Short abstract
This commentary is on the original article by Shavelle et al. on pages 1253–1260 of this issue.
When considering any treatment that may be life‐changing, it is important to have as much information as possible on long‐term outcomes. These issues first emerged in spina bifida in the 1960s. At that time neurosurgical centres offering closure of the back within the first 24 to 48 hours after birth showed a reduction in mortality by age 2 years from >90% to <40%. 1 However, although mildly affected individuals showed improved survival with less disability, it became increasingly evident that neurological deficits present at birth could not be reversed by treatment. This led to the survival of individuals with severe disability and problems with mobility, hydrocephalus, cognition, and continence. These complications were treated with multiple operations including cerebrospinal fluid shunts, and orthopaedic and urological procedures.
It became increasingly evident that for the most severely affected infants, their quality of life and the burden imposed on themselves and their families made it difficult to justify intensive treatment. 1 Cohorts of individuals who had been treated non‐selectively from birth were studied to identify predictors of long‐term survival and associated disability. 2 Evidence‐based selection criteria were published, including Lorber's four major adverse criteria: gross hydrocephalus, kyphosis, thoracolumbar sac, and complete paraplegia. 1 Early surgery was recommended only for those with fewer than two criteria. Our group was able to develop a simpler means of predicting long‐term outcome using the sensory level, which was accurately identifiable at birth and showed long‐term stability. 2 Data from our cohort of 117 consecutive cases of open spina bifida with 99% follow‐up over 50 years are freely available at: https://doi.org/10.24376/rd.sgul.14438780.v1.
Management of spina bifida has moved on from the priorities of the 1970s. In high‐income countries, routine advice for folate supplements in females planning pregnancy and folate fortification of food has reduced the prevalence of spina bifida; and fetal ultrasound scans have facilitated selective termination of those with identifiable anatomical abnormalities. This means that many fewer infants are born with severe spina bifida. The next goal is to focus on quality of life for the current, generally more mildly affected generation of individuals with spina bifida. Quality of life includes achieving major life goals such as living independently, walking, having a job, and driving a car. 3 , 4
Shavelle et al. analysed the life expectancy at different ages in a cohort of 1659 individuals born with open spina bifida. 5 Their data derived from the Client Development Evaluation Reports of individuals with spina bifida who had received services from the California Department of Developmental Services from 1986 to 2019. Their data covered 22 102 client years and included 356 deaths. They based their analysis on three major functional abilities: walking, feeding, and continence. They expressed their life expectancy data for males and females in different age brackets by seven descriptive permutations.
The main weakness of the research is the limited parameters available for analysis and that these do not necessarily remain stable over time. For example, mobility tends to decline with age and weight gain. 3 They were also unable to evaluate the anatomical level of the lesion as this was recorded in only 20%. In addition, theirs is not a birth cohort and excludes individuals with severe disabilities who died before age 5 years. But Shavelle et al. successfully estimated life expectancy for individuals with spina bifida of different ages and levels of disability.
One way forward is to improve recording of clinical parameters available for analysis. We suggest that sensory level might serve as a single parameter that is stable over time and has demonstrable value in predicting important clinical outcomes. 2 , 3 , 4
ACKNOWLEDGEMENTS
Open access publishing facilitated by The University of Sydney, as part of the Wiley ‐ The University of Sydney agreement via the Council of Australasian University Librarians.
This commentary is on the original article by Shavelle et al. on pages 1253–1260 of this issue.
DATA AVAILABILITY STATEMENT
Not required.
REFERENCES
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Data Availability Statement
Not required.