The relationship between severe hypertensive diseases of pregnancy and moderate-severe bronchopulmonary dysplasia.

OBJECTIVE: Determine the association between severe hypertensive disease of pregnancy (HDP) with moderate-severe bronchopulmonary dysplasia (BPD) in preterm infants (< 31 weeks' gestation). STUDY DESIGN: Preterm birth cohort study of 693 mother-infant dyads. Severe HDP was defined as severe preeclampsia, HELLP syndrome or eclampsia. The outcome was moderate-severe BPD classified at 36 weeks corrected gestational age, per the NICHD Consensus statement. RESULTS: 225 (32%) mothers developed severe HDP and 234 (34%) infants had moderate-severe BPD. There was an interaction between severe HDP and gestational age (p = 0.03). Infants born at < 25 weeks gestation to mothers with HDP had increased odds for moderate-severe BPD compared to infants of normotensive mothers delivering at the same gestational age. Infants born > 28 weeks to mothers with severe HDP had decreased odds for the outcome, though not statistically significant. CONCLUSIONS: Severe HDP has a differential effect on the development of moderate-severe BPD based on gestational age.

A Third-Year Medical School Ophthalmology Curriculum for a Longitudinal Integrated Clerkship Model.

Background Longitudinal Integrated Clerkships (LICs) are innovative educational models that allow medical student continuity with patients, preceptors, colleagues, and health care systems. Given their benefits, the number of LICs continues to increase. We share a pilot model for an ophthalmology LIC curriculum at the University of Colorado School of Medicine targeted for students to see patients through transitions of care. Methods A needs assessment was performed including literature search, interviews with expert faculty, and a precurricular student questionnaire. Based on our findings, we developed a pilot two-part curriculum consisting of an introductory lecture and a half-day clinical experience designed to integrate patient eye care into the LIC model. At the end of the year, students completed a questionnaire assessing attitude, confidence, and knowledge. Precourse data were collected from students in the academic year (AY) 2018/2019 to aid with the needs assessment. Postcourse data were collected after completion of the curriculum from students in AY 2019/2020. Data from questionnaire were intended to improve our curricular experience. Results Our curriculum was piloted between the 2019 and 2020 AY. The completion rate of our curriculum was 100%. The questionnaire response rate was 90% in pre- and postcurricular groups ( n =15/17 and n =9/10, respectively). Hundred percent of students from both groups responded that it is "very important"/"important" for all physicians to be able to identify when ophthalmology referral is indicated. After the intervention, there were significant differences in the rate of students responding that they were "confident" diagnosing acute angle-closure glaucoma (36 vs. 78%, p =0.04), treating a chemical burn (20 vs 67%, p =0.02), and diagnosing viral conjunctivitis (27 vs. 67%); 90% of students reported increased confidence in longitudinal care of patients in the eye clinic. Conclusions Medical students believe in the importance of ophthalmic education regardless of their specialty of choice. We present a pilot model to introduce ophthalmology within an LIC model. Future studies with a larger sample are needed to determine the impact of this model in terms of knowledge acquisition and relationship between curriculum and ophthalmology interest among students. Our curriculum can be adapted to other underrepresented specialties in the medical school curriculum and is easily exportable to other LICs.

Risk of Refractive Prediction Error After Cataract Surgery in Patients with Thyroid Eye Disease.

