Prevalence rates of spina bifida in Alberta, Canada: 2001-2015. Can we achieve more prevention?

BACKGROUND: Despite a substantial prevention of neural tube defects with mandatory folic acid (FA) fortification, a significant number of cases still exist in Alberta, Canada, particularly spina bifida (SB). The purpose of this study was to review cases with SB to provide a possible explanation as to why SB is still prevalent in Alberta. METHODS: Cases with SB born between 2001 and 2015, ascertained by the Alberta Congenital Anomalies Surveillance System, were reviewed. Cases were classified as lipomeningomyelocele, syndrome/recognized condition, chromosome, associated multiple congenital anomalies, and isolated. The notice of birth forms were reviewed to determine FA supplement use before and/or during pregnancy. Socioeconomic status (SES) was also examined. RESULTS: The majority of cases were isolated (58%). The total prevalence of SB for 2001-2015 was 0.37/1,000 births, with isolated SB being 0.21/1,000 births. Urinary and congenital heart defects were the most frequently identified associated anomalies. FA supplementation could not be determined for 69% of our cases because of a lack of completeness of the notice of birth forms. There was no significant difference regarding SES between mothers of cases and all mothers in Alberta. CONCLUSIONS: It is important to examine cases with isolated SB to determine why mandatory FA fortification has not completely prevented SB and to identify which cases are not folate-responsive. A more concerted effort of public health education and promotion with the identification of women with suboptimal folate status and a better understanding of the role of other micronutrients is necessary.

Stability of orofacial clefting rate in alberta, 1980-2011.

Objective : To determine the prevalence and trends of orofacial clefts in Alberta (Canada) over a 33-year period (1980 through 2011) and to determine whether the trends differ for subcategories of orofacial clefts for the period from 1997 through 2011. Design : A prevalence study based on the Alberta Congenital Anomalies Surveillance System, which has multiple sources of ascertainment, capability of verification, and an upper age limit of 1 year. Inclusion : All live born and stillborn babies and fetal deaths less than 20 weeks' gestation (including terminations of pregnancy) born in Alberta of mothers who reside in Alberta. Results and Conclusions : Rates for cleft lip with or without cleft palate and cleft palate only have been very stable over the 33-year period (1980 through 2011). These rates include all clefts (isolated, syndromes, recognizable conditions, chromosomal and multiple congenital anomalies). Ascertainment of fetal deaths less than 20 weeks' gestation began in 1997. There are trends for the 1997 through 2011 cohort with a marginally significant increase for cleft lip with or without cleft palate in the isolated category and a significant decrease for cleft palate, mainly in the associated groups. The impact of folic acid fortification and/or multivitamins/folic acid supplementation reports in the literature have shown no consensus with respect to a change in the prevalence of orofacial clefts. It is unclear whether folic acid fortification has had any impact in Alberta.

Birth defect registries: the vagaries of management- the British Columbia and Alberta case histories.

Birth defect surveillance is of increasing interest and importance, especially since the discovery that folic acid fortification or supplementation can prevent a large proportion of neural tube defects. Funding is a constant problem, but management or policy can also lead to changes in ascertainment and quality, and even to the threat of actual closure. This is a case study of 2 Canadian registries-British Columbia and Alberta-from an historical point of view. The lessons here are applicable to many registries or surveillance systems. To succeed, 4 things must be in place-stated objectives of the program, funding (preferably government, but may start with a grant or foundation), support from public health departments, and someone to champion the cause. The importance of medical consultants cannot be overstated.

Changes in frequencies of select congenital anomalies since the onset of folic acid fortification in a Canadian birth defect registry.

OBJECTIVES: Fortification of grain products with folic acid has been shown to significantly reduce the occurrence of neural tube defects (NTDs) in Canada and elsewhere. However, the impact on non-NTD anomalies has not been well studied. METHODS: Using the Alberta Congenital Anomalies Surveillance System (ACASS), we examined changes in occurrence of select congenital anomalies where folic acid supplementation with multivitamins had previously been suggested to have an effect. Anomalies documented in the ACASS 1992-1996 (pre-fortification) were compared to 1999-2003 (post-fortification). RESULTS: A significant decrease in spina bifida (OR 0.51, 95% CI 0.36-0.73) and ostium secundum atrial septal defects (OR 0.80, 95% CI 0.69-0.93) was evident, but there was a significant increase in obstructive defects of the renal pelvisand ureter (OR 1.45, 95% CI 1.24-1.70), abdominal wall defects (OR 1.40, 95% CI 1.04-1.88) and pyloric stenosis (OR 1.49, 95% CI 1.18-1.89). CONCLUSIONS: Consistent with other studies, a 50% reduction in spina bifida was associated with the post-fortification time period. Supporting the possibility that folic acid fortification may play a role in preventing other birth defects, a 20% reduction in atrial septal defects was also associated. The increase in abdominal wall defects, most notably gastroschisis, is likely related to pre-existing increasing trends documented in several regions around the world. The increase in pyloric stenosis and obstructive urinary tract defects was not expected and any causal relationship with folic acid fortification remains unclear. Similar studies by other birth defects surveillance systems in Canada and elsewhere are needed to confirm these trends.