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Learn about the growing practice of fetal surgery from Darrell Cass, MD and how he led Cleveland Clinic's first in utero fetal surgery that repaired a spina bifida birth defect in a nearly 23-week-old fetus.

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Innovations in Fetal Surgery with Dr. Darrell Cass

Podcast Transcript

Scott Steele:  Butts & Guts: A Cleveland Clinic podcast exploring your digestive and surgical health from end to end.

Welcome to another episode of Butts & Guts. I'm your host, Scott Steele, the chairman of colorectal surgery here at the the Cleveland Clinic in beautiful Cleveland, Ohio. Very pleased to have one of my close friends, Dr. Darrell Cass, who is a pediatric surgeon and also the director of fetal surgery and the director of the Fetal Center here at the Cleveland Clinic. Darrell, welcome to Butts & Guts.

Darrell Cass: Thank you. It's great to be here.

Scott Steele: So, we always like to start off with all of our guests to give us a little bit of background about yourself, where you're from, where'd you train, and how did it come to the point that you're here at the Cleveland Clinic.

Darrell Cass: Wow. That's a little bit of a long story. I grew up in Los Angeles. I went to Stanford for college and then UCLA for medical school, and then I trained in general surgery at UCSF, and that's probably the most important aspect of this story because that's where I met Mike Harrison and Scott Adzick who people consider the fathers and the developers of fetal surgery as a field. So, that was just pure luck, and I was very fortunate to be at UCSF at that time. I became very enamored in this whole field of fetal surgery. It was early in its development at the time.

From there, I went to finish my training in pediatric surgery in Houston at Texas Children's Hospital where I was very fortunate to be there at a good time where I was able to build a fetal surgery program, and I was in Houston for 18 years. And I think you're one of the reasons I'm here at the Cleveland Clinic. I was there for 18 years. We built a world-class fetal treatment center, but I was ready for some change, but I was looking for different opportunities.

It was a very exciting time in the field of fetal surgery. Beginning in 2011, the field really began to change, and perhaps we can get into that, but there were a lot of centers in the United States that were now becoming interested in building their own programs, and the Cleveland Clinic proved to be one of those. And I had heard great things about the Cleveland Clinic from you, having been here and built a great colorectal department. I knew that they had an amazing reputation in adult medicine and surgical care of all types, and I just thought it was going to be a great opportunity to build a new service line for the Cleveland Clinic in fetal surgery. And so, I moved here about a year-and-a-half ago.

Scott Steele: Well, we're very glad to have you here. So, let's go through kind of the 50,000-foot view first. So, when people hear of fetal surgery, man, those two terms don't necessarily go hand-in-hand, and it can't be a lot of that going on in the world. So, tell us, just in very broad terms, what is fetal surgery?

Darrell Cass: Well, that's a great question. So, fetal surgery, one simple way to think about it is treating conditions that I, as a pediatric surgeon, might treat normally in the neonatal intensive care unit. Normally, we would treat them after birth, but it's simply using those same surgical principles and techniques but treating them before the baby is born, and why the heck would you ever do that? Because it does pose risk. It actually poses risk for the mom as well as for the baby, but the only reason we do that is to try to optimize the outcome. And the field got created initially because we were trying to prevent fetal death. So, that was kind of black and white. If you didn't do something, the fetus dies. If you do something, you have an opportunity to possibly save that baby's life.

And so, that's how the field originally evolved, but more recently we've gotten into trying to improve the long-term outcomes. And fetal surgery for spina bifida is the perfect example of that where it turns out if we fix that spina bifida defect before the infant is born, we improve their long-term, lifelong outcome as best we know. And that's like a new paradigm that we're in, and that's also the paradigm that's led to more growth in the field of fetal surgery.

Scott Steele: Yeah, and we're going to get into spina bifida a little bit later. So, fetal surgery, is that rampant? Is there lots of different places for that, or is there a few centers, or how does this work?

Darrell Cass: 14 years ago, there were three comprehensive fetal treatment centers in the world. So, that was San Francisco, Philadelphia, and Houston. Since then, in the United States alone, there's about 20 fetal treatment centers, and those centers all have different capabilities. Mostly when I'm talking fetal surgery now, it's centers that are doing fetal surgery of spina bifida, but if we try to talk about doing fetal surgery for other conditions, whether it's diaphragm, hernia, maybe there's seven centers. If it's taking out a fetal lung lesion or a fetal tumor successfully, then that's whittling down to about three centers in the world again.

