Emergency Ultrasound Unveils Intermediate-High Risk Saddle Pulmonary Embolism with Extensive Bilateral Clot Burden, Masquerading as Micturition Syncope: A Case Report.

BACKGROUND: Isolated syncope as the manifestation of pulmonary embolism (PE) is a rare and diagnostically challenging presentation that often leads to delayed or missed diagnosis, increasing morbidity and mortality. In spite of emphasizing cardiovascular etiologies of syncope, current guidelines offer essentially no guidance in establishing a diagnostic workup for PE in these patients. By performing bedside echocardiography, emergency physicians can accurately identify concerning features suggestive of PE in patients with syncope. CASE REPORT: A 78-year-old man, receiving ertapenem via a peripherally inserted central catheter for treatment of extended spectrum ß-lactamase urinary tract infection, presented to the emergency department for isolated syncope with collapse while urinating. Arriving asymptomatic with normal vital signs and a benign physical examination, a presumptive diagnosis of micturition syncope was made. However, subtle vital sign changes on reassessment prompted performance of a point-of-care echocardiogram, which revealed signs of right heart strain. A computed tomography angiogram confirmed a saddle PE with extensive bilateral clot burden. Catheter-directed thrombectomy was performed via interventional radiology, with successful removal of pulmonary emboli. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Pulmonary embolism presenting as isolated syncope represents a daunting diagnostic dilemma, as emergency physicians may not consider it, or anchor on more benign etiologies of syncope. Although lacking sufficient sensitivity to rule out PE, point-of-care echocardiography to evaluate for signs of right heart strain can quickly and effectively point toward the diagnosis, while also assessing for other emergent cardiovascular causes of syncope. Given the lack of evidence-based guidance concerning PE presenting as syncope, bedside echocardiography should be highly considered as a part of the emergency physician's diagnostic workup, especially in patients with abnormal vital signs.

Long-Term Stability of Melanocytomas With Persistent Vascular Activity Without Malignant Transformation.

Purpose: To present 2 cases of large atypical melanocytomas that simulate melanoma. Methods: The largest risk factors for malignant transformation from melanocytoma into malignant melanoma are a combination of lesions with a thickness greater than 2 mm, visual symptoms, and tumor margin at the disc. The patients in this report were chosen because they both presented these factors with their lesions. Results: Because the lesions were properly identified as melanocytomas of the optic disc, the decision was made to monitor them closely and treat the associated vascular activity. Ultrasounds and close observations are key in differentiating these benign lesions from malignant melanomas. Conclusions: Both patients experienced long-term stability with intravitreal injections when needed for vascular activity.

Gene Expression Profile Class Change in a Case of Aggressive, Recurrent Melanoma.

Purpose: To report a novel case of a recurrent melanoma that had a change in its genetic expression profile (GEP) class over a 2-year period. Methods: This retrospective case study evaluated a patient with a recurrent uveal melanoma that changed classes from 1A to 1B. Results: A large melanoma was first treated with brachytherapy, and during that time genetic testing revealed a class 1A tumor. Two years later the tumor was noted to be enlarging, and the patient elected for enucleation. Subsequent GEP showed a class 1B tumor. Conclusions: An aggressive and large recurrent uveal melanoma that had changed from a class 1A to a class 1B tumor on subsequent GEP testing has never been reported before to our knowledge. It may imply that a recurrent or aggressive tumor has more mutations over time that could lead to a higher risk for metastasis. The natural course of a tumor's GEP class should be explored further.

Circumscribed Choroidal Hemangioma Simulating Choroidal Melanoma on Advanced Ultrawide-Field Pseudocolor Retinal Imaging: A Case Series.

The purpose of this case series was to evaluate the features of circumscribed choroidal hemangioma on pseudocolor ultrawide-field (UWF) retinal images simulating choroidal melanoma and compare it to fundoscopic appearance. All four patients underwent full ophthalmological examination, including dilated fundus examination, ultrasonography, and UWF imaging (UWFI). All circumscribed choroidal hemangioma appeared clinically as orange-red choroidal lesions which were echodense with regular internal structure on ultrasonography. All lesions appeared green-grey in color on pseudocolor UWFI. Pseudocolor UWFI of circumscribed choroidal hemangioma represents distortion of true color appearance and may simulate choroidal melanoma. [Ophthalmic Surg Lasers Imaging Retina 2023;54:292-296.].

Clinical Outcomes of Combined Phacoemulsification With Intraocular Lens Placement and Microincision Vitrectomy in Adult Vitreoretinal Disease.

