggests that acute exudative polymorphous vitelliform maculopathy may be directly associated with the use of BRAF inhibitors as treatment for metastatic cutaneous melanoma, or indirectly by triggering autoimmune-paraneoplastic processes. Future identification of similar associations is required to unequivocally link vemurafenib and/or pembrolizumab to acute exudative polymorphous vitelliform maculopathy in metastatic melanoma.

SYSTEMIC BETA-BLOCKERS AND RISK OF PROGRESSION TO NEOVASCULAR AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To determine whether oral beta-blockers (BBs) are associated with the development of neovascular age-related macular degeneration (nAMD). METHODS: Retrospective cohort study of patients from 2000 to 2014 using data from a large national U.S. insurer's administrative medical claims database. Patients with nonexudative AMD who initiated (index date) BB, a calcium channel blocker (CCB), an angiotensin-converting enzyme/angiotensin receptor blocker, or a diuretic. Patients were excluded for <2 years in the plan before the index date, any history of nAMD or diagnosis, or treatment for an ocular disease that could be confused with nAMD. Hazard of developing of nAMD was the main outcome measure. Primary analysis compared BB with CCB patients with BB versus the other classes as secondary analyses. In addition, a sensitivity analysis was performed between BB and CCB cohorts using 1:1 propensity score matching. Cox proportional hazard regression was performed to estimate the hazard ratio (HR) of developing nAMD at 90, 180, and 365 days for BB. Covariates of interest included demographic information, year of index date, number of antihypertensive medications, and other comorbid systemic conditions. RESULTS: Eighteen thousand seven hundred and fifty-four BB patients and 12,784 CCB patients met criteria for inclusion. After controlling for covariates, patients on BB had a lower hazard for nAMD at both 90 and 180 days than patients on CCB (HRs: 0.67-0.71; P < 0.01 for both) and diuretics (HRs: 0.55-0.62; P < 0.01). Patients on BB versus angiotensin-converting enzyme/angiotensin receptor blocker at all time points and BB versus CCB and diuretics at 365 days did not have a significantly lower association with nAMD (HR: 0.73-0.85; P > 0.06 for all comparisons). A sensitivity analysis using propensity score matching yielded similar results with patients on BB significantly less likely to develop nAMD at 90 and 180 days (HR: 0.70-0.76; P < 0.049 for both) but not at 365 days (HR: 0.88; P = 0.30) compared with patients on CCB. CONCLUSION: No evidence was found that BB usage increased the hazard for nAMD relative to other antihypertensive medications.

Anterior chamber fibrinoid syndrome after cataract extraction in a patient on ibrutinib for B-cell chronic lymphocytic leukemia: a case report and review of the literature.

BACKGROUND: Ibrutinib is a tyrosine kinase inhibitor commonly used in patients with chronic lymphocytic leukemia. Based on the published literature, it has a very sound ophthalmologic safety profile. In the following, we describe a case of anterior chamber fibrinoid syndrome in a patient on ibrutinib for B-cell chronic lymphocytic leukemia after uncomplicated cataract extraction. CASE PRESENTATION: A 75-year-old white man with B-cell chronic lymphocytic leukemia on ibrutinib therapy and without significant past ocular history presented 1 day after uncomplicated phacoemulsification with in-the-bag intraocular lens implantation with multiple, discrete, pigmented cords in the anterior chamber. His vision was 20/100 and intraocular pressure was 43 mmHg. There was no hypopyon, hyphema, or cellular reaction. The dilated fundus examination was unremarkable. He was diagnosed as having fibrinoid syndrome and started on topical prednisolone, brimonidine, timolol-dorzolamide, and orally administered acetazolamide. Within 2 weeks, the fibrin cords disappeared completely, vision improved to 20/30, and the intraocular pressure normalized off all medications. CONCLUSIONS: The precise etiology of fibrinoid syndrome remains unclear. This is the first case of fibrinoid syndrome in a patient on ibrutinib, which is known to cross the blood-brain barrier and induce intraocular changes. It is important to differentiate this syndrome from toxic anterior segment syndrome and endophthalmitis, and to initiate appropriate treatment. The fibrin bands tend to be exquisitely sensitive to topical steroids and to resolve within a few weeks without sequelae.