Spina bifida

Children's relies upon the combined expertise and experience of pediatric neurosurgeons, orthopedic surgeons and urologists to treat spina bifida. We feel strongly, and experience has shown, that our interdisciplinary approach provides the best possible chance for your child, living with spina bifida, to successfully grow and to flourish.

About our Spina Bifida Program

The Spina Bifida Program was created to serve 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.

Our team includes experienced, dedicated professionals who work together to provide and recommend care and education that benefits each child. The staff is committed to maximizing each child's quality of life.

We provide a team approach when evaluating patients by utilizing a multidisciplinary care model. During clinic visits, each patient will meet with a variety of specialists that will address patient and family concerns, gather information, examine the child, review results and recommend a plan of care.

Our specialists

  • Our Advanced Practice Nurse and clinic coordinator for the Spina Bifida program is available by calling (414) 266-2690. Her roles include teacher, case manager, advocate and consultant. The Advanced Practice Nurse also provides prenatal counseling for families who know their baby will be born with spina bifida. She also assists with care management when patients are having procedure or receiving care within the hospital. The Advanced Practice Nurse will also assist with coordinating appointments, triaging calls from parents, completing employment or insurance paperwork and referring patients to other specialties within Children's Hospital of Wisconsin.
  • Pediatric neurosurgeons will perform the initial surgery and continue to monitor the spinal cord and brain.
  • Urologists work to protect kidney function and specialize in the urinary tract and bowel function.
  • Orthopedists specialized in problems of the bones, including the spinal column.
  • Physical medicine and rehabilitation address issues related to growth and development, wheelchair positioning, bracing and seating.
  • Our social worker will help families cope and connect with community resources and government services.
  • Our dietitian works with nutritional issues and problems related to growth, weight and provided education and counseling on daily nutritional choices.

A wheelchair specialist is also available.

Clinic appointments

Appointments for the Spina Bifida Program can be made by calling (414) 266-4840. The program takes place on the second and third Tuesday of every month. A confirmation letter will be sent to the home prior to the appointment.
You can find us on the first floor of the Clinics Building in suite B140.

What is spina bifida?

Spina bifida refers to a collection of spinal birth defects and is also known as myelodysplasia or spinal dysraphism and is the most common neural tube defect (NTD). Learn more about spina bifida.

Spina bifida occurs when the fetal spinal cord fails to properly close before birth. Neural tube disorders are problems that relate to the developing brain and spinal cord while a child is still in the womb, and are among the most common and most serious of birth defects.

Spina bifida has three forms: spina bifida occulta, meningocele, and myelomeningocele, which range from mild to severe. While we don’t know what causes spina bifida, it appears to be inherited and may also relate to dietary and environmental factors.

Signs of spina bifida

Today, diagnostics have advanced such that we can determine before birth whether a child will be born with some form of spina bifida, though it helps to understand what to expect.

  • An abnormal appearance to the back, which may range from a hairy path, birthmark or dimple to a sac that pops out from the spine
  • No feeling below any unusual marks on the back
  • Paralysis of the legs
  • Constipation or incontinence
  • Hydrocephalus – “water on the brain”
  • Bone problems
  • Learning disabilities

Spina bifida education and prevention

The spina bifida program team at Children’s knows that education plays an important role in disease prevention. Our team members participate in extensive outreach in order to educate the community about the condition and lifestyle factors that can help prevent neural tube disorders. We visit schools and community agencies and offer prenatal counseling for families who know their baby will be born with spina bifida.

Children’s also cares for the entire patient family by displaying a large communication board in our clinic to keep you informed on the latest advances in treatment and care, as well as activities and announcements for families living with spina bifida.

A spina bifida resource room is available on clinic days and spina bifida family forums, which include parent panels, are offered periodically on a variety of topics.

Diagnosis & treatment

Children's uses blood tests, prenatal ultrasounds and amniocentesis to diagnose your child. Early detection is important because it prepares you and your family for the challenges ahead that come with welcoming a child with spina bifida.

Treatment for spina bifida begins at birth or, sometimes, before. 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 sometimes the brain), complications are not uncommon.

