Automated 3D Fetal Brain Segmentation Using an Optimized Deep Learning Approach.

BACKGROUND AND PURPOSE: MR imaging provides critical information about fetal brain growth and development. Currently, morphologic analysis primarily relies on manual segmentation, which is time-intensive and has limited repeatability. This work aimed to develop a deep learning-based automatic fetal brain segmentation method that provides improved accuracy and robustness compared with atlas-based methods. MATERIALS AND METHODS: A total of 106 fetal MR imaging studies were acquired prospectively from fetuses between 23 and 39 weeks of gestation. We trained a deep learning model on the MR imaging scans of 65 healthy fetuses and compared its performance with a 4D atlas-based segmentation method using the Wilcoxon signed-rank test. The trained model was also evaluated on data from 41 fetuses diagnosed with congenital heart disease. RESULTS: The proposed method showed high consistency with the manual segmentation, with an average Dice score of 0.897. It also demonstrated significantly improved performance (P < .001) based on the Dice score and 95% Hausdorff distance in all brain regions compared with the atlas-based method. The performance of the proposed method was consistent across gestational ages. The segmentations of the brains of fetuses with high-risk congenital heart disease were also highly consistent with the manual segmentation, though the Dice score was 7% lower than that of healthy fetuses. CONCLUSIONS: The proposed deep learning method provides an efficient and reliable approach for fetal brain segmentation, which outperformed segmentation based on a 4D atlas and has been used in clinical and research settings.

Regional Differences in Gamma-Aminobutyric Acid and Glutamate Concentrations in the Healthy Newborn Brain.

BACKGROUND AND PURPOSE: Gamma-aminobutyric acid and glutamate system disruptions may underlie neonatal brain injury. However, in vivo investigations are challenged by the need for special 1H-MR spectroscopy sequences for the reliable measurement of the neurotransmitters in this population. We used J-edited 1H-MR spectroscopy (Mescher-Garwood point-resolved spectroscopy) to quantify regional in vivo gamma-aminobutyric acid and glutamate concentrations during the early postnatal period in healthy neonates. MATERIALS AND METHODS: We prospectively enrolled healthy neonates and acquired Mescher-Garwood point-resolved spectroscopy spectra on a 3T MR imaging scanner from voxels located in the cerebellum, the right basal ganglia, and the right frontal lobe. CSF-corrected metabolite concentrations were compared for regional variations and cross-sectional temporal trends with advancing age. RESULTS: Fifty-eight neonates with acceptable spectra acquired at postmenstrual age of 39.1 (SD, 1.3) weeks were included for analysis. Gamma-aminobutyric acid (+ macromolecule) (2.56 [SD, 0.1]) i.u., glutamate (3.80 [SD, 0.2]), Cho, and mIns concentrations were highest in the cerebellum, whereas NAA (6.72 [SD, 0.2]), NAA/Cho, Cr/Cho, and Glx/Cho were highest in the basal ganglia. Frontal gamma-aminobutyric acid (1.63 [SD, 0.1]), Glx (4.33 [SD, 0.3]), Cr (3.64 [SD, 0.2]), and Cho concentrations were the lowest among the ROIs. Glx, NAA, and Cr demonstrated a significant adjusted increase with postmenstrual age (ß = 0.2-0.35), whereas gamma-aminobutyric acid and Cho did not. CONCLUSIONS: We report normative regional variations and temporal trends of in vivo gamma-aminobutyric acid and glutamate concentrations reflecting the functional and maturational status of 3 distinct brain regions of the neonate. These measures will serve as important normative values to allow early detection of subtle neurometabolic alterations in high-risk neonates.

Cerebral Perfusion Is Perturbed by Preterm Birth and Brain Injury.

