Characterizing the Subcortical Structures in Youth with Congenital Heart Disease.

BACKGROUND AND PURPOSE: Congenital heart disease is a leading cause of neurocognitive impairment. Many subcortical structures are known to play a crucial role in higher-order cognitive processing. However, comprehensive anatomic characterization of these structures is currently lacking in the congenital heart disease population. Therefore, this study aimed to compare the morphometry and volume of the globus pallidus, striatum, and thalamus between youth born with congenital heart disease and healthy peers. MATERIALS AND METHODS: We recruited youth between 16 and 24 years of age born with congenital heart disease who underwent cardiopulmonary bypass surgery before 2 years of age (n = 48) and healthy controls of the same age (n = 48). All participants underwent a brain MR imaging to acquire high-resolution 3D T1-weighted images. RESULTS: Smaller surface area and inward bilateral displacement across the lateral surfaces of the globus pallidus were concentrated anteriorly in the congenital heart disease group compared with controls (q < 0.15). On the lateral surfaces of bilateral thalami, we found regions of both larger and smaller surface areas, as well as inward and outward displacement in the congenital heart disease group compared with controls (q < 0.15). We did not find any morphometric differences between groups for the striatum. For the volumetric analyses, only the right globus pallidus showed a significant volume reduction (q < 0.05) in the congenital heart disease group compared with controls. CONCLUSIONS: This study reports morphometric alterations in youth with congenital heart disease in the absence of volume reductions, suggesting that volume alone is not sufficient to detect and explain subtle neuroanatomic differences in this clinical population.

Rehabilitation service utilization in children and youth with cerebral palsy.

AIM: To describe the pattern of use of rehabilitation services in children and adolescents with cerebral palsy (CP), and to identify factors associated with use. METHODS: In this study, parents of 91 school-age children and 167 adolescents with CP completed a questionnaire regarding educational and rehabilitation resources received within the last 6 months. Rehabilitation services included occupational therapy (OT), physical therapy (PT), speech language pathology (SLP), psychology and special education. Demographic characteristics were documented and developmental and functional status was assessed. Relationships between service utilization and sociodemographic factors, functioning and school setting were determined. RESULTS: Over half of children (53.2%) and adolescents (57.5%) were in regular schools; however, 41% of these required special education resources. The remainder (42.5-46.8%) was in special schools. The majority of children (84.6%) were receiving at least one rehabilitation service although this decreased (68.1%) in adolescence. PT and OT were most common and services were provided predominantly in the school setting. Services were primarily weekly direct interventions at school age, with weekly interventions or consultations most common for adolescents. Younger age was associated with service receipt at school age only. Children with greater motor limitations, lower IQ and greater activity limitations were more likely to receive OT, PT, SLP or special education. Children in segregated schools were significantly more likely to receive rehabilitation services, when compared with children in regular schools. CONCLUSIONS: The majority of children and youth received one or more services. Individuals with greater motor or cognitive challenges were more likely to receive a range of school-based services from rehabilitation specialists. When compared with children of school age, adolescents were less likely to receive services and when provided, services were more likely to be consultative. Services may need to be more optimally organized through childhood to enhance benefits to children with CP across activity limitation profiles.

Respect for autonomy in the healthcare context: observations from a qualitative study of young adults with cerebral palsy.

BACKGROUND: Respect for patient autonomy is a cornerstone of contemporary medical ethics and clinical practice. In its different shapes and forms (e.g. being informed, being engaged in discussions and decisions about medical care and being supported in developing healthcare preferences and choices), patient autonomy has been fostered by both paediatric and adult professional societies. The transition from paediatric to adult care creates a complex situation where autonomy for medical decisions shifts to the developing adolescent. More specific challenges to respect for autonomy may be experienced by young adults with cerebral palsy in the transition period where, for example, language and motor impairments may affect communication skills and this may be conflated with cognitive disability. AIM: To characterize perspectives towards autonomy in the healthcare context for young adults with cerebral palsy. METHOD: We carried out semi-structured interviews with 14 young adults (aged 18-25) with cerebral palsy. The audiotaped interviews were transcribed verbatim and analysed using a conventional thematic qualitative content analysis. RESULTS: Participants displayed a range of attitudes towards autonomy, suggesting that the value of autonomy is considered in light of competing values and of context. Testimonials from participants demonstrated that both contextual (e.g. ill-adapted health care, lack of specialized public transport) and relational (e.g. attitudes towards parental involvement in decision making) factors negatively or positively impact autonomy. CONCLUSION: We observed that there were four key elements interwoven in participants' characterization of autonomy: the coupling of decisional and physical autonomy, the influences of family and society on autonomy, the influence of healthcare professionals on autonomy and the need for preparation for autonomy.

Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society.

OBJECTIVE: To make evidence-based recommendations concerning the evaluation of the child with a nonprogressive global developmental delay. METHODS: Relevant literature was reviewed, abstracted, and classified. Recommendations were based on a four-tiered scheme of evidence classification. RESULTS: Global developmental delay is common and affects 1% to 3% of children. Given yields of about 1%, routine metabolic screening is not indicated in the initial evaluation of a child with global developmental delay. Because of the higher yield (3.5% to 10%), even in the absence of dysmorphic features or features suggestive of a specific syndrome, routine cytogenetic studies and molecular testing for the fragile X mutation are recommended. The diagnosis of Rett syndrome should be considered in girls with unexplained moderate to severe developmental delay. Additional genetic studies (e.g., subtelomeric chromosomal rearrangements) may also be considered in selected children. Evaluation of serum lead levels should be restricted to those children with identifiable risk factors for excessive lead exposure. Thyroid studies need not be undertaken (unless clinically indicated) if the child underwent newborn screening. An EEG is not recommended as part of the initial evaluation unless there are historical features suggestive of epilepsy or a specific epileptic syndrome. Routine neuroimaging, with MRI preferred to CT, is recommended particularly if abnormalities are found on physical examination. Because of the increased incidence of visual and auditory impairments, children with global developmental delay may undergo appropriate visual and audiometric assessment at the time of diagnosis. CONCLUSIONS: A specific etiology can be determined in the majority of children with global developmental delay. Certain routine screening tests are indicated and depending on history and examination findings, additional specific testing may be performed.

Early rehabilitation service utilization patterns in young children with developmental delays.

OBJECTIVE: There is a growing demand for interdisciplinary rehabilitation services for children with developmental disabilities, however, information is lacking on service utilization patterns. This study describes the type, frequency and location of initial rehabilitation services provided to young children recently diagnosed with a developmental delay. METHODS: A telephone survey was conducted on a consecutive cohort of children referred to subspecialists for the first investigation of their delay. RESULTS: Out of 129 respondents, only 30 (23%) did not receive any rehabilitation services within the first 6 months following medical evaluation, and 61 (47%) received two or more services. Services were provided most frequently in the hospital setting, especially for occupational or physical therapy (73-80%). Services were less often rendered in a rehabilitation centre (5-13%) or in the community (< 10%). Services were provided privately for a subset, particularly for speech language pathology and psychology (34% and 30% respectively). Interventions were typically given weekly or bi-weekly. Children receiving physical therapy were significantly younger whereas children referred to speech language pathology were older than the age of our cohort at intake. Overall, parents' educational level did not influence services received with the exception of private services, which were more likely to occur in children of fathers with university education. CONCLUSIONS: The findings would suggest that long waiting times and lack of resources may limit access to comprehensive services, particularly in community settings. Service utilization patterns were not consistent within types of developmental disability, suggesting that formal and co-ordinated interdisciplinary programmes are not in place for this population of interest.

Long-term developmental outcome of asphyxiated term neonates.

Asphyxia remains one of the main causes of later disability in term infants. Despite many publications identifying possible predictors of outcome in this population of interest, little is known of the long-term developmental outcome of asphyxiated term neonates. Observational studies have largely focused on short-term outcomes, with an emphasis on significant neurologic sequelae and intellectual impairments. This article reviews the literature that has described the developmental outcome of asphyxiated term newborns. As part of this review, we have also highlighted the evolution of the definition of asphyxia and delineated appropriate markers that should be used in future research on this population.

Functional limitations in young children with congenital heart defects after cardiac surgery.

