History and Funding

The Registry was set up in 1986 following the Chernobyl disaster. It registers fetuses/babies with congenital anomalies born after 1 January 1985. The Registry has been a member of EUROCAT since 1995. It is funded by research grants provided by the Styrian Government on an annual basis.

Population Coverage

The Registry covers all births to residents of the province of Styria (Population-based I = All mothers resident in defined geographic area), which amounts to a total of approximately 10,000 births annually.

Sources of Ascertainment

Paediatric cardiology centres supply systematic case lists and diagnostic details to the registry. There is one central cytogenetic lab in Styria, which covers the whole population and provides a list of all abnormal prenatal and neonatal karyotypes annually. The registry has no direct electronic link to Cytogenetics, but close contact almost every day. So the local cytogenetic database can be used easily, although indirectly.
The Registry operates as a research programme with voluntary participation of hospitals. Information is gathered from 48 sources once per year. Sources consist of 34 minor or major obstetric hospitals, 2 pathology services, 11 child health services, including specialised departments for diagnosis and treatment, and one cytogenetic lab. 48% of cases are reported by more than one source. In the remaining 52% of cases, only one source provided data. Since 2002 most of the information is gathered electronically (see above). Fetuses/babies with anomalies are registered if diagnosed before birth, at birth or during the first year of life.

Maximum Age at Diagnosis

Up to 1 year of age.

Terminations of Pregnancy for Fetal Anomaly (TOPFA)

Terminations of pregnancy following prenatal diagnosis of congenital anomalies are registered. (Termination of pregnancy for socio-economic reasons is legal in all cases up to 12 weeks post conception).
For TOPFA there is no upper limit of gestational age by law, if serious psychological or health problems for mother or the fetus were to be expected, and labour has not started. But if a non-lethal congenital anomaly is diagnosed late, most obstetricians in Austria would follow the maternal wish for TOPFA only up to end of 23 weeks’ gestation. Thereafter, pregnancies with severe anomalies, but viable fetus, may be terminated after fetal analgesia and fetocide, if a commission agrees with the maternal wish to terminate. Non-viable forms of congenital anomalies may of course be terminated at any stage of gestation.

Prenatal screening policy

The official policy regarding prenatal diagnosis is: pregnant women are offered 3 ultrasound scans (8-12, 18-22 and 30-34 weeks' gestation) according to a booklet called 'Mother-child Passport'. More scans are done in most cases, like combined testing and late assessment of fetal growth and wellbeing.

Stillbirth and Early Fetal Deaths

Stillbirths with congenital anomalies are registered. Stillbirth definition by law is: late fetal death from a Crown Foot Length >=35cm and from 01.01.95 a limit of >=500g. If the fetus is smaller, but shows clear vital signs after delivery for some time before dying, it is counted as stillbirth as well.
There is no lower gestational age or weight limit for registration of congenital anomaly in early fetal deaths/spontaneous abortions. Autopsy rates in 1999 were as follows: still births 63%, induced abortions 68%, early neonatal death (0-7 days) 70% (estimate), later deaths 1 week to 1 year 70% (estimate) and almost all deaths with congenital anomaly.

Exposure Data / Availability

Exposure information (eg. maternal occupation, intake of drugs or illnesses during pregnancy) is not available. Data about techniques of prenatal screening (ultrasound, serum markers) and prenatal diagnosis are systematically collected. Maternal residency is recorded and can be used for evaluating the sub-regional pattern of birth defects.

Denominators and Controls Information

Information on all births is available from birth certificates, registered nationaly by Statistics Austria.

Registry Description References

Haeusler M, Berghold A, Schoell W, Hofer P, Schaffer M (1992), ”The influence of the post-Chernobyl fallout on birth defects and abortion rates in Austria”, Am J Obstet Gynecol , Vol 167, pp 1025-1031.
Haeusler M, Berghold A, Stoll C, Barisic I, Clementi M and the EUROSCAN Study group (2002), “Prenatal ultrasonographic detection of gastrointestinal obstruction: results from 18 European congenital anomaly registries”, Prenatal Diagn, Vol 22, pp 616-23.

Ethics & Consent

The registry did not require ethics committee approval in order to collect and store data, but this will be sought for in the near future.
Parental consent is not asked for, and we do not need maternal informed consent. The reason is, that data are kept confidentially on a university computer for scientific use only, and data are published or transferred to central EUROCAT registry in a non personalized form.

Address for Further Information

Prof Martin Haeusler, Registry Leader, Styrian Malformation Registry, Med. University of Graz,
Humboldtstr. 12, AT 8010 Graz, Austria
Tel: +43 699 12 22 22 12
Fax: +43 316 683 683
Email: haeuslerm@aon.at

Prof Andrea Berghold, Head of the Institute for Medical Informatics, Statistics and Documentation, Med. University of Graz,
Auenbruggerplatz 2, AT-8036 Graz, Austria
Tel: +43 31 6 38513201
Fax: +43 31 6 38513590
Email: andrea.berghold@medunigraz.at