1/122. brain dysgenesis in Cornelia de lange syndrome.The neuropathological findings in a neonatal case of Cornelia de lange syndrome (CDLS) were described. Two different types of lesions were revealed in the affected brain. The first type was classified as perinatal hypoxic-ischemic brain damage, associated with cyanotic congenital heart anomalies: subarachnoideal, intraventricular, and parenchymal hemorrhage, and multiple necrosis in the cerebral white matter, basal ganglia, internal capsule, thalamus, mammillary bodies and dentate nucleus. This type may be non-specific and common in premature babies dying soon after birth. On the other hand, the second type was classified as congenital dysgenesis of the brain: microbrachycephaly, immature or simple convolution pattern of the cerebral gyri, thickened leptomeninges, persistent subpial granule cells, hypoplasia of the anterior thalamic nuclei, neurohypophysis, lateral geniculate body, cerebral peduncle, ventral pons and cerebellar internal granular layer, and heterotopic cell nests in the cerebellar white matter. This type may indicate that the maturation of the brain can be disturbed in the fetal period, particularly in the mid-gestational period. In conclusion, pathognomonic or specific changes of CDLS might be absent in the brain. However, congenital dysgenesis of the brain, especially that found in the diencephalon and the cortico-ponto-cerebellar system, may constitute morphologic evidence explaining the severe growth retardation and neurological abnormalities in CDLS.- - - - - - - - - - ranking = 1keywords = cerebral, parenchymal, brain (Clic here for more details about this article) |
2/122. Disseminated intravascular meconium in a newborn with meconium peritonitis.A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.- - - - - - - - - - ranking = 0.31020641260416keywords = cerebral, brain (Clic here for more details about this article) |
3/122. Case of the month: January 1999--fetus with echogenic mass in third ventricle.A 29-week gestational age newborn male infant was found to have an echogenic mass in the 3rd ventricle by prenatal ultrasound 2 weeks prior to delivery. At delivery he was poorly responsive and had hydrocephalus and ascites. A CT scan after birth showed cerebral infarction, amorphous tissue in the left hemisphere and numerous calcifications. Despite supportive treatment he died 4 days after birth. Postmortem examination of the brain revealed marked distortion of the architecture and a supratentorial undifferentiated neoplasm consistent with a PNET. The tumor showed extensive areas of hemorrhage and necrosis and involvement of lateral and third ventricles, brain parenchyma, and meninges.- - - - - - - - - - ranking = 0.31478925998189keywords = cerebral, brain (Clic here for more details about this article) |
4/122. Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions.Auditory neuropathy is defined as absent or severely distorted auditory brainstem responses with preserved otoacoustic emissions and cochlear microphonics. This entity can be found in various circumstances including pre-lingual children. An almost universal characteristic reported from adult patients is the ineffectiveness of traditional hearing aids. Adequate management of pre-lingual cases therefore remains an open problem. This paper describes two pre-lingual children whose follow-up data demonstrated a selective loss of the otoacoustic emissions, whereas the cochlear microphonics remained preserved. In one of the patients, hearing aid fitting as soon as she lost her otoacoustic emissions proved successful. These findings have important implications for the operational definition of the condition, since one must be prepared to encounter cases with absent otoacoustic emissions. The present data also demonstrate that conventional amplification can benefit pre-lingual auditory neuropathy cases, at least once they have lost their otoacoustic emissions.- - - - - - - - - - ranking = 0.0045828473777275keywords = brain (Clic here for more details about this article) |
5/122. Cerebral blood flow velocities in an infant with moyamoya disease.moyamoya disease is a progressive cerebrovascular disorder with bilateral occlusion of the basal circulation and development of collateral blood supply. In a 6-month-old female with multifocal ischemic infarctions, transcranial pulsed Doppler sonography revealed extremely high and low cerebral blood flow velocities, dampened waveforms, reversed flow, and musical murmurs. magnetic resonance angiography revealed different degrees of vascular stenosis and an abnormal collateral network. moyamoya disease was confirmed by conventional angiography at the age of 10.5 months. Pulsed-wave transcranial Doppler sonography is a noninvasive screening method in infants at risk of moyamoya disease.- - - - - - - - - - ranking = 0.30562356522643keywords = cerebral (Clic here for more details about this article) |
