Spina Bifida

Prep for fetal surgery involves kickball, chicken breasts

Spina Bifida | Johns Hopkins Medicine

To rehearse for complex surgery on the spinal cords of fetuses, surgeons are practicing on kickballs and chicken breasts.

These items, combined with high-tech 3D printing technology, are providing surgical teams with the practice they need to perform a complex but minimally invasive new surgical repair of a particular form of spina bifida.

“A kickball is about the size of a uterus at that time in pregnancy.”

Spina bifida is the failure of the spinal column to close normally during early fetal development. It occurs in about three to four of every 10,000 pregnancies. It can result in permanent nerve damage if left untreated.

The new procedure, called fetoscopic myelomeningocele repair, involves a maternal fetal medicine specialist and a pediatric neurosurgeon working together. A handful of hospitals, including Johns Hopkins Hospital, have adopted the surgery. They use two small ports rather than an open, large incision in the womb of the mother carrying the affected fetus.

Training has been challenging, the team at Johns Hopkins reports. To address the difficulty, they prepared for their first such procedure last year on the fetus of a 31-year-old woman 25 weeks pregnant by creating practice models.

The surgeons used a 10-inch diameter kickball secured to a Plexiglas base to mimic a uterus.

“A kickball is about the size of a uterus at that time in pregnancy,” says Jena L. Miller, assistant professor of gynecology and obstetrics at the Johns Hopkins University School of Medicine. “It holds its shape pretty well when sealed and, un other trainers for laparoscopic surgery, it’s not see-through, so it’s a more realistic model.”

A doll in the kickball

The surgeons used ultrasound to obtain an accurate image of the fetal spine and lesion. Then, they created mesh models of the region to be operated on and generated a 3D-printed model of the area using flexible materials.

To practice, the team cut two slits in the top of the kickball to serve as ports for surgical instruments. Inside the kickball, they had placed the 3D-printed model with its silicone cover secured to a plastic fetus over a layer of marbles to mimic the intraoperative motion and instability of the fetus.

Besides practicing on the 3D-printed model, they also used a section of a skin-on chicken breast secured to a model of a fetus—placed inside the kickball—to get a better sense of touch for operating on multiple layers of tissue. This was helpful, Miller says, because chicken has more realistic properties than the 3D printed model.

Seven live surgeries

The more common, standard approach to spina bifida repair is to close the spine as soon after birth as possible, Miller says. For some patients, though, prenatal—or fetal—surgery is performed by making an incision on the mother’s abdomen and womb to expose the baby’s back, close the spinal opening, sew up the womb and maternal abdomen, and let the pregnancy continue.

Although that approach can successfully reduce the risk of spinal cord damage and disability, it carries risks for the mother’s health and her ability to sustain future pregnancies. Johns Hopkins is using the new approach instead of this more invasive form of fetal surgery.

The first live surgery went well, and the team was able to make a complete watertight closure without complications. The woman had a vaginal delivery at term and the newborn has not required any additional procedures. The team has since used the technique to prepare for six additional cases.

Surgery and stem cells might cure spina bifida

“Repetitive practice by a dedicated surgical team in a patient-matched model lets us know exactly what to anticipate specific to each case,” Miller says. The goal of rehearsals, Miller adds, is to identify potential obstacles and decrease operating time and risks.

While the new procedure is promising, the surgeons caution that more study is needed to improve training, continue advancing the surgical technique and reduce surgical time and potential risks.

The surgical team described its approach in a letter in the journal Ultrasound in Obstetrics & Gynecology. Coauthors of the letter were from Johns Hopkins and the US Army’s Medical Modeling and Simulation Innovation Center in Frederick, Maryland. The Fetal Health Foundation paid for the work.

Source: Johns Hopkins University

Source: https://www.futurity.org/spina-bifida-fetal-surgery-1714812-2/

In utero surgery rescues neurological function at birth in sheep with spina bifida

Spina Bifida | Johns Hopkins Medicine

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Spina Bifida | Johns Hopkins Center for Fetal Therapy

Spina Bifida | Johns Hopkins Medicine
 

Spina bifida is a common birth defect caused by incomplete closure of the spine in the womb. 

