Blake's Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI : A Pictorial Review.

Purpose: The clinical variability of Blake's pouch cysts (BPC) may range from asymptomatic via ataxia to sequelae of decompensated hydrocephalus. On the other hand, Dandy-Walker malformation (DWM) and cerebellar vermis hypoplasia generally correlate with less favorable neurologic development. The aim was to illustrate the potential of prenatal and postnatal neuroimaging to distinguish a BPC or persistent BP from other posterior fossa malformations.

Delayed fenestration of Blake's pouch with or without vermian hypoplasia: fetal MRI at 3 tesla versus 1.5 tesla.

Background: Fetal magnetic resonance imaging (MRI), mainly performed at standard field strength, plays a role in the classification of posterior fossa malformations. In the context of early second-trimester screening, upward rotation of the cerebellar vermis per se is usually compatible with a more favorable outcome than Dandy-Walker malformation and profound vermian hypoplasia. Delayed fenestration of Blake's pouch may either mimic vermian hypoplasia by compression or be associated with it in individual cases.

Like father, like son. Physical Activity, Dietary Intake, and Media Consumption in Pre-School-Aged Children.

An imbalance between energy input and energy needs contributes to the growing incidence of overweight children. Pre-schoolers normally like to move, but even at this young age, they are already affected by a lack of physical activity and a high amount of screen time. Media consumption contributes to unhealthy diets and extends the length of time spent sitting.

A novel therapeutic approach for LPIN1 mutation-associated rhabdomyolysis--The Austrian experience.

Introduction: Lipin 1 gene (LPIN1) mutations lead to cellular energy deficiency and cause up to 50% of the rhabdomyolysis episodes seen in pediatric patients. These episodes are associated with poor prognosis, as treatment options have been limited. We propose a novel therapeutic strategy based on prevention and early treatment of catabolism.

The burden of pneumococcal meningitis in Austrian children between 2001 and 2008.

Unlabelled: The present study was conducted to evaluate the burden of pneumococcal meningitis in Austrian children between 2001 and 2008. Clinical outcome was retrospectively analyzed both on discharge and on follow-up investigations. This study was based on a prospective multicentre surveillance study on hospitalized invasive pneumococcal infections in Austrian children with a total annual "study population" of about 399,000 children aged below 5 years per year.

Is it possible to make a reliable prognosis within the first hour of life for very low birth weight infants delivered after preterm premature rupture of membranes?

Background: One third of all preterm births are due to preterm premature rupture of membranes (pPROM). An accurate prognostic evaluation after admission to the neonatal intensive care unit is necessary.

Objective: The aim of this study was to identify prognostic factors within the first hour of life for mortality, short-term pulmonary morbidity, chronic lung disease (CLD) and severe cerebral morbidity in very low birth weight (VLBW) infants after pPROM.

Perinatal outcome of preterm infants <1500 g after IVF pregnancies compared with natural conception.

Objective: In vitro fertilisation (IVF) pregnancies are at increased risk for adverse perinatal outcome including very low birth weight infants. The purpose of this study was to find out whether the perinatal outcome of preterm infants <1500 g after IVF is different from those in naturally conceived pregnancies.

Patients And Methods: This retrospective cohort study included preterm infants <1500 g born between 1999 and 2007 in a tertiary perinatal referral centre.

Uncommon manifestations of neuroborreliosis in children.

Lyme borreliosis is a tick-borne spirochetal infection which affects the skin, joints, heart and nervous system. Children with a neuroborreliosis usually present with a facial nerve palsy or aseptic meningitis, but the spectrum also includes other rare manifestations. We report four unusual cases of childhood neuroborreliosis and show that seizures with regional leptomeningeal enhancement, acute transverse myelitis, meningoradiculitis with pain and paraesthesia and cranial nerve palsies other than facial nerve palsy can be the leading symptoms of children with neuroborreliosis.

The impact of risk factors and more stringent diagnostic criteria of gestational diabetes on outcomes in central European women.

Objectives: In the face of the ongoing discussion on the criteria for the diagnosis of gestational diabetes (GDM), we aimed to examine whether the criteria of the Fourth International Workshop Conference of GDM (WC) select women and children at risk better than the World Health Organization (WHO) criteria.

Design And Setting: This was a prospective longitudinal open study in five tertiary care centers in Austria.

Patients And Outcome Measures: The impact of risk factors, different thresholds (WC vs.

Preterm birth and disruptive cerebellar development: assessment of perinatal risk factors.

Objective: Abnormal cerebellar development was recently recognized to be related to prematurity. Aim of the present study was to evaluate preterm birth and possible peri- and postnatal risk factors associated with this type of brain injury.

Patients And Methods: We report on a series of 35 very low birth weight infants (birth weight 986+/-257g S.

Disrupted cerebellar development in preterm infants is associated with impaired neurodevelopmental outcome.

The unfavorable impact of prematurity on the developing cerebellum was recently recognized, but the outcome after impaired cerebellar development as a prematurity-related complication is hitherto not adequately documented. Therefore we compared 31 preterm patients with disrupted cerebellar development to a control group of 31 gender and gestational age matched premature infants with normal cerebellar development. Supratentorial brain injuries during the neonatal period were comparable between the groups.

Disruption of cerebellar development: potential complication of extreme prematurity.

Background And Purpose: Infants with very low birth weight are at high risk for cerebral lesions. Although supratentorial brain damage is a common radiologic finding, posterior fossa pathologies are rare. We studied the morphology of cerebellar involvement in a large series of 28 premature infants born before the 30th week of gestation to define typical patterns and identify possible risk factors for this pathology.

