Clinical Utility of Electroretinograms for Evaluating Vigabatrin Toxicity in Children.

PURPOSE: To determine changes in the clinical treatment of pediatric patients taking vigabatrin for seizure control in response to results of electroretinogram (ERG) performed for retinal toxicity screening. METHODS: The authors retrospectively reviewed the medical records of patients who received ERGs at Children's Hospital of Colorado from 2009 to 2012. Age, indication for ERG, ERG data, and clinical management of vigabatrin were extracted from the records. ERGs were interpreted according to LKC Technologies normative values. A physician trained in ERG analysis interpreted each ERG. RESULTS: One hundred seventy ERGs were performed during the study period, and 147 ERGs were available for analysis. Every patient received general anesthesia for the procedure. Thirty-three ERGs were performed in 29 patients specifically as screening for retinal toxicity due to vigabatrin use, and 30 were available for analysis. Within this cohort, only 2 ERGs were normal (6.6%), and 28 were abnormal (93.3%). In patients who received abnormal results, 1 patient discontinued vigabatrin in response to the screening. CONCLUSIONS: In this study cohort, clinical management generally did not change in response to an abnormal screening result. Given the need for general anesthesia in the pediatric population receiving ERG testing, and minimal change in clinical decision-making in the face of abnormal results, ERG screening for retinal toxicity due to vigabatrin in the pediatric cohort should be reconsidered. [J Pediatr Ophthalmol Strabismus. 2021;58(3):174-179.].

Validation of WINROP for detecting retinopathy of prematurity in a North American cohort of preterm infants.

BACKGROUND: WINROP (weight, insulin-like growth factor 1, neonatal, retinopathy of prematurity) is a web-based retinopathy of prematurity (ROP) risk algorithm that uses postnatal weight gain as a surrogate of insulin-like growth factor-1 (IGF-1) to predict the risk of severe ROP in premature infants. The purpose of this study was to validate the web-based algorithm WINROP in detecting severe (type 1 or type 2) ROP in a North American cohort of infants. METHODS: The records of consecutive infants who underwent ROP examinations between 2008 and 2011 were reviewed retrospectively. Infants were classified into categories of "alarm" (at risk for developing severe ROP) and "no alarm" (minimal risk for severe ROP). RESULTS: A total of 483 were included. Alarm occurred in 241 neonates (50%), with the median time from birth to alarm of 2 weeks. WINROP had a sensitivity of 81.8% (95% CI, 67.3%-91.8%) and specificity of 53.3% (95% CI, 48.5%-58.0%) for identifying infants with severe ROP. Eight of the 44 infants with severe ROP were not detected (5 with type 1 and 3 with type 2). Of these 8 infants, 7 (88%) had birth weight in excess of the 70th pecentile. With additional weight data entry, sensitivity of WINROP rose to 88.6%. CONCLUSIONS: Very preterm infants (gestational age of ≤27 weeks) with relatively high birth weight for gestational age may not be detected by WINROP as high risk for developing severe ROP.

Colorado retinopathy of prematurity model: a multi-institutional validation study.

PURPOSE: The Colorado retinopathy of prematurity (ROP) prediction model (CO-ROP), developed using a cohort of infants from Colorado, calls for ROP examination of infants meeting all of the following criteria: gestational age of ≤30 weeks, birth weight of ≤1500 g, and a net weight gain of ≤650 g between birth and 4 weeks of age. The purpose of this study was to perform an external validation to assess the sensitivity and specificity of the CO-ROP model in a larger cohort of babies screened for ROP from four academic institutions in the United States. METHODS: The medical records of neonates screened for ROP according current national guidelines was conducted at 4 US academic centers were retrospectively reviewed. Sensitivity, specificity, and respective 95% confidence intervals in detecting ROP using CO-ROP were calculated for type 1, type 2, and any grade of ROP. RESULTS: A total of 858 cases were included. The CO-ROP algorithm had a sensitivity of 98.1% (95% CI, 93.3%-99.8%) for type 1 ROP, 95.6% (95% CI 78.0-99.9%) for type 2 ROP, and 95.0% (95% CI, 93.1-97.4%) for all grades of ROP. The CO-ROP model would have reduced the total number of infants screened by 23.9% compared to current 2013 screening guidelines. CONCLUSIONS: CO-ROP demonstrated high sensitivity in predicting ROP and would have greatly reduced the number of infants needing examination.

