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Bill Klaproth (Host):  So, let’s talk about the ex utero intrapartum treatment or the EXIT procedure. In this episode, we’re going to learn about the intricacies of the EXIT procedure that doctors perform while the baby is partially born and still in the mother’s whom. We will also learn about the role each medical expert plays in making sure the procedure runs smoothly for both mom and baby. This is Pediatric Insights; Advances and Innovations with Children’s Health, where we explore the latest in pediatric care and research. I’m Bill Klaproth. With me, is Dr. David Schindel, a Pediatric Surgeon at Children’s Health and Associate Professor at UT Southwestern and Dr. Sushmita Yallapragada, a Neonatologist and Medical Director of the Fetal Center at Children’s Health and Assistant Professor at UT Southwestern.

Dr. Schindel and Dr. Yallapragada thank you for your time. Dr. Schindel, let’s start with you. Why do you wait until just before birth for the EXIT procedure?

David Schindel, MD (Guest):  So, these babies are babies that have been identified as having a lesion typically in the head and neck or chest region that might or is very likely to impact their airway upon birth. And in the time of modern imaging, ability to identify these babies who are at risk of losing their airway at birth; obviously gives us an opportunity to intervene. And so, what you are trying to do is you are trying to let the baby mature to the extent fully possible within the uterus but then be prepared to deliver them in a controlled fashion at a time when their lungs are mature enough to be able to oxygenate their blood appropriately.

So, typically, the goal is to get around 34 to 36 weeks gestation before you consider an EXIT procedure.

Host:  Dr. Schindel, let me stay with you. Can you explain to us how the EXIT procedure works?

Dr. Schindel:  So, basically it takes advantage of the most efficient oxygenating machine ever invented and that’s the human placenta. The placenta obviously delivers oxygenated blood to the baby transporting oxygen across the placenta from mother to baby. And we basically take advantage of that uteroplacental circulation so that as the placenta is delivering oxygenated blood to the baby, we control the natural response of the uterus which is to contract and for the placenta to separate from the uterus itself.

We inhibit that process and by maintaining a placental uterine volume as well as use of tocolytics to prevent separation; and then while the baby is receiving oxygenated blood through the placenta; we then work to establish an appropriate airway for the baby so that we can then once we have an airway and that might be either through simply placing an endotracheal tube, it might be performing a tracheostomy, it might be performing a resection of a mass if we cannot actually obtain access to the trachea. I’ve done a thoracotomy which is an incision on the chest to remove a chest mass during and EXIT. But the goal is to establish an airway, to establish a viable route of delivering oxygen to the baby and then once that happens; we can then disrupt the placental circulation in the normal fashion by dividing the umbilical cord and then at that point, the maternal fetal medicine specialist take care of mom and the neonatologists take care of the baby.

Host:  Right. So, you oxygenate the baby through the placenta until after the procedure is performed. So, Dr. Yallapragada, I’m going to bring you in. So, Dr. Schindel just said that an EXIT procedure is generally performed when there’s a mass that has impacted a baby’s airway. Can you talk about the other pathologies that would require an EXIT procedure?

Sushmita Yallapragada, MD (Guest):  Sure. So, the EXIT procedure can really be divided into three major categories. And there are multiple pathologies within those categories that we can go through. But I’ll kind of hit the highlights. So, an EXIT to airway is the first category. So an EXIT procedure to help a baby with airway stabilization. So, as Dr. Schindel talked about, babies with a cervical teratoma or lymphangioma which is a type of tumor that might be blocking their trachea, compressing it, deviating it, making it difficult for the baby to oxygenate and ventilate after birth. Another example would be congenital high airway obstruction syndrome, micrognathia and a variety of different types of chest masses.

A second category would be EXIT to ECMO or extracorporeal membrane oxygenation. There are babies that are born with a specific disease called congenital diaphragmatic hernia where there is an abnormality in the diaphragm which then allows the stomach contents and the gut contents to herniate through that opening and can cause maldevelopment of the lungs. And so many of those babies will require an EXIT procedure to then go onto ECMO or basically oxygenation support outside of the womb.

There are also severe congenital heart defects that fall into that same category of EXIT to ECMO.

