Survival and disability in a cohort of neural tube defect births in Dublin, Ireland

✍ Scribed by Marie Sutton; Leslie E. Daly; Peadar N. Kirke

• Publisher: John Wiley and Sons
• Year: 2008
• Tongue: English
• Weight: 110 KB
• Volume: 82
• Category: Article
• ISSN: 1542-0752
• DOI: 10.1002/bdra.20498

✦ Synopsis

Abstract

BACKGROUND:

Neural tube defects (NTDs) are a major cause of death and disability. Periconceptional folic acid prevents up to 70% of these malformations but public health campaigns to increase use of supplements have had disappointing results: The proposed mandatory fortification of bread products in Ireland has raised concerns about possible side effects. We examined data collected on a cohort of children born with NTDs in an era before fortification/supplementation to illustrate the serious consequences in terms of survival and disability.

METHODS:

All 623 infants born with NTDs in the Dublin area between 1976 and 1987 were included. Information was collected on mortality and length of survival for those who died, and for those who survived at least 5 years, interview‐based data were collected on age, place of residence, prevalence of hydrocephalus, degree of disability, schooling, and IQ.

RESULTS:

Seventy‐four births (12%) were stillborn. Of the livebirths only 41% were alive at 5 years. Factors associated with mortality were type of lesion, level of lesion, presence of other defects, hydrocephalus, year of birth, and gestation. Of the children who survived to 5 years or more, 75% had a disability and 56% were severely disabled. Type of lesion and level of lesion influenced disability risk.Of the survivors, 51% of children had mobility limitations, 59% were incontinent, 42% had hydrocephalus, and 17% had intellectual disability.

CONCLUSIONS:

These findings illustrate the devastating consequences of NTDs and underline the importance of effective intervention programs with folic acid for prevention. Birth Defects Research (Part A) 2008. © 2008 Wiley‐Liss, Inc.