UNLABELLED: With the recent dramatic decline in mortality rates of infants undergoing open-heart surgery (OHS), there is growing concern regarding neurodevelopmental sequelae. Outcome studies have primarily focused on delineating developmental impairments; however, the impact on function and family burden has not been investigated. The objective of this study was to determine the prevalence of functional limitations and burden of care of young children with congenital heart defects (CHD) after OHS. STUDY DESIGN: One hundred thirty-one eligible infants with CHD undergoing their first OHS were recruited prospectively. Patients were assessed pre- and postoperatively, and again 12 to 18 months after surgery. Functional assessments included the WeeFIM (Functional Independence Measure) and the Vineland Adaptive Behavior Scale. RESULTS: For the WeeFIM, mean quotients were 84.3 +/- 23.8 (self-care), 77.2 +/- 30.0 (mobility), and 92.4 +/- 27.8 (cognition), with an overall quotient of 83.8 +/- 23.4. Only 21% of the cohort was functioning within their expected age range. Moderate disability was noted in 37%, while only 6% demonstrated a severe disability. For the Vineland scale, mean score for daily living skills was 84.4 +/- 17.6, and 80.3 +/- 15.9 for socialization. Functional difficulties in daily living skills were documented in 40%, whereas >1/2 had poor socialization skills. Factors enhancing risk for functional disabilities included perioperative neurodevelopmental status, microcephaly, length of deep hypothermic circulatory arrest, length of stay in the intensive care unit, age at surgery, and maternal education. CONCLUSIONS: The high prevalence of functional limitations and dependence in activities of daily living is currently underappreciated in the clinical setting, and deserves additional attention by pediatricians and developmental specialists.

Hyperbaric oxygen for children with cerebral palsy: a randomised multicentre trial. HBO-CP Research Group.

BACKGROUND: The use of hyperbaric oxygen for children with cerebral palsy has spread worldwide, despite little scientific evidence of efficacy. We did a randomised trial to assess the efficacy and side-effects of this form of therapy in children with cerebral palsy. METHODS: 111 children with cerebral palsy aged 3-12 years were randomly assigned hyperbaric oxygen (n=57) or slightly pressurised room air (n=54). All children received 40 treatments over 2 months. Hyperbaric oxygen treatment was 1 h in 100% oxygen at 1.75 atmospheres absolute (ATA); children on slightly pressurised air received air at 1.3 ATA (the lowest pressure at which pressure can be felt, thereby ensuring the maintenance of masking). The main outcome measure was gross motor function. Secondary outcomes included performance in activities of daily living, attention, working memory, and speech. FINDINGS: For all outcomes, both groups improved over the course of the study, but without any difference between the two treatments. The score on the global gross motor function measure increased by 3.0% in the children on slightly pressurised air and 2.9% in those on hyperbaric oxygen. The mean difference between treatments was -0.40 (95% CI -1.69 to 0.90, p=0.544). Other changes were seen in speech, attention, memory, and functional skills. Ear problems occurred in 27 children treated by hyperbaric oxygen and in 15 treated with hyperbaric air (p=0.004). INTERPRETATION: In this study, hyperbaric oxygen did not improve the condition of children with cerebral palsy compared with slightly pressurised air. The improvement seen in both groups for all dimensions tested deserves further consideration.

Profile of referrals for early childhood developmental delay to ambulatory subspecialty clinics.

The objective of this study was to determine the profile and pattern of referral to subspecialty clinics of young children with suspected developmental delay together with the factors prompting their referral. All children under 5 years of age referred to either developmental pediatrics or pediatric neurology clinics at a single tertiary hospital over an 18-month period were prospectively identified. Standardized demographic and referral information were collected at intake, final developmental delay subtype diagnosed was identified, and referring physicians were surveyed regarding factors prompting referral. A total of 224 children met study criteria. There was a marked male preponderance (166/224), especially among those with either cognitive or language delay. Two delay subtypes, global developmental delay and developmental language disorder, accounted for two thirds of the diagnoses made. For slightly more than one third of the children (75/224), the delay subtype diagnosed following specialty evaluation was different from that initially suspected by the referring physician. A mean delay of 15.5 months was observed for the cohort as a whole between initial parental concern and specialty assessment. For referring physicians, the major factor prompting referral was the severity of the observed delay. The most important aspects of the specialty evaluation according to referral sources were the identification of a possible etiology and confirmation of delay. A profile of referrals and the rationale thereof for a cohort of children with suspected developmental delay is presented that, although locale specific, has implications for service provision and training.

Association between electroencephalographic findings and neurologic status in infants with congenital heart defects.

