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Quality Report of the OSF Publication Series

Congenital anomalies

Relevance of statistical data

The Register of Congenital Malformations at the National Institute for Welfare and Health (THL) was es-tablished in 1963. The main purpose of the Register is to continuously monitor the prevalence and kind of congenital anomalies for an early identification of any new environmental factors that potentially cause foetal defects, and for the prevention of congenital anomalies by influencing these factors (monitoring / surveillance). Since 1993, the production of statistics for national and international purposes has become an increasingly important element of the Register's activities. Such statistical data are used for monitoring congenital anomalies nationally and regionally, for planning prenatal screening and diagnostics, as well as treatment of congenital anomalies, and for conducting research on congenital anomalies.

The Statistical Report of the Register of Congenital Malformations contains information on the number and prevalence rates (per 10 000 births) of congenital anomalies detected in stillbirths or in live births, on an annual basis, both nationally and by hospital district. The national-level data gives the numbers of cases with congenital anomalies among perinatal deaths and infant deaths, as well as the percentages of such cases of all infant deaths at the same age. In addition, the Report contains statistical data on major foetal malformations and other birth defects detected in selective terminations of pregnancy, and analyses the impact of such terminations on the national prevalence of cases with congenital anomalies. The annual numbers and prevalence rates of certain internationally monitored congenital anomalies are given at the national level, while a more detailed analysis is presented of neural tube defects, Down's syndrome and orofacial clefts. The statistics begin from 1993, after which year the Register data have had an adequate coverage and reliability.

The Statistical Report aims to provide up-to-date information on the numbers and prevalence rates of congenital anomalies to health-care professionals, administrators, planning officials and researchers working in the area of reproductive health and congenital anomalies, and any other people in need of such information.

The collection of data is based on the Act on the Statistical Actions of the National Research and Devel-opment Centre for Welfare and Health (STAKES; currently the National Institute for Health and Welfare, THL) (409/2001), as well as on the Act on Nation-wide Health Care Registers (556/1989) and the subsequent Statute (774/1989).

Description of methods used in statistical research

The Register of Congenital Malformations receives data on congenital anomalies from hospitals, health-care professionals and cytogenetic laboratories. It also draws data from the Medical Birth Register, the Care Register for Health Care (including Information on Outpatient Services in Specialised Health Care), the Register on Induced Abortions, and the Register of Visual Impairment, all maintained by THL, as well as from the data provided by the National Supervisory Authority for Welfare and Health (Valvira), and from the Cause of Death Statistics, maintained by Statistics Finland. The diagnoses obtained from these data sources are confirmed by contacting the hospitals that have given treatment to the infant/foetus. Notification of congenital anomaly should be made as soon as possible after the detection of a congenital anomaly after birth or termination of pregnancy. Although the Register mainly collects data from the first year of the infant for monitoring, it also collects data on subsequently detected congenital anomalies for statistics and research.

The Statistical Report shows statistics on cases with congenital anomalies included into the Register of Congenital Malformations, i.e. live births, stillbirths or selective abortions in Finland with at least one detected major congenital anomaly and with a mother who has been resident in Finland at the time of the delivery and also during most of the pregnancy.

The Statistical Report only gives information on major congenital anomalies as defined in the Register of Congenital Malformations, that is, structural anomalies, chromosomal defects and congenital hypothyroidism. Major congenital anomalies do not include hereditary diseases and other diseases not associated with congenital anomalies, dysfunction of organs or tissues, developmental disabilities, congenital infections, isolated minor dysmorphic features, normal variations and common less significant congenital anomalies included in the exclusion list of the Register. This practice complies largely with that of the European Registers of Congenital Anomalies EUROCAT.

From the notification forms and other sources, the data are stored in the Register electronically. The register is maintained by THL pursuant to the Act on Nation-wide Health Care Registers (566/1989) and Section 8 of the subsequent Statute (774/1989). Data check-ups are made regularly, missing cases and case-specific data are added from the Medical Birth Register, for instance, and any unclear cases and diagnoses are checked and ascertained by contacting the treating hospitals.

Correctness and accuracy of data

The data are correct if they have been reported correctly. Several notifications to the Register may be concerned with the same infant or foetus, specifying previously received data and ascertaining diagnoses of congenital anomalies. In case of uncertainty, the treating hospitals are contacted in order to check the data. The Register data are also compared with data from the Medical Birth Register, the Care Register for Health Care (including Information on Outpatient Services in Specialised Health Care), the Register on Induced Abortions and the Register of Visual Impairment, all maintained by THL, with data provided by the National Supervisory Authority for Welfare and Health (Valvira) as well as with data from the Cause of Death Statistics, maintained by Statistics Finland, whereby case-specific data are complemented, any missing cases with congenital anomalies are added to the Register, and diagnoses are confirmed by contacting the treating hospitals.

