Spina bifida

The Fetal Concerns Center helps families like yours every day. We offer the latest diagnostic tools and treatment options for spina bifida. From diagnosis to birth, the Fetal Concerns Center uses a team approach to teach and support families.

The Spina Bifida Program at Children’s Wisconsin was created to serve pediatric patients with all levels of spina bifida and related conditions. Our goal is to help children with spina bifida stay healthy, gain independence and become confident in their abilities.

What is spina bifida?

Spina bifida is a defect in the development of the spine, resulting in a lack of skin and tissue covering the spinal cord. The neural tube usually closes by the fourth week of pregnancy, but in spina bifida, the neural tube does not close completely, leaving the baby’s delicate spine open to possible injury. A neural tube defect is among the most common and serious of birth defects, and it can occur anywhere along the backbone. The types of spina bifida include:

• Spina bifida occulta — a mild form of spina bifida in which the spinal cord and the surrounding structures remain inside the baby, but the back bones in the lower back area fail to form normally. There may be a hairy patch, dimple or birthmark over the affected area. Other times, there may be no visible abnormalities.
• Meningocele — a moderate form of spina bifida in which a fluid-filled sac is visible outside of the baby’s back. The sac does not contain the spinal cord or nerves.
• Myelomeningocele — a severe form of spina bifida in which the spinal cord and nerves develop outside of the baby’s body and are contained in a fluid-filled sac that is visible outside of the back. These children typically have weakness and loss of sensation below the defect. Problems with bowel and bladder function are also common. A majority of babies with myelomeningocele will also have hydrocephalus, a condition that causes the fluid inside of the head to build up, increasing pressure and expanding the skull bones to a larger than normal size. 

Approximately 85 percent of neural tube defects occur in the lower back area; 15 percent are located in the back of the neck or upper back areas.

What causes spina bifida?

A neural tube defect (meningocele or meningomyelocele) can occur anywhere along the backbone. This illustration provides the names and locations of the vertebral column bones.

Although we don’t know what causes this condition, it appears to be inherited in some cases, and it may also involve dietary and environmental factors. Neural tube defects, including spina bifida (open spine) and anencephaly (open skull), are seen in one to two out of 1,000 live births.

During pregnancy, the human brain and spine begin as a flat plate of cells, which rolls into a tube. If all or part of the neural tube fails to close, leaving an opening, this is known as an open neural tube defect (ONTD). In more than 90 percent of cases, an ONTD occurs without a prior family history of these defects. ONTDs result from a combination of genes inherited from both parents, coupled with environmental factors. Environmental factors that may contribute to ONTDs include uncontrolled diabetes in the mother, certain prescription medications and a lack of folic acid. ONTDs are seen five times more often in females than males.

Additional risk factors include:

• Maternal age (spina bifida is more commonly seen in teenage mothers)
• History of miscarriage
• Birth order (first-born infants are at higher risk)
• Socioeconomic status (children born into lower socioeconomic families are at higher risk for developing spina bifida — possibly because their mothers are more likely to have a poor diet lacking essential vitamins and minerals)

Prenatal diagnosis of spina bifida

Illustration of a meningomyelocele or myelomeningocele which contains cerebral spinal fluid, meninges and spinal cord.

Early detection is important because it prepares your family for the challenges that come with welcoming a child with spina bifida. Your doctors may use the following prenatal tests to diagnose this condition:

• Blood tests — The American College of Obstetrics and Gynecology recommends a blood test for all pregnant women. This blood test, usually done between the 15th-20th weeks of pregnancy, measures alpha-fetoprotein (AFP) levels and other biochemical markers in the mother’s blood to determine whether her pregnancy is at increased risk for an ONTD. AFP is normally produced by the fetus and crosses the placenta into the mother's blood, and babies with an ONTD tend to have elevated levels of this protein. Although this test cannot tell for certain whether a fetus has an ONTD, it indicates whether further testing is necessary.
• Prenatal ultrasound (also called sonography) — a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues and organs. Ultrasounds are used to view internal organs as they function and to assess blood flow through various vessels. Prenatal ultrasound may be able to detect an ONTD in the majority of cases.
• Amniocentesis — a procedure that involves inserting a long, thin needle through the mother’s abdomen into the amniotic sac to withdraw a small sample of the amniotic fluid for examination. The fluid is then tested to determine the presence or absence of an open neural tube defect. Small or closed defects may not be picked up by this test.

