INTRAOPERATIVE OPTICAL COHERENCE TOMOGRAPHY DURING VITREORETINAL SURGERY FOR DENSE VITREOUS HEMORRHAGE IN THE PIONEER STUDY.

PURPOSE: To evaluate the feasibility and utility of intraoperative optical coherence tomography (OCT) during pars plana vitrectomy surgery for dense vitreous hemorrhage. METHODS: The Prospective Assessment of Intraoperative and Perioperative OCT for Ophthalmic Surgery study examined the utility of intraoperative OCT in ophthalmic surgery. Intraoperative scanning was performed with a microscope-mounted spectral domain OCT system. This report is a case series of those eyes undergoing pars plana vitrectomy for dense central vitreous hemorrhage that precluded preoperative OCT assessment. Intraoperative OCT images were qualitatively evaluated for retinal abnormalities that might impact intraoperative or perioperative management. Clinical variables were collected and assessed. Surgeon assessment of intraoperative OCT utility was also evaluated. RESULTS: Twenty-three eyes were identified and included. The etiology for the vitreous hemorrhage was proliferative diabetic retinopathy (19 eyes, 82.6%), horseshoe retinal tear (1 eye, 4.3%), retinal vein occlusion with neovascularization (1 eye, 4.3%), presumed polypoid choroidal vasculopathy (1 eye, 4.3%), and presumed retinal arterial macroaneurysm (1 eye, 4.3%). Intraoperative OCT revealed epiretinal membrane (14 eyes, 60.9%), macular edema (14 eyes, 60.9%), posterior hyaloid traction (1 eye, 4.3%), and retinal detachment (1 eye, 4.3%). Surgeon feedback suggested that intraoperative OCT impacted surgical decision making in eyes where membrane peeling was performed. CONCLUSION: Intraoperative OCT during pars plana vitrectomy for vitreous hemorrhage may provide physicians with clinically relevant information that may impact surgical management, perioperative management, and patient outcomes.

Prolonged hypotony maculopathy following uneventful strabismus surgery.

Hypotony is a rare postoperative complication of strabismus surgery. Resolution has been reported to occur within 1 month of surgery. Here, we describe the case of a 14-year-old boy with prolonged hypotony maculopathy following uneventful bilateral medial rectus recession. The hypotony resolved without long-term sequela after 7 months of treatment with topical steroids and atropine. Ultrasound biomicroscopy revealed a ciliary body effusion, which we hypothesize was the cause of decreased aqueous humor production and hypotony.

Assessment of scheduling for patients referred for an abnormal red reflex.

Scheduling lag time is the time between the date of an appointment and the date an appointment was created in the scheduling system. We sought to determine the scheduling lag time in a pediatric ophthalmology clinic for children <8 years of age, referred by a primary care provider for an abnormal red reflex (ARR), cataract, leukocoria, or retinoblastoma. The median scheduling lag time was 1.8 days (IQR, 0.8-5.0). Of the 106 total appointments, 89 (84%) were completed as scheduled, 12 (11%) were canceled, and 5 (5%) were no-shows. Ten patients (12%) were never seen. There was a shorter scheduling lag time for completed visits (median, 1.1 days; IQR, 0.2-4.0) than canceled visits (median, 12.0 days; IQR, 3.1-48.5; P < 0.001). Monitoring of the scheduling lag time for urgent referrals may be a metric for quality improvement in pediatric ophthalmology.

Retinopathy of prematurity screening and risk mitigation during the COVID-19 pandemic.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has significantly disrupted the delivery of healthcare. Although most nonurgent ophthalmology visits at Boston Children's Hospital were canceled, premature infants at risk for retinopathy of prematurity (ROP) still required timely, in-person care during the initial 3-month period of the infection surge in Massachusetts. The purpose of the current study was to report our protocols for mitigating risk of exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) between infants and eye care providers and to compare examination rates and results with the same 3-month period in 2019. METHODS: During the infection surge, we added new infection control measures and strengthened existing ones. Additional personal protective equipment was used, and the number of ophthalmologists rotating in the three high-capacity NICUs we service was limited. RESULTS: More infants required ROP examinations during the study period in 2020 than in the same period in 2019, but fewer examinations were performed. There were no cases of missed progression to severe ROP during this time and no known transmission of SARS-CoV-2 between ROP patients and ophthalmology staff. CONCLUSIONS: Overall, effective ROP care was safely provided during the COVID-19 pandemic, and contact with this vulnerable population was minimized.