Is the use of early nasal CPAP associated with lower rates of chronic lung disease and retinopathy of prematurity? Nine years of experience with the Vermont Oxford Neonatal Network.

Objective: The neonatal regional tertiary care center of the University of Vienna (VC) has been a member of the Vermont Oxford Neonatal Network (VONN) since 1994. During the period 1994--2002, important differences between the VC and the VONN in both pre- and postnatal management and in late morbidities such as chronic lung disease (CLD) and severe retinopathy of prematurity (ROP) were observed. We hypothesize that stabilization of very-low-birth-weight (VLBW) infants on nasal continuous positive airway pressure (NCPAP) immediately after birth, combined with a restrictive use of artificial ventilation, might be responsible for lower rates of CLD and ROP.

[Gestational diabetes mellitus].

Gestational diabetes (GDM) is defined as any degree of glucose intolerance with onset or first recognition during pregnancy and is associated with increased feto-maternal morbidity as well as long-term complications in mothers and offspring. GDM is diagnosed by an oral glucose tolerance test (OGTT) or fasting glucose concentrations in the diabetic range. In case of a high risk for GDM/type 2 diabetes (history of GDM or prediabetes [impaired fasting glucose or impaired glucose tolerance]; malformation, stillbirth, successive abortions or birth-weight > 4500 g in previous pregnancies) performance of the OGTT (120 min; 75 g glucose) is recommended already in the first trimester and--if normal--the OGTT should be repeated in the second/third trimester.

The clinical impact of magnetic resonance imaging in fetuses with central nervous system anomalies on ultrasound scan.

Objectives: Magnetic resonance imaging (MRI) was performed on fetuses with sonographically diagnosed central nervous system (CNS) anomalies to determine frequency and type of cases in which fetal MRI adds clinically relevant information.

Patients And Methods: Forty-three cases presenting with CNS anomalies by ultrasonography were investigated by MRI. The potential effect of MRI on parental counseling and pre- or postnatal management were the main endpoints.

Preterm male infants need more initial respiratory support than female infants.

Unlabelled: Female infants have a significantly greater chance of surviving than male infants at similar birthweights and gestational ages, and have an advantage over males for a better outcome with less morbidity.

Conclusion: The exact mechanisms responsible for the gender differences, with a better chance of survival of female infants, remain to be determined. The paper by Elsmén et al.

Brain abscesses after Serratia marcescens infection on a neonatal intensive care unit: differences on serial imaging.

Serratia are known to be a possible cause of severe cerebral infections in neonates. We describe imaging of three premature infants infected with Serratia marcescens. Born in the 31( st), 25( th) and 28( th) weeks of gestation, they presented with signs of septicaemia on postnatal days 9, 24 and 32.

Infantile sialic acid storage disease and protein-losing gastroenteropathy.

We report on a boy who presented at birth with gastroschisis and thereafter developed the characteristic clinical symptoms of infantile sialic acid storage disease within the first two months of life. Measurements of free sialic acid excretion (tenfold increase) in the urine and a 15-fold elevation of free sialic acid in cultured fibroblasts proved the diagnosis. The clinical course was complicated by hypertrophic cardiomyopathy, recurrent infections, hypothyroidism, and intestinal protein losses, which had never been described before in an infantile sialic acid storage disease patient.

Diagnosis and prevention of neural tube defects.

Purpose Of Review: Neural tube defects, including anencephaly, meningomyelocele and encephalocele, are among the most common birth defects that result in severe mortality and morbidity. Neural tube defects occur with an incidence of 1-5 per 1000 births, showing marked geographic, ethnic and temporal variations. Although clear evidence exists on preventability of a large proportion of neural tube defects by periconceptional folic acid intake, only a low percentage of women are following the recommendations.

MHC gene related effects on microglia and macrophages in experimental autoimmune encephalomyelitis determine the extent of axonal injury.

Myelin-oligodendrocyte-glycoprotein (MOG)-induced experimental autoimmune encephalomyelitis (EAE) in rats is a chronic inflammatory demyelinating disease of the central nervous system (CNS) strongly mimicking multiple sclerosis (MS). We determined the involvement of macrophages and microglia in the lesions of MOG-EAE in relation to different major histocompatibility complex (MHC, RT1 in rat) haplotypes. We used intra-RT1 recombinant rat strains with recombinations between the RT1a and RT1u haplotypes on the disease permissive LEW non-MHC genome.

Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: a hereditary compound structure in duodenal atresia?

Duodenal atresia predominantly represents an isolated entity, but could also be part of a complexly structured intestinal malformation. We report four children, including two from one family, with duodeno-jejunal atresia associated with malrotation, volvulus, and absent parietal attachment of the mesentery. Gross absence of the mesentery and absence of distal parts of the superior mesenteric artery were the most remarkable findings.

Sleep-wake cycles in preterm infants below 30 weeks of gestational age. Preliminary results of a prospective amplitude-integrated EEG study.

In the newborn, presence of sleep-wake cycles indicates integrity and maturity of the central nervous system. By spectral EEG analysis and polygraphic recordings subtle variations of EEG background activity and behavioural patterns corresponding to early sleep-wake cycles have been found in preterm infants as young as 27 weeks of gestation. The emergence of sleep-wake cycles at early gestational ages may have a positive predictive value for long-term neurological outcome.