The specialists at Children’s perform careful assessments of your child in order to determine what body systems are effected and to what degree. Depending on the results of the assessments, and your input as the parent, specialized treatments may include catheterization to help with urine elimination, bowel programs and even the use of therapeutic electrical stimulation and biofeedback.

Surgery is often recommended, especially in moderate to severe cases that leave a significant amount of the delicate spinal cord exposed to the outside world.

In some cases, it’s possible to treat spina bifida before a baby is born. The Fetal Concerns Center at Children’s now performs prenatal spina bifida surgery to repair the spinal defect while the baby is still in the womb. Studies show that prenatal surgery can provide significantly better outcomes than traditional surgery post-birth. However, it may not be an option in every case. Doctors at the Fetal Concerns Center will help you decide whether prenatal surgery is the right option for you and your baby.

What are the types of spina bifida?

The types of spina bifida include the following:

  • 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 area of the defect. Other times, there may be no abnormalities in the area.
  • Meningocele - a moderate form of spina bifida in which a fluid-filled sac is visible outside of the back area. 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 body and are contained in a fluid-filled sac that is visible outside of the back area. These babies 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, causing pressure inside of the head to increase and the skull bones to expand to a larger than normal size.
    Approximately 85 percent of defects are found in the lower back area. The remaining 15 percent of the defects are located in the back of the neck or upper back areas.

What causes spina bifida?

Spina bifida is a type of neural tube defect. 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, called the neural tube. If all or part of the neural tube fails to close, leaving an opening, this is known as an open neural tube defect (or ONTD). This opening may be left exposed (80 percent of the time), or covered with bone or skin (20 percent of the time).

Anencephaly and spina bifida are the most common types of ONTD, while encephalocele (in which there is a protrusion of the brain or its coverings through the skull) is much rarer. Anencephaly occurs when the neural tube fails to close at the base of the skull, while spina bifida occurs when the neural tube fails to close somewhere along the spine.

In over 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. For this reason, ONTDs are considered multifactorial traits, meaning "many factors," both genetic and environmental, contribute to their occurrence.

Some of the environmental factors that may contribute to ONTDs include uncontrolled diabetes in the mother, and certain prescription medications. According to the Centers for Disease Control and Prevention (CDC), the occurrence rate of ONTDs can vary from state to state and from country to country. The rate of ONTD occurrence in Arkansas is 7.8 out of 10,000 births and 30 out of 10,000 births in Washington. Ireland has the highest number of documented cases, where spina bifida occurs in approximately 4.2 out of 1,000 live births. The factors causing these differences are not well understood.

ONTDs are seen five times more often in females than males. Once a child with an ONTD has been born in the family, the chance for an ONTD to occur again is increased to 3 to 5 percent. It is important to understand that the type of neural tube defect can differ the second time. For example, one baby could be born with anencephaly, while a second baby could have spina bifida (not anencephaly).

Prevention of neural tube defects

Because the neural tube closes 28 to 32 days after conception and before many women are aware they are pregnant, normal development of the brain and spinal cord may be affected during these first three to eight weeks of pregnancy by the following:

  • Genetic problems
  • Exposure to hazardous chemicals/substances
  • Lack of proper vitamins and nutrients in the diet
  • Infection
  • Prescription drug and alcohol consumption

Although many factors are related to the development of spina bifida, research has found that folic acid (vitamin B-12), a nutrient found in some green, leafy vegetables, nuts, beans, citrus fruits, and fortified breakfast cereals, can help reduce the risk of neural tube defects. For this reason, the American College of Medical Genetics (ACMG) and the Centers for Disease Control and Prevention (CDC) recommend that all women of childbearing age take a multivitamin containing folic acid. If a couple has had a previous child with an ONTD, a larger amount of folic acid is recommended and can be prescribed by the woman's physician or healthcare provider. This allows the woman to take it for one to two months prior to conception, and throughout the first trimester of pregnancy, to reduce the risk of another child with ONTD.

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. It is thought that a poor diet, lacking essential vitamins and minerals, may be a contributing factor)

What are the symptoms of spina bifida?

