Search

Patients with congenital heart disease (CHD) demonstrate altered structural brain network connectivity. However, there is large variability between reported results and little information is available to identify those patients at highest risk for brain alterations. Thus, we aimed to investigate if network connectivity measures were associated with the individual patient's cumulative load of clinical risk factors and with family‐environmental factors in a cohort of adolescents with CHD. Further, we investigated associations with executive function impairments. In 53 adolescents with CHD who underwent open‐heart surgery during infancy, and 75 healthy controls, diffusion magnetic resonance imaging and neuropsychological assessment was conducted at a mean age of 13.2 ± 1.3 years. Structural connectomes were constructed using constrained spherical deconvolution tractography. Graph theory and network‐based statistics were applied to investigate network connectivity measures. A cumulative clinical risk (CCR) score was built by summing up binary risk factors (neonatal, cardiac, neurologic) based on clinically relevant thresholds. The role of family‐environmental factors (parental education, parental mental health, and family function) was investigated. An age‐adjusted executive function summary score was built from nine neuropsychological tests. While network integration and segregation were preserved in adolescents with CHD, they showed lower edge strength in a dense subnetwork. A higher CCR score was associated with lower network segregation, edge strength, and executive function performance. Edge strength was particularly reduced in a subnetwork including inter‐frontal and fronto‐parietal‐thalamic connections. There was no association with family‐environmental factors. Poorer executive functioning was associated with lower network integration and segregation. We demonstrated evidence for alterations of network connectivity strength in adolescents with CHD — particularly in those patients who face a cumulative exposure to multiple clinical risk factors over time. Quantifying the cumulative load of risk early in life may help to better predict trajectories of brain development in order to identify and support the most vulnerable patients as early as possible. [ABSTRACT FROM AUTHOR]

In addition to the primary aim of seizure freedom, a key secondary aim of pediatric epilepsy surgery is to stabilize and, potentially, optimize cognitive development. Although the efficacy of surgical treatment for seizure control has been established, the long‐term intellectual and developmental trajectories are yet to be delineated. We conducted a systematic review and meta‐analysis of studies reporting pre‐ and postsurgical intelligence or developmental quotients (IQ/DQ) of children with focal lesional epilepsy aged ≤18 years at epilepsy surgery and assessed at >2 years after surgery. We determined the IQ/DQ change and conducted a random‐effects meta‐analysis and meta‐regression to assess its determinants. We included 15 studies reporting on 341 patients. The weighted mean age at surgery was 7.1 years (range =.3–13.8). The weighted mean postsurgical follow‐up duration was 5.6 years (range = 2.7–12.8). The overall estimate of the mean presurgical IQ/DQ was 60 (95% confidence interval [CI] = 47–73), the postsurgical IQ/DQ was 61 (95% CI = 48–73), and the change was +.94 IQ/DQ (95% CI = −1.70 to 3.58, p =.486). Children with presurgical IQ/DQ ≥ 70 showed a tendency for higher gains than those with presurgical IQ/DQ < 70 (p =.059). Higher gains were determined by cessation of antiseizure medication (ASM; p =.041), not just seizure freedom. Our findings indicate, on average, stabilization of intellectual and developmental functioning at long‐term follow‐up after epilepsy surgery. Once seizure freedom has been achieved, ASM cessation enables the optimization of intellectual and developmental trajectories in affected children. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) patients are at risk for alterations in the cerebral white matter microstructure (WMM) throughout development. It is unclear whether the extent of WMM alterations changes with age, especially during adolescence when the WMM undergoes rapid maturation. We investigated differences in WMM between patients with CHD and healthy controls from childhood until early adulthood in a pooled sample of children, adolescents, and young adults. The association between WMM and EF was assessed. Patients with CHD (N=78) and controls (N=137) between 9 and 32 years of age underwent diffusion tensor imaging and an executive function test-battery. Mean fractional anisotropy (FA) was calculated for each white matter tract. Linear regression tested age and group effects (CHD vs control) and their interaction on FA. Relative Variable Importance (RI) estimated the independent contribution of tract FA, presence of CHD, CHD complexity, and parental education to the variability in EF. Mean FA was lower in patients compared to controls in almost all tracts (p between 0.057 and <0.001). WMM alterations in patients were not different depending on age (all interaction effects p>0.074). Predictors of EF were CHD group (RI=43%), parental education (RI=23%), CHD complexity (RI=10%), FA of the hippocampal cingulum (RI=6%) and FA of the corticospinal tract (RI=6%). The lack of group-FA-interactions indicates that the extent of altered FA remains similar across age. Altered FA is associated with EF impairments. CHD is a chronic disease with cerebral and neurocognitive impairments persisting into adulthood and, thus, long-term follow-up programs may improve overall outcome for this population. [ABSTRACT FROM AUTHOR]

