Revision of Initial Referral Diagnosis following Genotypic Confirmation of Familial Exudative Vitreoretinopathy.

PURPOSE: To describe common referral diagnoses of FEVR (Familial Exudative Vitreoretinopathy) DESIGN: Retrospective case series. SUBJECTS: Patients with phenotypic FEVR evaluated by the pediatric retina service at Bascom Palmer Eye Institute from March 1, 2019 to May 31, 2023. METHODS: A retrospective detailed review of history, imaging, and genetic testing was performed for all patients included in the study. MAIN OUTCOME MEASURES: The primary outcome measures included genetic evaluation, clinical imaging and characteristics. Secondary outcome measures were age and stage of disease at diagnosis. RESULTS: Fifty-five patients with phenotypic and genetically confirmed FEVR were identified. Of these, the initial diagnosis was incorrect upon referral in 33 cases (60%). The referral diagnoses included Coats disease (n =2, 7%), toxoplasmosis (n=2, 7%), retinal detachment (n=6, 18%), persistent fetal vasculature (PFV) (n =5, 15%), suspected inherited retinal disease (n=2, 7%), myopia (n=3, 9%), retinopathy of prematurity (ROP) with preterm gestational age (n=7, 21%), and ROP at moderate to late preterm gestational age (n=6, 18%). The mean age at diagnosis was 8.78 years and 6.42 years for patients with revised diagnosis versus initial diagnosis of FEVR, respectively (p =0.4). There was no correlation between age of diagnosis and stage of disease (p =0.8). CONCLUSIONS: A high index of suspicion for FEVR must be maintained given the heterogeneity of phenotypes. Atypical cases of PFV, Coats disease, and ROP should prompt fluorescein angiography and genetic evaluation.

Clinical Outcomes in Patients With Traumatic Dehiscence of Penetrating Keratoplasty Grafts.

PURPOSE: The purpose of this study was to report clinical characteristics and outcomes of surgical repair for patients with traumatic dehiscence of penetrating keratoplasty (PKP) grafts. METHODS: Retrospective, consecutive chart review of patients evaluated at Bascom Palmer Eye Institute between 2015 and 2020 with traumatic dehiscence of penetrating keratoplasty grafts. RESULTS: The study cohort consisted of 65 eyes of 65 patients. The mean age at presentation was 72 years (SD 18), with a male predominance (65%). The most common indications for PKP included keratoconus (42%), corneal scar (31%), and Fuchs corneal dystrophy (8%). Dehiscence occurred as a result of blunt trauma in 94% of cases, and the mean wound length was 6.4 clock hours (SD 2.4), with a predominance of inferior dehiscence. The mean presenting visual acuity (VA) was 2.45 logMAR (SD 0.41), and the mean final VA was 2.17 logMAR (SD 0.99). Graft failure occurred in 64% of patients, and 22% underwent repeat PKP. When stratified by indication for corneal transplantation (keratoconus vs. other), there was no significant difference in graft age at the time of rupture, final VA, rate of graft failure, or rate of repeat PKP. CONCLUSIONS: Traumatic dehiscence of corneal grafts remains a rare but serious subtype of ocular trauma with generally poor visual prognoses. Presenting VA along with severity of trauma and posterior segment involvement tend to be the worst prognostic factors in final visual outcome.

Patients With Dragged Optic Disc Vessels and Retinal Folds: Clinical Features, Multimodal Imaging, and Histopathology.

BACKGROUND AND OBJECTIVE: To describe the multimodal imaging and histopathological features of patients with dragged optic disc vessels (DODV). PATIENTS AND METHODS: This is a retrospective, observational analysis using multimodal imaging of eyes with DODV in patients with retinal vascular diseases including familial exudative vitreoretinopathy, inflammatory disease, and others. In addition, two additional enucleated eyes with DODV underwent histopathological analysis. RESULTS: Of the 13 patients, eight were girls and five were boys. Mean age was 5 years (ranging from 4 months to 10 years old). Of the 15 eyes, 12 (80%) demonstrated temporal dragging and three (20%) had nasal dragging. Retinal vascular abnormalities were present in 11 of the fellow eyes. Multimodal imaging demonstrated features of DODV including direction of traction, outer retinal thickening, increased flow, and other features. Only two (13.3%) eyes required surgical intervention. Mean follow-up was 14 (range 3 to 30 months) months. At last follow-up all eyes remained stable. Last visual acuity ranged from 20/100 to counting fingers. As well, two enucleated globes of adults with retinopathy of prematurity underwent histopathologic evaluation, showing optic nerve fibers that extended from the optic nerve into the DODV. CONCLUSIONS: DODV is a sign of various late-stage retinal vascular diseases, associated with poor visual function. Multimodal imaging and histopathology can assist in understanding the disease pathology. [Ophthalmic Surg Lasers Imaging Retina 2023;54:634-642.].

