Patricia Riley

Severe bronchopulmonary dysplasia increases risk for later neurological and motor sequelae in preterm survivors.

Preterm children who develop severe chronic lung disease may be developmentally compromised by exposure to hypoxic episodes. This study aims to determine if children with severe bronchopulmonary dysplasia (BPD) who required home oxygen therapy were at greater risk for neurological and motor deficits at school age than preterm peers without BPD. This study evaluated 27 subjects with BPD and 27 preterm control infants matched for gestational age, birth weight, sex, and year of birth at a mean age of 9.9 years (2.0 SD) using standardized neuromotor outcome measures. Pair‐matched comparisons and regression analyses were used to determine if subjects with BPD were at increased risk for neuromotor sequelae. Neurological abnormalities, including subtle neurological signs, cerebral palsy, microcephaly, and behavioral difficulties were highly prevalent in the BPD group (71% compared with 19% in control group, P<0.005). Over half the BPD cohort had difficulties in gross and/or fine motor skills. There were significant differences in postural stability between groups. Duration of hospitalization and home oxygen treatment, and decreased lung function at school age, markers of severity of illness, correlated with motor outcomes. The findings underline the importance of preventing the cardiorespiratory complications associated with chronic lung disease to minimize disability in preterm children. For children with severe BPD, better recognition and subsequent remediation of neuromotor impairments that manifest at school age may help maximize their functional potential.

PROGNOSTIC SIGNIFICANCE OF THE AUDITORY BRAINSTEM EVOKED RESPONSE HIGH‐RISK NEONATES

The prognostic significance of the auditory brainstem evoked response (ABR) was examined in this prospective study of neonates at risk for neurodevelopmental sequelae. ABR testing was performed in the neonatal period (37 to 45 weeks conceptional age) and at two and/or six months corrected age. 34 high‐risk newborns and 14 controls were followed to one year of age, when they received neurological and developmental assessments. Increased I to III and I to V interwave latencies predicted gross motor delay at one year, with a positive predictive power of 83 per cent and a specificity of 94.7 per cent. Increased brainstem conduction, dispersal of waves III to V and V/I amplitude ratio abnormalities predicted abnormal neurological findings at one year, with positive predictive values of 100, 100 and 80 per cent, respectively. A standard clinical examination of the newborn, performed on the at‐risk and control infants at 40 weeks conceptional age, was not found to be strongly predictive of neurodevelopmental deficits at one year.

Sequelae of caffeine treatment in preterm infants with apnea.

Growth, neurologic, and ophthalmologic assessments were done in 21 low-birth-weight infants given caffeine for neonatal apnea and in 21 matched control infants. Caffeine significantly decreased the need for and the duration of mechanical ventilation. No difference in growth and development was noted between the control and caffeine-treated infants. A high incidence of cicatricial retrolental fibroplasia was observed in both control (10/21) and caffeine-treated infants (7/18).

The neonatal presentation of Prader-Willi syndrome revisited

We describe 6 newborns evaluated for hypotonia, later diagnosed with Prader-Willi syndrome despite the absence of the classical neonatal features of this syndrome. Specific genetic testing for Prader-Willi syndrome should be considered for all neonates with undiagnosed central hypotonia even in the absence of the other major features of this syndrome.

Influence of gestational age, birth weight, and asphyxia on neonatal neurobehavioral performance

The objectives of this study were to determine the influence of birth weight, gestational age, and asphyxia on neurobehavioral performance at 40 weeks conceptional age, and to demonstrate that this performance may differ in preterm high-risk infants when using low-risk preterm rather than healthy term infant norms. A cross-sectional comparative design was used to compare neurologic behavior in healthy and high-risk newborns at 40 weeks conceptional age using the Einstein Neonatal Neurobehavioral Assessment Scale. Seventy-four high-risk newborns were selected from a consecutive sample of asphyxiated, very-low-birth-weight, and small-for-gestational-age neonates. Controls did not fulfill any of the high-risk criteria and had no pre- or perinatal complications. There were 37 healthy term and 17 low-risk preterm controls. Statistically significant differences occurred between high-risk newborns and term controls for the total score (P < .001) as well as for most individual items (P < .05). Analysis of high-risk subgroups revealed that small-for-gestational-age and term asphyxiated newborns had the most abnormal responses. When comparing test performance between preterm high-risk and term controls, the majority of test items achieved significance (P < .05); however, when compared to low-risk preterm controls, fewer items were abnormal, and the probability level on abnormal items was less significant than expected. This disparity in interpretation of neurobehavioral status in preterm high-risk neonates using a preterm versus term reference sample warrants further investigation. Appropriate normative standards for preterm infants require definition because it may increase the utility of the neonatal neurologic examination and its prognostic value.

