[Trends in antenatal diagnosis, pregnancy termination and perinatal mortality in infants with congenital heart disease: evaluation in the general population of Paris 1983-2000]. / Evolution du diagnostic prénatal, des interruptions de grossesse et de la mortalité périnatale des enfants avec cardiopathie congénitale: évaluation en population générale à Paris entre 1983 et 2000.

OBJECTIVE: To examine population-based overall and malformation-specific trends in the prenatal diagnosis, pregnancy termination, and perinatal mortality for congenital heart disease (CHD) during a period of rapid progress in prenatal diagnosis and medical management of CHD and to explore the impact of prenatal diagnosis on early neonatal mortality for specific (isolated) cardiac malformations. METHODS: A total of 1982 cases of CHD, which were not associated with a known chromosomal anomaly, were obtained from the Paris Registry of Congenital Malformations. Main outcome measures were trends in the proportions diagnosed and terminated prior to birth, stillbirth and early (<1 day, one-week) neonatal mortality for (1) all cases; (2) all cases excluding isolated ventricular septal defects; and (3) malformation-specific trends for transposition of great arteries, hypoplastic left heart syndrome, coarctation of aorta, and tetralogy of Fallot. Analyses included cusum and binomial regression models for analysis of the trends during 1983-2000. RESULTS: Prenatal diagnosis rates for CHD increased from 23.0% (95%CI: 19.0-27.4) in 1983-1988 to 47.3% (95%CI: 43.8-50.8) in 1995-2000. Termination rates increased between 1983 and 1989 (9.9%; 95%CI: 7.2-13.2) and 1989 and 1994 (14.7%; 95%CI: 12.3-17.4) but seemed to remain stable thereafter. Other than for hypoplastic left heart syndrome, pregnancy termination was exceptional for the other 3 specific malformations examined. Early neonatal mortality decreased to less than a third in the period 1995-2000 as compared with 1983-1989 (risk ratio, first week mortality: 0.31; 95%CI: 0.18-0.53). First week mortality was significantly lower for cases of transposition of great arteries diagnosed before birth (risk difference: 15.4%; 95% CI: 4.0-26.7). CONCLUSION: Progress in clinical management, together with policies for increased access to prenatal diagnosis, has resulted in both a substantial increase in the prenatal diagnosis and considerable reductions in early neonatal mortality of CHD in the Parisian population.

[Prevalence and prenatal diagnosis of congenital malformations in the Parisian population: twenty years of surveillance by the Paris Registry of congenital malformations]. / Prévalence et diagnostic prénatal des malformations en population parisienne. Vingt ans de surveillance par le Registre des malformations congénitales de Paris.

OBJECTIVES: To assess overall and malformation-specific trends in the prevalence, prenatal diagnosis and pregnancy termination of congenital malformations in the Parisian population. METHODS: We used data from the Paris Registry of Congenital Malformations, which includes all births and pregnancy terminations with structural birth defects or chromosomal anomalies. Data on total and live birth prevalence were available for the period 1981-2000 and for prenatal diagnosis and pregnancy terminations for 1983-2000. Twenty malformations were selected for malformation-specific analyses due to their higher frequency and consistent definitions in different classification systems. RESULTS: For the period 1981-2000, the overall total prevalence of malformations was 3.2%. Prenatal diagnosis rates consistently increased from 16.2% (95% CI, 13.8-18.6) of cases with malformation in 1983 to 69.1% (95% CI, 66.7-71.5) in 2000. Pregnancy terminations increased from 8.8% (95% CI, 7.0-10.8) of cases with malformation in 1983 to 30.1% (95% CI, 27.7-32.6) in 2000. CONCLUSION: Registries of congenital malformations provide population-based data on the prevalence of malformations and their associations with other anomalies. Registry-based data can also be used to evaluate the impact of prenatal testing policies for congenital malformations.

[Are there any changes in Down Syndrome prevalence in France following the implementation of the measurement of nuchal translucency and maternal serum screening?]. / La mesure de la clarténucale et le dosage des marqueurs sériques commencent-ils à modifier l'incidence de la trisomie 21 en France.