Spina Bifida Treatment: Why Choose Johns Hopkins

  • We understand the urgency of a spina bifida diagnosis. The Center for Fetal Therapy is available to take your call at any time and will see you as soon as possible.
  • Before choosing a treatment strategy, we counsel our patients on all options available to them for the management of spina bifida, from prenatal surgery to postnatal management. We work collaboratively with our patients to create a care plan that works best for mother, child and family.
  • Our fetal surgeons are leading experts in prenatal closure techniques for spina bifida, including minimally invasive fetoscopic repair of myelomeningocele.
  • Our team is at the forefront of research into advanced treatments. We were one of the first centers to recognize the benefits of prenatal closure, and our research shows that when the defect is repaired before birth by fetal surgery, infants are less ly to require shunt treatment for hydrocephalus. These infants also have improved nerve function and development compared with infants who receive standard surgery after birth.
  • Your child will receive coordinated, long-term care from the many advanced care specialties of Johns Hopkins Children’s Center. Our experts in advanced brain imaging, pediatric neurosurgery and neonatal-perinatal medicine, among others, will support your family from diagnosis through delivery and childhood care.

Request an appointment phone 410-502-6561

Patient Story: Fetoscopic Surgery for Spina Bifida

Fetoscopic Surgery for Spina Bifida | Keri’s Story

At 21 weeks pregnant, Keri and Scott learned their unborn child, Harper, had a defect in her spinal column known as spina bifida.

Wanting the best possible outcome for her, they elected for fetoscopic surgery.

Learn how the expert teams at the Johns Hopkins Center for Fetal Therapy and Johns Hopkins Children’s Center ensured both mom and baby were cared for from surgery through delivery and beyond.

Spina Bifida Treatment: What to Expect

At your first appointment, you will meet with our multispecialty team who will perform a detailed evaluation to determine the severity of the defect. Diagnostic procedures may include:

  • Fetal ultrasound
  • Genetic counseling
  • MRI

Following your initial consultation, we will review the results of these tests and your treatment options with you. Then we will work together to develop a care plan that is best for you.

If you are a good candidate for fetal surgery, surgical treatment options may include minimally invasive fetoscopic surgery or open surgery.

The goal of surgery is to restore normal anatomy as much as possible and treat any of the secondary effects of spina bifida such as hydrocephalus.

Whether spina bifida repair is performed before or after birth, your child will require a long-term care team who specializes in managing spina bifida.

Learn more about spina bifida repair before birth.

Our Spina Bifida Specialists

Drs. Rosner, Baschat and Miller of the Johns Hopkins Center for Fetal Therapy.

Our multidisciplinary team includes fetal medicine specialists and surgeons, pediatric neurosurgeons, neonatologists, pediatric plastic surgeons, orthopaedic surgeons, urologists, pediatric physical medicine therapists, nurses, genetic counselors and social workers who work together to ensure your child receives the full-spectrum of spina bifida care to give him or her the best quality of life possible.

Fetal Medicine & Surgery

Jena Miller, M.D.
Ahmet Baschat, M.D.
Mara Rosner, M.D., M.P.H.

Anesthesiology

David Berman, M.D.
Jamie Murphy, M.D.

The Johns Hopkins Center for Fetal Therapy research team.

Johns Hopkins experts have been at the forefront of research into the benefits and proven outcomes of prenatal spina bifida repair and, particularly, of the use of minimally invasive fetal endoscopic surgery (fetoscopy), for both mom and baby. Our research includes:

Source: https://www.hopkinsmedicine.org/gynecology_obstetrics/specialty_areas/fetal_therapy/conditions-we-treat/spina_bifida.html

ICTR in the News: Kickballs, Chicken and 3-D Models Help Johns Hopkins Surgeons Prepare for Complex Fetal Surgeries

Spina Bifida | Johns Hopkins Medicine

Kickball used to mimic womb Credit: Johns Hopkins Medicine

By combining high-tech 3-D printing technology with everyday items such as a kickball and pieces of chicken breast, surgeons at Johns Hopkins report they have devised an innovative way to “rehearse” a complex minimally invasive surgical repair of open lesions on fetal spinal cords inside the womb.

