Decision making in anomalous aortic origin of a coronary artery.

INTRODUCTION: There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform. AREAS COVERED: The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA. EXPERT OPINION: Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA. A multi-disciplinary team with a focus on shared decision-making with the patients/families is likely necessary to optimize outcomes. Long-term follow-up and research are needed to improve our understanding of AAOCA.

Incidence and Risk Factors of Visual Impairment in Patients with Angioid Streaks and Macular Neovascularization.

OBJECTIVE: To estimate the incidence and risk factors of visual impairment and complications in eyes with macular neovascularization (MNV) because of angioid streaks (ASs). DESIGN: Longitudinal multicenter retrospective cohort study. SUBJECTS: Patients with AS-associated MNV treated with anti-VEGF agents and a follow-up of > 3 months. METHODS: Clinical and MNV characteristics were collected at baseline. Visual acuity (VA) values and the presence of atrophy or fibrosis were collected at each visit. MAIN OUTCOME MEASURES: Rate of VA change over time and associated factors; the incidence rate of moderate-to-severe visual impairment (MSVI) and blindness and hazard ratio (HR) of candidate risk factors for MSVI; the incidence rate of fibrosis and macular atrophy. RESULTS: Overall, 84 eyes of 66 patients (39 men, 58%) with a mean (standard deviation) age of 55.7 (13.8) years were followed for a mean (standard deviation) of 67.7 (48.5) months. The median number of anti-VEGF doses per eye was 13. The average rate (95% confidence interval [CI]) of visual loss was +0.04 (0.02-0.06) logarithm of the minimum angle of resolution/year (P < 0.001); the visual loss was faster in nonnaive eyes (P = 0.007) and those with better baseline VA (P < 0.001); it was slower in eyes with pattern dystrophy-like features (P = 0.04). The incidence rates (95% CI) of MSVI and blindness were 10.4 (6.88-15)/100-eye-years and 2.33 (1.12-4.29)/100-eye-years. A higher number of injections (HR [95% CI] = 0.45 [0.19-0.94] for receiving ≥ 13 injections vs. < 13; P = 0.03) was protective against MSVI. The incidence rates (95% CI) of fibrosis and macular atrophy were 24.1 (17.5-32.3)/100-eye-years and 14.3 (10.1-19.6)/100-eye-years. CONCLUSIONS: Eyes with MNV-related AS had a high rate of visual impairment and propensity to macular fibrosis and atrophy. A higher number of injections yielded better chances of maintaining good VA, suggesting the need for intensive treatment. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

LV Prasad Eye Institute EyeSmart electronic medical record-based analytics of big data: LEAD-Uveitis Report 1: Demographics and clinical features of uveitis in a multi-tier hospital based network in Southern India.

Purpose: To describe the demographics and epidemiology of uveitis presenting to a multi-tier ophthalmology hospital network in Southern India. Methods: Cross-sectional hospital-based study of 19,352 patients with uveitis presenting between March 2012 and August 2018. Results: In total, 1,734,272 new patients were seen across the secondary and tertiary centers of our multi-tier ophthalmology hospital network during the study period. Among them, 25,353 eyes of 19,352 patients were diagnosed with uveitis and were included in the study. Uveitis constituted 1.11% of all cases. The majority of patients were male (60.33%) and had unilateral (68.09%) affliction. The most common age group was 21-50 years with 12,204 (63.06%) patients. The most common type of uveitis was anterior uveitis, which was seen in 7380 (38.14%) patients, followed by posterior uveitis in 5397 (23.89%) patients. Among the infectious causes, tuberculosis was the most common etiology (2551 patients, 13%) followed by toxoplasmosis (1147 patients, 6%). Conclusion: Uveitis constituted 1.11% of all cases presenting to our clinics. It was more common in the age group of 21-50 and was predominantly unilateral. Anterior uveitis was the most common subtype seen in 38%.

Flanged intraocular lens (IOL) implantation with scleral pockets - A modification of flanged IOL technique (E-Flanged IOL) for secondary lens implantation.