BACKGROUND AND PURPOSE: Early disturbances in systemic and cerebral hemodynamics are thought to mediate prematurity-related brain injury. However, the extent to which CBF is perturbed by preterm birth is unknown. Our aim was to compare global and regional CBF in preterm infants with and without brain injury on conventional MR imaging using arterial spin-labeling during the third trimester of ex utero life and to examine the relationship between clinical risk factors and CBF. MATERIALS AND METHODS: We prospectively enrolled preterm infants younger than 32 weeks' gestational age and <1500 g and performed arterial spin-labeling MR imaging studies. Global and regional CBF in the cerebral cortex, thalami, pons, and cerebellum was quantified. Preterm infants were stratified into those with and without structural brain injury. We further categorized preterm infants by brain injury severity: moderate-severe and mild. RESULTS: We studied 78 preterm infants: 31 without brain injury and 47 with brain injury (29 with mild and 18 with moderate-severe injury). Global CBF showed a borderline significant increase with increasing gestational age at birth (P = .05) and trended lower in preterm infants with brain injury (P = .07). Similarly, regional CBF was significantly lower in the right thalamus and midpons (P < .05) and trended lower in the midtemporal, left thalamus, and anterior vermis regions (P < .1) in preterm infants with brain injury. Regional CBF in preterm infants with moderate-severe brain injury trended lower in the midpons, right cerebellar hemisphere, and dentate nuclei compared with mild brain injury (P < .1). In addition, a significant, lower regional CBF was associated with ventilation, sepsis, and cesarean delivery (P < .05). CONCLUSIONS: We report early disturbances in global and regional CBF in preterm infants following brain injury. Regional cerebral perfusion alterations were evident in the thalamus and pons, suggesting regional vulnerability of the developing cerebro-cerebellar circuitry.

In vivo assessment of placental and brain volumes in growth-restricted fetuses with and without fetal Doppler changes using quantitative 3D MRI.

OBJECTIVE: The relationship between placental and fetal brain growth is poorly understood and difficult to assess. The objective of this study was to interrogate placental and fetal brain growth in healthy pregnancies and those complicated by fetal growth restriction (FGR). STUDY DESIGN: In a prospective, observational study, pregnant women with normal pregnancies or pregnancies complicated by FGR underwent fetal magnetic resonance imaging (MRI). Placental, global and regional brain volumes were calculated. RESULTS: A total of 114 women (79 controls and 35 FGR) underwent MRI (median gestational age (GA) 30 weeks, range 18 to 39). All measured volumes increased exponentially with advancing GA. Placental, total brain, cerebral and cerebellar volumes were smaller in FGR compared with controls (P<0.05). Increasing placental volume was associated with increasing cerebral and cerebellar volumes (P<0.05). CONCLUSION: Quantitative fetal MRI can accurately detect decreased placental and brain volumes in pregnancies with FGR and may provide insight into the timing and mechanisms of brain injury in FGR.

Brain Injury in Neonates with Complex Congenital Heart Disease: What Is the Predictive Value of MRI in the Fetal Period?

BACKGROUND AND PURPOSE: Brain injury in neonates with congenital heart disease is an important predictor of adverse neurodevelopmental outcome. Impaired brain development in congenital heart disease may have a prenatal origin, but the sensitivity and specificity of fetal brain MR imaging for predicting neonatal brain lesions are currently unknown. We sought to determine the value of conventional fetal MR imaging for predicting abnormal findings on neonatal preoperative MR imaging in neonates with complex congenital heart disease. MATERIALS AND METHODS: MR imaging studies were performed in 103 fetuses with confirmed congenital heart disease (mean gestational age, 31.57 ± 3.86 weeks) and were repeated postnatally before cardiac surgery (mean age, 6.8 ± 12.2 days). Each MR imaging study was read by a pediatric neuroradiologist. RESULTS: Brain abnormalities were detected in 17/103 (16%) fetuses by fetal MR imaging and in 33/103 (32%) neonates by neonatal MR imaging. Only 9/33 studies with abnormal neonatal findings were preceded by abnormal findings on fetal MR imaging. The sensitivity and specificity of conventional fetal brain MR imaging for predicting neonatal brain abnormalities were 27% and 89%, respectively. CONCLUSIONS: Brain abnormalities detected by in utero MR imaging in fetuses with congenital heart disease are associated with higher risk of postnatal preoperative brain injury. However, a substantial proportion of anomalies on postnatal MR imaging were not present on fetal MR imaging; this result is likely due to the limitations of conventional fetal MR imaging and the emergence of new lesions that occurred after the fetal studies. Postnatal brain MR imaging studies are needed to confirm the presence of injury before open heart surgery.

Elucidating Metabolic Maturation in the Healthy Fetal Brain Using 1H-MR Spectroscopy.

