Is spina bifida fatal. Spina Bifida Survival Rates: 40-Year Study Reveals Unexpected Deaths and Life Expectancy

21.08.202324.06.2023 by alexxlab

What is the life expectancy for individuals with open spina bifida. How does mortality rate compare to the general population. What are the most common causes of unexpected death in spina bifida patients. How can long-term survival be improved for those with spina bifida.

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Understanding Open Spina Bifida: A 40-Year Longitudinal Study

Open spina bifida is a congenital condition that significantly impacts life expectancy and quality of life. A groundbreaking 40-year study conducted in the UK has provided crucial insights into the long-term outcomes for individuals born with this condition. The research, led by Pippa Oakeshott and colleagues, followed a cohort of 117 infants with open spina bifida from birth to an average age of 40 years.

The study’s findings shed light on survival rates, causes of death, and unexpected mortality among spina bifida patients. This comprehensive analysis offers valuable information for healthcare providers, patients, and families affected by this condition.

Survival Rates and Mortality in Open Spina Bifida

The study revealed stark differences in mortality rates between individuals with open spina bifida and the general population. Here are some key findings:

One-third of the cohort (40 out of 117) died before reaching the age of 5 years
An additional 26% (31 out of 117) died during the following 35 years
The mortality rate for spina bifida patients was over 10 times higher than the national average

Do these statistics indicate a need for improved early intervention and long-term care strategies? The data suggests that while early childhood mortality is significant, there are also substantial risks throughout adulthood for individuals with open spina bifida.

Unexpected Deaths: A Concerning Trend

One of the most striking findings of the study was the high rate of sudden and unexpected deaths among spina bifida patients. Half of the deaths (16 out of 31) occurring after the age of 5 were classified as sudden and unexpected. These fatalities shared several characteristics:

All occurred in the community setting
Each case was followed by a coroner’s autopsy
The deaths were not anticipated by caregivers or medical professionals

This high rate of unexpected mortality raises important questions about the long-term medical management and monitoring of individuals with open spina bifida. Are there warning signs or risk factors that could be identified to prevent these sudden deaths?

Common Causes of Unexpected Death

The study identified several frequent causes of unexpected death among spina bifida patients:

Epilepsy
Pulmonary embolus
Acute hydrocephalus
Acute renal sepsis

Understanding these common causes can help healthcare providers develop more targeted monitoring and preventive strategies for patients with open spina bifida. How can awareness of these risks improve long-term care and potentially reduce mortality rates?

Early Intervention and Long-Term Care in Spina Bifida

The study highlighted the importance of early intervention and consistent long-term care for individuals with open spina bifida. Among the cohort, 89% (82 out of 92) of those who survived to age 1 year had a cerebrospinal fluid shunt installed. This high rate of early intervention underscores the complex medical needs of these patients from infancy.

Despite these early interventions, the elevated mortality rate throughout adulthood suggests that ongoing medical management remains crucial. How can healthcare systems better support the lifelong needs of individuals with open spina bifida?

Comparing Spina Bifida Mortality to General Population

The study’s findings starkly illustrate the increased mortality risk for individuals with open spina bifida compared to the general population. With a death rate more than 10 times the national average, it’s clear that this condition significantly impacts life expectancy.

This disparity raises important questions about healthcare equity and the allocation of resources for individuals with complex congenital conditions. What systemic changes could help reduce this mortality gap and improve outcomes for spina bifida patients?

Improving Long-Term Survival in Spina Bifida

The high rate of unexpected deaths identified in the study suggests that there may be opportunities to improve long-term survival for individuals with open spina bifida. Some potential areas for improvement include:

Enhanced monitoring protocols for known risk factors
Improved patient and caregiver education about potential complications
Development of specialized long-term care programs for adults with spina bifida
Research into predictive markers for sudden health deterioration
Integration of mental health support to address the psychological impact of living with a chronic condition

By focusing on these areas, healthcare providers and researchers may be able to reduce the rate of unexpected deaths and improve overall life expectancy for individuals with open spina bifida.

The Role of Community-Based Care in Spina Bifida Management

The study noted that all unexpected deaths occurred in community settings, highlighting the importance of robust community-based care for individuals with open spina bifida. This finding suggests that there may be gaps in the current model of community healthcare for these patients.

Improving community-based care could involve:

Regular home health visits for high-risk patients
Telemedicine options for frequent check-ins with specialists
Community education programs to increase awareness of spina bifida complications
Support groups for patients and caregivers to share experiences and coping strategies

How can healthcare systems better integrate community-based care into the long-term management of spina bifida? By strengthening these community connections, it may be possible to identify and address potential health crises before they become life-threatening.