PURPOSE: To investigate the risk of unexpected refractive prediction error after cataract surgery in patients with thyroid eye disease (TED) at the University of Colorado. PATIENTS AND METHODS: A retrospective observational study was performed using records of patients who underwent cataract surgery (2014 to 2018) who were included in a Cataract Surgery Outcomes database. Any patient with documentation of thyroid eye disease (TED) in the medical record was classified as TED. Post-operative refraction error greater than or equal to ±1.0 diopter from the target refraction was the main outcome of this study. Eyes with history of refractive surgery, ocular trauma, retinal detachment, non-Graves' disease thyroid conditions or Graves' disease without TED, and eyes without refractive error at follow-up were excluded. RESULTS: A total of 5716 eyes from 3692 patients who underwent cataract surgery were analyzed. Sixty-five eyes of thirty-nine patients (1.1%) had TED. Former and/or current cigarette use was associated with having TED (p = 0.0504). Patients with TED had a statistically significant shorter axial length as compared to eyes without TED (p = 0.0257). Three hundred and forty-nine eyes (6.1%), including 9 eyes (13.8%) in patients with TED, had refractive prediction error greater than ±1.0 diopter following surgery (univariate OR = 2.5, 95% CI: 1.1-5.7, p = 0.0274). After multivariate analysis controlling for race, tobacco use, combined surgery, and axial length, TED was associated with an increased risk of our primary outcome, refractive prediction error greater than ±1.0 diopter (OR = 2.4, 95% CI: 1.0-5.7, p = 0.0506). CONCLUSION: Patients with TED are at increased risk for refractive prediction error following cataract surgery. Discussion with patients regarding their risk and possible need for glasses following surgery is important for setting realistic patient expectations.

Ocular Disorders in the Newborn.

Visual development begins at birth and continues throughout childhood. Ocular pathology can lead to permanent visual impairment and subsequent problems in overall development and school performance as well as signify serious systemic disease or even life-threatening malignancies. Prompt identification of congenital or early acquired ocular pathology via regular bedside ocular examinations and assessments of visual behaviors by neonatal clinicians is a critical component of newborn care. The goal of this article is to review the components of the newborn eye examination, describe key ocular findings and diagnoses that are common or critical to identify in the newborn period, and offer guidance on necessity and timing of ophthalmology referral.

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.].

Trends in Retinopathy of Prematurity over 12 Years in a Colorado Cohort.

PURPOSE: To determine trends in retinopathy of prematurity (ROP) in a Colorado cohort between 2006 and 2017 and compare trends in risk factors between our cohort and statewide data. METHODS: A retrospective cohort study was conducted by the use of records from two registry databases: 1) an academic center's ROP registry, and 2) vital statistics birth data from the Colorado Department of Public Health and Environment (CDPHE). ROP was categorized as severe (type 1 or type 2), low grade (not type 1 or type 2), or no ROP. Other variables included in the analyses were gestational age and birth weight at delivery, and infant mortality. Trends over time were evaluated for both registry databases using generalized linear models. RESULTS: In our ROP registry cohort of 1,267 eligible infants, 134 (10.6%) developed severe ROP and 279 (22%) developed low-grade ROP. We found no overall trend in severe ROP rates (p = .23), and a decreasing trend in rates of low-grade ROP (p < .01) over the study period. Trends in gestational age, birth weight, and mortality rates remained stable during the study period in both the ROP registry and the CDPHE cohorts. CONCLUSION: The rate of severe ROP in our ROP registry cohort did not change over time. There was evidence of a decreasing trend in low grade ROP during the 12-year study period that was not explained by a change in the primary ROP risk factors in either the ROP registry cohort or the Colorado statewide data.

Cost comparison of different treatment approaches of dacryocystitis and dacrocystocele.

PURPOSE: Congenital dacrocystocele with potential for dacryocystitis are common ophthalmic findings in children. There are multiple surgical approaches to open the mucocele. In this study, we look at the financial impact of these different approaches. METHODS: A retrospective chart review of 17 patients with dacrocystocele or dacryocystitis was performed. We examined four approaches: (1) bedside nasal endoscopy with marsupialization of nasolacrimal duct (NLD) cyst, (2) surgically performed nasal endoscopy with marsupialization of NLD cyst, (3) NLD probe, and (4) a combination of procedures. Cost of the procedure and length of anesthesia were collected. Reoccurrence of symptoms and disease post-procedure were also collected. RESULTS: The lowest cost billed procedure was bedside nasal endoscopy performed by an otolaryngologist (US$435; n = 1). A nasal endoscopy (n = 2) performed in the operating room (OR) had an average OR fee of US$14,557 [standard deviation (SD): US$7598] for 108.5 (SD: 87.0) min of operating time. An NLD probe (n = 5) performed by pediatric ophthalmologists resulted in an average OR fee of US$5540 (SD: US$1752) for 31.0 min (SD: 8.6 min) of operating time. A combination of both nasal endoscopy and NLD probing (n = 9) had an average OR fee US$10,325 (SD: US$4137) for 69 min (SD: 34.5 min) of operating time. CONCLUSION: This is the first study looking at cost benefit of four different approaches to treating dacrocystoceles/dacryocystitis. A NLD probe was a low-cost OR intervention and had the shortest operating time. The combination procedure was more cost-effective than nasal endoscopy or NLD probing alone.

