Medical Minute 5-11: Tales from the Womb: Saving Evan from Spina Bifida

By: Vanessa Welch Email
By: Vanessa Welch Email

He plays with passion. Shooting hoops. Making baskets. Evan Terrell is not just something special on the court. Evan Terrell is nothing short of a medical miracle.

"Most people would never know he had spina bifida. Nothing slows him down," said Kristie Terrell.

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.

"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.

"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. Doctor Noel Tulipan pioneered the surgery at Vanderbilt University.

"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," said Noel Tulipan, M.D., Professor and Director of Pediatric Neurosurgery Vanderbilt University Medical Center.

Another benefit? 90% 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.

"It always makes me nervous to see him jump, but I always let him jump cause I never thought I would see it," said Brian Terrell.

Another score… for medicine.

For more information on other series produced by Ivanhoe Broadcast News contact John Cherry at (407) 691-1500, jcherry@ivanhoe.com.

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MEDICAL BREAKTHROUGHS - RESEARCH SUMMARY:

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.
(SOURCE: Kishealth.org)

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 2½ 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:
Craig Boerner
Vanderbilt University Medical Center
Nashville, TN
Craig.boerner@vanderbilt.edu

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THE FOLLOWING IS AN IN-DEPTH INTERVIEW WITH THE DOCTOR FROM THE STORY ABOVE:

Noel Tulipan, M.D., Professor and Director of Pediatric Neurosurgery Vanderbilt University Medical Center, discusses a way to operate on babies before they're even born!

There are some very exciting results from this study?

Dr. Noel Tulipan: Yes. The study has been going on for seven or eight years so it took a long time to finish. It’s really the culmination of work we started way back in the early 90’s, a project we’ve been working on for almost 20 years now. It’s very gratifying to see that the results came out so well.

Can you tell me a little bit about spina bifida and some of the things the children who have it may have to worry about if they don’t have the surgery prenatally?

Dr. Noel Tulipan: Spina Bifida is a congenital disease, which means it’s something that you are born with. It’s a defect of the spinal cord that actually occurs about four weeks after the baby is conceived. A lot of the time it happens even before a mom knows she is pregnant. What results is a little piece of spinal cord is exposed at the lower part of the spine. The result of that is that there is damage to that spinal cord and there can be varying degrees of damage. That lower part of the spinal cord really controls the leg function and also the bowel and bladder function. All of these kids have some degree of loss of bowel and bladder function as well as leg weakness. And then, in addition to that, they have a condition called hydrocephalus, water on the brain in 90% of the cases, so they also need a treatment for that as well. It can be a very devastating disease and, to me, the hydrocephalus is the worst part of the disease because it affects the brain Also the treatment for hydrocephalus is not perfect, so we often have to bring the patients back for multiple operations for that part of their disease

I spoke with a woman whose child was in the study and her biggest concern was with the hydrocephalus and the shunts they would have to put in.

Dr. Noel Tulipan: Right. Exactly. The device we put in to drain the fluid on the brain is called a shunt and the shunts, as I said, are very imperfect devices. They tend to fail unexpectedly. They have a pretty high rate of infection which requires replacement of the shunt and so patients tend to come back over and over again with problems with their shunt or even more than that false alarms for problems with their shunts. If they have bad headaches, let’s say, or other symptoms of pressure in their head they end up in the emergency department many, many times during their childhood. So, it’s really something that hangs over their heads for their entire lives.

Have you found that need has gone away by performing this surgery in the womb?

Dr. Noel Tulipan: It hasn’t completely gone away, but it is greatly reduced by a significant amount, almost 50%. Our original studies that we did before the protocol was started and also the randomized trial, the MOMS trial, both showed there was about a 50% reduction in the need for shunting in kids with spina bifida if they have their backs repaired in the uterus.

What are some of the other advantages? I know when speaking with this particular woman, she said her child was still going to have bowel problems even with the surgery.

Dr. Noel Tulipan: It’s not as though this procedure is a miracle cure. It doesn’t totally cure the disease. Our original studies didn’t even show much improvement in their leg function but, in fact, the recent study, the randomized study, does show an improvement in their ability to walk without crutches, walkers or canes and that kind of thing, so it does have a benefit as far as that goes as well. But, almost every one of these children is going to have some degree of bowel or bladder dysfunction. It varies among the different patients.

