About 166,000 Americans are living with spina bifida.
The disease strikes before birth, often crippling a child's chances of walking and causing a dangerous build-up of fluid on the brain.
Surgery usually takes place within a few days after birth, but now results from a new study have opened up the way for kids to get treatment before birth.
Evan terrell is nothing short of a medical miracle.
Evan's mom Kristie Terrell said, “Most people would never know he had spina bifida. Nothing slows him down."
Doctors first diagnosed him in the womb. His tiny spinal cord stuck out of his back. The defect often leads to difficulty walking and brain damage.
Kristie said, "We never, at that point, expected the running or the jumping or the kicking or the sprinting or any of the other wonderful things that he's able to do."
But because of surgery done before Evan was even born, when his mom was just 24 weeks pregnant, he can do all that and more.
Dr. Noel Tulipan, M.D., professor and Director of Pediatric Neurosurgery at Vanderbilt University Medical Center said, "You make a small incision in the uterus. We don't take the baby out of the uterus. We just position the baby, so we can see what's important to us and fix it."
Results of the seven-year trial show fetal surgery significantly reduces the risk of water on the brain and paralysis. Dr. Tulipan pioneered the surgery at Vanderbilt University.
Dr. Tulipan said, "One of the beauties of a fetus is that they heal much better than even a baby, and before a certain age, they can actually heal without a scar."
Another benefit is that 90-percent of babies born with spina bifida will need a shunt to relieve fluid buildup in the brain. Prenatal surgery cuts that risk in half.
Giving kids like Evan the chance to do what boys do.
Evan’s dad Brian Terrell said, "It always makes me nervous to see him jump, but I always let him jump cause I never thought I would see it."
The in-utero surgery still has risks.
Women are five-times more likely to deliver a premature baby and are also more likely to develop fluid in the lungs.
Surgeons at Vanderbilt are already looking for ways to reduce complications.
BACKGROUND: Spina bifida is a common birth defect of the spine. In Latin, the term literally means "split or open spine." It occurs when the two sides of the embryo's spine fail to join together, leaving an open area. Myelomeningocele is the most severe form of spina bifida, and about one in 3,000 babies is born with this type. When the bone around the spine doesn't form properly, it causes part of the spinal cord to stick out of the baby's back. Sometimes, other membranes may push through this opening in the back. About 10 percent of these babies die after birth.
One common problem for children with spina bifida is hindbrain herniation. That's where part of the brain and brainstem slips into the spinal column. Nearly 90 percent of babies born with Myelomeningocele have a buildup of fluid on the brain, which is known as hydrocephalus. The exact cause of spina bifida is unknown.
TREATMENT: While spina bifida can be treated, there is no cure. Affected newborns have to undergo an operation just a few days after birth, so the spinal cord can be pushed back into the spinal column. The only way to alleviate the pressure of hydrocephalus is to insert a tube or shunt to drain it. The first in-utero spinal cord repair surgery was done at Vanderbilt University in 1997. In this procedure, mothers are given anesthesia, which also sedates the fetus. The mother's abdomen and uterus are opened so as not to bother the placenta, which nourishes the fetus. The fetus is not removed from the womb.
CLINICAL TRIAL: A New England Journal of Medicine study found that the most serious complications from the defect can be lessened through prenatal repair surgery. During the clinical trial, 158 women found that brain malformations were reversed in one-third of the fetuses. The trial was conducted at Vanderbilt University Medical Center, the Children's Hospital of Philadelphia and the University of California, San Francisco. It was stopped early because of overwhelmingly positive results. Half the mothers had the surgery between their 19th and 25th weeks of pregnancy. The rest carried their babies to term, and the surgery was performed a few days later. After one year, researchers found only 40 percent of babies in the prenatal surgery group had to get shunts compared with 82 percent who had surgery after birth. Researchers also found that after 21/2 years, children who had their spinal surgery in-utero had better leg function. Forty-two percent of the children who had been operated on in the womb could walk without crutches compared to only half that amount in the postnatal surgery group. Dr. Noel Tulipan, from Vanderbilt University Medical Center, says the earlier the surgery is performed, the better because the longer the spinal cord is exposed to amniotic fluid, the more it can be damaged.
(SOURCE: New England Journal of Medicine)
RISKS: Eighty percent of the babies in the prenatal surgery group were born somewhat premature and their mothers had a higher risk of experiencing a ruptured uterus. Pregnant women who have the surgery will have to deliver their future babies by C-section. (Source: Vanderbilt University)
FOR MORE INFORMATION, PLEASE CONTACT:
Vanderbilt University Medical Center