Intraoperative OCT Angiography in Pediatric Patients with Persistent Fetal Vasculature.

PURPOSE: To describe abnormalities of the optic nerve microvasculature in patients with persistent fetal vasculature (PFV) and their fellow eyes using OCT angiography (OCTA). DESIGN: Cross-sectional study. SUBJECTS: Fifty eyes of 25 patients with PFV who underwent prospective imaging using supine OCTA during examination under anesthesia at Bascom Palmer Eye Institute from March 1, 2019, to December 31, 2022. METHODS: OCT angiography images of the optic nerve of the included patients were analyzed with a primary focus on blood flow. Demographic, clinical, and treatment factors were compared with morphologic changes in the optic disc microvasculature. MAIN OUTCOME MEASURES: Prevalence of optic nerve microvascular abnormalities on OCTA in the affected and fellow eyes of patients with PFV. RESULTS: A total of 50 eyes from 25 patients were reviewed, and 28% (7/25) met image quality criteria for OCTA analysis. Optic nerve OCTA showed a persistent hyaloid artery (PHA) in all (7/7) PFV eyes analyzed. Of these, flow on OCTA was detectable in 57% (4/7). A Bergmeister papilla was evident in 100% (25/25) fellow eyes, of which flow was detected in 68% (17/25). Fluorescein angiography (FA) demonstrated blood flow within the stalk in 40% (10/25) of PFV eyes and within the Bergmeister papilla in 25% (6/25) of fellow eyes. Similar findings of abnormal blood flow and presence of fibrovascular stalk were seen in both treatment-naïve and treated groups. CONCLUSIONS: OCT angiography allows for high-resolution visualization of subtle vascular abnormalities that are not readily apparent using RetCam FA and may serve as a useful noninvasive test to confirm the patency of the PHA and Bergmeister papilla in children. The results of the present study suggest that PFV may be a bilateral and asymmetric process. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Evolving Use of Regional versus General Anesthesia for the Surgical Repair of Open Globe Injuries.

PURPOSE: The purpose of the current study is to report outcomes with the evolving use of regional anesthesia with monitored anesthesia care (RA-MAC) vs general anesthesia (GA) in the repair of open globe injuries. DESIGN: Retrospective, consecutive, comparative, nonrandomized clinical study. METHODS: The study includes 507 eyes of 507 patients with open globe injuries treated with either RA-MAC or GA at a tertiary referral center between 2015 and 2020. There was no predetermined protocol for selection of anesthesia method. However, based on experience and findings of prior research by this group, regional anesthesia with monitored anesthesia care was typically selected initially and changed to general anesthesia if warranted after evaluation of the patient and discussion with the surgeon. The main outcome measure was visual acuity at last follow-up. Results were compared to previously published study groups between 1995 and 2014. RESULTS: Primary closure of open globe injury was performed under RA-MAC anesthesia in 462 patients (91%) and under GA in 45 patients (9%). Zone 1, 2, and 3 injuries were recorded in 251, 170, and 86 patients, respectively. Zone 1 (96%, 240 of 251 patients) or zone 2 (92%, 156 of 170 patients) (P < .001) were more likely to be repaired under RA-MAC vs zone 3 injuries (76%, 65 of 86 patients). The improvement from presenting visual acuity was similar for the 2 anesthesia groups, 0.52 logMAR and 0.46 logMAR for RA-MAC and GA, respectively (P = .68, CI -0.3 to 0.2). The use of RA-MAC anesthesia for open globe injuries has increased at our institution from 64% in 1995-1999 to 91% in the present study, 2015-2020 (P < .00001). CONCLUSION: The current study demonstrates that with anesthesiologists experienced in ophthalmic regional anesthesia techniques, and appropriate case selection, RA-MAC can be safely used as an alternative to general anesthesia for open globe repair. Considerations when employing RA-MAC include a patient's ability to cooperate, position, and communicate for the duration of the globe repair.