Purpose: To evaluate the safety and clinical outcomes of combined phacoemulsification with intraocular lens (IOL) placement and microincision vitrectomy surgery (MIVS) in adult patients with concomitant cataract and vitreoretinal disease. Methods: A consecutive series of patients with comorbid vitreoretinal disease and cataract who had combined phacoemulsification with IOL placement and MIVS was retrospectively analyzed. The main outcome measures were visual acuity (VA) and intraoperative and postoperative complications. Results: The analysis comprised 648 eyes of 611 patients. The median follow-up was 26.9 months (range, 12-60 months). The most common vitreoretinal pathology was intraocular tumor (53%). The best-corrected Snellen VA improved from 20/192 at baseline to 20/46 at the 12-month follow-up. The most frequent intraoperative complication was capsule tear (3.9%). The most common postoperative adverse events after 3 months of follow-up (mean, 24 months) were vitreous hemorrhage (3.2%) and retinal detachment (1.8%). No patient developed endophthalmitis. Conclusions: Combined phacoemulsification with IOL placement and MIVS is a safe, effective technique to manage a broad range of vitreoretinal diseases in patients with significant cataract.

Sequential Short Interval Anti-VEGF for the Treatment of Stage 4 Retinopathy of Prematurity.

A 23-month-old girl born at 23 weeks' gestational age with a birth weight of 453 g and retinopathy of prematurity (ROP) stage 4A in 360° and plus disease in both eyes was treated in another institution with laser; her treatment was complicated with two cardiac arrests during the procedure. The patient was referred and treated at 31 weeks with repeated intravitreal bevacizumab injection in both eyes secondary to progressive ROP. Treatment with multiple anti-vascular endothelial growth factor (VEGF) therapy as monotherapy led to the resolution of a retinal detachment and full vascularization of the retina in both eyes. To the best of the authors' knowledge, this is the first description of a patient with stage 4A ROP treated solely with anti-VEGF after failed laser treatment that led to complete resolution of a retinal detachment without surgical intervention. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:159-163.].

Erdafitinib-Induced Secondary Maculopathy.

Purpose: This work presents a case of secondary maculopathy associated with the use of erdafitinib (Balversa) for the management of bladder urothelial carcinoma with bony metastasis. Methods: A case report is presented. Results: A 58-year-old Hispanic man presented with blurry vision 3 weeks after starting erdafitinib for the management of bony metastases associated with urothelial carcinoma. A comprehensive evaluation identified multiple areas of subretinal fluid induced by erdafitinib. Throughout treatment, the ocular condition progressed, causing worsening of vision; this led to discontinuation of the drug. Discontinuation was associated with visual and anatomic function improvement. Conclusions: Fibroblast growth factor receptor (FGFR) plays a major role in maintaining mature and premature retinal pigment epithelium cells. Drugs that inhibit the FGFR pathway block the activation of the mitogen-activated protein kinase pathway, leading to synthesis of antiapoptotic proteins. Erdafitinib is associated with ocular toxicity and leads to multifocal pigment epithelial detachments associated with secondary subretinal fluid.

Atypical Event Profiling of Intra-Arterial Chemotherapy.

Purpose: Three cases of atypical events following intra-arterial chemotherapy for the treatment of retinoblastoma are presented. Methods: Case report. Results: One patient had acute orbital swelling with proptosis, another with extravasation of the chemotherapeutic agent, and the final with total ipsilateral hearing loss. Conclusions: These cases underscore the importance of maintaining close follow-up when using intra-arterial chemotherapy for treatment of retinoblastoma.

Retinoblastoma in the Presence of Persistent Fetal Vasculature.

Purpose: This work aims to report the challenging diagnosis, treatment, and follow-up of a patient with persistent fetal vasculature (PFV) and retinoblastoma (RB). Methods: A 22-month-old boy presented with unilateral RB stage VB in the right eye and PFV in both eyes. The patient was treated with transpupillary laser ablation and systemic chemotherapy. Results: Treatment resulted in complete tumor regression. Two years after the last systemic chemotherapy treatment, magnetic resonance imaging (MRI) showed increased signal intensity with progressive optic nerve enhancement, where intraneural malignancy could not be excluded. Enucleation of the right eye was performed. Histopathologic review showed no residual active malignancy in the enucleated globe. Conclusions: This case demonstrates the importance of a thorough clinical examination to establish the correct diagnosis and to rule out RB before any surgery. This case also highlights the importance of regular follow-ups after tumor regression with full a ophthalmologic examination, B-scan, and periodic MRI.

