Congenital Anomalies In England Research Articles

Academic achievement at ages 11 and 16 in children born with congenital anomalies in England: A multi-registry linked cohort study.

Children born with major congenital anomalies (CAs) have lower academic achievement compared with their peers, but the existing evidence is restricted to a number of specific CAs. To investigate academic outcomes at ages 11 and 16 in children with major isolated structural CAs and children with Down or Turner syndromes. This population-based cohort study linked data on approximately 11,000 school-aged children born with major CAs in 1994-2004 registered by four regional CA registries in England with education data from the National Pupil Database (NPD). The comparison group was a random sample of children without major CAs from the background population recorded in the NPD that were frequency matched (5:1) to children with CAs by birth year, sex and geographical area. Overall, 71.9%, 73.0% and 80.9% of children with isolated structural CAs achieved the expected attainment level at age 11 compared to 78.3%, 80.6% and 86.7% of the comparison group in English language, Mathematics and Science, respectively. Children with nervous system CAs as a whole had the lowest proportion who achieved the expected attainment at age 11. At age 16, 46.9% of children with CAs achieved the expected level compared to 52.5% of their peers. Major CAs were associated with being up to 9% (95% confidence interval [CI] 8%, 11%) and 12% (95% CI 9%, 14%) less likely to achieve expected levels at ages 11 and 16, respectively, after adjustment for socioeconomic deprivation. Although many children with isolated CAs achieved the expected academic level at ages 11 and 16, they were at higher risk of underachievement compared to their peers. These stark yet cautiously encouraging results are important for counselling parents of children with specific CAs and also highlight the possible need for special education support to reduce potential academic difficulties.

Primary-school recorded special educational needs in children born with major congenital anomalies in England: A population-based study using the Education and Child Health Insights from Linked Data database

ObjectivesWe provide a national overview of survival to primary school and recorded special educational needs (SEN) provision among children with hospital identified major congenital anomalies (MCAs) born in England. We also report changes before and after government reform of SEN in 2014.
 MethodsWe created a cohort of 6,180,400 singleton children born in England between 1 September 2003 and 31 August 2013 using linked administrative health and education records (from the ‘ECHILD’ database). MCAs were identified using hospital admission and mortality records during infancy. We used at least one record of SEN in state-school records as a proxy for SEN provision. We quantified: survival to age 5 using Kaplan-Meier survival analysis; the prevalence of recorded SEN during primary school Years 1 to 6; and the difference in proportion of children with recorded SEN in Year 1 before and after the 2014 government SEN reforms.
 ResultsChildren with any MCA had 5-year survival rates of 95.1% (95% confidence interval, CI, 95.0, 95.2), compared with 99.7% (95% CI 99.7, 99.7) among children without a MCA. 41.5% (75,202/181,328) of children with an MCA attending state-school between Year 1 and 6 had any recorded SEN compared with 25.6% (1,282,979/5,008,624) of children without a MCA. Of the 12 system-specific MCA subgroups, children with chromosomal, nervous system and eye anomalies had the largest prevalence of recorded SEN. The prevalence of recorded SEN decreased by 4.9% (95% CI -5.3, -4.4) for children with any MCA compared with a reduction of 4.3% (95% CI -4.4, -4.2) for children without a MCA, when comparing pupils in Year 1 before and after 2014.
 ConclusionRecorded SEN among children with hospital identified MCAs was markedly higher than for those without MCAs, however more than half had no recorded SEN. Our findings suggest government reform in 2014 reduced SEN provision for children with MCAs.

Trends of Hospital Admissions Due to Congenital Anomalies in England and Wales between 1999 and 2019: An Ecological Study.

Objectives: To investigate the trends in congenital anomalies-related hospital admissions in England and Wales. Methods: This was an ecological study that was conducted using hospital admission data taken from the Hospital Episode Statistics database in England and the Patient Episode Database for Wales. Congenital malformations, deformations and chromosomal abnormalities hospital admissions data were extracted for the period between April 1999 and March 2019. Results: Hospital admission rate increased by 4.9% [from 198.74 (95% CI 197.53–199.94) in 1999 to 208.55 (95% CI 207.39–209.71) in 2019 per 100,000 persons, trend test, p < 0.01]. The most common hospital admissions causes were congenital malformations of the circulatory system, the musculoskeletal system, genital organs, and the digestive system. The most notable increase in hospital admissions rate was observed in congenital malformations of the respiratory system (1.01-fold). The age group below 15 years accounted for 75.1% of the total number of hospital admissions. Males contributed to 57.5% of the whole number of hospital admission. Hospital admission rate between females was increased by 6.4% [from 162.63 (95% CI 161.10–164.16) in 1999 to 173.05 (95% CI 171.57–174.54) in 2019 per 100,000 persons]. Hospital admission rate between males was increased by 3.4% [from 236.61 (95% CI 234.72–238.50) in 1999 to 244.70 (95% CI 242.92–246.49) in 2019 per 100,000 persons]. Conclusions: Males had a higher percentage of hospitalisation compared to females. Further studies to investigate the factors associated with higher hospitalisation rate among males are needed.

