Innovations in Fetal Surgery Part II: Treating Lung Malformations

Podcast Transcript

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

Hi everybody, and welcome back to another episode of Butts & Guts. I'm your host, Scott Steele, the Chair of Colorectal Surgery here at the Cleveland Clinic, in beautiful Cleveland, Ohio, and we're very pleased to have Dr. Darrell Cass back. This is appearance two on Butts & Guts. His previous one was in 2019. Darrell is the Director of Fetal Surgery here at the Cleveland Clinic, and the Director of the Fetal Care Center. Darrell, welcome back to Butts & Guts.

Darrell Cass: Thank you so much for having me.

Scott Steele: So we're going to entitle this one Innovations in Fetal Surgery, Part Two, and we're going to talk a little bit about treating lung malformations, but before we get there, for our listeners who have not heard your wonderful previous podcast, why don't you give us a little bit of background about yourself, where you were born, where'd you grow up, where'd you train, and finally coming here to Cleveland?

Darrell Cass: I'm from California. I was born in the Los Angeles region in the Valley. I went to Stanford University, and then UCLA for medical school, and I was incredibly lucky to do my general surgery training at UCSF in San Francisco, where I met the world leaders in the field of fetal surgery, really, the world innovators, Michael Harrison, Scott Adzick, and immediately I was attracted to the whole field of fetal surgery, the concepts of trying to do things before birth, to try to improve the outcome of babies with these complex different types of birth defects.

I then did some time as a research fellow at Children's Philadelphia, as well as in San Francisco. I did my pediatric surgery training in Houston, Texas at Texas Children's Hospital, and then I had the great fortune of staying on as staff there, where I developed the third fetal surgery program in the United States, or really, the world at that time. And we became very, very, very successful, but then this amazing opportunity came to join the Cleveland Clinic about two and a half years ago. In fact, you were a motivating factor, because you and I have been friends for a while. I knew that you came here and built this amazing program, and had nothing but great things to say, and I decided what the heck, let's come and see what the Cleveland Clinic is all about, and I've been incredibly happy to make that move.

Scott Steele: Well, we're sure glad to have you here, and we've had the opportunity here of kind of following in the footsteps of giants. So let's take a high level overview. I know you mentioned it briefly, but what is fetal surgery? I mean, that sounds scary to me, and I know what you do. So for the listeners out there, what does that mean?

Darrell Cass: It's essentially doing what we do as pediatric surgeons, where we treat babies with different kinds of birth defects. It's essentially doing similar things, but doing it before the baby is born, so before the umbilical cord is cut. In the most dramatic example, we do treatments in the middle of development, halfway through pregnancy, where we might open up the uterus and fix some problem in the fetus, and then try to close the uterus back up, and hope the pregnancy can continue all the way to the normal time of birth.

But there's ways we can do that, either with ultrasound guidance, we can do things by putting a scope in, called Fetoscopic surgery, and then sometimes we do interventions just at the time of birth. So we take advantage of the placenta and the fact that the fetus is getting oxygen, getting rid of carbon dioxide at the time of delivery, and we do things to stabilize the fetus, when we can predict that they're going to have immediate distress after birth if we don't do something, and then we stabilize them, and then we cut the umbilical cord, and that, we call an EXIT procedure.

Scott Steele: I would encourage all the listeners, in 2019, Dr. Cass joined us here on Butts & Guts to talk about Cleveland Clinic's first in utero fetal surgery that repaired a spina bifida birth defect, and this was in a nearly 23 week old fetus. And so, please go back and listen to that fascinating episode. But get us up to date since then, kind of where are you at since then, how's the fetal surgery program evolved, and what sort of additional innovative practices have you been involved with?

Darrell Cass: Thanks for that question. So when I first got here about two and a half years ago, it was important that we worked to build a team. So that involves maternal-fetal medicine, pediatric radiology to improve fetal MRI capabilities, anesthesiology, obstetric and pediatric anesthesiology, other obstetric providers, and then other pediatric specialists that have different expertise, urology, neurology, other types of things. Neurosurgery is really important. So we put the team together, and we started doing simulations and practices, and then our first fetal surgery, just as you mentioned, was in February of 2019. Since then, our program has continued to grow and develop. We've since done 13 fetal surgery procedures.

