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.].

Endophthalmitis caused by Burkholderia cepacia complex (BCC): clinical characteristics, antibiotic susceptibilities, and treatment outcomes.

PURPOSE: To report the clinical characteristics, antibiotic susceptibilities, and review the literature of Burkholderia cepacia complex (BCC) associated endophthalmitis. STUDY DESIGN: Retrospective, observational case series. METHODS: Clinical and microbiology records were reviewed for patients evaluated at the Bascom Palmer Eye Institute and diagnosed wisth culture-confirmed endophthalmitis due to BCC. Antibiotic susceptibility profiles were generated using standard microbiologic protocols via an automated VITEK system. RESULTS: Endophthalmitis associated with BCC was diagnosed in three patients. Infection occurred in the setting of post-penetrating keratoplasty (PKP), glaucoma filtering surgery, and suspected trauma. All isolates demonstrated in vitro susceptibility to ceftazidime and meropenem. Presenting visual acuity (VA) ranged from hand motion to light perception. Initial treatment strategies included intravitreal ceftazidime (2.25 mg/0.1 mL) and vancomycin (1.0 mg/0.1 mL) injections with fortified topical antibiotics in 2 patients, and surgical debridement of a corneoscleral melt with patch graft along with both topical fortified antibiotics oral antibiotics in the third patient. In all 3 patients, there was no VA improvement at last follow-up, as 2 eyes ultimately underwent enucleation and 1 eye exhibited phthisis bulbi at last follow-up. BCC related endophthalmitis was reviewed among 13 reports. Treatment outcomes were generally poor and antibiotic resistance was common. These BCC isolates cases demonstrated broad resistance patterns, with susceptibilities to ceftazidime (58%), ciprofloxacin (53%), and gentamicin (33%). CONCLUSIONS: Endophthalmitis caused by B. cepacia is a rare clinical entity with generally poor visual outcomes despite prompt treatment with appropriate antibiotics.

NATIONWIDE DEMOGRAPHIC DISPARITIES IN UNITED STATES EMERGENCY DEPARTMENT VISITS IN PATIENTS WITH RHEGMATOGENOUS RETINAL DETACHMENT.

BACKGROUND/PURPOSE: Within the evolving landscape of healthcare in the United States (US), delineating the demographic nuances and financial implications of emergent conditions, such as rhegmatogenous retinal detachment (RRD), is paramount. This study seeks to analyze the demographic and hospital billing amount/cost of service disparities in RRD visits to emergency departments (EDs) nationwide. METHODS: We conducted a retrospective, cross-sectional, population-based study using International Classification of Diseases, 10th revision , and Current Procedural Terminology codes in the 2016 to 2019 Nationwide Emergency Department Sample databases to identify RRD visits. The analysis included demographics, hospital billing amount, and cost of service of RRD ED management. RESULTS: A total of 12,492 RRD encounters were identified with men constituting 64% and a prominent age group being 50 to 64 years (49.3%). Most patients (90%) were managed in metropolitan teaching hospitals, predominantly in the southern U.S. region (56.1%). Private insurance covered 45% of patients. Same-day RRD repair odds increased in November and December. Whites had a higher likelihood for same-day treatment. Hospital billing amount rose from $23,600 in 2016 to $30,354 in 2019, with stable mean total cost of service. Rhegmatogenous retinal detachment ED visit incidence did not show seasonal variation ( P = 0.819). CONCLUSION: Most patients with RRD in U.S, EDs were middle-aged men, with Whites more likely to receive same-day repair. There was no sex bias observed in same-day repair decision-making. Although hospital billing amount increased over the study period, total cost of service remained stable. The incidence of RRD ED visits showed no seasonal variation.

Retinal displacement following repair of rhegmatogenous retinal detachment.