Phenotyping congenital anomalies in administrative hospital records.

BackgroundCongenital anomalies are a major cause of co‐morbidity in children. Diagnostic code lists are increasingly used to identify congenital anomalies in administrative health records. Evidence is lacking on comparability of these code lists.ObjectivesTo compare prevalence of congenital anomalies and prognostic outcomes for children with congenital anomalies identified in administrative health records using three different code lists.MethodsWe developed national cohorts of singleton livebirths in England (n = 7 354 363, 2003‐2014) and Scotland (n = 493 556, 2003‐2011). Children with congenital anomalies were identified if congenital anomaly diagnosis was recorded at birth, during subsequent hospital admission or as cause of death before 2 years old. We used three code lists: the EUROCAT list for congenital anomaly surveillance in Europe; the Hardelid list developed to identify children with chronic conditions (including congenital anomalies) admitted to hospital in England; and the Feudtner list developed to indicate children with complex chronic conditions (including congenital anomalies) admitted to hospitals in the United States. We compared prevalence, and risks of postnatal hospital readmission and death according to each code list in England and Scotland.ResultsPrevalence of congenital anomalies was highest using the EUROCAT list (4.1% of livebirths in England, 3.7% in Scotland), followed by Hardelid (3.1% and 3.0% of livebirths, respectively) and Feudtner (1.8% and 1.5% of livebirths, respectively). 67.2%‐73.3% of children with congenital anomalies in England and 65.2%‐77.0% in Scotland had at least one postnatal hospital admission across the three code lists; mortality ranged between 42.6‐75.4 and 41.5‐88.7 deaths per 1000 births in England Scotland, respectively. The risk of these adverse outcomes was highest using Feudtner and lowest using EUROCAT code lists.ConclusionsThe prevalence of congenital anomalies varied by congenital anomaly code list, over time and between countries, reflecting in part differences in hospital coding practices and admission thresholds. As a minimum, researchers using administrative health data to study congenital anomalies should report sensitivity analyses using different code lists.

The relationship between social deprivation and prevalence of congenital anomalies in England (2015-2016); an NCARDRS analysis

The relationship between social deprivation and prevalence of congenital anomalies in England (2015-2016); an NCARDRS analysis

Phenotyping congenital anomalies in England and Scotland: a comparison of three coding clusters using retrospective hospital data

ABSTRACT&#x0D; ObjectivesWe aimed to compare prevalence rates of complex congenital anomalies (CCAs) in the first two years of life in England and Scotland using two published CCA diagnosis code lists (phenotypes) developed to identify children who require high levels of healthcare.&#x0D; ApproachWe developed birth cohorts of singleton, hospital live births between 1998-2011 using birth registration, hospitalisation and mortality register datasets from England (n=8,157,213) and from Scotland (n=731,377) from. Children were followed up until 2 years of age.&#x0D; We compared prevalence rates of congenital anomalies using two code lists developed to identify CCAs: a UK chronic condition code list developed by Hardelid et al. and a US medical complexity code list developed by Feudtner et al. Children were classified as having a CCA if a diagnosis code was included in any admission during follow-up as a primary or subsidiary diagnosis.&#x0D; To assess the level of healthcare attention required, we compared the proportion of children who had at least one re-admission to hospital in the first two years of life.&#x0D; ResultsRates of CCAs in England and Scotland were higher using the Hardelid phenotype (2.8% for both countries) than when using the Feudtner phenotype (1.8% in England and 1.5% in Scotland). Rates of CCAs increased in both England and Scotland between 1998 and 2011. The increase was more notable using the Hardelid phenotype (37.2% and 20.2%, in England and Scotland, respectively) compared to the Feudtner phenotype (35.6% and 13.0%). Prevalence rates using the Hardelid phenotype were similar to EUROCAT figures. The proportion of children with at least one re-admission to hospital was higher in the Feudtner code list (69.2% in England and 84.8% in Scotland), than for Hardelid’s code list (67.9% and 72.0% in England and Scotland, respectively). The proportions of children with any of the CCA phenotypes were higher than compared to children without CCAs (24.4% in England and 25.8% in Scotland).&#x0D; ConclusionPrevalence of CCAs and associated hospital use vary depending on choice of CCA code list. Our findings suggest that Feudtner’s code list identifies children with higher level of healthcare needs. The impact of coding methods for defining CCAs in administrative hospital records should be explored and externally validated when comparing prevalence of CCAs and associated hospital use between countries.&#x0D; The impact of coding method for defining CCAs in administrative hospital records should be explored and externally validated when comparing prevalence and admission rates between countries.