A third category would be EXIT to resection. So, a couple of examples of those might be a congenital pulmonary airway malformation or CPAM and secondarily a bronchopulmonary sequestration. And these would be extreme cases.

Host:  Got it. Dr. Yallapragada, staying with you, what are the risks then for baby and mom through this procedure?

Dr. Yallapragada:  Yes. We really start this counseling for the family and for mom prenatally. We talk a lot about the risks for the baby and the mom so that there is an expectation set before we even begin the procedure. We get the team together et cetera. So, most moms when they deliver babies, they are either delivering a normal vaginal delivery where they have an epidural in which is really just regional anesthesia, or they deliver via cesarean section where they also have an epidural in and perhaps other adjunctive anesthesia, but most moms do not require general anesthesia. By that I mean, the mom herself is intubated with a breathing tube and she is put to sleep entirely.

And so in the cases of an EXIT procedure, the mom because of all the reasons that Dr. Schindel explained earlier, the mom does have to go under general anesthesia. And we have to control the uterus, the contractions and the relaxation of the uterus ourselves which does put the mom at higher risk. Because she will be under general anesthesia. Now, the baby, the fetus, might feel some of that general anesthesia and might be a little bit slower to respond as far as breathing on his or her own or bringing up the heart rate or being as active. In addition, because of the type of anesthesia we have to give the mom, there is a higher risk that mom will bleed excessively or hemorrhage and so that is a big risk for mom as well.

As far as the baby, the risk is really despite all of our efforts to get an airway in or remove the mass or place a tracheostomy; that we may not be able to do those things despite all of our best efforts. And so, mortality is a risk.

Host:  So, Dr. Schindel, Dr. Yallapragada just mentioned the team in her last answer. There’s a story on the Children’s.com website about a baby named Hunter who had the EXIT procedure performed by you and your team, ultimately saving his life. The story talks about the highly specialized team that you assembled for the EXIT procedure. First off, can you talk about your role in this team and then after, I’d like to ask you who else is on generally an EXIT procedure team.

Dr. Schindel:  I started the Exit team in 2007. We’ve done a number of cases since then. They are not highly common. The members of the team for the most part have been people that I’ve worked with for a long time who I’ve known to be excellent at their job as well as have a team mentality. These sorts of procedures are potentially stressful and the way that we mitigate that stress at least amongst the team is to be well-prepared and to have kind of an understanding and a great working relationship amongst the team members.

And so, in 2007, the team was created in part because of a baby that was somewhat of a surprise if you will in the sense that it had a chest mass that was causing some trouble and who the neonatologist and the maternal fetal medicine docs were looking for a way to help the baby. And so, this was something that we created at a relatively spur of the moment if you will.

The team is a multidisciplinary. The current team members for the most part – the physicians are for the most part the same since 2007 with some small variations. The nurses with the exception of one or two are all relatively new to the team at least in the last year or so. The main member of the team besides myself, which is a Pediatric Surgeon, the main member of the team is Pediatric Ears, Nose and throat physician that I have worked with for a number of years named Roman Johnson. Dr. Johnson is a fantastic pediatric ears, nose and throat specialist and he and I collaborate very closely not only on the EXIT team but in general.

And basically, what we do is to look at these cases on an individual fashion, individual manner and discuss what if in fact airway is at risk, what we need to do to address it. And fortunately the imaging that is so – the modern imaging is so fantastic that we really can get a pretty good holographic view in our minds what this – what the airway might look like and what we need to do about it.

We also have as part of the team is our Pediatric Anesthesiologist. Again, with the multidisciplinary approach, my view of putting the team together has always been about putting the experts into a room and then let them go to work. Our Pediatric Anesthesiologist intubate probably up to 100 patients a day in terms of the operating rooms and the radiology rooms and et cetera. And so they look at pediatric airways on a daily basis. And they are quite expert in acquiring airways that are difficult dealing with complicated issues and so, Dr. Derek Walsh has been our main Pediatric Anesthesiologist and then for this last case that we did in October, Dr. Eddie Kiss and Dr. Umar Kan also participated in the EXIT.