The procedure, called fetoscopic myelomeningocele repair, is performed by a maternal fetal medicine specialist and pediatric neurosurgeon working together to correct a particular form of spina bifida, a birth defect marked by failure of the spinal column to close normally during early fetal development. It occurs in about three to four of every 10,000 pregnancies and can result in permanent nerve damage if left untreated.

The standard of care for the condition is surgery to close the spine as soon after birth as possible, says Jena L. Miller, M.D., an assistant professor of gynecology and obstetrics at the Johns Hopkins University School of Medicine and member of the Johns Hopkins Center for Fetal Therapy.

For select patients, prenatal—or fetal—surgery is performed by making an incision on the mother’s abdomen and womb to expose the baby’s back, close the spinal opening, sew up the womb and maternal abdomen, and let the pregnancy continue.

Although that approach can successfully reduce the risk of spinal cord damage and disability, it carries risks for the mother’s health and her ability to sustain future pregnancies.

A handful of hospitals, including The Johns Hopkins Hospital, are performing the surgery minimally invasively through two small ports rather than through open, large incisions on the maternal womb.

But, the Johns Hopkins surgeons say, training to do it has been challenging. To address the difficulty, a Johns Hopkins team prepared for its first such procedure last year on the fetus of a 31-year-old woman 25 weeks pregnant by creating models on which to practice.

The work is described in a letter to the editor published online Aug. 29, 2017, in the journal Ultrasound in Obstetrics & Gynecology.

First, the surgeons report, they used ultrasound technology to obtain an accurate image of the fetal spine and lesion.

Then, they created mesh models of the region to be operated on and generated a 3-D print model of the area using flexible materials such as Tango (a material with rubber properties) and NinjaFlex (a filament with elasticity), adding a combination of silicones to produce a skin cover.

The surgeons also used a 10-inch diameter kickball secured to a Plexiglas base to mimic a uterus. “A kickball is about the size of a uterus at that time in pregnancy,” Miller says. “It holds its shape pretty well when sealed and un other trainers for laparoscopic surgery, it’s not see-through so it’s a more realistic model.”

For their practice sessions, the team cut two slits in the top of the kickball to serve as ports for surgical instruments. Then, inside the kickball, they placed the 3-D printed model with its silicone cover secured to a plastic fetus over a layer of marbles to mimic the intraoperative motion and instability of the fetus. Then they practiced the surgical steps in that environment.

Besides practicing on the 3-D printed model, they also practiced on a section of a skin-on chicken breast secured to a model of a fetus—placed inside the kickball—to get a better sense of touch for operating on multiple layers of tissue. This was helpful, Miller says, because chicken has more realistic properties than the 3-D printed model.

During their planning, the team members also used a variety of instruments, suture materials and techniques until they achieved consistent times and outcomes for the individual steps of the operation.

Their first live surgery went well, consistent with their practice on the 3-D model, and they were able to make a complete watertight closure without complications. The woman had a vaginal delivery at term and the newborn has not required any additional procedures.

The team has since used the technique to prepare for six additional cases.

“Repetitive practice by a dedicated surgical team in a patient-matched model lets us know exactly what to anticipate specific to each case,” Miller says.

The goal of such rehearsals, Miller adds, is to pre-identify potential obstacles, and decrease operating time and risks.

While the new procedure is promising, the surgeons caution that more study is needed to improve training, continue advancing the surgical technique, and reduce surgical time and potential risks of needing to convert to open fetal surgery.

Coauthors of the editorial were Edward Ahn, Juan R. Garcia, Andrew Satin and Ahmet A. Baschat of Johns Hopkins, and G T. Miller of the U.S. Army Medical Research and Materiel Command’s Medical Modeling and Simulation Innovation Center in Frederick, Maryland.

The work was supported by the Fetal Health Foundation.

