ACR Appropriateness Criteria® Orbital Imaging and Vision Loss-Child.

Orbital disorders in children consist of varied pathologies affecting the orbits, orbital contents, visual pathway, and innervation of the extraocular or intraocular muscles. The underlying etiology of these disorders may be traumatic or nontraumatic. Presumed location of the lesion along with the additional findings, such as eye pain, swelling, exophthalmos/enophthalmos, erythema, conjunctival vascular dilatation, intraocular pressure, etc, help in determining if imaging is needed, modality of choice, and extent of coverage (orbits and/or head). Occasionally, clinical signs and symptoms may be nonspecific, and, in these cases, diagnostic imaging studies play a key role in depicting the nature and extent of the injury or disease. In this document, various clinical scenarios are discussed by which a child may present with an orbital or vision abnormality. Imaging studies that might be most appropriate (based on the best available evidence or expert consensus) in these clinical scenarios are also discussed. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

A Novel Video-Based Skills Curriculum for Incoming Ophthalmology Residents.

BACKGROUND: Given limited pre-residency ophthalmology exposure, skill training for PGY-2 ophthalmology residents is essential. However, orientation experiences vary, and skills acquisition is often not measured. OBJECTIVE: A novel video-based orientation curriculum was developed and implemented to standardize and effectively teach ophthalmic examination skills to incoming ophthalmology residents. METHODS: An instructional video library (VL) on ophthalmic exam skills was created in 2020. Prior to any instruction, PGY2s were recorded performing basic ophthalmic exams (BOE) using slit-lamp recording smartphone adapters. After a 2-week orientation involving live teaching, practice, and self-directed library review, ophthalmic exams were again recorded. A 36-point ophthalmic exam skills checklist expanding upon the Ophthalmic Clinical Evaluation Exercise (OCEX) was developed for scoring videos. Residents also completed pre- and post- surveys assessing their comfort with the ophthalmic exam. RESULT: 7 of 11 incoming PGY-2 residents (63.7%) ophthalmology residents participated. Average recorded OCEX score improved from 16.5 ± 5.8 to (p = .0002) to 30.9 ± 2.7. Surveyed resident comfort with the exam increased from 2.4 ± 0.6 to 4.2 ± 0.5 on a 5-point Likert scale (p < .0001). CONCLUSION: Our video library orientation curriculum was effective in rapidly increasing resident comfort and BOE skills. With the launch of the integrated internship model, the VL curriculum may be effective for training ophthalmology PGY1s, medical students and non-ophthalmology providers.

Creating ophthalmology experiences in undergraduate medical education: pilot of a cased-based learning ophthalmology tool.

PURPOSE: To evaluate medical student perceptions of a novel ophthalmology resource delivered through facilitated workshops in the core clerkship curriculum. METHODS: We created www.2020sim.com, a free case-based learning (CBL) ophthalmology tool, adapted from NephSIM (www.nephsim.com). The tool was first piloted with the internal medicine (IM) residents. After confirming a need, we focused on undergraduate medical education (UME) by expanding the 20/20 SIM content and partnering with the neurology (pilot academic year [AY] 2020-2021) and pediatric clerkships (pilot AY 2021-2022) to deliver a facilitated one-hour ophthalmology workshop within each clerkship's didactic curriculum. We evaluated the tool using pre- and post-surveys and knowledge assessments. RESULTS: Of 80 IM residents, 33 (41.3%) completed the needs assessment. Of the 25 residents who attended the workshop, 23 (92.0%) completed the exit survey. IM residents reported discomfort in several ophthalmology domains (9 of 14 rated mean score < 3.0), confirming a need. Most (n = 21/23, 91.3%) rated the tool as good/excellent. Of 145 neurology clerkship students, 125 (86.2%) and at least 88 (60.7%) students completed the pre- and post-test/exit surveys, respectively. On average, participants highly rated the tool, perceiving 20/20 SIM to be relevant to their education [4.1 (0.8)]. Mean pre- to post-test knowledge scores increased from 7.5 to 8.5/10.0 points (p < 0.001). Of the 136 pediatric clerkship students, 67 (49.3%) and 51 (37.5%) completed the pre- and post-surveys, respectively. Respondents perceived increased comfort with ophthalmology topics after the facilitated workshop [3.8 (0.8)]. Mean pre- to post-test knowledge scores trended from 1.8 to 2.0/5.0 points (p = 0.30). Collectively, 20/139 (14.4%) of exit survey respondents visited www.2020sim.com within 1 month after the workshop. CONCLUSION: After identifying areas of greatest need with residents, we partnered with core clerkships to deliver cross-disciplinary ophthalmology content in UME. We found high engagement with 20/20 SIM, with trends toward increased knowledge.

