1/69. 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 = brain (Clic here for more details about this article) |
2/69. 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 = 0.16666666666667keywords = brain (Clic here for more details about this article) |
3/69. 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 = 0.33333333333333keywords = brain (Clic here for more details about this article) |
4/69. 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 = 0.16666666666667keywords = brain (Clic here for more details about this article) |
5/69. 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 = 166.68471053504keywords = haemorrhage (Clic here for more details about this article) |
6/69. 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 = 66.673884214016keywords = haemorrhage (Clic here for more details about this article) |
7/69. Continuous insulin infusion: promoting growth in low birth weight infants.Development of improved technologies in neonatal care has yielded increasing numbers of surviving low birth weight (LBW) infants who have challenged methods of supportive care. As researchers and practitioners have focused on respiratory, cardiac, and other body system requirements, nutritional support has been low on the priority list. Support for growth and maximized neurologic development, rather than simply for survival, has been the challenge in care of small and sick newborns. Use of insulin to enhance glucose tolerance in LBW infants, and thereby maximize growth, is a management modality that can be initiated early in the infant's course and is facilitated by implementation of clear and consistent policies and procedures. Continuous insulin infusions have been demonstrated to enhance glucose uptake and utilization--facilitating neonatal growth and in turn enhancing brain growth and developmental outcome.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
8/69. Spontaneous activity in premature infants affects bone strength.OBJECTIVE: Determination of bone strength of lower extremities in very low birth weight (VLBW) premature infants with central nervous system pathology resulting in reduced unilateral spontaneous leg movements. STUDY DESIGN: Quantitative ultrasound (QUS) measurements of speed of sound (SOS) of the tibiae of both legs in three VLBW premature infants with brain insult and unilateral reduced spontaneous activity. Results were compared to QUS measurements of both legs in healthy premature infants. Measurements were performed by the same investigator who was blinded to the clinical course of the participants. RESULTS: Reduced spontaneous activity of one leg due to brain pathology resulted in decreased tibial SOS in the affected side. There was no difference in bone SOS between the legs of the healthy controls. CONCLUSION: Spontaneous movements (mainly antigravity flexion and extension) are important for bone structure and mineralization in VLBW premature infants. QUS may become an important diagnostic modality for the evaluation, treatment, and follow-up of bone strength and osteopenia in this unique population.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
9/69. Screening and follow up assessment in three cases of auditory neuropathy.Three children with auditory neuropathy are described. Two were detected via a targeted neonatal hearing screening programme based on auditory brain stem response testing, and one via the routine health Visitor Distraction Test. Auditory neuropathy is an important but poorly understood disorder which has implications on planning future hearing screening policy and management of hearing impairment.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
10/69. serratia marcescens in the neonatal intensive care unit: re-emphasis of the potentially devastating sequelae.BACKGROUND: It is known that infections with serratia marcescens can take a progressive course in preterm infants and that meningoencephalitis with this pathogen exhibits an extremely bad neurologic prognosis. methods AND RESULTS: We report on five cases of septicemia with serratia marcescens in preterm infants during a nosocomial outbreak. Three patients developed meningoencephalitis with brain abscesses. Mild clinical and laboratory findings of infection contrasted with destructive findings on MRI scan. All five patients survived, those with isolated bacteremia without neurologic sequelae. CONCLUSION: When serratia marcescens is isolated from any source in a neonatal intensive care unit, preventive measures including strict hygiene and cohorting of infants must be implemented immediately since this pathogen seems to exhibit specific affinity for the central nervous system and serratia marcescens meningoencephalitis takes a progressive and destructive course despite antibiotic therapy.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
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