Comparison of Different Surgical Approaches to Inferior Oblique Overaction.

BACKGROUND: The purpose of this study is to compare surgical outcomes of recession, anteriorization, and myotomy for the treatment of inferior oblique overaction (IOOA). METHODS: A retrospective chart review of all patients undergoing IOOA correction from July 2010 to March 2017 at the Children's Hospital of Colorado was performed. Preoperative grading of IOOA (+0.5 to +4.0) was compared to post-operative IOOA (0 to +4.0). The goal was reduction of IOOA to 0, but any decrease in IOOA was measured. RESULTS: There were a total of 260 patients with 357 eyes. Gender and age were similar across surgery types. A 94.6% of eyes had a decrease in IOOA with recession (n = 165) of the inferior oblique while 86.1% decreased to no IOOA. Anteriorization of the inferior oblique (n = 115) decreased overaction in 97.4% of eyes with 81.7% improving to zero degree of IOOA. Myotomy of the inferior oblique (n = 77) was found to decrease overaction in 98.7% of eyes and reduce IOOA to zero in 88.3%. There was no significant difference among type of surgery and outcome. CONCLUSION: All three surgical interventions were found to be equally successful in reducing the amount of IOOA.

IRF2BPL gene mutation: Expanding on neurologic phenotypes.

Heterozygous loss of function variants in the IRF2BPL are a newly described cause of neurodevelopmental disabilities and epilepsy. As of 2019, fewer than 20 patients have been described in the published literature. This article reports an additional case of a patient with a pathogenic IRF2BPL variant and offers a comprehensive review of the published cases of individuals with IRF2BPL variants, in order to help expand the phenotype. The patient has a history of infantile spasms evolving into drug-resistant epilepsy with underlying epileptic encephalopathy consistent with Lennox-Gastaut syndrome. While at the extreme end of the spectrum, his phenotype is consistent with those previously described. Our literature review highlights the wide range of phenotypes exhibited by those with diseases related to IRF2BPL gene variants. This article also briefly discusses other comorbidities seen in the patient and those previously reported. While the molecular underpinnings of the role of IRF2BPL gene in the central nervous system are newly established, the specifics of its effects elsewhere have yet to be delineated. Furthermore, its pathogenesis in other organ systems is not yet understood and could be of importance from a management perspective.

Relationship between severe bronchopulmonary dysplasia and severe retinopathy of prematurity in premature newborns.

BACKGROUND: Bronchopulmonary dysplasia (BPD) and retinopathy of prematurity (ROP) are two adverse sequelae of preterm birth associated with abnormal vascular development. The purpose of this study was to characterize the relationship between these two outcomes at a single institution. METHODS: The medical records of infants screened for ROP at the University of Colorado Hospital between January 2012 and December 2017 were reviewed retrospectively. ROP was classified according to Early Treatment Retinopathy of Prematurity (ET-ROP) criteria; BPD, according to the 2010 Criteria from the National Institute for Child Health and Human Development. We examined the relationship between moderate-severe BPD and the development of severe ROP (type 1 or 2) using univariate analysis and multivariable logistic regression with the odds ratio as a measure of association. Covariates included gestational age and birth weight at delivery. RESULTS: A total of 625 cases were reviewed. Of these, 64 infants (10%) developed severe ROP and 176 (28%) infants developed moderate-severe BPD. We found a significant relationship between these two outcomes following adjustments for gestational age, birth weight, and multiparity (OR = 3.2; 95% CI, 1.6-6.5 [P < 0.01]). CONCLUSIONS: In our cohort of preterm infants, we found a significant relationship between moderate-severe BPD with severe ROP. We hypothesize that these two neonatal outcomes have links with a common pathogenesis.

Frequency of pediatric traumatic cataract and simultaneous retinal detachment.