The current technique for implanting flangedintraocular lens (IOL) suffers from complications like haptic exposure and tilting of the implanted IOL. We describe a modification of the currently described technique to obviate its shortcomings. Five eyes of five patients with a minimum of 1 year of follow-up were included. In this technique, two scleral pockets were made nasal and temporal to embed the flanged haptics. The primary outcome measure was the improvement in visual acuity (VA) postoperatively and the secondary outcome measures were postoperative complications. The primary objective of this current modification is to simplify the surgical technique for secondary IOL implantation and make it more replicable and predictive. The mean age of the patients was 19.44 years. The mean preoperative VA was 0.44 logMAR which improved to 0.26 logMAR at the 6-week postoperative visit. The mean follow-up was 496+/- 80 days. The maximum follow-up was 647 days. There were no postoperative complications such as haptic exposure, hypotony, or IOL tilt in any cases. The new E-flanged IOL technique has good visual outcomes and does not have postoperative complications. It has less intraoperative manipulation and complications.

Photodynamic therapy as a treatment option for peripapillary pachychoroid syndrome: a pilot study.

BACKGROUND: To investigate the anatomical and functional results in eyes with peripapillary pachychoroid syndrome (PPS) undergoing photodynamic therapy (PDT). METHODS: A total of 25 eyes from 23 patients with PPS treated with PDT were retrospectively evaluated in this multicentric study. Main outcome measure was the proportion of eyes that achieved treatment success, defined as a decrease in both subretinal fluid (SRF) height and central subfield thickness (CST), at 3 months after PDT compared to baseline. Secondary outcomes were the change in CST, SRF, and best-corrected visual acuity (BCVA) 3 months after treatment and predictive factors for treatment success. When available, data between 3 and 12 months were also reviewed. RESULTS: Treatment success was achieved in 16 eyes (64%). In the total cohort, CST decreased significantly from 356 ± 118 µm at baseline to 282 ± 90 µm and 270 ± 91 µm at 1 and 3 months, respectively (p < 0.001). Maximal SRF height decreased significantly from 102 ± 83 µm at baseline to 38 ± 46 µm and 32 ± 42 µm at 1 and 3 months, respectively (p < 0.001), and remained stable at month 6 (29 ± 44 µm) and month 12 (23 ± 35 µm). BCVA improved significantly from baseline to month 3 (p = 0.021). CONCLUSIONS: PDT can be considered an efficacious treatment option in patients with PPS. Prospective data with longer follow-up in a bigger cohort are needed in order to determine the optimal treatment algorithm in this relatively novel disease.

Retinal toxicities of systemic anticancer drugs.

Newer anticancer drugs have revolutionized cancer treatment in the last decade, but conventional chemotherapy still occupies a central position in many cancers, with combination therapy and newer methods of delivery increasing their efficacy while minimizing toxicities. We discuss the retinal toxicities of anticancer drugs with an emphasis on the mechanism of toxicity. Uveitis is seen with the use of v-raf murine sarcoma viral oncogene homolog B editing anticancer inhibitors as well as immunotherapy. Most of the cases are mild with only anterior uveitis, but severe cases of posterior uveitis, panuveitis, and Vogt-Koyanagi-Harada-like disease may also occur. In the retina, a transient neurosensory detachment is observed in almost all patients on mitogen-activated protein kinase kinase (MEK) inhibitors. Microvasculopathy is often seen with interferon α, but vascular occlusion is a more serious toxicity caused by interferon α and MEK inhibitors. Crystalline retinopathy with or without macular edema may occur with tamoxifen; however, even asymptomatic patients may develop cavitatory spaces seen on optical coherence tomography. A unique macular edema with angiographic silence is characteristic of taxanes. Delayed dark adaptation has been observed with fenretinide. Interestingly, this drug is finding potential application in Stargardt disease and age-related macular degeneration.

Sight-threatening intraocular infection in patients with COVID-19 in India.

