Article

The aim of our study was to investigate survival and causes of death in a
complete cohort of open spina bifida at the mean age of 40 years. METHOD: We
conducted a community-based, prospective study of 117 consecutive infants (50
males, 67 females) with open spina bifida whose backs were closed non-selectively
within 48 hours of birth between 1963 and 1971 at Addenbrooke's Hospital, Cambridge, UK. Of those who survived to age 1 year, 89% (82 out of 92) had a
cerebrospinal fluid shunt. In 2007, all survivors were surveyed by postal
questionnaire backed up by telephone interview. Details of deaths were obtained
from the Office for National Statistics, medical records, and autopsy reports,
and Kaplan-Meier survival curves were constructed.
RESULTS: One in three of the
cohort (40/117) died before the age of 5 years. A further 26% (31/117) died
during the next 35 years, over 10 times the national average. Half the deaths
(16/31) after the age of 5 were sudden and unexpected. All occurred in the
community and were followed by a coroner's autopsy. The most frequent causes of
these unexpected deaths were epilepsy, pulmonary embolus, acute hydrocephalus,
and acute renal sepsis. The prognosis for survival was strikingly poor in those
with the most extensive neurological deficit. Only 17% (7/42) of those born with
a high sensory level (above T11) survived to the mean age of 40 years, compared
with 61% (23/38) of those with a low sensory level (below L3; p=0.001).
INTERPRETATION: Doctors and care planners need to be aware that, contrary to
previous suggestions, there is continuing high mortality throughout adult life in
individuals with open spina bifida, and many deaths are unexpected.

Langue : ANGLAIS