History and Funding
The Congenital Anomaly Register and Information Service (CARIS) collect data on all cases of congenital anomaly born to mothers normally resident in Wales. Data collection commenced on 1st January 1998 and includes any baby where pregnancy ended on or after this date. The Wales register joined EUROCAT in 1998. The register is based at Singleton Hospital, Swansea and since 1st October 2009 became part of Public Health Wales NHS trust. The register aims to collect data which can be used to describe the pattern of congenital anomalies across Wales. This should help:
- Build up and monitor the picture of congenital anomalies in Wales
- Assess interventions intended to help prevent or detect congenital anomalies
- Plan and co-ordinate provision of health services for affected babies and children
- Assess possible clusters of birth defects and their causes
The register has a lead clinician, who is an obstetrician and a director of information, who is a consultant in Public Health.
In 2005 an expert advisory group was set up to help set objectives for the register. The group consists of a fetal medicine consultant, an obstetrician, a paediatrician, a medical geneticist, a consultant radiologist, a lay representative, a consultant in public health, the director of Antenatal Screening Wales and a senior medical officer from the Welsh Assembly Government. The group meets twice yearly.
Population Coverage
The Registry is population based and covers the entire country of Wales with an annual number of births of around 35,000 currently.
Sources of Ascertainment
Reporting is voluntary. The Register relies upon multi-source reporting including: antenatal clinics, delivery units, paediatric departments, ophthalmology, cytogenetics, pathology, orthopaedics, maxillo-facial and regional centres of paediatric surgery. Each delivery unit has a nominated coordinator to help ensure good reporting. Register staff also visit units to help with data collection. Registration covers all fetuses with prenatally diagnosed anomalies. There is no lower age limit so fetal losses and early terminations with anomalies are registered. All live born babies with structural anomalies are registered if diagnosed before their 1st birthday, but all chromosomal anomalies and syndromes are registered, even if diagnosed later.
Babies in North Wales needing specialist services are referred to Liverpool and in South Wales travel to Bristol for cardiac surgery. Both paediatric cardiology centres in Cardiff and Liverpool supply systematic case lists and diagnostic details to the registry, including details of antenatally detected cases. These lists cover the whole population requiring paediatric cardiology services and are provided annually.
The cytogenetic laboratory in Cardiff provides a download of data of all abnormal karyotypes on a quarterly basis. This includes details of demographic details, procedure, and reason for karyoptying. Cases reported include antenatal and all children born since 1998. This covers most of the population. Some cases in North Wales are karyotyped in Liverpool, whilst a few cases in Mid Wales are referred via Shrewsbury to Birmingham. This means that the register cannot guarantee 100% coverage of the Welsh population; although many of these cases are known and reported back to the register.
Maximum Age at Diagnosis
Maximum age at diagnosis is set at 1 year of age. This is currently under review. Chromosomal anomalies and syndromes are an exception to this rule. In these cases, it is often found that a registration has already been made for other anomalies diagnosed within the 1st year.
Electronic reporting of inpatient data has presented a challenge, as it has highlighted unreported cases from years before. It is not always clear precisely when the diagnosis was originally made. These cases require follow-up to ensure that the diagnosis is correct as well as to verify the time of diagnosis. Regular data downloads should lessen this problem in the future and give better ascertainment.
Terminations of Pregnancy for Fetal Anomaly (TOPFA)
Termination of pregnancy is legal under certain grounds up to 24 weeks of gestation. If congenital anomaly is diagnosed, there is no upper gestational age limit for termination in cases of major anomaly. Terminations of pregnancy for Fetal Anomaly are registered whatever the gestational age.
Stillbirth and Early Fetal Deaths
Stillbirth definition: 24 weeks gestation (late fetal death after 23 weeks + 6 days gestation). Stillbirths of 24 weeks or more are registered. Early fetal deaths/spontaneous abortions have no lower limit for inclusion on the register (earliest recorded is 8 weeks gestation.)
Exposure Data Availability
Exposure information: information on maternal drug use, smoking, alcohol, maternal and paternal diseases and occupations, outcomes of previous pregnancies and assisted conception is available. Folic acid supplementation before and during pregnancy is also collected. Drugs are coded to the ATC classification. Since 2005 BMI has also been added to the dataset.
Denominators and Controls Information
Denominator data is obtained from the Office for National Statistics. Where this is not timely or readily available, then data may be used from the National Community Child health System.
Registry Description References
The registry’s description and work was most recently described in our 10th Annual report. This is available on www.wales.nhs.uk/sites3/Documents/416/Caris%20Ann%20rep%20%28Eng%29%20final.pdf
Ethics & Consent
The registry requires ethics committee approval in order to collect and store data and this comes from Leicester Regional MREC committee, under the auspices of the British Network of Congenital Anomaly Registers (BINOCAR). Approval is renewed every 5 years, next due in 2014.
National legislation requires informed consent but the registry is exempt from this. The register has been granted exemption under section 251 of the Health Service Act 2006. Parents have to ask for the removal of the child from the Register (opt-out).
Information governance standards, policies and protocols are agreed and set by a committee within Public Health Wales.
Address for Further Information
Mr David Tucker,
Public Health Wales
Congenital Anomaly Register and Information Service for Wales (CARIS),
Level 3 - West Wing,
Singleton Hospital,
Sketty Lane,
Swansea, SA2 8QA, United Kingdom
Tel: +44 1792 285241
Fax: +44 1792 285242
Email: david.tucker2@wales.nhs.uk