Medication Utilization at School Age for Children Born Preterm.

We explored medication use by children born preterm at 5-8 years of age. Compared with children born at full term, children born preterm had higher medication use that included most therapeutic classifications. Although asthma and chronic lung disease influenced use, prematurity remained an independent risk factor.

Short-Term Neurodevelopmental Outcome in Congenital Diaphragmatic Hernia: The Impact of Extracorporeal Membrane Oxygenation and Timing of Repair.

OBJECTIVES: The purpose of this study was to assess the need and timing of extracorporeal membrane oxygenation in relation to congenital diaphragmatic hernia repair as modifiers of short-term neurodevelopmental outcomes. DESIGN: Retrospective study. SETTING: A specialized tertiary care center. PATIENTS: Between June 2004 and February 2016, a total of 212 congenital diaphragmatic hernia survivors enrolled in our follow-up program. Neurodevelopmental outcome was assessed at a median age of 22 months (range, 5-37) using the Bayley Scales of Infant Development, third edition. Fifty patients (24%) required extracorporeal membrane oxygenation support. Four patients (8%) were repaired prior to cannulation, 25 (50%) were repaired on extracorporeal membrane oxygenation, and 21 (42%) were repaired after decannulation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children with congenital diaphragmatic hernia, who required extracorporeal membrane oxygenation scored on average 4.6 points lower on cognitive composite (p = 0.031) and 9.2 points lower on the motor composite (p < 0.001). Language scores were similar between groups. Mean scores for children with congenital diaphragmatic hernia repaired on extracorporeal membrane oxygenation were significantly lower for cognition (p = 0.021) and motor (p = 0.0005) outcome. Language scores were also lower, but did not reach significance. A total of 40% of children repaired on extracorporeal membrane oxygenation scored below average in all composites, whereas only 9% of the non-extracorporeal membrane oxygenation, 4% of the repaired post-extracorporeal membrane oxygenation, and 25% of the repaired pre-extracorporeal membrane oxygenation patients scored below average across all domains. Only 20% of congenital diaphragmatic hernia survivors repaired on extracorporeal membrane oxygenation support scored within the average range for all composite domains. Duration of extracorporeal membrane oxygenation support was not associated with a higher likelihood of adverse cognitive (p = 0.641), language (p = 0.147), or motor (p = 0.720) outcome. CONCLUSIONS: Need for extracorporeal membrane oxygenation in congenital diaphragmatic hernia survivors is associated with worse neurocognitive and neuromotor outcome. Need for congenital diaphragmatic hernia repair while on extracorporeal membrane oxygenation is associated with deficits in multiple domains. Overall time on extracorporeal membrane oxygenation did not impact neurodevelopmental outcome.

Why do children with severe bronchopulmonary dysplasia not attend neonatal follow-up care? Parental views of barriers.

AIM: To assess in children with severe bronchopulmonary dysplasia at a corrected age of 18-36 months: (i) Neonatal follow-up clinic attendance rates; (ii) Parent-identified reasons for difficulty attending neonatal follow-up. METHODS: Mixed methods study utilising semi-structured phone interviews with parents of infants eligible for follow-up with severe bronchopulmonary dysplasia (defined as gestational age <32 weeks and requiring ≥30% FiO2 and/or >2 L nasal cannula at 36 weeks post-menstrual age) at 18-36 months corrected age. Questions addressed barriers to neonatal follow-up attendance. Enrolment continued to saturation (no new themes emerging). RESULTS: A total of 58 infants (69% male) were enrolled. Infants were 26 ± 2.1 weeks gestational age and birth weight 794 ± 262 g. At 28 ± 5.8 months corrected age, 26% had never attended neonatal follow-up clinic, 16% stopped attending before discharge, 5% were discharged, and 53% were still followed. Longer travel distance from home to follow-up clinic was associated with poorer attendance. Parent-generated items related to neonatal follow-up barriers were coded into four themes: Logistics, Time, Perceptions and Emotional Stress. CONCLUSION: Despite high risk of developmental delay in infants with severe bronchopulmonary dysplasia, neonatal follow-up rates are suboptimal. Careful review of parent-identified barriers could be utilised to develop targeted strategies to improve neonatal follow-up attendance in this high-risk population.

Developmental outcomes of very preterm infants with tracheostomies.

