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Low-dose and very low-dose intravitreal bevacizumab (IVB) have been reported to be successful in short-term treatment of type 1 retinopathy of prematurity (ROP), down to an initial dose of 0.004 mg. We now report 12-month outcomes for these infants.Masked, multicenter, dose de-escalation study.One hundred twenty prematurely born infants with type 1 ROP.A cohort of 120 infants with type 1 ROP in at least 1 eye from 2 sequential dose de-escalation studies of low-dose IVB (0.25 mg, 0.125 mg, 0.063 mg, and 0.031 mg) or very low-dose IVB (0.016 mg, 0.008 mg, 0.004 mg, and 0.002 mg) to the study eye; the fellow eye (if also type 1) received 1 dose level higher of IVB. After primary success or failure at 4 weeks, clinical management was at investigator discretion, including all additional treatment.Reactivation of severe ROP by 6 months corrected age, additional treatments, retinal and other ocular structural outcomes, and refractive error at 12 months corrected age.Sixty-two of 113 study eyes (55%) and 55 of 98 fellow eyes (56%) received additional treatment. Of the study eyes, 31 (27%) received additional ROP treatment, and 31 (27%) received prophylactic laser therapy for persistent avascular retina. No trend toward a higher risk of additional ROP treatment related to initial IVB doses was found. However, time to reactivation among study eyes was shorter in eyes that received very low-dose IVB (mean, 76.4 days) than in those that received low-dose IVB (mean, 85.7 days). At 12 months, poor retinal outcomes and anterior segment abnormalities both were uncommon (3% and 5%, respectively), optic atrophy was noted in 10%, median refraction was mildly myopic (-0.31 diopter), and strabismus was present in 29% of infants.Retinal structural outcomes were very good after low- and very low-dose IVB as initial treatment for type 1 ROP, although many eyes received additional treatment. The rate of reactivation of severe ROP was not associated with dose; however, a post hoc data-driven analysis suggested that reactivation was sooner with very low doses.

Background There remains debate regarding the need for venous imaging in pediatric intracranial hypertension. Methods Records of patients aged 18 years or younger who were evaluated in the intracranial hypertension clinic at Nationwide Children's Hospital in Columbus, Ohio, were reviewed. Past medical history, diagnostic evaluation, and presenting symptoms were examined to evaluate differences in symptomatology presentation and risk factors in patients with pediatric intracranial hypertension with and without thrombosis. Results A total of 226 patients met inclusion criteria, 145 were diagnosed with primary intracranial hypertension, 81 with secondary intracranial hypertension, with 17 noted to have venous sinus thrombosis as the cause of their secondary intracranial hypertension. Of those with thrombosis, 41.2% did not have any thrombosis risk factors. Headache was the most prominent symptom, present in 73.8% (n = 107) of patients with primary intracranial hypertension, 87.5% (n = 56) of patients with secondary intracranial hypertension without thrombosis, and 82.4% (n = 14) with thrombosis. Conclusions The only clinically significant difference in presenting symptomatology between the thrombosis and the other groups was nausea or vomiting. Predisposing factors to develop thrombosis were absent in 41.2% of patients. Hence, the need for venous imaging in pediatric intracranial hypertension cannot be clearly determined by clinical presentation or risk factors alone. Patients with indwelling catheters should receive imaging in the region of their catheter to rule out catheter-associated thrombosis.