Regional variation in the prevalence of congenital anomalies in England and Wales, 2005-2009

AimThe British Isles Network of Congenital Anomaly Registers (BINOCAR) provides surveillance of congenital anomalies in England and Wales.1 The aim of this study is to investigate if there is regional...

A new national data collection for congenital anomalies in England and Wales

AimIn 2009 the National Congenital Anomaly System (NCAS) run by the Office for National Statistics (ONS) ceased data collection. In 2011, the Department of Health funded the British Isles Network...

Monitoring the Prenatal Detection of Structural Fetal Congenital Anomalies in England and Wales: Register-based Study

To provide current population-based prevalence and prenatal diagnosis rates (PND) for specified major congenital anomalies in England and Wales to enable monitoring of the Fetal Anomaly Screening Programme (FASP). Secondary analysis of prospectively collected registry data. Seven multiple-source, population-based congenital anomaly registers, members of the British Isles Network of Congenital Anomaly Registers (BINOCAR) in 2005 and 2006. 2,883 births with congenital anomalies from a total of 601,545 live and stillbirths. PND and birth prevalence of selected congenital anomaly groups/subtypes (anencephaly, spina-bifida, serious cardiac, diaphragmatic hernia, gastroschisis, exomphalos, bilateral renal agenesis, lethal/severe skeletal dysplasia, cleft lip with or without cleft palate [CL + /- P]). Of the selected anomaly groups, the most frequently reported were serious cardiac (14.1 per 10,000 births [95% CI 13.0-15.2]) and CL + /- P (9.7 per 10,000 births [8.9-10.5]); the least frequent were bilateral renal agenesis and lethal/severe skeletal dysplasia (< 1.5 per 10,000 births). The PND varied for different anomalies from 53.1% (95% CI 43.5-65.2) for serious cardiac anomalies to 99.6% (95% CI 97.9-100.0) for anencephaly. Least variation in PND rates was for anencephaly (range 98.9-100%) and gastroschisis (93.5-100%); greatest variation was for serious cardiac (43.5-65.2%) and lethal/severe skeletal dysplasias (50.0-100%). BINOCAR registers can, uniquely, provide contemporary data on PND and birth prevalence rates to enable monitoring of the ultrasound component of FASP at a national and regional level, allowing comparisons between populations to be made, planning of resources facilitated and assistance for parents making informed decisions on whether to enter the screening programme.

Air Pollution and Risk of Congenital Anomalies in England, 1991-99

Air Pollution and Risk of Congenital Anomalies in England, 1991-99

Ambient air pollution and risk of congenital anomalies in England, 1991–1999

ObjectivesTo investigate whether there is an association between risk of congenital anomaly and annual ward level exposure to air pollution in England during the 1990s.MethodsA geographical study was conducted across...

Geographic density of landfill sites and risk of congenital anomalies in England

Objective: To investigate the risk of congenital anomalies in relation to an index of geographic density of landfill sites across 5×5 km grid squares in England. Methods: 2 km zones...

Chlorination Disinfection By-Products and Risk of Congenital Anomalies in England and Wales