Advancing Research in Spina Bifida Care

This 40-year study provides a wealth of data that can inform future research and care strategies for individuals with open spina bifida. Some potential areas for further investigation include:

Genetic factors that may influence long-term outcomes
Development of new surgical techniques to address spina bifida complications
Exploration of novel therapies to manage associated conditions like epilepsy and hydrocephalus
Investigation of potential biomarkers for predicting acute health deterioration
Studies on the impact of lifestyle factors on long-term survival in spina bifida patients

By continuing to advance our understanding of open spina bifida, researchers and healthcare providers can work towards improving outcomes and quality of life for affected individuals.

The Importance of Longitudinal Studies in Rare Conditions

The 40-year duration of this study underscores the value of long-term, longitudinal research in understanding rare and complex conditions like open spina bifida. Such studies provide invaluable insights that cannot be gained from shorter-term research or isolated case reports.

Key benefits of longitudinal studies in rare conditions include:

Ability to track natural disease progression over decades
Identification of long-term complications that may not be apparent in shorter studies
Opportunity to evaluate the effectiveness of early interventions on adult outcomes
Potential to uncover unexpected findings, such as the high rate of sudden deaths in this cohort

How can the medical research community encourage and support more long-term studies of rare conditions? The insights gained from such research have the potential to significantly improve patient care and outcomes.

Psychological Impact of Living with Spina Bifida

While the study focused primarily on physical health outcomes, it’s important to consider the psychological impact of living with open spina bifida. The constant medical management, potential for sudden health crises, and awareness of increased mortality risk can all take a significant toll on mental health.

Addressing the psychological aspects of spina bifida may involve:

Integration of mental health screening into routine care
Provision of counseling services tailored to the unique challenges of living with spina bifida
Development of peer support programs to connect patients with others who share similar experiences
Education for families and caregivers on supporting the emotional well-being of individuals with spina bifida

By addressing both the physical and psychological aspects of open spina bifida, healthcare providers can work towards improving overall quality of life and potentially reducing stress-related health complications.

The Future of Spina Bifida Care: Integrating Technology and Personalized Medicine

As medical technology continues to advance, there are new opportunities to improve care for individuals with open spina bifida. Some promising areas of development include:

Wearable devices for continuous monitoring of vital signs and early detection of complications
Artificial intelligence algorithms to analyze patient data and predict potential health crises
Advanced imaging techniques for more precise diagnosis and treatment planning
Gene therapy approaches to address the underlying causes of spina bifida
3D printing technology for creating customized assistive devices and surgical implants

How can healthcare systems effectively integrate these technological advancements into existing care protocols? By embracing innovation and personalized medicine approaches, it may be possible to significantly improve outcomes and quality of life for individuals with open spina bifida.

Ethical Considerations in Long-Term Spina Bifida Care

The study’s findings also raise important ethical questions about long-term care for individuals with complex congenital conditions. Some key considerations include:

Balancing aggressive medical intervention with quality of life concerns
Addressing potential disparities in access to specialized care
Navigating end-of-life decisions in the context of unexpected health crises
Ensuring patient autonomy in long-term care decisions
Allocating healthcare resources for rare conditions with high care needs

How can healthcare systems and policymakers address these ethical challenges while striving to improve outcomes for individuals with open spina bifida? Engaging in open dialogue with patients, families, healthcare providers, and ethicists is crucial to developing compassionate and effective care strategies.

Global Perspectives on Spina Bifida Care

While this study was conducted in the UK, open spina bifida affects individuals worldwide. It’s important to consider how care approaches and outcomes may vary in different healthcare systems and cultural contexts.

Some factors that may influence global spina bifida care include:

Variations in access to specialized medical care and early interventions
Differences in social support systems and community resources
Cultural attitudes towards disability and chronic health conditions
Economic factors affecting long-term care availability
Disparities in research funding and clinical trial participation

How can the global medical community collaborate to improve spina bifida care worldwide? Sharing knowledge, resources, and best practices across borders could help reduce disparities and improve outcomes for all individuals living with this condition.

The Role of Patient Advocacy in Advancing Spina Bifida Care

Patient advocacy groups play a crucial role in advancing care and research for conditions like open spina bifida. These organizations can:

Raise public awareness about the challenges faced by individuals with spina bifida
Lobby for increased research funding and improved healthcare policies
Provide support and resources for patients and families
Facilitate connections between patients and healthcare providers
Contribute to the development of patient-centered care guidelines

How can healthcare systems and researchers better collaborate with patient advocacy groups to improve spina bifida care? By amplifying the voices of those directly affected by the condition, these partnerships can help ensure that care strategies and research priorities align with the needs and experiences of patients and families.

Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study

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. 2010 Aug;52(8):749-53.

doi: 10.1111/j.1469-8749.2009.03543.x.

Epub 2009 Dec 9.