Case Series: Small Full-thickness Macular Hole Treated with Topical Dorzolamide and Bevacizumab Intravitreal Injections.

SIGNIFICANCE: This article reports two cases reported of combined treatment with topical dorzolamide and intravitreal bevacizumab injections, successfully treating small full-thickness macular hole without the need for traditional macular hole surgery. PURPOSE: This study aimed to report the potential effectiveness of combination treatment with topical dorzolamide and bevacizumab intravitreal injection therapy for small macular holes. CASE REPORTS: Case 1 was a 68-year-old woman presented with distorted and decreased vision in her right eye for 9 months. Fundus examination showed a full-thickness macular hole, which was confirmed with spectral domain optical coherence tomography measuring 96 µm. Treatment with topical dorzolamide and intravitreal bevacizumab 1.25 mg/0.05 mL injections was initiated. Spectral domain optical coherence tomography showed gradual improvement of the full-thickness macular hole with complete hole closure and visual improvement in just 1 month. Case 2 was a 73-year-old woman who presented with distorted and decreased vision in her right eye for 8 months. Fundus examination showed a full-thickness macular hole in the right eye, confirmed with spectral domain optical coherence tomography, measuring 76 µm. Treatment with topical dorzolamide and intravitreal bevacizumab 1.25 mg/0.05 mL injections was initiated. Spectral domain optical coherence tomography showed gradual improvement of the full-thickness macular hole with complete closure at 2 months and improvement in visual acuity. CONCLUSIONS: The combination of topical dorzolamide and bevacizumab intravitreal injection is a reasonable consideration for small macular holes with vascular components before considering surgery. Both cases had early macular closure in less than 3 months.

Case Report: Two Genetically Distinct Choroidal Melanomas in the Same Eye Treated with Endolaser Therapy.

SIGNIFICANCE: This case report highlights the merits of using fine needle aspiration biopsy to obtain gene expression profiling of individual choroidal melanomas when more than one tumor arises in the same eye. It is also the first such case to document laser ablation therapy as the primary treatment. PURPOSE: This report describes a case of two primary choroidal melanomas with different genetic profiles in the same eye. CASE REPORT: An 80-year-old man presented to the office with a neoplasm of uncertain behavior in the left eye. The patient's visual acuity and IOP in the left eye, respectively, at the time of his first visit to the office were 20/25 and 8 mmHg. A dilated fundus examination revealed that there were two choroidal lesions in the left eye. The macular lesion was classified as type 1A, and the ciliochoroidal lesion was classified as type 1B. The patient underwent a vitrectomy of the left eye, followed by endolaser ablation of the tumors. The patient was also injected with bevacizumab. To date, the patient is free of known metastasis. Most recently, his visual acuity and IOP in the left eye were 20/30 and 14 mmHg, respectively. CONCLUSIONS: Although rare, multiple melanomas in the same eye may have differing genetic profiles, which may alter prognosis and management, depending on the class of tumor detected.

Case Report: Endogenous Candida Endophthalmitis in Cornelia de Lange Syndrome: Atypical Stellate Neuroretinitis.

SIGNIFICANCE: This study aimed to highlight the association of stellate neuroretinitis occurring secondary to endogenous candidemia. PURPOSE: We report an unusual presentation of endogenous Candida endophthalmitis as a stellate neuroretinitis in the setting of Cornelia de Lange syndrome. CASE REPORT: A 34-month-old girl with severe Cornelia de Lange syndrome and a history of parenteral nutrition dependence requiring a chronic central venous catheter presented with bilateral endophthalmitis secondary to candidemia. In one eye, the endophthalmitis had the atypical presentation as a stellate neuroretinitis. CONCLUSIONS: This case represents a unique association of stellate neuroretinitis secondary to Candida infection in a patient with Cornelia de Lange syndrome.

Brolucizumab: Evaluation of Compassionate Use of a Complex Anti-VEGF Therapy.