And again, these gentlemen are – deal with pediatric airways every day and are trusted colleagues. In the case that we did in October, that baby had an immature teratoma of the neck that was obstructing the airway. And two days later after some fetal imaging; the baby underwent surgery to have that resected and the anesthesiologist who helped us with the airway at the time of the Exit, helped participate in putting that baby to sleep 48 hours later. So, the continuity of care provided some degree of comfort if you will, maybe even understanding of the complexity of the airway so that we can then move to the next phase after the EXIT in a safe fashion.

The other people obviously, Patricia Santiago-Munoz who is the maternal fetal medicine specialist. She’s the main MFM how helps us with this and then Dr. Yallapragada. But the unsung heroes of this team is the nursing staff. The nursing staff are absolutely amazing. Again, these ladies and gentlemen, they work with us in the operating room on a daily basis and so they know us inside and out. They know the equipment that we like to use. They know – they communicate with us in a way that’s efficient and they are just unsung heroes of all of this.

And I always try to make it a point to recognize them. Mary Reese is a nurse who is one of the Charge Nurses in the operating room at Children’s and she has been over the years responsible for ensuring that our equipment is functional, that we have all the equipment that we need. Because basically the EXIT procedure is a combination of multiple procedures that we do on a daily basis. Things like intubating, tracheostomies, neck mass resections, chest tube placements, central line placements, thoracotomies. We do these procedures pretty frequently, but the EXIT is a coordinated effort that combines all these procedures into one procedure.

And so, that’s a whole lot of equipment that needs to be managed, organized, kept functional, and kept at a moment’s notice. And Mary Reese has been extremely valuable in making sure that that happens. Literally, if the – if one of these cases was to become a reality, if another case was to present itself; Mary is definitely the first person I call.

And then there’s a whole other group of nurses that help in the – that are either ears, nose and throat nurses or pediatric surgical nurses and so, they help us on a daily basis in the operating room and again, they are the folks that make sure that the equipment is working, pass the equipment to us, stay organized. They are just phenomenal. And then the ears, nose and throat nurses that help Roman Johnson on a daily basis are just fantastic at what they do.

Dr. Yallapragada:  And I’ll just add a little bit onto that. So, the neonatology team is actually involved on a couple of different levels. Not only is the neonatologist scrubbed into the procedure to help secure the airway a long with anesthesia and ENT. We also then have a neonatal resuscitation team and our team is lead by Dr. Myer Wycoff and it is an internationally recognized neonatal resuscitation program. So, one the baby is actually separated from mom, the neonatology team takes over. And that team consists of a couple of neonatologists, trainees for neonatology or neonatology fellows, residents, pediatric residents, nurses, neonatal ICU nurses, neonatal ICU respiratory therapists that all come together to really make sure that the baby is doing well post EXIT and is safe and secure and ready for transfer.

Host:  Well thanks for adding that in Dr. Yallapragada. So, Dr. Schindel, how do you put a plan together for all of these multiple procedures in a coordinated effort? How do you plan for an EXIT procedure?

Dr. Schindel:  Well I think that it starts with just trying to learn something about the baby. Obviously, not every – we’ve done a number of EXITs and I can say with great confidence that I don’t think any two of them are alike. They have some general similarities but there’s always something subtle. The EXIT team, the EXIT protocol, the EXIT plan, the surgical strategy if you will is basically predicated on the idea of keeping multiple contingencies into play. So, what I mean by that is that if you try to maintain, you try to obtain the airway in the simplest way possible. And then if you can’t get it that way, then you move onto the next route. And then if you can’t get it that way, you move up to the next route. And if you can’t get it that way, you keep moving – you keep planning for these contingencies, but your goal is to obtain the airway in the simplest fashion.

And once you do obtain the airway, the EXIT is done. Now, for example, the case in October, that baby, we were unable to visualize the airway with just the laryngoscope and we then ultimately did visualize the airway with a bronchoscope and obtained the airway then directly by intubating over the bronchoscope. But the baby’s neck mass was mobile enough that we could pull it if you will, off the airway to the point where the endotracheal tube could be passed beyond it and appropriately ventilate the baby. And at that point, the EXIT was finished, and we gave the baby to Dr. Yallapragada.