Referral patterns for infantile cataracts in two regions of the United States.

BACKGROUND: Delayed treatment of congenital or infantile cataracts can cause deprivation amblyopia. Prompt diagnosis and surgical intervention is critical for optimal outcomes. This study assessed referral patterns for congenital or infantile cataracts in two regions of the United States. METHODS: The medical records of children 0-1 years of age with congenital or infantile cataracts at Stanford University (2008-2018) and Emory University (2010-2015) were reviewed retrospectively. RESULTS: A total of 111 children were included. Of these, 82 (74%) were initially evaluated by a primary care doctor, of whom 40 (49%) were referred directly to a pediatric cataract surgeon. Of 61 newborns 0-2 months of age, 9 (15%) were initially referred to an eye care provider before 6 weeks of age, but the initial evaluation by a pediatric cataract surgeon was delayed until after 6 weeks of age. Referral patterns were similar between the two institutions (P = 0.06). CONCLUSIONS: Many children with congenital of infantile cataracts are initially referred by a primary care doctor to an eye care provider who does not perform pediatric cataract surgery. Nevertheless, the majority of newborn infants with cataracts were evaluated by a pediatric cataract surgeon before 6 weeks of age.

Microphthalmia and orbital cysts in DiGeorge syndrome.

We report the case of a 4-month-old boy diagnosed with DiGeorge syndrome with novel ocular features. The patient was diagnosed through genetic testing, with a noted 22q11.2 deletion, and had the additional clinical findings of cardiac anomalies, Hirschsprung's disease, and intracranial microhemorrhages. Eye findings included bilateral microphthalmia, persistent fetal vasculature, chorioretinal coloboma, and a unilateral orbital cyst. Given no known additional inciting exposures, a dysgenic mechanism resulting in failed closure of developmental fissures associated with the chromosomal deletion likely gave rise to these combined pathologies.

A Virtual Clinical Reasoning Case for Medical Students Using an Ophthalmology Model: A Case of Red Eye.

Introduction: Triggered by the COVID-19 pandemic, medical education has moved online, tasking medical educators with developing virtual learning experiences. This is particularly challenging for less-represented disciplines, such as ophthalmology. We designed a red eye clinical reasoning case for preclinical medical students, which can be delivered virtually, using video conference software. Methods: We developed a 90-minute red eye/clinical reasoning workshop for which prereading was assigned to students. We then delivered a virtual development session to nonophthalmologist copreceptors and provided a session faculty guide. The entire first-year medical student class (No. = 140) participated in one of four identical workshops, which included virtual small- and large-group discussions. Students completed a knowledge pre- and posttest, and an optional session postsurvey. Results: Knowledge gains from pretest (No. = 94) to posttest (No. = 73) were statistically significant (p < .05), with average scores improving from 57% to 70%. Overall, students were satisfied, rating the following items 4 or 5 out of 5: session (86%, No. = 31), virtual format (83%, No. = 30), and if they recommended future use (69%, No. = 35). Discussion: This novel, virtual clinical reasoning case simulated small- and large-group learning, achieved knowledge gains, and was well received by students. Minor technical challenges were encountered but successfully remedied, without apparent disruption to learning. This virtual medical education model can be used to enhance ophthalmology education in preclinical medical students and can be adapted for virtual design of other curricular content.

Reversal of severe vergence anomaly associated with convergence insufficiency.

Central disruption of fusion refers to a subject's inability to fuse images, resulting in constant diplopia. Severely reduced vergences, or vergence anomalies, producing markedly decreased fusional amplitudes resembling fusional disruption have not been reported previously with convergence insufficiency. We report 3 patients with severe vergence anomalies in the setting of convergence insufficiency.

Congenital corneal blood staining secondary to hemorrhagic persistent fetal vasculature.

An African American girl born at 37 weeks via spontaneous vaginal delivery to a 33-year-old woman was noted on delivery to have a unilateral absent red reflex in the right eye, which was enlarged. Intraocular pressure was elevated, and the cornea had a straw-colored opacity. B-scan ultrasonography of the right eye showed diffuse hyperechoic vitreous opacities and a retrolental mass, with a hyperechoic band stretching from the optic disk to the posterior lens. Neuroimaging showed a unilateral enlarged globe, intraocular hemorrhage, and persistent fetal vasculature, with no other intracranial pathology. An anterior chamber washout revealed liquified blood; the presence of corneal blood staining was confirmed. A spontaneous intraocular hemorrhage associated with persistent fetal vasculature was suspected, leading to secondary glaucoma and corneal blood staining.