Working memory is frequently impaired in children with complex congenital heart disease (CHD), but little is known about the functional neuronal correlates. Sleep slow wave activity (SWA; 1–4.5 Hz EEG power) has previously been shown to reliably map neurofunctional networks of cognitive abilities in children with and without neurodevelopmental impairments. This study investigated whether functional networks of working memory abilities are altered in children with complex CHD using EEG recordings during sleep. Twenty-one children with complex CHD (aged 10.9 [SD: 0.3] years) and 17 typically-developing peers (10.5 [0.7] years) completed different working memory tasks and an overnight high-density sleep EEG recording (128 electrodes). The combined working memory score tended to be lower in children with complex CHD (CHD group: −0.44 [1.12], typically-developing group: 0.55 [1.24], d = 0.59, p =.06). The working memory score and sleep SWA of the first hour of deep sleep were correlated over similar brain regions in both groups: Strong positive associations were found over prefrontal and fronto-parietal brain regions – known to be part of the working memory network – and strong negative associations were found over central brain regions. Within these working memory networks, the associations between working memory abilities and sleep SWA (r between −.36 and.58, all p <.03) were not different between the two groups (no interactions, all p >.05). The current findings suggest that sleep SWA reliably maps working memory networks in children with complex CHD and that these functional networks are generally preserved in these patients. [ABSTRACT FROM AUTHOR]

PURPOSE: This study aimed to describe outcomes of motor function with a special focus on ambulation ability at 36 months among children with open prenatal repair of spina bifida aperta (SB). METHODS: A prospective cohort study was conducted including 87 patients with open prenatal repair of SB at the investigating center born between 2010 and 2018. Anatomic lesion level and motor function level in the neonatal period, as well as motor function level, ambulation status, and use of orthotics and assistive devices at 36 months were assessed. RESULTS: At 36 months, ambulation was assessed in 86 children; of those, 86% (n = 74) were ambulating. Independent of ambulation, orthotics were worn in 81.6% (71/87) and assistive devices in 47.1% (41/87). Children with a lower lumbar or sacral motor function level were the first to reach independent ambulation and were more likely to ambulate at 36 months than children with higher motor function levels (p = <.001). The anatomic lesion level determined on the neonatal MRI correlated with ambulation status at 36 months (p = < 0.001). CONCLUSION: At 36 months, most children with open prenatal repair for SB showed favourable ambulation status. However, most still used assistive devices or orthotics. Anatomic lesion level on neonatal MRI, motor function level during the neonatal period, and motor function level at 36 months were associated with ambulation status at 36 months. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Children with univentricular congenital heart disease undergoing staged surgical palliation are at risk for impaired neurodevelopmental (ND) outcome. Little is known about the long-term effects on brain growth until school age. METHODS In a prospective two-centre study, consecutive patients undergoing stage I (Hybrid or Norwood) to stage III (Fontan procedure) were evaluated by 2 serial cerebral magnetic resonance imaging examinations, somatic growth and ND testing before Fontan procedure at 2 years of age (Bayley-III) and after Fontan at 6–8 years of age (Wechsler Intelligence Scale for Children-third edition). Magnetic resonance imaging findings were compared with 8 healthy controls. Medical and sociodemographic characteristics were documented and related to cerebral and ND findings. RESULTS We examined 33 children (16 female) at a mean age of 2.3 (0.35) and 6.8 (± 0.7) years. The mean Bayley-III cognitive scales were 99.1 (9.9), language scales 98.4 (11.9) and motor scales 98.5 (13.8) at the first examination. Follow-up at school age showed a mean total IQ of 86.7 (13.6). The rate of structural brain lesions increased from 39% at 2 years to 58% at school age. Bayley-III language scale (P  = 0.021) and mean Wechsler Intelligence Scale for Children-third edition (P  = 0.019) were lower in children with pathological MR findings. Total brain volume (P  < 0.001), total grey matter volume (P  = 0.002), deep grey matter volume (P  = 0.001) and white matter volume (P  < 0.001) were smaller in patients compared to age- and gender-matched healthy controls. CONCLUSIONS Smaller brain volumes and structural brain lesions in complex congenital heart defect patients at school age are associated with impaired ND outcome. For the evaluation of predictive surgical or clinical factors, larger multicentre studies are needed. [ABSTRACT FROM AUTHOR]

