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Purpose: To compare a single surgeon's surgical outcomes for treating intermittent exotropia using bilateral lateral rectus recession (BLR), unilateral lateral rectus recession and medial rectus resection (RR), and unilateral lateral rectus recession and medial rectus plication (RP)., Methods: A retrospective review of all surgeries for basic intermittent exotropia between 2015 and 2023 was performed. Only patients with initial correction using BLR, RR, or RP were included. Exclusion criteria included age older than 18 years, vertical deviation, any nonrefractive ocular diagnoses, prior ocular surgery, and inadequate follow-up., Results: There were 460 patients identified; 123 met inclusion criteria with 54 in the BLR group, 41 in the RR group, and 28 in the RP group. The average pre-operative distance alignment (and standard error) values for the BLR, RR, and RP groups were 25.07 (7.35), 22.44 (5.95), and 23.84 (6.42) prism diopters (PD), respectively. At 1 year, the postoperative distance alignment values for the BLR, RR, and RP groups were 8.72 (7.89), 7.46 (6.31), and 12.83 (6.82) PD, respectively ( P = .03). A subanalysis found a significant difference between the BLR and RP ( P = .02) and RR and RP ( P = .02) groups. There was no difference between the BLR and RR groups ( P = .57)., Conclusions: This study of three surgical approaches for intermittent exotropia found RP had a significantly larger angle of exodeviation compared to BLR and RR at 1 year of follow-up. Both BLR and RR were equally effective approaches for treating intermittent exotropia. [ J Pediatr Ophthalmol Strabismus . 2024;61(4):287-290.] .

Background: Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist., Methods: An institutional database of patients 18 years and younger followed in the institution's pediatric intracranial hypertension clinic was examined for opening pressure changes in PIH at diagnosis, before medication wean, and following medication wean, as well as to examine whether measurements at the time of diagnosis differed between those with and without disease recurrence., Results: Forty-two patients were included in this study; 36% were male and the mean age at diagnosis was 11.01 years. Treatment duration averaged 9.68 months in those without recurrence and 8.5 months in those with recurrence. Average body mass index percentile of patients with disease recurrence was 83.7 and 72.1 in those without recurrence (P = 0.16). Average opening pressure values of all patients at diagnosis, prewean, and postwean was 36.53 cm H 2 O, 30.7 cm H 2 O, and 31.1 cm H 2 O, respectively. There was no statistically significant difference in opening pressures across these time points (P = 0.14). The change in opening pressure from diagnosis to postwean was statistically significant with a reduction of 5.18 cm H 2 O (P = 0.04). There was no statistical difference between change in opening pressure at diagnosis versus postwean between those with and without recurrence (P = 0.17)., Conclusions: This clinical observational study suggests that mean opening pressure measurements in patients with PIH remain elevated both before and after medication wean despite papilledema resolution and patient-reported PIH symptoms. Clinically, this suggests that other features such as signs of optic disc edema and symptoms should be used to inform a clinical determination of disease recurrence and treatment course., Competing Interests: Declaration of competing interest S.C. Aylward receives honoria as associate editor of Pediatric Neurology. The remaining authors have no relevant conflicts of interest to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Classically, peripheral vascular changes in the retina in patients with neuronal ceroid lipofuscinosis type 2 (CLN2) are described as vascular attenuation seen in the late stages of disease on the Weill Connell Ophthalmic Severity Score (WCOSS) staging system. We describe isolated, mild, peripheral vasculitis with peripheral arteriolar dropout identified by fluorescein angiography in patients with a WCOSS grade of stage 2. We believe this vasculitis represents an early vasodegenerative phase of disease that leads to the vascular attenuation seen in later stages of the disease., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To report 2-year ocular and developmental outcomes for infants receiving low doses of intravitreal bevacizumab for type 1 retinopathy of prematurity (ROP)., Methods: A total of 120 premature infants (mean birthweight, 687 g; mean gestational age, 24.8 weeks) with type 1 ROP were enrolled in a multicenter, phase 1 dose de-escalation study. One eye per infant received 0.25 mg, 0.125 mg, 0.063 mg, 0.031 mg, 0.016 mg, 0.008 mg, 0.004 mg, or 0.002 mg of intravitreal bevacizumab; fellow eyes when treated received one dosage level higher. At 2 years, 70 of 120 children (58%) underwent ocular examinations; 51 (43%) were assessed using the Bayley Scale of Infant and Toddler Development., Results: Correlation coefficients for the association of total dosage of bevacizumab with Bayley subscales were -0.20 for cognitive (95% CI, -0.45 to 0.08), -0.15 for motor (95% CI, -0.41 to 0.14), and -0.19 for language (95% CI, -0.44 to 0.10). Fourteen children (21%) had myopia greater than -5.00 D in one or both eyes, 7 (10%) had optic nerve atrophy and/or cupping, 20 (29%) had strabismus, 8 (11%) had manifest nystagmus, and 9 (13%) had amblyopia., Conclusions: In this study cohort, there was no statistically significant correlation between dosage of bevacizumab and Bayley scores at 2 years. However, the sample size was small and the retention rate relatively low, limiting our conclusions. Rates of high myopia and ocular abnormalities do not differ from those reported after larger bevacizumab doses., (Copyright © 2023. Published by Elsevier Inc.)