The Colorado-retinopathy of prematurity model (CO-ROP): postnatal weight gain screening algorithm.

PURPOSE: To describe a novel retinopathy of prematurity (ROP) screening model incorporating birth weight, gestational age, and postnatal weight gain that maintains sensitivity but improves specificity in detecting all grades of ROP compared to current 2013 screening guidelines. METHODS: The medical records of 499 neonates from a single tertiary referral center who met the 2013 screening guidelines for ROP were retrospectively reviewed. Weekly weights were analyzed using standard logistic regression to determine the age at which the weekly net weight gain best predicted the development of ROP, which was designated as the postnatal weight gain criterion. The 2013 birth weight and gestational age criteria were included in an "and" fashion to form the CO-ROP model. Sensitivities and specificities in detecting high grade (type 1 and 2) and all grades of ROP were calculated. RESULTS: The CO-ROP model screens infants with a gestational age at birth of ≤30 weeks and birth weight of ≤1500 g and net weight gain of ≤650 g between birth and 1 month of age. In our cohort, CO-ROP had a sensitivity of 100% (95% CI, 92.1%-100.0%) for high-grade (type 1 and 2) ROP and 96.4% (95% CI, 92.3%-98.7%) for all grades of ROP. It would reduce the number of infants screened by 23.7% compared to 2013 guidelines. Calibrating the model to detect only high-grade ROP would result in a 45.9% reduction in the total number of infants screened. CONCLUSIONS: CO-ROP is a simple model that maintains a statistically similar sensitivity in detecting all grades of ROP while significantly reducing the total number of required ROP screenings compared to 2013 guidelines. The study had a small sample size but shows promise for future research and clinical efforts.

Pharmacokinetics and safety of intravenous cidofovir for life-threatening viral infections in pediatric hematopoietic stem cell transplant recipients.

Children undergoing hematopoietic stem cell transplantation (HSCT) are at risk for life-threatening viral infections. Cidofovir is often used as a first-line agent for adenovirus infections, despite the absence of randomized controlled trials with HSCT patients, and as a second-line agent for resistant herpesvirus infections. The frequency and severity of adverse effects, particularly nephrotoxicity, in pediatric HSCT recipients are unclear, and pharmacokinetics (PK) of cidofovir in children have not previously been reported. This study was an open-label, nonrandomized, single-dose pilot study to determine the safety and PK of cidofovir in pediatric HSCT recipients with symptomatic adenovirus, nucleoside-resistant cytomegalovirus (CMV) or herpes simplex virus (HSV), and/or human papovavirus infections. Subsequent dosing and frequency were determined by clinical response and side effects, as assessed by the treating physician. Blood and urine samples were obtained from patients for PK studies and assessment of toxicity and virologic response. Twelve patients were enrolled (median age, 9 years; 33.5 days posttransplantation). Four of seven patients with adenovirus infection were successfully treated and eventually cleared their infections. Four of twelve patients died of disseminated viral disease and multiorgan failure. Two of twelve patients had evidence of acute kidney injury after the first dose, and one of these patients developed chronic kidney disease; two other patients developed late nephrotoxicity. The mean drug half-life was 9.5 h. There was no correlation between nephrotoxicity and plasma maximum concentration, clearance, or half-life. PK were similar to those reported for adults, although the drug half-life was significantly longer than that for adults. Cidofovir was well tolerated in the majority of patients. However, effective therapeutic strategies are urgently needed to support patients until immune reconstitution is achieved.

Pediatric traumatic hyphema: a review of 138 consecutive cases.

