survival of anencephaly

OBJECTIVES: To estimate the extent to which late termination of pregnancy (gestational period > 24 weeks) is practised, to find out what fetal diseases and abnormalities lead to termination and to obtain insight into the clinical judgement and decision-making process by gynaecologists with regard to termination. DESIGN: A retrospective study. PLACE: Province of North Holland, The Netherlands. METHODS: An anonymous printed questionnaire, was sent to all associations or departments of gynaecology in the 21 hospitals in North Holland...

The purpose of this investigation was to examine the sex distribution of deaths from spina bifida in birth cohorts exposed and unexposed to severe periconceptional famine. For this purpose, we compared the risk of deaths from spina bifida between birth cohorts exposed and unexposed to the Dutch Hunger Winter of 1944-1946. In males, the relative risk of death from spina bifida was 2.62 [95% confidence interval (CI) = 1.14-6.01]. In females, the relative risk for spina bifida was 0.59 (95% CI = 0.14-2.37). The sex ratio (male:female) for deaths from spina bifida in the exposed birth cohort was 2...

OBJECTIVE: To determine the live birth prevalence of neural tube defects (NTD) in the Netherlands and describe the clinical picture. DESIGN: Descriptive. SETTING: TNO Prevention and Health, Leiden, the Netherlands. METHOD: Data collected through active surveillance of NTD on a monthly basis by paediatricians throughout the country. RESULTS: From October 1993 to September 1995, 203 infants with NTD were registered...

The paired-class homeobox-containing gene, Cart1, is expressed in forebrain mesenchyme, branchial arches, limb buds and cartilages during embryogenesis. Here, we show that Cart1-homozygous mutant mice are born alive with acrania and meroanencephaly but die soon after birth-a phenotype that strikingly resembles a corresponding human syndrome caused by a neural tube closure defect. Developmental studies suggest that Cart1 is required for forebrain mesenchyme survival and that its absence disrupts cranial neural tube morphogenesis by blocking the initiation of closure in the midbrain region that ultimately leads to the generation of lethal craniofacial defects...

OBJECTIVE: To gain insight into the extent to which late termination of pregnancy (gestational age > 24 weeks) is practised and to find out what foetal diseases and abnormalities lead to termination. DESIGN: An exploratory, descriptive, retrospective study. SETTING: Province of North Holland, the Netherlands. METHODS: An investigation, based on an anonymous printed questionnaire, was conducted among all associations/departments of gynaecologists in the 21 hospitals in North Holland...

Two infants with anencephaly survived for 7 and 10 months without the need for prolonged assisted mechanical ventilation. One infant prospectively fulfilled all four criteria of the Medical Task Force on Anencephaly, making diagnosis almost certain. Prolonged survival of anencephalic infants is important not only to provide adequate counseling, but also for its relevance to organ transplantation.

To investigate the survival of infants with neural tube defects in Western Australia from 1966 to 1990, cases of neural tube defects were ascertained from multiple sources. Survival of infants with anencephaly, spina bifida, and encephalocoele was examined separately, and four birth cohorts were compared: 1966-1972 (when most surviving infants were actively treated), 1973-1979 (a period of stringent application of selection criteria for treatment), 1980-1985 (some relaxation of selection criteria), and 1986-1990 (further relaxation of selection criteria)...

We present three probands with partial trisomies 2p21-23 due to ins(4;2)(q21;p21p23) pat, 2p23-pter due to t(2;4)(p23;q35)mat, and 2p21-pter due to t(2;11)(p21;q23.3)mat. More than 50 cases of partial trisomy 2p have been reviewed and some abnormalities, unusual for most other types of structural autosomal imbalance, have been found in patients with inherited forms of 2p trisomy and in their non-karyotyped sibs. Neural tube defects (anencephaly, occipital encephalocele, and spina bifida) were found in five probands and 4/6 affected non-karyotyped sibs...

The intrauterine fetal death in a gemelar pregnancy is a rare complication with an incidence from 0.5 to 6.8% of all gemelar pregnancies with a conservative management for the mother and the surviving fetus. The complications are due to the passage of tromboplastic substance from the death fetus to the surviving fetus, through intravascular communications specially in monocorionic placenta producing nervous central system, kidney and skin alterations, mainly in the surviving fetus and coagulation system alterations in the mother...