Neurologic status is of concern in infants with congenital heart defects undergoing open heart surgery. The association between perioperative electroencephalography (EEG) with acute neurologic status and subsequent outcome was examined in a cohort of 60 infants. Preoperative EEG and neurologic examinations were performed within 1 to 2 days prior to surgery (n = 27) and postoperatively (n = 47). Prior to surgery, 15 of 27 infants had normal EEG, whereas 5 had epileptiform activity and 9 had disturbances in background activity that were primarily moderate (8/9) and diffuse (7/9). Postoperatively, only 17 of 47 infants had normal recordings. Newborns (<1 month) were more likely (P< .001) to demonstrate EEG abnormalities than infants. Epileptiform activity was documented in 15, whereas 28 had background abnormalities that were moderate-severe (22/28) and diffuse (20/28) in most. Epileptiform activity prior to surgery was always associated with an abnormal neurologic examination, and this association persisted postoperatively (86%). Moderate to severe background abnormalities in the postoperative EEG was also strongly associated with acute neurologic abnormalities (93%). Severe background abnormalities (n = 5) were 100% predictive of death or severe disability. Long-term follow-up revealed that all children with normal postoperative EEGs had positive neurologic outcomes (P = .04); however, there were many false positives. Perioperative EEG abnormalities increased the likelihood for acute neurologic findings, whereas normal recordings following surgery were reassuring with regard to a favorable outcome.

Etiologic yield of autistic spectrum disorders: a prospective study.

At present, the etiologic yield in community-derived samples of young children with an autistic spectrum disorder is not known. To address this question, all young children (under 5 years of age) referred for an initial assessment to ambulatory pediatric neurology or developmental pediatric clinics at a tertiary university center over an 18-month period for a suspected developmental delay were prospectively identified. Specific diagnostic testing was left to the discretion of the evaluating physician. In all, 50 children with an autistic spectrum disorder were assessed. Detailed history or physical examination was informative with respect to suggesting the possibility of an underlying etiology in a minority (10/50,20%). Genetic studies (FMR-1, karyotype), electroencephalography (EEG), and neuroimaging were carried out in a majority (42/50, 34/50, and 33/50, respectively) of the children, for the most part on a screening rather than an indicated basis (31/42, 34/34, and 28/33, respectively). Etiologic yield was low (1/50, 2%), with only a single child identified with a possible Landau-Kleffner variant on sleep EEG tracing. The results suggest an evaluation paradigm with reference to etiologic determination for young children with autistic spectrum disorder that does not presently justify metabolic or neuroimaging on a screening basis. Recurrence risk and treatment implications, however, suggest that strong consideration be given to genetic (FMR-1, karyotype) testing and EEG study despite a relatively low yield.

Etiologic determination of childhood developmental delay.

To determine the etiologic yield in young children with developmental delay referred to sub-specialty clinics for evaluation. Over an 18-month period, all children less than 5 years of age referred to the ambulatory pediatric neurology or developmental pediatrics clinics of the Montreal Children's Hospital for initial evaluation of a suspected developmental delay were enrolled. Features evident on history or physical examination were determined at intake as were the laboratory tests (and their rationale) requested by the evaluating physicians. Six months post initial assessment, detailed chart review was undertaken to determine if an etiology was found and the basis for such a determination. Bivariate and multivariate logistic regression was used to test for associations between factors present at intake and successful ascertainment of an underlying etiology. Two hundred and twenty-four children met study criteria. Etiologic yield varied across childhood developmental delay subtypes, and was 44/80 for global developmental delay [GDD] (55%), 13/22 for motor delay [MD] (59.1%), 3/72 for developmental language disorders [DLD] (4.2%), and 1/50 for autistic spectrum disorders [ASD] (2%). For GDD, the presence of historical features or findings on physical examination was associated with greater likelihood for successful etiologic determination with the following items significant in multiple logistic regression analysis; microcephaly, antenatal toxin exposure, focal findings. For MD, physical findings or the co-existence of a cerebral palsy symptom complex predicted a successful search for etiology. For both groups, the severity of the delay did not predict etiologic yield. For both groups, a small number of etiologic categories accounted for the majority of diagnoses made. Etiologic yield in childhood developmental delay is largely dependent on the specific developmental delay subtype. Paradigms for systematic evaluation of this common child health problem can be suggested, however they await validation.

Involvement of occupational therapy departments in research: a provincial survey.