OBJECTIVES: To evaluate the neurodevelopmental outcomes of very preterm (<30 weeks) infants who underwent tracheostomy. STUDY DESIGN: Retrospective cohort study from 16 centers of the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network over 10 years (2001-2011). Infants who survived to at least 36 weeks (N = 8683), including 304 infants with tracheostomies, were studied. Primary outcome was death or neurodevelopmental impairment (NDI; a composite of ≥1 of developmental delay, neurologic impairment, profound hearing loss, severe visual impairment) at a corrected age of 18-22 months. Outcomes were compared using multiple logistic regression. We assessed the impact of timing by comparing outcomes of infants who underwent tracheostomy before and after 120 days of life. RESULTS: Tracheostomies were associated with all neonatal morbidities examined and with most adverse neurodevelopmental outcomes. Death or NDI occurred in 83% of infants with tracheostomies and 40% of those without (OR adjusted for center 7.0, 95% CI 5.2-9.5). After adjustment for potential confounders, odds of death or NDI remained higher (OR 3.3, 95% CI 2.4-4.6), but odds of death alone were lower (OR 0.4, 95% CI 0.3-0.7) among infants with tracheostomies. Death or NDI was lower in infants who received their tracheostomies before, rather than after, 120 days of life (aOR 0.5, 95% CI 0.3-0.9). CONCLUSIONS: Tracheostomy in preterm infants is associated with adverse developmental outcomes and cannot mitigate the significant risk associated with many complications of prematurity. These data may inform counseling about tracheostomy in this vulnerable population.

Progesterone for Neurodevelopment in Fetuses With Congenital Heart Defects: A Randomized Clinical Trial.

Importance: Neurodevelopmental outcomes for children with congenital heart defects (CHD) have improved minimally over the past 20 years. Objectives: To assess the feasibility and tolerability of maternal progesterone therapy as well as the magnitude of the effect on neurodevelopment for fetuses with CHD. Design, Setting, and Participants: This double-blinded individually randomized parallel-group clinical trial of vaginal natural progesterone therapy vs placebo in participants carrying fetuses with CHD was conducted between July 2014 and November 2021 at a quaternary care children's hospital. Participants included maternal-fetal dyads where the fetus had CHD identified before 28 weeks' gestational age and was likely to need surgery with cardiopulmonary bypass in the neonatal period. Exclusion criteria included a major genetic or extracardiac anomaly other than 22q11 deletion syndrome and known contraindication to progesterone. Statistical analysis was performed June 2022 to April 2024. Intervention: Participants were 1:1 block-randomized to vaginal progesterone or placebo by diagnosis: hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and other CHD diagnoses. Treatment was administered twice daily between 28 and up to 39 weeks' gestational age. Main Outcomes and Measures: The primary outcome was the motor score of the Bayley Scales of Infant and Toddler Development-III; secondary outcomes included language and cognitive scales. Exploratory prespecified subgroups included cardiac diagnosis, fetal sex, genetic profile, and maternal fetal environment. Results: The 102 enrolled fetuses primarily had HLHS (n = 52 [50.9%]) and TGA (n = 38 [37.3%]), were more frequently male (n = 67 [65.7%]), and without genetic anomalies (n = 61 [59.8%]). The mean motor score differed by 2.5 units (90% CI, -1.9 to 6.9 units; P = .34) for progesterone compared with placebo, a value not statistically different from 0. Exploratory subgroup analyses suggested treatment heterogeneity for the motor score for cardiac diagnosis (P for interaction = .03) and fetal sex (P for interaction = .04), but not genetic profile (P for interaction = .16) or maternal-fetal environment (P for interaction = .70). Conclusions and Relevance: In this randomized clinical trial of maternal progesterone therapy, the overall effect was not statistically different from 0. Subgroup analyses suggest heterogeneity of the response to progesterone among CHD diagnosis and fetal sex. Trial Registration: ClinicalTrials.gov Identifier: NCT02133573.

Early neurodevelopmental outcome of infants with high-risk fetal lung lesions.