OBJECTIVE: We correlated sonographic findings with fetal outcomes in women with unsuspected twin pregnancies who had sonography in the second trimester as part of a screening program for maternal serum alpha-fetoprotein (MSAFP) level and history of neural tube defect. MATERIALS AND METHODS: The study group consisted of 97 women with twin pregnancies who participated in a screening program for MSAFP level and history of neural tube defect. Seventy-three had normal MSAFP levels, 21 had elevated MSAFP levels, and two had low MSAFP levels...

This premature girl demonstrated electrocerebral inactivity in four records over a ten day period. After another eight days with no records, low voltage activity was present at a time when clinical responsiveness increased although the child never developed a prognostically favorable clinical state. The clinical responses never became well developed and the outlook never appeared favorable. If electrocerebral inactivity can be followed by active cortical function in a child who at best had islands of neurons on a deafferented cortex, then some recovery may potentially be possible in the premature child with post bleeding distress and electrocerebral inactivity with apparently present hemisphere tissue...

Neural tube defects (NTDs) occur in at least 1 in every 1000 babies born in South Africa. The incidence is uncertain but appears to be highest in Whites and Coloureds and lowest in Blacks. Spina bifida is a particularly serious problem because many affected babies survive and require extensive, costly treatment and care. A high maternal serum alpha-fetoprotein (AFP) level may indicate that the fetus has an NTD. Enzyme-linked immunosorbent assay is well suited to mass screening of maternal sera. The Natal population is used as a model here, and it is calculated that of some 122000 pregnancies annually 38725 might be screened...

There is little available literature on survival data in the form of lifetables for babies born with anencephaly. These data would be valuable in advising parents, who often request very specific information on the length of time their child might survive. Survival is examined for a cohort of anencephalic infants in a well-defined population. Sex-specific survival tables are given, since these are the most practical to use for counselling purposes. These data show that over 40 percent of liveborn anencephalic infants (51% males; 34% females) survive longer than 24 hours, and of these, 5 percent are still alive on the seventh day...

The question whether to terminate pregnancy during the third trimester involves a moral conflict. We argue that such termination is morally justifiable if two conditions are fulfilled: first, that the fetus is afflicted with a condition that is either incompatible with postnatal survival for more than a few weeks or characterized by the total or virtual absence of cognitive function; and second, that highly reliable diagnostic procedures are available for determining prenatally that the fetus fulfills either of the two parts of the first condition...

In Britain alone about 2,400 infants (0.3 percent of total live births) are born with spina bifida each year. Nearly half of these die at or shortly after birth, but a great majority of the remainder who survive are seriously handicapped. Many attempts have been made in the past to diagnose such cases early enough in pregnancy so that timely termination of such a pregnancy can be offered to the mother. In the last decade amniotic fluid alpha fetoprotein has been used successfully at many centers in the United Kingdom as a marker for pre-natal diagnosis of neural tube defects such as open lesions of spina bifida and anencephaly...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Data are presented from the South Wales Congenital Malformation Survey (92,982 births 1964-6 inclusive) showing that within areas in South Wales there exists an inverse relation between previous spontaneous abortion rate and the prevalence at birth of neural tube defect (anencephaly or spina bifida cystica or both). This relation is independent of social class, parity, and maternal age, and is not likely to be explained by area differences in accuracy of reporting previous spontaneous abortions.On the basis of these findings a hypothesis is advanced which proposes that the incidence of neural tube defects is uniform throughout South Wales and that the present substantial and relatively stable differences in area prevalence are controlled by small area differences in mortality of malformed embryos...

A twin is described who was born with ileal atresia and hydranencephaly, the co-twin having died at about 24 weeks' gestation. If a macerated or autolysed twin is found at birth, the paediatrician should be alerted to the possibility of serious and not immediately obvious defects in the surviving infant.

Clinical and financial gains and losses accruing from five different options for screening for open neural tube defects were estimated, based principally on the results of detailed monitoring of inputs and outcomes and of process costs in the South Wales Anencephaly and Spina Bifida Study. As well as estimating the overall clinical costs of a screening service it was shown that if the prevalence, including terminations, of open neural tube defects is between 1.25 and five per 1000 births the financial cost of avoiding the birth of a seriously handicapped child who would survive for more than 24 hours is in the range 9000 pounds- 54000 pounds depending on the option adopted and the prevalence of the condition in the target population...