For the Media

Source: https://ictr.johnshopkins.edu/news_announce/ictr-in-the-news-kickballs-chicken-and-3-d-models-help-johns-hopkins-surgeons-prepare-for-complex-fetal-surgeries/

Guidelines for the Care of People with Spina Bifida Preface

Spina Bifida | Johns Hopkins Medicine

Guidelines for the Care of People with Spina Bifida
 – An Initiative of the Spina Bifida Association

Preface to the Fourth Edition

Spina Bifida is the most commonly-occurring complex congenital birth defect associated with long-term survival.

With this understanding, along with the knowledge of the multiple medical and psychosocial issues that people with Spina Bifida face, the Guidelines for Spina Bifida Health Care Services Throughout the Lifespan were first published by the Spina Bifida Association of America (now known as the Spina Bifida Association, SBA) in 1990 and revised in 1995.

Both editions were the culmination of several years of work by SBA’s Professional Advisory Council (PAC), as well as numerous consultants under the editorial leadership of Karen Rauen, RN, MSN. These guidelines were limited contemporary knowledge and expert opinion.

Research on outcomes in Spina Bifida has been sparse.

In that light, a symposium entitled: Evidence-Based Practice in Spina Bifida: Defining a Research Agenda was convened May 9-10, 2003 to identify the current evidence related to Spina Bifida, identify research gaps and priorities, and to foster new directions and funding for research.

Sponsors included the Centers for Disease Control and Prevention, Agency for Healthcare Research and Quality, the National Institutes of Health (Office of Rare Diseases), and the U. S. Department of Education.

Additional supporting agencies included the National Institute of Child Health and Human Development, the Interagency Committee on Disability Research, and the Spina Bifida Association. (A summary manuscript, edited by Gregory Liptak, MD, MPH, is available from the Spina Bifida Association, 1600 Wilson Blvd, Suite 800, Arlington, VA 22209.)

This meeting highlighted that much of the research in Spina Bifida was case series; very few randomized control trials or representative cohort studies had been performed on any topic on people with Spina Bifida. Research related to adults with Spina Bifida was nearly nonexistent.

The primary goal of the evidence-based review was achieved: directions for research were clarified. The third edition of the Guidelines for Spina Bifida Health Care Services Throughout the Lifespan, edited by Mark Merkens, MD, was published in 2006 by SBA.

The guidance included in the third edition was reviews generated from the evidence-based conference as well as expert consensus.

Guidelines for the Care of People with Spina Bifida are the fourth edition of the Guidelines, and the result of three years of planning, literature review and content development by nearly 100 volunteers.

The new Guidelines were needed to ensure that all people living with Spina Bifida receive the best and most up-to-date care possible and because previous versions did not have robust coverage of the care needs of adults.

Additionally, the fourth version features a new title that reflects greater respect and understanding for the people who are impacted by living with Spina Bifida.

In other words, the fourth edition Guidelines were developed to treat and care for the people who live with Spina Bifida, not just the conditions associated with this birth defect. Finally, this fourth edition features a number of new topics, including Transition and Quality of Life, important to the health and well-being for all people living with Spina Bifida.

Despite the efforts resulting from the “Evidence-Based Practice in Spina Bifida: Developing a Research Agenda” conference in 2003, the extensive literature review was done for the fourth edition of the Guidelines continues to identify that research in Spina Bifida remains limited. Where evidence exists, it is included.

For other recommendations, the collective judgment of expert working groups determined the appropriateness of assessments and interventions to be considered. The workgroups used the consensus-building methodologies of Single Text Procedure and Nominal Group Techniques.

These recognized guidelines development methodologies allow the inclusion of expert opinion for aspects of care for which medical evidence does not exist or is not robust.

Since the publication of the third edition of the Guidelines, there have been advances in health care service delivery concepts related to improving the care of children with medical complexity, including Spina Bifida. These concepts will be important in ensuring the full implementation of the fourth edition of the Guidelines for the Care of People with Spina Bifida.

The first is that care coordination is an essential component of health care delivery.

