Neurological level at birth predicts survival to the mid-40s and urological deaths in open spina bifida: a complete prospective cohort study

AIM: To conduct a 50-year complete, community-based, prospective cohort study to investigate long-term survival, causes of death, and influence of level of the lesion in treated open spina bifida. METHOD: The cohort comprised 117 consecutive cases whose backs were closed non-selectively at birth between 1963 and 1971 in Cambridge, UK. In 2013 we surveyed the survivors (n=39, 18 males, 21 females; mean age 46y, range 43–49y) by postal questionnaire and telephone interview. We compared outcomes in those born with a neurological deficit in terms of sensory and motor levels of L1 and above versus L2 and below. RESULTS: Two-thirds of the cohort (78/117) had died. Causes of death were cardiorespiratory (n=26), neurological (n=24), urological (n=22), or other (n=6). Only the urological deaths were related to level of the lesion: there were none in those with a sensory level of L2 or below (p<0.001). Birth findings also predicted survival: of the 57 infants with a neurological level of L1 or above, only 12% (n=7) survived compared with 55% (30/55) of the remainder (p<0.001). INTERPRETATION: The increased mortality in those born with an extensive neurological deficit was mainly due to urological deaths. Neurological level, particularly the sensory level, is the best predictor of long-term outcome and should be assessed routinely at birth. WHAT THIS PAPER ADDS: In open spina bifida, sensory level predicts urological life expectancy. Death due to urological causes only occurred in those with a sensory level of T6–L1. Neurological level predicts outcome better than cutaneous or radiological level. Sensory level should be assessed in all infants with open spina bifida. This article is commented on by Worley on pages 594–595 of this issue.