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Introduction: Several studies demonstrate the relationship between preterm birth and a reduced thalamus volume at term-equivalent age. In contrast, this study aims to investigate the link between the thalamic growth trajectory during the early postnatal period and neurodevelopment at two years of age. Methods: Thalamic volume was extracted from 84 early MRI scans at postmenstrual age of 32.33 (± 2.63) weeks and 93 term-equivalent age MRI scans at postmenstrual age of 42.05 (± 3.33) weeks of 116 very preterm infants (56% male) with gestational age at birth of 29.32 (± 2.28) weeks and a birth weight of 1158.92 (± 348.59) grams. Cognitive, motor, and language outcomes at two years of age were assessed with Bayley Scales of Infant and Toddler Development Third Edition. Bivariate analysis was used to describe the clinical variables according to neurodevelopmental outcomes and multilevel linear regression models were used to examine the impact of these variables on thalamic volume and its relationship with neurodevelopmental outcomes. Results: The results suggest an association between severe brain injury and thalamic growth trajectory (ß coef = -0.611; p < 0.001). Moreover, thalamic growth trajectory during early postnatal life was associated with the three subscale scores of the neurodevelopmental assessment (cognitive: ß coef = 6.297; p = 0.004; motor: ß coef = 7.283; p = 0.001; language: ß coeficient = 9.053; p = 0.002). Discussion: These findings highlight (i) the impact of severe brain injury on thalamic growth trajectory during early extrauterine life after preterm birth and (ii) the relationship of thalamic growth trajectory with cognitive, motor, and language outcomes.

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The purpose of this study is to define the impact of early brain growth trajectory in very low birth weight infants (VLBWI) on neurological prognosis at 2 years, assessed using sequential ultrasound (US) scans. This is a prospective cohort study with consecutive inclusion of VLBWI ≤ 32 weeks gestational age and ≤ 1500 g at birth. Total brain volume (TBV) was assessed using sequential 3D-US from birth to discharge. Prognosis at 2 years (corrected age) was assessed using the Bayley Scales of Infant and Toddler Development Third Edition. TBV showed slower growth with postmenstrual age (PMA) in those VLBWI who had an adverse cognitive prognosis compared to those with good cognitive prognosis (mean difference in TBV between prognostic groups from 4.56 cm3 at 28 weeks to 42.58 cm3 at 43 weeks) as well as in those with adverse language prognosis (mean difference in TBV from 2.21 cm3 at 28 weeks to 26.98 cm3 at 43 weeks) although other variables showed more impact than TBV on language prognosis (gestational age at birth, brain injury at term, and socioeconomic status). No association was found between TBV and motor prognosis. Brain growth rate was also significantly higher in those VLBWI who presented good cognitive scores (18.78 + (0.33 × (PMA-33)) cm3/week) compared to those with adverse cognitive outcome (13.73 + (0.64 × (PMA-33)) cm3/week).  Conclusion: Early altered brain growth is associated with poor cognitive prognosis at 2 years of age. Using sequential US monitoring, we can detect early brain growth deviation in patients who will have adverse cognitive outcomes. What is known: • The prediction of neurodevelopmental outcome of VLBWI is mostly based on the presence of brain injury in US and structural magnetic resonance imaging (MRI) at term. • Some studies have related brain volume measured on MRI at term with neurodevelopment outcome. What is new: • VLBWI with adverse cognitive prognosis at two years of age present smaller brain volumes detectable by sequential US during NICU admission. • Brain volume can be estimated from 2D and 3D US and has prognostic value in VLBWI.

