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OBJECTIVES: To investigate the survival up to age 10 for children born alive with a major congenital anomaly (CA). METHODS: This population-based linked cohort study (EUROlinkCAT) linked data on live births from 2005 to 2014 from 13 European CA registries with mortality data. Pooled Kaplan-Meier survival estimates up to age 10 were calculated for these children (77 054 children with isolated structural anomalies and 4011 children with Down syndrome). RESULTS: The highest mortality of children with isolated structural CAs was within infancy, with survival of 97.3% (95% confidence interval [CI]: 96.6%-98.1%) and 96.9% (95% CI: 96.0%-97.7%) at age 1 and 10, respectively. The 10-year survival exceeded 90% for the majority of specific CAs (27 of 32), with considerable variations between CAs of different severity. Survival of children with a specific isolated anomaly was higher than in all children with the same anomaly when those with associated anomalies were included. For children with Down syndrome, the 10-year survival was significantly higher for those without associated cardiac or digestive system anomalies (97.6%; 95% CI: 96.5%-98.7%) compared with children with Down syndrome associated with a cardiac anomaly (92.3%; 95% CI: 89.4%-95.3%), digestive system anomaly (92.8%; 95% CI: 87.7%-98.2%), or both (88.6%; 95% CI: 83.2%-94.3%). CONCLUSIONS: Ten-year survival of children born with congenital anomalies in Western Europe from 2005 to 2014 was relatively high. Reliable information on long-term survival of children born with specific CAs is of major importance for parents of these children and for the health care professionals involved in their care. [ABSTRACT FROM AUTHOR]

Context: There is little consistency in the use of instruments for measuring self-reported quality of life (QoL) in young children., Objective: To systematically review studies of self-reported QoL in children aged <12 years with congenital health conditions, and to examine the agreement between self- and proxy-reports., Data Sources: Literature databases (MEDLINE, EMBASE, Web of Science, PsychINFO) were systematically searched, reference lists of eligible studies were scanned., Study Selection: We included studies published in English between January 1989 and June 2013 which used validated instruments to assess self-reported QoL in children aged <12 years with a distinct congenital health condition identified in early infancy., Data Extraction: We extracted data on study design, objective, sample characteristics, QoL assessment instrument, statistical techniques and results., Results: From 403 full-text articles assessed for eligibility, 50 studies underwent detailed review, and 37 were included in a narrative synthesis. Children's self-reported QoL was assessed by using a variety of generic and/or condition-specific instruments, with the Pediatric Quality of Life Inventory being the most frequently used (25% [9 studies]). Regardless of the condition or the instrument used, children often reported QoL similar to the reference population, except for lower scores in the physical functioning/health domain. There were differences between younger and older age groups according to QoL domain. The child's perception of QoL differed from that of his or her parents, in particular for subjective domains such as emotional functioning, and these differences were age related. The main limitation of the review resulted from the lack of published studies on self-reported QoL in young children, in particular, lacking both self-reports and proxy reports. Existing studies demonstrated wide variability in the QoL instruments used and approaches to statistical analyses, lack of information about the formation of the study sample (response rate; comparison of responders and nonresponders) and low sample sizes in the age group of interest., Conclusions: The reviewed studies demonstrated that, even for younger children, both child and parent perspectives are essential to understanding the impact of a condition on a child's QoL., (Copyright © 2014 by the American Academy of Pediatrics.)

Objective: To examine the influence of fetal and maternal characteristics on the survival of children born with Down syndrome., Methods: We used prospectively collected population-based data on Down syndrome extracted from the UK Northern Congenital Abnormality Survey (NorCAS), January 1, 1985-December 31, 2003, matched to hospital and national mortality records to January 29, 2008, to determine survival status of liveborn children. Survival to 20 years was estimated by using Kaplan-Meier methods. Cox proportional hazards regression was used to examine factors that predict survival., Results: A total of 1115 Down syndrome pregnancies were notified to NorCAS during the 19 years, a total prevalence of 16.8 (95% CI, 15.8-17.8) per 10,000 live births and stillbirths. Of these, 5.4% resulted in a spontaneous fetal loss (late miscarriage ≥ 20 weeks and stillbirth), 31.7% in a termination of pregnancy, and 63.0% in a live birth. Survival status was known for 95.3% of live births; 16.6% resulted in a death. Year of birth (P < .001), gestational age at delivery (P < .001), standardized birth weight (P < .001), karyotype (P < .01), and presence of additional structural anomalies (P < .001) were significant predictors of survival. Infant gender, plurality, maternal age, and maternal deprivation were not significant predictors of survival., Conclusions: These robust estimates of predictors of survival are important for the prenatal counseling of parents whose pregnancy is affected by Down syndrome and for health care planning for the future care needs of these children.