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 (about 13% of births in Austria). The size of Styria is 16.401 km2 which is 19,5% of Austria.

Sources of Ascertainment

Fetuses/babies with congenital anomalies are registered if diagnosed before birth, at birth or during the first year of life – according to EUROCAT guidelines. Since 2002 there is a computerized patient recording system of the University Hospital / Med. Univ. of Graz and ICD 10 codes and admission details of each patient are documented. So we get each year the list of newborns and patients with ICD 10 codes up to one year of life who had been admitted to the University Hospital from all over Styria for diagnoses, treatment and eventually operation. Based on this list we look into the individual electronic charts for diagnostic and treatment details. The valuable data of the Human Genetics Institute, which covers the whole region, are sought separately because there is no electronic access to the data from outside the institute. The same procedure is used for the postmortem charts of the Pathology Department. About half of the cases are reported by more than one source.

Maximum Age at Diagnosis

Up to 1 year of age.

Terminations of Pregnancy for Fetal Anomaly (TOPFA)

Terminations of pregnancy of course are always based on the maternal/parental wish. TOPFA 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 and are not registered). 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/parental wish for TOPFA only up to the 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/parental 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 three ultrasound scans (8-12, 18-22 and 30-34 weeks' gestation) according to a booklet called “Mother-child Passport” which will be renamed 2023 in “Parents-child-passport”. The screening in experienced hands is paid by the health insurance only if the mother is > 35 yeas of age. More scans are done in most pregnancies, like combined testing and late assessment of fetal growth and wellbeing. Sadly, most screening scans are done on a very basic ultrasound level, which causes a low prenatal detection rate.

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 regional pattern of birth defects.

Denominators and Controls Information

Information on all births is available from birth certificates, registered nationally 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.
Boyd PA, Haeusler M, Barisic I (2011), EUROCAT Report 9: Surveillance of Congenital Anomalies in Europe 1980-2008, Birth Defects Research (Part A), Vol 91:S1
Boyd PA, Haeusler M, Barisic I, Loane M, Garne E, Dolk H (2011), The EUROCAT Network – Organization and Processes, Birth Defects Research (Part A), Vol 91:S2-S15

Ethics & Consent

The registry got ethical approval of the Ethics Committee of the Medical University of Graz, each year an extension has to be applied for . Parental consent is generally asked for electronic data recording if admitted to hospital or coming for diagnostic purposes into outpatient wards. Maternal consent to record explicitly congenital anomalies of the fetus/neonate is not sought for. The data are registered on a university computer for scientific use. The maternal name and individual data are kept on a university data base separate from the EUROCAT DMS, linked by a number. DMS Data are analysed or transferred to the EUROCAT Central Registry in Ispra 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

 

Last updated: 17.04.2023