BACKGROUND AND PURPOSE: (1)H-MRS provides a noninvasive way to study fetal brain maturation at the biochemical level. The purpose of this study was to characterize in vivo metabolic maturation in the healthy fetal brain during the second and third trimester using (1)H-MRS. MATERIALS AND METHODS: Healthy pregnant volunteers between 18 and 40 weeks gestational age underwent single voxel (1)H-MRS. MR spectra were retrospectively corrected for motion-induced artifacts and quantified using LCModel. Linear regression was used to examine the relationship between absolute metabolite concentrations and ratios of total NAA, Cr, and Cho to total Cho and total Cr and gestational age. RESULTS: Two hundred four spectra were acquired from 129 pregnant women at mean gestational age of 30.63 ± 6 weeks. Total Cho remained relatively stable across the gestational age (r(2) = 0.04, P = .01). Both total Cr (r(2) = 0.60, P < .0001) as well as total NAA and total NAA to total Cho (r(2) = 0.58, P < .0001) increased significantly between 18 and 40 weeks, whereas total NAA to total Cr exhibited a slower increase (r(2) = 0.12, P < .0001). Total Cr to total Cho also increased (r(2) = 0.53, P < .0001), whereas total Cho to total Cr decreased (r(2) = 0.52, P < .0001) with gestational age. The cohort was also stratified into those that underwent MRS in the second and third trimesters and analyzed separately. CONCLUSIONS: We characterized metabolic changes in the normal fetal brain during the second and third trimesters of pregnancy and derived normative metabolic indices. These reference values can be used to study metabolic maturation of the fetal brain in vivo.

Pregnancy outcomes in two growth restricted fetuses with in utero cerebral lactate.

Elevated cerebral lactate is increasingly detected by magnetic resonance spectroscopy in the human fetus diagnosed with various pathologic conditions. However, the significance of detectable cerebral lactate remains uncertain. We present two cases of fetal cerebral lactate with adverse pregnancy outcomes.

Assessment of MRI-Based Automated Fetal Cerebral Cortical Folding Measures in Prediction of Gestational Age in the Third Trimester.

BACKGROUND AND PURPOSE: Traditional methods of dating a pregnancy based on history or sonographic assessment have a large variation in the third trimester. We aimed to assess the ability of various quantitative measures of brain cortical folding on MR imaging in determining fetal gestational age in the third trimester. MATERIALS AND METHODS: We evaluated 8 different quantitative cortical folding measures to predict gestational age in 33 healthy fetuses by using T2-weighted fetal MR imaging. We compared the accuracy of the prediction of gestational age by these cortical folding measures with the accuracy of prediction by brain volume measurement and by a previously reported semiquantitative visual scale of brain maturity. Regression models were constructed, and measurement biases and variances were determined via a cross-validation procedure. RESULTS: The cortical folding measures are accurate in the estimation and prediction of gestational age (mean of the absolute error, 0.43 ± 0.45 weeks) and perform better than (P = .024) brain volume (mean of the absolute error, 0.72 ± 0.61 weeks) or sonography measures (SDs approximately 1.5 weeks, as reported in literature). Prediction accuracy is comparable with that of the semiquantitative visual assessment score (mean, 0.57 ± 0.41 weeks). CONCLUSIONS: Quantitative cortical folding measures such as global average curvedness can be an accurate and reliable estimator of gestational age and brain maturity for healthy fetuses in the third trimester and have the potential to be an indicator of brain-growth delays for at-risk fetuses and preterm neonates.

Prevalence and spectrum of in utero structural brain abnormalities in fetuses with complex congenital heart disease.

BACKGROUND AND PURPOSE: Brain injury is a major complication in neonates with complex congenital heart disease. Preliminary evidence suggests that fetuses with congenital heart disease are at greater risk for brain abnormalities. However, the nature and frequency of these brain abnormalities detected by conventional fetal MR imaging has not been examined prospectively. Our primary objective was to determine the prevalence and spectrum of brain abnormalities detected on conventional clinical MR imaging in fetuses with complex congenital heart disease and, second, to compare the congenital heart disease cohort with a control group of fetuses from healthy pregnancies. MATERIALS AND METHODS: We prospectively recruited pregnant women with a confirmed fetal congenital heart disease diagnosis and healthy volunteers with normal fetal echocardiogram findings who underwent a fetal MR imaging between 18 and 39 weeks gestational age. RESULTS: A total of 338 fetuses (194 controls; 144 with congenital heart disease) were studied at a mean gestational age of 30.61 ± 4.67 weeks. Brain abnormalities were present in 23% of the congenital heart disease group compared with 1.5% in the control group (P < .001). The most common abnormalities in the congenital heart disease group were mild unilateral ventriculomegaly in 12/33 (36.4%) and increased extra-axial spaces in 10/33 (30.3%). Subgroup analyses comparing the type and frequency of brain abnormalities based on cardiac physiology did not reveal significant associations, suggesting that the brain abnormalities were not limited to those with the most severe congenital heart disease. CONCLUSIONS: This is the first large prospective study reporting conventional MR imaging findings in fetuses with congenital heart disease. Our results suggest that brain abnormalities are prevalent but relatively mild antenatally in fetuses with congenital heart disease. The long-term predictive value of these findings awaits further study.

