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

Abstract

Objectives: A few enterprising adults with shunt treated spina bifida live independently in the community, have a job in competitive employment, and drive to work in their own car. By contrast others with similar disability but lacking their motivation remain dependent on care and supervision. The aim 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 with open spina bifida had their backs closed regardless of the severity of their condition. When reviewed in 1997 every case was ascertained. Sixty had died and the 57 survivors had a mean age of 30. These were assigned to two groups: achievers and non-achievers, according to their attainments in independence, employment, and use of a car.

Results: Of the 57 survivors nine had no shunt and eight of these were achievers. All were of normal intelligence (IQ>/=80) and only one was severely disabled. Of the 48 with shunts only 20 were achievers (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 required a revision and 11 (69%) were achievers; 10 had had revisions only during infancy and five (50%) were achievers; 22 had had revisions after their second birthday and only four (18%) were achievers (p<0.001). Elective revisions were not performed in this cohort and in 75% of patients revisions had been preceded by clear symptoms of raised intracranial pressure.

Conclusion: Revisions of the shunt, particularly after the age of 2, are associated with poor long term achievement in adults with spina bifida.