Neurology News

Advances In The Treatment Of Spina Bifida

Fetal spina bifida surgery is one of the most exciting developments in the history of the treatment of birth defects. It reduces the need to drain fluid from the brain, improves mobility and improves the chances that the child can walk. The most complex procedure available, qualified candidates for fetal surgery in myelomeningocele require considerable commitment from the mother who chooses the procedure and extensive surgical experience. Fetal surgery for spina bifida is not a cure, but studies have shown that prenatal repairs offer better results than traditional postnatal repairs. (9)


Another important advance in the management of myelomeningocele was the introduction of fetal repair. Improved prenatal detection has led to comprehensive planning of the prenatal clinical course, delivery and maternity care of infants with spina bifida. Formal diagnoses, including specific subtypes, are made at birth and in the operating theatre for fetal repairs. (3)

Since the late 1990s, fetal repair in fetuses of various gestational ages and variables has been tried with promising success (12-15). Although there is a significant risk for maternal and fetal dyad, experts in this field agree that a randomized controlled trial is needed to prove that the benefits outweigh the risks. In 2003, the Management of Myelomeningocele Study (MOMS) was launched to determine the safety and efficacy of fetal surgery in spina bifida (16). MOMS was conducted by three centers with extensive expertise in fetal surgery: the Childrens Hospital of Philadelphia (CHOP), the University of California, San Francisco (UCSF), and Vanderbilt University in collaboration with George Washington University, which acted as a data coordination center. (3)

Amy J. Houtrow, MD, MPH, PhD, associate professor and vice chair of the Department of Physical Medicine and Rehabilitation at the University of Pittsburgh, and colleagues investigated the effects of hydrocephalus shunt placement on developmental differences at 30 months of age in children with spina bifida participating in the Management of Myelomeningocele Study (MOMS). Patients with spina bifida who have hydrocephalus (excess cerebrospinal fluid shunting) are often used to treat the disorder. A generalized linear model was used to adjust factors that differ between the groups at the starting line. Additional analyses were performed to assess the impact on the severity of the disease. Among children with hydrocephalus, 27% were hunted for hydrocephalus (10.8%) and those without hydrocephalus (3.6%), compared to 30 months on multiple developmental scales. (4)

About 80% to 90% of children with spina bifida develop hydrocephalus. Hydrocephalus is a condition in which excess cerebrospinal fluid (CSF) accumulates in the ventricles (fluid-containing cavities in the brain) and increases pressure in the head. Most of these children require a ventricular shunt to control the accumulation of spinal fluid. The shunt persists throughout the life of the individual and may need to be replaced several times. (8)

A shunt is a small hollow tube that helps drain fluid from a baby’s brain and protect it from too much pressure. For babies with spina bifida and hydrocephalus, surgeons use a shunt. Additional surgeries are required to change the shunt when the baby grows and the tube becomes clogged or infected. (0)

Spina bifida occulta can cause problems when a child enters puberty. During this time in childhood, the spinal cord is attached to the spine. The growth spurt of adolescence begins when the nerves in the umbilical cord are stretched. (7)

Meningocele is the most severe form of spina bifida, affecting about 1,000 live births. In meningocele, the spinal cord is fully developed and undamaged. In severe spina bifida occulta, meningoceles can be surgically repaired and there is little or no nerve damage. The operation can be performed at any time during infancy. (7)

Complications are common and there are questions about the benefits of correcting spinal malformations in people with myelomeningocele. Infections are common in patients with spina bifida, particularly neurogenic blisters. The regression of the spinal cord can occur without manifestation. There is an increased risk during surgical procedures. (2)

Given the high risk of complications and lack of long-term follow-up, the American College of Obstetricians and Gynecologists (ACOG) currently recommends that fetal surgery should only be considered in specialized centers with teams with fetal surgery experience. Babies born with spina bifida must be exposed to the part of the spinal cord that is repaired to prevent further injury or infection. Neurosurgeons place nerve tissue in the spinal canal, near muscles and skin. (8)

If the baby has an open spina bifida myelomeningocele, doctors can perform surgery to close the spine before the baby is born. This is an important procedure for both the mother and the baby and may not be available where you live. Contact a doctor who works with Spina Bifida babies and expectant mothers about the pros and cons of this surgery. (0)

Many babies born with spina bifida are born with hydrocephalus, also known as water brain. Hydrocephalus means there is extra fluid in the brain. This causes the space around the brain, the so-called ventricles, to become larger and the head to swell. This condition must be followed and treated to prevent brain injury. (0)

An advanced ultrasound can detect signs of spina bifida, such as an open spine or certain features of your baby’s brain that may indicate this condition. Expert ultrasound scans are also effective in detecting spina bifida and assessing its severity. An expert who uses ultrasound is also effective in assessing the severity. Prenatal ultrasound may confirm a diagnosis of the disease, but your doctor may also request an amniocentesis. During this procedure, your doctor will use a needle to remove a sample of fluid from the amniotic fluid surrounding the baby. (1)

Since 1997, when the first open fetal surgery to repair spina bifida was performed, more than 20 medical centers across the country have provided uterine repairs to qualified mothers (see map of most sites here). Some of these centers, such as the Fetal Care Center in Dallas, have expanded their coverage to include more open fetal surgeries in 2018. Others, such as Childrens Hospital of Philadelphia, are known for their extensive history and experience, having performed more than 350 spina bifida surgeries and more than 1,700 fetal surgeries for various conditions. Talented surgeons who have pioneered in northern Ohio and other areas of the United States have developed and refined medical techniques in this area over the past 20 years, but additional options are not yet known. With these new options, mothers have an alternative form of treatment to calm the swelling tide of shock and grief that overwhelms them after a prenatal diagnosis of a fetal abnormality. (5)