Comprehensive Spina Bifida Treatment at Our Pediatric Neurology & Neurosurgery Program

The Pediatric Neurology & Neurosurgery Program at The Department of Neurosurgery at Rutgers Health and RWJBarnabas Health offers comprehensive care for the birth defect spina bifida, including it’s most severe form, myelomeningocele. Our board-certified pediatric neurologists, neurosurgeons, and other health care professionals use a multidisciplinary approach to give your child the best treatment possible to correct the defect and preserve existing spinal cord function.

Infants with myelomeningocele (pronounced my-uh-lo-muh-NING-go-seel) must be treated quickly and with the utmost care by a multidisciplinary team of physicians. Treatment for myelomeningocele involves surgery within the first 24 hours of life to close the spinal defect and preserve existing spinal cord function. Follow-up treatment is just as important, and most patients must have ongoing care throughout childhood. If your baby has been diagnosed with spina bifida in-utero, you have options for treatment, sometimes even before the baby is born. Our team of pediatric neurology experts can help you determine an individualized treatment plan for your baby and we ensure high-quality aftercare.

What Is a Myelomeningocele?

Myelomeningocele is a severe neurological condition where the spinal column and canal do not properly close before birth. This very rare birth defect occurs about once per every 800 births, according to the National Institutes of Health. It is the most severe form of spina bifida, which means “split spine” in Latin. While many cases of spina bifida only have mild symptoms, babies with myelomeningocele often have permanent nerve damage and other disabilities. These may include developmental delays, paralysis, lack of bladder and bowel control, Chiari II malformation, and as many as 90% of babies also have hydrocephalus (a buildup of excess fluid on the brain). In addition, babies with this condition are at high risk of life-threatening infections. For these reasons, it’s crucial for babies with myelomeningocele to undergo surgery within 24 hours of birth to minimize the risk of infection or bacterial meningitis, as well as to preserve existing function of the spinal cord.

A myelomeningocele defect can happen anywhere along the spinal cord, but is most common in the lower back in the lumbar and sacral areas of the spine. Nerve damage occurs below the level of the defect, and causes symptoms such as paralysis, numbness, or other loss of function. Usually, babies with higher myelomeningoceles have the most severe symptoms.

Myelomeningocele forms during the first month of fetal development, which means it is very difficult to successfully prevent it, as many women are unaware of being pregnant during the first month of gestation. The American College of Medical Genetics and the Centers for Disease Control and Prevention (CDC) recommend that women who are actively trying to conceive should take a multivitamin containing folic acid, as this may prevent spina bifida. This is because folic acid has been shown to help with proper development of the baby’s neural tube, which is what develops into the baby’s brain and spinal cord.

Babies with myelomeningocele exhibit the following:

  • Bones of the vertebrae do not form normally;

  • A small sac extends through the mid-to lower back, covered with a membrane, protruding through an opening in the spine;

  • The sac contains cerebrospinal fluid (CSF) and meninges (tissues that protect the spinal cord); and

  • The sac may also contain parts of the spinal cord, including nerves.

What Causes Myelomeningocele?

Myelomeningocele, like other forms of spina bifida, has no known cause. The reason the neural tube of the spine does not close in fetal development could be due to a variety of factors, but none are proven. Influencing factors could include genetics, maternal age, maternal nutrition, mothers with a history of miscarriage, and/or environmental factors. A woman who has had one child born with myelomeningocele has a slightly increased risk of having another child with the condition, however, there is still only a very remote chance.

How Is Myelomeningocele Diagnosed?

Prenatal Diagnosis
Often, myelomeningocele is diagnosed before the baby is born, either through a blood test called the quadruple screen, which checks for certain conditions, or through and ultrasound. Amniocentesis, a procedure in which amniotic fluid is extracted from the mother’s uterus, can confirm a diagnosis of myelomeningocele.

Postnatal Diagnosis
Physicians also find myelomeningocele after birth. Usually a diagnosis of spina bifida is apparent at birth, with the protrusion of the sac along the infant’s spine, but imaging tests can confirm the diagnosis. These tests may include X-ray, ultrasound, CT scans and MRI scans.