OBJECTIVE: To evaluate the impact of prenatal screening for Down Syndrome in France in relation to the important changes in the French prenatal screening policy during the years 1996-1997: measurement of nuchal translucency and maternal serum screening, for all women. PATIENTS AND METHOD: Data are those published from three congenital anomalies registries implanted in Centre-Est, Paris and Bas-Rhin areas, from 1990 to 2001. RESULTS: The livebirth prevalence of Down Syndrome decreased from 1/950 in 1990 to 1/1500 in 2000-2001. This decrease was observed from 1994 onwards but has proved stronger since 1996, in spite of the observed increase in the total prevalence partly explained by changes in the maternal age distribution. Extrapolated to all births in France, the calculated annual number of Down Syndrome births would decrease from 800-900 in 1990 to 500-600 in 2001. Consequently, the proportion of induced terminations following prenatal diagnosis increased from 38.6% in 1990 to 75.5% in 2001. In addition, the mean gestational age of the induced terminations is decreasing. CONCLUSION: It seems reasonable to assume that the observed trends followed the changes in the French national policy for prenatal screening: a mass screening for all women, whatever the maternal age.

Mothers' knowledge of screening for trisomy 21 in 1999: a survey in Paris maternity units.

OBJECTIVE: To assess mothers' knowledge of screening tests for trisomy 21. STUDY DESIGN: Interview of all women who had recently delivered a healthy child and were present in 15 Paris maternity units during one of the two non-consecutive days in June 1999 (N = 734). RESULTS: Two-third said that they had access to a nuchal translucency measurement (NTM) and to maternal serum screening (MSS), and 16% to amniocentesis. Thirty-eight percent of the women who had NTMs and 69% of those who had serum screening said that they had been informed of the need for amniocentesis if the results were abnormal. Among the women who had amniocentesis, 20% did not know the risk of miscarriage and 41% had not been informed about the possibility of terminating the pregnancy if trisomy 21 was diagnosed. CONCLUSIONS: Mothers' knowledge about the screening tests for trisomy 21 remains fragmentary. Providing comprehensive information about all these tests should be considered in early pregnancy so that women can make informed choices.

[Opinions of couples on care during medical termination of pregnancy]. / Opinions des couples sur la prise en charge lors d'une interruption médicale de grossesse.

OBJECTIVES: To study the medical care, staff attitudes and patients' satisfaction from the decision to the post-intervention medical visit for termination of pregnancy for fetal abnormalies. PATIENTS AND METHODS: All patients and their spouses having a termination of pregnancy at the "Unite de Medecine Foetale" in Port-Royal Hospital between November 1996 and July 1997 were contacted for the study. A self-administered questionnaire was mailed six to eight weeks after intervention. Forty seven women and 42 men returned a completed questionnaire, the response rates were respectively 68% and 61%. RESULTS: The patients and their spouses rated globally very high their satisfaction about the care received. The delay before intervention, the length and pain of labour were rated less positively. The factors associated with satisfaction were the quality of the relationship with the staff, and of information. Positive feelings about delivery were linked with the consideration and relief of pain. Most respondents mentioned that their physical and psychological state has improved at the moment of the survey but the psychological distress subsisted or has increased in one fourth of the cases. On the whole the answers made within the couples were correlated. CONCLUSION: The positive results should be moderated by the number of non-respondents. In a context of very high rates of satisfaction, psychological distress is still present for one respondent out of four, six to eight weeks after termination of pregnancy for fetal abnormalies.

On the symmetry of limb deficiencies among children with multiple congenital anomalies.

In humans, unpaired organs are placed in a highly ordered pattern along the left-right axis. As indicated by animal studies, a cascade of signaling molecules establish left-right asymmetry in the developing embryo. Some of the same genes are involved also in limb patterning. To provide a better insight into the connection between these processes in humans, we analysed the symmetry of limb deficiencies among infants with multiple congenital anomalies. The study was based on data collected by the International Clearinghouse for Birth Defects Monitoring Systems (ICBDMS). Registries of the ICBDMS provided information on infants who, in addition to a limb deficiency, also had at least one major congenital anomaly in other organ systems. We reviewed 815 such cases of which 149 cases (18.3 %) were syndromic and 666 (81.7 %) were nonsyndromic. The comparisons were made within the associated limb deficiencies, considering the information on symmetry, using a comparison group with malformations associated not involved in the index association. Among the non-syndromic cases, the left-right distribution of limb deficiencies did not differ appreciably between limb deficiency subtypes (e.g., preaxial, transverse, longitudinal). The left-right distribution of limb anomalies did not differ among most types of non-limb anomalies, though a predominance of left-sided limb deficiencies was observed in the presence of severe genital defects - odds ratio [OR], 2.6; 95 % CI, 1.1-6.4). Limb deficiencies (LDs) were more often unilateral than bilateral when accompanied by gastroschisis (OR, 0.1) or axial skeletal defects (OR, 0.5). On the contrary, LDs were more often bilateral than unilateral when associated with cleft lip with or without cleft palate (OR, 3.9) or micrognathia (OR, 2.6). Specifically, we found an association between bilateral preaxial deficiencies and cleft lip, bilateral amelia with gastroschisis and urinary tract anomalies, and bilateral transverse deficiencies and gastroschisis and axial skeleton defects. Of 149 syndromic cases, 62 (41.6 %) were diagnosed as trisomy 18. Out of the 30 cases of trisomy 18 with known laterality, 20 cases were bilateral. In the remainder the right and left sides were equally affected. Also, in most cases (74.4 %) only the upper limbs were involved. In conclusion the left-right distribution of limb deficiencies among some non-limb anomalies may suggest a relationship between the development of the limb and the left-right axis of the embryo.

