Superficial and Deep Capillary Plexus Nonperfusion in Nonaccidental Injury on OCTA.

A 5-year-old Caucasian male with a history nonaccidental injury (NAI) at age 1 presented with new onset no light perception in the left eye due to a closed funnel retinal detachment. The right eye showed optic nerve pallor, peripheral vascular attenuation, and leakage. Optical coherence tomography angiography (OCTA) revealed significant parafoveal attenuation of the superficial vascular plexus, intermediate capillary plexus, and the deep capillary plexus. This correlated with inner and middle retinal layer thinning temporal to the fovea and preservation of the ellipsoid zone. The peripapillary vascular plexus was preserved. Laser photocoagulation was performed to the nonperfused peripheral retina and intravitreal bevacizumab was injected. OCTA may be used in patients with NAI to characterize macular ischemia changes. Attenuation of the superficial, intermediate, and deep capillary plexuses in our patient may represent chronic ischemic retinal changes that arise from traumatic injury to the vitreoretinal interface and inner retina in NAI.

Unrecognized ROPER in a child with a novel pathogenic variant in ZNF408 gene.

BACKGROUND: Familial exudative vitreoretinopathy (FEVR) is a rare hereditary disorder characterized by abnormal or incomplete retinal angiogenesis commonly inherited in an autosomal dominant fashion. Up to 50% of FEVR cases are linked to known genetic mutations affecting retinal vasculature development. PURPOSE: To report a case, a novel pathogenic variant of the ZNF408 gene associated with a case of FEVR in a premature male. MATERIALS AND METHODS: Case report. RESULTS: A 10-month-old male who was born prematurely at 34 weeks' gestation in the Dominican Republic was referred for persistent avascular retina. The baby was treated with bilateral intravitreal ranibizumab injections for retinopathy of prematurity (ROP) with the presence of plus disease. Fundus examination several months after treatment revealed the absence of tortuosity of the vessels with avascular periphery; fluorescein angiography (FA) confirmed peripheral avascularity and demonstrated irregular sprouts of vascularization in the absence of neovascularization. We performed genetic testing under the suspicion of FEVR and results identified a heterozygous mutation in the ZNF408 gene on chromosome 11, c.1307 C > T. CONCLUSION: FEVR is an important differential diagnosis in premature infants with retinopathy, as clinical presentation can overlap with common findings in ROP. Maintaining high suspicion for the disease is especially critical in cases with findings unusual for ROP. FEVR in the presence of prematurity has been well described, falling under the proposed term ROPER. Genetic testing is key to confirm diagnosis.

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.

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.

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.

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.

Lens-Induced Uveitis Triggered by Intravitreal Injection 40 Years after Primary Congenital Cataract Surgery with Aphakia.

We report a case of a 42-year-old male with a history of bilateral congenital cataract surgery performed at 2 years of age. The patient was left with aphakia, secondary glaucoma, and a history of diabetic macular edema in the setting of diabetes mellitus type 1. The right eye became prephthisical from his congenital surgical repair, and his left eye presented with an acute pseudo-endophthalmitis developing after the seventh intravitreal injection to treat the macular edema. The eye then presented with decrease in vision, periocular injection, and a diffuse inflammatory reaction focused around the anterior residual lens capsule. The patient underwent surgical removal of the residual capsule and primary vitrectomy repair of the eye, achieving a significant improvement in visual symptoms and recovery of visual and anatomic function.

Case Report: Suprachoroidal Hemorrhage after Chiropractic Manipulation of the Neck.

SIGNIFICANCE: The case report highlights the possible complications of undergoing neck manipulation within a critical time period after intravitreal injection. PURPOSE: This study aimed to describe a case of traumatic hemorrhagic choroidal detachment after cervical manipulation during a chiropractic treatment session. CASE REPORT: A 43-year-old male patient with a history of complex rhegmatogenous retinal detachment repair and recurrent cystoid macular edema presented with decreased vision and sudden pain in the right eye after chiropractic manipulation of the neck, status post-intravitreal injection of triamcinolone, which was performed earlier that day. Vision in the right eye was hand motion and 20/20 in the left eye. IOPs were 8 and 11 mmHg, respectively. Slit lamp examination of the right eye revealed blood-tinged steroid residues in the anterior chamber. There was no view to the posterior pole. Ultrasonography showed a lobulated mass with heterogeneous echogenicity consistent with a large hemorrhagic choroidal detachment. No central kissing was observed. Left eye examination was unremarkable. CONCLUSIONS: With the increasing use of complementary and alternative medicine, a better understanding of potential complications to raise awareness is becoming essential.

Multimodal Imaging of Large Optic Disc Coloboma: A Report of Three Cases.

Optic disc coloboma (ODC) is a rare congenital anomaly of the optic nerve. Optical coherence tomography (OCT) helps to monitor the complications when there is associated retinoschisis, retinal detachment, or peripapillary neovascularization, while being minimally invasive. OCT angiography could help to better understand this entity from a vascular perspective. We report multimodal imaging of 3 children with large ODC associated with cat eye syndrome, CHARGE syndrome (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities), and branchio-oculo-facial syndrome.

Disaster Preparedness Impact In A Complex Ophthalmology Practice: A Review Of Patients Receiving Intravitreal Injections.

PURPOSE: A private tertiary care ophthalmology practice was analyzed to see how Hurricane Irma affected patient care. METHODS: Eighty-two patients (96 eyes) that missed their scheduled appointments due to Hurricane Irma were reviewed. Patients were being treated with intravitreal injection therapy for diabetic retinopathy, radiation retinopathy, vein occlusions, choroidal neovascularization, or neovascular age-related macular degeneration. The authors assessed patients' visual acuity and macular thickness changes before the hurricane and their initial visit after the storm. Emergency preparedness was also evaluated in this setting. RESULTS: Patients on average were delayed 19.6 days. On average, best-corrected visual acuity was 20/97 (0.7 logMAR) before the hurricane and 20/82 (0.6 logMAR) after the storm. The average central macular thickness change was -1.30 µm. Intraocular pressure was not significantly affected and there were no ocular complications associated with the delay in therapy. A back-up electrical system was in place to maintain appropriate temperature for storage of medications and proper access to medical records. CONCLUSION: The authors believe that minimizing delay in treatment was the most important contributing factor to preventing worsening of ocular disease. Ophthalmology clinics should strive for appropriate treatment modality regarding injection intervals prior to a natural disaster and emergency planning with excellent patient communication both before and after natural disasters.