Emily A. McCourt

Measurement of Subfoveal Choroidal Thickness Using Spectral Domain Optical Coherence Tomography

BACKGROUND AND OBJECTIVEnTo compare subfoveal choroidal thickness (SFCT) in normal patients and those with known ocular pathology using spectral domain optical coherence tomography (SD-OCT).nnnPATIENTS AND METHODSnThis retrospective, observational case series was conducted at a tertiary care center where 194 consecutive eyes from 102 patients were imaged. Patients were not included or excluded based on presence or absence of pathology. One masked observer imaged the choroid and a second masked observer measured SFCT. Multivariate analysis was used and a statistical model created to analyze the changes in SFCT induced by age, diabetic retinopathy, glaucoma, wet and dry age-related macular degeneration, and other posterior pole pathology.nnnRESULTSnThe mean SFCT of the 194 eyes studied was 246.59 ± 93.17 μm with a mean age of 55.50 ± 19.70 years. A strong negative relationship was found between age and SFCT (R(2) = 0.42), with an average 3.09-μm decrease in SFCT per additional year of age. Subgroup analysis demonstrated that patients with diabetic retinopathy, wet or dry age-related macular degeneration, and glaucoma all had SFCT measurements that were statistically significantly less than those of normal patients. However, when regression analysis was used to control for age, this difference was no longer significant.nnnCONCLUSIONnNo differences were found in SFCT in patients with glaucoma, macular degeneration, or diabetic retinopathy compared to eyes lacking pathology when age was counted as a confounding variable. Age has a strong inverse relationship with SFCT, independently confirming prior studies and creating a foundation for more research on the relationship between ocular pathology and choroidal thickness.

Ocular disease in the cobalamin C defect: A review of the literature and a suggested framework for clinical surveillance

The association between combined methylmalonic acidemia and homocystinuria of cblC type (cobalamin C defect, cblC) and ocular disease is now well recognized, and is a significant component of morbidity and disability associated with the condition. In this review, through collation of historically reported cases of early- and late-onset cblC and previously unreported cases, we have attempted to characterize the epidemiology, clinical features, and pathomechanisms of individual ocular features of cblC. These data suggest that maculopathy and nystagmus with abnormal vision are extremely common and affect the majority of children with early-onset cblC, usually before school age; strabismus and optic atrophy are also seen at relatively high frequency. The timing of progression of macular disease may coincide with a critical period of postnatal foveal development. Maculopathy and retinal disease may be subclinical and show only partial correlation with the extent of visual deficits, and visual deterioration may be relentlessly progressive in spite of aggressive treatment of biochemical abnormalities. In later-onset forms of the disease, visual loss and ocular complications appear to be infrequent. Finally, we discuss investigational strategies in diagnosing and characterizing eye disease in individuals with cblC, explore possible therapeutic avenues that may attenuate progression and severity of eye disease, and propose a clinical surveillance guideline for monitoring progression of ocular disease in children and adults with cblC.

Pediatric traumatic hyphema: a review of 138 consecutive cases

PURPOSEnTo report the demographics and outcomes in children (<18xa0years of age) who developed hyphema from ocular trauma and were subsequently cared for at a tertiary medical center.nnnMETHODSnThe medical records of consecutive patients seen at Childrens 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.nnnRESULTSnA total of 138 cases of unilateral hyphema were included, with 88% occurring in boys (mean age, 10.1xa0years; 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 1xa0months follow-up, and no patient experienced a rebleeding event. Most of the 33 patients with elevated IOP were managed medically; 4 (12%) required surgery.nnnCONCLUSIONSnThe 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.

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

PURPOSEnTo 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.nnnMETHODSnThe 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.nnnRESULTSnThe CO-ROP model screens infants with a gestational age at birth of ≤30xa0weeks and birth weight of ≤1500xa0g and net weight gain of ≤650xa0g between birth and 1xa0month 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.nnnCONCLUSIONSnCO-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.

Isolated group B streptococcal endogenous endophthalmitis simulating retinoblastoma or persistent fetal vasculature in a healthy full-term infant.