Purpose: 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., Design: Masked, multicenter, dose de-escalation study., Participants: One hundred twenty prematurely born infants with type 1 ROP., Methods: 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., Main Outcome Measures: 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., Results: 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., Conclusions: 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., (Copyright © 2022 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: The proportion of children with recurrent signs and symptoms of intracranial hypertension after medication wean has been reported to be between 18% and 50%. Few studies have reported intracranial hypertension recurrence risk in children while adjusting for each individual's observed follow-up time after medication wean. In addition, the role of intracranial hypertension etiology on the risk of disease recurrence has not been widely studied., Methods: The medical charts of patients with intracranial hypertension treated with intracranial pressure-lowering medication were analyzed retrospectively for disease recurrence. Baseline characteristics from diagnosis were recorded in addition to information regarding duration of therapy, medication wean, and recurrence. Survival analyses as well as Poisson regression models with time under observation as an offset were performed., Results: One hundred and thirty-three patients were included in the study. The cumulative risk of intracranial hypertension recurrence increased rapidly within the first six months after medication wean and was 1.5% at one month, 9.5% at three months, and 20% at six months. This risk leveled off near 12 to 18 months., Conclusions: While the cumulative risk of intracranial hypertension recurrence increases most dramatically within the first six months after medication wean, it does not appear to taper until 12 to 18 months. Given the possibility of delayed or asymptomatic recurrences, long-term follow-up is ideal, although patients can likely be seen less frequently after the first 12 to 18 months after medication wean., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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 H 2 O. 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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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., (© 2020 American Academy of Sleep Medicine.)

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 if 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 who were followed in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data was 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 was 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. There was a statistically significant difference in total sleep disturbance score (control mean 44.3; patient mean 48.1; n=33 pairs, t=-2.2, p=0.035) as well as subscale scores of sleep onset delay (control mean 1.4; patient mean 1.7; n=52 pairs, t=-2.53, p=0.014), parasomnias (control mean 8.5; patient mean 9.5; n=42 pairs, t=-2.59, p=0.013), and sleep disordered breathing (control mean 3.1; patient mean 3.4; n=44 pairs, t=-2.61, p=0.013). There was no difference found in bedtime resistance, sleep duration, sleep anxiety, night wakings, and daytime sleepiness subscales. Furthermore, there was no difference in total sleep disturbance score between patient subsets including: primary versus 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., (© 2020 American Academy of Sleep Medicine.)

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.]., (Copyright 2019, SLACK Incorporated.)

