Innovations in Fetal Surgery Part III: Removal of a Tumor on the Heart

Podcast Transcript

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

Hi, everybody. And welcome to another episode of Butts & Guts. I'm your host, Scott Steele, the Chair of Colorectal Surgery here at the Cleveland Clinic in be beautiful Cleveland, Ohio. And today I'm extremely pleased to welcome appearance number three on Butts & Guts, with the last one being in 2020. And that's Dr. Darrell Cass, who's the Director of Cleveland Clinic's Fetal Surgery and Fetal Care Center. Darrell, welcome back to Butts & Guts.

Dr. Darrell Cass: Thank you, Scott. It's my honor to be here.

Dr. Scott Steele: So we're super excited to have you. And today we're going to talk a little bit about something you've had and something that is so unique to a very small cohort of people out there. And that's the ability to perform fetal surgery, and one of which, that you are that person. And we're going to talk a little bit about removal of a life threatening tumor on the heart.

So for those of you who didn't listen to episodes one and two, we're going to cover your background a little bit first. So just give us the broad overview, where you're from, where'd you train, and how did it come to the point that you're here at the clinic?

Dr. Darrell Cass: Yeah, Scott. So I grew up in Southern California, and by pure luck, I matched in general surgery at UCSF beginning in 1991. And at that time it was really the epicenter of development of fetal treatments and fetal surgery. And I was honored to get a chance to work with Mike Harrison and Scott Adzick at that location. And that launched a long career that involved research and fellowships and training. I came to the Cleveland Clinic about four years ago from Houston, Texas. I was in Houston for about 18 years, where we were able to form a fetal surgery program.

And four years ago, you were one of the motivating factors for me to join Cleveland Clinic because you were so happy being here. And I felt like this was going to be an amazing opportunity to develop fetal surgery at this amazing world class healthcare or organization that has an international outreach. And for that reason, I came to beautiful Cleveland, Ohio, and we've had some success at building a fetal treatment program here.

Dr. Scott Steele: Well, we are super excited to have you here. And so on this episode, we're going to discuss the newest innovation in fetal surgery performed by you and your team. But before we get in there, if I'm listening out there, I'm like, wait a minute. Did I hear fetal surgery? What is fetal surgery? So can we just start there and give us a broad overview of what that is?

Dr. Darrell Cass: Well, maybe you've never watched Grey's Anatomy, because that's been one of the features in Grey's Anatomy through decades, is fetal surgery. So it's a simple idea, and it's an incredibly complex idea to execute. The simple idea is there are congenital conditions that a fetus or an infant can be born with that simply, if you can fix those conditions before they're born, you can improve their outcome. In the most dramatic examples, the fetus develops a problem that, unfortunately is going to kill the fetus before they even have the chance to be born. Those are life threatening conditions.

There are other conditions where that fetus might suffer lifelong disability or severe complications, or have a risk dying after birth. But the simple idea is to do something to the unborn patient that will improve their long term outcome. And there's a lot of ways we can do that. We can do it with ultrasound guidance. We can do it with needles and small trocars, fetoscopically. Or in the most dramatic cases, we actually open the mother's uterus up and we expose the fetus, and we actually do pediatric surgery on the fetus to fix that problem.

Dr. Scott Steele: So Darrell, you and I have had multiple talks and you've been featured on national prominent TV shows, but tell us a little bit about this most recent surgery you and your team of surgeons performed. And you're very humble, but what different type of teams from the Cleveland Clinic were part of it that you were able to lead in this successful surgery?

Dr. Darrell Cass: Yeah, Scott, this was just an amazing ... actually, it was one of the highlights of my career. Basically, we were able to create synergy with our fetal surgery team, which is already quite complex, and our congenital heart surgery team, which operates on babies and fixes their hearts after they're born, newborns, older infants. And we came together to treat this incredibly rare problem in this amazing family and this amazing young boy that we were able to address.