It is increasingly recognized that research is necessary to advance the practice of occupational therapy. The objective of this study was to determine whether occupational therapy departments in Quebec were commonly involved in research, and whether participation varied with the size of department and type of work setting. Secondly, enablers and barriers to participation in research were identified. A random sample of occupational therapy departments, stratified by size, were surveyed by mail. Participation rate was 74.8% (107/143). Participation in research was noted in half of the departments surveyed (51/107), most frequently as collaborator. Involvement in research was associated (p < .001) with a greater number of therapists in the department. Furthermore, occupational therapy departments in rehabilitation centres and in university hospitals were more likely to participate in research (79% and 90% respectively), whereas participation was lower for long-term care facilities (29%) and community health clinics (38%). When asked to rank factors that may facilitate participation in research, the highest rankings were given to: time allotted to research, research as a priority for administration, adequate financial support, and presence of an occupational therapy researcher and a research centre on site. This survey demonstrates that a number of factors can promote or prevent the realization of research activities within the clinical setting. Strategies are proposed to enhance the integration of research into clinical practice.

Etiologic yield of single domain developmental delay: a prospective study.

OBJECTIVE: To determine the etiologic yield in young children with single domain developmental delay (either developmental language disorder or isolated motor delay) after a specialty diagnostic evaluation. METHODS: During an 18-month period, all children <5 years of age, who were consecutively referred to pediatric neurology or developmental pediatric clinics at a single tertiary pediatric center, were prospectively enrolled. Etiologic yield was determined after completion of clinical assessments and selected laboratory studies requested by the evaluating physician. RESULTS: Seventy-two children (60 boys) were found to have a developmental language disorder, and 22 children (11 boys) had isolated motor delay, of whom 6 had an associated diagnosis of cerebral palsy. An etiologic diagnosis was rarely made in the children with developmental language disorder (3/72, 4.1%). Laboratory investigations (metabolic, cytogenetic, imaging), aside from audiometry, were uniformly uninformative. In those children with isolated motor delay, an etiology was apparent in more than half (13/22, 59%). Slightly more than half (7/13, 54%) of etiologies identified in this group were potentially preventable. Successful etiologic determination in children with motor delay often had an impact on recurrence risk estimation, medical management, or specific therapy offered (8/13, 62%). The presence of physical findings on initial assessment was found to be highly predictive of successful etiologic determination in children with isolated motor delay (13/17 vs 0/5, P =.002). CONCLUSION: Etiologic yield differs substantially according to the subgroup of single domain developmental delay.

Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.

BACKGROUND: Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. OBJECTIVE: To describe the preoperative and postoperative neurodevelopmental status of newborns and infants with CHDs. STUDY DESIGN: One hundred thirty-one children (56 newborns and 75 infants) were evaluated before and after surgery by using standardized neurobehavioral (newborn) and motor assessments (infant) and neurologic examinations. RESULTS: In newborns, neurobehavioral abnormalities were documented in >50% before surgery, with abnormalities persisting in most after surgery. In infants, neurodevelopmental abnormalities were observed in 38% before surgery. There was a significant association between preoperative and postoperative neurodevelopmental status, with status remaining unchanged in most. Newborns with acyanotic heart lesions were more likely to demonstrate neurologic compromise than those with cyanotic defects. For infants, arterial oxygen saturations <85% were significantly associated with an abnormality. There was a trend for a longer circulatory arrest time to be associated with greater risk for neurologic sequelae in newborns, whereas prolonged cardiopulmonary bypass was an important risk factor for infants. CONCLUSIONS: Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.

Prediction of outcome at school age in neonatal intensive care unit graduates using neonatal neurologic tools.

Prediction of outcome for neonatal intensive care unit graduates is clinically useful to counsel families effectively and target those who may benefit from early interventions. Evoked potentials have proven prognostic value of neurologic outcomes in early childhood; however, their long-term predictive validity remains to be determined. The objective of this prospective study was to determine the long-term predictive value of three neonatal neurologic assessments: brainstem auditory evoked potentials, somatosensory evoked potentials, and the Einstein Neonatal Neurobehavioral Assessment Scale. Seventy-eight high-risk newborns and 28 healthy controls were recruited and were assessed in the newborn period using these tests. At 8 to 9 years of age, 42 subjects and 13 controls were re-evaluated for developmental progress using a range of psychologic, sensorimotor, and neurologic measures. Findings indicated that the somatosensory evoked potential was most accurate at predicting outcome at school age, with high specificity (83-100%) across all domains tested and good sensitivity (80-100%) for intellectual performance and sensorimotor abilities. The brainstem auditory evoked potential was limited by false-negatives, whereas the neonatal neurobehavioral assessment yielded many false-positives. This study provides new evidence that associations between neonatal somatosensory evoked potentials and developmental sequelae continue to be significant at school age.