At the core, patient- and family-centered care within a medical home is a foundational component; outcomes are optimized when there is cross-sector collaboration among the multiple medical systems and providers, community services, and support agencies with whom families and people with Spina Bifida interact.

While effective care coordination typically requires dedicated paid personnel, care coordination activities are not the sole responsibility of a single individual or provider. Rather, all people who interact with patients and families have a role to play in care coordination.

The second concept, in the context of patient- and family-centered care, is that for people with Spina Bifida, care providers may be provided via a medical neighborhood with team-based care.

Within this framework is co-management with defined roles, data sharing, and collaborative care protocols among primary care, community-based services, and subspecialty care.

Full implementation of these Guidelines to optimize outcomes for people with Spina Bifida cannot rest with the Spina Bifida clinic alone. Indeed, guidance provided on many topics should be implemented through primary care providers and efforts of community services.

While the Spina Bifida clinic may direct the overall health care planning in many cases, optimal care is best achieved as a partnership between families and people with Spina Bifida, primary and subspecialty care providers, health systems, and community services.

These Guidelines were developed to serve people with Spina Bifida and those who care for them. It is essential to remember that several factors influence how an individual or family member uses the education and written information they are provided.

This is imperative, particularly when reaching across potential obstacles such as cultural and/or language differences. It is known that the dynamics that modify the incidence of Spina Bifida are multifactorial, such as the well-documented higher incidence of Spina Bifida among people of Hispanic origin.

Thus, it is increasingly critical for health care and community service providers to consider how a family’s language, level of acculturation, and cultural constructs of care (e.g.

, the concept of self-management and independence from others) directly influence their understanding and reception of the health care message along with their willingness to change behavior.

10 Moreover, since over 20% of the US population older than five years of age speaks a language other than English at home, when possible, all families with limited English proficiency ought to be supported with additional health care navigation services, along with oral and written information provided in their preferred language.11

Executive Committee

  • Timothy J. Brei, MD, Spina Bifida Association Medical Director; Developmental Pediatrician, Professor, Seattle Children’s Hospital
  • Sara Struwe, MPA, Spina Bifida Association President & Chief Executive Officer
  • Patricia Beierwaltes, DPN, CPNP, Guideline Steering Committee Co-Chair; Assistant Professor, Nursing, Minnesota State University, Mankato
  • Brad E. Dicianno, MD, Guideline Steering Committee Co-Chair; Associate Medical Director and Chair of Spina Bifida Association’s Professional Advisory Council; Associate Professor, Department of Physical Medicine and Rehabilitation, University of Pittsburgh School of Medicine
  • Nienke Dosa MD, MPH, Guideline Steering Committee Co-Chair; Upstate Foundation Professor of Child Health Policy; SUNY Upstate Medical University
  • Lisa Raman, RN, MScANP, MEd, former Spina Bifida Association Director, Patient and Clinical Services
  • Jerome B. Chelliah, MD, MPH, Johns Hopkins Bloomberg School of Public Health

Acknowledgments

Julie Bolen, PhD, MPH, Lead Health Scientist, Rare Disorders Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention

Adrienne Herron, PhD Behavioral Scientist, Intervention Research Team, National Center for HIV/AIDS, Viral Hepatitis, STD, and TB Prevention, Centers for Disease Control and Prevention

Judy Thibadeau, RN, MN, Spina Bifida Association Director, Research and Services; former Health Scientist, National Spina Bifida Program, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention

The development of these Guidelines was supported in part by Cooperative Agreement UO1DD001077, funded by the Centers for Disease Control and Prevention. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Centers for Disease Control and Prevention or the Department of Health and Human Services.

Source: https://www.spinabifidaassociation.org/resource/guidelines-for-the-care-of-people-with-spina-bifida-preface/

Spina Bifida

Spina Bifida | Johns Hopkins Medicine

Linkedin Pinterest Brain, Nerves and Spine Brain Tumor What You Need to Know

  • Spina bifida is term used to identify a set of conditions that affect the development of the spinal cord and vertebrae.
  • The three main types of spina bifida are myelomeningocele, lipomeningocele and spina bifida occulta.
  • Spina bifida is often diagnosed in utero before a baby is born.
  • Children with spina bifida are often cared for by a variety of medical professionals, including a neurologist, orthopaedist and physical therapist, among others.
  • Treatment may include surgery, bracing, physical therapy and other forms of medical care each child’s needs.