So this little fetus developed what's called an intrapericardial teratoma. A teratoma is the most common tumor that we see in babies, but they're incredibly rare. So this problem is well less than one in 100,000 live births. And unfortunately, a tumor grew on this little infant's heart, on the left side of the heart. And it grew rapidly in the period of 22, 23, 24 weeks, and it was completely squishing the heart. And essentially, the fetus was dying from heart failure because the blood circulating back from the placenta couldn't circulate properly. And the fetus was essentially dying and we had to do something.

Dr. Scott Steele: So Darrell, walk us through a little bit about what that is. I mean, you don't have to go into the gruesome details for our listeners out there, but overview, what does that involve? How do you do it?

Dr. Darrell Cass: So first, we diagnose this problem with a big team that involved our pediatric cardiologist, our pediatric cardiac surgeon, our maternal fetal medicine doctors, our radiologists, et cetera. And there were a lot of options. We counseled the family incredibly carefully about pros and cons and risks and benefits. And then we felt that if we did not intervene that this fetus was going to die within either days to a week or so, based on the time course of what was happening. And the family put their trust in us, and we then performed open fetal surgery for this problem.

So that involved making a C-section like incision on the mother after she was under a very careful anesthetic. And then we exposed the uterus and we figure out where the placenta is and we figure out how the baby is lying within the uterus. And we had to move him around to get him in the best spot, because it's really important we don't hurt the placenta, which is essentially the baby's life source, essentially, where he gets his nutrition and his oxygen, et cetera. And then we had to open the uterus in a very careful way where there's no bleeding, and we keep the membrane sac in place. And then we brought his arms out, but we kept his head and body inside the uterus. Dr. Najm then placed an IV, which we were able to use to give medications and some fluid.

And then Dr. Najm did a heart surgery just like he might do after birth, where we open up the chest, expose the heart. We saw this huge tumor that was the same size as this little fetus's heart, essentially. It was the same size and it was completely blocking the left side of the heart. Dr. Najm carefully carved this tumor off of the heart. And then, it was unbelievable what we saw. Essentially, the left side of the heart popped open. When the tumor was in place, there was essentially no blood circulating at all in the left side of the heart, it was all shutting around. After the tumor was removed, the left atrium, the left ventricle started to fill. We started to see more normal fetal circulation.

And essentially, it was a miracle, because this procedure had only been done successfully at one place in the world previously, and that was in Philadelphia. Other people had tried this very rare procedure and not been successful. And then we closed the uterus back up and we closed the mom back up. And then amazingly, everything went well from that point forward. And little Rylan was then born 10 weeks later. So he had all that time to recover and grow and for his heart to recover. And after he was born, he's essentially done great ever since then. And now he's over five months of age and doing awesome.

Dr. Scott Steele: Well, Darrell, that sounds like an incredible feat and an incredible team effort, and something like it's out of the future or out of the movies. But so glad that we were able to provide that for that family and for that young man. So how did the family end up coming to Cleveland Clinic for care to meet with you?

Dr. Darrell Cass: As we've grown our programs, there are a growing number of maternal fetal medicine specialists in our region or nationally that know about our expertise. And so this family is from Western Pennsylvania. They were getting care in Eastern Ohio, and the docs that they were seeing diagnosed this problem perfectly accurately, and promptly referred them to come see us for possible solutions. And we had been working with this group for a little while, and I think we've taken great care of their patients previously. And I think they felt like this would give the family the best opportunity to be able to save their little boy, if anybody could.

Dr. Scott Steele: So Darrell, I know it's hard to do broad strokes on questions like these, but in general ... very, very big level. When is fetal surgery recommended and when can it wait until after the baby is born? And at what stage of pregnancy is typically the earliest where you could recommend fetal surgery?