Background Cryopyrin-associated periodic syndrome is characterized by periodic fever, rash, and joint pain. Papilledema rarely occurs. We present our series of patients with cryopyrin-associated periodic syndrome who clinically met the diagnostic criteria for Muckle-Wells syndrome and our experience with secondary intracranial hypertension. Methods Retrospective review of all patients with cryopyrin-associated periodic syndrome at Nationwide Children's Hospital from October 2015 to September 2017. Results Eighteen children met inclusion criteria: 15 females and three males, aged 1.5 to 16.2 years. Fifteen had periodic fever genetic testing; three had a known genetic defect identified, eight had a defect identified not currently known to be associated with cryopyrin-associated periodic syndrome, and four had no defect identified. Six patients (30%) developed headaches and were diagnosed with secondary intracranial hypertension. Lumbar puncture opening pressures ranged from 28 to 45 cm H2O. Only one patient had papilledema. Initial treatment was medical in all cases, by increasing interleukin-1 inhibitor dose and/or acetazolamide. One patient required a ventriculoperitoneal shunt for headache management. No visual acuity loss was detected. All six patients with secondary intracranial hypertension had a known genetic mutation or genetic variant of unknown significance; five involved the NLRP3 gene. Conclusions In our series of 18 patients with cryopyrin-associated periodic syndrome, secondary intracranial hypertension occurred at a higher than expected rate. We suspect that genetic defects involving the NLRP3 gene may be a risk factor. Papilledema was present in only one patient. Physicians treating cryopyrin-associated periodic syndrome should be aware of this vision-threatening association and potential therapeutic approach.

Importance Intravitreous bevacizumab (0.25 mg to 0.625 mg) is commonly used to treat type 1 retinopathy of prematurity (ROP), but there are concerns about systemic toxicity, particularly the risk of neurodevelopmental delay. A much lower dose may be effective for ROP while reducing systemic risk. Previously, after testing doses of 0.25 mg to 0.031 mg, doses as low as 0.031 mg were found to be effective in small cohorts of infants. Objective To find the lowest dose of intravitreous bevacizumab effective for severe ROP. Design, Setting, and Participants Between April 2017 and May 2019, 59 premature infants with type 1 ROP in 1 or both eyes were enrolled in a masked, multicenter, dose de-escalation study. In cohorts of 10 to 14 infants, 1 eye per infant received 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg of intravitreous bevacizumab. Diluted bevacizumab was prepared by individual research pharmacies and delivered using 300-µL syringes with 5/16-inch, 30-guage fixed needles. Analysis began July 2019. Interventions Bevacizumab intravitreous injections at 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg. Main Outcomes and Measures Success was defined as improvement by 4 days postinjection and no recurrence of type 1 ROP or severe neovascularization requiring additional treatment within 4 weeks. Results Fifty-five of 59 enrolled infants had 4-week outcomes completed; the mean (SD) birth weight was 664 (258) g, and the mean (SD) gestational age was 24.8 (1.6) weeks. A successful 4-week outcome was achieved for 13 of 13 eyes (100%) receiving 0.016 mg, 9 of 9 eyes (100%) receiving 0.008 mg, 9 of 10 eyes (90%) receiving 0.004 mg, but only 17 of 23 eyes (74%) receiving 0.002 mg. Conclusions and Relevance These data suggest that 0.004 mg may be the lowest dose of bevacizumab effective for ROP. Further investigation is warranted to confirm effectiveness of very low-dose intravitreous bevacizumab and its effect on plasma vascular endothelial growth factor levels and peripheral retinal vascularization.