Brain perfusion in encephalopathic newborns after therapeutic hypothermia.

BACKGROUND AND PURPOSE: Cerebral perfusion patterns in neonates with HIE after therapeutic hypothermia have not been well described. The objectives of this study were to compare global and regional perfusion between infants with HIE and neonate controls and to relate measures of cerebral perfusion to brain injury on conventional MR imaging in neonates with HIE. MATERIALS AND METHODS: Term encephalopathic neonates meeting criteria for hypothermia between June 2011 and January 2012 were enrolled in this prospective observational study. MR imaging-ASL was performed in the second week of life. Comparisons were made with data from neonate controls who underwent the same imaging protocol. NIRS measures of cerebral oxygenation during and immediately after hypothermia were also evaluated in a subset of patients. Secondary analyses were performed to assess cerebral perfusion and oxygenation differences by pattern of injury on qualitative MR imaging interpretation. RESULTS: We enrolled 18 infants with HIE and 18 control infants. Mean global CBF and regional CBF in the basal ganglia, thalamus, and anterior white matter were higher in cases compared with controls. Infants with HIE with injury on MR imaging, however, had lower CBF (significant in the thalamus) compared with those with normal MR imaging. Decreased FTOE by NIRS further differentiated patients with HIE with injury on MR imaging. CONCLUSIONS: Disturbed cerebral perfusion is observed in the second week of life in some babies with HIE despite treatment with hypothermia. Infants with HIE with injury on MR imaging have lower regional CBF in the thalamus compared with those without injury, possibly representing pseudonormalization of CBF and low metabolic demand after progression to irreversible brain injury.

Delayed cortical development in fetuses with complex congenital heart disease.

Neurologic impairment is a major complication of complex congenital heart disease (CHD). A growing body of evidence suggests that neurologic dysfunction may be present in a significant proportion of this high-risk population in the early newborn period prior to surgical interventions. We recently provided the first evidence that brain growth impairment in fetuses with complex CHD has its origins in utero. Here, we extend these observations by characterizing global and regional brain development in fetuses with hypoplastic left heart syndrome (HLHS), one of the most severe forms of CHD. Using advanced magnetic resonance imaging techniques, we compared in vivo brain growth in 18 fetuses with HLHS and 30 control fetuses from 25.4-37.0 weeks of gestation. Our findings demonstrate a progressive third trimester fall-off in cortical gray and white matter volumes (P < 0.001), and subcortical gray matter (P < 0.05) in fetuses with HLHS. Significant delays in cortical gyrification were also evident in HLHS fetuses (P < 0.001). In the HLHS fetus, local cortical folding delays were detected as early as 25 weeks in the frontal, parietal, calcarine, temporal, and collateral regions and appear to precede volumetric brain growth disturbances, which may be an early marker of elevated risk for third trimester brain growth failure.

Neuroimaging and cerebral palsy in children.

Cerebral palsy (CP) is a description of a spectrum of central nervous system (CNS) impairments that affect mobility, communication, intellectual ability, and neurobehavior as a result of developmental brain dysfunction. CP is the most common contributor to motor disability in children with prevalence of about 2-3/1000 live births globally. Presently, no curative therapies or successful methods of prevention on a population level are available for children with one of the cerebral palsy syndromes. Despite these challenges, orthopedic, rehabilitation, neuropharmacological, and other management interventions can help maintain mobility, prevent deformity, and promote quality of life for children with CP. Typically, the diagnosis of CP is based on clinical observations and parent concerns regarding delays in attaining motor milestones (e.g., rolling, sitting, crawling, walking), not on laboratory testing or neuroimaging. However, since 2004 the American Academy of Neurology (AAN) has recommended that neuroimaging of the CNS be part of diagnostic process for cerebral palsy. Although the guideline was initially met with controversy and criticism, neuroimaging has allowed a broader appreciation of timing of lesions, extent of white matter involvement, and the complexity of the motor spectrum of disability. In this article we shall describe the major types of neuroimaging techniques and review their roles in identification and evaluation of children with one of the cerebral palsy syndromes. The authors will focus on the emerging knowledge of how brain structure can inform us about children's functioning, especially among children with prematurity, recognizing that we are only beginning to understand brain plasticity and developmental resiliency.

Cerebellar cleft: confirmation of the neuroimaging pattern.