Dr. Darrell Cass: Well, that's a great question. It's an incredibly complex question, because there's so many different conditions for which we can do different types of fetal surgeries, and there are different types. So for example, there's twin-twin transfusion, and the treatment is using a laser to divide little blood vessels on the placenta. And that intervention, we can go down as low as 16 weeks or so. And we don't usually do it more than 22, 23, 24 weeks. So that one's a little earlier. When we're talking open fetal surgery, about the earliest you go is 22-ish weeks. And it just, it has to do with how the uterus is forming and how the membrane sac is forming and complications that can happen. And when you start going too earlier in time, the fetus often is just not able to get through the operation.

So for a problem such as is this, if we wait longer, if we get 28 weeks, 29 weeks, actually we then ... at that point, we may consider delivering the fetus. And then treating him at the time of delivery or after delivery. So the window is 22 to 26-ish weeks, is when we have to do these types of dramatic, open fetal surgery cases. There's other conditions such as spina bifida or myelomeningocele, an open neural tube defect, where the fetal surgery is not life threatening, it's life enabling.

So in that instance, fetuses that have myelomeningocele seal have a high risk of extreme disability. They can develop hydrocephalus, they can have weak legs, they can lose bowel and bladder control. The fetal surgery is designed to limit those disabilities. And so that's more of an elective operation that we do between 22 to 26 weeks. And we can even offer that surgery fetoscopically, although that's still investigational as to which is the best approach.

Dr. Scott Steele: So what's the typical process? If I'm a family out there and something's going on and they hear this and they feel like they know someone or they themselves have to get in contact with our Fetal Care Center, how do they go about that? Did they talk to the OB-GYN, maternal fetal medicine, their PCP? And then what can a family expect when they have a visit with you or one of the colleagues at the Fetal Care Center?

Dr. Darrell Cass: Well, first of all, we're happy to help in any way, whatever is needed. Families can come to us in a number of different ways. Sometimes their regular obstetrician knows about us and may call us to refer a patient. Or more commonly the maternal fetal medicine doctors that they're seeing diagnose the problem, know what the options are, and then refer the family to our fetal care program. Sometimes families find us themselves. They search Dr. Google. They have friends, their support groups. And sometimes they come to us directly, and the family contacts us directly. And sometimes it's through the web, things like that.

The first stop would be our fetal care nurse coordinators, Carrie, Sue, Debbie. The family calls our phone number, they email us, and those coordinators will respond promptly and call the family and find out what their situation and their concerns are. They can help get records that might be needed to help with an assessment. And then they set up a consultation, and we can do this any way. We can do it virtually, we can do a phone call. More commonly, the family ... actually, we plan a day for them to come and get some testing done. It might be a fetal echo, we might get a fetal MRI, might do an ultrasound.

And then to get different evaluations, whatever is needed in that particular case. It might be with a cardiologist. In this case, a cardiac surgeon. A pediatric surgeon, a maternal fetal medicine, a geneticist, a urologist. There's lots of different consultations that might be needed for that particular problem that that family is concerned about. And then we try to communicate back with the referring doctors and the referring providers. And then we come up with a comprehensive treatment plan. Sometimes it's just to provide an opinion, sometimes it's to assume care, and sometimes it's to even do a fetal intervention or a fetal treatment. And that process is taken very carefully with lots of consultations. We discuss risks and benefits, and then we really try to do the right thing for each individual family.

Dr. Scott Steele: So what's on the horizon as far as additional Fetal Care Center research that you and your team hopes to undertake in the near future?

Dr. Darrell Cass: Heart problems is one of our primary interests, and Dr. Najm and our cardiology team are thinking about new approaches to complex cardiac problems. So fetuses can be born with different kinds of birth defects involving the heart. And some of them have very poor prognosis. There have been some fetal interventions that have mixed results. And we're thinking about ways to approach some of these more rare, challenging problems with different kinds of fetal surgical techniques. And so that's something we're actively working on now.

