Vitreoretinal Abnormalities during Macular Hole Repair in Alport Syndrome.

PURPOSE: To describe abnormal vitreoretinal findings during macular hole repair in Alport syndrome (AS). METHODS: Case report of preoperative, intraoperative, and postoperative findings related to macular hole surgery in a patient with AS. OBSERVATIONS: A 50-year-old woman with Alport syndrome was found to have bilateral full-thickness macular holes. Surgery was recommended for her left eye given recent onset of vision loss and smaller hole size (313 um). Intraoperatively, the vitreous was found to have fine fibrillar strands and to be abnormally adherent to the retinal surface. There was little to no internal limiting membrane (ILM) present. Vitrectomy was performed with posterior cortical hyaloid peeling and 15% C3F8 gas tamponade. Two months after surgery, the macular hole was successfully closed. CONCLUSIONS: Abnormal vitreous composition and adherence to the retinal surface may contribute to macular hole formation in AS patients. Standard surgical approaches including ILM peeling may not be feasible, though thorough removal of the posterior cortical hyaloid may be sufficient to achieve macular hole closure.

Evaluation of Virtual Vitreoretinal Surgery Fellowship Interview and Match Experience.

Purpose: To evaluate applicants' and programs' experiences during the 2019 and 2020 fellowship application seasons. Methods: An anonymous survey of vitreoretinal surgery fellowship program directors (PDs) (n = 21) and applicants from the 2019 traditional (n = 24) and 2020 virtual (n = 17) match cycles (before and during COVID-19 pandemic, respectively) was conducted. The questions assessed demographics, interview experiences, and overall interview costs. Statistical significance was determined using unpaired and paired 2-sided t tests for applicants and PDs, respectively (P < .05). Results: In 2020, 17.6% of applicants and 15.8% of PDs strongly agreed that they were able to convey themselves well during the interview compared with 50% and 73.7%, respectively, in 2019 (P = .002 and P < .001, respectively). In 2020, 5.9% of applicants and 10.5% of PDs strongly agreed that they gained a good understanding of their counterpart compared with 41.7% and 47.4%, respectively, in 2019 (P < .001 and P = .01, respectively). Regarding cost, 83.3% of applicants and 21.1% of programs spent more than $2000 in 2019, whereas 17.6% of applicants and no program did so in 2020. Conclusions: Although virtual interviews allowed fellowship recruitment to continue during the pandemic, both applicants and PDs expressed uncertainty regarding their abilities to portray themselves and gauge those with whom they interviewed. The benefits of virtual interviews, including decreased cost, increased efficiency, and convenience, must be weighed against these factors.

Acute macular neuroretinopathy associated with acute promyelocytic leukemia.

PURPOSE: To describe the first reported case of acute macular neuroretinopathy (AMN) associated with acute promyelocytic leukemia in a young Asian-Indian male. OBSERVATIONS: We review the clinical and multimodal imaging findings in our patient that are characteristic of AMN. CONCLUSIONS AND IMPORTANCE: Ophthalmologists should be aware of the association of leukemia with AMN and consider hematologic work-up when assessing patients with AMN without the prototypical history or risk factors.

A Multiple Evanescent White Dot Syndrome-like Reaction to Concurrent Retinal Insults.

