Congenital hydrocephaly

The results of neuropsychological investigation of 808 children and adolescents from the age of 7 to 18, out of whom 689--patients with local damages of brain (tumors, arachnoidal cysts, vessel pathology, congenital hydrocephaly), 119--health ones were analyzed. All patients, healthy and ailing were divided into four age groups: preschoolers 7-9 years old, preteenagers 10-12 years old, teenagers 13-15 years old, and adolescents 16-18 years old. Were detected the disorders of cognitive functions and there dependence on the character of pathologic processes of the brain and age...

Ultrasound has an important role in the detection and follow- up of intrauterine infection. Viral infections are a major cause of fetal morbidity and mortality. Transplacental transmission of the virus, even in sub-clinical maternal infection, may result in a severe congenital syndrome. Prenatal detection of viral infection is based on fetal sonographic findings and PCR to identify the specific infectious agent. Most affected fetuses appear sonographically normal, but serial scanning may reveal evolving findings...

BACKGROUND: The objective of this study was to assess, in a large data set from Swedish Medical Health Registries, whether maternal obesity and maternal morbid obesity were associated with an increased risk for various structural birth defects. METHODS: The study population consisted of 1,049,582 infants born in Sweden from January 1, 1995, through December 31, 2007, with known maternal weight and height data. Women were grouped in six categories of body mass index (BMI) according to World Health Organization classification...

We report on a 6-month-old boy with craniosynostosis, pseudohypoparathyroidism type 1a (PHP1A), and a GNAS gene mutation. He had synostoses of the coronal, frontal, and sagittal sutures, brachyturricephaly, and hydrocephaly. He also had congenital hypothyroidism, round face, full cheeks, shortness of limbs, mild developmental delay, and muscular hypotonia. Because of progressive hydrocephaly, the synostosis was corrected surgically but circulatory decompensation led to disseminated intravascular coagulation and cerebral infarctions...

OBJECTIVES: To describe surgical experience in the repair of anterior skull base defects, we studied patients with congenital or acquired defects. According to a multidisciplinary evaluation, all subjects underwent surgery performed by an ENT surgeon in order to avoid craniotomy. DESIGN: Retrospective study of patients who were treated for an anterior skull base defect between 2000 and 2005 at a tertiary referral centre. MATERIALS AND METHODS: Twelve patients had surgery to correct anterior skull base defects...

Currently available data on the relationship between the prevalence of isolated congenital malformations and parental age are inconsistent and frequently divergent. We utilised the data from the Polish Registry of Congenital Malformations (PRCM) to accurately assess the interplay between maternal and paternal age in the risk of isolated non-syndromic congenital malformations. Out of 902 452 livebirths we studied 8683 children aged 0-2 years registered in the PRCM. Logistic regression was used to simultaneously adjust the risk estimates for maternal and paternal age...

BACKGROUND: Although thyroid disorders are present in approximately 3% of pregnant women, little is known about the association between maternal thyroid disease and birth defects. METHODS: We assessed the association between maternal thyroid disease, thyroid medication use, and 38 types of birth defects among 14,067 cases and 5875 controls in the National Birth Defects Prevention Study, a multisite, population-based, case-control study. Infants in this study were born between October 1997 and December 2004...

CONTEXT: Evidence suggests an association between maternal obesity and some congenital anomalies. OBJECTIVE: To assess current evidence of the association between maternal overweight, maternal obesity, and congenital anomaly. DATA SOURCES: MEDLINE, EMBASE, CINAHL, and Scopus (January 1966 through May 2008) were searched for English-language studies using a list of keywords. Reference lists from relevant review articles were also searched. STUDY SELECTION: Observational studies with an estimate of prepregnancy or early pregnancy weight or body mass index (BMI) and data on congenital anomalies were considered...

Infections acquired in utero or during the birth process are a significant cause of fetal and neonatal mortality and an important contributor to early and later childhood morbidity. Advances in ultrasound, invasive prenatal procedures and molecular diagnostics have allowed in utero evaluation and given rise to more timely and accurate diagnosis in infected fetuses. Transplacental transmission of the infectious agent, even in subclinical maternal infection, may result in a severe congenital syndrome. Prenatal detection of infection is based on fetal sonographic findings and polymerase chain reaction to identify the specific agent...

INTRODUCTION: The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. OBJECTIVES: First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. MATERIALS AND METHOD: Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999...