The data content and the data collection practices of the Register of Congenital Malformations were revised in 1985, 1993 and 2005. From 1993 onwards the data coverage can be regarded as very good although there have been no coverage analyses since the 1993 revision. The prevalence of cases with congenital anomalies corresponds to the normal prevalence described in the literature and reported internationally. The prevalence rates of different types of congenital anomalies have also been consistent with the findings of other national and international studies on congenital anomalies. Since 2005, data on congenital malformations have also been derived from the statistics on Information on Outpatient Services in Specialised Health Care, which has further improved the total coverage of the Malformation Register to some extent.

As the Register of Congenital Malformations also draws data from other registers, its data sets are not complete until after two calendar years from the birth, whereby the final data are published in the Statistical Report. In addition, the Register issues a report on preliminary data on congenital anomalies after one calendar year from the birth or the selective termination of pregnancy. As regards most cases with severe congenital anomalies, the coverage of the preliminary statistics has been found to be over 90 per cent. The preliminary statistical data concerning a majority of internationally monitored types of anomalies show an almost complete coverage. Preliminary statistics are complemented during the following calendar year, being subsequently published in the Statistical Report.

Any errors identified in the statistics will be corrected.

Timeliness and promptness of published data

The Statistical Report of the Register of Congenital Malformations is compiled annually by THL. In gen-eral, it is published in March or April. From 1993 onwards, the statistics are complete, excluding the two previous calendar years before the publication of the Report only. The preliminary data are from end of the first calendar year after the birth, termination of pregnancy or spontaneous abortion. As it is possible that congenital anomalies are not diagnosed or their principal cause, such as a chromosomal defect, is not identified until at a later stage in the infant's life, the numbers in the final annual statistics may change slightly over the years - this, however, only concerns a few individual cases.

Accessibility and transparency/clarity of data

The Statistical Report is sent to hospitals and other units submitting data to the Register, in addition to being published on the THL website at http://www.thl.fi/statistics/congenitalmalformations. The text material of the Report describes major findings and specifies concepts, definitions, symbols and methods. In order to give a better general idea of the situation longer time series have been used in some of the figu-res than elsewhere in the statistics of the Statistical Report. The graphs of the time series have been smoothed by the Microsoft Excel data analysis software in order to show the continuous formation of congenital anomalies. Further information on the numbers and prevalence rates of congenital anomalies is available from the Register.

Comparability of statistical data

The statistical data collected after the 1993 revision of the Register of Congenital Malformations are not directly comparable with the Register's earlier data, as the revision considerably improved the coverage and quality of the data compared with the data for 1963-1992. In addition to changes in the data collection and registration practices of the Register, and in the degree to which hospitals fulfil their notification obligations, there have been changes in the definitions, classifications, coding systems, diagnostics and treatment of congenital anomalies and related mortality rates over the decades. With the development of prenatal screening and diagnostics, selective terminations of pregnancy have increased. The impact of these developments has been seen particularly as regards certain severe congenital anomalies. In the late 1980s, the coverage of the Register was considerably reduced as a result of the 1985 revision of the Register. Moreover, the reform of the Act on Induced Abortion in 1985, and the redefinition of stillbirth from January 1st 1987 onwards affect the comparability of data entered in the Register in different years and decades. The 1986-1992 data of the Register are being complemented by congenital anomaly data drawn from other registers. The use of the statistics on the Information of Outpatient Services in Special-ised Health Care as data source for the Malformation Register in 2005 has improved the total coverage of the Register to some extent, but has not affected the coverage of severe malformations in the Register.

Beginning from 1993, the annual statistical data presented in the Statistical Report are mutually comparable; the modification of the data collection practices in 2005 does not reduce the comparability of data on severe malformations. Internationally, the statistics are of high quality and comparable. The prevalence rates of preliminary statistics are comparable with the final annual statistics. In some hospital districts, the degree of meeting the notification requirement is lower than usual, as a result of which the total coverage of cases with congenital anomalies may be somewhat lower within these districts than elsewhere in the country.

The concept of cases with congenital anomalies (births, terminations of pregnancy or spontaneous abortions involving congenital anomalies), and that of major congenital anomalies, as well as the definitions and classifications of major congenital anomalies, and the data content of the Register have remained unchanged since 1993. The basic definitions used (ICD-10) have remained the same.

Clarity and consistency

The Statistical Report uses established international concepts and classifications. They are mostly consistent with other national registers and databases that contain data on congenital anomalies.

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Published 10.3.2006, Updated 9.2.2011

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Last updated 9.2.2011
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