Pippa Oakeshott¹, Gillian M Hunt, Alison Poulton, Fiona Reid

Affiliations

Affiliation

¹ Community Health Sciences, St George’s, University of London, London, UK. [email protected]

PMID:
20015251
DOI:
10.1111/j.1469-8749.2009.03543.x

Free article

Pippa Oakeshott et al.

Dev Med Child Neurol.

2010 Aug.

Free article

. 2010 Aug;52(8):749-53.

doi: 10.1111/j.1469-8749.2009.03543.x.

Epub 2009 Dec 9.

Authors

Pippa Oakeshott¹, Gillian M Hunt, Alison Poulton, Fiona Reid

Affiliation

¹ Community Health Sciences, St George’s, University of London, London, UK. [email protected]

PMID:
20015251
DOI:
10.1111/j.1469-8749.2009.03543.x

Abstract

Aim:

The aim of our study was to investigate survival and causes of death in a complete cohort of open spina bifida at the mean age of 40 years.

Method:

We conducted a community-based, prospective study of 117 consecutive infants (50 males, 67 females) with open spina bifida whose backs were closed non-selectively within 48 hours of birth between 1963 and 1971 at Addenbrooke’s Hospital, Cambridge, UK. Of those who survived to age 1 year, 89% (82 out of 92) had a cerebrospinal fluid shunt. In 2007, all survivors were surveyed by postal questionnaire backed up by telephone interview. Details of deaths were obtained from the Office for National Statistics, medical records, and autopsy reports, and Kaplan-Meier survival curves were constructed.

Results:

One in three of the cohort (40/117) died before the age of 5 years. A further 26% (31/117) died during the next 35 years, over 10 times the national average. Half the deaths (16/31) after the age of 5 were sudden and unexpected. All occurred in the community and were followed by a coroner’s autopsy. The most frequent causes of these unexpected deaths were epilepsy, pulmonary embolus, acute hydrocephalus, and acute renal sepsis. The prognosis for survival was strikingly poor in those with the most extensive neurological deficit. Only 17% (7/42) of those born with a high sensory level (above T11) survived to the mean age of 40 years, compared with 61% (23/38) of those with a low sensory level (below L3; p=0.001).

Interpretation:

Doctors and care planners need to be aware that, contrary to previous suggestions, there is continuing high mortality throughout adult life in individuals with open spina bifida, and many deaths are unexpected.

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Spina Bifida Life Expectancy, Causes, Symptoms & Treatment

Did you know that one in every 1,000 babies is born with spina bifida? For those who don’t know, spina bifida is a birth defect where the spinal cord does not close properly. As a result, there can be problems with bladder and bowel control, movement, and even learning.

While it’s impossible to predict how spina bifida will affect someone long-term, it’s important to be aware of some of the potential health issues a person with spina bifida might face. In this blog post, we’ll discuss spina bifida life expectancy, causes, treatment, and common health issues people with spina bifida may experience. We hope this information provides some insight and inspiration as you navigate your journey with spina bifida.

What Problems Does Spina Bifida Cause?

Some of the most common problems include:

Spinal cord damage

One of the most common Spina Bifida symptoms is damage to the spinal cord. This can lead to a loss of sensation and mobility, as well as problems with bowel and bladder control.

Musculoskeletal problems

Spina bifida can cause a variety of musculoskeletal problems, including scoliosis, kyphosis, and lordosis. These problems can be painful and may require surgery to correct.

Hydrocephalus

Hydrocephalus is a condition in which fluid accumulates in the brain, causing pressure on the brain tissue. It is a common complication of Spina Bifida and can often be treated with surgery.

Spinal deformities

Spina bifida can cause the spine to curve abnormally, which can lead to back pain and difficulty walking. Spinal deformities can often be corrected with surgery.

Nervous system problems

Spina bifida can damage the nerves that control movement and sensation, leading to paralysis, weakness, and numbness. Nervous system problems can often be helped with surgery, physical therapy, and other treatments.

What Are the Long-Term Effects of Spina Bifida?

Poor bladder control: This can be a major problem for people with spina bifida as it can lead to urinary infections and incontinence. There are a number of treatments available, including medication, surgery, and special exercises.
Bone disease: Spina bifida can cause a number of problems with the bones, including scoliosis (curvature of the spine) and kyphosis (humpback). These problems can be treated with surgery, braces, and physical therapy.
Learning difficulties: Many people with spina bifida have difficulty learning new things and processing information. This can be helped with special education and therapy.
Loss of mobility in legs: Spina bifida can cause paralysis of the legs, which can make it difficult to walk or even stand. There are a number of treatments available to help people with Spina Bifida regain mobility, including surgery, physical therapy, and specially-designed braces or wheelchairs.
Depression: People with spina bifida can often feel isolated and alone. This can lead to depression, which can be treated with medication, therapy, and support groups.