Validity of the Red Reflex Exam in the Newborn Eye Screening Test Cohort.

BACKGROUND AND OBJECTIVE: The validity of the red reflex exam has yet to be tested against new methods of wide-angle imaging that may improve early detection of neonatal ocular pathology. The authors aimed to determine the validity of the pediatrician's red reflex exam using 130° wide-angle external and fundus digital imaging as a gold standard. PATIENTS AND METHODS: This was a prospective cohort study of 194 healthy, term newborns enrolled in the Newborn Eye Screening Test study at Lucile Packard Children's Hospital from July 25, 2013, to July 25, 2014. Red reflex screening was performed by a pediatrician in the newborn nursery and wide-angle fundus digital imaging was performed by a neonatal intensive care unit-certified nurse. The main outcome measure was the validity of the pediatrician's red reflex exam (unweighted kappa [κ] statistic, sensitivity, specificity). RESULTS: Compared to no subjects with abnormal red reflex exams reported in the pediatrician's notes, 49 subjects demonstrated one or multiple ocular abnormalities on 130° wide-angle fundus imaging (κ = 0.00). The pediatrician's red reflex exam had a sensitivity of 0.0% (95% CI, 0.0%-7.3%) and specificity of 100.0% (95% CI, 97.5%-100.0%) for the detection of ocular abnormalities. CONCLUSION: This study demonstrates the ability of wide-angle fundus imaging to detect fundus abnormalities not otherwise identified by standard newborn red reflex screening prior to hospital discharge. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:103-110.].

Revisiting secondary capsulotomy for posterior capsule management in pediatric cataract surgery.

PURPOSE: To evaluate the development and treatment of visual axis opacification following pediatric cataract extraction with intraocular lens placement (IOL) without primary posterior capsulotomy and anterior vitrectomy (PPC+AV). METHODS: The medical records of children who underwent cataract extraction and IOL at an academic medical center were reviewed retrospectively for development of posterior capsular opacification (PCO) to identify risk factors for development of treatment-requiring posterior capsular opacification. RESULTS: A total of 63 eyes of 47 children 7 months to 16 years of age were included. The rate of PCO formation following cataract extraction without PPC+AV was 90%. Of those, 96% required a secondary capsular procedure to clear the visual axis; 55% had a clear visual axis after 1 procedure, almost exclusively with a YAG capsulotomy, and 3.5% did not require any secondary capsular procedure. Younger age was the only statistically significant characteristic associated with both PCO formation and need for more than one secondary capsular procedure. Children <3 years of age had an average of 2.1 capsular procedures. CONCLUSIONS: Cataract extraction and IOL without PPC+AV leads to an expected high rate of PCO formation, which can be effectively managed with a secondary capsular procedure in all age groups. Leaving the posterior capsule intact at primary surgery is an option to discuss with parents to avoid a more complicated primary surgery.

Treatment of Nystagmus in Brainstem Cavernous Malformation with Botulinum Toxin.

We report a long-term eye movement study of a 68-year-old female with pontomedullary junction cavernous malformation whose dysconjugate nystagmus was treated with retrobulbar botulinum toxin A injections. Sequential, bilateral retrobulbar injections of botulinum toxin A were performed. Injections immediately decreased oscillopsia and nystagmus, and improved visual acuities. One to three months following injection, three-dimensional infrared oculography measured a significant 39-100% (P = 0.001) decrease in nystagmus amplitudes at multiple dimensions. This improvement diminished by six months in the right eye but sustained for about one year in the left eye. Over two years, botulinum toxin A injections were performed twice in the left eye and five times in the right eye. Our study supported the safe and effective use of repetitive retrobulbar botulinum toxin A injections in symptomatic nystagmus that failed medical therapy.

What colour are newborns' eyes? Prevalence of iris colour in the Newborn Eye Screening Test (NEST) study.