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BACKGROUND: Preterm infants develop smaller brain volumes compared to term newborns. Our aim is to study early brain growth related to perinatal factors in very low birth weight infants (VLBWI). METHODS: Manual segmentation of total brain volume (TBV) was performed in weekly 3D-ultrasonographies in our cohort of VLBWI. We studied the brain growth pattern related to term magnetic resonance image (term-MRI). RESULTS: We found different brain growth trajectories, with smaller brain volumes and a decrease in brain growth rate in those VLBWI who would later have an abnormal term-MRI (mean TBV 190.68 vs. 213.9 cm3; P = 0.0001 and mean TBV growth rate 14.35 (±1.27) vs. 16.94 (±2.29) cm3/week; P = 0.0001). TBV in those with normal term-MRI was related to gestational age (GA), being small for gestational age (SGA), sex, and duration of parenteral nutrition (TPN) while in those with abnormal term-MRI findings it was related to GA, SGA, TPN, and comorbidities. We found a deceleration in brain growth rate in those with ≥3 comorbidities. CONCLUSIONS: An altered brain growth pattern in VLBWI who subsequently present worst scores on term-MRI is related to GA, being SGA and comorbidities. Early ultrasonographic monitoring of TBV could be useful to detect deviated patterns of brain growth. IMPACT STATEMENT: We describe the brain growth pattern in very low birth weight infants during their first postnatal weeks. Brain growth may be affected in the presence of certain perinatal factors and comorbidities, conditioning a deviation of the normal growth pattern. The serial ultrasound follow-up of these at-risk patients allows identifying these brain growth patterns early, which offers a window of opportunity for implementing earlier interventions.

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BACKGROUND: New ultrasound measurements to diagnose diaphragmatic dysfunction, including diaphragmatic shortening fraction (DSF), have been studied in adults and children, but there are no data on reference values for neonates. OBJECTIVE: To describe DSF reference values for term neonate (TN) and preterm neonate (PTN), and to calculate its reproducibility. METHODS: We included asymptomatic TN and PTN during their first 24 hours of life. We measured DSF at the zone of apposition in both hemithoraces. Reproducibility of image acquisition, including inter- and intra-rater agreement of the measurements were calculated among an experienced and a novel operator (after completion of a 1-day course on lung ultrasound [LU] and performance of 10 diaphragm ultrasounds [DUs] under supervision), and a more-trained examiner (completion of a 1-day course on LU and performance of 60 DUs under supervision). RESULTS: Two groups of 33 TN and 33 PTN were studied. Median DSF values did not differ between the groups, although diaphragm thickness was higher in the TN group. Intra-observer reproducibility: the intraclass correlation coefficient (ICC) was 0.95 (95% confidence interval [95% CI] 0.86-0.98). Interobserver reproducibility with novel operator had an ICC of 0.42 (95% CI -0.74 to 0.81), and with a more experienced operator improved to 0.76 (95% CI 0.27-0.92). Both intra- and interobserver agreement were high. CONCLUSIONS: Asymptomatic TN and PTN have similar DSF values in the first 24 hours of life. The intra- and interobserver agreement is high. Reproducibility is acceptable, but intensive training is necessary to perform adequate DU.

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Desde su aparición, las técnicas percutáneas han ido sustituyendo la cirugía convencional como tratamiento de primera línea en los defectos septales interauriculares. Los dispositivos Amplatzer fueron los primeros aprobados por la Food and Drug Administration, y su colocación se ha convertido en un procedimiento habitual en cardiología pediátrica. La aparición de endocarditis bacteriana sobre este tipo de dispositivos es muy infrecuente. Se presenta el caso de una endocarditis bacteriana en un paciente pediátrico portador de un dispositivo Amplatzer, que se manejó de forma conservadora con antibioterapia intravenosa, con evolución satisfactoria.

Since their introduction, percutaneous techniques have been replacing conventional surgery as a first-line treatment for septal defects. Amplatzer devices were the first to be approved by the Food and Drug Administration, and their placement has become a standard procedure in pediatric cardiology. Bacterial endocarditis of intracardiac devices such as the Amplatzer septal occluder is very infrequent. We report a case of bacterial endocarditis in a pediatric patient with an Amplatzer device, who developed an infectious endocarditis six years after its placement and received conservative management with intravenous antibiotics, with satisfactory evolution.

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Since their introduction, percutaneous techniques have been replacing conventional surgery as a first-line treatment for septal defects. Amplatzer devices were the first to be approved by the Food and Drug Administration, and their placement has become a standard procedure in pediatric cardiology. Bacterial endocarditis of intracardiac devices such as the Amplatzer septal occluder is very infrequent. We report a case of bacterial endocarditis in a pediatric patient with an Amplatzer device, who developed an infectious endocarditis six years after its placement and received conservative management with intravenous antibiotics, with satisfactory evolution.