PURPOSE: To describe a clinical picture resembling classic multiple evanescent white dot syndrome (MEWDS) potentially triggered by previous or concurrent, apparently unrelated, ocular events and to provide a literature review of similar presentations. DESIGN: Retrospective chart series and literature review. PARTICIPANTS: Consecutive patients diagnosed with MEWDS at the Feinberg School of Medicine, Northwestern University, Chicago, Illinois, and the IRCCS San Raffaele Scientific Institute, Milan, Italy, between July 2019 and June 2020. METHODS: Charts of patients were reviewed. Ophthalmic history, best-corrected visual acuity, spectral-domain OCT results, OCT angiography results, fundus autofluorescence results, ultra-widefield fluorescein angiography results, and indocyanine green angiography results were collected. A PubMed-based search was carried out for similar presentations using the terms MEWDS and white spot syndromes. MAIN OUTCOME MEASURES: An ocular history positive for previous or concurrent ocular events in patients with MEWDS was sought in our cohort and the existing literature. RESULTS: Five eyes of 4 patients (2 females; age range, 16-81 years) were included. The first eye had a history of bilateral Best vitelliform dystrophy and unilateral choroidal neovascularization. The second eye had angioid streaks complicated by choroidal neovascularization and underwent prior thermal laser photocoagulation. The third eye had a history of high myopia and a scleral buckle procedure for retinal detachment. The fourth patient had bilateral idiopathic retinochoroiditis. We identified 16 case reports from 5 previous publications that support a MEWDS-like reaction to previous ocular insults. CONCLUSIONS: We suggest a MEWDS-like reaction may be elicited by ocular events in a subset of susceptible patients. We hypothesize that damage to the outer retina may play a role in triggering the local inflammatory response.

SEROUS MACULAR DETACHMENT ASSOCIATED WITH WALDENSTROM MACROGLOBULINEMIA MANAGED WITH IBRUTINIB: A CASE REPORT AND NEW INSIGHTS INTO PATHOGENESIS.

PURPOSE: To report a case of serous macular detachment in a patient with Waldenstrom macroglobulinemia treated with ibrutinib. METHODS: The patient underwent a complete ophthalmic examination and imaging at presentation and at follow-up visits up to 13 months. RESULTS: At presentation, there were serous macular detachments bilaterally with no dye leakage on fluorescein angiography or vasculature abnormalities on optical coherence tomography angiography. After treatment with ibrutinib, there was near resolution of the patient's retinopathy with an improvement in vision at 13 months' follow-up. CONCLUSION: Serous macular detachments in Waldenstrom macroglobulinemia-associated retinopathy may be due to the disruption of the retinal pigment epithelium pump mechanism by hyperglobulinemia. The favorable course of this patient, treated with the novel tyrosine kinase inhibitor ibrutinib, suggests this may be the preferred treatment for Waldenstrom macroglobulinemia patients with associated retinopathy.

The Role of Optical Coherence Tomography Angiography in Ranibizumab-Treated Choroidal Neovascularization in Choroidal Osteoma.

In this study, we report the initial evaluation of choroidal neovascularization (CNV) secondary to choroidal osteoma and subsequent response to anti-vascular endothelial growth factor (anti-VEGF) treatment monitored with optical coherence tomography angiography (OCT-A). A 38-year-old female presented with an initial visual acuity of 20/150 in the left eye. Clinical examination revealed a choroidal osteoma. OCT demonstrated both subretinal and intraretinal fluid. OCT-A was performed and showed CNV. A course of ten treatments with ranibizumab showed an improvement of visual acuity to 20/30-3, improvement of subretinal and intraretinal fluid, as well as attenuation of CNV. Our report demonstrates OCT-A as a useful tool for both initial evaluation of CNV and following treatment response to anti-VEGF therapy.

REVIEW OF OPHTHALMIC AND BREASTFEEDING MEDICINE EVIDENCE: Real and Theoretical Risks of Intravitreal Anti-Vascular Endothelial Growth Factor Administration in Lactating Women.