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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the incidence of surgical intervention in pediatric intracranial hypertension (IH), evaluate the visual outcomes of surgically managed patients, and identify potential predictors for surgical intervention., Methods: The medical records of patients with primary and secondary IH at Nationwide Children's Hospital from 2010 to 2017 were reviewed retrospectively. Presenting characteristics of medically and surgically managed patients were compared, and the clinical courses of surgically managed patients were reviewed., Results: A total of 129 medically and 14 surgically managed patients were included. The surgical incidence was 9.8%. Final visual acuity in 27 of 28 surgically managed eyes was 20/25 or better. In combined primary and secondary IH patients, elevations in body mass index (BMI; OR = 1.06; 95% CI, 1.01-1.11; P = 0.022) and lumbar puncture opening pressures ≥52 cm H 2 O (OR = 6.17; 95% CI, 1.93-19.67; P = 0.002) were significantly associated with the likelihood of surgical intervention when assessed by univariate logistic regression; grade of papilledema >2 was of marginal significance. After controlling for BMI, a lumbar puncture opening pressure of ≥52 cm H 2 O was more likely to result in surgery (adjusted OR = 4.69; 95% CI = 1.39-15.98; P = 0.013)., Conclusions: Most pediatric IH can be treated medically. Patients with lumbar puncture opening pressures ≥52 cm H 2 O at the time of diagnosis are at a higher risk of surgical intervention and should be monitored closely. Elevations in presenting BMI and grade of papilledema may also increase the odds of surgery., (Copyright © 2019. Published by Elsevier Inc.)

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 <4 years old with newly diagnosed increased ICP as determined by using direct measurement and/or clinical criteria. Infants who were premature, neonates, and suspected survivors of abusive head trauma were excluded on the basis of nonocular findings. Fundus examinations were performed; extent, number, and type of RH in each of 4 distinct retinal zones were recorded., 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., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Drs Levin and Feldman have provided paid expert testimony in child abuse cases; the other authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2019 by the American Academy of Pediatrics.)

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., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Background: Cerebral sinovenous thrombosis (CSVT) has been proposed as an alternative cause of retinal hemorrhage (RH) in children being evaluated for abusive head trauma. This study investigated the prevalence and characteristics of RH in children with CSVT., Methods: The medical records of children >6 weeks of age with newly diagnosed CSVT and fundus examination by an ophthalmologist were examined retrospectively. Primary outcomes were presence and patterns of RH., Results: A total of 29 children (median age, 9 years; range, 7 weeks to 17 years) were studied. Of these, 5 (17%) had RH, in 4 of whom RH were peripapillary, superficial, intraretinal, and adjacent to a swollen optic disk. In the fifth child, who had meningitis, sepsis, and multiple cerebral infarcts, there were a moderate number of posterior pole intraretinal hemorrhages. Eighteen children (62%) had optic disk swelling. In 13 children, cerebrospinal fluid opening pressure was recorded (range, 27-59 cm H 2 O). CSVT risk factors included meningitis, mastoiditis, and hypercoagulability., Conclusions: RH in pediatric CSVT was uncommon. When RHs were present, the appearance matched RH patterns known to be caused by medical conditions, such as raised intracranial pressure and sepsis, also present in these children. These findings suggest that the RHs are due to these other causes and not directly to CSVT itself. In children with CSVT, if RHs are multilayered, extend beyond the peripapillary region into the rest of the posterior pole or retinal periphery, or occur in the absence of optic disk swelling, another etiology for the RH should be sought., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

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 H 2 O (range: 21-65 cm H 2 O). 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.

Maddox proposed that the perceived nearness of a target could influence the ocular vergence response. Proximal inputs have been used to refer to all static and dynamic cues to depth other than disparity and blur. In this paper, we review a number of studies in which proximal influences have been assessed. While general agreement exists that proximal contributions are significant when blur and disparity cues are absent (open-loop conditions), there are conflicting reports on the role of proximal vergence and accommodation under closed-loop conditions., (© 2016 Optometry Australia.)