Scott Steele: So, Darrell, one of the things that you've brought here is this ability that we had not had. So, tell us about this most recent in-utero fetal surgery that your team recently performed.

Darrell Cass: When we got here, that was my job was to build a fetal surgery department, and the first thing that we needed to do was to assemble a team because these are all team efforts that involve a well-organized, highly functioning team, and that team includes anesthesiologists. It includes, in this case, neurosurgeons. It includes an OR team of nurses and surgical providers. It includes maternal fetal medicine. It includes radiologists that can diagnose the problem with fetal MRI.

So, we begin assembling that team, and it turns out there was tons of interest. It's a cool field, and so, a lot of people are excited to be involved in it. And then, after we assembled the team, we began preparing for doing actual fetal surgeries, and we did that by a number of different ways. First was educational conferences and sharing information. All of the different specialists then contacted colleagues of theirs that they were friends with that were doing fetal surgery at other programs and got information from them. And then, as a unit, we began visiting other fetal surgery centers. Even though I was very experienced at doing this, I felt it was important for all the different team members to go and see it for themselves.

So, we went and visited programs. We went to Ann Arbor, Michigan. We went to Cincinnati. We went to Denver, and we watched those teams doing fetal surgery, and that was awesome for me because I had been in San Francisco. I had been in Philadelphia, and I created the program in Houston, but I had not gone and seen my friends do surgery in these other places, and I'm sure you've had the same experience. And it was eye-opening. I learned some things. I shared some information that maybe helped them, but it turns out there was variability that I didn't realized existed in how people do things. But our team gained experience, and then, we began simulating, and we did walkthroughs and simulations of fetal surgery, and then, we felt like we were ready to do our first fetal surgery case here at the Cleveland Clinic.

Scott Steele: To some of our listening audience out there, they may have seen you in the past when it was all over national media when you captained a team that dealt with conjoined twins. With that experience, did some of that help or at least guide this entire process?

Darrell Cass: Well, as surgeons, it's not necessarily natural for us to be team players. You want your surgeon to be confident, to know what they're doing, and feel like they can be in charge and do an effective operation. In fact, as doctors, I would say it isn't natural in our training to work in teams, but that is the current era. We know that the best medicine is generated by comprehensive, well-functioning teams of different specialists that can work together effectively, and this conjoined twin operation that I helped to captain several years ago now was a great experience where we had to put together a team that had 20 different specialists working together.

The operation took 26 hours in a very complex, well-orchestrated surgery to separate two baby girls effectively that involved the chest and the lungs and the heart and the genitourinary system, their intestines, pelvis, uterus, very complex operation. And I had a really good opportunity to kind of help build that team to help us work together effectively, and it was incredibly successful, and I think that did help prepare this effort at building a team that can do an effective fetal surgery operation.

Scott Steele: So, tell us a little bit more about this first surgery here.

Darrell Cass: Well, first of all, we are incredibly grateful for this family that came to us with this diagnosis of their baby girl having spina bifida. At first, it's a shock to hear that because all of us are expecting pregnancies to have healthy babies, and when you hear of a diagnosis that's concerning like this, it really breaks your heart.

Scott Steele: So, tell the audience a little bit as background before you go into the surgery. What is spina bifida?

Darrell Cass: Oh, yeah. It's a great question. So, spina bifida simply means the spine is separated in two parts. It's a common term. There's other technical terms, myelomeningocele, myelocystocele. There are subtypes of spina bifida, but simply, as you may know, the spinal column, the vertebral column, is actually is a bony element that has a tube, and the spinal cord runs in the middle of that. And in spina bifida, the back of that bony column is not formed properly, and the spinal cord is malformed, and it's exposed out to the world, out to the uterine environment before birth and out to the world after the baby is born. And that leads to problems with that spinal cord in terms of how it functions.

Fortunately, it just affects the spinal cord and what that area controls, which is the strength of the legs, the ability to poop properly, the ability to urinate properly. Obviously, constipation is something that this podcast deals with commonly, and children with spina bifida have a real problem with that. Their bowels can't empty properly because of the spinal cord dysfunction.