What are the Causes of Spina Bifida?

There are several possible causes of spina bifida, including:

Genetic factors: Spina bifida is often caused by a combination of genetic factors such as mutations in the gene responsible for spinal cord formation.

Environmental factors: Exposure to certain environmental toxins, such as organic solvents used in agriculture, may increase the risk of spina bifida.

Nutritional deficiencies: The occurrence of spina bifida may be influenced by dietary deficiencies of folic acid, a type of vitamin B.

Medical conditions: Spina bifida is more common in people with certain medical conditions, such as diabetes and obesity.

What Is The Life Expectancy of Spina Bifida?

One of the most frequently asked questions about spina bifida is, “What is the life expectancy?” The answer, unfortunately, is not a simple one.

Most children born with spina bifida today live into adulthood. However, the severity of the condition can range from very mild to very severe, and their life expectancy can vary greatly as a result.

There are many factors that can affect life expectancy in people with spina bifida, including:

The level of disability
Associated medical conditions
Access to quality care.

People with spina bifida who have milder forms of the condition and do not have any other major health problems can expect to have a normal lifespan. However, those with more severe forms of spina bifida or who have other associated health problems (such as hydrocephalus) may have a shorter life expectancy.

There have been significant improvements in the care of people with spina bifida over the past few decades, and this has led to a significant increase in life expectancy. With access to quality medical care and support, people with spina bifida can expect to lead long and healthy lives.

Conclusion

Spina bifida is a severe birth condition caused by improper development of the spinal cord. This can result in both physical and mental challenges. Spina bifida can be treated either surgically, with medication, or through other means. If you or a loved one has been diagnosed with Spina Bifida, it is essential to be aware of the possible health complications that may arise.

No one should face Spina Bifida on their own. People with spina bifida can lead full and happy lives with the proper support. If your child exhibits symptoms of spina bifida, consult POBAR, a pediatric orthopedic clinic immediately. We are specialists in treating this illness. Contact us and feel free to schedule an appointment!

Spina bifida. What is spina bifida?

IMPORTANT
The information in this section should not be used for self-diagnosis or self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.

Spina bifida is a malformation of the spinal column, which consists in the underdevelopment of the arches and / or bodies of one or more vertebrae with the formation of a defect in the wall of the spinal canal, through which the membranes and tissues of the spinal cord can prolapse with the formation of congenital spinal hernia. Clinical manifestations are observed in the formation of a hernia or compression of the spinal cord by connective tissue and fatty growths. It is diagnosed clinically, as well as with the help of radiography, CT and MRI of the spine. Treatment is surgical only. The operation is aimed at eliminating the existing defect, but in no way can affect the existing congenital pathology of the spinal cord itself.

Causes of spina bifida
Classification of spina bifida
Symptoms of spina bifida
Complications of spina bifida
Spina bifida diagnostics
Treatment and prognosis of spina bifida
Prices for treatment

General

Spina bifida is a congenital anomaly of the spinal column, in which a defect in the bone tissues that form the spinal canal is formed at the level of one or more vertebrae. The spinal cord passes through the canal, which can prolapse at the level of the formed defect, forming a congenital spinal hernia. In modern neurology and neonatology, spina bifida is also referred to by the Latin term spina bifida. The prevalence of this anomaly in the development of the spine among the population, according to some data, is up to 20%. At the same time, the incidence of congenital spinal hernia does not exceed 1 case per 3000 newborns. Most often, the anomaly is localized in the lumbosacral spine. 99% of congenital hernias of the spinal cord are formed in the back of the spinal canal. The contents of the hernia may vary. An exit to the hernial sac of the tissues of the spinal cord is observed in 75% of cases.

Spina bifida

Causes of spina bifida

Spina bifida is caused by disorders in the formation of the neural tube, which occurs at the 3rd week of pregnancy and ends by the 23rd day of gestation. Spina bifida is a multifactorial pathology. Hereditary mechanisms, spontaneous mutations and exposure to teratogenic factors may play a role in its development.

The latter include intrauterine infections (cytomegaly, measles, rubella, herpes), hyperglycemia in diabetes mellitus, constant overheating of the body of a pregnant woman (insolation, hot baths, sauna), taking pregnant pharmaceuticals with a teratogenic side effect (for example, antibiotics, anticonvulsants), folate deficiency acids, intoxication (drug addiction, alcoholism, substance abuse, occupational hazards), radiation. The situation is aggravated by the fact that often at such early stages of gestation, a woman does not know about her pregnancy and does not comply with the necessary protective regime aimed at excluding possible teratogenic effects as much as possible.