BackgroundIncreased risk of various congenital anomalies has been reported to be associated with trihalomethane (THM) exposure in the water supply.ObjectivesWe conducted a registry-based study to determine the relationship between THM concentrations and the risk of congenital anomalies in England and Wales.MethodsWe obtained congenital anomaly data from the National Congenital Anomalies System, regional registries, and the national terminations registry; THM data were obtained from water companies. Total THM (< 30, 30 to < 60, ≥60 μg/L), total brominated exposure (< 10, 10 to < 20, ≥20 μg/L), and bromoform exposure (< 2, 2 to < 4, ≥4 μg/L) were modeled at the place of residence for the first trimester of pregnancy. We included 2,605,226 live births, stillbirths, and terminations with 22,828 cases of congenital anomalies. Analyses using fixed- and random-effects models were performed for broadly defined groups of anomalies (cleft palate/lip, abdominal wall, major cardiac, neural tube, urinary and respiratory defects), a more restricted set of anomalies with better ascertainment, and for isolated and multiple anomalies. Data were adjusted for sex, maternal age, and socioeconomic status.ResultsWe found no statistically significant trends across exposure categories for either the broadly defined or more restricted sets of anomalies. For the restricted set of anomalies with isolated defects, there were significant (p < 0.05) excess risks in the high-exposure categories of total THMs for ventricular septal defects [odds ratio (OR) = 1.43; 95% confidence interval (CI), 1.00–2.04] and of bromoform for major cardiovascular defects and gastroschisis (OR = 1.18; 95% CI, 1.00–1.39; and OR = 1.38; 95% CI, 1.00–1.92, respectively).ConclusionIn this large national study we found little evidence for a relationship between THM concentrations in drinking water and risk of congenital anomalies.

Spatial Versus Spatiotemporal Disease Mapping

ISEE-495 Objective: Disease mapping models analyze the geographical variation of disease risk. Data for this type of analysis are usually aggregated over a period of time, thus neglecting the temporal dimension. Recent advances in the subject have extended the pure spatial models to include time and also space-time interaction. Although a number of benefits can be expected from their higher flexibility, they also imply further data disaggregation. This associated increase in sparseness may lead to a loss of power to detect areas with local excess risk. The objective of this work is to compare the performance of spatial only versus spatiotemporal models to detect areas with unusually high risk. We also propose rules for detecting such areas using the space-time interaction. Material and Methods: We set up a simulation study using data on congenital anomalies in England between 1983 and 1998. We considered 9 possible scenarios and generated 50 data replicas for each of them. We fitted Bayesian spatial only and spatiotemporal hierarchical models and compared the results obtained. Results: Both type of models provided very similar estimations of the spatial risks, and no appreciable loss of power was observed. Nevertheless, the richness of the spatiotemporal model provided further information such as time trend and departures from main spatial and temporal patterns through the space-time interaction terms. The latter in particular let us to propose rules with high specificity and reasonable sensitivity to discriminate areas with excess risks stable and unstable over time. Conclusions: Decomposing variation of risks into space and time, as opposed to space only, leads to a number of benefits in terms of interpretability and helps detecting areas with unusually high risks, thus outweighing the significant extra computational effort needed to fit them.

Water Chlorination By-Products and Congenital Anomalies in England and Wales

ISEE-400 Objective: We have conducted a study to examine the relation between trihalomethanes (THMs) and congenital anomalies in 12 water regions in the United Kingdom. Material and Methods: The congenital anomaly data were provided by the National Congenital Anomalies Registry (NCAR), the Local Congenital Anomalies registries (LCARs), and the national Terminations registry. THM data based on sampling of tap water within water supply zones were provided by the water companies and linked to the birth data via residential postcode using a Geographical Information System (GIS). For each birth, a modeled estimate of THM exposure was obtained for the first trimester of pregnancy. We used 3 categories of total THM (TTHM) exposure (<30, 30 to <60, and 60+ mg/L), total brominated exposure (<10, 10 to <20, and 20+ mg/L), and bromoform exposure (<2, 2 to <4, and 4+ mg/L). Limited information on potential confounders was available on sex, maternal age, and measures of socioeconomic status (SES) at small-area scale. A total of 2,605,226 pregnancy outcomes were included in the study. Results: In general, there did not seem to be an association between THM exposure and risk of congenital anomalies. For some congenital anomaly subgroups, there tended to be a negative exposure response relationship with TTHM exposure, but only for exomphalos, and cleft palate only and broad urinary defects as part of a multiple syndrome were they statistically significant. The exposure-response relationships with total brominated THMs and bromoform for some subgroups of congenital anomalies tended to be mostly positive but not statistically significant, with the exception of obstructive urinary defects, which showed a negative exposure-response relationship with brominated THMs, which was statistically significant (P < 0.05), and isolated respiratory defects and some groups of cleft lip and palate, which showed a negative relationship with bromoform, which was not statistically significant. Conclusions: We found little evidence for a relationship between trihalomethanes and congenital anomalies.

Congenital anomaly surveillance in England—ascertainment deficiencies in the national system

Objective Firstly, to assess the completeness of ascertainment in the National Congenital Anomaly System (NCAS), the basis for congenital anomaly surveillance in England and Wales, and its variation by defect,...