Desde su aparición, las técnicas percutáneas han ido sustituyendo la cirugía convencional como tratamiento de primera línea en los defectos septales interauriculares. Los dispositivos Amplatzer fueron los primeros aprobados por la Food and Drug Administration, y su colocación se ha convertido en un procedimiento habitual en cardiología pediátrica. La aparición de endocarditis bacteriana sobre este tipo de dispositivos es muy infrecuente. Se presenta el caso de una endocarditis bacteriana en un paciente pediátrico portador de un dispositivo Amplatzer, que se manejó de forma conservadora con antibioterapia intravenosa, con evolución satisfactoria.

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Listeriosis is a sporadic infectious disease, which affects high-risk populations, such as the elderly, pregnant women, newborns, and immunocompromised patients. During pregnancy, listeriosis usually presents like a mild non-specific infection, but it may be responsible for fetal loss, preterm labor, early onset neonatal sepsis, and neonatal death. We report the case of a late stillbirth secondary to maternal chorioamnionitis. Listeria monocytogenes was isolated from the amniotic fluid and the fetal pleural fluid. The fetal autopsy revealed a disseminated inflammatory response with multi-organ involvement. This case illustrates the importance of the prevention and the diagnosis of listeriosis during gestation and may help us to understand the physiopathology of fetal loss due to listeriosis.

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We describe our experience with helmet-delivered continuous positive airway pressure in five preterm newborns. We analyzed oxygen requirement, arterial oxygen saturation, respiratory rate, medium arterial pressure, heart rate, apneic spells and patient's comfort. The patients' vital signs or pain scale were not different before and after treatment.

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Listeriosis is a sporadic infectious disease, which affects high-risk populations, such as the elderly, pregnant women, newborns, and immunocompromised patients. During pregnancy, listeriosis usually presents like a mild non-specific infection, but it may be responsible for fetal loss, preterm labor, early onset neonatal sepsis, and neonatal death. We report the case of a late stillbirth secondary to maternal chorioamnionitis. Listeria monocytogenes was isolated from the amniotic fluid and the fetal pleural fluid. The fetal autopsy revealed a disseminated inflammatory response with multi-organ involvement. This case illustrates the importance of the prevention and the diagnosis of listeriosis during gestation and may help us to understand the physiopathology of fetal loss due to listeriosis.

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Germinal matrix hemorrhage-intraventricular hemorrhage (GMH-IVH) remains a serious complication in the preterm newborn. The significant increase of survival rates in extremelye preterm newborns has also contributed to increase the absolute number of patients developing GMH-IVH. However, there are relatively few available animal models to understand the underlying mechanisms and peripheral markers or prognostic tools. In order to further characterize central complications and evolution of GMH-IVH, we injected collagenase intraventricularly to P7 CD1 mice and assessed them in the short (P14) and the long term (P70). Early complications at P14 included ventricle enlargement, increased bleeding, and inflammation. These alterations were maintained at P70, when increased tau phosphorylation and decreased neurogenesis were also observed, resulting in impaired learning and memory in these early adult mice. We additionally analyzed peripheral blood biomarkers in both our mouse model and preterm newborns with GMH-IVH. While MMP9 levels were not significantly altered in mice or newborns, reduced gelsolin levels and increased ubiquitin carboxy-terminal hydrolase L1 and tau levels were detected in GMH-IVH patients at birth. A similar profile was observed in our mouse model after hemorrhage. Interestingly, early changes in gelsolin and carboxy-terminal hydrolase L1 levels significantly correlated with the hemorrhage grade in newborns. Altogether, our data support the utility of this animal model to reproduce the central complications and peripheral changes observed in the clinic, and support the consideration of gelsolin, carboxy-terminal hydrolase L1, and tau as feasible biomarkers to predict the development of GMH-IVH.

