Impact of Macrocephaly, as an Isolated Trait, on EEG Signal as Measured by Spectral Power and Multiscale Entropy during the First Year of Life.

Macrocephaly has been associated with neurodevelopmental disorders; however, it has been mainly studied in the context of pathological or high-risk populations and little is known about its impact, as an isolated trait, on brain development in general population. Electroencephalographic (EEG) power spectral density (PSD) and signal complexity have shown to be sensitive to neurodevelopment and its alterations. We aimed to investigate the impact of macrocephaly, as an isolated trait, on EEG signal as measured by PSD and multiscale entropy during the first year of life. We recorded high-density EEG resting-state activity of 74 healthy full-term infants, 50 control (26 girls), and 24 macrocephalic (12 girls) aged between 3 and 11 months. We used linear regression models to assess group and age effects on EEG PSD and signal complexity. Sex and brain volume measures, obtained via a 3D transfontanellar ultrasound, were also included into the models to evaluate their contribution. Our results showed lower PSD of the low alpha (8-10 Hz) frequency band and lower complexity in the macrocephalic group compared to the control group. In addition, we found an increase in low alpha (8.5-10 Hz) PSD and in the complexity index with age. These findings suggest that macrocephaly as an isolated trait has a significant impact on brain activity during the first year of life.

Ultrasound findings in rapidly involuting congenital hemangioma (RICH) - beware of venous ectasia and venous lakes.

BACKGROUND: Bleeding, ulceration and cardiac failure can occur in rapidly involuting congenital hemangioma (RICH). Bleeding intensity ranges from superficial to life-threatening. OBJECTIVE: To determine whether there are sonographic criteria associated with an increased risk of bleeding, ulceration or cardiac failure in RICH in order to identify children who need close monitoring or prophylactic treatment. MATERIALS AND METHODS: This retrospective single-center study included RICH patients over a period of 13 years. We evaluated sonographic features of RICH on B-mode and Doppler ultrasound. We correlated the occurrence of bleeding, ulceration and cardiac failure with four sonographic findings: (1) visible vessel, (2) venous ectasia, (3) venous lake and (4) arteriovenous shunting. RESULTS: We included 24 patients. Ulceration occurred in five cases, bleeding in four cases, one of which was life-threatening. Cardiac failure was observed more frequently in RICH with venous lakes (P=0.028). Bleeding and ulceration appeared more frequently in RICH with venous ectasia and venous lakes. Cardiac failure was associated with the presence of venous ectasia. All children with cardiac failure or ulceration had arteriovenous shunts. CONCLUSION: RICH with venous lakes on ultrasound is prone to develop bleeding, cardiac failure and ulceration. This association was only significant for cardiac failure.

Predicting Perinatal Outcome from Prenatal Ultrasound Characteristics in Pregnancies Complicated by Gastroschisis.

INTRODUCTION: The objective of the study was to establish the predictive value of prenatal ultrasound markers for complex gastroschisis (GS) in the first 10 days of life. MATERIAL AND METHODS: In this retrospective cohort study over 11 years (2000-2011) of 117 GS cases, the following prenatal ultrasound signs were analyzed at the last second- and third-trimester ultrasounds: intrauterine growth restriction, intra-abdominal bowel dilatation (IABD) adjusted for gestational age, extra-abdominal bowel dilatation (EABD) ≥25 mm, stomach dilatation, stomach herniation, perturbed mesenteric circulation, absence of bowel lumen and echogenic dilated bowel loops (EDBL). RESULTS: Among 114 live births, 16 newborns had complex GS (14.0%). Death was seen in 16 cases (13.7%): 3 intrauterine fetal deaths, 9 complex GS and 4 simple GS. Second-trimester markers had limited predictive value. Third-trimester IABD, EABD, EDBL, absence of intestinal lumen and perturbed mesenteric circulation were statistically associated with complex GS and death. IABD was able to predict complex GS with a sensitivity of 50%, a specificity of 91%, a positive predictive value of 47% and a negative predictive value of 92%. DISCUSSION: Third-trimester IABD adjusted for gestational age appears to be the prenatal ultrasound marker most strongly associated with adverse outcome in GS.

Phenotype and outcomes of very early onset and early onset inflammatory bowel diseases in a Montreal pediatric cohort.

Objectives: The incidence of very-early-onset inflammatory bowel disease (VEO-IBD) and early-onset IBD (EO-IBD) is increasing. Here, we report their phenotype and outcomes in a Montreal pediatric cohort. Methods: We analyzed data from patients diagnosed with IBD between January 2014 and December 2018 from the CHU Sainte-Justine. The primary endpoint was to compare the phenotypes of VEO-IBD and EO-IBD. The secondary endpoints involved comparing outcomes and rates of steroid-free clinical remission (SFCR) at 12 (±2) months (m) post-diagnosis and at last follow-up. Results: 28 (14 males) and 67 (34 males) patients were diagnosed with VEO-IBD and EO-IBD, respectively. Crohn's disease (CD) was more prevalent in EO-IBD (64.2% vs. 39.3%), whereas unclassified colitis (IBD-U) was diagnosed in 28.6% of VEO-IBD vs. 10.4% of EO-IBD (p < 0.03). Ulcerative colitis (UC) and IBD-U predominantly presented as pancolitis in both groups (VEO-IBD: 76.5% vs. EO-IBD: 70.8%). Combining all disease subtypes, histological upper GI lesions were found in 57.2% of VEO-IBD vs. 83.6% of EO-IBD (p < 0.009). In each subtype, no differential histological signature (activity, eosinophils, apoptotic bodies, granulomas) was observed between both groups. At 12 m post-diagnosis, 60.8% of VEO-IBD and 62.7% of EO-IBD patients were in SFCR. At a median follow-up of 56 m, SFCR was observed in 85.7% of VEO-IBD vs. 85.0% of EO-IBD patients. Conclusion: The rate of patients in SFCR at 1-year post-diagnosis and at the end of follow-up did not significantly differ between both groups.

