Long-term outcome in open spina bifida.
BACKGROUND
Doctors need reliable data on outcome in order to help parents faced with difficult decisions about termination of an affected pregnancy or treatment after birth.


OBJECTIVE
To determine survival, health and lifestyle at the mean age of 30 years in a complete cohort of adults born with open spina bifida.


METHODS
Prospective cohort study.


METHODS
Well-documented cohort of 117 consecutive cases of open spina bifida whose backs were closed non-selectively at birth between 1963 and 1971.


METHODS
Survivors (age range = 26 to 33 years) were surveyed by postal questionnaire and telephone interview. The main outcome measures were the health, independence and lifestyle of the survivors in terms of living in the community, driving a car and working in open employment.


RESULTS
Ascertainment was 100%. Sixty (51%) had died, mainly the most disabled. Of the 57 survivors, 84% had a cerebrospinal fluid (CSF) shunt, 70% had an IQ of 80 or more, 37% lived independently in the community, 39% drove a car, 30% could walk more than 50 metres and 26% were in open employment. However one-third (19) still needed daily care, three were on respiratory support, two were blind, two had diabetes mellitus, and one was on dialysis. Mortality, disability and achievement reflected the neurological deficit that had been recorded in infancy in terms of sensory level. Attainment and independence were reduced in those who had needed revision of CSF shunt.


CONCLUSIONS
The survivors in this unselected cohort showed a wide range of outcome from apparent normality to very severe disability. This reflected both the extent of their original neurological deficit and events in the history of their CSF shunt.
Department of Community Health Sciences, St George's Hospital Medical School, London SW17 0RE. oakeshot@sghms.ac.uk
Pippa
P
Oakeshott
Gillian M
GM
Hunt
D002557:Cerebrospinal Fluid Shunts; D003657:Decision Making; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D011788:Quality of Life; D012684:Sensory Thresholds; D016137:Spina Bifida Cystica / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D013116:Spinal Cord / Q000503:physiopathology / Q000601:surgery; D013997:Time Factors