PURPOSE: Intraocular infection in patients with COVID-19 could be different in the presence of treatment with systemic corticosteroid and immunosuppressive agents. We describe the epidemiology and microbiological profile of intraocular infection in COVID-19 patients after their release from the hospital. METHODS: We analyzed the clinical and microbiological data of laboratory-confirmed COVID-19 patients from April 2020 to January 2021 presenting with features of endogenous endophthalmitis within 12 weeks of their discharge from the hospital in two neighboring states in South India. The data included demography, systemic comorbidities, COVID-19 treatment details, time interval to visual symptoms, the microbiology of systemic and ocular findings, ophthalmic management, and outcomes. RESULTS: The mean age of 24 patients (33 eyes) was 53.6 ± 13.5 (range: 5-72) years; 17 (70.83%) patients were male. Twenty-two (91.6%) patients had systemic comorbidities, and the median period of hospitalization for COVID-19 treatment was 14.5 ± 0.7 (range: 7-63) days. Infection was bilateral in nine patients. COVID-19 treatment included broad-spectrum systemic antibiotics (all), antiviral drugs (22, 91.66% of patients), systemic corticosteroid (21, 87.5% of patients), supplemental oxygen (18, 75% of patients), low molecular weight heparin (17, 70.8% of patients), admission in intensive care units (16, 66.6% of patients), and interleukin-6 inhibitor (tocilizumab) (14, 58.3% of patients). Five (20.8%) patients died of COVID-19-related complications during treatment for endophthalmitis; one eye progressed to pan ophthalmitis and orbital cellulitis; eight eyes regained vision >20/400. Fourteen of 19 (73.7%) vitreous biopsies were microbiologically positive (culture, PCR, and microscopy), and the majority (11 patients, 78.5%) were fungi. CONCLUSION: Intraocular infection in COVID-19 patients is predominantly caused by fungi. We suggest a routine eye examination be included as a standard of care of COVID-19.

Cytomegalovirus retinitis in patients of non-Hodgkin's lymphoma: clinical presentations and outcomes.

BACKGROUND: Cytomegalovirus (CMV) retinitis in patients with Non-Hodgkin's Lymphoma (NHL) can occur even in the presence of high CD 4 counts and can behave differently when compared to CMV retinitis in human immunodeficiency (HIV) patients. It, therefore, becomes important to understand its varied presentations and the challenges in management of these cases. The aim of this study was to analyse the various patterns of presentations and outcomes of CMV Retinitis in patients with NHL. STUDY DESIGN: A retrospective chart review of seven eyes of four patients of NHL presenting with CMV retinitis between June 2017 and May 2020 was done. METHODS: Clinical patterns of CMV Retinitis, CD4 counts at the time of presentation and the duration of treatment along with recurrences and time for recurrence of retinitis were assessed. RESULTS: Granular or indolent retinitis (6 out of 7 eyes) was the commonest form of CMV retinitis in patients of NHL. Three patients had a presenting CD4 count above 150 cells/mm3 and none of them were below 50 cells/mm3. Floaters were the commonest presenting complaint. All patients had vitritis and majority of the patients (3 out of 4) had anterior chamber (AC) inflammation. Two out of the 4 patients had a recurrence (mean time 33.8 days) after stopping the maintenance phase of ganciclovir and one patient had significant myelosuppression related to oral valganciclovir which required discontinuation of the drug. CONCLUSION: CMV retinitis in NHL patients is usually of an indolent or granular type and can occur even in the presence of high CD4 counts as compared to patients with HIV. These patients may require a long term maintenance in view of frequent recurrences after discontinuation of treatment.

Radiation Retinopathy: Detection and Management Strategies.

A gradual shift in trend from primary enucleation to globe salvaging radiation therapy for the management of ocular tumors has resulted in the rise of several post-treatment ocular complications including radiation retinopathy. Radiation retinopathy is a chronic, progressive, and occlusive vasculopathy that can manifest anytime between 1 month to 15 years after starting radiation therapy. The aim of treatment in most of these cases is to prevent further vision loss. Treatment options such as laser photocoagulation, anti-vascular endothelial growth factor and intraviral steroids have been described. However, despite several advances in diagnostic and therapeutic modalities, a significant proportion of eyes with radiation retinopathy eventually go blind. This review summarises some of the clinical features, investigative modalities, and recent therapeutic strategies used in the management of radiation retinopathy.

Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery.

BACKGROUND: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. OBJECTIVE: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. MATERIALS AND METHODS: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. RESULTS: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CONCLUSION: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

Decreased Quality of Life in Children With Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden death in the young. We sought to determine quality of life (QOL) in patients/families affected by AAOCA. METHODS: Patients with AAOCA (8-18 years) were prospectively included from January 2016 to May 2017. Parent proxy and patient Pediatric Cardiac Quality of Life Inventory (PCQLI) were used to evaluate QOL and Pediatric Quality of Life Inventory (PedsQL) Family Impact Module to assess the impact of AAOCA on families, as primary outcomes. Secondary outcomes included peer relationship, anxiety, and depression assessed using patient-reported outcomes measurement information system. Patients deemed high-risk were offered surgery/exercise restriction. Generalized linear mixed regression models were used to determine significant predictors of outcomes. RESULTS: Fifty-three patients, the majority (n = 31, 59%) unrepaired, and 49 caregivers were included. Using PCQLI, patient and parent proxy QOL scores were similar to published scores for children with long-QT syndrome. Patients' QOL score was associated with exertional symptoms, perceived chronic disease, and altered parent's concentration ability. Likewise, parent proxy QOL scores were associated with mother's living situation, exertional symptoms, parent missing work for ≥1 day, and disturbed parental functioning at work. Family impact scores were associated with lower maternal education, among other measures. Risk categories or surgical status did not impact patient, parent proxy reported, or family impact QOL. CONCLUSION: Anomalous aortic origin of a coronary artery is associated with decreased QOL as perceived by patients and caregiver and is associated with numerous facets of family functioning. These findings are independent of risk categorization or surgical status.

Long-term outcomes of half-fluence photodynamic therapy and eplerenone in chronic central serous chorioretinopathy: A comparative study.

AIMS: To compare the long-term outcomes in chronic central serous chorioretinopathy (cCSC) following half-fluence photodynamic therapy (HF-PDT) and oral eplerenone treatment. METHODS: This retrospective comparative study included consecutive patients of cCSC treated with either HF-PDT or eplerenone. The treatment outcomes of the two groups were analyzed at 3-month, 6-month, and 12-month post-treatment. RESULTS: This study included 20 eyes (20 patients) in HF-PDT group, and 18 eyes (18 patients) in eplerenone group. All baseline parameters in HF-PDT and eplerenone groups were comparable including neurosensory detachment height (217.05 ± 140.25 µm vs 178.05 ± 164.24 µm respectively, p = 0.09), best-corrected visual acuity (BCVA) (logMAR 0.24 ± 0.13 vs logMAR 0.46 ± 0.37 respectively, p = 0.1), and subfoveal choroidal thickness (SFCT) [427.4 ± 117.4 µm vs 456.38 ± 119.25 µm respectively, p = 0.45]. HF-PDT resulted in complete resolution of neurosensory detachment in higher proportion of eyes compared to eplerenone at each follow-up visits (3 months: 90% vs 27.7%, p = 0; 6 months: 100% vs 61.1%, p = 0.003; and 12 months: 100% vs 70%, p = 0.03) with a significantly shorter duration to resolution (3.3 ± 0.9 months vs 5.8 ± 3.3 months respectively, p = 0.02). Intergroup comparison showed no significant difference between mean BCVA (p = 0.38 at 3 months, p = 0.14 at 6 months, and p = 0.19 at 12 months). Mean SFCT at 12 months of the two groups differed significantly (p = 0.003) due to increased choroidal thickness of eplerenone group. CONCLUSION: HF-PDT has a superior efficacy to achieve faster, greater and long-lasting resolution of subretinal fluid in cCSC compared to eplerenone therapy.

Impaired Myocardial Perfusion on Stress CMR Correlates With Invasive FFR in Children With Coronary Anomalies.