BACKGROUND/PURPOSE: There is limited research regarding the consequences of treating lactating mothers with intravitreal anti-vascular endothelial growth factor (VEGF) agents. Balancing the need for vision-saving treatment, the benefits of breastfeeding, and the concern for affecting the newborn can present a conflict for both mothers and ophthalmologists. This review summarizes the state of the literature regarding the use of intravitreal anti-VEGF agents during breastfeeding along with details about their pharmacology. RESULTS: Bevacizumab and aflibercept have Fc domains subjecting them to FcRn recycling and extending their half-life compared with ranibizumab which is an antibody fragment and lacks the Fc domain. Case reports and small studies have shown that ranibizumab has the lowest serum concentration after intravitreal injection and the least effect on plasma-free VEGF concentrations and breastmilk VEGF levels. CONCLUSION: Clinical and pharmacologic data suggest that ranibizumab has less systemic circulation and effect on maternal serum and breastmilk VEGF levels when compared to bevacizumab and aflibercept. However, there is significant need for further research on the degree and duration to which intravitreal agents circulate systemically, what fraction is transferred into breastmilk and is absorbed, and whether this results in any functional adverse effects to the infant. Other factors to consider in the medical decision-making of lactating mothers necessitating intravitreal anti-VEGF treatment include the gestational and post-natal age of the child and whether it is feasible to avoid breastfeeding for the half-life duration of the intravitreal agent rather than ceasing breastfeeding altogether.

Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman.

PURPOSE: To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. OBSERVATIONS: Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. CONCLUSIONS AND IMPORTANCE: Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology.

Morphological Implications of Vascular Structures Not Visualized on Optical Coherence Tomography Angiography in Retinal Vein Occlusion.

BACKGROUND AND OBJECTIVE: Advanced retinal imaging can improve understanding of retinal vein occlusion (RVO) pathology. The authors aimed to characterize the vascular pathology of RVO on en face optical coherence tomography (OCT) and OCT angiography (OCTA). PATIENT AND METHODS: This was a cross-sectional study including 17 eyes with RVO. The authors identified discordance between vasculature on en face OCT and flow on OCTA, which was correlated with structural findings at the corresponding location on OCT B-scans. RESULTS: Six eyes had vessels that were seen on OCT without flow on OCTA. The most clinically relevant finding was preserved inner retinal layers in areas where the en face OCT showed collaterals that appeared nonperfused on OCTA. CONCLUSIONS: The authors' findings indicate that collaterals can appear on en face OCT without flow on OCTA in RVO and may be associated with relatively preserved inner retinal structures. Clinicians should consider multimodal imaging to evaluate RVO, including both OCT and OCTA. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:392-396.].

Calcified amorphous tumor: A rare cause of central retinal artery occlusion.

PURPOSE: We report the case of a central retinal artery occlusion secondary to presumed embolus from a calcified amorphous tumor of the heart, a very rare non-neoplastic cardiac mass. OBSERVATIONS: A 60-year-old female presented with acute unilateral vision loss of the left eye. Examination revealed hand motion visual acuity of the left eye and a left relative afferent pupillary defect. Fundoscopy showed whitening of the macula with a cherry red spot, consistent with a central retinal artery occlusion. Initial workup was unremarkable, including hypercoagulability labs, magnetic resonance imaging of the brain, and magnetic resonance angiography of the head and neck. Transthoracic echocardiogram (TTE) showed calcification of the mitral valve but no masses. Subsequently, transesophageal echocardiogram (TEE) was performed, which revealed a mobile calcified amorphous tumor of the heart. CONCLUSIONS: Calcified amorphous tumor of the heart is a very rare cardiac mass that may cause retinal artery occlusion. TEE is a more sensitive imaging modality to assess for potential cardio-embolic sources if TTE is unrevealing.

Amiodarone: A potential risk factor for retinal phototoxicity.

