Link between the CSF shunt and achievement in adults with spina bifida

Objectives-A few enterprising adults with shunt treated spina bifida live i ndependently in the community, have a job in competitive employment, and dr ive to work in their own car. By contrast others with similar disability bu t lacking their motivation remain dependent on care and supervision. The ai m of this study was to identify events in the history of their shunt which may have influenced their subsequent achievement. Methods-Between June 1963 and January 1971 117 babies born in East Anglia w ith open spina bifida had their backs closed regardless of the severity of their condition. When reviewed in 1997 every case was ascertained. Sixty ha d died and the 57 survivors had a mean age of 30. These were assigned to tw o groups: achievers and non-achievers, according to their attainments in in dependence, employment, and use of a car. Results-Of the 57 survivors nine had no shunt and eight of these were achie vers. All were of normal intelligence (IQ greater than or equal to 80) and only one was severely disabled. Of the 48 with shunts only 20 were achiever s (OR 11.2, 95% confidence interval (95% CI) 1.3-96.8). Lack of achievement in these 48 was associated with revisions of the shunt, particularly when revisions were performed after the age of 2. Sixteen patients had never req uired a revision and 11 (69%) were achievers; 10 had had revisions only dur ing infancy and five (50%) were achievers; 22 had had revisions after their second birthday and only four (18%) were achievers (p<0.001). Elective rev isions were not performed in this cohort and in 75% of patients revisions h ad been preceded by clear symptoms of raised intracranial pressure. Conclusion-Revisions of the shunt, particularly after the age of 2, are ass ociated with poor long term achievement in adults with spina bifida.