Faculty Bibliography

PURPOSE/OBJECTIVE:To describe the vascular anatomy and intraluminal flow characteristics of segmental retinal arteritis (SRA) using structural and angiographic optical coherence tomography (OCT). METHODS:Retrospective case series of consecutive patients presenting with SRA. All patients were evaluated at presentation with fundus photography, spectral domain OCT, and OCT angiography. One patient was imaged with dense B-scan OCT angiography. RESULTS:Three eyes of three male patients were evaluated. All examinations were consistent with reactivation of ocular toxoplasmosis with an area of active retinochoroiditis adjacent to a focal chorioretinal scar. Spectral domain OCT through areas of SRA noted on clinical examination demonstrated areas of hyperreflectivity circumscribing the affected vessel with a normoreflective lumen. Optical coherence tomography angiography and dense B-scan OCT angiography demonstrated narrowing of the intraluminal flow signal that correlated with areas of segmental hyperreflectivity on spectral domain OCT. Vascular sections proximal and distal to areas of SRA showed normal flow signal. CONCLUSION/CONCLUSIONS:Vessels with SRA demonstrated hyperreflectivity highlighting the vessel wall on spectral domain OCT. Optical coherence tomography angiography showed narrowing of the flow signal within these segments suggesting reduced lumen diameter. Coupling these finding with previous indocyanine green imaging findings in SRA, the collective data suggest the plaques are localized within the vessel wall to either the endothelium or the muscular tunica media without occlusion of the vessel lumen.

PURPOSE/OBJECTIVE:This review article is meant to serve as a reference guide and to assist the treating physician in making an appropriate selection and duration of an antimicrobial agent. METHODS:Literature review. RESULTS:Infections of the posterior segment require prompt medical or surgical therapy to reduce the risk of permanent vision loss. While numerous options exist to treat these infections, doses and alternative therapies, especially with contraindications for first-line therapy, are often elusive. Antimicrobial agents to treat posterior segment infections can be administered via various routes, including topical, intravitreal, intravenous, and oral. CONCLUSIONS:Although there are many excellent review articles on the management of endophthalmitis, we take the opportunity in this review to comprehensively summarize the appropriate antimicrobial regimen of both common and rare infectious etiologies of the posterior segment, using evidence from clinical trials and large case series.

Purpose : To evaluate the diagnostic yield and clinical impact of the SPARK/Invitae gene panel in patients with known or suspected inherited degenerative retinal disease, in comparison with traditional clinical assessments. Methods : Patients of the authors' clinical practices obtained genetic screening at no charge via the SPARK/Invitae ID your IRD genetic testing panel, which ranged from 248 genes to 293 genes. Over 16 months, tests were submitted for 87 patients and results were available for 70 patients. Clinical diagnoses prior to submitting the gene panel . By continuing to use our website, you are agreeing to included retinitis pigmentosa; Stargardt disease; Best vitelliform dystrophy; Leber our privacy policy. congenital amaurosis; choroideremia; achromatopsia; cone-rod dystrophy; congenital stationary night blindness; occult macular dystrophy; and familial dominant drusen in addition to patients with normal clinical findings and unclear diagnoses. Results : Of 70 patients, SPARK/Invitae considered the results ''Positive or Potentially Positive'' in 24 cases (34.3%), Carrier in 16 cases (22.9%) and ''Uncertain'' in 30 cases (42.9%). Uncertain results comprised patients with only Variants of Uncertain Significance. Patients categorized as a Carrier by SPARK/Invitae but who demonstrated pathogenic genetic changes correlating to the clinical diagnosis were considered to be in agreement with the clinical impression. The genetic diagnosis agreed with the clinical diagnosis in 30/70 (42.9%) total patients. Test results were consistent with the clinical impression in 13/26 (50.0%) retinitis pigmentosa cases, 6/8 (75.0%) Stargardt patients, 3/7 (42.9%) cone-rod dystrophy cases, and 2/4 (50.0%) Best vitelliform dystrophy patients. Gene testing helped elucidate diagnoses in two patients with unclear clinical impressions: one panel showed autosomal recessive achromatopsia and the other showed a carrier state for autosomal recessive retinitis pigmentosa. Of four patients with normal clinical exams, none had diagnostic results: all showed Uncertain findings. Conclusions : The SPARK/Invitae gene panel provided a genetic diagnosis consistent with the clinical impression in about 40 percent of patients. Retinitis pigmentosa and Stargardt disease were the most common clinical diagnoses and the diagnoses most often confirmed by testing. Genetic screening also assisted in clarifying unknown diagnoses for two patients