Retinal displacement following the repair of rhegmatogenous retinal detachment (RRD) has been reported in recent studies. It was described as vertical movement of the retinal relative to its original location, as evidenced by retinal vessel printing on fundus autofluorescence imaging. This review reports the current literature on retinal displacement. We conducted an English literature search using Medline, PubMed, Embase, and Web of Science. We have reviewed 22 articles describing the diagnosis, frequency, and possible risk factors for retinal displacement. The reported rate of retinal displacement ranged from 6.4% to 62.8%, and the possible risk factors included the detachment-to-repair time, location and extent of RRD, macula-off RD, presence of subretinal fluid, use of perfluorocarbon, use of tamponade, postoperative facedown positioning, type of RRD repair, and presence of proliferative vitreoretinopathy, especially high grade. This review increases awareness of retinal displacement and its associated visual effects.

Endophthalmitis caused by Burkholderia cepacia complex (BCC): Clinical characteristics, antibiotic susceptibilities, and treatment outcomes.

Purpose: To report the clinical characteristics, antibiotic susceptibilities, and review the literature of Burkholderia cepacia complex (BCC) associated endophthalmitis. Study design: Retrospective, observational case series. Methods: Clinical and microbiology records were reviewed for patients evaluated at the Bascom Palmer Eye Institute and diagnosed with culture-confirmed endophthalmitis due to BCC. Antibiotic susceptibility profiles were generated using standard microbiologic protocols via an automated VITEK system. Results: Endophthalmitis associated with BCC was diagnosed in three patients. Infection occurred in the setting of post-penetrating keratoplasty (PKP), glaucoma filtering surgery, and suspected trauma. All isolates demonstrated in vitro susceptibility to ceftazidime and meropenem. Presenting visual acuity (VA) ranged from hand motion to light perception. Initial treatment strategies included intravitreal ceftazidime (2.25 mg/0.1 mL) and vancomycin (1.0 mg/0.1mL) injections with fortified topical antibiotics in 2 patients, and surgical debridement of a corneoscleral melt with patch graft along with both topical fortified antibiotics oral antibiotics in the third patient. In all 3 patients, there was no VA improvement at last follow-up, as 2 eyes ultimately underwent enucleation and 1 eye exhibited phthisis bulbi at last follow-up. BCC related endophthalmitis was reviewed among 13 reports. Treatment outcomes were generally poor and antibiotic resistance was common. These BCC isolates cases demonstrated broad resistance patterns, with susceptibilities to ceftazidime (58%), ciprofloxacin (53%), and gentamicin (33%). Conclusions: Endophthalmitis caused by B. cepacia is a rare clinical entity with generally poor visual outcomes despite prompt treatment with appropriate antibiotics.

Phenotypic expansion of KCNJ13-associated snowflake vitreoretinal degeneration.

INTRODUCTION: An 18-year old highly myopic woman presented with bilateral retinoschisis associated with a unilateral macular hole in the right eye and vitreomacular traction in the left eye. METHODS: Genetic studies disclosed a heterozygous pathogenic variant in the KCNJ13 gene was identified (c.484C>T (p.Arg162Trp)), consistent with a diagnosis of snowflake vitreoretinal degeneration (SVD). RESULTS: While there were no corneal guttata, juvenile cataracts, or perivascular sheathing in this case, salient features of SVD included a fibrillar vitreous structure, crystalline retinopathy, and flattened optic nerves. The patient developed a FTMH in the left eye at 17 months follow up, followed by a rhegmatogenous retinal detachment (RRD) requiring 2 surgical repairs. CONCLUSION: This case expands on the spectrum of clinical features in SVD, including retinoschisis and FTMH. It also characterizes optical coherence tomography findings in this rare disease entity. We emphasize the importance of using panel-based genetic testing to clinically distinguish and further define atypical vitreoretinopathies.