This is a problem that we can diagnose before birth. There are blood tests that the mom can take that raise our suspicion for that, and it can be confirmed, then, with ultrasound. We do fetal MRIs to better accurately diagnose that problem. So, traditionally, that defect is fixed after the baby is born, but it turns out that there's evidence that during the in-utero environment, there's ongoing damage that's happening to that spinal cord because we can see early on in the middle of the pregnancy, the ultrasound can follow those babies moving their legs, kicking their feet, moving their ankles. But we can see that they seem to lose that function during the pregnancy, and by the end of the birth, their leg strength seems to be much weaker.

So, that raised the question of whether we can do something earlier to help improve how these children can do. And there was an amazingly designed prospective, randomized trial that was conducted from about 2001 to 2011 where they tested doing fetal surgery for spina bifida against the traditional postnatal surgery, and it proved that there was a real benefit to fetal surgery repair of that defect before the baby was born.

Scott Steele: So, this family came to you with a baby with spina bifida, and walk us through that.

Darrell Cass: Yeah. So, they came to us. The first goal of any consultation is to make sure that the family understands everything we know about it. We explained what the problem was, what it meant. We explained the traditional surgical repair, and then, we offered fetal surgery. The mom and the fetus were appropriate candidates to consider for fetal surgery. We made it very clear that this could be the first case that was done at the Cleveland Clinic or in northern Ohio. I explained my experience with fetal surgery but that we had assembled a new team. I felt like we were prepared, but it would be the team's first endeavor here at the Cleveland Clinic. I explained that we could refer them to other places. We'd be happy to facilitate that, and amazingly, the family put their trust in us. So, then, we did our first fetal surgery case here at the Cleveland Clinic, and it was just an amazing experience.

Scott Steele: And that's incredible. So, if I'm a listener out there and pregnant, when is fetal surgery recommended, and then, when can it wait until after the baby is born?

Darrell Cass: Yeah. So, fetal surgery is recommended for a number of different types of conditions. The first are placental problems and twin problems. So, one of the problems is called twin-twin transfusion syndrome. It's a complication of two twins when they're sharing a placenta. There were traditional treatments, but we now know that the best treatment is to go into the uterus and to use a little laser to divide abnormal blood vessels that connect those two twins abnormally, and that leads to the best outcome.

That procedure is being offered, and that's the most commonly done fetal surgery intervention.

Spina bifida is probably number two. We do open fetal surgery repair so spina bifida. There's an investigational approach where we're working on doing fetoscopic repair. That has pros and cons that have to be carefully studied in order to know if that's going to be a long-term approach. And then, we do fetal surgery for a lot of other very rare conditions. If a fetus sometimes develop lung malformations, they've been called CCAMs or CPAMs.

Usually, those can be best treated after the baby is born, but on very rare instances, those grow very rapidly before birth. They squish the fetus's heart. They cause what's called fetal hydrops, which is fetal heart failure, and then, either the fetus dies, or you go in and do some kind of fetal surgery. You can do fetal surgery for fetuses that have tumors. Sometimes, those tumors, which are called teratomas, can have high blood flow that causes the heart to go into strain. If that heart starts going into signs of heart failure, those fetuses will die unless they have in-utero treatment. There's other rare conditions, amniotic bands, that might benefit from lysis. There's things where we can do shunts for, like fetuses that have bladder outlet obstruction. We have to go in and put a shunt into the fetus's bladder.

Scott Steele: Darrell, you mentioned a lot of things, and I understand that a lot of them are more on the rare side, and that's just the way things work in terms of your field, but how many of those things, and roughly, are we offering here now at the Cleveland Clinic?

Darrell Cass:  We can do virtually every type of fetal intervention here at Cleveland Clinic. Our center is new, but fortunately, I've had 20 plus years’ experience in this field, and I'm very experienced with all of these different types of treatments, and I can confidently say that we can offer treatments for all of these conditions.

Scott Steele:  So, I'm a mom at home, and I have one of these conditions, and I'm perusing the internet or, hopefully, listening to Butts & Guts and come across this. And so, what can a family expect in a visit to your office?

Darrell Cass:  Well, first of all, that it's going to be a team visit. So, we are going to do our very best to coordinate their care to make sure they understand all of their appointments and what the agenda for the consultation is going to be. They're going to have an opportunity to see the center. They're going to get expert imaging, whether it's with ultrasound, echocardiogram looking at the fetus's heart or fetal MRI, which will be immediately interpreted, and that information will be shared with them.