OBJECTIVE: To report a consecutive series of compassionate, off-label use of intravitreal brolucizumab as a rescue therapy for complex, non-responsive macular edema. This report delineates primary diagnosis, indications for treatment, adverse events, and visual and anatomic outcomes after intravitreal brolucizumab. METHODS: A retrospective review of a consecutive clinical case series of 110 eyes treated with intravitreal brolucizumab between January 1st and March 1st. 2020. All patients were included if they received intravitreal brolucizumab in an off-label delivery and had ongoing macular edema in the setting of prior, multiple intravitreal anti-VEGF and/or intravitreal triamcinolone acetonide. All patients had spectral domain OCT documented before, at the time of, and in serial follow-up after intravitreal brolucizumab. RESULTS: Ninety-eight of 98 patients had marked decrease in macular edema. Indications for treatment were assigned to the primary etiologic diagnosis leading to the macular edema secondary to radiation retinopathy, complex epiretinal membrane, or complex diabetic retinopathy. In this series, sdOCT central point thickness decreased by an average of 71.5 microns, subretinal fluid resolved, and visual acuity was improved in 40% (greater than two Snellen lines) and stable in 60% (within two Snellen lines). No patient experienced a severe adverse event to specifically include vitritis and/or vasculitis. CONCLUSION: In this series, brolucizumab intravitreal injection was associated with significant improvement in macular edema in each diagnostic category. No serious complications to treatment were found in this series. Brolucizumab, though associated with known intraocular inflammation and vasculitis, demonstrated marked benefit in these complex eyes previously unresponsive to aggressive intravitreal pharmacotherapy.

Aflibercept for Radiation Maculopathy (ARM Study): Year-2 Extension of a Prospective Clinical Study.

Purpose: This work describes the 2-year results of the Aflibercept for Radiation Maculopathy (ARM) randomized clinical study that evaluated intravitreal vascular endothelial growth factor antagonist therapy in radiation maculopathy delivering aflibercept using a second-year collapsed, every-6-weeks, treat-and-adjust interval. Methods: Forty patients were enrolled in an institutional review board-approved clinical trial and randomly assigned to aflibercept treatment via 1 of 2 regimens: fixed, every-6-week treatment or variable, treat-and-adjust therapy centered around 6 weeks. All patients had a diagnosis of treated uveal melanoma with documented tumor control, and they had visually compromising radiation maculopathy. At conclusion of year 1, the first 30 patients were offered a collapsed single-arm variable of an every-6-weeks treat-and-adjust aflibercept injection schedule for an additional treatment year. Results: Baseline best-corrected visual acuity (BCVA) was 20/63 at ARM study entry 20/62 at the institution of the year-2 extension. At ARM study entry baseline, spectral domain-optical coherence tomography mean central retinal thickness was 432 µm and was 294 µm at the same institution. At the 2-year study's conclusion, 76.7% (23 of 30) of eyes were better than 20/50, and only 6.7% (2 of 30) ended with a BCVA below 20/200. Final mean BCVA was 20/62 and final mean spectral domain-optical coherence tomography central retinal thickness was 286 µm, but as in year 1, this reduction in number of injections was not statistically significant. Conclusions: Aflibercept is effective in treating radiation maculopathy with maintained visual acuity at 2 years but continues to require an ongoing treatment approach to stabilize radiation maculopathy.

Five-Year Follow-up of Microincisional Vitrectomy Surgery, Endolaser Tumor Ablation, and Gene-Expression Profiling in Small Uveal Melanoma.

Purpose: This work evaluates a microincisional vitrectomy surgical (MIVS) approach to endolaser ablation of small uveal malignant melanoma by incorporating genetic tumor classification as a means to avoid radiotherapy while maintaining local tumor control without compromising visual acuity (VA). Methods: An institutional review board-approved, single-surgeon, retrospective analysis was conducted of a consecutive case series of all patients with tumors less than 2.5 mm in apical thickness who underwent MIVS, endolaser tumor ablation, fine-needle aspiration biopsy (FNAB), and intravitreal triamcinolone acetonide for small uveal melanoma between 2012 and 2015. Results: A total of 226 patients underwent FNAB from January 2012 to January 2015 for uveal melanoma. All 58 patients treated for a small uveal melanoma were included. This group of patients had a minimum follow-up of 60 months (range, 60-93 months). At initial diagnosis, subretinal fluid was present in 52 eyes (89.1%), macular edema was present in 24 eyes (41.4%), and epiretinal membrane was present in 11 eyes (20.1%). Fifty-six specimens (96.5%) received a molecular classification of either class 2 (4 of 56, 7.1%) or class 1 (52 of 56, 92.8%). Initial VA was 20/40 or better in 26 eyes (44.8%), and final VA was 20/40 or better in 48 of 58 eyes (82.8%). Conclusions: Endolaser tumor ablation delivered at MIVS surgery enables excellent tumor control (98.3%) and improves VA to better than 20/40 in more than 80% of treated eyes. FNAB achieves molecular classification in 96.5% of all patients undergoing treatment for small choroidal melanoma independent of tumor size.