CHANGE IN CHORIOCAPILLARIS FLOW DEFICITS WITHIN TEARS OF THE RETINAL PIGMENT EPITHELIUM IMAGED WITH SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: Choriocapillaris (CC) flow deficits (FDs) were measured in the areas exposed by tears of the retinal pigment epithelium (RPE) before and after their onset to determine their change over time. METHODS: Patients enrolled in a prospective, swept-source optical coherence tomography angiography (SS-OCTA) study were retrospectively reviewed for RPE tears, and scans were evaluated before and after RPE tear formation. Choriocapillaris flow deficits were measured within the bed of the tear and within a symmetric control region. RESULTS: Three patients with RPE tears were imaged before tear formation and for at least 16 months afterward. When the baseline and first posttear visit were compared, CC FDs decreased by 1.0% in the tear region and 1.7% in the control region ( P = 0.84). When the 16-month follow-up visits were compared with the first post-RPE tear visits, CC FDs decreased by 1.9% in tear regions and increased by 1.3% in control regions ( P = 0.37). CONCLUSION: No significant changes in CC FDs were observed before and after RPE tear formation and for 16 months afterward, suggesting that CC FDs can be reliably detected in the presence of an intact RPE and the absence of the RPE did not affect CC perfusion for at least 16 months.

Recurrent, bilateral endogenous Candida endophthalmitis with multiple focal chorioretinal lesions: management with pars plana vitrectomy and focal endolaser.

PURPOSE: Bilateral endogenous Candida endophthalmitis (ECE) treatment usually involves administering systemic and intravitreal antifungal medications. In advanced cases with vitreous seeding, pars plana vitrectomy (PPV) is considered. The use of focal endolaser treatment to chorioretinal lesions has not been reported. We present a case of bilateral recurrent ECE treated with PPV and endolaser to elevated focal lesions. CASE: A 45-year-old diabetic male presented with decreased visual acuity in both eyes (20/50 right eye, 20/150 left eye) and was found to have bilateral ECE with moderate vitritis and chorioretinal lesions. The initial treatment consisted of multiple intravitreal voriconazole injections to both eyes as well as systemic antifungal therapy. Resolution of ECE occurred after three months, but one year later despite therapy recurred bilaterally. Patient underwent PPV with endolaser to the elevated chorioretinal lesions in both eyes. One year later, his vision improved to 20/40 in both eyes, focal lesions were flat and resolved along with the ECE. CONCLUSION: Advanced or recurrent ECE that is refractive to intravitreal antifungal therapy may be treated with PPV. Endolaser therapy to the chorioretinal lesions is an additional local option that can resolve the activity of ECE.

Endophthalmitis Caused by Kocuria Kristinae.

Kocuria is an anaerobic, Gram-positive bacterium, which has been rarely reported to cause endophthalmitis following cataract surgery, intravitreal injections, penetrating ocular trauma, and also secondary to endogenous sources. Visual prognosis is often guarded, with no previous cases reporting a final visual acuity better than 20/60. We describe a young female patient who developed culture-proven Kocuria kristinae endophthalmitis associated with a traumatic scleral rupture. Visual acuity at 2 months of follow-up improved from light perception to 20/50 after treatment with intravitreal antimicrobial therapy and pars plana vitrectomy.

SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY DETECTION OF BRUCH MEMBRANE AND CHORIOCAPILLARIS ABNORMALITIES IN SORSBY MACULAR DYSTROPHY.

PURPOSE: Swept-source optical coherence tomography angiography (SS-OCTA) was used to analyze Bruch membrane (BM) and choriocapillaris (CC) abnormalities in undiagnosed family members with Sorsby macular dystrophy (SMD). METHODS: In a family with SMD ( TIMP3 Tyr191Cys), SS-OCTA imaging was performed using the 6 × 6 mm scan patter and previously validated algorithms to detect abnormalities in BM and the CC, as well as the presence of reticular pseudodrusen and macular neovascularization. Genetic analyses were performed for TIMP3 mutations. RESULTS: Of eight family members, two were previously diagnosed with SMD and six were asymptomatic. SS-OCTA imaging of the 33-year-old proband revealed type 1 macular neovascularization in the left eye and bilateral reticular pseudodrusen, thickening of BM, CC thinning, and increases in CC flow deficits. A TIMP3 mutation was confirmed. His niece, despite having no clinical evidence of SMD, showed BM thickening and CC thinning on SS-OCTA. A TIMP3 mutation was confirmed. The proband's younger nephew and niece also carried the TIMP3 mutation without clinical evidence of SMD. Two additional members had normal examinations, unremarkable SS-OCTA findings, and no TIMP3 mutation. CONCLUSION: Swept-source optical coherence tomography angiography imaging can detect BM and CC abnormalities in vivo in subjects unaware of their TIMP3 status in a family with SMD.

