Spina bifida is a birth defect in which an area of the spinal column doesn’t form properly, leaving a section of the spinal cord and spinal nerves exposed through an opening in the back. The two main spina bifida treatment options are fetal surgery during pregnancy or surgery on the baby right after birth. In this video, fetal surgeons and other team members from Children's Hospital of Philadelphia (CHOP) explain spina bifida treatment options, the benefits of spina bifida surgery before birth, the team required to perform this complex procedure, and the support services CHOP offers families. The video also shares spina bifida fetal surgery stories from the perspective of families.
Gregory Heuer, MD, PhD: When a mom decides to have an operation on her unborn baby, nothing could be more vulnerable to a person than at that moment.
I remember the first time I did an operation before the baby was born, and I left the room and I immediately said to no one, but I said it out loud, we should not be able to do that.
And then when that baby was born, I felt pride. I know I shouldn't, but it feels really good to have a positive impact on the baby. Dr. Adzick always says, when you save a baby, you save a lifetime.
Julie S. Moldenhauer, MD: In the operating room at the time of the fetal spina bifida closure, it is a very well-choreographed dance that happens. There are pediatric surgeons; there's a neurosurgeon; there's maternal fetal medicine specialist; there is a pediatric cardiologist; there's anesthesia, anesthesia techs, OR nurses, OR techs, circulating nurses; it's a very large team.
Spina bifida is a birth defect in which the spine and the vertebrae do not form correctly very early in the pregnancy.
N. Scott Adzick, MD: As an early embryo, four to six weeks, this tube’s suppose to form and close, all the tissue to protect the spinal cord. And in spina bifida, for reasons we don't really completely understand, that closure doesn't occur.
Julie S. Moldenhauer, MD: Your spinal cord acts as your information superhighway between your brain and your extremities, and it allows your brain to tell your arms and your legs what to do and to maintain control over your bladder and your bowel functions.
Gregory Heuer, MD, PhD: Because the spinal cord is outside the body and it's exposed to the environment, over time there's irreversible damage to the spinal cord.
Julie S. Modenhauer, MD: When couples are faced with a diagnosis of spina bifida in their baby, the two main treatment options are: fetal surgery while the baby is still in mother's womb or surgery on the baby right after birth. What's really important to understand is that the fetus surgery is not a cure. The goal for the fetal surgery is that maybe we can minimize or reduce some of the long-term effects of spina bifida.
For kids who have spina bifida, the long-term issues that we worry about are things like difficulty controlling their bladder and controlling their bowel. We worry about them not being able to walk on their own; they may need to use a wheelchair. Kids are also at risk for developing hydrocephalus, or water on the brain is what some people call it, and so they may need to have a shunt placed in order to keep the pressure down in their head.
Hopefully we’ll improve the likelihood that the baby can walk longer term. Hindbrain herniation is another one of those big scary terms that we hope we can reverse with the prenatal surgery.
Hindbrain herniation is a common finding for babies who have spina bifida, where the posterior part of the brain starts to come down into the top of the spinal canal.
Stephanie Rodriguez, Miah's Mom: The first hospital that we were at offered to do fetal surgery for us. And we asked them how many times they have done it, and they said we would be the first, and that just for us, was not an option. We just felt that our daughter, we wanted her to have the absolute best care possible even though she hadn't been born yet, but we just knew we had to come here, just there was no other option for us.
N. Scott Adzick, MD: Our team at CHOP, we're very fortunate to have done a lot of the groundwork experimentally for spina bifida, to have done some of the first cases. And because of our expertise, there's no other center in the galaxy that's done more fetal surgery for spina bifida than we have at CHOP.
Lori J. Howell, DNP, MS, RN: There are layers and layers and layers of expertise that we drop on, on a daily basis. It really is a giant team specialized with various backgrounds and education with a singular purpose — best outcome for mom and baby.
Stephanie Rodriguez, Miah's Mom: The doctors during the consultation let us know what surgery entailed. They let us know they would be doing an incision across my belly as if it were a c-section, and then they would be opening up my uterus and they were going to use an ultrasound to find her, to make sure just her back came up out into the world. It's just mind blowing that it's possible. It's just mind blowing.
Gregory Heuer, MD, PhD: All this team has to work sort of seamlessly; have to work at some level without communicating with each other, know what each member's doing. And so it takes a lot of teamwork. And that teamwork's built over years of working together.
So the first thing we close is the dura, that's the covering to your brain and your spine. Then we close the muscle; then we close the skin. By closing those layers over the top, we're protecting the spinal cord from any ongoing damage. So we're hoping that we can have an impact on those nerves and keep as many nerves as we can functioning so the baby has more function.
Julie S. Modenhauer, MD: So, typically after having had fetal surgery, moms remain on bed rest in the hospital for about four days. And then after they're discharged from the hospital, they're discharged with a wheelchair and they have very minimal activity for about the first three to four weeks.
And if everything looks good on a follow-up exam, we slowly allow her to increase her activity level. After fetal surgery, moms are seen every week for both a prenatal visit and an ultrasound. We plan for mom to have a cesarean delivery at 37 weeks.
Lori J. Howell, DNP, MS, RN: The Garbose Family Special Delivery Unit opened June 8th, 2008, and it was the first birthing unit dedicated exclusively to healthy mothers whose unborn baby has a birth defect.
Rachel, Maverick's mom: I think it's easier to deal with stuff that you know than you don't know, and this whole time has been a very unknown. Unknown whether we were going to make it to 37 weeks, unknown what … how he's going to come out; how he's going to do; how I would do, you know. But at least after today, it will all be on the table. So it'll be so much easier to deal with. I'm excited. I'm excited to no longer have to be in a wheelchair and on bed rest. I can't wait to go walking again.
Julie S. Modenhauer, MD: So if mom opts to deliver here in the Special Delivery Unit, immediately after birth, the baby is evaluated by the neonatal surgical team, stabilized, and then immediately goes to the Neonatal Intensive Care Unit and starts down a very involved care pathway that includes: neurosurgery, surgery, urology, and specific imaging so that we can best assess the outcomes from the fetal surgery and make a detailed care plan going forward.
Susan S. Spinner, MSN, RN: Some of our families are relocating from far away, across the country, maybe from around the world.
We surround the family with layers and layers of support, so we support them with psychosocial services, with nursing support, and they may not realize the effect that the diagnosis and the treatments and relocating to our center may have on the entire family. There are issues with anxiety, there maybe marital issues, and we address those with our psychosocial team.
Stephanie, Miah's mom: Miah still has daily medical care that she has to have done. She has no function of her bowel or bladder. She gets therapy four days a week, so some days are definitely harder than other days. She has great function right now and can run around and play.
Jeremiah, Miah's dad: We forget she has spina bifida some days, but you know, we still worry about her bowel and bladder, going to school and trying to regulate that and combat that when she's at school. We're blessed where she is right now. If that's all we have to deal with, we got out lucky.
Stephanie, Miah's mom: It was scary for me at first, but now it's just so normal for us. Miah just doesn't know any different and I think, I think sometimes as parents we stress more over what their life is going to possibly be like than how they actually perceive it.
N. Scott Adzick, MD: So you might ask why come to the Center for Fetal Diagnosis and Treatment at CHOP? The reason is that as a patient, as a mother carrying a baby with a birth defect, you want the best information from an incredibly caring team; you want to know all the options, and you want the best possible care. That's what we provide.
Stephanie, Miah's mom: Today, Miah is an incredible, happy little girl. She is able to be a typical child. She's able to play. She just started gymnastics, and she's going to be starting kindergarten in the fall, and she's just incredible.