We do have a plan to develop more fetoscopic approaches to different kinds of problems. There are pros and cons. We have the technology, and we have to figure out the right time to use it for the right patient. And that's something we're actively working on as well.

Dr. Scott Steele: You know, Darrell, I've been interviewing surgeons from all around the world since 2013, and I've never asked him this question. Do you get nervous performing some of these procedures?

Dr. Darrell Cass: That's a great question, it's totally fair. I'm sure you know this too. I do complicated surgeries on babies, on children, and this fetal surgery. And I guess I know that I've learned this about myself. In the most challenging circumstances, I often might be nervous, but I end up ... my brain focuses on what I'm going to do to help that fetus. And so that takes everything away.

I try to keep a sense of humor in how the team interacts and our teamwork. I try to make sure everybody is listened to. We go through and all introduce ourselves constantly. We all talk constantly, so that every team member is listened to and felt like an important part of the team, because they all are. And then we just do the best we can to help that mom and that little fetus in a safe way, and the most productive and efficient way possible. I guess I feel a little bit blessed that I'm pretty good at this.

Dr. Scott Steele: Well, that's a great answer. And I'm always amazed at how quickly hours fly by when you're so focused. And so, Darrell, you have been on Butts & Guts a couple of different times. I've asked you a couple of different rounds of quick hitters, so I'm going to try some new ones on this time out. So first of all, what was your favorite car?

Dr. Darrell Cass: My favorite car would ... oh, that I've owned or that I would love to have?

Dr. Scott Steele: Yeah. You can answer both.

Dr. Darrell Cass: My favorite car that I've owned is a BMW M6, period. But my favorite car might be a Ferrari, like a Ferrari California. But ...

Dr. Scott Steele: That will never come. But what was your first car, by the way?

Dr. Darrell Cass: A Ford Anglia, which is like a ... it's a British design Ford vehicle. I also had a Ford Pinto, Scott.

Dr. Scott Steele: Fantastic. I will have to look up the other one.

Dr. Darrell Cass: Do you remember the Pinto?

Dr. Scott Steele: Yep. I absolutely do. So second one, sweet or sour?

Dr. Darrell Cass: Sour.

Dr. Scott Steele: Third, as most people know you, you enjoy working out and everything. So what type of music do you listen to when you're working out?

Dr. Darrell Cass: Lately, Dua Lipa.

Dr. Scott Steele: There we go. And then finally, you've lived in a bunch of different places. What's the favorite place that you've ever lived?

Dr. Darrell Cass: Yeah. Well, I love Cleveland, but I loved living in Diamond Heights San Francisco at the top of the hill with a view of the Golden Gate Bridge and the bay from my small studio apartment.

Dr. Scott Steele: Leave it to a California person to say California. So give us a final take home message for our listeners regarding fetal care surgery.

Dr. Darrell Cass: Well, it's a rapidly growing and evolving field. We're here to just simply help, either refer doctors or families. We're happy to do a phone call, to do imaging, to provide a second opinion. And then we promise to do the very best to take care of each family and each patient, and to do the right thing for that family and that patient. And there's always options. There's always surgical options. There's always nonsurgical options and there's always things we can do. And we promise to just provide a patient and family centered environment to deliver the very best care.

Dr. Scott Steele: Well, that's super exciting. And again, we're so glad to have you here. So for more information about Cleveland Clinic's Fetal Care Center, visit clevelandclinic.org/fetalcarecenter. That's clevelandclinic.org/fetal, F-E-T-A-L care, C-A-R-E, center, C-E-N-T-E-R. And to make an appointment with the Fetal Care Center, please call 216.444.9706. That's 216.444.9706.

And remember, it's important for you and your family ... you hear me say this all the time. To continue to receive medical care, regular checkups and screenings. And rest assured, here at the Cleveland Clinic, we're taking all the necessary precautions to sterilize our facilities and protect our patients and caregivers. Darrell, Dr. Cass, thank you so much for joining Butts & Guts.

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

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