STUDY OBJECTIVES: There is a well-established association between headache disorders and sleep disturbances in children, but it is unknown whether sleep disturbance plays a role in pediatric intracranial hypertension. The objective of this study was to examine sleep issues related to pediatric intracranial hypertension. METHODS: Patients with intracranial hypertension in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data were collected from the electronic medical record in addition to patient and parent completed questionnaires. Information on sleep behaviors was gathered using the Children’s Sleep Habits Questionnaire, and control data were obtained from patient siblings. Statistical analyses were performed using paired t tests or two-sample t tests, as appropriate. RESULTS: Sixty-two pairs of patients and matched sibling controls were compared. We found a statistically significant difference in total sleep disturbance score (control mean, 44.3; patient mean, 48.1; n = 33 pairs, t = −2.2, P = .035), as well as subscale scores of sleep onset delay (control mean, 1.4; patient mean, 1.7; n = 52 pairs, t = −2.53, P = .014), parasomnias (control mean, 8.5; patient mean, 9.5; n = 42 pairs, t = −2.59, P = .013), and sleep-disordered breathing (control mean, 3.1; patient mean, 3.4; n = 44 pairs, t = −2.61, P = .013). No difference was found in bedtime resistance, sleep duration, sleep anxiety, night awakenings, and daytime sleepiness subscales. Furthermore, no difference was found in total sleep disturbance score between patient subsets, including primary vs secondary intracranial hypertension, body mass index, pubertal status, presence of headaches, or intracranial hypertension treatment. CONCLUSIONS: This observational study suggests that pediatric intracranial hypertension is associated with a modest increase in sleep disturbances. CITATION: Kornbluh AB, Thompson K, Mcmahen G, et al. Sleep disturbance in pediatric intracranial hypertension. J Clin Sleep Med. 2020;16(7):1099–1105.

To evaluate the sensitivity and specificity of single, independent, nonphysician trained reader (TR) gradings in the Telemedicine Approaches to Evaluating Acute-phase Retinopathy of Prematurity (e-ROP) study.Secondary analyses of image grading results from 1,235 infants of birth weights1251 g. Two of three TRs independently graded image sets; discrepancies were adjudicated by the reading center director (an ophthalmologist) to reach final grading. Sensitivity and specificity of each TR grading and final grading was calculated by comparing gradings to clinical examination results.Of 7,808 double graded image sets, TR1 graded 5,165; TR2, 3,787; and TR3, 6,664. Compared to final grading for referral warranted retinopathy of prematurity (RW-ROP), two TRs had relatively lower sensitivity (TR1, 75% vs 79% [P = 0.03]; TR2, 73% vs 77% [P = 0.02]) and specificity (TR1, 80% vs 83% [P 0.001]; TR2, 82% vs 83% [P = 0.09]). TR3 had similar sensitivity (83% vs 83% [P = 0.78]) and specificity (83% vs 84% [P = 0.02]). Compared to final grading, TR1 had lower sensitivity for zone I ROP (47% vs 56% [P = 0.04]) and stage ≥3 ROP (71% vs 77% [P = 0.002]); TR2 had lower sensitivity for stage ≥3 ROP (69% vs 77% [P 0.001]) and lower specificity for all three components (P 0.001); TR3 had lower sensitivity for detecting plus disease (23% vs 35% [P 0.001]) and similar sensitivity for zone I ROP and stage ≥3 ROP.There is a small but significant decrease in the sensitivity and specificity for RW-ROP when single-reader grading is compared to double adjudicated grading.

Ocular causes of headaches include headaches associated with refractive error (HARE), convergence insufficiency (CI), and accommodative spasm (AS). HARE is more severe in patients with a high amount of refractive error. CI or AS patients can have diplopia and strabismus. Both CI and AS can be associated with head trauma or other systemic disorders. Headaches due to ocular causes are frontally localized occurring near the end of the day and are associated with increased amount of near work. HARE is treated with glasses while CI or AS may need other therapies such as prism, eye drops, surgery, or orthoptic exercises.