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., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: Raised intracranial pressure (ICP) has been proposed as an isolated cause of retinal hemorrhages (RHs) in children with suspected traumatic head injury. We examined the incidence and patterns of RHs associated with increased ICP in children without trauma, measured by lumbar puncture (LP)., Methods: Children undergoing LP as part of their routine clinical care were studied prospectively at the Children's Hospital of Philadelphia and retrospectively at Nationwide Children's Hospital. Inclusion criteria were absence of trauma, LP opening pressure (OP) ≥ 20 cm of water (cm H2O), and a dilated fundus examination by an ophthalmologist or neuro-ophthalmologist., Results: One hundred children were studied (mean age: 12 years; range: 3-17 years). Mean OP was 35 cm H2O (range: 20-56 cm H2O); 68 (68%) children had OP >28 cm H2O. The most frequent etiology was idiopathic intracranial hypertension (70%). Seventy-four children had papilledema. Sixteen children had RH: 8 had superficial intraretinal peripapillary RH adjacent to a swollen optic disc, and 8 had only splinter hemorrhages directly on a swollen disc. All had significantly elevated OP (mean: 42 cm H2O)., Conclusions: Only a small proportion of children with nontraumatic elevated ICP have RHs. When present, RHs are associated with markedly elevated OP, intraretinal, and invariably located adjacent to a swollen optic disc. This peripapillary pattern is distinct from the multilayered, widespread pattern of RH in abusive head trauma. When RHs are numerous, multilayered, or not near a swollen optic disc (eg, elsewhere in the posterior pole or in the retinal periphery), increased ICP alone is unlikely to be the cause.