PURPOSE: Traumatic cataract in children is a treatable cause of vision loss. In cases of simultaneous retinal detachment, the prognosis for visual recovery is often poor. The purpose of this study was to investigate risk factors for concurrent retinal detachment in patients with traumatic cataract. METHODS: A retrospective review of patients diagnosed with traumatic cataract at Children's Hospital Colorado between 2005 and 2014 was conducted. Demographics, mechanism of injury, and incidence of retinal detachment were recorded. Logistic modeling with generalized estimating equations to account for correlation of eyes within patients was used to analyze associations between potential risk factors and retinal detachment. RESULTS: A total of 62 total eyes with traumatic cataract were included: 52 patients presented with unilateral cataract; 5 presented with bilateral cataracts. Mean patient age was 8.4 ± 4.1 years (range, 0-16 years), and 83% of patients were male. A total of 9 eyes (14.5%) had comorbid retinal detachment. Traumatic cataracts caused by self-injurious hitting were more likely to present with simultaneous retinal detachment than those caused by other mechanisms of injury (OR = 24.0; 95% CI, 3.8-153.3; P = 0.0010). CONCLUSIONS: Patients with traumatic cataract who display self-injurious behavior are at higher risk for concurrent retinal detachment. These patients can often only be examined under sedation. Ophthalmologists should counsel families of high-risk patients and consider involving retinal specialists in surgical planning.

Pediatric ocular trauma caused by recreational drones: two case reports.

Drones are increasingly being used by children and adults recreationally and commercially. The propeller blades when spinning at high speeds may cause serious harm to the eye and orbital structures. We report 2 cases of injuries to the eye and orbital structures caused by drones.

T-tubule remodeling and increased heterogeneity of calcium release during the progression to heart failure in intact rat ventricle.

A highly organized transverse-tubule (TT) system is essential to normal Ca2+ cycling and cardiac function. We explored the relationship between the progressive disruption of TTs and resulting Ca2+ cycling during the development of heart failure (HF). Confocal imaging was used to measure Ca2+ transients and 2-D z-stack images in left ventricular epicardial myocytes of intact hearts from spontaneously hypertensive rats (SHR) and Wistar-Kyoto control rats. TT organization was measured as the organizational index (OI) derived from a fast Fourier transform of TT organization. We found little decrease in the synchrony of Ca2+ release with TT loss until TT remodeling was severe, suggesting a TT "reserve" characterized by a wide range of TT remodeling with little effect on synchrony of release but beyond which variability in release shows an accelerating sensitivity to TT loss. To explain this observation, we applied a computational model of spatially distributed Ca2+ signaling units to investigate the relationship between OI and excitation-contraction coupling. Our model showed that release heterogeneity exhibits a nonlinear relationship on both the spatial distribution of release units and the separation between L-type Ca2+ channels and ryanodine receptors. Our results demonstrate a unique relationship between the synchrony of Ca2+ release and TT organization in myocytes of intact rat ventricle that may contribute to both the compensated and decompensated phases of heart failure.

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.

Specialty Practice and Cost Considerations in the Management of Uveitis Associated With Juvenile Idiopathic Arthritis.

PURPOSE: To evaluate whether cost, prior insurance authorization concerns, and subspecialty practice influence therapeutic decisions in the treatment of uveitis associated with juvenile idiopathic arthritis. METHODS: A total of 2,965 pediatric ophthalmologists, uveitis specialists, retina specialists, and rheumatologists across the United States were surveyed via e-mail regarding their choice in long-term therapy for a hypothetical patient with uveitis associated with juvenile idiopathic arthritis. Outcomes of interest were differences in therapy choice based on cost/prior authorization and specialty practice. RESULTS: There were significant differences in the use of methotrexate and biologics among specialists, both with and without consideration for cost and prior authorization. CONCLUSIONS: Physicians in four different specialties who treat uveitis associated with juvenile idiopathic arthritis agree on methotrexate as a first-line treatment choice and a biologic immunosuppressive medication as a second choice, but there are significant differences between the specialties in their use of these medications. Cost and insurance considerations did not affect therapy selection. [J Pediatr Ophthalmol Strabismus. 2016;53(4):246-251.].