Purpose To determine the accuracy of identifying referral-warranted retinopathy of prematurity (RW-ROP, defined as any zone I ROP, stage 3 or worse, or plus disease) from retinal image sets using three grading protocols: a single optic disk–centered image, a set of 3 horizontal images, and a 5-image set. Methods In this secondary analysis of images from the e-ROP study, a weighted sample of 250 image sets from 250 infants (125 with RW-ROP and 125 without RW-ROP) was randomly selected. The sensitivities and specificities for detecting RW-ROP and its components from a single disk center image, along with nasal and temporal retinal images, were calculated and compared with the e-ROP grading of RW-ROP of all 5 retinal images (disk center and nasal, temporal, superior, and inferior retinal images). Results RW-ROP was identified with a sensitivity of 11.2% (95% CI, 6.79%-17.9%) using a single disk center image, with a sensitivity of 70.4% (95% CI, 61.9%-77.9%) using 3 horizontal images, and a statistically higher sensitivity of 82.4% (95% CI, 75.0%-89.0%) using all 5 images ( P = 0.002). The specificities were 100%, 86.4%, and 90.4%, respectively. For grading using 3 horizontal images, sensitivity was 14.3% for plus disease, 25% for zone I ROP, and 71.2% for stage 3 or worse compared to 40.8%, 50%, and 79.8% for grading using 5-image sets, respectively. Conclusions Both a single, disk-centered, posterior pole image and 3 horizontal images were less effective than a 5-image set in determining the presence of RW-ROP on qualitative grading by trained readers.

Differentiating true optic nerve edema from pseudo-optic nerve edema is a diagnostic dilemma faced by pediatric ophthalmologists. This case series suggests that oral fluorescein angiography is equivalent to intravenous fluorescein angiography in making this distinction. [ J Pediatr Ophthalmol Strabismus . 2019;56:e68–e72.]

OBJECTIVES: Increased intracranial pressure (ICP) has been suggested in legal settings as an alternative cause of retinal hemorrhages (RHs) in young children who may have sustained abusive head trauma. We assessed the prevalence and characteristics of RHs in children with increased ICP. METHODS: We conducted a prospective, multicenter study of children RESULTS: Fifty-six children (27 boys) were studied (mean age 15.4 months; range 1–43 months). All of the children had elevated ICP that required intervention. One child had papilledema. No child (0%; 95% confidence interval: 0%–6.4%) or eye (0%; 95% confidence interval: 0%–3.3%) was found to have an RH. Causes of increased ICP included hydrocephalus, intraventricular hemorrhage, congenital malformations, malfunctioning shunts, and the presence of intracranial space-occupying lesions. CONCLUSIONS: Although acute increased ICP can present in children with a pattern of peripapillary superficial RHs in the presence of papilledema, our study supports the conclusion that RHs rarely occur in the absence of optic disc swelling and do not present beyond the peripapillary area in the entities we have studied.

Importance The first Postnatal Growth and Retinopathy of Prematurity Study (G-ROP-1) developed new screening criteria with 100% sensitivity for type 1 retinopathy of prematurity (ROP) and 30% reduction of infants requiring examinations in a retrospective development cohort of 7483 infants from 29 North American hospitals in 2006-2012. Infants meeting 1 or more of the following criteria undergo examinations: gestational age less than 28 weeks or birth weight less than 1051 g; weight gain less than 120 g during age 10 to 19 days, weight gain less than 180 g during age 20 to 29 days, or weight gain less than 170 g during age 30 to 39 days; or hydrocephalus. Objective To evaluate the generalizability of the G-ROP screening criteria in a new cohort of at-risk infants. Design, Setting, and Participants This prospective validation cohort study (G-ROP-2) was conducted at 41 hospitals in the United States and Canada (25 G-ROP-1 hospitals and 16 new hospitals) from September 8, 2015, to June 13, 2017, among 3981 premature infants at risk for ROP and with known ROP outcomes. Main Outcomes and Measures Sensitivity for Early Treatment for Retinopathy of Prematurity Study type 1 ROP and potential reduction in infants receiving examinations. Results Among the 3981 infants in the study (1878 girls and 2103 boys; median gestational age, 28 weeks [range, 22-35 weeks]; median birth weight, 1072 g [range, 350-4080 g]; 1966 white; 942 black; 321 Latino; 120 Asian; 22 Native Hawaian or Pacific Islander; and 25 American Indian or Alaskan Native), the G-ROP criteria correctly predicted 219 of 219 cases of type 1 ROP (sensitivity, 100%; 95% CI, 98.3%-100%), while reducing the number of infants undergoing examinations by 35.6% (n = 1418). In a combined G-ROP-1 and G-ROP-2 cohort of 11 463 infants, the G-ROP criteria predicted 677 of 677 cases of type 1 ROP (sensitivity, 100%; 95% CI, 99.4%-100%), reducing the number of infants receiving examinations by 32.5% (n = 3730), while current criteria (birth weight Conclusions and Relevance This study found that the G-ROP screening criteria were generalizable on validation and, if used clinically in the United States and Canada, could reduce the number of infants receiving examinations. The large G-ROP cohorts provide evidence-based screening criteria that have higher sensitivity and higher specificity (fewer infants receiving examinations) for type 1 ROP than currently recommended guidelines.