EVALUATION OF RETINAL DISPLACEMENT FOLLOWING PRIMARY SCLERAL BUCKLING FOR MACULA-INVOLVING RHEGMATOGENOUS RETINAL DETACHMENT.

PURPOSE: To evaluate the incidence and degree of retinal displacement following scleral buckling surgery for macula-involving rhegmatogenous retinal detachment. METHODS: Retrospective interventional case series comprised of patients treated with primary scleral buckling procedure without gas tamponade for macula-involving rhegmatogenous retinal detachment and imaged postoperatively with fundus autofluorescence imaging between June 1, 2016 and July 25, 2021. Clinical notes, operative reports, fundus autofluorescence photographs, and optical coherence tomography images were reviewed. The presence and degree of retinal displacement were recorded. RESULTS: Twelve eyes of 11 patients were included. One (8%) eye with an epiretinal membrane demonstrated 0.1 mm of retinal displacement along the superior arcade and in the superotemporal periphery. The remainder of eyes (92%) did not show any identifiable signs of retinal displacement. CONCLUSION: Retinal displacement does not seem to be a frequent complication of primary scleral buckling surgery for macula-involving rhegmatogenous retinal detachment.

Laser-induced choroidal neovascularization detected on optical coherence tomography angiography in patients with diabetic retinopathy.

PURPOSE: To report two cases of laser-induced choroidal neovascularization (CNV) using swept source optical coherence tomography (SS-OCTA) imaging in patients with proliferative diabetic retinopathy. OBSERVATIONS: Two patients developed CNV adjacent to laser scars, and these neovascular lesions were detected on SS-OCTA imaging. Historically, both patients had subretinal fluid associated with these lesions. Visual acuity for both patients was 20/25 in the affected eyes. Both patients were observed over multiple years with stable CNV and visual acuity. CONCLUSIONS AND IMPORTANCE: SS-OCTA was able to detect laser-induced CNV in a rapid and non-invasive manner. When there is no foveal involvement and excellent visual acuity, we recommend close observation since the CNV may not progress.

Diagnosing Persistent Hypertransmission Defects on En Face OCT Imaging of Age-Related Macular Degeneration.

PURPOSE: A training exercise was performed to study the ability of graders to reliably identify precursor lesions to geographic atrophy (GA), known as persistent choroidal hypertransmission defects (hyperTDs), using en face OCT images from eyes with nonexudative age-related macular degeneration (AMD). DESIGN: Intergrader agreement study. PARTICIPANTS: Eleven graders participated in this exercise. METHODS: Formal training on how to identify persistent hyperTDs on en face OCT images was provided to the graders. A persistent hyperTD was defined as a bright lesion having a greatest linear dimension (GLD) of at least 250 µm. Training consisted of a tutorial session followed by the grading of 3 pretest exercises, each consisting of 3 cases. After all graders scored 100% on the pretest exercises, they performed a final exercise consisting of 30 en face OCT images from 29 eyes with nonexudative AMD containing 107 hyperTDs that each grader needed to evaluate. The cases contained a variety of AMD-related atrophic lesions. MAIN OUTCOME MEASURES: The sensitivity, positive predictive value (PPV), and modified accuracy were assessed for each grader. RESULTS: A total of 1177 hyperTDs from 30 en face OCT images were reviewed by the graders. The mean sensitivity, PPV, and modified accuracy for all the graders were calculated to be 99.0%, 99.2%, and 98.2%, respectively. There was a 97% agreement observed between all the graders (first-order agreement coefficient [AC1] = 0.97). Internal graders from the Bascom Palmer Eye Institute had a slightly higher agreement compared with the external graders (AC1 = 0.98 vs. AC1 = 0.96). The hyperTDs most often incorrectly identified included the following features: (1) hyperTDs containing hypotransmission defect cores, (2) single hyperTDs that were incorrectly graded as 2 separate lesions, and (3) hyperTDs with borderline GLDs that were close to 250 µm. CONCLUSIONS: The accurate detection of persistent hyperTDs on en face OCT images by graders demonstrates the feasibility of using this OCT biomarker to identify disease progression in eyes with nonexudative AMD, especially when used as a clinical trial end point in studies designed to test new therapies that may slow disease progression from intermediate AMD to GA.

