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

Abstract

AimTo 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.MethodThe 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.ResultsTwo-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).InterpretationThe 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 addsIn 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.

Walking and living independently with spina bifida: a 50‐year prospective cohort study

“…10,11 Using data from the 2016 and 2017 review and previous reviews, 11,15-20 we analysed trends in walking at the mean ages of 9 years, 18 years, 25 years, 30 years, 35 years, 40 years, 45 years, and 50 years, and trends in living independently from mean age 25 to 50 years. 12, 16 We used the logrank test with Kaplan-Meier curves to compare survival in those born with a sensory level below L3 versus the remainder. 11 We used Fisher's exact test to explore possible predictors of walking and living independently at age 50 years.…”

“…17 Sleep apnoea and mid-brain elongation may also be risk factors for sudden death. [14][15][16]19 Not needing a CSF shunt, or not needing a shunt revision, is associated with independence. The two major factors predicting disability are the neurological deficit at birth and a history of CSF shunt.…”

“…After a meticulous neurological examination, all had their backs closed within 48 hours of birth; 15 if required, a cerebrospinal fluid (CSF) shunt was inserted. 11,[16][17][18][19] The reviews at home and school at the mean ages of 4 years and 9 years included clinical examination; in later reviews, only those still attending Addenbrooke's Hospital (51%, 35/69 at mean age 18y) were examined. Until 2012, the cohort had been reviewed about every 5 years with no loss to follow-up.…”

“…16,17,19 Details of deaths up to August 2017 were obtained from the Office for National Statistics and the Health and Social Care Information Centre (flagged cohort MR564), backed by information from medical records and autopsy reports. Carers included parents, partners, and/or health care staff.…”

Neuropathic bladders: Investigation and treatment through their lifetime

“…Historically, children with neurogenic bladders would go on to renal failure and suffer a significantly reduced life expectancy. 1,2 However, proper management, treatment, and expectations can result in excellent health and improvements in quality of life. Although the classic example is the child with a myelomeningocele, a significant number of our patients have other spinal cord defects, including lipomyelomeningocele, fatty filum, and occult tethered cords.…”