Importance Intravitreous bevacizumab (0.25 to 0.625 mg) is increasingly used to treat type 1 retinopathy of prematurity (ROP), but there remain concerns about systemic toxicity. A much lower dose may be effective while reducing systemic risk. Objective To find a dose of intravitreous bevacizumab that was lower than previously used for severe ROP, was effective in this study, and could be tested in future larger studies. Design, Setting, and Participants Between May 2015 and September 2016, 61 premature infants with type 1 ROP in 1 or both eyes were enrolled in a masked, multicenter, phase 1 dose de-escalation study. One eye of 10 to 14 infants received 0.25 mg of intravitreous bevacizumab. If successful, the dose was reduced for the next group of infants (to 0.125 mg, then 0.063 mg, and finally 0.031 mg). Diluted bevacizumab was delivered using 300 µL syringes with 5/16-inch, 30-gauge fixed needles. Interventions Bevacizumab injections at 0.25 mg, 0.125 mg, 0.063 mg, and 0.031 mg. Main Outcomes and Measures Success was defined as improvement in preinjection plus disease or zone I stage 3 ROP by 5 days after injection or sooner, and no recurrence of type 1 ROP or severe neovascularization requiring additional treatment within 4 weeks. Results Fifty-eight of 61 enrolled infants had 4-week outcomes completed; mean birth weight was 709 g and mean gestational age was 24.9 weeks. Success was achieved in 11 of 11 eyes at 0.25 mg, 14 of 14 eyes at 0.125 mg, 21 of 24 eyes at 0.063 mg, and 9 of 9 eyes at 0.031 mg. Conclusions and Relevance A dose of bevacizumab as low as 0.031 mg was effective in 9 of 9 eyes in this phase 1 study and warrants further investigation. Identifying a lower effective dose of bevacizumab may reduce the risk for neurodevelopmental disability or detrimental effects on other organs.

We aimed to determine the frequency of intracranial hypertension without headache in children. We retrospectively analyzed patients evaluated in a pediatric intracranial hypertension referral center. Patients were divided into 2 groups depending on whether they complained of headache at the time of presentation. Age, body mass index, and opening cerebrospinal fluid pressures were considered continuous variables and compared by Wilcoxon rank-sum test because of non-normality. A P value of .05 was considered significant. A total of 228 charts were reviewed; 152 patients met the criteria for intracranial hypertension and 22/152 patients (14.5%) met the criteria of optic nerve edema without headache. There were clinically significant differences in age and body mass index between the 2 groups. The group without headache was typically younger and not obese. The opening pressure and modified opening pressure were not clinically significant between the 2 groups.

Objective We analyzed the incidence and demographic features of pediatric intracranial hypertension. Methods Inpatient and outpatient encounters of children aged 18 years or younger who were diagnosed with primary (idiopathic) or secondary intracranial hypertension between January 2010 and December 2013 were identified. Data were collected from a subspecialty clinic devoted to intracranial hypertension and the sole children's hospital in a large Midwestern city of the United States. Estimated incidence rates were calculated based on the number of newly diagnosed patients in our hospital's primary service area, which includes seven central Ohio counties. Sex, race, body mass index, socioeconomic status, and geographic distribution were also noted. Results A total of 74 pediatric patients were diagnosed with intracranial hypertension (49 primary/idiopathic and 25 secondary) between January 2010 and December 2013. Using census data, we determined the pediatric population in our service area during the four-year period. The Ohio Hospital Association's database indicated that 92.3% of patients aged 0 to 17 years residing in the region sought care at our institution. By combining these data, we calculated an annual incidence of primary and secondary intracranial hypertension of 0.63 and 0.32 per 100,000 children, respectively. Conclusions The estimated annual incidence of pediatric primary intracranial hypertension in our seven county service area in central Ohio is similar to previous pediatric reports from other countries and is 67% of that reported in the US adult population.