Impact of brain overgrowth on sensorial learning processing during the first year of life.

Macrocephaly is present in about 2-5% of the general population. It can be found as an isolated benign trait or as part of a syndromic condition. Brain overgrowth has been associated with neurodevelopmental disorders such as autism during the first year of life, however, evidence remains inconclusive. Furthermore, most of the studies have involved pathological or high-risk populations, but little is known about the effects of brain overgrowth on neurodevelopment in otherwise neurotypical infants. We investigated the impact of brain overgrowth on basic perceptual learning processes (repetition effects and change detection response) during the first year of life. We recorded high density electroencephalograms (EEG) in 116 full-term healthy infants aged between 3 and 11 months, 35 macrocephalic (14 girls) and 81 normocephalic (39 girls) classified according to the WHO head circumference norms. We used an adapted oddball paradigm, time-frequency analyses, and auditory event-related brain potentials (ERPs) to investigate differences between groups. We show that brain overgrowth has a significant impact on repetition effects and change detection response in the 10-20 Hz frequency band, and in N450 latency, suggesting that these correlates of sensorial learning processes are sensitive to brain overgrowth during the first year of life.

Solid Pseudo-Papillary Tumor Mimicking as Complicated Pseudocyst: Multimodality Imaging and Pathological Correlation.

Pancreatic neoplasm is very rare in the pediatric population. Malignant tumors represent less than 0.2% of pediatric cancer-related mortality. Pancreas lesions can be from exocrine or endocrine origin or present themselves as cystic masses. Clinical, biological, and radiological findings usually are sufficient to establish diagnosis, but in some cases, they may be misleading. We present the case of a young patient presenting a pancreatic mass where anatomical and metabolic characteristics of the lesion were discordant to the final diagnosis.

Computer-aided lateral ventricular and brain volume measurements in 3D ultrasound for assessing growth trajectories in newborns and neonates.

For newborns and neonates, ultrasound (US) is the most common imaging modality used for examinations due to its accessibility and ease of use. However, precise volume measurements remain limited in 2D, while MRI in newborns is typically avoided because of immobilization issues which may require sedation. The objective of this study is to assess and validate the lateral ventricular and total brain volumes obtained with an automatic segmentation method using cerebral trans-fontanelle 3D US. Infants aged between 2 and 8.5 months old were recruited, with both MRI and 3D US acquired on the same day was used to validate ventricular and brain volume measurements in comparison to MRI. Lateral ventricles were segmented on both the US (manually and with a proposed automatic fusion-based approach) and MRI, while brain volumes were estimated with an automatic segmentation method. Volumetric 3D US measurements were then evaluated with respect to age distribution. For the comparison between MRI and 3D US, strong inter-class correlations (ICC) were found for the ventricle volumes (manual: 5.9% ± 2.5% difference (ICC = 0.99); automatic: 6.0% ± 2.6% difference (ICC = 0.98)), as well as the total brain size, with a 3.0% ± 1.3% difference (ICC = 0.98). There was no statistically significant difference based on t-test and f-test for the lateral ventricles volume (t-test: p = 0.542) and (f-test: p = 0.738) and for the total brain volume (t-test: p = 0.412) and (f-test: p = 0.685) between MRI and 3D US. This study demonstrates that 3D US can be used to automatically assess lateral ventricular and total brain volumes with no significant difference to the MRI acquisitions. The highest correlations were obtained for infants under 8 months when the fontanelle is open.

Predictive factors at birth of the severity of gastroschisis.

AIM: To establish children born with gastroschisis (GS). METHODS: We performed a retrospective study covering the period from January 2000 to December 2007. The following variables were analyzed for each child: Weight, sex, apgar, perforations, atresia, volvulus, bowel lenght, subjective description of perivisceritis, duration of parenteral nutrition, first nasogastric milk feeding, total milk feeding, necrotizing enterocolitis, average period of hospitalization and mortality. For statistical analysis, descriptive data are reported as mean ± standard deviation and median (range). The non parametric test of Mann-Whitney was used. The threshold for statistical significance was P < 0.05 (Two-Tailed). RESULTS: Sixty-eight cases of GS were studied. We found nine cases of perforations, eight of volvulus, 12 of atresia and 49 children with subjective description of perivisceritis (72%). The mortality rate was 12% (eight deaths). Average duration of total parenteral nutrition was 56.7 d (8-950; median: 22), with five cases of necrotizing enterocolitis. Average length of hospitalization for 60 of our patients was 54.7 d (2-370; median: 25.5). The presence of intestinal atresia was the only factor correlated with prolonged parenteral nutrition, delayed total oral milk feeding and longer hospitalization. CONCLUSION: In our study, intestinal atresia was our predictive factor of the severity of GS.