A novel dominant CRX mutation causes adult-onset macular dystrophy.

BACKGROUND: To present the ophthalmological findings of a mother and son initially diagnosed with benign concentric annular macular dystrophy (BCAMD) and later discovered to carry a novel nonsense mutation in the cone-rod homeobox (CRX) gene (19q13.3). MATERIALS AND METHODS: Patients received ophthalmic examinations and diagnostic testing. The proband underwent sequencing of 131 retinal dystrophy genes. His mother had targeted testing of the identified sequence variations in CRX and BBS12 (4q27). RESULTS: The proband (Case I) presented at age 35 years for routine care. He had several male and female relatives with adult-onset retinal degeneration. Over 11 years, visual acuity (VA) dropped from 20/20 OU to 20/25 OD and 20/50 OS. Ophthalmoscopy showed bull's eye macular lesions OU. The proband's mother (Case II) presented at age 57 years with unilateral, progressive vision loss. Over 5 years, VA fluctuated 20/80-20/400 OD and remained 20/25 OS. Ophthalmoscopy showed similar findings to Case I. A clinical diagnosis of BCAMD was given because of the characteristic retinal lesion, preserved VA, and presumed autosomal dominant inheritance. Genetic testing identified a novel nonsense mutation in CRX (c.766C>T; p.Gln256Ter) in both patients. The son was also heterozygous for a missense mutation in BBS12 (c.1859A>G; p.Gln620Arg), which was absent in Case II. CONCLUSIONS: CRX mutations are associated with a variety of clinical phenotypes, including an adult-onset macular dystrophy that simulates BCAMD with a bull's eye macular lesion and fairly well preserved VA.

The Frequency of Optical Coherence Tomography Testing in Glaucoma at a Single Academic Medical Center.

PURPOSE: To determine the frequency of optical coherence tomography (OCT) examinations compared with clinical examinations and visual field (VF) tests in patients with 5 types of glaucoma. PATIENTS AND METHODS: A retrospective, longitudinal cohort study was conducted of 5154 patients treated between 2003 and 2010 at a single academic medical center. Patients were classified using billing records as having primary open-angle glaucoma, low-tension open-angle glaucoma (NTG), pigmentary open-angle glaucoma, chronic angle-closure glaucoma, or pseudoexfoliation glaucoma. Analysis of variance, χ test, and exact χ test were performed to identify associations between glaucoma type and test frequency. RESULTS: Pigmentary open-angle glaucoma and NTG patients had a higher rate of undergoing at least 2 VFs (94.4%, 94.9%), and chronic angle-closure glaucoma patients had a lower rate of undergoing at least 2 OCTs (25.3%) than all other glaucoma types. NTG patients also had the highest rate of undergoing at least 2 OCTs and at least 2 VFs (36.6%). Overall, the rate of clinical examinations (2.68 examinations/y) exceeded the rates of OCTs (1.39 examinations/y), which exceeded the rate of VF tests (1.24 tests/y). There were no differences in OCT frequency between glaucoma types (0.91 to 1.63 OCTs/y). Within each glaucoma diagnosis, patients had clinical examinations more frequently than OCTs and clinical examinations more frequently than VFs. Primary open-angle glaucoma and pseudoexfoliation glaucoma patients also had OCTs more frequently than VFs. More patients had at least 2 VF tests than at least 2 OCTs (4481 vs. 1679). CONCLUSION: The relative use of clinical examinations, VF testing, and OCT imaging varies among glaucoma diagnoses.

The Use of a Mobile Van for School Vision Screening: Results of 63 841 Evaluations.