OBJECTIVE: To evaluate the neurodevelopmental outcome of infants with high-risk fetal lung lesions defined as (1) requiring fetal intervention and/or ex utero intrapartum therapy (EXIT), or (2) acute respiratory decompensation postnatally necessitating emergent resection within 48 h of life. METHODS: We reviewed the medical records of 13 consecutive patients with high-risk fetal lung lesions who were enrolled in our prospective interdisciplinary follow-up program. Neurodevelopmental status was evaluated using the Bayley Scales of Infant Development-III (children ≤3 years, n = 12), or the Wechsler Preschool and Primary Scale of Intelligence-III (children ≥4 years, n = 1). RESULTS: Eight children (62%) underwent prenatal intervention (EXIT, n = 6; fetal resection, n = 1; intrauterine shunt placement, n = 1), and 5 (38%) required emergent resection postnatally. Median age at evaluation was 25 months (range: 5-80). Average scores for cognitive development were found in all children assessed under 3 years of age. The one child who was tested for cognitive ability at 6 years of age scored in the borderline range of intellectual functioning. For language outcome, 15% scored above average, 54% scored within the average range, and 31% had mild deficits. Overall, 77% scored within the average range for neuromotor outcome, while 23% scored within the mildly delayed range. None of the children had severe delays. Cognitive, language, and psychomotor scores were similar between both groups. Hypotonicity was found in 23%. Autism was suspected in one child who underwent an EXIT procedure and was postnatally diagnosed with mosaic trisomy 18. CONCLUSION: The majority of children with high-risk fetal lung lesions have age-appropriate neurodevelopmental scores.

Health literacy of parents of very preterm infants at NICU admission and discharge: a prospective cohort study.

OBJECTIVE: To determine the prevalence of limited health literacy in parents of infants born ≤32 and 0/7 weeks and if health literacy changes during hospitalization. STUDY DESIGN: Multi-site, prospective cohort study measuring health literacy using the Parent Health Literacy Activities Test, which estimates caregivers' ability to complete tasks such as reading prescription labels and preparing bottles. Data were analyzed using parametric and nonparametric comparison tests and multivariable regression to control for confounders. RESULT: Of the 137 participants, 31% missed ≥3 questions of 8. Scores were not associated with admission characteristics or NICU complications. Lower scores were associated with lower nurses' (rho 0.20, p = 0.04) but not parents' (rho -0.12, p = 0.22) ratings of discharge readiness. Scores improved slightly from admission to discharge (p = 0.049). CONCLUSION: Many parents have difficulty answering questions related to basic infant care tasks. NICUs should ensure that communication and discharge planning are mindful of health literacy.

Autism spectrum disorder and neurodevelopmental delays in children with giant omphalocele.

OBJECTIVE: To determine the prevalence and identify risk factors of autism spectrum disorders (ASDs) and neurodevelopmental delays in giant omphalocele (GO) survivors. MATERIALS AND METHODS: The study cohort consists of 47 GO survivors enrolled in our follow-up program between 07/2004 and 12/2015. All patients underwent assessments at 2 years of age or older. Outcomes were assessed by either the Bayley Scales of Infant Development II (prior 2006) or III (after 2006), or the Wechsler Preschool and Primary Scale of Intelligence (children older than 4 years). ASD diagnosis was made based on the Diagnostic and Statistical Manual of Mental Disorders IV (prior to 2014) or 5 criteria. RESULTS: The prevalence of ASD in GO children is 16 times higher than the general population (P = 0.0002). ASD patients were more likely to be diagnosed with neurodevelopmental and neurofunctional delays, language disorders, and genetic abnormalities (P < 0.01). While 53.2% of GO children scored within the average range for all developmental domains, 19.1% scored within the mildly delayed and 27.7% in the severe delayed range in at least one domain. Prolonged respiratory support, pulmonary hypertension, gastroesophageal reflux disease, feeding problems, prolonged hospitalization, abnormal BAER hearing screen, presence of delayed motor coordination, and hypotonicity were associated with delayed scores (P < 0.05). CONCLUSIONS: There is a significant rate of ASD in GO survivors. Neurodevelopmental delays, language delays, and genetic abnormalities were strongly associated with ASD. Neurological impairments were present in nearly half of GO children. Surrogate markers of disease severity were associated with below average neurodevelopmental scores. Level of evidence Level IV.

Rate and Risk Factors Associated with Autism Spectrum Disorder in Congenital Diaphragmatic Hernia.