Myelomeningocele Symptoms

The main symptom of myelomeningocele is a small sac protruding from the mid- to lower back, and this is evident at birth. It contains a portion of the spinal cord and nerves and it is exposed through the skin.

The main symptoms of myelomeningocele include:

  • Partial or complete paralysis of legs

  • Partial or complete loss of sensation

  • Weakness of the hips, legs, and/or feet

  • Hydrocephalus (also called “open skull,” which involves excessive fluid buildup inside the skull and presents with an abnormally large head due to increased pressure causing skull bones to expand)

  • Chiari II malformation

In addition, it is common for spina bifida patients in general and myelomeningocele patients in particular to have learning difficulty or intellectual disability. This may be apparent if your baby has a short attention span, difficulty understanding or expressing language, or difficulty with math.

How Is Myelomeningocele Treated?

Infants born with myelomeningocele spina bifida require intensive care and cannot go home with their parents until their condition is stable and they have been cleared by the neonatal intensive care unit (NICU) doctors. Myelomeningocele must be treated quickly to minimize the risk of permanent brain damage, blindness, repeated seizures, and other disabilities.

Fetal Surgery for Myelomeningocele

In recent years, neurosurgeons have pioneered a new, experimental technique for correcting spina bifida during fetal development, before the 26th week of gestation. This type of surgery involves an incision similar to one made during a C-section, although the fetus is not removed from the uterus. The purpose of the surgery is to close the neural tube defect to prevent further loss of nerve function. Fetal surgery has been shown to have better results than performing the surgery on a baby after birth. However, this highly specialized surgery is not feasible for every patient. Those women who are candidates for this surgery should only see an experienced expert in fetal surgery in conjunction with neonatal intensive care.

Fetal surgery for myelomeningocele is a possibility for certain pregnant mothers. Patients who may be eligible for fetal surgery meet the following criteria:

  • Gestational age between 19 to 26 weeks

  • Defect must start between T1-S1 vertebrae

  • Chiari II malformation MRI diagnosis

Exclusion guidelines for fetal surgery, or those fetuses which are not considered good candidates, are:

  • Multifetal pregnancies (twins, triplets, etc.)

  • Maternal contraindications

  • High maternal body mass index

  • Kyphosis in fetus (curved spine)

  • Short cervix

  • Pre-term labor has started

  • Placenta previa or placental abruption

  • Uterine abnormality

  • Inability to comply with follow-up requirements

Surgery for Babies Born with Myelomeningocele

Within the baby’s first 24 hours of life, surgery is necessary to cover the spinal cord to limit the risk of infection, preserve existing function of the spinal cord, and prevent further damage. Any nerve damage or functions lost as a result of the defect cannot be improved through surgery, as this damage is permanent.

The surgery involves closing the covering around the spinal cord, called the dura matter. Then, the muscles that surround the spinal cord are closed. Finally, the skin is closed over the open area. The surgeon may need to graft skin from the baby’s back or bottom to make a flap to close the open area.

Surgery for babies born with myelomeningocele often have hydrocephalus, which is caused by too much cerebrospinal fluid in their head. This also requires surgical intervention to prevent brain damage. Surgery involves inserting a temporary drain, or shunt, in the first few days after birth. Some babies have the shunt inserted 4 to 8 weeks after birth if their hydrocephalus is not a medical emergency. Other babies are not born with hydrocephalus at birth, and develop it later. In these cases, the neurologist will wait to see if it develops later and treat it at that point in time.

Prognosis of Myelomeningocele

Those born with myelomeningocele need ongoing, comprehensive care, as they usually have complex nervous system problems. These problems may include movement difficulties, loss of sensation, and lack of bowel or bladder control.

Each child is different. We will cater your child’s treatment plan to their needs, and work closely with other experts to ensure your child gets the best possible, coordinated, long-term care plan.

Request an appointment online now or call 833-656-3876.