Hearing loss in congenital diaphragmatic hernia (CDH) survivors: is it as prevalent as we think?

PURPOSE The incidence of sensorineural hearing loss (SNHL;>20 dB loss) in CDH survivors is debated. We evaluated long-term audiological outcomes at a single tertiary care center with ECMO capability and an established neonatal follow-up program. METHODS With REB approval, records of CDH survivors from 2000 to 2010 were retrospectively analyzed. Demographic, postnatal, and audiometric information was gathered. All underwent auditory brainstem response (ABR) or otoacoustic emissions screening before discharge and complete audiological surveillance. Thirty-three patients were evaluated to age 4+ years with others continuing follow-up. RESULTS Forty-three patient records were reviewed with 1 excluded (transferred to another institution). Median GA and BW were 39 weeks (35-41) and 3.1 kg (2-4), respectively. Median ventilation days were 10 (2-189) with 34 infants ventilated 5+ days. Sixteen (36%) received HFOV, 21 (49%) iNO, and 5 (12%) ECMO. The median time to CDH repair was 3 days (1-23), and 11 (26%) required patch repair. Nine infants (21%) received diuretics and oxygen after discharge. Audiological surveillance identified only one patient with SNHL (received HFO, iNO, and patch repair). CONCLUSION Neonatal screening identifies CDH survivors at risk for hearing difficulties but must be followed with comprehensive testing until school age. The incidence of SNHL may be less than previously reported in this population.

Participation and enjoyment of leisure activities in adolescents born at ≤ 29 week gestation

BACKGROUND AND OBJECTIVES Motor, cognitive, social and behavioral problems have been found to persist in adolescents born extremely preterm. Leisure participation has been associated with health benefits; however, few studies have explored leisure participation in this population. The aim of this study was to describe leisure participation in adolescents born at ≤29week gestation. Secondary aims were to identify potential differences in participation related to sex, age, motor competence, and cognitive ability, and between adolescents born preterm and their siblings born at term. METHODS This cross-sectional study included 128 adolescents (mean age: 16.0years; 67 females) with a mean gestational age of 26.5weeks. All participants, as well as 22 siblings born at term, completed the Childrens Assessment of Participation and Enjoyment. RESULTS Participation levels were highest in social and recreational activities, and lowest in active-physical and skill-based activities. Boys participated in more active-physical activities (p=0.01) and more often (p<0.001) than girls, whereas girls had higher participation levels in social and self-improvement activities (p<0.05) than boys. Older adolescents engaged in more social activities (p=0.01) and more frequently (p=0.002) than younger adolescents. Overall, participants with poor motor and cognitive skills had lower participation levels in active-physical and social activities. Adolescents born preterm participated in fewer recreational activities compared to term-born siblings (p=0.013). CONCLUSIONS Engagement in active-physical and skill-based leisure activities needs to be promoted in boys, and especially in girls with a history of prematurity. Activities should be adapted to sex and individual skill level in order to promote participation.

Child and environmental factors associated with leisure participation in adolescents born extremely preterm.

BACKGROUND Developmental impairments persist among adolescents born extremely preterm, and these individuals are at an increased risk for chronic disease later in life. Participating in active and positive leisure activities may act as a buffer against negative outcomes, but involvement in active-physical and skill-based activities is low in youth born preterm. AIMS To explore the child and environmental determinants of leisure participation among adolescents born extremely preterm. STUDY DESIGN Cross-sectional study. SUBJECTS Participants were recruited from the hospitals Neonatal Follow-Up Program and included 128 adolescents born preterm (mean gestational age: 26.5 weeks). OUTCOME MEASURES Leisure participation was assessed using the Childrens Assessment of Participation and Enjoyment. Potential determinants were assessed using standardized tests and questionnaires. Selected factors were entered into five separate multivariable regression models. RESULTS Child and environmental factors contributed between 21% (skill-based) and 52% (active physical) of the adjusted variance for participation intensity. Lower gestational age was associated with greater participation in recreational activities. Male sex, higher maternal education and better motor competence were associated with involvement in active-physical activities. Being older and feeling socially accepted were associated with participation in social activities. Families oriented to hobbies and higher maternal education were associated with participation in skill-based activities. Preference was the strongest determinant of participation in all five leisure activities. CONCLUSIONS Activities should be adapted to individual skill level, include family and peers, foster social acceptance and be driven by the adolescents preferences. Although certain factors cannot be modified, they can be used to identify adolescents at risk for low participation.