PURPOSE: To report the only known case, to our knowledge, of amiodarone induced retinal phototoxicity following vitrectomy surgery. OBSERVATIONS: A 66-year-old male presented with visual acuity of 20/150 OS secondary to an epiretinal membrane (ERM). Patient was on oral amiodarone for atrial fibrillation. Baseline spectral domain optical coherence tomography (SD-OCT) revealed an ERM with retinal thickening and schisis. The patient underwent an uncomplicated pars plana vitrectomy and membrane peel using standard vitrectomy settings and illumination. Triamcinolone was used to stain the ERM intraoperatively. ICG was not used. On post-operative day one, vision was count finger (CF) at 1'. At post-operative week one, vision was unchanged and SD-OCT showed macular edema. At post-operative month one, vision remained CF at 1' and macular edema resolved with residual pigmentary changes and subretinal fibrosis resembling phototoxic damage. SD-OCT at one month showed resolution of macular edema, retinal pigment epithelium hyperplasia and an indistinct ellipsoid layer. Fluorescein angiography did not show any neovascularization. At three month follow-up, patient's vision, exam and OCT findings remained unchanged. CONCLUSIONS AND IMPORTANCE: Many pharmacologic agents have the ability to alter a patient's sensitivity to solar or artificial radiation. Drugs act as photosensitizers that lead to photochemical damage. Amiodarone has been reported to have such photosensitizing properties in humans. This report describes a case of retinal phototoxicity from intraoperative light exposure photosensitized by systemic amiodarone use.

SEMIAUTOMATED QUANTITATIVE APPROACH TO CHARACTERIZE TREATMENT RESPONSE IN NEOVASCULAR AGE-RELATED MACULAR DEGENERATION: A Real-World Study.

PURPOSE: To perform a quantitative study of the vascular microstructure in actively treated choroidal neovascularization by optical coherence tomographic angiography. METHODS: Patients undergoing individualized anti-vascular endothelial growth factor therapy of minimum 12 months duration were included in this cross-sectional observational study and imaged using optical coherence tomographic angiography. En face optical coherence tomographic angiography images were analyzed for quantitative features, such as junction density, vessel length, and lacunarity using validated software (Angiotool). Patients were divided into 2 groups depending on their individualized treatment interval: "good responders, treated less frequently than 6 weeks" versus "poor responders, treated every 6 weeks or more frequently." Nonparametric testing was used to assess differences between these groups. RESULTS: Twenty-five eyes of 23 consecutive patients with a median 58-month history of choroidal neovascularization, treated by median of 34 anti-vascular endothelial growth factor injections, were included in the analysis. There was no significant difference between any of the microvascular choroidal neovascularization features between the 2 groups (P > 0.05). CONCLUSION: The semiautomated vessel segmentation software provides an objective and quantitative approach for choroidal neovascularization characterization. The consistently nonsignificant outcomes between the groups may provide evidence to support the "normalization hypothesis." This would suggest that regardless of treatment interval, individualized therapy in these eyes established vessel stability.

Relapsed Burkitt Lymphoma Presenting as an Isolated Infiltrative Optic Neuropathy.

We report a case of relapsed sporadic Burkitt lymphoma (BL) presenting as an isolated infiltrative optic neuropathy. This is a single-patient, retrospective case report of a patient seen by the ophthalmology service at our institution. To our knowledge, our case is the first to report isolated infiltrative optic neuropathy associated with sporadic BL as the sole manifestation of recurrence. The possibility of disease relapse should be considered for patients with a history of lymphoreticular malignancy who present with acute visual symptoms suggestive of optic neuropathy.

Fundus Autofluorescence Patterns of Submacular Fluid Resolution Following Repair of Macula-Involving Rhegmatogenous Retinal Detachments.

BACKGROUND AND OBJECTIVE: Submacular fluid (SMF) can persist for months to years following rhegmatogenous retinal detachment (RD) repair. The authors' objective was to describe fundus autofluorescence (FAF) and optical coherence tomography (OCT) findings associated with the absorption of persistent submacular fluid (SMF) following RD repair. PATIENTS AND METHODS: Retrospective review of clinical data and FAF and OCT imaging from sequential postoperative visits in a cohort of patients with persistent SMF following RD repair. RESULTS: In 11 of 13 eyes with persistent SMF, patches of decreased FAF signal corresponded to SMF on OCT. In eight eyes, there was a hypo- to hyperautofluorescence transition at the time of SMF resolution. These areas of increased FAF signal correlated with inner segment/outer segment (IS/OS) junction loss on OCT. CONCLUSION: FAF imaging can be informative when following SMF after RD repair; a hypo- to hyper-FAF signal transition correlates with SMF resolution and photoreceptor loss. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1020-1029.].