PURPOSE: This study aims to assess the birth prevalence of iris colour among newborns in a prospective, healthy, full-term newborn cohort. METHODS: The Newborn Eye Screening Test (NEST) study is a prospective cohort study conducted at Lucile Packard Children's Hospital at Stanford University School of Medicine. A paediatric vitreoretinal specialist (DMM) reviewed images sent to the Byers Eye Institute telemedicine reading centre and recorded eye colour for every infant screened. Variables were graphed to assess for normality, and frequencies per subject were reported for eye colour, sex, ethnicity and race. RESULTS: Among 192 subjects screened in the first year of the NEST study with external images of appropriate quality for visualization of the irides, the birth prevalence of iris colour was 63.0% brown, 20.8% blue, 5.7% green/hazel, 9.9% indeterminate and 0.5% partial heterochromia. The study population was derived from a quaternary care children's hospital. We report the birth prevalence of iris colour among full-term newborns in a diverse prospective cohort. CONCLUSION: The study demonstrates a broad range of iris colour prevalence at birth with a predominance of brown iris coloration. Future studies with the NEST cohort will assess the change in iris colour over time and whether the frequencies of eye colour change as the child ages.

Retinal and Optic Nerve Hemorrhages in the Newborn Infant: One-Year Results of the Newborn Eye Screen Test Study.

PURPOSE: To report the birth prevalence, risk factors, characteristics, and location of fundus hemorrhages (FHs) of the retina and optic nerve present in newborns at birth. DESIGN: Prospective cohort study at Stanford University School of Medicine. PARTICIPANTS: All infants who were 37 weeks postmenstrual age or older and stable were eligible for screening. Infants with known or suspected infectious conjunctivitis were excluded. METHODS: Infants born at Lucile Packard Children's Hospital (LPCH) from July 25, 2013, through July 25, 2014, were offered universal newborn screening via wide-angle digital retinal photography in the Newborn Eye Screen Test study. Maternal, obstetric, and neonatal factors were obtained from hospital records. The location, retinal layer, and laterality of FH were recorded by 1 pediatric vitreoretinal specialist. MAIN OUTCOME MEASURES: Birth prevalence of FH. Secondary outcomes included rate of adverse events, risk factors for FH, hemorrhage characteristics, and adverse events. RESULTS: The birth prevalence of FH in this study was 20.3% (41/202 infants). Ninety-five percent of FHs involved the periphery, 83% involved the macula, and 71% involved multiple layers of the retina. The fovea was involved in 15% of FH cases (birth prevalence, 3.0%). No cases of bilateral foveal hemorrhage were found. Fundus hemorrhages were more common in the left eye than the right. Fundus hemorrhages were most commonly optic nerve flame hemorrhages (48%) and white-centered retinal hemorrhages (30%). Retinal hemorrhages were found most frequently in all 4 quadrants (35%) and more often were multiple than solitary. Macular hemorrhages most often were intraretinal (40%). Among the risk factors examined in this study, vaginal delivery compared with cesarean section (odds ratio [OR], 9.34; 95% confidence interval [CI], 2.57-33.97) showed the greatest level of association with FH. Self-identified ethnicity as Hispanic or Latino showed a protective effect (OR, 0.43; 95% CI, 0.20-0.94). Other study factors were not significant. CONCLUSIONS: Fundus hemorrhages are common among newborns. They often involve multiple areas and layers of the retina. Vaginal delivery was associated with a significantly increased risk of FH, whereas self-identified Hispanic or Latino ethnicity was protective against FH in this study. The long-term consequences of FH on visual development remain unknown.

Pediatric eye injuries due to nonpowder guns in the United States, 2002-2012.

PURPOSE: To identify epidemiologic trends in nonpowder gun-related pediatric eye injuries and to determine factors associated with severe injury requiring hospital admission. METHODS: US emergency department data on pediatric eye injuries between 2002 and 2012 were reviewed using the National Electronic Injury Surveillance System. Literature review was conducted to determine trends in visual outcomes after treatment and use of eye protection. RESULTS: In 2012 roughly 3,161 children were treated in US emergency departments for nonpowder gun-related eye injuries. Since 2010 rates of severe nonpowder gun pediatric eye injury have increased by over 500% (P = 0.039). Specifically, while rates of hospital admission due to paintball gun eye injury have dropped precipitously (P = 0.0077), rates of admissions for air gun eye injuries have increased by over 600% since 2010 (P = 0.033). Children sustaining eye injury due to air guns are more likely to be diagnosed and admitted with foreign body or ocular puncture injury. Roughly 28% of documented cases of airsoft or BB gun-related injury had visual acuity worse than 20/50 after initial treatment. Over 98% of injuries occurred without eye protection. CONCLUSIONS: Air guns are rising in popularity and now account for the majority of pediatric eye injuries requiring hospital admissions. These eye injuries occur without ocular protection and may lead to permanent eye injury. Increasing regulations for eye protection, sales, and usage of air guns are needed to prevent serious pediatric eye injuries.