Aim: We assessed motor and intellectual outcome in triplets at school age and investigated the predictive value of perinatal and demographic factors. Methods: Seventy-one live-born newborn infants (24 triplet pregnancies) were prospectively enrolled at birth. At the age of 6 years, 58 children (31 males, 27 females; mean gestational age 31.2wks [SD 2.2wks]; mean birthweight 1622g [SD 440g]) returned for a neurodevelopmental examination. A comparison group for triplets born before 32 gestational weeks comprising 26 gestational age-, birthweight-, and sex-matched singletons was also recruited (mean gestational age 30.1wk [SD 1.5wk]; mean birthweight 1142g [SD 210g]; 12 males, 14 females). The Zurich Neuromotor Assessment was used to examine motor performance, and intellectual abilities were assessed with the Kaufman Assessment Battery for Children (K-ABC). Results: Motor performance and movement quality in these individuals was significantly reduced compared with the test norms for all motor tasks (p less than 0.001) other than static balance. The mean values on the Mental Processing Composite (95.3, SD 8.4) and the Achievement Scale (90.1, SD 13.8) of the K-ABC were also lower than those in the test reference (p less than 0.05 and p less than 0.01 respectively). Triplets born at less than 32 weeks' gestation showed poorer pure motor and adaptive gross motor performance (both p less than 0.05) than, but similar intellectual performance to, the gestational age-, birthweight- and sex-matched singletons. Poor outcome was predicted by low socio-economic status and by intertriplet birthweight discordance (both p less than 0.01). Interpretation: Triplets were at an increased risk of mild motor and intellectual impairments. This finding is important for tailoring therapeutic interventions for these children and for parental counselling. Very preterm triplets showed similar outcomes to the singleton comparison children, except that they had poorer motor performance. Low socio-economic status was a major risk factor for impaired intellectual development. In addition, birthweight discordance may also be considered a predictor for poor long-term motor and intellectual outcome in triplets.

Background To investigate the long-term neurodevelopmental outcome in children after hemolytic uremic syndrome (HUS) and to compare outcome dependent on central nervous system (CNS) involvement during HUS. Methods A single-center retrospective cohort of 47 children was examined at a median age of 10.6 (range 6-16.9) years and a median follow-up of 7.8 (range 0.4-15.3) years after having had HUS. Intellectual performance was assessed with the German version of the Wechsler Intelligence Scale 4th version and neuromotor performance with the Zurich Neuromotor Assessment (ZNA). The occurrence of neurological symptoms during the acute phase of HUS was evaluated retrospectively. Results Mean IQ of the whole study population fell within the normal range (median full scale IQ 104, range 54-127). Neuromotor performance was significantly poorer in the domains 'adaptive fine,' 'gross motor,' 'static balance' (all p < 0.05) and 'associated movements' (p < 0.001); only the 'pure motor' domain was within the normal reference range. Neurological findings occurred in 16/47 patients (34%) during acute HUS. Neurodevelopmental outcome was not significantly different between children with or without CNS involvement. Conclusions Our follow-up of children after HUS showed a favorable cognitive outcome. However, neuromotor outcome was impaired in all study participants. Neurological impairment during acute HUS was not predictive of outcome. Keywords Intellectual * Motor * Neurocognitive outcome * Central nervous system involvement * Hemolytic uremic syndrome, Introduction Hemolytic uremic syndrome (HUS) is a multi-organ and life-threatening disease characterized by hemolytic anemia, thrombocytopenia and acute renal injury. HUS is also one of the most frequent causes of [...]