Accuracy Assessment of Outpatient Telemedicine Encounters at an Academic Ophthalmology Department.

Purpose We assess the clinical accuracy of direct-to-patient real-time outpatient video visit encounters at our eye center. Design This was a retrospective longitudinal study. Subjects and Methods Patients who completed a video visit over a 3-week period between March and April 2020 were included. Accuracy assessment was determined by comparing diagnosis and management from the video visit with subsequent in-person follow-up over the next year. Results A total of 210 patients (mean age 55±18 years) were included, of whom 172 (82%) were recommended a scheduled in-person follow-up encounter after their video visit. Among the 141 total patients who completed in-person follow-up, 137 (97%) had a diagnostic agreement between telemedicine and in-person evaluation. Management plan agreed for 116 (82%), with the remainder of visits either escalating or deescalating treatment upon in-person follow-up with little substantive change. Compared with established patients, new patients had higher diagnostic disagreement following video visits (12 vs. 1%, p =0.014). Acute visits trended toward more diagnostic disagreement compared with routine visits (6 vs. 1%, p =0.28) but had a similar rate of management change on follow-up (21 vs. 16%, p =0.48). New patients were more likely to have early unplanned follow-up than established patients (17 vs. 5%, p =0.029), and acute video visits were associated with unplanned early in-person assessments compared with routine video visits (13 vs. 3%, p =0.027). There were no serious adverse events associated with the use of our telemedicine program in the outpatient setting. Conclusions Video visits had high diagnostic and management agreement with subsequent in-person follow-up encounters.

Use of Integrated Intraoperative Ocular Coherence Tomography in Pediatric Cataract Surgery: Thinking outside the Box.

The advent of integrated intraoperative optical coherence tomography (i2OCT) has opened the door for safer and more complex surgeries in the retina and cornea. However, to limit its use to just two subspecialties within ophthalmology is an opportunity lost for many other subspecialties. Here, we describe the use of i2OCT in pediatric cataract surgery in circumstances that are not traditionally considered for i2OCT use. The specific circumstances include: evaluation and treatment of a child following cataract extraction with lens implantation who has failed two attempts at YAG capsulotomy; lysis of post-trauma keratolenticular adhesion in an opaque cornea; surgical removal of secondary lens epithelial cell proliferation after pediatric cataract extraction with lens implantation, and identification of posterior capsular openings and defects. In each case the use of i2OCT allowed the procedure to be completed safely and successfully or informed the surgeon, allowing modification of the surgical decision tree.

Nonresponse to Health-Related Social Needs Screening Questions.

BACKGROUND AND OBJECTIVES: Although clinical settings are increasingly screening for social determinants of health, essential questions about optimal screening remain. We aimed to assess primary care contexts of individuals choosing not to answer questions about health-related social needs and to compare screening question response with subsequent use of resource information. METHODS: We compared caregiver responses to an electronic survey administered during a child's emergency department visit and through telephone follow-up 2 weeks later by responses to questions about health-related social needs (no social needs endorsed, ≥1 endorsed, none endorsed but ≥1 question not answered). RESULTS: Of 146 respondents, 42 (29%) endorsed ≥1 health-related social need. Additionally, 19 (13%) endorsed no social needs but did not answer ≥1 question. Compared with those denying all social needs and those endorsing ≥1 social need, respondents who did not answer social needs screening questions reported longer duration since their child's last primary care visit, lower perceptions of primary care, and less social support. For the 61 respondents participating in the 2-week follow-up survey, reported use of a community resource packet was 37% among those who had reported a social need, 26% among those who had denied all social needs, and 0% among those who had not answered ≥1 social needs questions. CONCLUSIONS: Clinicians and systems implementing screening for health-related social risks should plan for individuals who choose not to respond to specific items and may also wish to consider strategies that do not rely on screening and disclosure, particularly in communities known to have high prevalence of social needs.

Serial, Visually-Evoked Potentials for the Assessment of Visual Function in Patients with Craniosynostosis.

This study aimed to evaluate the effect of craniofacial surgical intervention on the visual pathway's function by comparing pre- to post-operative patterned, visually-evoked potentials (pVEP). A retrospective review was conducted on craniosynostosis patients who had pre- and post-craniofacial surgery pVEP testing. The pVEP measured grade in terms of amplitude latency and morphology of the waveforms. The pre- and post-operative results were compared. The study identified 63 patients (mean age at preoperative pVEP of 16.9 months). Preoperatively, 33 patients (52.4%) had abnormal pVEP. Nine patients had evidence of intracranial hypertension, and of those, eight (88.9%) had abnormal pVEP. Within 6 months postoperatively, 24 of 33 patients (72.7%) with abnormal preoperative pVEP developed normal postoperative pVEP, while all 30 patients with normal preoperative VEP maintained their normal results postoperatively. Significant improvements in pVEP latency in patients with broad or delayed latency waveforms was evident for subjects with preoperative grades 2-4 (grade 2, p = 0.015; grade 3, p = 0.029; grade 4; p = 0.007), while significant postoperative increase in amplitude was significant for patients with abnormally low amplitude grade 3 and 5 waveforms (grade 3, p = 0.011; grade 5, p = 0.029). Serial pVEP testing represents a useful tool for the early detection of visual pathway dysfunction and follow up visual pathway function in craniosynostosis. Surgical intervention for craniosynostosis can result in the reversal of preoperative pVEP abnormalities seen in these patients, resulting in the normalization of the pVEP waveform, amplitude and latency, depending on the preoperative pVEP abnormality.

