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Long-term survival of individuals with myelomeningocele
Beth Ellen Davis1, Colleen M Daley, David B Shurtleff
1Department of Pediatrics, Madigan Army Medical Center, Tacoma, WA, USA. BethEllen.Davis@nw.amedd.army.mil
Insights
Survival analysis for myelomeningocele (MM) patients shows improved childhood survival after 1975. However, adults with MM and shunted hydrocephalus face decreased longevity after age 34.
Area of Science:
- Neurology
- Pediatric Medicine
- Public Health
Background:
- Myelomeningocele (MM) is a complex congenital condition impacting long-term survival.
- Historical data indicates significant mortality in infancy for MM patients.
- Cerebral spinal fluid (CSF) shunting has been associated with improved survival rates.
Purpose of the Study:
- To analyze long-term survival into adulthood for myelomeningocele (MM) patients.
- To compare survival curves based on defect severity and birth year (pre- and post-1975).
- To investigate the impact of shunted hydrocephalus on adult longevity in MM survivors.
Main Methods:
- Retrospective review of 904 myelomeningocele (MM) patient records over 43 years.
- Survival analysis comparing patient cohorts born before and after 1975.
- Statistical comparison of survival probabilities between shunted and non-shunted patients.
Main Results:
- Infant mortality was a significant factor in decreased survival before 1975.
- After 1975, adolescent survival was similar regardless of shunt status (p = 0.17).
- Adult MM patients (age 16+) with shunted hydrocephalus showed significantly decreased survival probability after age 34 compared to non-shunted individuals (p = 0.03).
Conclusions:
- While childhood survival has improved for MM patients born after 1975, long-term outcomes require attention.
- Adults with myelomeningocele and shunted hydrocephalus are at increased risk for reduced longevity.
- Further research is needed to understand and mitigate long-term risks associated with shunted hydrocephalus in adult MM survivors.
Abstract:
The objectives of this study were to extend survival analysis into adulthood for patients with myelomeningocele (MM) and to compare survival curves for patients born with varying defect severity before and after 1975. We have reviewed existing data for 904 patients with MM seen in a large multidisciplinary children's clinic over 43 years. Before 1975, a major contributor to decreased survival is death during infancy. The presence of cerebral spinal fluid shunting is a major contributor to increased survival. After 1975, survival to adolescence is similar regardless of shunt status (p = 0.17). For all patients alive at age 16, a significant decrease in survival probability after age 34 years was found for individuals with shunted hydrocephalus compared to those without a shunt (p = 0.03). Although childhood survival for individuals born after 1975 is not related to shunt status, adults with MM and shunted hydrocephalus may be at risk for decreased longevity.