Limb defects associated with major congenital anomalies: clinical and epidemiological study from the International Clearinghouse for Birth Defects Monitoring Systems.

Although limb defects associated with other congenital anomalies are rarely studied, they may provide insights into limb development that may be useful for etiologic studies and public health monitoring. We pooled data from 11 birth defect registries that are part of the International Clearinghouse for Birth Defects Monitoring Systems. We identified 666 infants, born from 1983 through 1993, who had a non-syndromal limb defect plus at least one other major malformation (rate 12.9/100,000 population). We used observed/expected ratios and log-linear models to detect association patterns. We found that specific limb defects occurred with relatively distinct sets of malformations. Preaxial limb defects occurred more frequently with microtia, esophageal atresia, anorectal atresia, heart defects, unilateral kidney dysgenesis, and some axial skeleton defects; postaxial defects with hypospadias; transverse defects with craniofacial defects, micrognathia, ring constrictions, and muscular defects; intercalary defects with omphalocele; split hand/foot with encephalocele; and amelia with anorectal atresia, omphalocele, severe genitalia defects, unilateral kidney dysgenesis, gastroschisis, and ring constriction. Log-linear modeling identified higher order associations among some of these same malformations.

Maternal occupational risk factors for oral clefts. Occupational Exposure and Congenital Malformation Working Group.

OBJECTIVES: This study investigated the role of maternal exposures at work during pregnancy in the occurrence of oral clefts. METHODS: The occupational exposures of 851 women (100 mothers of babies with oral clefts and 751 mothers of healthy referents) who worked during the first trimester of pregnancy were studied. All the women were part of a multicenter European case-referent study conducted using 6 congenital malformation registers between 1989 and 1992. In each center, the mother's occupational history, obtained from an interview, was reviewed by industrial hygienists who were blinded to the subject's status and who assessed the presence of chemicals and the probability of exposure. Odds ratios (OR) were estimated by a multivariate analysis including maternal occupation or occupational exposures during the first trimester of pregnancy and possible confounding factors such as center of recruitment, maternal age, urbanization, socioeconomic status, and country of origin. RESULTS: After adjustment for confounding factors, cleft palate only was significantly associated with maternal occupation in services such as hairdressing [OR 5.1, 95% confidence interval (95% CI) 1.0-26.0] and housekeeping (OR 2.8, 95% CI 1.1-7.2). The analysis suggests that the following occupational exposures are associated with orofacial clefts: aliphatic aldehydes (OR 2.1, 95% CI 0.8-5.9) and glycol ethers (OR 1.7, 95% CI 0.9-3.3) for cleft lip with or without cleft palate and lead compounds (OR 4.0, 95% CI 1.3-12.2), biocides (OR 2.5, 95% CI 1.0-6.0), antineoplastic drugs (OR 5.0, 95% CI 0.8-34.0), trichloroethylene (OR 6.7, 95% CI 0.9-49.7), and aliphatic acids (OR 6.0, 95% CI 1.5-22.8) for cleft palate only. CONCLUSIONS: Due to the limited number of subjects, these results must be interpreted with caution. However, they point out some chemicals already known or suspected as reproductive toxins.

Tobacco and alcohol use during pregnancy and risk of oral clefts. Occupational Exposure and Congenital Malformation Working Group.