We recently described the neuroimaging and clinical findings in 6 children with cerebellar clefts and proposed that they result from disruptive changes following prenatal cerebellar hemorrhage. We now report an additional series of 9 patients analyzing the clinical and neuroimaging findings. The clefts were located in the left cerebellar hemisphere in 5 cases, in the right in 3, and bilaterally in one child who had bilateral cerebellar hemorrhages as a preterm infant at 30 weeks gestation. In one patient born at 24 weeks of gestation a unilateral cerebellar hemorrhage has been found at the age of 4 months. Other findings included disordered alignment of the folia and fissures, an irregular gray/white matter junction, and abnormal arborization of the white matter in all cases. Supratentorial abnormalities were found in 4 cases. All but 2 patients were born at term. We confirm the distinct neuroimaging pattern of cerebellar clefts. Considering the documented fetal cerebellar hemorrhage in our first series, we postulate that cerebellar clefts usually represent residual disruptive changes after a prenatal cerebellar hemorrhage. Exceptionally, as now documented in 2 patients, cerebellar clefts can be found after neonatal cerebellar hemorrhages in preterm infants. The short-term outcome in these children was variable.

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.

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.

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.

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.

Developmental progress of children with congenital heart defects requiring open heart surgery.

Recent advances for infants requiring early open heart surgery have resulted in a dramatic decline in mortality and severe morbidity. The developmental progress of these new survivors is currently being defined. Causes contributing to brain injury are multifactorial, and may involve preoperative, intraoperative, and postoperative events. Before surgery, these children often exhibit hypotonia, poor state regulation, microcephaly, and developmental delays. These findings are particularly prevalent in newborns. In the acute postoperative period, neurodevelopmental deficits continue to manifest clinically. Long-term follow-up studies indicate that subtle neurological deficits and global developmental lags are characteristic of this population. Overall, severe neurological sequelae are uncommon; however, mild to moderate developmental disabilities are prevalent. Functional limitations, academic achievements, and health-related quality of life are areas that deserve further attention.

Neurologic status of newborns with congenital heart defects before open heart surgery.

UNLABELLED: Controversy exists regarding the integrity of the nervous system in the newborn with a congenital heart defect who must undergo corrective or palliative open heart surgery. Neurodevelopmental sequelae have been primarily attributed to surgical procedures without standardized evaluation of the preoperative neurologic status. OBJECTIVE: To determine whether newborns with congenital heart defects demonstrate abnormalities in neurobehavioral status before surgery. STUDY DESIGN: In this prospective study, a standardized neonatal neurobehavioral assessment and a neurologic examination were conducted independently in a consecutive series of 56 neonates referred to our hospital for investigation of open heart surgery. RESULTS: Neurobehavioral and neurologic abnormalities were documented in greater than half of the cohort and included hypotonia, hypertonia, jitteriness, motor asymmetries, and absent suck. Poor state regulation (62%) and feeding difficulties (34%) also were commonly observed. Furthermore, 3 subjects had seizures, 35.7% were microcephalic, and 12.5% were macrocephalic. The overall likelihood of neurobehavioral abnormalities was not enhanced by indicators of cardiorespiratory compromise. Interestingly, newborns with acyanotic congenital heart defects were more likely to demonstrate neurologic compromise than were those with cyanotic defects. CONCLUSIONS: Findings suggest that the prevalence of neurobehavioral abnormalities before surgery in newborns with congenital heart defects has been underappreciated and would indicate that factors other than intraoperative procedures should be considered in the genesis of brain injury in this population.congenital heart defects, neurologic examination, newborn.

Agreement between the neonatal neurological examination and a standardized assessment of neurobehavioural performance in a group of high-risk newborns.

The degree of agreement between the neonatal neurological examination and a standardized neurobehavioural assessment was investigated in a group of 32 newborns with congenital heart defects. A paediatric neurologist performed a neurological examination, and an occupational therapist administered the Einstein Neonatal Neurobehavioural Assessment Scale on all subjects. Both examiners independently evaluated each subject, and were blinded to the diagnosis, to perinatal status and to each other's clinical findings. Statistical analysis demonstrated a significant association between the overall impression between both examiners (p < .0001), with a crude agreement of 96.9%. Sixteen neonates were determined to be normal and 15 abnormal by both examiners, with disagreement in only one subject. Although two distinct approaches were employed in the neurological assessment of high-risk newborns, both assessments evaluate the maturity and integrity of the immature central nervous system. The results demonstrate a strong agreement between these two approaches, suggesting that the neonatal neurological examination is consistent and valid.