Classification of spina bifida

Allocate hidden and open spina bifida. Hidden (spina bifida occulta) is a small bone defects (for example, non-closure of the vertebral arches) without the formation of a spinal hernia. Open spina bifida (spina bifida aperta) includes rachischisis and herniated spinal cord. Any type of spina bifida can be accompanied by the growth of fibro-adipose tissue, filling bone defects, penetrating into the subarachnoid space and causing compression of the spinal cord.

Depending on the contents of the hernial sac, there are 4 forms of congenital spinal hernias. With meningocele, only cerebrospinal fluid (CSF) is in the hernial sac. Meningoradiculocele, in addition to cerebrospinal fluid, contains the membranes of the spinal cord. In myelomeningocele, the hernia consists of cerebrospinal fluid, membranes, and tissues of the spinal cord. Myelocystocele is a complete prolapse of a segment of the spinal cord with membranes, accompanied by eversion and expansion of the spinal canal. Rakhishizis is a spina bifida in the most severe form, when a bone defect of great length is combined with non-fusion of the membranes of the spinal cord and skin. In this case, the spinal cord is visible through a gaping hole in the spine. This pathology leads to death.

Symptoms of spina bifida

Latent spina bifida is usually asymptomatic. Clinical manifestations manifest in cases of proliferation of fibro-adipose tissue with compression of the roots and other structures of the spinal cord. Most often, the debut of the clinic falls on adolescence. Characterized by radicular pain of the type of sciatica, which the patients themselves describe as a sensation of the passage of an electric current. On the skin in the area of a bone defect, as a rule, there is a tuft of hair and / or a pigment spot, sometimes a papilloma.

A meningocele is characterized by a visible bulge on the spine covered by a thin layer of skin. Such spina bifida occurs without the development of a neurological deficit. Meningoradiculocele is a protrusion that occupies the area from 4 to 5 vertebrae. The nature of neurological disorders depends on the number of roots involved in the hernial sac and the location of the hernia. The clinic can vary from mild paresis to a pronounced decrease in muscle strength, sensory and pelvic disorders. Myelomeningocele and myelocystocele involve 6 to 8 vertebrae and are not covered by skin, characterized by severe paralysis, lack of sensation, urinary incontinence, and encopresis.

Spina bifida is often accompanied by hydrocephalus. It can be combined with other malformations: Chiari anomaly, curvature of the spine (scoliosis, kyphosis), clubfoot, congenital limb deformities, hip dysplasia, congenital heart defects, multiple dermatofibromas, lipomas, teratomas.

Complications of spina bifida

Spina bifida with the formation of a hernia is dangerous by infection of the hernial sac. As a rule, such a complication occurs in the absence of the skin over the hernial protrusion. In addition, infection can occur with ruptures and other damage to the thinned skin covering the hernia. In such cases, reddening of the tissues of the hernial sac and surrounding skin is observed. Infection is dangerous by the spread of inflammation to the meninges with the development of meningitis, the infection enters the bloodstream with the onset of sepsis.

Another complication of congenital spinal hernias can be a rupture of the hernial sac with the outflow of cerebrospinal fluid (liquorrhea), which leads to a drop in cerebrospinal fluid pressure and also opens the gate for infection. In addition, spina bifida can be complicated by increasing compression of the spinal cord, which eventually leads to irreversible changes in the spinal tissues.

Spina bifida diagnostics

Hidden spina bifida is often not diagnosed during life. The diagnosis of congenital spinal hernia is established by a neonatologist together with a neurologist according to the clinic and the results of palpation – palpation of the bone defect of the spine. Clarification of the diagnosis is carried out using CT and MRI of the spine. CT of the spine to a greater extent allows you to identify existing bone defects and deformities, MRI – to obtain information about the state of the spinal cord and its membranes, to establish the contents of the hernial sac. In younger children, CT is not performed due to high radiation exposure. In the absence of the possibility of a tomographic examination, an X-ray of the spine is used.

CT scan of the lumbar spine. Absence of complete fusion of the L5 arch (Spina bifida)

It is mandatory to conduct a study of somatic organs to identify concomitant congenital pathology. For this purpose, a biochemical analysis of blood and urine, urography, CT scan of the kidneys, chest x-ray, ultrasound of the heart, ultrasound of the abdominal cavity, MSCT of the abdominal cavity, radiography of bones and joints are prescribed. Specialists of various profiles can be involved in the diagnosis as necessary: nephrologist, cardiologist, gastroenterologist, etc.

In some cases, prenatal diagnosis is possible. Spina bifida can be suspected according to obstetric ultrasound, amniocentesis, followed by a study of the concentration of acetylcholinesterase and α-fetoprotein in the amniotic fluid. Prenatally diagnosed spina bifida is an indication for caesarean section, which helps to prevent injury to the spinal hernia during natural childbirth.