BACKGROUND: Invasive fractional flow reserve (FFR) is considered the gold standard to evaluate coronary artery flow. Stress cardiovascular magnetic resonance (sCMR) is an emerging non-invasive tool to evaluate myocardial perfusion in children. We sought to compare sCMR with FFR to determine impaired intracoronary flow in children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridge (MB) who presented concern for myocardial ischemia. METHODS: From December 2012 to May 2019, AAOCA and/or MB patients (<20 years old) were prospectively enrolled and underwent sCMR and FFR. Abnormal sCMR included perfusion/regional wall-motion abnormality in the involved coronary distribution. FFR was performed at baseline and with dobutamine/regadenoson and considered abnormal if <0.8 in the affected coronary segment. RESULTS: Of 376 patients evaluated, a total of 19 (age range, 0.2-17 years) underwent 24 sets of sCMR and FFR studies, with 5 repeat studies following intervention. Types of anomalies included 6 isolated MB/normal CA origins, 5 single CAs, 5 left AAOCAs, and 3 right AAOCAs. Seventeen patients (89.5%) had MB/intramyocardial course - 14 involving the left anterior descending coronary artery and 3 with multivessel involvement. sCMR correlated with FFR in 19/24 sets (7 sCMR and FFR positive, 12 sCMR and FFR negative) and it did not correlate in 5/24 sets. The positive percent agreement was 77.8%, negative percent agreement was 80.0%, and overall percent agreement was 79.2%. CONCLUSIONS: Assessment of myocardial perfusion using non-invasive sCMR concurred with FFR, particularly if performed with close proximity in time, and may contribute to risk stratification and decision making in children with AAOCA and/or MB.

Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

Miliary Retinal Lesions in Ocular Syphilis: Imaging Characteristics and Outcomes.

Purpose: To describe full thickness miliary retinal lesions in ocular syphilis.Methods: Retrospective chart review of patients with serologically confirmed ocular syphilis. Retinal miliary lesions in three cases of Syphilitic uveitis, in immunocompetent individuals are described. Case 1 and case 2 were positive for both VDRL (venereal disease research laboratory) and TPHA (Treponema pallidum hemagglutination), case 3 was VDRL negative but TPHA positive.Results: Miliary lesions were small round to oval, yellow retinal lesions, measuring less than » disc diameter size, with distinct margins, involving complete thickness of retina on OCT, in a pillar like manner, associated with ground glass retinitis, outer retinal placoid lesion or with retinal vasculitis. In cases 1 and 3, these healed with pigmentation.Conclusion: In contrast to similar lesions described, retinal miliary lesions seem to involve full thickness of the retina on OCT and may heal with pigmentation. These lesions may be characteristic of ocular syphilis.

Intra-lenticular caterpillar seta in ophthalmia nodosa.

PURPOSE: To report the clinical course of ophthalmia nodosa with a retained lenticular seta misdiagnosed and treated as non-infectious posterior uveitis for 7 consecutive years. METHODS: Meticulous clinical examination led to discovery of the caterpillar seta embedded in the crystalline lens and the intravitreal setae. RESULTS: Lens-sparing pars plana vitrectomy and removal of free-floating vitreal seta resulted in complete resolution of vitritis and uveitis. CONCLUSION: Embedded seta within a clear lens may remain sequestered, and may be left untouched under close observation, precluding a clear lens extraction in such patients.

Systemic immunosuppressive therapies for uveitis in developing countries.

There are multiple approaches to inhibit inflammatory molecules and pathways in noninfectious uveitis. The cornerstone of local and systemic anti-inflammatory treatment is corticosteroid therapy. Corticosteroids remain the most potent and efficacious drugs for treating intraocular inflammation. However, their long-term use is limited by their medium- and long-term side effects, which are a major concern. The approach taken to limit corticosteroid side effects is to introduce steroid-sparing agents that suppress the inflammatory pathways and immune response differently than corticosteroids. There are several classes of such drugs that are affordable, effective, and generally well-tolerated. Relatively recently, an increasing range of biologic agents has become available to treat intraocular inflammation. However, the relatively expensive cost of these therapies limits their use in the developing world. This systemic review aimst to discuss the use of corticosteroids and different immunosuppressive regimens in the management of various uveitides.