6/122. Thrombosis of the deep cerebral veins with excessive bilateral infarction in a premature infant with the thrombogenic 4G/4G genotype of the plasminogen activator inhibitor-1.We report on a preterm infant with deep cerebral venous thrombosis, a rare condition in this age group. This premature infant had a gestational age of 33 weeks and normal development until day 18, when he presented with tonic seizures and a tense fontanelle. Ultrasound and computed tomography revealed bilateral haemorrhagic infarction of the whole region drained by the deep cerebral veins, including the periventricular white matter, thalamus and choroid plexus. The child was homozygous for the 4G allele of the plasminogen activator inhibitor-1 (PAI-1) 4G/5G promoter polymorphism. CONCLUSION: In patients with bilateral cerebral infarction, thrombosis of the deep cerebral veins should be considered. In addition the role of prothrombotic risk factors, including PAI-1 4G/5G promoter polymorphism, in cerebral vein thrombosis should be clarified in a multicentre study.- - - - - - - - - - ranking = 2.7506120870379keywords = cerebral (Clic here for more details about this article) |
7/122. Anaesthetic management of liver haemorrhage during laparotomy in a premature infant with necrotizing enterocolitis.The case of a 680 g premature baby who developed massive spontaneous liver haemorrhage during laparotomy for necrotizing enterocolitis is reported. The infant survived due to rapid and massive fluid administration, including transfusion of large volumes of blood and blood products, in combination with high dose inotropic support and the surgical use of packing with thrombostatic sponges. Good venous access, including two central venous lines, turned out to be very useful.- - - - - - - - - - ranking = 12.731288017032keywords = haemorrhage (Clic here for more details about this article) |
8/122. cerebral infarction in newborns: report of two cases.Neonates with cerebral infarction do not present with specific symptoms and the condition is usually insidious, so many atypical cases are not diagnosed properly during the neonatal stage. Normal neurological examination results may be found in newborns who have actually had a cerebral infarction insidiously. We present two newborns with cerebral infarction. One had clinical symptoms of seizures. brain computed tomography showed a low-attenuated area and magnetic resonance angiography showed a decreased caliber and number of cerebral artery branches. The other had normal neurological examination results. He was referred to our hospital due to cyanosis. brain sonography revealed a focal hyperechoic area and T2 weighted magnetic resonance image showed an increased signal intensity area. The incidence, etiologies, clinical and radiographic findings are also reviewed.- - - - - - - - - - ranking = 1.2224942609057keywords = cerebral (Clic here for more details about this article) |
9/122. Combination thrombolytic and anticoagulant therapy for bilateral renal vein thrombosis in a premature infant.We report a premature infant with bilateral renal vein thrombosis and renal failure at 1 month of age who underwent treatment with recombinant tissue plasminogen activator and heparin sulfate. Combination thrombolytic and anticoagulation therapy was complicated by an intraventricular and intraparenchymal cerebral hemorrhage without resolution of the renal vein thrombosis. With increasing reports of successful use of this therapy for venous thrombosis, we suggest caution and continued study of its use in the premature infant and newborn.- - - - - - - - - - ranking = 0.36125578528077keywords = cerebral, parenchymal (Clic here for more details about this article) |
10/122. Unilateral parenchymal haemorrhagic infarction in the preterm infant.A unilateral parenchymal haemorrhage associated with a germinal matrix-intraventricular haemorrhage (GMH-IVH) is still an important problem in the preterm infant and especially in those who are very immature. This type of lesion is now considered mainly to be caused by impaired drainage of the veins in the periventricular white matter and is often referred to as a venous infarction. The risk factors and neonatal imaging findings, as well as neurodevelopmental outcome and imaging data in infancy, of this type of lesion differ from those found in children with bilateral periventricular leukomalacia. An effort should, therefore, always be made to make a distinction between these two types of lesions. In our experience it is possible to make this distinction in most cases, when performing both sequential ultrasonography as well as selective magnetic resonance imaging during the neonatal period.- - - - - - - - - - ranking = 5.3706763070845keywords = haemorrhage, parenchymal (Clic here for more details about this article) |
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