Treatment and prognosis of spina bifida

Open spina bifida needs surgical treatment. The operation is carried out by neurosurgeons in a planned manner in the first days of a newborn’s life. It helps to prevent the development of complications and the growth of neurological deficit, to eliminate the existing cosmetic defect. In case of violation of the integrity of the hernial sac and liquorrhea, the operation is performed in an urgent manner. Contraindications to surgical treatment may be severe disorders of the somatic organs due to concomitant congenital pathology.

The operation is performed using general anesthesia. Its essence is to close the existing defect. An incision is made along the surface of the hernial protrusion. Nerve formations are carefully separated and retracted inside behind the spinal membranes. The membranes are sutured in layers, the skin defect is closed. Complications of surgical intervention can be increasing hydrocephalus, infection of the wound, divergence of its edges, trophic disorders in the area of the postoperative wound that interfere with its healing. A lumboperitoneal shunt may be used to prevent hydrocephalus.

In the postoperative period, rehabilitation treatment is carried out, aimed at increasing muscle strength and range of motor activity, preventing joint contractures. Various methods of physiotherapy, physiotherapy exercises, massage are used. The outcome of the operation largely depends not on the skills of the neurosurgeon, but on the form of the spinal hernia. If spina bifida is not accompanied by the involvement of spinal cord tissues in the hernial sac, then after the operation there is a complete or significant restoration of neurological functions. With myelomeningocele and myelocystocele, the existing neurological deficit persists after surgery, since surgical intervention is not able to restore the altered tissues of the spinal cord.

You can share your medical history, what helped you with the treatment of spina bifida.

Sources

self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.
what is it, treatment, operation, consequences
Spina bifida is an anomaly of intrauterine development of the spine in the fetus. With this pathology of embryogenesis, the spinal cord, together with the hard shell of the spinal cord, splits the spine. In other words, spina bifida is also referred to as spina bifida. The disease also carries many side pathologies that pose a threat to human life.
The name of the pathology comes from the Latin terms “Spina” and “bifida” – back and splitting. In practice, it is considered a severe developmental anomaly, which is difficult to treat therapeutically, requires surgical intervention and long-term rehabilitation.
Back bifida in adults is an extremely rare disease, because children often have severe deviations and do not live to be 3-4 years old. Given the fact that myelomeningocele is the most common, the most severe form, developmental pathology is fatal.
Contents of the article
Spinal anatomy
The spine consists of 33-35 vertebrae, divided into 5 sections – cervical, thoracic, lumbar, sacral, coccygeal. There are 7 vertebrae in the cervical, 12 in the thoracic, 5 in the lumbar and sacral, while the vertebrae of the sacral spine merge and form a powerful structure – the sacrum, which supports the spinal column.
The coccygeal vertebrae are unstable and represent the remains of an atavism – an underdeveloped tail. A person can have from 2 to 5 coccygeal vertebrae, which also fuse and form the coccyx. Each vertebra has a body, an opening, a transverse and spinous process, and also, depending on the department, its own characteristics.

All the openings of the spine fold into the dorsal column, which carries the spinal cord. The spinal cord is part of the central nervous system, which is responsible for the transmission of impulses from the brain, controls the functioning of internal organs and is responsible for the work of unconditioned reflexes.
According to its structure, the spinal cord consists of the bodies of the second effector and first afferent neurons, axons and dendrites of the nuclei of the spinal cord and brain. The spinal cord has membranes that protect it from damage and infection by foreign organisms. The spinal cord has posterior and anterior horns that produce spinal nerves.
Spinal nerves form nerve plexuses – cervical, brachial, lumbar and sacral, which innervate different groups of muscles and human skin. If these plexuses are damaged, various dysfunctions of internal organs and loss of motor activity will be observed.