ACUTE- AND DELAYED-ONSET ENDOPHTHALMITIS AFTER OPEN GLOBE INJURIES: Clinical Features and Visual Acuity Outcomes.

PURPOSE: The purpose of the study was to report the clinical features and best-corrected visual acuity outcomes in patients with acute- and delayed-onset endophthalmitis after open globe injuries. METHODS: The study included a retrospective, comparative, consecutive case series of patients with endophthalmitis after open globe injury between January 2016 and October 2020 at the Bascom Palmer Eye Institute. RESULTS: Acute-onset endophthalmitis accounted for 16 of 20 cases (80%), and all cases were diagnosed at the initial examination. Delayed-onset endophthalmitis cases, occurring more than 2 weeks after injury, accounted for 4 of 20 cases (20%) and were because of Zone 1 wound leaks and infections. Factors associated with endophthalmitis included presence of a retained intraocular foreign body (11/20 [55%]) and delay of presentation >24 hours (15/20 [75%]) ( P < 0.001 and 0.002, respectively). The mean presenting best-corrected visual acuity was logMAR 1.64 (20/800), and the mean best-corrected visual acuity at the last follow-up was logMAR 1.22 (20/300). CONCLUSION: In patients with open globe injury-related endophthalmitis, visual acuity outcomes are generally poor. Despite intravitreal antibiotics at primary closure, delayed-onset endophthalmitis cases may develop in the setting of compromised Zone 1 wound integrity.

CENTRAL RETINAL VEIN OCCLUSION IN 12-YEAR-OLD GIRL WITH METHYLENETETRAHYDROFOLATE REDUCTASE MUTATION: A CASE REPORT AND REVIEW OF THE LITERATURE.

PURPOSE: This case report describes a central retinal vein occlusion in a healthy 12-year-old girl who developed retinal neovascularization at 24 years of age. To the knowledge of the authors, this is the longest time between a reported pediatric central retinal vein occlusion event and neovascularization. METHODS: The patient underwent a full history, physical examination, and laboratory workup to determine potential risk factors contributing to the vascular event. Fundus photographs, optical coherence tomography, and fluorescein angiography were performed throughout the patient's treatment course. RESULTS: Family history was noncontributory, but laboratory testing revealed a mildly elevated homocysteine level and homozygous C677T mutation in methylenetetrahydrofolate reductase. As a result, she was started on folate supplementation. The patient has had no further ocular or systemic thrombotic events to date. CONCLUSION: Pediatric patients presenting with central retinal vein occlusion should undergo a systemic workup and require long-term follow-up to avoid complications, such as intraocular hemorrhage, tractional retinal detachments, and neovascular glaucoma.

MULTIMODAL IMAGING OF BILATERAL IDIOPATHIC MULTIFOCAL RETINAL PIGMENT EPITHELIAL DETACHMENTS IN YOUNG PATIENTS.

PURPOSE: The aim of this study was to describe two cases of multiple epithelial detachments (pigment epithelial detachments [PEDs]) occurring in otherwise young, healthy patients. METHODS: The medical and imaging records of two cases were reviewed retrospectively. RESULTS: Multiple serous nonvascularized PEDs were present in two young, healthy patients who underwent multimodal imaging. The first patient, a 38-year-old woman, was incidentally found to have a visual acuity of 20/20 in both eyes and multiple bilateral PEDs. Imaging and a systemic work-up was negative for secondary causes, and at 36 months, vision remained stable although some of the PEDs had coalesced. The second patient, a 32-year-old woman, presented with distortion and perceived scotomas in both eyes progressive over the course of 2 years. The visual acuity was 20/20, and multimodal imaging confirmed the presence of bilateral PEDs. CONCLUSION: Bilateral idiopathic multifocal retinal PEDs in otherwise healthy young adults is a rare condition. Longer term follow-up is needed to evaluate secondary complications and visual outcomes.

AN 82-YEAR-OLD WOMAN WITH RETINAL VASCULAR SHEATHING AND VITREOUS HEMORRHAGE.