Specialists you will work with

Spina bifida requires a multidisciplinary team of experts. Your child’s health care team could include:

• Maternal fetal medicine specialist (before birth)
• Fetal surgeon (if prenatal surgery is selected)
• Neurosurgeon
• Orthopedist
• Urologist
• Physical Medicine and Rehabilitation
• Nurse Specialist
• Physical/occupational therapist
• Dietician
• Social worker

How does spina bifida affect my baby?

Spina bifida ranges in severity. Some children have mild or no symptoms. Others have lifelong disability, including inability to control their bowel or bladder, paraplegia (little or no ability to move the legs) or quadriplegia (little or no ability to move the arms and legs). The most severe form of spina bifida is myelomeningocele. For a small percentage of children, spina bifida can even be fatal.

The primary goal of managing spina bifida is to prevent infection and to preserve the spinal cord and nerves that are exposed outside of the body. Children with spina bifida, especially those with both brain and spinal cord involvement, require complex medical care and neurosurgery accompanied by careful monitoring. Because spina bifida affects the spinal cord (and oftenthe brain), complications are not uncommon. Babies and children with spina bifida are also very susceptible to breaking their bones since their bones may be weaker than normal, and they may experience learning problems.

The specialists at Children’s perform careful assessments of your child in order to determine what body systems are affected and to what degree. Most often neurosurgery is required within 24-48 after birth to cover the delicate spinal cord exposed to the outside world.

Does my baby need a special hospital?

Babies born with a meningocele or a myelomeningocele usually require care in the neonatal intensive care unit (NICU) for evaluation and for surgery to close the defect, if it wasn’t closed prenatally. If you deliver at Froedtert, your baby will receive top-notch care in Children’s Wisconsin's Level IV Neonatal Intensive Care Unit, which is ranked as one of the best in the nation. We will closely monitor your baby, using ultrasounds to check his or her head, kidneys and bladder, and MRI to check the spine and brain. Your child might require a catheter to help with urine elimination or a shunt to drain excess fluid from the brain.

Prenatal surgery options

Historically, patients with spina bifida have been treated with surgery after birth, but it’s now possible to treat some cases while a baby is still in the womb. Studies show that prenatal surgery can provide significantly better outcomes than traditional surgery post-birth. 

Prenatal surgery is not a cure for spina bifida, and your child may still have problems with walking and urine/bowel function. Also, not every baby with spina bifida is a candidate for prenatal surgery. Learn more about fetal myelomeningocele repair.

How does spina bifida affect my pregnancy?

You will be closely monitored for the rest of your pregnancy. If you undergo prenatal surgery, you will need to remain on modified bed rest after the surgery to prevent the risk of preterm delivery, and you will need to deliver your baby via a scheduled cesarean section at 37 weeks, unless you go into labor earlier. Most Milwaukee-area patients continue to receive care at the Fetal Concerns Center and then deliver at the Froedtert & the Medical College of Wisconsin Birth Center, though in some cases it’s possible for mothers to receive follow-up care and deliver at another hospital closer to home. Your baby will stay in the hospital until he or she has recovered from surgery and is strong enough to go home.

How do you treat spina bifida?