OBJECTIVES: This study examined the relationship between maternal tobacco and alcohol consumption during the first trimester of pregnancy and oral clefts. METHODS: Data were derived from a European multicenter case-control study including 161 infants with oral clefts and 1134 control infants. RESULTS: Multivariate analyses showed an increased risk of cleft lip with or without cleft palate associated with smoking (odds ratio [OR] = 1.79, 95% confidence interval [CI] = 1.07, 3.04) and an increased risk of cleft palate associated with alcohol consumption (OR = 2.28, 95% CI = 1.02, 5.09). The former risk increased with the number of cigarettes smoked. CONCLUSIONS: This study provides further evidence of the possible role of prevalent environmental exposures such as tobacco and alcohol in the etiology of oral clefts.

Current French practices for prenatal diagnosis of trisomy 21: a population-based study in Paris, 1992-97.

The results and limitations of current French practices for prenatal diagnosis (PND) of trisomy 21 were examined, using population-based data from the Paris Registry for 1992-97 (219 000 births). Of 670 cases of trisomy 21 reported, 71.0 per cent were terminations of pregnancy (TOP). The PND rate among mothers 38 years and older, all of whom were offered amniocentesis, was 89.9 per cent. Nearly all affected births in this age class followed maternal decisions, either to refuse amniocentesis or continue the affected pregnancy. In younger mothers, the overall French prenatal screening policy (three ultrasound examinations plus serum screening from January 1997) led to an overall PND rate of 67.3 per cent; it reached 78.8 per cent in 1997. Ultrasound accounted for 73.4 per cent of diagnosed cases. Increased detection by nuchal translucency measurement is clearly visible from 1996 onward. The birth prevalence, 8.7 per 10 000 births, diminished only slightly over the study period. The increase observed in the total number of cases in 1996 and 1997, concomitant with PND practice trends, may be due primarily to earlier TOP, which precedes miscarriages that would otherwise have occurred without being recorded. Future trends in prevalence among births must be observed carefully.

Clusters of birth defects: emergency and management. A review of some publications.

Published reports of clusters of congenital anomalies, traditionally defined as an aggregation in time and/or space of malformed cases, are interesting to analyse in terms of emergence, management and initiator of a public health decision. Through some examples of clusters for which a suspected source has been suggested, for those having identified important causal relationship or for those where the cluster appeared without any explanation, the paper shows the different steps that were taken after the initial 'alarm' and the time spent between the alarm and a final conclusion. If basic keys such as accurate field investigation, reliable estimation of the expected number and etiological evaluation, are the rule, the handling should remain flexible to take into account the particularity of each cluster. Among the clusters of congenital anomalies published in the last 20 years, very few of them were clearly explained.

Therapeutic drug use during pregnancy: a comparison in four European countries. OECM Working Group. Occupational Exposures and Congenital Anomalies.

A drug utilization study was performed using data of the OECM study on Occupational Exposures and Congenital Malformations, which was conducted in six European Registries of Congenital Anomalies (two in France, two in Italy, one in Great Britain, and one in The Netherlands): the mothers were interviewed after delivery for exposures during pregnancy, including use of therapeutic drugs. The analysis of drug use considered only the 1134 control mothers of healthy newborns, and focused on the first trimester of pregnancy: 36.2% of the interviewed mothers used at least one drug (excluding vitamins and minerals) during the first trimester. This rate varied from 22.5% in Glasgow to 50.3% and 44.2% in the French centers. Anti-infectives were the most frequent drugs (12.3% of mothers), then antinauseants (10.6%), and treatments for threatened abortion (5.5%). Important variations between countries were observed, reflecting different medical attitudes towards drug use during pregnancy.

Congenital malformations in twins: an international study.

Data provided by nine registries based in European and Latin America countries were analyzed to assess whether there is an excess of malformations in twins compared to singletons. Specific congenital malformations were coded according to the ninth revision of the International Classification of Diseases (ICD). Malformation rates and rate ratios (RR) for twins compared to singletons were calculated for each registry, and the homogeneity of the RRs was tested using the test of Breslow and Day. If departure from homogeneity in the different registries was not significant, registry-adjusted RRs with 95% confidence intervals were calculated. Overall, among 260,865 twins, 5,572 malformations were reported. A total of 101 different types of malformations or groups of defects was identified, and a homogeneous estimate of the RRs among registries was found for 91.1% of the malformations. Thirty-nine of the 92 malformations with homogeneous estimates of RRs were more common in twins than in singletons. For the remaining nine malformations, heterogeneous estimates of RRs were obtained. This study confirms the majority of already known associations and further identifies previously unreported malformations associated with twins. In conclusion, there is an excess of malformations in twins compared with singletons, and all anatomical sites are involved. The number of specific malformations associated with twins is higher than that previously reported in smaller studies.