Serial Visual Evoked Potentials in Patients with Craniosynostosis and Invasive Intracranial Pressure Monitoring.

This study aimed to detect the ability of pattern visual evoked potentials to detect visual pathway dysfunction in a cohort of patients with craniosynostosis who also had invasive intracranial pressure measurement. A retrospective review was conducted on craniosynostosis patients who had invasive intracranial pressure measurement and at least one pattern visual evoked potentials test. Reversal pattern visual evoked potentials were performed with both eyes open. Thirteen patients met the inclusion criteria (mean age at intracranial pressure measurement, 5.7 years). Seven patients had raised intracranial pressure, and of these, five (71.4 percent) had abnormal or deteriorated pattern visual evoked potentials parameters on serial testing, whereas all patients (100 percent) with normal intracranial pressure had normal pattern visual evoked potentials amplitude and latency. Four of the five patients (80 percent) with raised intracranial pressure and abnormal pattern visual evoked potentials did not show evidence of papilledema. The mean latency in patients with raised intracranial pressure (118.7 msec) was longer than in those with normal intracranial pressure (108.1 msec), although it did not reach statistical significance (p = 0.09), whereas the mean amplitude in patients with raised intracranial pressure (12.4 µV) was significantly lower than in patients with normal intracranial pressure (23.3 µV) (p = 0.03). The authors' results showed that serial pattern visual evoked potentials testing was able to detect visual pathway dysfunction resulting from raised intracranial pressure in five of seven craniosynostosis patients, and of these five patients, 80 percent had no evidence of papilledema, demonstrating the utility of serial pattern visual evoked potentials in follow-up of the visual function in craniosynostosis patients. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Diagnostic, II.

RASopathy in Patients With Isolated Sagittal Synostosis.

RASopathy is caused by dysfunction in the MAPK pathway, and include syndromes like Noonan syndrome (NS), NS with multiple lentigines (formerly known as Leopard syndrome), cardiofaciocutaneous (CFC), Legius syndrome, capillary malformation-arteriovenous malformation, neurofibromatosis type 1, and Costello syndrome. When counted together, RASopathies affect 1/1000 live births, and are characterized by cardiovascular manifestations, short stature, developmental delay, renal, urogenital, skin/skeletal abnormalities, and dysmorphic appearance. NS-one of the most common RASopathies-occurs in 1/1000 to 1/2500 live births. On the other hand, the frequency of CFC is unknown, but it is one of the rarest RASopathies, with estimates of only a few hundred cases worldwide. However, its phenotype overlaps with that of NS. In this case series, we describe 5 patients with a clinical and genetic diagnosis of RASopathy-either NS or CFC-all of whom were also diagnosed with isolated sagittal synostosis (ISS). Medical records from ophthalmology, cardiology, plastic surgery, medical genetics, cleft craniofacial, and neurosurgery were used to determine patient history. In our cohort, late presentation of ISS was the predominant form of ISS presentation. We hope this report further characterizes the burgeoning relationship between RASopathy and ISS. Furthermore, these findings support including sagittal synostosis among the presenting features in the clinical phenotype of RASopathies. Ethical approval was obtained from the university's institutional review board.

The Incidence of Chiari Malformations in Patients with Isolated Sagittal Synostosis.