The spine has a number of intervertebral joints, which contain ligaments between the vertebrae and vertebral discs – shock absorbers, which are located between the vertebral bodies. The spinal disc consists of the gelatinous body and the fibrous ring – when the latter ruptures, the roots of the spinal cord are infringed – an intervertebral hernia.
The spine is also surrounded by two layers of muscles – deep and superficial. Both are responsible for the stability of the spine and the ability of a person to turn the body to the right and left, bend and unbend, and bend. On top of the muscles there are skin coverings that protect the muscles and the organs underlying them from damage by external factors
What is spina bifida?
This pathology, according to embryology, develops at the ninth week of pregnancy. It is an incomplete closure of the developing neural tube – the future spinal cord and spine. At the same time, splitting of the vertebral body is noticeable above the affected area.
Depending on the severity of the disease, the vertebral body may be split or partially – the spinous process will be absent, the vertebral arch will be partially fused. In a more severe stage, the vertebral arch will be completely absent and the nerve fibers will come out. In this case, a skin pouch with a spinal cord or without a skin covering is formed externally.
See also: spina Bifida in children.
Disease grades
Spina bifida occurs most often at the level of the lumbosacral junction – at the level of the L5 and S1 vertebrae. Allocate a form of moderate and severe severity. With moderate severity, surgical intervention has the goal of healing from the disease, and in most cases the lost functions of the spinal cord are restored.
Moderate severity is characterized by splitting of a vertebra with loss of its function without damage to the spinal cord, or with minor damage.
If the severity of the disease is severe, the treatment of the disease will carry the goal of alleviating the patient’s condition – removing severe symptoms of the disease, restoring organ function. In this case, a complete recovery is almost impossible. The following stages are distinguished for back bifida:
- Meningocele;
- Lipomeningocele;
- Myelomeningocele.
Meningocele is a moderate disease. The spinal canal is not closed, the meninges partially protrude through the back in the form of a sac in which cerebrospinal fluid accumulates. The most common is the meningocele of the lumbar vertebrae, however, there are pathologies in the thoracic and cervical regions. The severity of the consequences will depend on how high the anomaly is located.
Lipomeningocele is characterized by the fact that lipoid, or fatty, tissue perforates the membrane of the spinal cord and presses it, disrupting its function. In some cases, instead of destroying the spinal membrane, only thickening of the fatty and connective tissues that press down on the spinal cord is possible.
In this case, there will be dysfunction of the internal organs that are innervated along the course of the spinal cord. The lower the organ is located, the lower will be the section of the spinal cord that innervates it. Parasympathetic innervation is located at the level of the sacral plexus, therefore, often, if the lumbar region is damaged, the pelvic organs – the rectum, bladder, will not function normally.
Myelomeningocele is considered the most difficult diagnosis to treat and carries the most severe consequences for the patient. The spinal cord with myelomeningocele falls out – it becomes visible along with the roots. The complexity varies – the spinal cord can be simply noticeable – there are no integumentary tissues of the lower back, or fall out.
Myelomeningocele is considered the most severe form – the outcome almost always involves the loss of lower limbs and intestines, however, with proper therapy and high-quality surgical intervention, it is possible to restore the shape of the spine and save a person’s life.
Symptoms
Symptoms vary depending on the form of the disease. These include various degrees of visibility of the spinal cord and deformities at the level of different parts of the spine:
- Paralysis of the limbs;
- Speech disorders;
- Deformities of the bones of the spine – scoliosis, deformity of the ribs, pelvis;
- Displacement of the medulla;
- Dysfunction of organ systems;
- Disorders of the integumentary layers of the body;
- Problems with back muscle mass;
- Disorders in the work of the heart and lungs.
- Visible symptoms.
In essence, all symptoms are different consequences of deformation. Visible abnormalities are the most significant diagnostic symptom. With hidden splits, spots in the area of \u200b\u200bpathology, dips in the back will be noticeable.
With the development of a more severe form of the disease, more serious external symptoms can be observed – retrograde displacement of the vertebra, lack of skin, skin sac containing spinal mass.
Paralysis and speech disorders
Injury to the spinal cord will result in paralysis of the upper or lower extremities. Different types of damage are possible – if spina bifida is closer to the lower back, only paralysis of the lower extremities and pelvic muscles will be observed. If the paralysis is above the 2nd vertebra of the thoracic region, paralysis of the whole body is usually added to the damage to the legs.
Speech disorders depend on the magnitude of the upward displacement of the spine. At the same time, the spinal cord begins to put pressure on the head sections of the brain, causing their deformation. Deformation leads to a delay in the development of speech, organ functions, visual impairment. In addition, it is possible to develop hydrocephalus – dropsy of the brain, which leads to the death of the child.
Spinal disorders
Spina bifida leads to various disorders in the spine. These include scoliosis, spondylolisthesis, kyphosis, lordosis, interarticular hernia or the absence of intervertebral discs. Scoliosis is characterized by the presence of curvature in the spine – one or more vertebrae deviate in a certain direction.
This happens due to a violation in the development of the vertebra – because of it, the entire overlying spine sags, and developmental dysfunction is observed in the lower vertebrae – due to the lack of pressure, growth of the lower vertebrae is possible, weakening of their bodies and arches, because under the pressure of their strength becomes higher.
Lordosis and kyphosis are essentially scoliosis that occurs in the frontal projection. With pathological kyphosis, there will be a distortion of the spine back, with lordosis – with forward. Changes anterior to the normal position of the spine will be called anterograde, posterior – retrograde. Normally, kyphosis and lordosis form the natural curves of the spine.
Spondylolisthesis refers to the displacement of a vertebra from its normal location – often occurs with the upper and lower vertebra from a split one. Splitting of a vertebra can also transmit to them – there will also be no spinous processes, an increase in the zone of prolapse of the spinal cord is possible.
Due to disorders of the vertebrae, the intervertebral disc may develop incorrectly – the fibrous membrane becomes thinner and, with sufficient pressure, is torn and pressed through, forming a hernia. The hernia presses down on the paravertebral roots and the spinal cord, causing a complication of the syndrome and severe pain.