Long-Term Favorable Visual Outcomes in Patients with Large Submacular Hemorrhage.

Submacular hemorrhage (SMH) has been reported to be toxic to the retina based on animal studies. However, observational studies of patients with neovascular-related SMH and those treated with intravitreal anti-vascular growth factor (anti-VEGF) therapy have shown many favorable visual acuity outcomes. We report two cases of neovascular-related SMH with ten or more years of follow-up. The first case was an 83-year old female with a history of nonexudative age-related macular degeneration in both eyes presenting with sudden decrease in vision (20/400) in her right eye due to a large SMH, treated with anti-VEGF therapy. Over the next following months, there was resolution of the hemorrhage and return of good visual acuity. At 10-year follow-up, visual acuity was 20/30 in the right eye. The second case was a 49-year old female with a history of presumed ocular histoplasmosis syndrome (POHS), presenting with sudden vision loss (20/400) in her right eye due to large, thick SMH. With observation and intermittent anti-VEGF therapy, there was resolution of the hemorrhage. At 30-year follow-up, visual acuity was 20/20 in the right eye.

Drug-related adverse effects of antivascular endothelial growth factor agents.

PURPOSE OF REVIEW: Antivascular endothelial growth factor (VEGF) agents have provided historic therapeutic breakthroughs in the treatment of retinal disease. New anti-VEGF agents are emerging for the treatment of retinal vascular diseases. Both systemic and ocular adverse effect need to be understood in managing patients. This review aims to highlight the adverse effects seen with routine use of bevacizumab, ranibizumab and aflibercept, as well as with new medications such as brolucizumab and abicipar. RECENT FINDINGS: We review the recent findings of intraocular inflammation (IOI) of brolucizumab and abicipar in the context of the efficacy and safety reported with the routine anti-VEGF agents. Specifically, brolucizumab has been reported to cause occlusive retinal vasculitis in the setting of IOI, which has not been seen in other anti-VEGF medications. In addition, abicipar appears to cause IOI at a higher rate of patients than other anti-VEGF agents have previously. SUMMARY: Newer anti-VEGF agents pose a significant risk of adverse events not seen with routine anti-VEGF agents.

Widefield en face optical coherence tomography monitoring of the peri-venular fern-like pattern of paracentral acute middle maculopathy.

PURPOSE: To demonstrate the ability of widefield en face swept source optical coherence tomography (OCT) imaging to monitor peri-venular fern-like patterns of paracentral acute middle maculopathy (PAMM) associated with retinal arterial occlusions. OBSERVATIONS: The peri-venular fern-like pattern of PAMM was diagnosed on the 12 × 12 mm en face structural OCT images from three patients. Fluorescein angiography images were unremarkable. Over time, all three patients demonstrated significant improvement in visual acuity with resolution of their peri-venular PAMM. CONCLUSIONS: The peri-venular fern-like pattern of PAMM is usually associated with retinal vein occlusions, but we identified three cases with this pattern in eyes with presumed incomplete retinal arterial occlusions. Our cases support the ischemic cascade theory that begins within the deep capillary plexus and ascends in the retina depending on the severity of the ischemic event. Using the 12 × 12 mm en face structural OCT images, we are able to demonstrate a wider area of ischemia in PAMM compared with the traditional 6 × 6 mm scans.