To determine the rate and predictors of autism spectrum disorder (ASD) in congenital diaphragmatic hernia (CDH). Between 06/2004 and 09/2015 a total of 110 CDH survivors underwent neurodevelopmental (ND) testing and screening for ASD, followed by a full autism diagnostic evaluation if indicated at our institution. We found a 9 time higher rate of ASD in CDH children compared to the general population (P = 0.0002). Multiple patient-related and clinical variables risk factors of ASD were identified by univariate analysis. However, only short-term and long-term neurodevelopmental delays were strongly associated with ASD in CDH by multivariate comparisons. There is a striking prevalence of ASD in CDH survivors and our findings suggest that all CDH children should be regularly screened for ASD.

Reduced oxygen concentration for the resuscitation of infants with congenital diaphragmatic hernia.

OBJECTIVE: To evaluate whether infants with congenital diaphragmatic hernia (CDH) can be safely resuscitated with a reduced starting fraction of inspired oxygen (FiO2) of 0.5. STUDY DESIGN: A retrospective cohort study comparing 68 patients resuscitated with starting FiO2 0.5 to 45 historical controls resuscitated with starting FiO2 1.0. RESULTS: Reduced starting FiO2 had no adverse effect upon survival, duration of intubation, need for ECMO, duration of ECMO, or time to surgery. Furthermore, it produced no increase in complications, adverse neurological events, or neurodevelopmental delay. The need to subsequently increase FiO2 to 1.0 was associated with female sex, lower gestational age, liver up, lower lung volume-head circumference ratio, decreased survival, a higher incidence of ECMO, longer time to surgery, periventricular leukomalacia, and lower neurodevelopmental motor scores. CONCLUSION: Starting FiO2 0.5 may be safe for the resuscitation of CDH infants. The need to increase FiO2 to 1.0 during resuscitation is associated with worse outcomes.

Short-Term Neurodevelopmental Outcome in Children Born With High-Risk Congenital Lung Lesions.

BACKGROUND: This study sought to evaluate neurodevelopmental outcome in survivors of high-risk congenital lung lesions (CLLs) who underwent prenatal intervention or postnatal surgery within the first month of life. METHODS: Forty-five high-risk CLL survivors underwent assessment using the Bayley Scales of Infant Development, 3rd Edition between July 2004 and December 2016. Scores were grouped as average, at-risk, and delayed based on SD intervals. Correlations between outcome and risk factors were analyzed by Fisher's exact test or two-sided t test as appropriate, with significant p values <0.05. RESULTS: Open prenatal intervention was required in 13 (28.9%) children (fetal surgical resection, n = 4 , ex utero intrapartum treatment, n = 9), whereas 32 (71.1%) children had respiratory distress postnatally and required resection within the first month of life. Mean age at follow-up was 19.3 ± 10.3 months. Mean composite scores were within the expected average range. A total of 62.2% scored within the average range for all domains. At-risk scores were found in 26.7% of children in at least one domain, and 11.1% had delays in at least one domain. Neurodevelopmental outcome was similar between treatment groups. Prolonged ventilator support and neonatal intensive care unit stay, need for supplemental oxygen at day of life 30, gastroesophageal reflux disease, and delayed enteral feeding were associated with neurologic delays (all p < 0.05). CONCLUSIONS: Neurodevelopmental scores for high-risk CLL survivors in infancy and toddlerhood are age appropriate. Neither fetal intervention nor the need for postnatal resection within the first month of life increases the risk of delays. Surrogate markers of a complicated neonatal course are predictive of adverse outcome.

Neurodevelopmental outcomes at 5years of age in congenital diaphragmatic hernia.