Purpose : To identify risk factors for endophthalmitis and poor visual outcomes in cases of retained intraocular foreign body (IOFB) and management strategies for these cases. Methods : A retrospective chart review was conducted in 88 eyes of 88 patients suffering traumatic injury with retained IOFB at the University of Michigan between January 2000 and December 2019. Medical records were reviewed to identify the nature of the injury, IOFB composition, and presenting characteristics of each eye as well as the surgical and antimicrobial strategies employed. Details of the injury, IOFBs, and clinical presentation were utilized to identify factors associated with clinical outcomes. Visual outcomes and development of endophthalmitis were additionally evaluated for association with treatment modalities. Results : This cohort developed endophthalmitis at a rate of 11.4% (4.5% presented with endophthalmitis, 7.1% of the remaining eyes developed endophthalmitis after initial intervention). Delayed presentation and organic IOFB were significantly associated with development of endophthalmitis. Retinal detachment, wound length greater than 5 mm, and poor presenting visual acuity were associated with poor final visual outcome. Antibiotic prophylaxis was given to all patients, though agents and routes of delivery varied. Primary and deferred removal of posterior segment IOFBs were associated with similar rates of endophthalmitis. Conclusions : Poor presenting visual acuity and severity of injury as measured by large wound and retinal detachment correlate with poor visual outcome. Prompt globe closure and antimicrobial prophylaxis is critical for infection prevention. Deferred IOFB removal may carry similar risk of endophthalmitis as primary removal

Purpose : To assess risk factors for development of endophthalmitis following open globe injury. Methods : A retrospective chart review of all patients treated for open globe injury at the University of Michigan from January 2000 through July 2017 was conducted. Exclusion criteria included intravitreal injection or intraocular surgery in the 30 days prior to injury or less than 30 days of follow-up. A total of 586 out of 993 eyes were included in the study. The main outcome measure was the rate of endophthalmitis in these eyes. Results : 25/586 eyes (4.3%) presented with endophthalmitis, or developed endophthalmitis following globe closure. Of these, 12/25 eyes (48.0%) presented with endophthalmitis and 13/25 eyes (52.0%) developed endophthalmitis after globe closure. Multivariate logistic regression analysis identified time to globe repair (OR 4.5, CI 1.9-10.7, p = 0.0008), zone I injury (OR 3.6, CI 1.1-11.0, p = 0.0282), and need for additional surgery (OR 5.5, CI 1.5-19.7, p = 0.0092) as factors associated with increased risk of developing endophthalmitis. Subconjunctival antibiotics (OR 0.3, CI 0.1-0.7, p = 0.0036) were associated with decreased risk of developing endophthalmitis. Conclusions : Prompt closure of the globe and use of subconjunctival antibiotics may reduce the risk of endophthalmitis in open globe injuries. Furthermore, a one-time dose of prophylactic antibiotics with same day discharge and delayed IOFB removal with intravitreal injection of antibiotics did not increase the rate of endophthalmitis. Open globe injuries can be visually devastating and understanding what factors predict and protect against endophthalmitis is paramount to achieving the best possible visual outcomes

PURPOSE/OBJECTIVE:To determine which spectral domain optical coherence tomography biomarkers of idiopathic macular hole (MH) correlate with the postoperative best-corrected visual acuity (BCVA) in anatomically closed MH. METHODS:Retrospective analysis of spectral domain optical coherence tomography scans of 44 patients presenting with MH followed for a mean of 17 months. Widths of MH aperture, base, and ellipsoid zone disruption were calculated from presenting foveal spectral domain optical coherence tomography B-scans. Macular hole base area and ellipsoid zone disruption area were calculated through the custom in-house software. RESULTS:Poorer postoperative BCVA correlated with increased preoperative choroidal hypertransmission (r = 0.503, P = 0.0005), minimum diameter (r = 0.491, P = 0.0007), and base diameter (r = 0.319, P = 0.0348), but not with preoperative ellipsoid zone width (r = 0.199, P = 0.2001). Applying en-face analysis, the BCVA correlated weakly with preoperative ellipsoid zone loss area (r = 0.380, P = 0.013), but not with preoperative MH base area (r = 0.253, P = 0.1058). CONCLUSION/CONCLUSIONS:Increased MH minimum diameter, base diameter, base area, and choroidal hypertransmission are correlated with a poorer postoperative BCVA. Ellipsoid zone loss measurements were not consistently correlated with a BCVA. Choroidal hypertransmission width may be an easy-to-visualize predictive imaging biomarker in MH surgery.