Long-Term Evaluation of MEK Inhibitor Retinal Toxicity With Multimodal Imaging.

MEK inhibitor chemotherapy, used in various malignancies, has been reported to be associated with serous retinal detachments. This study reports serial multimodal imaging focused primarily on infrared reflectance and spectral-domain optical coherence tomography (SD-OCT) in the monitoring of MEK inhibitor retinal toxicity. The benefit of OCT angiography in understanding the pathophysiology of MEK inhibitor toxicity is also shown.

Quantification of Change in Pigment Epithelial Detachment Volume and Morphology After Transition to Intravitreal Aflibercept in Eyes With Recalcitrant Neovascular AMD: 18-Month Results.

BACKGROUND AND OBJECTIVE: To quantitatively evaluate the change in pigment epithelial detachment (PED) morphology on spectral-domain optical coherence tomography (SD-OCT) 18 months after the transition to intravitreal aflibercept in patients with neovascular age-related macular degeneration (AMD) with PED recalcitrant to monthly intravitreal bevacizumab or ranibizumab. PATIENTS AND METHODS: Retrospective case series examining patients with neovascular AMD who had a persistent fibrovascular or serous PED on SD-OCT. PED volume was calculated by manually outlining the PED on individual OCT slices of the raster scan and multiplying by the pixel dimensions. RESULTS: Eleven eyes of 10 patients who had received an average of 25.7 ± 20.1 (range: 6 to 70) prior bevacizumab or ranibizumab injections over a period of 26.6 ± 19.8 months (range: 4 to 63) were included. PED volume decreased with aflibercept from 0.687 ± 0.837 mm(3) to 0.562 ± 0.705 mm(3) (P = .02), a decrease of 19% ± 12.27%. CONCLUSION: After 18 months of aflibercept, recalcitrant PED volumes were reduced by 19% while preserving visual acuity in eyes with neovascular AMD.

Small hyperreflective intraretinal material observable on spectral-domain optical coherence tomography in high myopia.

With the rapid adoption of spectral-domain optical coherence tomography (SD-OCT) in clinical practice and recent advances in technology, SD-OCT has become an important tool for detecting and managing retinal pathology. The diagnosis of degenerative changes in pathologic myopia can sometimes be difficult and delayed, as the clinical presentation may be subtle. In this series, SD-OCT proved useful in the detection of small hyperreflective intraretinal material in cases in which retinal changes are minimally visible and may explain visual changes that may not have been otherwise elucidated. The results show that SD-OCT in conjunction with clinical examination may provide useful information in the management of patients with pathologic myopia.

Suprachoroidal silicone oil migration following retinal detachment repair.

Silicone oil is a commonly used tamponade agent. We report the rare complication of a patient who presented with silicone oil in the suprachoroidal space following retinal detachment repair. The silicone oil was subsequently removed without any long-term complications.

Melanocytoma of the optic disc associated with visual field defects: clinical features and imaging characteristics.

The authors studied the clinical features, visual field, and spectral-domain optical coherence tomography (SD-OCT) characteristics of peripapillary melanocytoma associated with visual field defects in two eyes of two patients. Both eyes had hyperreflective anterior surface and posterior optical shadowing in the region of peripapillary melanocytoma on SD-OCT imaging. The retinal nerve fiber layer was thin adjacent to these lesions, and this corresponded to the opposite hemifield defect on Humphrey Visual Field testing. Three-dimensional imaging of the melanocytoma revealed an irregular elevation in the peripapillary region. SD-OCT, when used in conjunction with clinical examination and Humphrey Visual Field testing, provided useful information that may assist clinicians in treating patients with peripapillary melanocytoma, particularly when observing them over time.