OBJECTIVE: To evaluate neurodevelopmental sequelae in congenital diaphragmatic hernia (CDH) children at 5years of age. MATERIALS AND METHODS: The study cohort of 35 CDH patients was enrolled in our follow-up program between 06/2004 and 09/2014. The neurodevelopmental outcomes assessed at a median of 5years (range, 4-6) included cognition (Wechsler Preschool and Primary Scale of Intelligence [WPPSI], n=35), Visual-Motor-Integration (n=35), academic achievement (Woodcock-Johnson Tests of Achievement, n=25), and behavior problems (Child Behavior Check List [CBCL], n=26). Scores were grouped as average, borderline, or extremely low by SD intervals. RESULTS: Although mean Full (93.9±19.4), Verbal (93.4±18.4), and Performance (95.2±20.9) IQ were within the expected range, significantly more CDH children had borderline (17%) and extremely low (17%) scores in at least one domain compared to normative cohorts (P<0.02). The Visual-Motor-Integration score was below population average (P<0.001). Academic achievement scores were similar to expected means for those children who were able to complete testing. CBCL scores for the emotionally reactive (23%) and pervasive developmental problems scales (27%) were more likely to be abnormal compared to normal population scores (P=0.02 and P=0.0003, respectively). Autism was diagnosed in 11%, which is significantly higher than the general population (P<0.01). Univariate analysis suggests that prolonged NICU stay, prolonged intubation, tracheostomy placement, pulmonary hypertension, autism, hearing impairment, and developmental delays identified during infancy are associated with worse cognitive outcomes (P<0.05). CONCLUSION: The majority of CDH children have neurodevelopmental outcomes within the average range at 5years of age. However, rates of borderline and extremely low IQ scores are significantly higher than in the general population. CDH survivors are also at increased risk for developing symptoms of emotionally reactive and pervasive developmental problems. Risk of autism is significantly elevated. Disease severity and early neurological dysfunction appear to be predictive of longer-term impairments.

Growth trajectory and neurodevelopmental outcome in infants with congenital diaphragmatic hernia.

PURPOSE: The purpose of this study was to evaluate the impact of impaired growth on short-term neurodevelopmental (ND) outcomes in CDH survivors. METHODS: Between 9/2005-12/2014, 84 of 215 (39%) CDH survivors underwent ND assessment at 12months of age using the BSID-III. RESULTS: Mean cognitive, language, and motor scores were 92.6±13.5, 87.1±11.6, and 87.0±14.4, respectively (normal 100±15). 51% of patients scored 1 SD below the population mean in at least one domain, and 13% scored 2 SD below the population mean. Group-based trajectory analysis identified two trajectory groups ('high' and 'low') for weight, length, and head circumference (HC) z-scores. (Fig. 1) 74% of the subjects were assigned to the 'high' trajectory group for weight, 77% to the 'high' height group, and 87% to the 'high' HC group, respectively. In multivariate analysis, longer NICU stay (p<0.01) was associated with lower cognitive scores. Motor scores were 11 points higher in the 'high' HC group compared to the 'low' HC group (p=0.05). Motor scores were lower in patients with longer NICU length of stay (p<0.001). CONCLUSIONS: At 1 year, half of CDH survivors had a mild delay in at least one developmental domain. Low HC trajectory was associated with worse neurodevelopmental outcomes. TYPE OF STUDY: Prognosis Study/Retrospective Study. LEVEL OF EVIDENCE: Level II.

Use of Gastroesophageal Reflux Medications in Premature Infants After NICU Discharge.

OBJECTIVES: To describe the epidemiology and management of gastroesophageal reflux (GER) medications started in the first year of life for premature infants. METHODS: Retrospective review of a cohort of infants ≤35 weeks' gestation presenting for care by 168 days of age to a 30-site network between 2005 and 2009 (n = 2217) and followed to 3 years of age. Medication frequency, types, and duration of use were assessed. Logistic regression identified factors associated with treatment. RESULTS: Thirty-seven percent (812) were prescribed GER medications with 77% begun after NICU discharge. Ninety percent (727) received histamine-2 receptor antagonists, 33% (269) proton pump inhibitors, 22% (182) prokinetics; 40% (325) received >1 medication. Outpatient medication was initiated at 95 ± 69 days of life for total of 294 ± 249 days (interquartile ratio: 117-359). Feeding issues (adjusted odds ratio [aOR] 2.05, 95% confidence interval [CI]: 1.24-3.39) were associated with outpatient initiation. Forty-three percent (322) of infants started before 6 months were still on at 1 year of age associated with gestational age <32 weeks (aOR 1.76, 95% CI: 1.16-2.67), chronic lung disease (aOR 2.59, 95% CI: 1.29-5.22), and reactive airways disease (aOR 1.67, 95% CI: 1.05-2.65). CONCLUSIONS: Of the 37% of the cohort on GER medications, 77% were started after NICU discharge with prolonged use of medications. Feeding difficulties were associated with starting medication and markers of chronic lung disease with continuation of treatment. With uncertain evidence of efficacy, use of these medications in a high-risk population should be carefully evaluated.

Younger gestational age is associated with increased risk of adverse neurodevelopmental outcome during infancy in congenital diaphragmatic hernia.