Acute Orbital Compromise after Intra-Arterial Chemotherapy in a Complex Retinoblastoma Associated with 13q Deletion Syndrome.

INTRODUCTION: The intra-arterial chemotherapy (IAC) is increasingly used as a first-line therapy for retinoblastoma. The IAC has proved to be relatively safe. However, many local side effects of IAC have been described. CASE PRESENTATION: This case report describes a local side effect presenting as proptosis and myositis with vascular access difficulty of the middle meningeal artery, in a 2-year-old male with left eye diffuse multifocal stage Vb retinoblastoma complicated with retinal detachment. DISCUSSION/CONCLUSION: IAC is assured to provide as efficient results in eliminating the tumor as the systemic chemotherapy, without causing the systemic side effects. It has become an alternative to systemic chemotherapy. A better understanding of the local side effects is required.

Persistent Retinal Detachment in Retinoblastoma: The Challenges.

INTRODUCTION: Retinoblastoma (RB) is the most common eye tumor in children. There have been significant improvements in treatment options targeting killing the tumor while also conserving the eye and attempting to conserve functional vision. Retinal detachment (RD) is not an uncommon event and compromises the vision and sometimes RB treatment. MATERIALS AND METHODS: Retrospective review of 62 patients over a period of 8 years between 2012 and 2019 with eyes treated for RB and having persistent RD that did not resolve after complete tumor regression. RESULTS: Forty-two patients of these 62 cases developed RD (67%). The RD resolved in 35 patients (83% of RD), and 7 patients (16% of RD) developed a persistent RD. In all the persistent RD groups (7 patients/11 eyes), RB and RD were present simultaneously in the first ophthalmological assessment. Sex ratio was 2 females/5 males. The mean age of diagnosis was 11 months. All eyes had advanced RB stages. Eight eyes had local treatment with transpupillary laser, 6 eyes received IAC, and 3 patients received systemic chemotherapy. In 9 eyes, the RD had both exudative and tractional components. Only one eye had a pure tractional RD due to persistent fetal vasculature, and one eye had rhegmatogenous RD component with presence of a tear in addition to exudation. None of the eyes received RD surgical repair. CONCLUSION: Persistent RD occurs in eyes with advanced RB stages with complex RD with more than one component. The dilemma is performing a vitrectomy in eyes with cancer and poor visual outcome.

History through the eyes of a pandemic.

PURPOSE OF REVIEW: This review provides a historic perspective of the impact that major pandemics have had on human and their relationship with ophthalmology. The novel coronavirus epidemic is also analyzed, highlighting the relevance of the eye as a possible source of transmission, infection, and prognosis for the disease. RESULTS: Smallpox is suspected to be present for more than 12 000 years. However, trachoma seems to be the first recorded ophthalmological infectious disease. The deadliest pandemics include the bubonic plague, smallpox, and Spanish flu. The CoVID-19 epidemic is still developing and measures need to be implemented to prevent further escalation of the crisis. SUMMARY: Understanding the current facts in light of earlier historical evidence may help us prepare better to minimize the spread of infections in the future.

Impact of the COVID-19 Pandemic on Essential Vitreoretinal Care with Three Epicenters in the United States.

PURPOSE: To report the impact of COVID-19 on retina practices in three different "hot spot" cities in the United States. PATIENTS AND METHODS: The authors assessed data of encounters and intravitreal injections from March 16th to May 8th 2020, at different offices specializing in retina in the United States. All three practices are located in COVID-19 hot spot zones. One practice was in an academic setting, one practice was in a private multispecialty setting, and one practice was a solo private vitreo-retina practice. All practices were focused on emergent/urgent care, and the results were compared to preCOVID-19 weekly averages. RESULTS: A significant decrease in the number of encounters and injections was revealed in all three centers involved in this review. There was a decrease of 87% in encounters (156 patients were seen only) and a decrease of 58% (126 patients) in intravitreal injections in Weill Cornell Medical College in NYC and a decline of 59% (569 patients) in encounters and a decrease of 64% (280 patients) of intravitreal injections at the Ophthalmic Consultants of Boston and Tufts University School of Medicine in Boston. The decline at Miami Ocular Oncology & Retina in Miami was 37% (1198 patients) in the encounters and 30% (867 patients) in the injections. CONCLUSION: This manuscript documents a specific example illustrating that COVID-19 has led to a significant decrease in specialized health services. The degree of infection and mortality rate at each hot spot had a direct impact on the practice volume; however, the type of practice setting also played a role.