BACKGROUND: We report the incidence of Chiari malformation I (CMI) in a cohort of 377 patients with isolated sagittal synostosis (ISS), which is to the best of our knowledge the largest such series reported to date. METHODS: A retrospective review of patients seen at a single institution from 2007 to 2017 was completed. ISS, Chiari malformations (CMI and CMII) and hydrocephalus were diagnosed by a senior neuroradiologist (G.Z.). Patients who met the inclusion criteria were divided into early (group A) and late (group B) presenting groups, as well as operated (group I) and unoperated (group II) groups. The patients were further subdivided into group AI (early operated), group AII (early unoperated), group BI (late operated), and group BII (late unoperated). Once identified, patient notes were examined for the following data sets: date of birth, age of presentation, age at last follow-up, other systemic conditions as well as molecular testing results. Surgical interventions, ophthalmological, and other relevant data were recorded. Statistical analysis was run in the form of a chi-square test to identify a significant difference between each subgroup. A literature review of the incidence of Chiari malformations in patients with ISS was conducted. RESULTS: Three hundred seventy-seven patients constitute the study's total cohort (272 were males and 105 females). This cohort was divided into patients who underwent surgical repair of ISS (group 1: n = 200), and patients who did not (group 2: n = 177). The entire cohort was also divided into early (group A: n = 161) and late (group B: n = 216) presenting craniosynostosis. In the total cohort, 22/377 (5.8%) patients with CMI were identified. CMI was found in 14/200 (7.0%) patients in group I, and 8/177 (4.5%) patients in group II. CMI was found in 2/161 (1%) patients in group A, and 20/216 (9.2%) patients in group B. The incidence of CMI in group AI (early operated) was 2/151 (1.3%), in group AII (early unoperated) was 0/10, in group BI (late operated) was 11/49 (21%), and in group BII (late unoperated) was 9/167 (5.4%). Chi-square analysis revealed a significant difference between the incidence of CMI in the early-presenting (group A) and late-presenting (group B) groups (P = 0.001) and between the late-presenting operated (BI) and late-presenting unoperated (BII) groups (P = 0.001). The incidence of hydrocephalus was 1.6% (6/377) in the total cohort. However, all patients diagnosed with hydrocephalus came from group II (no surgical ISS correction). The incidence of hydrocephalus in group II was 3.3% (6/177). The incidence of hydrocephalus in group BII (late unoperated ISS) was 3.0% (5/167). The incidence of hydrocephalus in group AII (early unoperated ISS) was 9.0% (1/11). CONCLUSIONS: We noted the highest incidence of CMI-21%-in group BI (late-presenting operated). We noted hydrocephalus in group II (nonoperated), with the highest incidence of hydrocephalus found in the group BII (late-presenting unoperated) subgroup. We therefore recommend patients with ISS receive funduscopic examination to screen for raised intracranial pressure (ICP) associated with CMI and hydrocephalus, especially patients with late-presenting ISS.

Syndromic and Systemic Diagnoses Associated With Isolated Sagittal Synostosis.

Reports of systemic associations in patients with Isolated Sagittal Synostosis (ISS) are sparse. Craniofacial surgeons, and other providers, should be aware that a significant proportion of patients with ISS may have syndromic or systemic involvement. This study investigates the incidence of systemic disease and syndromic diagnosis in a cohort of patients presenting with ISS (ie, patients with sagittal synostosis without other sutural involvement). METHODS: This study consists of a retrospective review of patients diagnosed with ISS between 2007 and 2017 at a single institution. Patients were divided according to onset (early <1 year, late >1 year) of ISS. Patient notes were examined for congenital anomalies, systemic conditions, and molecular testing. Only patients with isolated sagittal fusion-meaning, patients with sagittal synostosis and no other sutural involvement-were included. RESULTS: Three hundred seventy-seven patients met the inclusion criteria: systemic conditions were identified in 188/377 (50%) of them. One hundred sixty-one patients with early onset (Group A), and 216 patients with late onset ISS (Group B) were identified. Systemic involvement was identified in 38% of Group A and 60% of Group B, which was statistically significant (P < 0.001). Forty-eight of 377 (13%) of patients had a syndromic diagnosis, and 79% of these were confirmed via genetic testing. Thirty-five percent of patients were diagnosed with central nervous system anomalies and 16% had craniofacial anomalies. CONCLUSIONS: Nearly 50% of the patients initially diagnosed with ISS were found to have some form of systemic involvement. This supports affording full pediatric and genetic evaluation with molecular testing to these children.

Use of neuroimaging measurements of optic nerve sheath diameter to assess intracranial pressure in craniosynostosis.

PURPOSE: This study aims to evaluate accuracy of optic nerve sheath diameter (ONSD) measurements obtained by magnetic resonance imaging (MRI) in patients with craniosynostosis (CS) for detection of high intracranial pressure (ICP) and to correlate MRI-derived ONSD measurements with those obtained by computed tomography (CT) scans in CS patients. METHODS: A retrospective review was conducted on CS patients who had MRI- and age-matched controls with normal MRI. Diagnosis of intracranial hypertension was based on presence of papilledema, direct ICP monitoring, and/or lumbar puncture. The search also included patients with MRI and CT done within 30 days apart. ONSDs were measured 3 mm behind the globe on both modalities. RESULTS: The study identified 56 CS patients (mean age 3.8 ± 3.47 years) and 49 controls (mean age 3.7 ± 3.62 years). Mean ONSD in patients with high ICP was significantly higher than in patients without high ICP (P = 0.0001) and in controls (P < 0.0001). The optimal ONSD threshold for predicting raised ICP in patients >1 year old was 6 mm (71.4% sensitivity, 89.7% specificity). Nineteen patients with 38 single-eye MRI/CT pairs were identified. Substantial agreement between both modalities resulted (r = 0.959, 95% CI 0.923-0.978), and Bland and Altman Plot analysis showed that 95% of measurements fell within limits of agreement (1.96 SD; ± 0.6 mm). CONCLUSION: In CS patients, ONSD measured by MRI represent indirect non-invasive means of ICP assessment. Both MRI and CT measurements of ONSD gave comparable results, and the use of CT-derived ONSD measurements may give some idea about ICP in CS patients.