If a prenatal ultrasound detects spina bifida, the Fetal Center at Riley Children’s Health offers fetal repair to close the birth defect to reduce morbidity and improve health outcomes.
What is spina bifida?
What is spina bifida?
Spina bifida refers to a congenital birth defect in which a baby’s spine does not form properly. Myelomeningocele (also referred to as an “open neural tube” defect) is the most severe form of spina bifida, where the spinal cord and nerves (also known as the “neural placode") develop on the outside of the body. Because of congenital malformations in the spinal cord and nerves, babies can lose function below the spinal level where the neural placode is located. If the neural placode is located higher up on a baby’s back, more nerves have not formed correctly and can result in weakness in the legs, difficulty with walking, and disturbances with bladder and bowel control.
Other commonly encountered forms of spina bifida include spina bifida occulta (the mildest form of spina bifida), meningocele, and lipomyelomeingocele. In these forms of spinal bifida, the spinal cord isn’t on the outside of the body and there is little to no nerve damage.
Medical conditions associated with myelomeningocele
The number and severity of problems in children with myelomeningocele varies depending on where the neural placode is located on the spine and how much of the spinal cord is affected. Possible health effects of myelomeningocele include:
- Hydrocephalus
- Chiari 2 malformation
- Loss of feeling and decreased function or paralysis
- Loss of bowel or bladder control and increased risk for urinary tract infections
- Heart problems
- Orthopedic issues, particularly clubfoot malformations
- Other neurological problems
How is myelomeningocele diagnosed?
Myelomeningocele can be diagnosed early in pregnancy and most commonly during the anatomy ultrasound at routine prenatal visits and prenatal ultrasounds. If diagnosed, mothers may be eligible for fetal surgery to repair the myelomeningocele before birth.
What is fetal myelomeningocele repair?
Myelomeningocele repair, also known as spina bifida or open neural tube defect repair, is a delicate fetal surgical procedure to close the neural placode in utero. Performed by fetal interventionalists and neurosurgeons, myelomeningocele repair is intended to prevent further damage to the baby’s spinal cord in the womb.
What are the potential benefits and risks of fetal myelomeningocele repair?
The results of the Management of Myelomeningocele (MOMs) Trial suggest that fetal repair of myelomeningocele can have several beneficial effects for the baby. Fetal surgery for spina bifida can lead to a reduced risk of progressive damage to the spinal cord and nerves caused by exposure to amniotic fluid, improved mobility and walking as well as reduce the development of symptomatic hydrocephalus. With advancements in fetal surgery, especially procedures such as myelomeningocele repair, the life expectancy for children with spina bifida often extends into adulthood despite being a congenital condition.
Potential risks include preterm labor and preterm birth.
Traditionally, the operation was completed through an “open approach.” This involved making a cesarean section-like skin incision to expose and open the uterus, as well as operate on the baby’s back. This was found to increase the risk of maternal complications during the remainder of pregnancy, including uterine rupture, increased risk of wound infection and prolonged time to recovery. Additionally, the baby was required to be delivered via cesarean section.
What is mini-laparotomy/percutaneous fetoscopic myelomeningocele repair?
What is mini-laparotomy/percutaneous fetoscopic myelomeningocele repair?
Given these maternal risks, our physicians were part of the pioneering team for a minimally invasive approach known as mini-laparotomy/percutaneous approach aimed at reducing the risk of large uterine incisions and achieving faster recovery for the mother, while maintaining the benefits to the baby.
The mother and fetus are eligible for surgery if:
- Mothers are at least 18 years old
- The fetus has a myelomeningocele lesion located between T1 and S1
- The fetus is between 19 and 26 weeks of gestation
- Mothers with a body mass index of less than 40 kg/m2
- Absence of uncontrolled maternal health conditions
- Absence of other associated severe fetal anomalies
- Normal fetal gestic testing confirmed via FISH, karyotype, or microarray
The mother is given general anesthesia and medication to prevent uterine contractions. The fetus also receives pain medication before the repair.
During the procedure, the fetal surgery team will perform a small skin incision on the mother’s abdomen and place three 4-millimeter ports into the uterus. These ports are used for the introduction of a small scope with a camera on the end (laparoscope) and surgical instruments into the womb to continue the operation on the baby. Some amniotic fluid is then removed and replaced with carbon dioxide gas to improve visualization.
The fetal surgery team then closes the spinal cord defect, protecting the fetal spinal cord and nerves from injury for the remainder of the pregnancy. The skin on the baby’s back is then closed to provide an additional layer of protection. The baby’s heart rate is checked frequently via ultrasound throughout the procedure.
Once the repair is complete, the amniotic fluid is replaced, and the ports are removed. The mother’s abdominal incision is then closed and gestation resumes.
What happens after fetal surgery?
When the surgery is complete, the baby can continue growing and developing in the womb. Both mother and child will then remain in the hospital for a few days while recovering and monitoring the baby’s status. For the remainder of the pregnancy, the Fetal Center and the primary referring provider will monitor both mother and baby.
Our Spina Bifida Program will also provide ongoing care for your family. Our team of experts helps families prepare for the birth of a child with spina bifida and care for children into adulthood.
Key Points to Remember
Key Points to Remember
- Myelomeningocele (also referred to as an “open neural tube” defect) is the most severe form of spina bifida, where the spinal cord and nerves (also known as the “neural placode") develop on the outside of the body.
- The number and severity of problems in children with myelomeningocele varies depending on where the neural placode is located on the spine and how much of the spinal cord is affected.
- Myelomeningocele can be diagnosed early in pregnancy and most commonly during the anatomy ultrasound at routine prenatal visits and prenatal ultrasounds.
- Myelomeningocele repair, also known as spina bifida or open neural tube defect repair, is a delicate fetal surgical procedure to close the neural placode in utero.
Please note that this surgery will be available to eligible patients in early 2025