The purpose of this study was to report the incidence and describe the characteristics of sixth cranial nerve (CN VI) palsy in paediatric patients with intracranial hypertension (IH). A retrospective chart review of central Ohio children diagnosed with IH over the 3-year period from 2010 to 2013 was conducted. IH without identifiable cause was defined as idiopathic intracranial hypertension (IIH), whereas IH with identifiable pathologic aetiology was deemed secondary intracranial hypertension (SIH). A subset of patients with CN VI palsy was identified. Data collected included patient age, gender, past medical history, aetiology of SIH, ophthalmic examination, lumbar puncture results, neuroimaging results, and response to treatment. Seventy-eight children with intracranial hypertension were included in the study. Nine (11.5%) children (four males, five females; median age 14, range: 3–18) were found to have a unilateral (n = 2) or bilateral (n = 7) CN VI palsy. Five children had IIH; the remaining four had SIH from cerebral venous sinus thrombosis (n = 2) and infection (n = 2). The mean lumbar puncture opening pressure for the nine patients with CN VI palsy was 40 cm H2O (range: 21–65 cm H2O). Papilloedema was present in 8/9 (89%) patients. One patient required a lumboperitoneal shunt, and two others required optic nerve sheath fenestrations in addition to medical management. All cases of CN VI palsy resolved with treatment. In our primary service area, the incidence of CN VI palsy is approximately 12% among paediatric IH patients. The majority of cases with CN VI palsy presented with papilloedema and all cases resolved with treatment of intracranial hypertension.

Purpose To compare keratometry measurements on a fixating patient with readings from the same nonfixating patient intraoperatively using the Nidek KM-500 handheld keratometer. Methods Consecutive patients who were scheduled for strabismus or nasolacrimal surgery between 5 and 11 years of age were included in the study. Handheld keratometry was performed preoperatively on both eyes with the child fixating and intraoperatively with the child anesthetized. Three readings were taken on each eye. The steepest and flattest corneal meridians were recorded. Intraclass correlation coefficients were calculated to assess reliability, and interchangeability was assessed by the use of the Bland-Altman method. Results Included in the study were 55 eyes of 28 patients. The average fixating keratometry reading was 44.10 ± 1.45 D for right eyes and 44.12 ± 1.42 D for left eyes. The average nonfixating keratometry reading was 44.06 ± 1.62 D for right eyes and 44.02 ± 1.54 D for left eyes. The intraclass correlation coefficient for the average keratometry obtained fixating versus nonfixating was 0.96 for right eyes and 0.95 for left eyes. The Bland-Altman analysis showed fairly large limits of agreement between readings, but most readings fall within the limits of variability. The mean time to obtain the intraoperative measurements was 4.26 minutes. Conclusions In our study the Nidek KM-500 handheld keratometer provided reliable readings when used intraoperatively on anesthetized nonfixating children and required minimal time to perform.