And our focus so far has really been program building and improving on technique, with some focus on maternal indications. As you know, this is a relatively new field. It's not practiced by many institutions, and there's tons that we can learn. What's the best time to operate? Are there maternal conditions that we have to take into consideration? Is it safe or not safe? For spina bifida, as our first case was, there's so much we have to learn. And so, we've been working on kind of perfecting our surgical technique and improving on indications. In the future, we're going to evolve into more fetoscopic procedures where it's less invasive, but you have to understand that that term, less invasive, is not quite like in your field because we still are opening the mom's stomach up, and then we're making it less invasive through the uterine wall. And there's technical challenges there, and the mom's experience has to be balanced in, because current fetoscopic things are taking a lot longer, and maybe the mom's going to be higher risk.

So, but those are areas of development for the future. There's a few clinical trials underway treating diaphragm hernia, which I believe we're going to get some results of that in the near future, and we hope to be leaders in the field of treating fetuses with diaphragmatic hernia.

Scott Steele: That's extremely exciting, and sometimes whenever I'm around you, I'm just amazed at some of the things that you can do. So let's talk a little bit more about first, what is a lung malformation? Let's start there, and then if you'd kindly then just take me into the next steps, as how does a pediatric fetal surgeon play a role in lung malformations?

Darrell Cass: Well, lung malformations is an area of my expertise and interest that's evolved over 30 years, actually. There's lots of names we give to these things, but essentially, they're congenital malformations of the lung. In the past, we've used terms like CCAM. Currently, there's a term that's in vogue called CPAM but essentially, it's malformed lung tissue, sometimes there's an extra blood supply that goes there that comes off of an anomalous source. Sometimes they're more cystic, sometimes they're more solid, and in fact, occasionally there can be neoplasms of the fetal lung. And in fact, I've had a rare experience treating neoplasms of the fetus, but those are incredibly rare.

So in general, these lesions, there's no cause that we know of. There's no genetic cause, there's nothing the parents do that cause these things, it's just essentially bad luck, and they can come in lots of different forms. Sometimes they're relatively small, and the fetus is completely asymptomatic. Sometimes they grow really rapidly before birth, usually between about 20 and 26 weeks, and in fact, they can actually cause fetal problems, in that case. They can squish the heart, they can squish the diaphragm, they can compress the normal other developing lung that's trying to grow during that time. And it turns out, it's important to try diagnose these things before birth, in order to try to optimize the outcome.

And it's an area where I've had the good fortune of really studying now for about 25 years and doing lots of research, first in the basic science arena, trying to understand what causes these things, looking at the molecular structure of these, looking at different proteins that may cause them, different animal models, and that was 25 years ago now. And then also now more recently, studying kind of the clinical effects of these, how we can best diagnose them, how we can best classify them, how we can best predict how they're going to actually, and it turns out we're pretty good at that these days. We can look at how they look by ultrasound and fetal MRI, and I can pretty accurately predict how the course is going to go, and we can then group a subset of these, called high risk lesions, that pose a risk of causing harm to the fetus, and we have to follow those very closely and sometimes, they can lead to even fetal death, in rare instances, if they're not followed closely enough.

In that setting, we will start to see signs of heart dysfunction or heart failure, and there's two choices, and what choice is best for a family is just really dependent upon the family. All we try to do is try to educate them on what we're seeing and what the options are, and to help decide with them, but in some instances, we might have to do open fetal surgery. So if we don't do something, the fetus dies, but what we have to do is we have to open the mom's uterus up. We have to take the mass out of the fetus, close the fetus back up, close the uterus up, and then hope the baby can recover.

And I've had an incredibly unique and unusual experience with that, having done that now six times with success in five of those cases, which is unique and an excellent outcome. And when I say success, I mean, kids that have later gone on to play T-ball, to hula hoop, to one kid even being an actor on the Disney Channel and getting his Screen Actors Guild card. So that's the type of outcome that can happen following open fetal surgery. So it's incredibly scary, but when we offer it, that's the outcome we're hoping for.