The baby Hunter that you referred to, that baby’s airway was so compromised by the mass that we had to resect the mass, make an incision, resect the neck mass and then do a tracheostomy, intubate the trachea directly in order to even visualize the airway at all. And so, each step in the EXIT is a preparation of preparing for all multiple contingencies.

So, the accuracy of the imaging is super important. It helps us prepare just like coach might prepare a football game having viewed the film of opposing team from their last game. You are preparing with the best plan possible. And because we have all these specialists, multidisciplinary specialists who have done – who do these things on relatively daily basis; but just not in the combined EXIT effort; everybody is relatively methodical about it.

While you are doing an EXIT for the baby, or why you are trying to obtain an airway for the baby probably one of the more important aspects of this is to remember that you have two patients on the table. You have a baby that the focus seems to be on but you have mom and mom may be the most important one of the two. And the health and safety of the mom is of paramount importance and we rely on our maternal fetal medicine specialists and our adult anesthesiologists or obstetric anesthesiologists to take care of the mom as we focus on the baby.

But taking care of mom is a huge part of this. And so, while you are preparing for an EXIT, you have to also keep in proper perspective the health of the mom and in many instances, this is likely to not be the mom’s last pregnancy. And so one metric of outcomes for our EXIT procedure is that we want to A: Protect the mom, the health of the mom and her fertility, her subsequent fertility. And we’ve been quite successful with that here UT Southwestern Children’s Health.

Host:  Right. Speaking of that, Dr. Yallapragada, let me turn to you again. Dr. Schindel was just talking about providing the EXIT procedure. What makes you unique in providing this procedure in the area?

Dr. Yallapragada:  Yeah, great question. I think what makes us so special is our three pronged approach. It really starts with our obstetrics and maternal fetal medicine docs. They are the ones that identify the mom, identify the issue in the fetus. They refer the mom for imaging. The interpret all of that. They put is all together and then present it to the rest of the team which is neonatology and surgery. And once that is completed, we then through the fetal center or Fetal Evaluation and Treatment Alliance Center here at Children’s; we then start the process of putting together a multidisciplinary team to come together and talk about this and come up with a plan.

The EXIT team is lead by Dr. Schindel and the Fetal Center and I’m the Medical Director here and so my role here was to really bring together the physicians, the social workers, the sort of support staff, the family, everyone together to talk about what the plan is beforehand and then set up anything going forward as far as logistics for the family and for the staff and physicians that are responsible for this going forward.

And so, I think coming together in our environment we have all of the pediatric subspecialists. We have surgical subspecialties, and medical subspecialties and a lot of talent in our group. And I think we all really work together very well. We communicate really well. And we are really here for the best interests of the patients both mom and baby. And so, coming together beforehand, coming up with a plan and really trying to identify any issues that could be going forward; I think what’s helpful in this situation as we prepare for this procedure. And putting together a team that is skilled not only on the social, emotional and logistic aspects but of course, the surgical, postnatal, resuscitation and the neonatal ICU side of it.

Host:  And as we wrap up Dr. Schindel, for any physician listening to this, what do you want them most to remember when it comes to the EXIT procedure?

Dr. Schindel:  I just think that for those physicians who are caring for an expectant mother and family who identifies a child with a congenital anomaly of the head and neck or chest, that they are concerned about the nature of the airway or the safety of delivering the baby spontaneously; that we are here to help them. We are here to help them through figure that out. There are some clear indications for an EXIT procedure and it’s not just having a mass or having some sort of anomaly. The indications are quite distinct. And we can help them figure that risk out and then if in fact the baby is at risk for an airway issue at birth; then there is a viable mechanism here in north Texas to help them take care of it.

Host:  Well Dr. Schindel and Dr. Yallapragada, this has been really informative. Thanks for your time.

Dr. Schindel:  You bet.

Dr. Yallapragada:  Thank you.

Host:  That’s Dr. David Schindel and Dr. Sushmita Yallapragada. And thank you for listening to Pediatric Insights. For more information please visit www.childrens.com/exit. That’s www.childrens.com/exit. And if you found this podcast helpful, please rate and review or share the episode and please follow Children’s Health on your social channels.