Treatment starts at birth, if not earlier. Treatment will be informed by the medical team’s assessment of your child’s condition and your input as the parent. Surgery can help manage the problems associated with spina bifida, but it cannot restore muscle function or sensation to a normal state. Surgical interventions may be needed for:

• Repair and closure of the neural tube defect
• Treatment of hydrocephalus
• Orthopedic problems (such as curvatures in the back, hip dislocations, ankle and foot deformities, and contracted muscles)
• Bowel and bladder problems (surgery may be required toreduce peeing and pooping accidents, for incontinence or constipation, or when the bladder does not empty completely)

Not all babies require surgical repair of spina bifida. Nonsurgical management of spina bifida may include:

• Rehabilitation such as physical, occupational, or speech therapy
• Positioning aids (used to help the child sit, lie or stand)
• Braces and splints (used to help bones to grow straight, promote muscle support, or help with walking)
• Medications
• Catheterization to help with urine elimination by learning how to empty your baby’s bladder by putting in a tube; and helping your baby to poop by abdominal massage, bicycling his/her legs, or putting in a suppository (medicine that helps with pooping)
• Therapeutic electrical stimulation and biofeedback to help with bladder/bowel issues

What about after surgery?

Your child’s medical team will give you detailed instructions on caring for your baby at home. Education may include the following:

• Monitoring your baby’s alertness and feeling the soft spot on his/her head to watch for hydrocephalus
• Examining the skin, especially over bony areas such as the elbows, buttocks, back of the thighs, heel and foot areas
• Promoting bowel and bladder function by learning how to empty your baby’s bladder by putting in a tube (urinary catheterization); and helping your baby to poop by abdominal massage, bicycling his/her legs, or putting in a suppository (medicine that helps with pooping)
• Ways to feed your baby and monitor your baby's nutrition
• Promoting activity and mobility
• Encouraging age-appropriate growth and development

Babies with spina bifida are at high risk for developing a latex allergy due to exposure to latex from multiple medical and surgical procedures. The medical team takes precautions to reduce the baby's exposure to products that contain latex, and your baby's health care providers can help you identify products that contain latex and also find products that are latex-free.

Will I be able to help care for my baby?

Yes. Our recently expanded and redesigned NICU was created to promote family bonding, with private rooms where parents can stay with their baby 24/7, and other special features for families’ comfort. Our on-call lactation specialists can help you successfully breastfeed and pump and store breast milk when direct breastfeeding isn’t an option.

When can my baby go home?

After surgery, the incision on your baby’s back needs to heal before he or she can go home. Some babies may need only a brief stay in the NICU, while others may need more time in the hospital to resolve health issues. Premature babies will likely need a longer NICU stay. After discharge, your child will require ongoing monitoring by Children’s Wisconsin's multidisciplinary Spina Bifida Program or another clinic close to your home.

What’s my baby’s long-term prognosis?

The full extent of this condition’s effect on your child may not be completely understood immediately after birth; it may be revealed as your child grows and develops. Since spina bifida is a lifelong condition that is not curable, management often focuses on preventing or minimizing deformities and maximizing the child's capabilities at home and in the community. Positive reinforcement, anticipatory guidance, and parental/family support will encourage your child to promote as much independence and self-confidence as possible.

What about future pregnancies?

Once a child with an open neural tube defect has been born in the family, the chance for an ONTD to occur again is increased to 3 to 5 percent. The type of neural tube defect can differ the second time. Your doctor will likely prescribe high doses of folic acid (preferably before you conceive) to reduce the odds of your baby developing spina bifida. If you underwent prenatal repair, the surgical scar on your uterus puts you at increased risk of uterine rupture during childbirth. You will need to wait two years between pregnancies and deliver all future babies via planned cesarean section no later than 37 weeks.

Clinical Care Partner with the National Spina Bifida Association (SBA)

The Spina Bifida program at Children's Wisconsin is designated as a clinical care partner in collaboration with the national Spina Bifida Association (SBA). This designation from SBA supports the excellent care that children with spina bifida receive at Children's. To be designated as a partner, the program must achieve 10 standards of care which use best practices to promote the best outcomes for people with Spina Bifida. The standards address such issues as:

• Ensuring the use of the guidelines for care of children with Spina Bifida
• Coordination of care through a multidisciplinary team
• A partnership with a prenatal evaluation center
• A transition program to continue to support the health of adults with Spina Bifida.

Children's Wisconsin joins only 30 clinics worldwide who have this designation and are able to collaborate with other providers and SBA, and to share ideas with a national network of providers that have also been recognized as SBA clinic care partners.