Background/Aims/UNASSIGNED:To determine the rate of endophthalmitis and assess risk factors for development of endophthalmitis following open globe injury (OGI). Methods/UNASSIGNED:A retrospective chart review of all patients treated for OGI at the University of Michigan from January 2000 to July 2017 was conducted. Exclusion criteria included intravitreal injection or intraocular surgery in the 30 days prior to injury or less than 30 days of follow-up. A total of 586 out of 993 open globe injuries were included in the study. The main outcome measure was the rate of endophthalmitis. Results/UNASSIGNED:In this study, 25/586 eyes (4.3%) had endophthalmitis. Of these, 12/25 eyes (48.0%) presented with endophthalmitis and 13/25 eyes (52.0%) developed endophthalmitis after globe closure. Multivariate analysis identified time to globe repair (OR 4.5, CI 1.9-10.7, p = 0.0008), zone I injury (OR 3.6, CI 1.1-11.0, p = 0.0282), and need for additional surgery (OR 5.5, CI 1.5-19.7, p = 0.0092) as factors associated with increased risk of developing endophthalmitis. Subconjunctival antibiotic injection at the time of globe closure (OR 0.3, CI 0.1-0.7, p = 0.0036) was associated with decreased risk of developing endophthalmitis. Conclusion/UNASSIGNED:Prompt globe closure and subconjunctival antibiotics may reduce the risk of endophthalmitis in OGI. Furthermore, our practice of a one-time dose of systemic prophylactic antibiotics, and intravitreal antibiotics if intraocular foreign body (IOFB) removal is delayed, was not found to increase the rate of endophthalmitis.

Purpose To evaluate the diagnostic yield and clinical impact of the SPARK/Invitae 248 gene panel in patients with known or suspected inherited degenerative retinal disease, in comparison with traditional clinical assessments including ERG testing. Methods Patients were drawn from the clinical practices of the authors. Genetic screening was provided by means of the SPARK/Invitae 248-gene panel at no charge to patients. Over the past year, results are available for 18 patients. Clinical diagnoses were Best vitelliform dystrophy (definite in one patient, suspected in three patients); retinitis pigmentosa (five affected patients, one likely female carrier); possible Stargardt disease (five patients); and one each with Leber congenital amaurosis, choroideremia, nyctalopia, and Norrie disease. Results Of the 18 studies returned, results were deemed by Invitae ''Positive'' in nine and ''Uncertain'' in nine. The genetic diagnosis agreed with the clinical diagnosis in eight cases (including two patients diagnosed with retinitis pigmentosa with homozygous mutations in USH2A, and one patient with the diagnosis of retinitis pigmentosa with compound heterozygotic mutations in NR2E3, which is also associated with enhanced S-cone syndrome). One patient with macular degeneration of uncertain etiology had a deletion in TPP1, which codes for ceroid lipofuscinosis. The remaining nine patients all returned heterozygous mutations considered ''variations of uncertain significance'' (VUS) ranging from two to eight different genes. Heterozygous VUS in ABCA4 were noted in two patients with the clinical diagnosis of Stargardt disease and in one with the clinical diagnosis of Best disease. Conclusion The SPARK/Invitae 248-gene panel provided a genetic diagnosis consistent with the clinical diagnosis in about half of cases and was diagnostic in one case where the clinical diagnosis was unclear. Retinitis pigmentosa and Stargardt disease were the most common genetic diagnoses. No cases of Leber congenital amaurosis were detected by genetic testing which had been misdiagnosed clinically.