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.

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.

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 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.

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.

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.

Aflibercept for Radiation Maculopathy Study: A Prospective, Randomized Clinical Study.

PURPOSE: To evaluate 2 treatment approaches to intravitreal vascular endothelial growth factor antagonist therapy in radiation maculopathy comparing aflibercept delivered by either a 6-week treatment interval or treat-and-adjust interval. DESIGN: Randomized, prospective clinical trial. METHODS: Forty consecutive patients were enrolled in an institutional review board-approved clinical trial and randomized to aflibercept treatment via 1 of 2 regimens: (1) fixed, every-6-weeks treatment or (2) variable, treat-and-adjust treatment centered around 6 weeks. All patients had a diagnosis of treated uveal melanoma with documented tumor control. All patients showed visually compromising radiation maculopathy confirmed by a decline in best-corrected visual acuity (BCVA) and spectral-domain (SD) OCT documentation of radiation maculopathy. MAIN OUTCOME MEASURES: Best-corrected visual acuity and SD OCT central retinal thickness at 1 year. RESULTS: Thirty-nine of 40 patients completed the trial (97.5%) with 1 year of follow-up. Baseline study entry BCVA was 20/63 and was maintained at 20/62 at study conclusion at 60 weeks (1 year). At baseline, SD OCT mean central retinal thickness was 432 µm and improved to 294 µm at 60 weeks (P < 0.02). At the study conclusion, 42.5% of eyes (17/40) showed better than 20/50 BCVA, and only 5% of eyes (2/40) showed a BCVA worse than 20/200. In the every-6-weeks interval treatment arm, patients received 9 injections, whereas in the treat-and-adjust study arm, patients received 8.4 injections (P = 0.88, not significant). One patient experienced an inflammatory response after aflibercept injection, but this did not occur again for this patient, nor for any other study injections (1/400 injections [0.0025%]). No patients demonstrated endophthalmitis or metastatic disease or died during the study window. CONCLUSIONS: Aflibercept seems to limit vision loss associated with radiation maculopathy. In this randomized, prospective clinical study, no difference was found between a fixed 6-week treatment interval and a variable treat-and-adjust interval because virtually all patients required treatment every 6 weeks and were not able to extend. Remarkably, almost half of all treated patients maintained BCVA of 20/50 or better throughout 1 year of treatment. Aflibercept is effective in treating radiation maculopathy, but requires an ongoing treatment approach.

Axial length development in children.

AIM: To study ocular axial lengths in pediatric subjects without intraocular pathology. METHODS: An Institutional Review Board-approved consecutive retrospective chart review of axial lengths measured in pediatric subjects who underwent examination under anesthesia due to positive family history of retinoblastoma or other inherited ocular disease. Only subjects without any intraocular pathology in either eye were included. Subjects were stratified into age groups. An axial length model using a logarithmic regression algorithm was calculated. RESULTS: Data from 330 eyes of 165 subjects were included in the study. The mean age at the time of examination was 30.62 (SD 18.04)mo. The steepest increase in axial length was present during the first 10mo of life. After 36mo, there was no statistically significant axial length growth. CONCLUSION: This study presents the biggest series of pediatric axial lengths in healthy eyes. The axial length model developed with these data may assist in the diagnosis and management of a wide variety of pediatric ophthalmic diseases.

Case Report: Importance of B-scan Ultrasonography for the Detection of Choroidal Melanoma.

SIGNIFICANCE: This case highlights the importance of using ultrasonography to evaluate an eye that has no clear view of the fundus. PURPOSE: Uveal melanoma stems from melanocytes found in the iris, ciliary body, and choroid, and it is the most common primary intraocular malignancy found in adults. The lesion is identified predominantly via fundus biomicroscopy and binocular indirect ophthalmoscopy. The authors present a unique case where visual evaluation of the fundus was not possible, and ultrasonography was used to assess the retina and choroid. CASE REPORT: A 38-year-old Hispanic man with prior ocular trauma presented with an eye that could not be clinically examined owing to complete pupil occlusion. The diagnosis of presumed uveal melanoma was made exclusively based on the outcome of ocular ultrasonography. Further histologic, antibody, and genetic testing was completed once the patient had undergone treatment of the affected eye and uveal melanoma was confirmed. CONCLUSIONS: In this rare instance, B-scan ultrasonography provided a finding that required the patient to undergo enucleation in an eye without visual potential. Genetic testing was then used to appropriately categorize the tumor as a class 1B melanoma, indicating that there is a risk of metastasis. Consequently, the patient is being monitored by a medical oncologist.

Sympathetic Ophthalmia in a 22-Month-Old Infant With Sturge-Weber Syndrome With Atypical Histopathological Correlation.

PURPOSE: Sturge-Weber syndrome (SWS) and sympathetic ophthalmia (SO) are unusual pathologies. Their association has not been previously reported. This unusual diagnosis can be elucidated by clinical suspicion. METHODS: Case report of a 22-month-old female with SO. RESULTS: SWS with a unilateral diffuse choroidal hemangioma. The exciting eye had multiple surgeries for glaucoma that required enucleation. Clinical picture of SO was found on the sympathizing eye. Atypical histopathology (nongranulomatous choroidal infiltrate and perivascular mononuclear cells) is found in the enucleated eye. CONCLUSIONS: To the best of the authors' knowledge, this is the first description of an association of SWS, diffuse choroidal hemangioma, infantile glaucoma, and SO. Many confounding factors are present (ie, multiple surgeries, risk of infectious endophthalmitis). Therefore, a high index of suspicion must be kept in mind to make the diagnosis. We aim to raise awareness of such a devastating condition in a pediatric patient with many comorbidities.