Time trends in neural tube defects prevalence in relation to preventive strategies: an international study.

OBJECTIVE: To examine time trends in neural tube defects (NTD) prevalence from 1987 to 1996 in relation to the primary prevention policies for folic acid supplementation strategies in different countries. DESIGN: Retrospective time trends analysis of NTD prevalence. SETTING: 11 birth defect registries of congenital malformations participating in the International Clearinghouse for Birth Defects Monitoring System, in the period from 1 July 1987 to 30 June 1996. SUBJECTS: 8207 live births, stillbirths and terminated pregnancies affected by anencephaly or spina bifida registered by the 11 participating centres 1987-1996. OUTCOME MEASURES: Prevalence rate ratios based on the annual rates, using the Poisson regression model. RESULTS: During the study period a significant fall in prevalence rates for all NTD is present in Atlanta (USA), England and Wales, Hungary and Japan, and a significant rise in Norway and South America. After adjusting for the secular trends observed in the earlier years of the study, no significant trend can be attributed to preventive strategies. Data on NTD prevalence are supplemented with information on folate awareness among some of the populations studied. CONCLUSION: There is no evidence that, up to the middle of 1996, any change in time trend was attributable to the introduction of national folate supplementation policies. The possible effectiveness of folate supplementation policies for the reduction of NTD clearly needs to be tried and studied for several more years. Considering that in the Western world about 50% of pregnancies are unplanned, a policy that rests on action taken before conception can only have limited success. Strategies based on food enrichment, such as was introduced in the USA from the beginning of 1998, may prove to be more successful.

Outborn status with a medical neonatal transport service and survival without disability at two years. A population-based cohort survey of newborns of less than 33 weeks of gestation.

OBJECTIVE: Prenatal events are thought to play an important role in long-term handicap, but the specific role of perinatal factors remains controversial. Our study, conducted in the context of this debate, aimed to break down the various components of perinatal management and to assess the relationship between these components and survival without disability at the age of two years. STUDY DESIGN: A prospective geographically-defined study was conducted in 1985 in the Paris metropolitan area. It covered 53430 births (stillbirths and live births), including 539 that occurred between 25 and 32 weeks gestation. The relationship between perinatal management and survival without disability was studied by a multivariate analysis (logistic regression). The analysis was restricted to a group of 202 infants born at 31 or 32 week's gestation, to avoid indication bias. RESULTS: An inborn status (delivery in a tertiary care facility) exerted a protective effect on survival without disability at the age of two years (Adjusted Odds Ratio (OR)=7.51 [1.51; 37.4]), even though the area we studied possessed an excellent Medical Neonatal Transport Service. Multiple pregnancies also seemed to have a protective effect (Adjusted OR=2.45 [0.96; 6.27]). No statistically significant association was seen between survival without disability at two years and the presence of a hospital staff paediatrician in the delivery room. CONCLUSION: These results lead us to consider what the concept of inborn/outborn represents in the perinatal management of infants at high risk.

Adoption and fostering of babies with Down syndrome: a cohort of 593 cases.

Recently, professionals in France have noticed an increase in newborns with Down syndrome (DS) being placed for adoption. The aim of this study was to investigate DS babies given up at birth for adoption and to consider the possible determinants of this in order to assess social acceptance of DS. A retrospective cohort of all living DS babies was collected from two birth-defect registries (Paris: 1981-1990; Marseilles area: 1984-1990). Follow-up data were collected: characteristics of the baby, biological parents and maternity units, age when given up for adoption, and type of foster care. The results showed that 19.4 per cent of infants with DS (115/593) were rejected by their parents. Multiple regression analysis indicated that foreign origin of the mother, area of residence, no associated major malformation, maternal age (15-24 years), and birth rank (> 2) variables were significantly associated with a lower placement rate. Among the 115 abandoned infants with DS, 88 came from unknown parentage (76.5 per cent). For half of them, adoptive placement (88/115) occurred before the age of 6 months. Socio-cultural attitudes play a great part in these family decisions. Equally important is the manner in which professionals propose adoption as an alternative to these parents of DS babies. They should be encouraged to consider all options before making a decision, so that the best solution can be found for the interest of all.