PURPOSE: This report describes a case of hereditary transthyretin amyloidosis in an 82-year-old woman who presented with bilateral retinal arteriolar vascular sheathing and vitreous hemorrhage in one eye. METHODS: The patient underwent a full history, physical examination, genetic workup, and cardiac imaging to establish the diagnosis. Fundus photographs, optical coherence tomography, fluorescein angiography, and indocyanine green angiography were performed throughout the patient's treatment course. RESULTS: Fundus examination demonstrated segmental sheathing of the retinal arterioles, telangiectasias, and intraretinal hemorrhages in both eyes without neovascularization. Fluorescein angiography revealed delayed filling of the nasal retinal arterioles in the right eye with severe temporal nonperfusion and areas hyperfluorescent segmental sheathing in both eyes. Ocular coherence tomography demonstrated retinal vessels with hyperreflective thickened walls. Cardiac amyloid nuclear scan was consistent with transthyretin-mediated amyloidosis amyloid heart disease, and genetic testing confirmed a heterozygous pathogenic transthyretin mutation: c.290 C>A (p.Ser97Tyr) also known as Ser77Tyr. CONCLUSION: Amyloidosis should be considered in the differential of vasculitis and vitreous hemorrhage with or without significant vitreous opacities. Ophthalmologists aware of this diagnosis should complete a full review of systems with specific focus on cardiac history and symptoms of peripheral neuropathy when amyloidosis is in the differential.

Outcomes in Patients with Suprachoroidal Hemorrhage After Anterior Segment Surgery.

Objective: The purpose of the current study is to report outcomes of suprachoroidal hemorrhage (SCH) after anterior segment surgery at a single institution, and to identify clinical features associated with visual prognosis. Methods and Analysis: Retrospective consecutive case series of patients with SCH occurring after anterior segment surgery. Results: The study includes 112 eyes of 112 patients between 2014 and 2020. There were 76 cases of non-appositional SCH versus 36 cases of appositional SCH. The mean presenting visual acuity for patients with non-appositional versus appositional SCH was 2.03 logMAR (SD 0.78) versus 2.39 logMAR (SD 0.43), respectively. Visual acuity outcomes generally remained poor at last follow-up: 64 (58%) patients had a visual acuity (VA) of ≤ 20/200, including 19 (17%) with light perception (LP), and 11 (10%) with no light perception (NLP). Regarding management of non-appositional versus appositional SCH, observation was selected in 46 (61%) vs 12 (33%), delayed drainage in 14 (18%) vs 15 (42%), delayed pars plana vitrectomy in 16 (21%) vs 13 (36%), and VA at last follow-up was 1.2 versus 1.86 logMAR (p=0.002). In patients that were observed, both appositional SCH (p=0.01) and duration of apposition (p=0.04) were correlated with worse outcome. Conclusion: Appositional SCH was associated with poorer visual outcomes compared to non-appositional SCH. Observation remains a reasonable management strategy for non-appositional SCH.

Reusable Fundus Model for Laser Retinopexy Simulation and Training.

BACKGROUND AND OBJECTIVE: To describe a simulation technique for laser barrier retinopexy as an option for ophthalmologists in training to practice slit lamp and laser indirect ophthalmoscopy (LIO) retinopexy. MATERIALS AND METHODS: A commercially available fundus model and laser printer labels were used to simulate laser retinopexy of horseshoe tears (HST). At two time points, seven first-year residents and seven second-year residents were asked to complete three rows of laser burns around HSTs and the times were recorded. RESULTS: Second-year residents completed simulated retinopexies in less time than first-year residents on both the slit lamp (P = .001) and with LIO (P = .001). Second-year residents were also statistically more efficient when retested at the second time point (P = .003 and P = .005, slit lamp and LIO, respectively). CONCLUSION: This novel educational model is able to capture differences in training level and increased experience among ophthalmology trainees, and may be beneficial as part of a structured teaching program. [Ophthalmic Surg Lasers Imaging Retina 2022;53:606-609.].

Role of corneal biopsy in the management of infectious keratitis.

PURPOSE OF REVIEW: The aim of this study was to review the existing literature and investigate the role of microbiologic culture and histopathologic examination of corneal biopsies in the management of infectious keratitis. RECENT FINDINGS: Corneal biopsy continues to be a significantly useful tool in the diagnosis and tailored management of infectious keratitis. Several techniques can be employed for tissue collection, handling and processing to optimize diagnostic yield and maximize safety, including emerging femtosecond laser-assisted biopsy. SUMMARY: Corneal opacities represent a significant cause of global blindness, and infectious keratitis is the most common cause. Organism identification in progressive infectious keratitis is essential for proper management. However, microbiological culture alone has a high rate of false-negative results. Records from the Bascom Palmer Eye Institute were retrospectively searched for patients between 1 January 2015, and 31 December 2019, who underwent corneal biopsy, therapeutic keratoplasty or endothelial graft removal for infectious keratitis and had specimens bisected and submitted for evaluation with both microbiologic culture and histopathologic examination. Detection of bacteria, fungus and mycobacteria was not statistically different between culture and histopathology. Microbiology and histopathology are complementary methods for the identification of causative microorganisms in corneal specimens with presumed infectious keratitis.