How many patients have you worked on?

Dr. Noel Tulipan: I’ve done well over 200 patients. There were only about thirty in the randomized protocol, but then we did an additional 170 plus patients prior to the randomized protocol.

Just so people can understand, how this is done? Can you walk us through a day in the operating room?

Dr. Noel Tulipan: Sure. Basically, the surgery is a combination between a cesarean section, which women have to deliver their babies, and my standard myelomeningocele repair, spina bifida repair. So, a woman would come to the operating room, be anesthetized, have an incision in the lower part of the abdomen, and we actually, what we call exteriorize the uterus. In other words, we pull it out of the abdomen, so it’s sitting on top of the abdomen like a big red soccer ball. Then the obstetricians make an incision in the abdomen and through that we can see the baby’s back. We don’t actually, take the baby out of the uterus during the procedure. So, I’m looking at the baby’s back where this defect is and I repair that much in the same way that I would after birth, which involves covering two layers of tissue over the spinal cord tissue. Then the uterus is closed back up, put back in the abdomen, the abdomen is closed up and that’s the end of the procedure.

You basically, pull the uterus out?

Dr. Noel Tulipan: Right. The uterus is obviously still attached to the mom but we can lift it out of the abdomen so it’s sitting on top of the abdominal wall. It’s easy to access that way and we can, localize the baby with the little ultra sound machine so we know exactly where the baby is. We make a little cut in the uterus, just like they would if they were delivering a baby from a C-section.
Then the obstetrician can work outside the uterus and position the baby so that I can see the defect through that opening in the uterus. From then on, for me, it’s a relatively straightforward repair process that is much the same as I would do in a baby after a normal delivery. Obviously, these fetuses are smaller than a term baby. They are like an extremely premature baby, basically. Technically, it’s a little more difficult and the tissues are a little more delicate but the actual process of the repair and the layers that we repair are the same for the prenatal repairs as would be for postnatal repair.

How long does a surgery like that take?

Dr. Noel Tulipan: In general, it takes about an hour and a half to two hours if you add together all the anesthesia, the obstetricians’ part, my part and then closing everything up again.

I know you said you have done over 200 surgeries but can you take us back to that first surgery because that must have been exciting.

Dr. Noel Tulipan: Yes, that was a very exciting experience obviously. There was a lot of publicity involved, a lot of excitement about the whole thing and it was a little scary because we didn’t really know what the results were going to be. At first we were concerned that maybe the babies would deliver immediately after the surgery and then be very premature and then very sick, but as it turns out it worked out quite well and the babies did well. We gained more confidence after doing several procedures and then there was a lot of demand for it after that.

Obviously, you have to be pretty brilliant at what you do to be able to go into the womb.

Dr. Noel Tulipan: Prior to this, I have done many of these myelomeningocele repairs, the spina bifida repairs. I was very familiar with the disease, very familiar with the anatomy, so it was not like the first time I had ever done anything like this. I had fifteen years of experience before, taking care of patients in the conventional fashion.

Still though, it is pretty incredible that you were able to do this.

Dr. Noel Tulipan: It certainly was a great experience and I still get a thrill out of it. For me it is a fun surgery. Actually, for me, the real challenge going forward is trying to figure out even better ways of doing this surgery.

You said you have been doing this, or the study has been going on, for seven years. I can imagine you take that first patient you worked on here that kid is seven years later and you can see that kid running around and not in a wheel chair as they might have been. You see them living a somewhat normal life. What’s that like for you?

Dr. Noel Tulipan: Once again, it’s very gratifying. In our original group of patients before the randomized protocol, we used to have yearly reunions where lots of them would come back and get together every summer. We would rent out a big park, or find someone who had a big backyard, and have a barbeque and get everybody in town. So, I would see these patients and a lot of them did exceedingly well. Once again, this is not a miracle cure and not every single patient does great, but a lot of the patients do much better than we would have expected if we had just done it in the standard fashion.