Group B streptococcus (GBS) is a potentially devastating neonatal pathogen that most commonly causes meningitis, sepsis, and pneumonia. It is also a very rare cause of endogenous endophthalmitis. We present the second case of endogenous endophthalmitis caused by GBS in a healthy newborn and the first case of endogenous endophthalmitis by GBS in a newborn mimicking retinoblastoma and resulting in enucleation.

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

PURPOSEnThe 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 ≤30xa0weeks, birth weight of ≤1500xa0g, and a net weight gain of ≤650xa0g between birth and 4xa0weeks 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.nnnMETHODSnThe 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.nnnRESULTSnA 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.nnnCONCLUSIONSnCO-ROP demonstrated high sensitivity in predicting ROP and would have greatly reduced the number of infants needing examination.

The relationship of the subtypes of preterm birth with retinopathy of prematurity

Background Retinopathy of prematurity is an adverse outcome of preterm birth and is a leading cause of childhood blindness. The relationship between the subtypes of preterm birth with retinopathy of prematurity is understudied. Objective To investigate whether there is a difference in the incidence of type 1 or type 2 retinopathy of prematurity in infants with preterm birth resulting from spontaneous preterm labor, a medical indication of preterm birth, or preterm premature rupture of the membranes. Study Design A retrospective cohort study was conducted of 827 infants screened for retinopathy of prematurity who were delivered at a single tertiary care center in Colorado. All infants fulfilled the American Academy of Pediatrics 2013 screening criteria for retinopathy of prematurity defined as “infants with a birth weight of ≤1500 g or gestational age of 30 weeks or less (as defined by the attending neonatologist) and selected infants with a birth weight between 1500 and 2000 g or gestational age of >30 weeks with an unstable clinical course, including those requiring cardiorespiratory support and who are believed by their attending pediatrician or neonatologist to be at high risk for retinopathy of prematurity.” Two independent reviewers masked to retinopathy of prematurity outcomes determined whether preterm birth resulted from spontaneous preterm labor, medical indication of preterm birth, or preterm premature rupture of the membranes. Discrepancies were resolved by a third reviewer. Data were analyzed with univariate and multivariable logistic regression. Results In our cohort, the frequency of preterm birth resulting from spontaneous preterm labor, medical indication of preterm birth, or preterm premature rupture of the membranes was 34%, 40%, and 26%, respectively. The mean gestational age (weeks, days) ± SD (range) in the cohort and across the preterm birth subtypes was as follows: entire cohort, 28 weeks, 6 days ± 2 weeks, 3 days (23 weeks, 3 days – 36 weeks, 4 days); spontaneous preterm labor, 28 weeks 1 day ± 2 weeks, 3 days (23 weeks, 3 days – 33 weeks, 4 days); medical indication of preterm birth, 29 weeks, 1 day ± 2 weeks, 2 days (24–36 weeks, 4 days); preterm premature rupture of the membranes, 28 weeks, 4 days ± 2 weeks, 1 day (24–33 weeks, 1 day). Among infants with type 1, type 2, or no retinopathy of prematurity, the incidence of type 1 or type 2 retinopathy of prematurity in births from spontaneous preterm labor, medical indication of preterm birth, and preterm premature rupture of the membranes was 37 of 218 (17%), 27 of 272 (10%), and 10 of 164 (6%), respectively. Adjusted for gestational age, birth weight, and multiparity and compared with the preterm premature rupture of the membranes group, the odds ratios of spontaneous preterm labor and medical indication of preterm birth for type 1 or type 2 retinopathy of prematurity were 6.1 (95% confidence interval, 1.8 to 20, P = .003) and 5.5 (95% confidence interval, 1.4 to 21, P = .01), respectively. Among neonates born after preterm premature rupture of the membranes, the probability of developing type 1 or type 2 retinopathy of prematurity was greatest in infants with rupture of membrane duration of up to 24 hours. After 24 hours, the probability of developing type 1 or type 2 retinopathy of prematurity declined. The odds of developing type 1 or type 2 retinopathy of prematurity was 9.0 (95% confidence interval 2.3 to 34, P = .002) in infants who had preterm premature rupture of the membranes ≤ 24 hours compared with infants who had preterm premature rupture of the membranes > 24 hours. Conclusion Type 1 or type 2 retinopathy of prematurity are adverse ocular outcomes linked with not only lower gestational age and birth weight at delivery but also with events in the intrauterine environment that trigger a preterm birth. The reduced incidence of type 1 or type 2 retinopathy of prematurity in the preterm premature rupture of the membranes group compared with other causes of preterm birth may be related to the perinatal therapies associated with preterm premature rupture of the membranes (such as corticosteroids, antibiotics, maternal–fetal surveillance), which may have an inhibitory effect on the development of retinopathy of prematurity. We suggest that the physiologic events that predispose infants to type 1 or type 2 retinopathy of prematurity begin before delivery.