Scott Steele: So truth or myth: the most common forms of fetal surgery involve taking the fetus out, operating on it and putting it back in?

Darrell Cass: Yeah, that would be a myth. First of all, we never actually try to take the baby out, but that would be what most people think, where the fetus actually [inaudible 00:10:41] completely out of the womb. We try to keep as much of the baby inside of the womb as we can, keeping the biblical cord okay, trying to keep the baby warm inside with the warm amniotic environment. So we don't really ever remove, we just try to open the uterus as small as possible, but then we do a lot of things now fetoscopically. Twin-twin transfusion is done completely with a small little scope, a small trocar, and a small single scope that goes in, and we use a laser to ablate communicating vessels between the two twin fetuses. So most common is fetoscopic or ultrasound guided interventions.

Scott Steele: Second, truth or myth: operating on fetal surgery is safe.

Darrell Cass: That one's tough. Let's see. The answer I suppose is true, because it is safe, but it's very tricky. So it's high risk. So it's important to have a very well-trained team of anesthesia, maternal fetal medicine, fetal surgeons, cardiology, because we are monitoring the baby throughout the procedure to make sure they're doing well, to ensure their safety, but it is very high risk and lots of complications can happen, but we can do it safely. The fetus can have an operation, and have a completely normal life thereafter.

Scott Steele: When is fetal surgery recommended? And you mentioned talking about indications, and this is probably the crux of the matter here. So when in your mind can it just not wait until the baby's delivered? And take apart those EXIT type of procedures that you talked about before. When do you got to go in there and say, "You know what? High risk. No question it's high risk," and I really appreciate your answer on the last one, but what are those indications where you're like, "Let's jump in there?"

Darrell Cass: The simple answer is we only do it when we have to. So we only do it when we know that by doing it, we're going to get the best outcome for that baby. The indications include twin pregnancies, complicated twin pregnancies. Twin-twin transfusion is a common one where we have to do laser ablation of communicating vessels between the two twins. We also do it for a baby that might have a bladder problem, a bladder outlet, they're not able to pee. We might have to do shunts. Sometimes we put shunts into a cystic area of the lung. Those are less invasive, but they're things that we might have to do.

Open fetal surgery, it's actually a proven benefit for spina bifida, for myelomeningocele. For most types of open neural tube defects, fetal surgery leads to the best long-term outcome for those babies. What I talked about fetal surgery for a lung lesion is incredibly rare, we also can do it for fetuses with a teratoma that becomes very vascular, causes high output cardiac failure, and hydrops, that also is a lethal condition unless you go in and treat that. And then there's a few areas where they're still under investigation for diaphragmatic hernia, occluding the trachea, called FETO, is an option, but it remains experimental, but I think we're going to have an answer to that in the next year. And then cardiac lesions, there are indications for fetuses that have congenital heart lesions that probably will help improve their outcome, but the evidence supporting it is less clear and they're still under investigation.

Scott Steele: So I'm a mom at home pregnant or about to get pregnant, and I think for whatever reason, I need to get in contact with a fetal care center. Is this something they talk to their OB-GYN about, or maternal fetal medicine specialist, or how does one go about even reaching you or knowing that you're an appropriate person to reach out to?

Darrell Cass: Yeah, that's a great question. I would start by your primary care team. Usually, it's a maternal fetal medicine specialist and there's many of these in every region of the United States and the world. I would start there, but you must understand that there is various experiences with these, and the types of things we're talking about here at the Cleveland Clinic are incredibly rare. And so, your team may not have a lot of experience, or maybe their information is just not updated, which is why we're doing this podcast, we're trying to improve everybody's understanding and the availability of these types of things.

