Spina bifida is a congenital defect resulting from the incomplete closing of the neural tube—the structure that forms the brain and spinal cord—during the first month of pregnancy. This condition affects the vertebrae, spinal cord, and surrounding nerves, leading to varying degrees of neurological impairment below the defect. While spina bifida is rarely fatal when proper medical management is received, the prognosis depends heavily on the defect’s severity and type. Modern medical advances have drastically improved survival rates, allowing most individuals with the condition to live well into adulthood.
Understanding the Types of Spina Bifida
Spina bifida exists in three primary forms, with the severity linked directly to the structural defect. The mildest and most common type is Spina Bifida Occulta, where a small gap exists in one or more vertebrae. The spinal cord and nerves remain fully contained, and this form is often asymptomatic, causes no long-term problems, and may only be discovered incidentally on an X-ray.
The next type is Meningocele, where the membranes protecting the spinal cord (meninges) push through the vertebral opening, forming a fluid-filled sac on the back. Since the spinal cord itself is typically not damaged, this form usually results in few or mild long-term health problems. Individuals with meningocele often have a good prognosis following surgical repair.
Myelomeningocele (MMC) is the most severe form and is most commonly associated with fatality risk. In MMC, a portion of the spinal cord and nerves protrude through the back within an exposed fluid-filled sac. Damage to the neural tissue results in significant neurological impairments, including paralysis, loss of sensation, and bowel or bladder control issues. The higher the defect occurs on the spine, the greater the extent of nerve damage and the risk of severe complications.
Mortality and Survival Rates
Immediate death from spina bifida is uncommon today due to prompt postnatal surgical closure and supportive care. Survival rates in developed countries with specialized care exceed 90% for individuals with Myelomeningocele, allowing them to survive well into adulthood. This is a significant improvement from historical rates before the widespread use of antibiotics and shunting procedures.
Mortality risk remains greatest during the first few years of life, primarily for those born with Myelomeningocele. Factors that decrease long-term survival include a high-level spinal cord lesion and the presence of brain abnormalities at birth. For those who survive infancy, the annual mortality rate is estimated to be around 0.3% to 0.4%, which is still higher than the general population.
The median age at death for patients with spina bifida has increased substantially, rising from 41 years in 2011 to 56 years in 2022, demonstrating the effect of ongoing medical advancements. While life expectancy is generally not typical compared to the general population, the difference has narrowed significantly. Causes of death in adults are often related to secondary complications rather than the initial defect, emphasizing the need for lifelong preventative care.
Major Medical Complications That Impact Prognosis
The primary drivers of fatality and severe morbidity in spina bifida are associated medical complications, not the structural defect itself. A major life-threatening risk is Hydrocephalus, where cerebrospinal fluid accumulates in the brain’s ventricles, causing pressure that damages brain tissue. Approximately 85% of individuals with Myelomeningocele require a ventriculoperitoneal (VP) shunt to drain this excess fluid. This device has been available since the mid-1950s.
Shunt malfunction, blockage, or infection is a significant cause of morbidity and mortality throughout a person’s life. Nearly half of shunts develop complications within the first year of placement. Another serious complication is Chiari II Malformation, which involves the lower brainstem and cerebellum descending into the upper spinal canal. This descent can compress the brainstem, leading to life-threatening symptoms such as difficulty swallowing, breathing abnormalities, and upper airway obstruction.
Recurrent or severe infections are secondary risks that significantly impact long-term prognosis and are common causes of death in adults. Chronic issues like urinary tract infections (UTIs) can lead to serious kidney damage and eventual renal failure if not managed aggressively. Respiratory failure, often a consequence of Chiari II Malformation or chronic issues, is another frequently reported cause of death.
Modern Medical Management and Long-Term Outlook
Modern medical management focuses on mitigating the risks of neurological damage and fatal complications, improving both survival and quality of life. The initial intervention is often immediate postnatal surgery to close the opening, preventing infection and further damage to exposed neural tissue. A more recent advancement is fetal surgery, or in utero repair, which closes the defect before birth. This procedure has been shown to reduce the rate of hydrocephalus requiring shunting and improve motor outcomes at 30 months of age compared to postnatal repair.
These interventions are supplemented by lifelong, multidisciplinary care involving neurosurgeons, urologists, orthopedic specialists, and rehabilitation teams. Advances like clean intermittent catheterization (CIC) have dramatically improved renal health and reduced the risk of kidney failure, a former major cause of death. Consistent monitoring and aggressive management of shunt function and urinary system health are foundational to maximizing longevity.
While chronic disability is common due to permanent nerve damage, most individuals with spina bifida who receive consistent, specialized care now live well into adulthood. The long-term outlook emphasizes that fatality is primarily a risk associated with preventable or treatable complications, not the condition itself.