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PURPOSE: Preterm small-for-gestational-age (SGA) infants are at risk for a high mortality rate and impaired cognitive development. Only a few studies have focused on amplitude-integrated EEG (aEEG) in preterm SGA infants. They have been shown to have a slower rate of brain maturation, but these findings have not consistently been related to neurodevelopmental outcomes. The aim of our study was to evaluate early aEEG monitoring in SGA compared with adequate-for-gestational-age preterms. METHODS: This prospective cohort study enrolled infants with very low birth weight who were admitted to the neonatal intensive care unit at Hospital Puerta del Mar, Cádiz, Spain, from June 2009 to September 2012. This study was a subanalysis of SGA from the global cohort previously described by our group. Adverse outcome included severe intraventricular hemorrhage and/or death. Cerebral function was monitored using aEEG recordings during the first 72 hours of life. RESULTS: Preterm SGA infants (18 SGA in the global cohort of 92 patients) had lower 1- and 5-minute Apgar scores, higher score for neonatal acute physiology perinatal extension II scores, and higher proportion of adverse outcomes. When comparing preterm adequate-for-gestational-age infants with SGA infants with good prognosis, those with SGA had more mature and continuous aEEG patterns. Low margin amplitude depression was not as severe in these patients, and a higher proportion of these patients developed sleep-wake cycles. CONCLUSIONS: The results of our study suggest that SGA infants with a good prognosis have a more mature aEEG pattern than preterm adequate-for-gestational-age patients with the same outcome. These findings support the brain sparing theory in SGA infants.

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Pulmonary sequestration is an unusual cause of heart failure in infants. We report a preterm newborn with signs of congestive heart failure supposed secondary to a ductus arteriosus that was finally diagnosed as a coexistent extralobar pulmonary sequestration. In this case, Doppler echocardiography was essential for diagnosis, revealing an abnormal systemic arterial supply to the sequestered lung and abnormal venous drainage.

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Chest pain is the second most common reason for referral to a pediatric cardiologist, because cardiovascular-related disorders are a major concern for children and their families when seeking medical attention. On the rare occasions when pediatric chest pain is a result of severe heart disease, it is usually associated with well-known cardiovascular risk factors such as fibrillinopathies. Type 1 fibrillinopathies are heritable disorders caused by mutations in the fibrillin genes that lead to a broad spectrum of connective tissue phenotypes ranging from Marfan syndrome, at the most severe end, to patients displaying mild marfanoid features, or milder Marfan (MM). We report the case of an adolescent patient with MM and suspected acute coronary syndrome, with chest pain and electrocardiographic changes suggestive of myocardial ischemia. Despite the low risk of coronary or aortic dissection/aneurysm in MM, these possibilities should be tested. Once they are ruled out, mitral valve prolapse should be considered as the main cause of chest pain with ischemic-like changes in the inferior electrocardiogram leads. We emphasize that clinical and echocardiographic follow-up over years is warranted in the pediatric population to ensure that the aortic root does not show progressive dilatation or a tendency to dissect. Finally, genotyping is clinically indicated for early and complete diagnosis in patients with MM as well as de novo Marfan syndrome to take advantage of educational and clinical programs for young carriers of the mutation.

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Chest pain is rare and usually benign in pediatrics. Cardiac etiology is even rarer. However, it is a symptom associated with ischemic heart disease and it imposes great social alarm, even in health care workers. Therefore, it is necessary to know the most common causes of this symptom in children, as well as serious diseases that can cause it, which require prompt medical attention. We report a case of chest pain associated with ischemic electrocardiographic changes in a patient with mitral valve prolapse and MASS phenotype (mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations), we review the mitral valve prolapse and stress the importance of knowing it in the pediatric setting.