PURPOSE: To present data from an established mobile screening program for children in the amblyogenic age group using gold-standard examination techniques by eye care professionals. DESIGN: Retrospective 12-year, cross-sectional study. METHODS: setting: Single center. STUDY POPULATION: All children enrolled in pre-kindergarten, kindergarten, and first grades in public schools in Cleveland, Ohio were eligible for evaluations. intervention procedure: An ophthalmic technician and/or optometrist examined children in a customized van that visits all elementary schools. The initial screening included testing of monocular near and distance visual acuity, stereoacuity, ocular alignment, motility, pupils, and external abnormalities. Children meeting the 2003 American Academy of Pediatrics vision screening referral criteria underwent cycloplegic retinoscopy and ophthalmoscopy by the on-site optometrist and received glasses. In addition, these children were referred to pediatric ophthalmology for ongoing care. MAIN OUTCOME MEASURE: Determination of the proportion of children with refractive errors, amblyopia, and/or strabismus. RESULTS: Between 2002 and 2014, 63 841 evaluations were performed, representing approximately 55% of eligible children. Overall, 6386 (10.0%) of children met 1 or more referral criteria: 5355 (8.39%) received glasses, 873 (1.37%) had amblyopia, and 1125 (1.76%) had strabismus. Over the 12-year period, there was no statistically significant change in the prevalence of strabismus (1.73%-2.24%, P = .91) or amblyopia (0.9%-2.08%, P = .13) among first grade students. CONCLUSIONS: Amblyopia, strabismus, and refractive errors are common in young children. During the study period, the rates of amblyopia and strabismus remained stable, indicating the need for continued vision screening and treatment services. A van-based model, traveling directly to schools, appears to be effective in reaching young children in underserved communities.

Prevalence of comorbid retinal disease in patients with glaucoma at an academic medical center.

BACKGROUND: Patients with various retinal diseases and patients who have undergone retinal procedures and surgeries have an increased risk of developing ocular hypertension and glaucoma. Little is known about the epidemiology of comorbid retinal diseases in glaucoma patients. This study evaluated the prevalence of retinal comorbidities in a population of patients with five types of glaucoma. METHODS: A longitudinal, retrospective study was conducted using International Classification of Disease (ICD-9) billing records from 2003 to 2010 at an academic medical center. Patients were classified as having primary open-angle glaucoma (POAG), low tension open-angle glaucoma (NTG), pigmentary open-angle glaucoma, chronic-angle closure glaucoma (CACG), or pseudoexfoliation glaucoma (PXG) if they had at least three clinic visits with the same ICD-9 code. Patients were classified as having a retinal comorbidity if they had two visits with the same code. Variables were analyzed with the independent t-test, χ (2) test, analysis of variance, or Fisher's exact test. RESULTS: A total of 5,154 patients had glaucoma, and 14.8% of these had a retinal comorbidity. The prevalence of comorbid retinal disease was higher in patients with POAG (15.7%) than in those with NTG (10.7%), PXG (10.1%), or pigmentary open-angle glaucoma (3.7%; P<0.05). Two hundred and two patients had diabetic retinopathy, with POAG patients (4.5%) having a higher prevalence than those with CACG (1.4%) or PXG (0.6%; P<0.001). There were 297 patients who had macular degeneration and both POAG (2.0%) and PXG patients (2.9%) had a higher prevalence of nonexudative macular degeneration than those with CACG (0%; P<0.01). Patients with comorbid retinal disease had a higher prevalence of blindness and low vision than those without comorbid retinal disease (1.97% versus 1.02%, P=0.02). CONCLUSION: The high prevalence of comorbid retinal disease and the nearly twofold increase in blindness and low vision in this population demonstrate the need for ophthalmologists to determine if patients have multiple etiologies for their vision loss. The higher prevalence of certain retinal diseases in POAG patients may reflect common pathophysiological processes that warrant further investigation.

Best's vitelliform macular dystrophy in 10- and 31-month-old siblings.

There are limited reports of Best's disease in children younger than 3 years. This is a case series of two family members aged 10 and 31 months with Best's disease. To the authors' knowledge, the 10-month-old male infant is the youngest patient with Best's disease documented by fundus photography.

No follow-up after positive newborn screening: medical neglect?

The current study examined medical professionals' behaviors related to reporting medical neglect when a family is noncompliant with follow-up services after a positive newborn screening result. Pediatric medical professionals within an urban medical campus were provided with five case vignettes in relation to different diseases. Medical professionals rated the severity of family noncompliance with follow-up services and indicated whether they would report suspected medical neglect to Child Protective Services (CPS). Physicians were more likely to report medical neglect than the other mandated reporters in the study. Logistic regression analyses found that medical professionals' perceptions of the severity of family noncompliance with services were significantly predictive of decisions to report medical neglect. Respondent gender and the method by which families were notified of screening results also significantly affected reporting behaviors in certain instances. Although all vignettes included information that met legal statutes for reporting neglect, medical professionals indicated that they would only report neglect 40-61% of the time across vignettes. Continued investigation of the rationale behind medical professionals' decision-making process and training protocols designed to improve mandated reporter knowledge and reporting behaviors are needed to further reduce bias and improve objectivity when considering ethical and professional obligations to report medical neglect.