Spina bifida is a group of congenital conditions involving the failure of normal development of the spinal cord and vertebrae. There are three main types of spina bifida, the most severe being myelomeningocele and the most mild being spina bifida occulta. Spina bifida will often result in varying degrees of weakness to the legs.

This muscle imbalance can then lead to the development of musculoskeletal problems, such as hip dislocation, joint contractures and clubfeet.

Myelomeningocele

This is the most common defect of the developing brain and spinal cord, called the neural tube. This birth defect develops by the third week of fetal gestation. Myelomeningocele is a fluid-filled area formed by the lining of the spinal cord and a web of spinal nerves. A child born with myelomeningocele presents with a fluid-filled sac to the back.

There is most often a defect in the skin as well, with no skin covering the mass. The bones of the spine fail to develop and do not close around the spinal cord. The sac protrudes through this defect and contains spinal nerve roots.

The child born with myelomeningocele will have a degree of muscle weakness and differences in skin sensation related to the level of the spinal cord involved.

Lipomeningocele

This type of spina bifida is associated with an overlying fatty tumor. At birth, the skin is intact over the underlying spinal cord and vertebral anomaly. There is associated muscle weakness and decreased sensation, relevant to the level of the spinal cord involved. The lower the spinal cord defect, the higher the level of physical functioning.

Spina Bifida Occulta

Typically an incidental finding, spina bifida occulta rarely presents with symptoms. The lowest lumbar vertebra is found to have a small midline defect. This spinal cord and spinal contents are not affected in this condition.

A child with this finding on an X-ray is expected to have no physical limitations, muscle weakness or increased risk of developing progressive spinal pathology. There is no need for routine checkups or activity limitations in the absence of symptoms.

A child could grow up and never know that he or she has this defect.

What are the signs and symptoms of spina bifida?

Spina bifida is typically screened for during routine prenatal exams. Many cases of spina bifida are detected with prenatal sonogram and blood work.

  • A midline vertebral body defect or cystic spinal cord lesion

  • Elevated maternal alpha-fetoprotein

If the diagnosis is not made prenatally, the newborn baby may present with:

  • A fatty or fluid-filled mass to the lower back

  • A hairy patch or lumbosacral skin marking

  • Weakness in the legs

What are the risk factors of spina bifida?

Factors that increase the risk of a mother having a child with spina bifida include:

  • Having a child or sibling with spina bifida

  • Having spina bifida herself

  • Deficit of folate (folic acid) in the maternal diet before pregnancy

  • Exposure to certain medications while pregnant (valproic acid)

Spina Bifida Diagnosis

The open neural tube defects are typically diagnosed prenatally. Those not diagnosed through prenatal sonogram and maternal blood work are diagnosed by physical exam at birth.

Spina Bifida Treatment

In the cases where an open myelomeningocele is present, surgery by a neurosurgeon is done within the first 48 hours of life. This surgery involves closure of the defect.

The musculoskeletal imbalances resulting from spinal cord defect are managed with a combination of physical therapy for strengthening and stretching, braces to support the knees and ankles while standing, and surgery to correct any problem bony misalignment.

The development of pressure sores is common. Daily skin checks and avoiding pressure from sitting and walking without proper support are taught at an early age. Assistive devices are often used to support mobility.

There are many specialists involved in the care of a child with spina bifida. These children are often best managed through a multidisciplinary approach, involving neurosurgery, orthopaedics, urology, orthotics, physical therapy, occupational therapy, primary care, nutrition and neuropsychology.

More information about spina bifida from Kennedy Krieger Institute.

While some birth defects can be prevented through prenatal care, it's important to know what treatments may exist if your fetus is diagnosed with a birth defect.

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/spina-bifida