Background The modified Dandy criteria and the newer diagnostic criteria for pseudotumor cerebri syndrome (PTCS) are both used to diagnose intracranial hypertension (IH). In comparison to the modified Dandy criteria, the PTCS criteria stratify the IH diagnosis into definite, probable, and suggested categories, exclude clinical symptoms, and use radiologic evidence for diagnosis. There is a lack of consensus on which criteria should be used in the pediatric population. The purpose of this study was to compare the diagnostic criteria for PTCS to the modified Dandy criteria and to identify limitations within both sets of criteria. Methods The PTCS criteria were retrospectively applied to 50 patients originally diagnosed with IH under the modified Dandy criteria. Results Of the 50 patients, 31 (62%) met diagnostic criteria for definite PTCS, 10 (20%) met criteria for probable PTCS, and 9 patients (18%) failed to meet sufficient PTCS criteria for diagnosis. Conclusions Although the PTCS criteria use objective data to make the IH diagnosis, we found subjective symptoms to be useful indicators of disease in this group of patients. Additionally, distinguishing probable from definite IH may not have clinical relevance, because both groups were treated similarly. The absence of radiographic evidence of IH should not preclude a diagnosis of the condition, as it was present in a minority of patients included in this study. Further research is needed to clarify the disease process in patients who present with signs and symptoms of elevated intracranial pressure but lack ocular pathology.

Importance Measurable competence derived from comprehensive and advanced training in grading digital images is critical in studies using a reading center to evaluate retinal fundus images from infants at risk for retinopathy of prematurity (ROP). Details of certification for nonphysician trained readers (TRs) have not yet been described. Objective To describe a centralized system for grading ROP digital images by TRs in the Telemedicine Approaches to Evaluating Acute-Phase Retinopathy of Prematurity (e-ROP) Study. Design, Setting, and Participants Multicenter observational cohort study conducted from July 1, 2010, to June 30, 2014. The TRs were trained by experienced ROP specialists and certified to detect ROP morphology in digital retinal images under supervision of an ophthalmologist reading center director. An ROP reading center was developed with standard hardware, secure Internet access, and customized image viewing software with an electronic grading form. A detailed protocol for grading was developed. Based on results of TR gradings, a computerized algorithm determined whether referral-warranted ROP (RW-ROP; defined as presence of plus disease, zone I ROP, and stage 3 or worse ROP) was present in digital images from infants with birth weight less than 1251 g enrolled from May 25, 2011, through October 31, 2013. Independent double grading was done by the TRs with adjudication of discrepant fields performed by the reading center director. Exposure Digital retinal images. Main Outcomes and Measures Intragrader and intergrader variability and monitoring for temporal drift. Results Four TRs underwent rigorous training and certification. A total of 5520 image sets were double graded, with 24.5% requiring adjudication for at least 1 component of RW-ROP. For individual RW-ROP components, the adjudication rate was 3.9% for plus disease, 12.4% for zone I ROP, and 16.9% for stage 3 or worse ROP. The weighted κ for intergrader agreement (n = 80 image sets) was 0.72 (95% CI, 0.52-0.93) for RW-ROP, 0.57 (95% CI, 0.37-0.77) for plus disease, 0.43 (95% CI, 0.24-0.63) for zone I ROP, and 0.67 (95% CI, 0.47-0.88) for stage 3 or worse ROP. The weighted κ for grade-regrade agreement was 0.77 (95% CI, 0.57-0.97) for RW-ROP, 0.87 (95% CI, 0.67-1.00) for plus disease, 0.70 (95% CI, 0.51-0.90) for zone I ROP, and 0.77 (95% CI, 0.57-0.97) for stage 3 or worse ROP. Conclusions and Relevance These data suggest that the e-ROP system for training and certifying nonphysicians to grade ROP images under the supervision of a reading center director reliably detects potentially serious ROP with good intragrader and intergrader consistency and minimal temporal drift.

It is now well recognized that idiopathic intracranial hypertension (IIH) can occur in children. Relative to the adult population, the demographic features and clinical presentation of IIH as well as the diagnosis and treatment guidelines for children are quite different. Although these differences are well established, specific diagnostic and treatment guidelines for pediatric patients have largely been based on the adult literature. Exciting new evidence is now available to assist the clinician in managing pediatric patients with IIH.