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El dolor torácico es poco frecuente y, por lo general, benigno en pediatría. La etiología cardíaca es más infrecuente aún. Sin embargo, es un motivo de consulta que se asocia a cardiopatía isquémica y crea gran alarma social, incluso en profesionales de la salud. Por tanto, es necesario conocer las causas más frecuentes de este síntoma en el niño, así como de patologías graves que lo pueden provocar y requieren tratamiento urgente. Presentamos un caso clínico de dolor torácico asociado a cambios isquémicos en el electrocardiograma en una paciente con prolapso de válvula mitral y fenotipo MASS (prolapso de válvula mitral, dilatación de raIz aórtica y alteraciones esqueléticas y cutáneas), revisamos el prolapso de válvula mitral y resaltamos la importancia de conocerlo en el ámbito pediátrico.(AU)

Chest pain is rare and usually benign in pediatrics. Cardiac etiology is even rarer. However, it is a symptom associated with ischemic heart disease and it imposes great social alarm, even in health care workers. Therefore, it is necessary to know the most common causes of this symptom in children, as well as serious diseases that can cause it, which require prompt medical attention. We report a case of chest pain associated with ischemic electrocardiographic changes in a patient with mitral valve prolapse and MASS phenotype (mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations), we review the mitral valve prolapse and stress the importance of knowing it in the pediatric setting.(AU)

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El dolor torácico es poco frecuente y, por lo general, benigno en pediatría. La etiología cardíaca es más infrecuente aún. Sin embargo, es un motivo de consulta que se asocia a cardiopatía isquémica y crea gran alarma social, incluso en profesionales de la salud. Por tanto, es necesario conocer las causas más frecuentes de este síntoma en el niño, así como de patologías graves que lo pueden provocar y requieren tratamiento urgente. Presentamos un caso clínico de dolor torácico asociado a cambios isquémicos en el electrocardiograma en una paciente con prolapso de válvula mitral y fenotipo MASS (prolapso de válvula mitral, dilatación de raÍz aórtica y alteraciones esqueléticas y cutáneas), revisamos el prolapso de válvula mitral y resaltamos la importancia de conocerlo en el ámbito pediátrico.

Chest pain is rare and usually benign in pediatrics. Cardiac etiology is even rarer. However, it is a symptom associated with ischemic heart disease and it imposes great social alarm, even in health care workers. Therefore, it is necessary to know the most common causes of this symptom in children, as well as serious diseases that can cause it, which require prompt medical attention. We report a case of chest pain associated with ischemic electrocardiographic changes in a patient with mitral valve prolapse and MASS phenotype (mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations), we review the mitral valve prolapse and stress the importance of knowing it in the pediatric setting.

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El dolor torácico es poco frecuente y, por lo general, benigno en pediatría. La etiología cardíaca es más infrecuente aún. Sin embargo, es un motivo de consulta que se asocia a cardiopatía isquémica y crea gran alarma social, incluso en profesionales de la salud. Por tanto, es necesario conocer las causas más frecuentes de este síntoma en el niño, así como de patologías graves que lo pueden provocar y requieren tratamiento urgente. Presentamos un caso clínico de dolor torácico asociado a cambios isquémicos en el electrocardiograma en una paciente con prolapso de válvula mitral y fenotipo MASS (prolapso de válvula mitral, dilatación de raIz aórtica y alteraciones esqueléticas y cutáneas), revisamos el prolapso de válvula mitral y resaltamos la importancia de conocerlo en el ámbito pediátrico.(AU)

Chest pain is rare and usually benign in pediatrics. Cardiac etiology is even rarer. However, it is a symptom associated with ischemic heart disease and it imposes great social alarm, even in health care workers. Therefore, it is necessary to know the most common causes of this symptom in children, as well as serious diseases that can cause it, which require prompt medical attention. We report a case of chest pain associated with ischemic electrocardiographic changes in a patient with mitral valve prolapse and MASS phenotype (mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations), we review the mitral valve prolapse and stress the importance of knowing it in the pediatric setting.(AU)

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Chest pain is rare and usually benign in pediatrics. Cardiac etiology is even rarer. However, it is a symptom associated with ischemic heart disease and it imposes great social alarm, even in health care workers. Therefore, it is necessary to know the most common causes of this symptom in children, as well as serious diseases that can cause it, which require prompt medical attention. We report a case of chest pain associated with ischemic electrocardiographic changes in a patient with mitral valve prolapse and MASS phenotype (mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations), we review the mitral valve prolapse and stress the importance of knowing it in the pediatric setting.

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