BACKGROUND: The purpose of the study was to investigate the impact of gestational age (GA) on short-term neurodevelopmental (ND) outcomes in congenital diaphragmatic hernia survivors. MATERIALS: Between 6/2004 and 2/2013, 135 consecutive CDH patients underwent ND assessment using the Bayley Scales of Infant Development-III at a median follow-up age of 13months (range, 5-36). ND delay was defined by a score of ≤85 in any of the composite scales. Severe impairment was defined as a score of ≤69 in at least one domain. The effect of GA was evaluated as continuous and categorical variables. GA at delivery was grouped into full term (39-41weeks), near term (37-38), late preterm (34-36), and preterm (24-33). RESULTS: Median GA at delivery was 38weeks (range, 24-41). Fifty (37%) patients were delivered full term, 59 (44%) near term, 16 (12%) late preterm, and 10 (7%) preterm. CDH children born before 39weeks' gestation were more likely to score below average (P=0.005) with corrected age for at least one composite score compared to full term peers. Cognitive (P=0.06) and language (P=0.08) scores tended to be lower in the near-term and late-preterm group compared to full-term CDH infants. Patients born near term and late preterm had significantly lower motor composite and fine motor scores compared to full-term children (P=0.009 and P<0.01, respectively). Preterm children scored the lowest in all composite scales (P<0.05). CONCLUSIONS: Compared to term infants, not only preterm but also late preterm and near-term CDH children carry an increased risk of ND delays. Motor performance appears most susceptible to earlier delivery.

Preterm Infant Attendance at Health Supervision Visits.

OBJECTIVES: To assess the adherence of premature infants with the American Academy of Pediatrics health supervision visit schedule, factors affecting adherence, and the association of adherence with preventive care. METHODS: Retrospective cohort of all infants ≤35 weeks' gestation, born 2005 to 2009, receiving care at a 30-site primary care network for at least 24 months (n = 1854). Adherence was defined as having a health supervision visit within each expected time period during the first 18 months of life. Logistic regression identified sociodemographic and medical factors associated with nonadherence and risk-adjusted association between nonadherence and outcomes. RESULTS: Only 43% received all expected health supervision visits. Those with Medicaid insurance (adjusted odds ratio [AOR] 0.46, 95% confidence interval [CI] 0.35-0.60), a visit without insurance (AOR 0.46, 95% CI 0.32-0.67), chronic illness (AOR 0.7, 95% CI 0.51-0.97), and black race (AOR 0.7, 95% CI 0.50-0.98) were less adherent, whereas provider continuity of care (AOR 2.89, 95% CI 1.92-4.37) and lower birth weight (AOR 1.67, 95% CI 1.02-2.73) increased adherence. Infants <100% adherent were less likely to be up to date with immunizations and receive recommended preventive care. In nearly half of missed visit windows, no health supervision visit was scheduled. CONCLUSIONS: Fewer than half of premature infants were fully adherent with the preventive health schedule with associated gaps in health monitoring and immunization delays. These data suggest the importance of health supervision visits and the need to explore scheduling facilitators for those at risk for nonadherence.

Neurodevelopmental outcome at one year of age in congenital diaphragmatic hernia infants not treated with extracorporeal membrane oxygenation.

BACKGROUND: We evaluated the neurodevelopmental (ND) outcome at one year of age for congenital diaphragmatic hernia (CDH) children who have not undergone extracorporeal membrane oxygenation (ECMO) treatment during the neonatal period. MATERIAL AND METHODS: Between 01/2005 and 06/2012, 63 consecutive CDH patients underwent ND assessment using the BSID-III at a median age of 12 months. ND delay was defined by a score of ≤ 85 in any of the composite scales. Severe impairment was defined as a score of ≤ 69 in at least one domain. RESULTS: Mean ± SD cognitive, language, and motor functions were 94 ± 14, 86 ± 14, 90 ± 15, respectively (normal 100 ± 15, P<0.01 for each). Forty-three-percent scored within the average range for all scales. Forty-four-percent had mild, and 13% had severe delays in at least one domain. Prolonged NICU stay, intubation and O2 requirement, fundoplication, abnormal BAERs, and tracheostomy were associated with lower scores in all domains. Right-sided CDH, male gender, lower 5 min APGAR, pulmonary hypertension, and delayed start of enteral feeding were predictive of lower cognitive and/or language scores. CONCLUSION: At one year of age, a high percentage of CDH children whose illness did not necessitate ECMO have below normal ND scores. Modifiable and non-modifiable factors are significant determinants of adverse outcomes.