Handwriting: current trends in occupational therapy practice.

The objective of this survey was to describe assessment and treatment approaches commonly used by occupational therapists for children exhibiting handwriting and related fine motor difficulties. Secondarily, the application of weights as a treatment modality was also explored. Fifty experienced paediatric occupational therapists from Ontario (46%), Quebec (22%) and six other Canadian provinces, were surveyed by telephone. The majority of therapists indicated that they evaluated gross/fine motor and perceptual skills, motor planning, quality of movement and sensory functioning for this population, while psychosocial and environmental factors were often not formally evaluated. Evaluations most commonly utilized included the Beery, Bruininks-Oseretsky and Gardner Tests. Standardized handwriting assessments were rarely employed. All used an eclectic treatment approach with sensorimotor most frequently selected (90%). Work setting and years of experience did not influence the treatment approach favoured.

Etiologic yield of subspecialists' evaluation of young children with global developmental delay.

OBJECTIVE: To determine the etiologic yield of subspecialists' evaluation of young children with global developmental delay. In addition, variables that may predict finding an underlying etiology were also identified. METHODS: All children <5 years of age, referred over an 18-month period to subspecialty services for initial evaluation of a suspected developmental delay, were prospectively enrolled. Diagnostic yield was ascertained after the completion of clinical assessments and laboratory investigations requested by the evaluating physician. RESULTS: Ninety-nine children (71 boys) were found to have global developmental delay; 96% had a mild or moderate delay documented. An etiologic diagnosis was determined in 44. Four diagnoses (cerebral dysgenesis, hypoxic-ischemic encephalopathy, toxin exposure, chromosomal abnormalities) accounted for 34 of 44 (77%) of the diagnoses made. The presence of co-existing autistic traits was associated with significantly decreased diagnostic yield (0/19 vs 44/80, P <.0001), whereas specific historical features (eg, family history, toxin exposure, and perinatal difficulty; 23/32 vs 21/67, P =.0002) and findings on physical examination (eg, dysmorphology, microcephaly, and focal motor findings; 35/48 vs 9/51, P <.0001) were significantly associated with identifying a diagnosis. Multiple logistic regression analysis identified antenatal toxin exposure, microcephaly, focal motor findings, and the absence of autistic traits as significant predictor variables for the identification of an etiology. CONCLUSION: An etiologic diagnosis is often possible in the young child with global developmental delay, particularly in the absence of autistic features. Etiologic yield is augmented by presence of specific findings on history or physical examination on initial assessment.

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, birthweight, 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.

Multimodality evoked potential findings in infants with congenital heart defects.

Evoked potentials are sensitive prognostic tools in young infants at risk for developmental disability. The objective of this prospective study was to determine whether infants with congenital heart defects demonstrate evoked potential abnormalities prior to or following open heart surgery, and to examine the association between these abnormalities and developmental status 1 year following surgery. A consecutive series of newborns (less than 1 month old) and infants (1 month to 2 years old) were recruited. Somatosensory and brain stem auditory evoked potentials were carried out before or after cardiac surgery, or both. One year later, neurologic examination and standardized measures of motor performance and functional independence were carried out. Twenty-seven newborns and 31 infants underwent perioperative somatosensory evoked potential recordings. Results indicate that perioperative somatosensory evoked potential abnormalities were common in newborns (41%) but not in infants (13%) with congenital heart defects. Brainstem conduction times were within normal limits in all subjects; however, 32% presented with mild elevations in hearing thresholds. All newborns with abnormal somatosensory evoked potentials had abnormal neurologic examinations both perioperatively and again 1 year after open heart surgery. Moreover, standardized developmental assessments 1 year following surgery indicate that all newborns with somatosensory evoked potential abnormalities had developmental deficits in one or more domains. Somatosensory evoked potential abnormalities in the perioperative period are common in newborns with congenital heart defects, and are strongly predictive of persistent developmental delay later.