The following are the most common symptoms of spina bifida. However, each baby may experience symptoms differently. Symptoms may include:

  • Abnormal appearance of the baby's back, varying from a small, hairy patch or a dimple or birthmark, to a sac-like protrusion that is found along the back bone area
  • Bowel and bladder problems (i.e., constipation, incontinence)
  • Loss of feeling below the area of the lesion, especially in babies born with a meningocele or myelomeningocele.
  • Inability to move the lower legs (paralysis)

The baby may also have other problems related to spina bifida that include the following:

  • Hydrocephalus (increased fluid and pressure in the head area; occurs in about 80 to 90 percent of cases)
  • Orthopedic (bone) problems
  • Subtle learning problems

The symptoms of spina bifida may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis.

How is spina bifida diagnosed?

Diagnostic tests can be performed during pregnancy to evaluate the fetus for spina bifida. The tests include the following:

  • Blood tests - The American College of Obstetrics and Gynecology (ACOG) recommends that a blood test be offered between 15 to 20 weeks to all women who are pregnant who have not previously had a child with an ONTD and who do not have a family history of ONTD. This blood test 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 a protein normally produced by the fetus that crosses the placenta into the mother's blood. Generally, if a fetus has an ONTD, the alpha-fetoprotein level in the mother's blood will be increased. Although this test does not tell for certain whether a fetus has an ONTD, it will determine which pregnancies are at greater risk, so that additional testing may be performed.
  • Prenatal ultrasound (Also called sonography) - a diagnostic imaging technique which 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, and may be used to examine other organs and body systems of the fetus.
  • 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.

Management of spina bifida

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. Specific management of spina bifida will be determined by your baby's physician based on:

  • Your baby's gestational age, overall health, and medical history
  • The extent and type of spina bifida
  • Your baby's tolerance for specific medications, procedures, or therapies
  • Expectations for the course of spina bifida
  • Your opinion or preference

A cesarean delivery is often performed to decrease the risk of damage to the spinal cord that may occur during a vaginal delivery. 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.

Pediatric spina bifida surgery

Surgery can help manage the problems, but it can not restore muscle function or sensation to a normal state. Surgical interventions may be needed for the following:

  • Repair and closure of the lesion
  • Treatment of hydrocephalus
  • Orthopaedic problems - Orthopaedic problems may include curvatures in the back, hip dislocations, ankle, and foot deformities, and contracted muscles. Babies and children with spina bifida are also very susceptible to breaking their bones since their bones may be weaker than normal.
  • Bowel and bladder problems - Bowel and bladder problems may require surgery to improve function in elimination, for incontinence, constipation, or when the bladder does not empty completely.

Following pediatric spina bifida surgery, you will receive instructions on caring for your baby at home. Education may include the following:

  • Examining the skin, especially over bony areas such as the elbows, buttocks, back of the thighs, heel, and foot areas. Recommendations may include changing your baby's position frequently to prevent skin breakdown and pressure sores.
  • Promoting bowel and bladder function
  • Ways to feed your baby and monitor your baby's nutrition
  • Promoting activity and mobility
  • Encouraging age-appropriate growth and development

Not all babies will require surgical repair of spina bifida. Non-surgical management of spina bifida may include the following:

  • Rehabilitation
  • Positioning aids (used to help the child sit, lie, or stand)
  • Braces and splints (used to prevent deformity, promote support or protection)
  • Medications

Latex precautions

Babies with spina bifida are at high risk for developing a latex allergy due to exposure to latex from multiple medical and surgical procedures. Precautions are taken by the healthcare team to reduce the baby's exposure to products that contain latex. Your baby's healthcare providers can help you identify products that contain latex and also find products that are latex-free.

Life-long considerations

Since spina bifida is a life-long 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 will encourage the child to strengthen his/her self-esteem and promote as much independence as possible.

The full extent of the problem is usually not completely understood immediately after birth, but may be revealed as the child grows and develops.

Future pregnancies

Genetic counseling may be recommended by your physician to discuss the risk of recurrence in a future pregnancy, as well as vitamin therapy (a prescription for folic acid) that can decrease the recurrence risk for ONTDs. Supplemental folic acid, a B vitamin, if taken one to two months prior to conception and throughout the first trimester of pregnancy, has been found to decrease the reoccurrence of ONTDs for couples who have had a previous child with an ONTD. You cannot obtain the proper amount of folic acid in a multivitamin. A prescription from an obstetrician or other healthcare provider is needed in order to receive the proper dosage.