CONCOMITANT PYOGENIC LIVER AND INTRAOCULAR ABSCESSES IN KLEBSIELLA ENDOGENOUS ENDOPHTHALMITIS: CASE REPORT AND REVIEW OF LITERATURE.

PURPOSE: To describe a rare presentation of Klebsiella pneumoniae endogenous endophthalmitis with concurrent intraocular and liver abscesses. METHODS: Case Report. RESULTS: A middle-aged man presented with a red and painful eye with a preceding febrile gastrointestinal illness. Examination and work-up revealed concomitant intraocular and liver abscesses that were treated by pars plana vitrectomy, drainage of subretinal abscess, inpatient admission, and systemic antibiotics. The patient ultimately underwent enucleation of the eye because of persistent pain and poor visual prognosis. CONCLUSION: Klebsiella pneumoniae endogenous endophthalmitis is a rare and potentially devastating disease that requires high clinical suspicion and early diagnosis. Practitioners should consider systemic etiologies and communicate guarded prognoses in this serious systemic disease.

Evaluation of Available Online Information Regarding Treatment for Vitreous Floaters.

OBJECTIVE: To assess the quality, content, and readability of information available online on vitreous floater information. DESIGN: Cross-sectional study. PARTICIPANTS: Not applicable. METHODS: Websites were generated using a Google search of "vitreous floaters treatment" and "[State]" and were analyzed using a standardized checklist of 22 questions. Readability was assessed using the Flesch Reading Ease score. Websites met qualification criteria if they represented U.S.-based institutions, if they provided clinical care and addressed vitreous floater treatment on their website. RESULTS: Of the 1,065 websites screened, 456 were included. Of these, 406 (89%) were private institutions, 24 (5.3%) were academic, and 26 (5.7%) were a combination of private and academic. The average readability score correlated to a 10th-12th grade reading level. Vitreous floater treatment was discussed on 283 (62.1%) websites and 63 (21.8%) websites discussed potential side effects. Google rank was inversely correlated with the depth of explanation (r = -0.114, p = .016). Observation was the main treatment recommended (55.8%, n = 158), followed by laser treatment (27.6%, n = 78), no specific treatment recommendation (11.3%, n = 32), and vitrectomy (5.3%, n = 15). Centers with vitreoretinal surgeons were 16.43 times more likely to recommend vitrectomy than those without vitreoretinal surgeons (p < .001). CONCLUSIONS: Online information about vitreous floater treatment is variable, and the material is at a higher than recommended reading level for health information. While treatment was discussed by nearly two thirds of websites, less than a quarter mentioned possible complications, and treatment recommendations varied significantly depending on physician training.

Cortical projections to the two retinotopic maps of primate pulvinar are distinct.

Comprised of at least five distinct nuclei, the pulvinar complex of primates includes two large visually driven nuclei; one in the dorsal (lateral) pulvinar and one in the ventral (inferior) pulvinar, that contain similar retinotopic representations of the contralateral visual hemifield. Both nuclei also appear to have similar connections with areas of visual cortex. Here we determined the cortical connections of these two nuclei in galagos, members of the stepsirrhine primate radiation, to see if the nuclei differed in ways that could support differences in function. Injections of different retrograde tracers in each nucleus produced similar patterns of labeled neurons, predominately in layer 6 of V1, V2, V3, MT, regions of temporal cortex, and other visual areas. More complete labeling of neurons with a modified rabies virus identified these neurons as pyramidal cells with apical dendrites extending into superficial cortical layers. Importantly, the distributions of cortical neurons projecting to each of the two nuclei were highly overlapping, but formed separate populations. Sparse populations of double-labeled neurons were found in both V1 and V2 but were very low in number (<0.1%). Finally, the labeled cortical neurons were predominately in layer 6, and layer 5 neurons were labeled only in extrastriate areas. Terminations of pulvinar projections to area 17 was largely in superficial cortical layers, especially layer 1.