Purpose: To report the clinical characteristics of infants with severe retinopathy of prematurity (ROP) and glaucoma-based on review of the Early Treatment for Retinopathy of Prematurity (ETROP) study., Methods: All infants randomized in the ETROP trial were included. Each infant developed high-risk prethreshold ROP in at least one eye. Infants were examined until 6 years postnatal age. The following data were collected: corneal clarity, depth of the anterior chamber, status of the optic nerve and cup/disk ratio, retinal structure, and visual acuity. The diagnosis of glaucoma was based on the investigators best clinical judgment. All infants diagnosed with glaucoma were included in the study., Results: A total of 12 of 718 eyes (1.67%) were diagnosed with glaucoma. Of these, 5 were first reported as having glaucoma at 9 months, 2 at 2 years, 1 each at 3 and 4 years, and 3 at 5 years. The anterior segment was shallow in 7 eyes. Three eyes had normal retinal structure, 1 had macular dragging, and 8 had retinal detachments involving the fovea (stage 4B or worse). At the 6-year follow-up examination only one eye with glaucoma had measurable vision., Conclusions: Although earlier treatment of significant ROP has resulted in better retinal structure and visual acuity outcomes, nearly 2% of the eyes with high-risk prethreshold ROP developed glaucoma at some point during the first 6 years of life., (Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To present strabismus data for children who participated in the Early Treatment for Retinopathy of Prematurity (ETROP) randomized trial., Methods: The prevalence of strabismus, categorized as present or absent, was tabulated for all children with history of high-risk prethreshold retinopathy of prematurity (ROP) who participated in the ETROP randomized trial and were examined at 9 months to 6 years of age. Relationships among strabismus and demographic measures, eye characteristics, and neurodevelopmental factors were analyzed., Results: Among the 342 children evaluated at 6 years, the prevalence of strabismus was 42.2%. Even with favorable acuity scores in both eyes, the prevalence of strabismus was 25.4%, and with favorable structural outcomes in both eyes the prevalence of strabismus was 34.2%. Of children categorized as visually impaired as the result of either ocular or cerebral causes, 80% were strabismic at the 6-year examination. Of 103 study participants who were strabismic at 9 months, 77 (74.8%) remained so at 6 years. Most strabismus was constant at both the 9-month (62.7%) and the 6-year examination (72.3%). After multiple logistic regression analysis, risk factors for strabismus were abnormal fixation behavior in one or both eyes (P < 0.001), history of amblyopia (P < 0.003), unfavorable structural outcome in one or both eyes (P = 0.025), and history of anisometropia (P = 0.04). Strabismus surgery was performed for 53 children. By 6 years, the cumulative prevalence of strabismus was 59.4%., Conclusions: Most children with a history of high-risk prethreshold ROP develop strabismus at some time during the first 6 years of life., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To investigate the effectiveness of 3 surgical preparation techniques in decreasing bacterial contamination of needles and suture material during strabismus surgery., Methods: Consecutive patients requiring 2-muscle strabismus surgery were randomized into 1 of 3 groups. In Group A, patients' periocular skin and bulbar conjunctivae underwent preparation with 5% povidone-iodine; the drape was placed without regard to eyebrows; and an open wire-loop lid speculum was used. Group B patients underwent the same preparation as Group A patients; however, the eyelashes and eyebrows were scrubbed with 5% povidone-iodine on cotton tip applicators, and the drape was placed to exclude the eyebrows from the surgical field. Group C patients underwent the same preparation as Group B patients; however, a bladed lid speculum was used during surgery to exclude some of the eyelashes from the surgical field. After the procedure, all needles and suture materials were sent separately for aerobic culture. The data were analyzed for differences in contamination rates between the groups., Results: Of 77 patients, 24 (31.4%) had either a needle and/or suture contaminant. Groups A, B, and C had mean contamination rates of 29.6%, 34.6%, and 29.2%, respectively. There was no significant statistical variation in contamination among the 3 groups. The most common organism identified was a coagulase-negative staphylococcus strain., Conclusions: More meticulous sterile preparation of the surgical field did not result in a meaningful reduction in suture or needle contamination rates during strabismus surgery., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To determine the efficacy of a home-based computer orthoptic program to treat symptomatic convergence insufficiency., Methods: A retrospective review of consecutive patients with symptomatic convergence insufficiency treated with a home-based computer orthoptic program was performed. Symptomatic convergence insufficiency was defined as: near point of convergence (NPC) >6 cm, decreased positive fusional vergence, exophoria at near at least 4(Δ) greater than at far, and documented complaints of asthenopia, diplopia, or headaches with reading or near work. The Computer Orthoptics CVS program was used for this study. Before beginning the computer orthoptic program, patients with an NPC >50 cm were given 4 base-in prisms and push-up exercises (NPC exercises with an accommodative target) for 2 weeks., Results: A total of 42 patients were included. Mean treatment duration was 12.6 weeks; mean follow-up, 8.5 months. Of the 42 patients, 35 were treated with the home-based computer orthoptic program and push-up exercises; the remaining 7 only used the computer orthoptic program. Because of a remote NPC, 5 patients were given base-in Fresnel prism before starting treatment. Baseline mean NPC was 24.2 cm; posttreatment mean NPC improved to 5.6 cm: 39 patients (92.8%) achieved an NPC of ≤6 cm (p < 0.001). Positive fusional vergence improved in 39 patients (92.8%). Fourteen patients reduced their near exophoria by ≥5(Δ). A total of 27 patients (64.2%) reported resolution of symptoms after treatment., Conclusions: In our study, home-based computer orthoptic exercises reduced symptoms and improved NPC and fusional amplitudes. The computer orthoptic program is an effective option for treating symptomatic convergence insufficiency., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Patients with cystinosis have risk factors known to be associated with secondary increased intracranial pressure. The authors report a series of patients with cystinosis and describe their experience in the diagnosis and management of increased intracranial pressure in this population. The ophthalmologist should be aware of this vision-threatening association., (Copyright 2010, SLACK Incorporated.)

