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Birth of a Breakthrough: Spina Bifida Video - CHOP

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Fetal Repair at CHOP: How It Works

The fetal repair at CHOP video details the extensive planning and multidisciplinary teamwork involved in the spina bifida prenatal surgery process. Participants in the surgery include several fetal surgeons, a pediatric neurosurgeon, a fetal cardiologist, an obstetrician, an anesthesiologist, surgical nurses and others. Spina bifida fetal repair at CHOP is a well-orchestrated and complex procedure that is performed with the safety of mother and unborn child as a foremost concern. Every precaution is taken to maintain the health and wellbeing of both patients during these tightly choreographed spina bifida fetal repair procedures.

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The Birth of a Breakthrough Video explores spina bifida diagnosis, treatment options, delivery and follow-up care at The Children’s Hospital of Philadelphia. Experts at CHOP’s Center for Fetal Diagnosis and Treatment have the world’s greatest collective experience in prenatal repair for spina bifida.
 

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Transcript: Fetal Repair at CHOP: How It Works


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Natalie E. Rintoul, MD: Once a family decides to pursue fetal surgery, things happen pretty rapidly.

N. Scott Adzick, MD: All the components of our rather large team meet to discuss the case and then have the parents come in, and we go through the entire process once again so that there's a clear understanding of what's involved and give them the opportunity to ask any additional questions.

Katherine Mulligan, mother: We had to be down here – I don't know if it was 5 o'clock or 6 o'clock, but it was very early in the morning. I was so nervous.

Michael Mulligan, father: And, you know, still a lot of consultations and things like that going on and, you know...

Katherine Mulligan, mother: Flurry of activity. They put an epidural in. It was the first thing they were going to do, and I had to be awake for that. You are knocked out for the surgery. And I remember bending over getting that epidural, and they're talking to me and all of a sudden, you go – Oh! I mean, if it hadn't hit us already, it hit me then: this is big time.

Michael Mulligan, father: Right.

Katherine Mulligan, mother: Seventeen days before that life was normal, and all of a sudden here we are in fetal surgery for something that's cutting edge.

Leslie N. Sutton, MD: We have a special OR that we use for fetal surgery. It's a very large OR because there's a lot of equipment that's there, and there are a lot of people involved in this. There are a couple of anesthesiologists. There's the fetal team.

Susan R. Miesnik, MSN: Two to three surgeons in the room. There's a cardiologist in the room who monitors the baby's heart through the entire procedure. There's a neurosurgeon who actually does the myelomeningocele repair. There is an obstetrician or maternal-fetal medicine specialist in the room who monitors the mother's uterus and performs ultrasound so that we make sure we put the incision in the uterus where we need to put it.

Mark P. Johnson, MD: And it all has to be carefully choreographed, so that everybody is focused on what their role is, what they need to do, but everybody has to work seamlessly together.

N. Scott Adzick, MD: The mother goes under deep general anesthesia. Deep general anesthesia is important for at least three reasons. It anesthetizes the mother, it anesthetizes the fetus, and also gives us very good uterine relaxation-- relaxation of the uterine muscle, which is very important for the operation. An ultrasound is done to look at the fetal position and then a sideways wound is made – almost hipbone to hipbone halfway between the belly button and the pubic bone to expose the uterus. Ofttimes the obstetrician will move the fetus within the uterus so that the myelomeningocele, or spina bifida lesion, is right underneath where we're going to make the cut in the uterus because the baby basically stays inside the uterus during the operation. The cut in the uterus is done again under ultrasound guidance – sterile, intraoperative, ultrasound guidance – with a uterine stapling device that fires absorbable staples that pinch off all the blood vessels and keep the membranes around the baby tacked up to the muscle layer of the uterus. During the operation the fetal cardiologist does monitoring of the fetal heart by echocardiography.

Jack Rychik, MD: We're able to look at blood flow patterns through the heart, the various structures, and the flow towards the different organs. Heart function. We look at the heart rate. Are able to feed that information back to the surgeons and to the anesthesiologist that's managing the mother.

N. Scott Adzick, MD: The neurosurgeon then comes in, and he and I do the myelomeningocele repair exactly like you would do in a newborn after birth.

Leslie N. Sutton, MD: I trim the sac of abnormal skin away from the nerve tissue. It's important to do that because that skin tissue, if I leave it behind, can become a cyst. It can be a problem later on. We then put the nerve tissue back into the spinal canal where it's supposed to be and then I try to get a three-layer closure. The first layer is the dura – that's the normal covering of the brain and the spinal cord. The second layer is the fascia – that's kind of like muscle-type tissue. And then the third layer is the skin.

N. Scott Adzick, MD: The goal is to cover the unprotected spinal cord so that it's a watertight protective closure. It needs to be watertight for two reasons. One, is that you want to stop the damage caused by amniotic fluid exposure for the rest of the pregnancy. And you also want to stop the leakage of cerebrospinal fluid out through the back that plays an important role in the development of hindbrain herniation and hydrocephalus. So if you stop the leak back here, the hindbrain can go back, and the hydrocephalus is less of an issue. Once the uterine wound is closed, the maternal abdomen is closed, including the skin, with a plastic surgical closure. All the layers are closed with an absorbable stitch that will dissolve once the mother's wound is healed.

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