OBJECTIVE: To compare ultrasound (US) and fetal autopsy findings in 2(nd) trimester termination of pregnancy because of structural fetal anomalies. METHODS: A total of 112 terminations of pregnancy (TOP) between 1999-2003 were reviewed retrospectively. The cases originated from a secondary and a tertiary Fetal Medicine unit in the south Stockholm area, using a common specialized perinatal pathology service. Karyotype was not known at the time of US examination. The findings were compared and classified into four groups according to the degree of agreement between US and autopsy...

BACKGROUND: The presence of a congenital malformation at birth is a cause of anxiety at an otherwise joyous occasion. Congenital malformations are a significant contributor to perinatal mortality. STUDY DESIGN: A retrospective study of external congenital abnormalities in singleton and twin births in rural eastern Nigeria over a 20 year period. RESULT: The incidence of congenital defects for all deliveries was 110.8/10,000 births. Of 1453 twins and 32206 singleton births, there were 58 and 315 congenital abnormalities, with incidence of 97...

OBJECTIVE: To investigate the technique of skull reconstruction for the congenital craniosynostosis. METHODS: Many technique of skull plasty were adopted to correct the congenital craniosynostosis. We advanced fronto-orbital, reversal change of the frontal bone to correct the brachycephalic, hydrocephaly acrocephaly, which were supported by parietal bone. Plum-plasty of the calvaria was performed for sagittal synostosis; Fronto-orbital plasty correct the trigonocephaly; for single coronal synostosis, which result to frontal plagiocephaly,we reversed both frontal sides, advanced orbital strip and tilted the invalid side ...

We report a male term newborn presenting with a congenital macrocephaly 3.5 standard deviations above the median, with a wide and tense anterior fontanel, splayed calvarial sutures, and muscular hypotonia. Antenatal head circumferences were repeatedly below the median. A postnatal head ultrasound showed a large right intracerebral mass with right lateral ventricle compression, right temporal horn dilation, and right frontal horn enlargement with lateral displacement. Additional imaging by computed tomography scan and magnetic resonance imaging was performed...

Three cases of congenital immature intracranial teratomas were examined by autopsy. The fetuses were diagnosed prenatally as macrocephalic and hydrocephalic due to skull-occupying lesions. These fetuses were born alive at 32, 31, and 36 weeks gestation, respectively, via Cesarean section. Intracranial teratoma in the first case was diagnosed by MRI and in the second and third cases by ultrasound. All tumors were deemed to be unresectable. Congenital CNS teratoma should be considered in the differential diagnosis of fetuses diagnosed with macrocephaly or hydrocephaly...

AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT)...

OBJECTIVE: To study the levels of prevalence and to describe the epidemiological features of birth defects in high-prevalence areas in China. METHODS: A population-based retrospective study was conducted in two counties of Shanxi province that including birth defects among fetuses and live births born after 20 weeks' gestational age in the study areas from 2002 through 2004. RESULTS: During 2002 - 2004, the prevalence of birth defects was 844...

BACKGROUND: It has been hypothesised that cerebral palsy (CP) and other congenital anomalies are attributable to feto-fetal transfusion problems in a monochorionic multiple gestation. Thus more than one organ could be compromised leading to the coexistence of two or more anomalies in a fetus. Such anomalies in a singleton birth may be attributable to early demise of the co-conceptus as a vanishing twin. AIM: To determine whether the coexistence of congenital anomalies and CP is greater than a chance finding by comparing the prevalence of congenital anomalies in children with CP with that in the general population of children...

Congenital infections are among the most pressing health care problems. Congenital infections are not reason of congenital malformation and perinatal mortality only, but also pathologies that can be revealed during first year of life. Frequency for congenital viral infection displayed from birth varies between 23% and 92%. The aim of the study was the investigation of inherent infection consequences (citomegaloviral infection, herpes infection and chlamidia) in children in different age groups. Under our observation were 81 children with congenital infections...

Walker Warburg syndrome (WWS) is the most severe of a group of multiple congenital disorders known as lissencephaly type II ( LIS Type II) associated with congenital muscular dystrophy and eye abnormalities. The POMT1 gene is the most frequently affected found in 20% of patients with WWS. We describe five fetuses with WWS in three non-related families carrying a same mutation in the POMT1 gene. All fetuses presented with tetra ventricular hydrocephaly, and arachnoidal neuroglial ectopia and cortical dysplasia characteristic of LIS type II...