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., (Copyright (c) 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Introduction: The MTI photoscreener (MTI) and the Welch-Allyn SureSight autorefractor are commonly used for preschool vision screening. We compared both of these methods on 100 consecutive patients in a prospective, randomized, masked, clinical trial conducted at a tertiary care center., Methods: One hundred patients between 1 and 6 years of age were included in the study. All participants underwent a comprehensive eye examination with cycloplegic refraction. Examination failure analysis was done on the SureSight data using the manufacturer's referral criteria, the Vision in Preschoolers study (VIP) 90% specificity criteria, the VIP 94% specificity criteria, and the referral criteria proposed by Rowatt and colleagues., Results: Data were successfully obtained on 76% of children using the SureSight and 96% with the MTI. The sensitivity and specificity of the SureSight to detect clinically significant amblyogenic factors using the manufacturer's criteria was 96.6 and 38.1%, using the VIP 90% criteria was 79.3 and 64.3%, using the VIP 94% criteria was 67.2 and 69.0%, and using criteria proposed by Rowatt and colleagues was 62.1 and 73.8%. The sensitivity and specificity of the MTI photoscreener was 94.8 and 88.1%, respectively., Conclusions: Using the manufacturer's referral criteria, the SureSight had a sensitivity equal to the MTI photoscreener; however, the specificity was low and over-referrals were anticipated. As specificity levels were increased, a substantial number of children with amblyogenic risk factors were not appropriately identified within our study population..

Purpose: To determine the relationship of central corneal thickness (CCT) and visual field loss between fellow eyes in primary open-angle glaucoma., Design: Retrospective, observational case series., Methods: Records review of glaucoma patients seen at local Veterans Administration eye clinic. Those with CCT measurements performed within one month of visual field testing were included. Patients were excluded with vision below 20/40 or disease that would affect visual fields. Intrasubject (between fellow eyes) differences in CCT, mean deviation (MD), and pattern standard deviation (PSD) were calculated by subtracting left eye value from right eye value., Results: Of the 100 subjects (94 males), the Spearman correlation coefficient between intrasubject differences in CCT vs intrasubject differences in MD was 0.36 (P = .0003). The Spearman correlation for differences in CCT vs differences in PSD was -0.31 (P = .0019)., Conclusions: Our study suggests that worse visual field changes tend to occur in the eye with the thinner cornea.

A 59-year-old woman presented with periocular pain and 1.5 mm of relative proptosis. A computed tomography scan demonstrated thickening of the orbital floor, and an endoscopic sinusectomy revealed chronic sinusitis. Although cases of maxillary sinus disease with orbital floor thickening have been reported, these findings are in contrast to cases of silent sinus syndrome, in which maxillary sinus disease induces thinning and inferior displacement of the orbital floor with secondary enophthalmos and hypoglobus. Thus, while chronic maxillary sinus disease may create negative antral pressure and inferior displacement of the orbital floor, whether orbital floor thinning or thickening occurs varies with each case. The present case represents a combination of maxillary sinusitis with orbital floor thickening and exophthalmos, a less common manifestation of maxillary sinus-related orbitopathy.

Free-radical-mediated oxidant damage can contribute to acute hepatitis. Vitamin E, a classic antioxidant, has been tested as a therapy for rodent acute hepatitis, but the protection achieved has not been complete. This study demonstrated that in rats, sodium diethyldithiocarbamate (DDC), a potent antioxidant, strongly depressed galactosamine-induced hepatitis in terms of serum alanine amino transferase activities and bile acids, though not in terms of serum beta-glucuronidase activities. A potential limitation for DDC use in humans, inhibition of copper metalloenzyme activities, did occur at the DDC dose used here. However, these effects were not severe. Thus, DDC could make a useful short term therapeutic drug for acute hepatitis., (Copyright 2002 Elsevier Science Ltd.)