Patient characteristics are important determinants of neurodevelopmental outcome during infancy in giant omphalocele.

OBJECTIVE: To examine patient-specific factors as potential predictors of neurodevelopmental (ND) outcome in children with giant omphalocele (GO). MATERIALS: Between 06/2005 and 07/2012, 31 consecutive GO survivors underwent ND assessment using the BSID-III at a median of 24months (range 6-35). ND delay was defined by a score of ≤84 in any composite score. Severe impairments were defined as a score of ≤69 in at least one domain. Correlations between ND outcome and patient-specific factors were analyzed by one-way ANOVA, chi-square, or logistic regression as appropriate. RESULTS: The mean cognitive score (86.8±16.8) was in the low average range. Mean language (83.2±21.1) and motor (81.5±16.2) scores were below average. Forty-six-percent scored within the average range for all scales. Mild deficits were found in 19%, and 35% had severe delays in at least one domain. Hypotonicity was present in 55%. Autism was suspected/confirmed in 13%. Predictors of lower ND scores were prolonged ventilator support (P<0.01), high-frequency oscillatory ventilation (P<0.01), tracheostomy placement (P<0.001), O2 supplementation at day of life 30 (P<0.02), pulmonary hypertension (P<0.02), delayed enteral feeding (P=0.01), need for feeding tube (P<0.001), GERD (P=0.05), abnormal BAER hearing screen (P<0.006), prolonged hospitalization (P=0.01), and failure to thrive (P=0.001). Autism was associated with delays in cognitive and language outcomes (P<0.03). Delayed staged closure (P=0.007), older age at final repair (P=0.03), and hypotonicity (P=0.02) were associated with motor dysfunction. CONCLUSIONS: Neurological impairments were present in more than half of GO survivors. Disease severity was associated with ND dysfunction. Autism and hypotonicity were often co-morbidities with ND delays and poor motor function.

Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery.

BACKGROUND: There has been increasing recognition of adverse neurodevelopmental sequelae in some children after repair of congenital heart defects. Even among children with the same cardiac defect, significant interindividual variation exists in developmental outcome. Polymorphisms of apolipoprotein E have been identified as a risk factor for worse neurologic recovery after central nervous system injury. METHODS: A single-institution prospective study of patients

"Benign" extra-axial fluid in survivors of neonatal intensive care.

OBJECTIVES: To identify the prevalence of "benign" extra-axial fluid (BEAF), the risk factors associated with this condition, and the natural history in "graduates" of neonatal intensive care. DESIGN: Cross-sectional study. SETTING: Neonatal follow-up clinic at a tertiary care center. PATIENTS: Seventy-seven infants with a head circumference greater than the 95th percentile by growth percentiles from either the National Center for Health Statistics or the Infant Health and Development Program growth percentile graphs who attended the Neonatal Follow-up Program at The Children's Hospital of Philadelphia between January 1, 1998, and December 31, 2001. MAIN OUTCOME MEASURES: Bronchopulmonary dysplasia, extracorporeal membrane oxygenation; development at 18 to 24 months. RESULTS: There were 26 infants (34%) in the BEAF group, 43 (56%) in the control group without extra-axial fluid, and 8 (10%) in the hydrocephalus group. Compared with the control group, infants with BEAF were more likely to have bronchopulmonary dysplasia or to require use of extracorporeal membrane oxygenation in the immediate neonatal period (risk ratio, 6.1; 95% confidence interval, 1.5-29.8). Measurements of head circumference in the BEAF group showed rapid growth between 3 and 12 months, followed by growth greater than and parallel to the 95th percentile. Head circumference measurements in the control group showed continued growth along the 95th percentile for age. Infants with BEAF were more likely than controls to develop cerebral palsy (risk ratio, 9.9; 95% confidence interval, 1.3-77.9) and to have evidence of developmental delay at adjusted ages 12 and 18 to 24 months. CONCLUSION: The presence of extra-axial fluid in macrocephalic survivors of neonatal intensive care is associated with an increased risk of developmental delay and cerebral palsy compared with control macrocephalic survivors.