Application of diffusion tensor tractography in pediatric optic pathway glioma.

OBJECT: Magnetic resonance imaging is commonly used in diagnosis and surveillance for optic pathway glioma (OPG). The authors investigated the role of diffusion tensor (DT) tractography in assessing the location of visual pathway fibers in the presence of tumor. METHODS: Data in 10 children with OPG were acquired using a 3T MRI generalized autocalibrating parallel acquisitions DT-echo planar imaging sequence (25 isotropic directions with a b value of 1000 seconds/mm(2), slice thickness 3 mm). Fiber tractography was performed, with seed regions placed within the optic chiasm and bilateral nerves on the coronal plane, including the tumor and surrounding normal-appearing tissue. Tracking was performed with a curvature threshold of 30°. RESULTS: For prechiasmatic lesions, fibers either stopped abruptly at the tumor or traversed abnormally dilated nerve segments. Similar findings were seen with chiasmatic lesions, with an additional arrangement in which fibers diverged around the tumor. For each patient, DT tractography provided additional information about visual fiber arrangement in relation to the tumor that was not evident by using conventional MRI methods. Retrospective reconstruction of visual fibers in 1 patient with new postoperative hemianopia revealed an unexpected superior displacement of the optic tract that might have been helpful information had it been applied to preoperative planning or surgical navigation. CONCLUSIONS: Optic pathway DT tractography is feasible in patients with OPG and provides new information about the arrangement of visual fibers in relation to tumors that could be incorporated into surgical navigation for tumor biopsy or debulking procedures.

Neovascularization in Purtscher's retinopathy.

We report a case of neovascularization secondary to Purtscher's retinopathy that showed minimal improvement with photocoagulation treatment. A 14-year-old boy with a history of cerebellar medulloblastoma presented with blurry vision and floaters after being struck by a motor vehicle while riding his bike. At presentation, visual acuity was 20/400 in his right eye and counting fingers in his left eye. Fundus examination showed disk edema, retinal whitening, and retinal hemorrhages in both eyes. Optical coherence tomography demonstrated thinning of the temporal retina and disruption of the inner segment-outer segment junction of the photoreceptor layer in the right eye and thickening and edema of the nasal macula, as well as a central foveal hyper-reflectivity, in the left eye. At the initial visit, there was no ischemia or neovascularization (NV). One month later, the patient developed NV of the disk and ischemia in the mid-periphery of the left eye. The patient underwent treatment with pan-retinal photocoagulation. The NV regressed, but visual outcome remained poor at his 5-month follow-up visit.

Novel technique for Nd:YAG posterior capsulotomy in pediatric patients.

A 7-year-old girl had posterior capsule opacification 2 years after cataract extraction without posterior capsulotomy. This report describes a novel technique for Nd:YAG posterior capsulotomy in pediatric patients for providers who do not have access to an overhead-mounted Nd:YAG laser.

Cyanoacrylate adhesive use in primary operation and reoperation in rabbit eye muscle surgery.

PURPOSE: To compare the performance of 2-octyl-cyanoacrylate to 6-0 polyglactin 910 suture in rabbit superior rectus muscle surgery after operation and reoperation procedures. METHODS: A prospective noninferiority trial was conducted in rabbits. Bilateral superior rectus muscle recessions were performed using cyanoacrylate in one eye and polyglactin suture in the other. At 5 weeks, reoperations to advance the superior rectus muscle were performed on 20 rabbits. Slippage, tensile strength, ease of reoperation, operative time, and inflammatory reaction were recorded. For the primary outcomes, the predetermined margin of noninferiority was 1 mm for slippage and 100 g for tensile strength. RESULTS: In both groups, the proportion of slippage > or =1 mm from the recession site was 1.9%. For the reoperation, it was 36.8% and 15.7% in the suture and cyanoacrylate groups, respectively, and the mean slippage was 0.60 mm and 0.42 mm. Mean tensile strength was 842.8 g for suture and 777.2 g for cyanoacrylate after the first operation and 877.73 g and 844.87 g after the reoperation. There was no difference between groups for surgical difficulty or inflammatory index. For the first operation, surgery using cyanoacrylate was on average 3.85 min faster than suture. CONCLUSIONS: Cyanoacrylate can achieve an adequate muscle-sclera bond in the immediate period after surgery to avoid major slippage and does not affect the long-term process of wound healing for both recession and advancement procedures. It is well tolerated and does not add technical difficulty even if used for reoperations. Because it eliminates the risk of globe perforation, cyanoacrylate may be a good alternative to sutures in strabismus surgery.