So, but start with your primary team, and then if you have questions, if you feel comfortable, great, if you have questions, if you want to get second opinions, we would be happy to provide that. We're still working on our website, but we have a fetal care center website at the Cleveland Clinic under the main Cleveland Clinic heading, that has all the contact information. Your primary contact here will be our fetal nurse coordinators who are an excellent team, who will take your story, help gather information from your caregivers, and then provide options for you to come here for a consultation. Sometimes I just talk to families on the phone. Actually, we've communicated via Facebook at times, or you may be appropriate to come here for a comprehensive evaluation. And we will try to ensure that it would be family-centered care, where we try to do one-stop shopping, where you can get all the testing you might need, all the consultations you might need, and I think usually, are able to leave here with better understanding of what the condition is and what the options are.

Scott Steele: So considering the time which we're recording this, I think one of the questions that often comes up is with coronavirus here as a part of our daily lives, at least for the foreseeable future, how are you keeping your families and your patients safe in this time?

Darrell Cass: Well, I think this is really one of the advantages of the Cleveland Clinic. The Cleveland Clinic is a world leader, and certainly a national leader in this coronavirus fight. We've been looked to be leaders in the whole care delivery process in this arena. And so, it's a delicate balance because we want to provide access to the care that you need, but provide it in the most safe way possible, and the Cleveland Clinic is trying to do that, and they've done it in an exceptional way. So when you do come in for a visit these days, you do get screened for possible COVID exposure. You get your temperature checked, you will wear a mask, which the whole world is doing that currently, so that's not a big surprise, and your care team will be wearing masks, and everything will be done to try to optimize the safety of all the families coming here for evaluation. I think the Cleveland Clinic are leaders in that regard.

Scott Steele: So what's on the horizon, as far as additional fetal care initiatives that your team hopes to undertake in this next segment, before we have you back on?

Darrell Cass: Thank you. Well, we are bringing in a new team member in August, who's an expert in fetoscopy, and care for fetuses with complicated twinning, so that's really exciting. He's joining us from Memphis, Tennessee, and so I hope maybe in the next year, we'll do an update that will have him being featured in all the things we do for caring for complicated twins. So we're going to move more toward fetoscopy, we're going to be leaders in the treatment of fetuses with complex forms of diaphragmatic hernia. We've already worked to develop programs in caring for neonates with that problem. We're going to provide probably more innovative techniques in how we repair spina bifida, and we're going to continue to excel and be on the forefront of treating all fetuses with lung malformations, CPAMs and CCAMs.

Scott Steele: So we close out here, we always like to end with some quick hitters. We've already asked you some, so we'll go with a next set of topics for you for our repeat guests here on Butts & Guts. So jelly or jam?

Darrell Cass: Jam, for sure.

Scott Steele: Salt or sweet?

Darrell Cass: Salt.

Scott Steele: And if you had to have sweet, is it hard candy or chocolate?

Darrell Cass: Chocolate.

Scott Steele: And finally, going back and talking to yourself back in the day and said, "You should really choose fetal surgery because of blank," what would that blank be?

Darrell Cass: It's incredibly rewarding, not only to treat the fetus and the baby, but just to engage with the family in the whole care process, and I would do the exact same thing again. It took a long time to get here and develop this program and expertise, but I would do it exactly the same way again.

Scott Steele: So how about a final take home message for our listeners, in regards to not only fetal surgery, but also treating these lung malformations?

Darrell Cass: I would say don't settle with there's nothing that can be done. I've heard that story many times, families hear that often in initial consultations. That's just not true. There really is always something that we can do, and we'd be happy to provide the state-of-the-art care here at the Cleveland Clinic.

Scott Steele: So for more information about Cleveland Clinic's Fetal Care Center, please visit clevelandclinicchildrens.org/fetalcare. That's clevelandclinicchildrens.org/fetalcare. And to make an appointment with a Cleveland Clinic children's specialist, please call (216) 444-9706. That's (216) 444-9706.

And truly remember, in times like these, it's important for you and your family to continue to receive medical care, and as Darrell said, rest assured, here at the Cleveland Clinic, we're taking all the necessary precautions to sterilize our facilities, protect our patients, and make this the safest place in the world to receive care. Darrell, thanks for joining us on Butts & Guts.

Darrell Cass: It's been 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.