Clinical manifestations and outcomes of ocular syphilis in Asian Indian population: Analysis of cases presenting to a tertiary referral center.

PURPOSE: To describe disease manifestations and outcomes of ocular syphilis in Asian Indian population. METHODS: Retrospective analysis of patients diagnosed with ocular syphilis at a tertiary referral center in India. Demographics, history, extraocular and ocular manifestations, ocular and systemic investigations, treatment and visual acuity outcomes were noted. All patients were diagnosed after necessary laboratory investigations including HIV ELISA (Human immunodeficiency virus, enzyme-linked immunosorbent assay), VDRL (venereal disease research laboratory), and TPHA (treponema pallidum hemagglutination). RESULTS: Totally, 20 patients with mean age at presentation 38.25 ± 9.76 were analyzed. 9/20 patients had bilateral involvement. 8/20 had concurrent HIV at presentation with an average CD4 counts of 592.25 ± 411.34 cells/microliter. The mean duration of symptoms at time of presentation was 15.45 ± 35.15 weeks. VDRL test was reactive in 45% (9/20) patients whereas, all patients had a reactive TPHA test. Clinical manifestations included outer retinal placoid chorioretinitis lesions (8/20, 40%), followed by retinitis mimicking acute retinal necrosis as the second most common phenotype (4/20, 20%). Other presenting manifestations noted were panuveitis, miliary retinitis lesions, retinal vasculitis, intermediate uveitis, and anterior uveitis. The clinical phenotypes in immunocompromised included panuveitis, acute retinal necrosis and isolated anterior uveitis. Mean follow up duration was 6.32 ± 6.15 months. An improvement in mean best corrected visual acuity (BCVA) of (0.63 LogMAR, approximately 6 Snellen lines, P < 0.02) was noted at last follow-up. CONCLUSION: Phenotypic manifestations of ocular syphilis are varied. Non-treponemal tests like VDRL may be unreliable when compared with treponemal tests in diagnosing ocular syphilis. Syphilitic uveitis is considered equivalent to neurosyphilis and is treated similar to neurosyphilis.

Instantaneous Wave-Free Ratio (iFR) Correlates With Fractional Flow Reserve (FFR) Assessment of Coronary Artery Stenoses and Myocardial Bridges in Children.

OBJECTIVES: Instantaneous wave-free ratio (iFR) has been proven to correlate with coronary flow reserve better than fractional flow reserve (FFR) and is non-inferior to FFR in guiding coronary revascularization in ischemic heart disease. There has been no study validating the utility of iFR in children. METHODS: We performed a retrospective review of clinically indicated cases in which both FFR and iFR were obtained at Texas Children's Hospital from July, 2016 to March, 2019. FFR and iFR were obtained at baseline. Adenosine FFR (FFRa) was used for assessment of coronary artery (CA) stenoses and diastolic dobutamine FFR (dFFRd) for myocardial bridges (MBs). FFRa or dFFRd ≤0.8 and iFR ≤0.89 indicated significant flow impairment. RESULTS: A total of 22 coronary arteries (9 CA stenoses and 13 MBs) were assessed in 20 patients with median age of 13 years (range, 4-21 years) and median weight of 60 kg (range, 19-110 kg). iFR correlated with FFRa (Spearman's rho, 0.87; P<.01) in CA stenoses and with dFFRd (Spearman's rho, 0.74; P<.01) in MBs and agreed with FFR in 20/22 cases (90.9%). In 1 patient with CA stenosis and 1 MB with normal FFR, iFR was positive and both patients underwent coronary revascularization. CONCLUSIONS: iFR correlated with FFR in the assessment of CA stenoses in children. iFR does not require administration of pharmacological agents; thus, it may reduce procedural time, cost, and complications, and result in more widespread adoption of invasive assessment of CA lesions in young patients.