The spectrum of congenital anomalies of the VATER association: an international study.

The spectrum of the VATER association has been debated ever since its description more than two decades ago. To assess the spectrum of congenital anomalies associated with VATER while minimizing the distortions due to small samples and referral patterns typical of clinical series, we studied infants with VATER association reported to the combined registry of infants with multiple congenital anomalies from 17 birth defects registries worldwide that are part of the International Clearinghouse for Birth Defects Monitoring Systems (ICB-DMS). Among approximately 10 million infants born from 1983 through 1991, the ICB-DMS registered 2,295 infants with 3 or more of 25 unrelated major congenital anomalies of unknown cause. Of these infants, 286 had the VATER association, defined as at least three of the five VATER anomalies (vertebral defects, anal atresia, esophageal atresia, renal defects, and radial-ray limb deficiency), when we expected 219 (P<0.001). Of these 286 infants, 51 had at least four VATER anomalies, and 8 had all five anomalies. We found that preaxial but not other limb anomalies were significantly associated with any combination of the four nonlimb VATER anomalies (P<0.001). Of the 286 infants with VATER association, 214 (74.8%) had additional defects. Genital defects, cardiovascular anomalies, and small intestinal atresias were positively associated with VATER association (P<0.001). Infants with VATER association that included both renal anomalies and anorectal atresia were significantly more likely to have genital defects. Finally, a subset of infants with VATER association also had defects described in other associations, including diaphragmatic defects, oral clefts, bladder exstrophy, omphalocele, and neural tube defects. These results offer evidence for the specificity of the VATER association, suggest the existence of distinct subsets within the association, and raise the question of a common pathway for patterns of VATER and other types of defects in at least a subset of infants with multiple congenital anomalies.

Congenital malformation and maternal occupational exposure to glycol ethers. Occupational Exposure and Congenital Malformations Working Group.

Glycol ethers are found in a wide range of domestic and industrial products, many of which are used in women's work environments. Motivated by concern about their potential reproductive toxicity, we have evaluated the risk of congenital malformations related to glycol ether exposure during pregnancy as part of a multicenter case-control study, conducted in six regions in Europe. The study comprised 984 cases of major congenital malformations and 1,134 controls matched for place and date of birth. Interviews of the mothers provided information about occupation during pregnancy, sociodemographic variables, and other potential risk factors (medical history, tobacco, alcohol, drugs). A chemist specializing in glycol ethers evaluated exposure during pregnancy, using the job description given by the mother, without knowledge of case or control status. We classified malformations into 22 subgroups. The overall odds ratio (OR) of congenital malformation associated with glycol ether exposure was 1.44 [95% confidence interval (CI) = 1.10-1.90], after adjustment for several potential confounders. The association with exposure to glycol ethers appeared particularly strong in three subgroups: neural tube defects (OR = 1.94; 95% CI = 1.16-3.24), multiple anomalies (OR = 2.00; 95% CI = 1.24-3.23), and cleft lip (OR = 2.03; 95% CI = 1.11-3.73). In this last subgroup, risk, especially of an isolated defect, tended to increase with level of exposure.

Management of the fetus with a correctable malformation in Paris maternity units: evolution 1985-1994.

OBJECTIVE: To analyze the evolution of the management of delivery and neonatal care in a population of children with correctable malformations born in Parisian maternity hospitals during the period 1985-1994. METHODS: Data were collected by the Paris Registry of Congenital Anomalies from 400,000 births recorded in Parisian maternity hospitals over a 10-year period. Chromosomal anomalies were excluded. The evolution between the first period (1985-1989) and the second (1990-1994) was analyzed for the following indicators in the management of liveborn children: place of delivery; frequency of prenatal diagnosis; transfer to intensive care units, and mortality. RESULTS: More than 60% of the births of malformed children took place in public maternity hospitals where better management is offered. Most of them were prenatally diagnosed, except for esophageal and anorectal atresia for which the rate of prenatal diagnosis was low. For malformations with poor prognoses (diaphragmatic and abdominal wall anomalies), the rate of deliveries in public maternity hospitals reached about 90%, mostly in those with intensive care units. The evolution between the two periods was characterized by a quicker transfer to intensive care units, during the first day of life for most cases. Lethality during the first day, which was already low during the first period, decreased further. The early neonatal mortality rate decreased for cardiac anomalies, but not significantly. The prognosis remained poor for diaphragmatic anomalies: 49% of liveborn children died during the first week of life.