I know you said they did better. In the study there were two babies that died?

Dr. Noel Tulipan: Yes. There were two babies in the prenatal group and two babies, actually, in the postnatal group. So, the actual mortality for the study was the same in both groups.

What are the implications for this? I would think if you’re successful treating these babies in utero are there other diseases or other ailment that could be treated in utero that you are considering doing that may be better doing it there instead of waiting until the baby is born?

Dr. Noel Tulipan: Well, I think there are quite a few, it really depends a lot on how we can improve the procedure to reduce some of the complications. Part of this procedure is taking on an increase risk of premature delivery, preterm labor, and those kinds of things. If we could get those better, than say less devastating diseases might be able to be treated prenatally. Let’s say we have a baby with cleft palate or some other congenital malformation, that would be amenable to surgery, one of the beauties of the fetus is that they heal much better than even a baby and before a certain age they can even heal without a scar. So, if you can consider doing surgeries like cleft palate or certain congenital heart defects and other defects like that, I think there are a lot of possibilities. For me, as a neurosurgeon this is certainly the most common congenital malformation that would be amenable to this kind of surgery. It’s fairly easy to diagnose prenatally so, we can get these patients referred as early as 14 – 16 weeks with improved diagnostics. There’s also an isolated disease of water on the brain without spina bifida. We have done four patients where we put shunts in prior to birth. I think that would be my other focus as a neurosurgeon, to try and improve that procedure. As it turns out, those patients didn’t do all that well. They didn’t do significantly better than patients who we treated postnatally, but I think if we learned how to pick the right patients I think that would be another procedure that we could easily do prenatally and it might help the patients out a great deal.

Is there an ideal candidate for this surgery? I noticed in the study that obese women were disqualified. What are the reasons behind who you choose?

Dr. Noel Tulipan: There are certain things that just make the surgery too risky compared to the benefits. Extreme obesity in the mother would be one. There are some babies with spina bifida that have very bad, devastating abnormalities of their spine when the elisions are very large or very high up on the spine. Those would probably not be good candidates because it would be very unlikely that we would be able to help them. Obviously, if the mother has other serious diseases, or if the fetus had other congenital malformations that’s another thing that would probably argue against doing this surgery prenatally. I would say, the majority of patients we see probably would be reasonable candidates.

Anything I missed that you think we should touch on?

Dr. Noel Tulipan: Once again, my main focus at this point, now that this study is done, is really to move on to bigger and better things and to see if we can improve the outcomes of the surgery. So, I plan to be working in the next few years on ways of improving the surgery to reduce the complication rate. I’m thinking of doing the surgery perhaps through endoscopes, so you wouldn’t have to make such a big hole in the uterus, or even doing it earlier on, say before 20 weeks gestation and maybe getting a better result from that, but I think there are a lot of things we can still do to even improve the results further.

You mentioned a picture.

Dr. Noel Tulipan: There are quite a few versions of that picture. One thing I think it is important to understand is that babies do not reach out of the uterus and try and grab you because they are all anesthetized at the time of surgery so I don’t want anyone thinking that these babies are awake and moving around while we are operating on them. When we anesthetize the mom, the fetuses are also anesthetized at the same time and we give them extra anesthesia. That (picture) was just something that happened during one of the surgeries. That arm came out there and we got photos of it, but there are certain misconceptions about the surgery or about that photo at the time.

I know you said you perform this procedure at around 20 weeks, but do most then go full term?

Dr. Noel Tulipan: The average baby in the study and also in our previous series went to about 34 weeks gestation. Term is 30 – 40 weeks so a little bit premature, but not extremely premature. Neonatologist, these days have gotten superb at taking care of patients at the 30 – 39 week range. Most of those babies do extremely well and don’t have many complications. It’s really only the babies that are born before 30 weeks that still have serious problems.

Your procedure is done 20 weeks of development, what would happen if the baby were delivered then?

Dr. Noel Tulipan: 20 weeks is not viable, so if we did a surgery at 20 weeks and the fetus is delivered immediately that fetus would not survive. There is still a possibility that we could do the surgery even earlier than twenty weeks. There’s no technical reason why that could not be done.


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