Amniotic membrane transplants in the pediatric population

PURPOSEnTo investigate the indications for and results of amniotic membrane transplantation (AMT) for the treatment of ocular disease in pediatric patients at a single institution.nnnMETHODSnThe medical records of patients <18xa0years of age who underwent AMT for ocular disease between January 1, 2003, and September 1, 2015, were reviewed retrospectively. Patients were determined to have reached a clinical endpoint if there was resolution of the ocular condition being treated after AMT placement, no additional surgery required for treatment of the ocular condition, and no active disease at most recent follow-up.nnnRESULTSnA total of 48 records were reviewed. Of these, 32 patients (67%) received AMT for treatment of ocular disease related to Stevens Johnson syndrome (SJS), 29 (94%) of whom reached the clinical endpoint. The remaining 16 patients (33%) underwent AMT for indications other than SJS, including difficult-to-treat corneal epithelial defects and ulcers, conjunctival reconstruction, and scarring after strabismus surgery. Of these, 80% reached the clinical endpoint. There were no adverse effects related to AMT in either group.nnnCONCLUSIONSnIn our series, AMT was used successfully and without complications.

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

BACKGROUNDnWINROP (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.nnnMETHODSnThe records of consecutive infants who underwent ROP examinations between 2008 and 2011 were reviewed retrospectively. Infants were classified into categories of alarm (atxa0risk for developing severe ROP) and no alarm (minimal risk for severe ROP).nnnRESULTSnA total of 483 were included. Alarm occurred in 241 neonates (50%), with the median time from birth to alarm of 2xa0weeks. 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 typexa02). 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%.nnnCONCLUSIONSnVery preterm infants (gestational age of ≤27xa0weeks) with relatively high birth weight for gestational age may not be detected by WINROP as high risk for developing severe ROP.

Cytomegalovirus retinitis in pediatric stem cell transplants: report of a recent cluster and the development of a screening protocol.

PURPOSEnThe incidence of cytomegalovirus (CMV) retinitis in the pediatric allogeneic hematopoietic stem cell transplant (HSCT) population is unknown. We report a cluster of 5 pediatric patients with CMV retinitis diagnosed in a 12-month period and compare this to the rate of CMV viremia and retinitis in the 4 years prior. Presented is the ophthalmic screening protocol developed in response to this experience.nnnDESIGNnRetrospective cross-sectional study.nnnMETHODSnA retrospective chart review was performed on patients at Childrens Hospital of Colorado (CHCO) who received allogeneic HSCT between January 2010 and December 2014. Fisher exact test was used to compare the proportion of CMV viremia and CMV retinitis in patients transplanted between January 2010 and December 2013 with those transplanted in 2014.nnnRESULTSnA total of 101 patients underwent allogeneic HSCT from January 2010 to December 2013; 32 (32%) tested positive for CMV viremia. No cases of CMV retinitis were identified. From January 2014 to December 2014, 28 patients underwent allogeneic HSCT; 13 patients (46%) had CMV viremia, not a statistically significant increase (Pxa0= .18). There were 5 cases of CMV retinitis diagnosed in those transplanted in 2014, a statistically significant increase compared with those transplanted in 2010-2013 (Pxa0= .0004). A multidisciplinary team was formed to review the literature and an ophthalmic screening protocol was developed.nnnCONCLUSIONnOur recent cluster of CMV retinitis in pediatric allogeneic HSCT patients may suggest a rise in incidence of CMV retinitis. We propose an ophthalmic screening protocol to diagnose retinitis in pediatric HSCT patients in the early, often asymptomatic stage.