PURPOSE: To report the demographics and outcomes in children (<18 years of age) who developed hyphema from ocular trauma and were subsequently cared for at a tertiary medical center. METHODS: The medical records of consecutive patients seen at Children's Hospital Colorado diagnosed with traumatic hyphema between September 1, 2003, and December 31, 2011, were retrospectively reviewed. The following data were recorded: patient age, parent/guardian-reported ethnicity, sex, injury location, visual acuity, and intraocular pressure (IOP) at presentation and follow-up. RESULTS: A total of 138 cases of unilateral hyphema were included, with 88% occurring in boys (mean age, 10.1 years; range, 1-19). Over 90% of injuries occurred in the home setting, with the most common mechanisms of injury being general play, projectiles from guns, and sports injuries occurring during games or practice. Only 3 patients had visual acuity <20/40 at 1 month's follow-up, and no patient experienced a rebleeding event. Most of the 33 patients with elevated IOP were managed medically; 4 (12%) required surgery. CONCLUSIONS: The majority of children with traumatic hyphema in this patient cohort were injured in the home setting. Very few patients underwent surgery for ocular hypertension, but higher IOP at presentation was associated with the need for surgical intervention. Outpatient care with activity restriction and topical medications usually led to resolution of hyphema without serious complications or visual loss.

Socioeconomics of retinopathy of prematurity care in the United States.

BACKGROUND AND PURPOSE: To elucidate the experience of pediatric ophthalmologists across the United States who care for infants with retinopathy of prematurity (ROP). METHODS: Seven hundred and ten surveys were mailed to U.S. members of the American Association of Pediatric Ophthalmology and Strabismus, and 283 (40%) of 710 surveys were completed. Surveys were reviewed and statistical analysis was completed by the authors. RESULTS: There was no uniformity of contract compensation or method for determining the value of ROP care. Almost half of the respondents felt they would generate more income if they did not perform ROP screening. Eighty percent of respondents that were happy with their ROP compensation had a contract for those services. One third of respondents had their malpractice insurance provided by the hospital. Retina specialists performed 40% of inpatient ROP screening and 53% of treatment. Most respondents continued to care for infants with ROP once discharged. CONCLUSIONS: In the United States, there is no uniform experience regarding compensation for ROP care, or a methodology for determining the value of services and coverage of liability insurance. These findings are consistent with previous studies. Lack of uniform compensation and high liability pose a threat to the future of ROP care.

Socioeconomics of retinopathy of prematurity in-hospital care.

OBJECTIVE: To determine if there was any uniform experience across the United States relative to retinopathy of prematurity (ROP) services provided, reimbursement, and malpractice insurance coverage. METHODS: An online pediatric ophthalmology listserv poll queried pediatric ophthalmologists regarding ROP screening, reimbursement, malpractice insurance, and call and consult coverage. RESULTS: Compensation for providing ROP services is quite variable around the United States. The Southern respondents reported the highest contract income while the Northeast reported the lowest. The mean annual contract income was $63 753 and the median annual contract income was $39 000. There was an even distribution between physicians vs hospitals providing malpractice coverage. There was also a fairly even distribution between physicians who do and do not provide consult and call coverage. CONCLUSIONS: Nationwide, there is no standard rate of compensation for ROP in-hospital care, coverage of liability insurance, or providing additional consult or on-call services. Income generation performing ROP screening examinations is roughly half what a pediatric ophthalmologist can generate by seeing patients in the clinic or performing surgery.

Incidental detection of bilateral corectopia by photo screening leads to the diagnosis of multiple sclerosis. A case report.

PURPOSE: To describe a case of an incidental finding of bilateral corectopia detected by photo screening which ultimately led to the diagnosis of multiple sclerosis. METHODS: Case presentation and literature review. RESULTS: Corectopia may be congenital or acquired. Midbrain corectopia is commonly caused by infarction and demyelinating disease can cause autonomic pupil abnormalities resulting in corectopia. CONCLUSION: A careful history and ocular examinatin can aid in determining the etiology of corectopia. Additional genetics or neurologic consultation may be necessary to diagnosis systemic disease.

Pseudohypopyon: Extramedullary relapse of acute myelogenous leukemia with poor prognosis.

An 11-month-old female presented to the emergency department with a 2-week history of fever, increasing fussiness, emesis, and decreased urine output. She was diagnosed with acute myelogenous leukemia. Systemic chemotherapy with intensified intrathecal cytarabine was started, and the patient achieved a clinical remission after the first course of induction. Towards the end of her second course of induction she developed pseudohypopyon in each eye on consecutive days, heralding a central nervous system relapse.