Pain syndromes due to dysfunction of the spine can cause the development of a pain focus in the brain, which in turn leads to epilepsy – spontaneous mass activity of neurons, which leads to loss of consciousness, convulsions, death.
Dysfunction of organs
In case of spinal cord disorders, various disorders in the spinal cord are observed, which lead to disorders of the innervation of the trunk organs. At the level of the lumbar and sacral plexus there are nerve endings that innervate the rectum, the organs of the genitourinary system.
At the level of the chest there are nerve roots, the innervation of which goes to the heart, lungs, stomach, liver, endocrine glands, small and large intestines. The brachial plexus, in addition to the muscles of the upper limbs, also gives branches to the heart and lungs. The neck is innervated by the muscles of the neck, pharynx, trachea, and partly by the organs of the mouth.
Therefore, in case of disorders in the lumbar region, there will be disturbances in the functioning of the smooth muscles of the genitourinary system. Spina bifida at the upper levels in case of damage to the spinal cord will cause disturbances in all underlying organs. Thus, the higher the damage, the more extensive the symptoms will be.
A deformed spine will cause extensive damage to the arteries and veins, which will disrupt the blood supply to the organs, causing necrosis, and necrotic lesions will affect all surrounding tissues, endangering the life of the child.
Other symptoms
Skin disorders are possible with myelomeningocele, in which there will be no skin. Violations in the work of the heart occur due to a violation of its sympathetic and parasympathetic innervation, which is responsible for the rhythm of the heart. Lungs due to disruption of the heart may be prone to edema.
The muscles of the back are highly dependent on the location of the vertebrae, since they push off mainly from the spines and are responsible for their location. With curvature, there may be noticeable damage, spasms or underdevelopment of the muscles. It should be noted that most of the symptoms are not necessarily signs of meningocele, since sometimes mild splitting occurs without external signs.
Treatment
Due to its specificity, spina bifida is not amenable to treatment, and all therapy is aimed at alleviating the consequences and stopping the symptoms and developing side syndromes. The main pathology in adults is the development of diseases of the urinary tract and rectum.
The methods include:
- Stabilization of the urination process;
- Relief of the processes of violation of independent urination and defecation;
- Split vertebrae surgery;
- Stimulation of affected organs;
- Relief of spinal syndromes.
Therapeutic methods
Since the fifth vertebra is the most affected, there is a violation in the processes of urine diversion, fecal incontinence, disruption of the sphincters. First of all, catheterization of the genitourinary tract is carried out with a sterile catheter, measures are taken to relax, or vice versa, to restore the work of the rectal sphincter.
Methods of physiotherapy, electrotherapy of the bladder, novocaine blockades and taking adrenoblockers restore the functioning of the bladder.
In some cases, these methods may not be effective because the spasm may be long and severe. In this case, they first try to relax the sphincter by administering botulinum toxin. It temporarily destroys the muscle, urgently relieving spasm. In this case, this is much more important, because due to prolonged retention of urine, intoxication of the body can develop, which will lead to death.
Surgical interventions
If this method is not applicable, then resort to surgical intervention. Often, to prevent intoxication and damage to the kidneys, a stoma is placed from the bladder. A stoma is an artificial opening between an organ and the environment. For her, they can use the tissues of the appendix, which have an affinity for the body and will not be rejected.
If the sphincters are damaged, plastic and bioorganic implants can be placed to replace it and allow you to restore the function of the bladder and rectum.
After the restoration of the urinary system, they begin to restore the spinal cord and the split vertebrae. These surgeries are considered difficult because any surgical interventions in the spinal cord are potentially dangerous due to the development of bacterial invasions under the membranes.
An injured vertebra is repaired with implants that form the normal appearance of the vertebra. At the same time, in addition to restoring the arch, they often replace the spinal disc, since during the time of the violation, destructive changes often begin in the fibrous ring.

After surgery, such patients are almost always severe, they are prescribed strict bed rest with a position “on the stomach” or “on the side”, since the suturing of the skin of the spine and healing takes a long time. Patients after such an operation require special and careful care.
Splits in the thoracic and cervical vertebrae
If the splitting process occurred in a higher section, therapy of the gastrointestinal tract must be added to the restoration of the bladder. Often, the patient is transferred to intravenous nutrition at this time, since the violations affect the enzymatic part of the stomach and duodenum, as well as intestinal motility disorders.