The first goal will be to make sure they understand the state-of-the-art diagnosis that their baby has, and then, we can talk about treatment options, whether it's just standard postnatal treatment, or it's in-utero treatment. And we can talk about the pros and cons of all of those, and then, we can orchestrate a follow-up plan of care working either with their obstetricians in wherever their current care is. In different instances, it might be just a consultation in which we can advise their treatment team, what we think is best.

In some instances, it may be appropriate to change care for plan delivery here at the Cleveland Clinic. Our goal is to simply provide the best diagnostic capabilities and then to provide the treatment options for each individual family, depending on what the situation is with that fetus to try to get the very best outcome possible.

Scott Steele:  I have to ask this question, even though I think I hopefully know the answer. Is it safe?

Darrell Cass:  It is safe. The cool thing about fetal surgery is there's two patients that we have to consider. There's the mother, who doesn't have any disease or condition herself, and then, there's the fetus that has a condition that requires treatment, and we have to balance the risks and benefits for each. As a center, we put the mom's health first. We would only offer treatments that would have the very minimal risk to the mother. If there was some high risk to the mother, then we may not be able to offer those treatments. So, the mom's health comes first, and then, within that confines, we do the very best we can to help the baby to achieve the best outcome possible.

Scott Steele:  So, what's on the horizon for fetal surgery?

Darrell Cass:  I think that there's a number of new developments. First is minimally invasive fetal surgery. I think we're working on that. I began working on that in Houston, and we will work on that here at the Cleveland Clinic where we can make smaller openings in the uterus to try to achieve the same outcome. I think there's a role for robotics in fetal surgery. We can argue about the benefits of the robot for other different disciplines, but in fetal surgery, I think it makes real sense. The problem is is that the instrumentation currently is a little bit big, but guaranteed, we're going to be minimizing the size as technology improves, and I believe there's going to be a role for robotics.

And then, there are some new treatments that are considered experimental currently. For example, FETO, which is tracheal occlusion for diaphragmatic hernia, currently is considered experimental, but I believe in the next year or two, we're going to have results of some randomized trials, which are going to prove a benefit. And we are well-positioned to provide that treatment here at the Cleveland Clinic.

Scott Steele:  Well, that's absolutely exciting stuff. I'd like to end up all of the podcasts with a couple of quick hitters. So, Darrell, what's your favorite sport?

Darrell Cass:  Baseball.

Scott Steele:  Darrell played at Stanford, was a catcher on the Stanford baseball team back in the day. What's your favorite meal?

Darrell Cass:  Filet mignon with king crab legs on the side.

Scott Steele:  Of course, it is, and what is the most recent book, nonmedical, that you've read?

Darrell Cass:  Probably a John Grisham novel on vacation. We're in the medical field, but I find the legal field very fascinating. So, I read one of his recent novels.

Scott Steele:  Which is circa 1990. And then, you're from Houston. You've been in a lot of different places. Tell the audience what's one of the things you like about Cleveland.

Darrell Cass:  First of all, it's beautiful. Cleveland's really pretty. The trees are amazing. There's a lake. There's a river running right through the center of downtown. I love that all the sports centers are downtown, and I see great opportunity for the future.

Scott Steele:  That's fantastic.

Darrell Cass:  I think it's a great city.

Scott Steele:  Yeah. Absolutely, and we're so glad that you've joined us here. So, for more information about Cleveland Clinic's Fetal Care Center, visit clevelandclinicchildrens.org/fetalcare. That's clevelandclinicchildrens.org/fetalcare. And to make an appointment with a Cleveland Clinic specialist, please call 216-444-9706. That's 216-444-9706. Darrell, thanks for joining us on Butts & Guts.

Darrell Cass:  It was my pleasure, Scott. Thank you so much.

Scott Steele:  That wraps things up here at Cleveland Clinic. Until next time, thanks for listening to Butts & Guts.

Butts & Guts

Butts & Guts

A Cleveland Clinic podcast exploring your digestive and surgical health from end to end. You’ll learn how to have the best digestive health possible from your gall bladder to your liver and more from our host, Colorectal Surgeon and President of the Main Campus Submarket, Scott Steele, MD.
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