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Scott Webb: One of the only comprehensive fetal centers in the country. The Chicago Institute for Fetal Health at Lurie Children's treats patients with complex pregnancies. Among the rarest and most serious conditions they see is twin to twin transfusion syndrome. A complication that affects 10 to 15% of identical twins. In this condition, the twin chair of placenta, which can lead to extreme prematurity and significant injury to major organs or even death.

Fetal surgeons like our guest, Dr. Er. Aha.

are sometimes able to correct this condition in utero. Today, we'll talk with him about this serious Condit. And the treatment for it.

This is Precision: Perspectives on Children's Surgery, the podcast of Lurie Children's Hospital. I'm Scott Webb. Doctor, thanks so much for your time today. We have a lot to talk about. We're gonna talk about the Chicago Institute for Fetal Health, twin transfusion syndrome, and so much more so as we get rolling here, tell us about the Chicago Institute for Fetal Health. What makes it so unique?

Dr. Amir Alhajjat: The really unique part of Chicago Fetal Health is how we deliver our care. The pregnant patient comes in for an evaluation. On that day of evaluation, they get all the necessary diagnostic, all the testing that they need, whether it's an MRI, an echo and ultrasound, and it's all state of the art diagnostics. We try to arrive at as accurate diagnosis as we can. And then we have a multidisciplinary team of experts. This team includes experts that have had combined decades of experience in treating the pregnant patient and the fetus and children.

And they meet together first and try to arrive as accurate diagnosis and as accurate information and picture that we can and then provide the family, provide the parents with what we know just based on the results that we have, and to paint the picture as accurate as possible, provide them with guidance and options and pathways. And the really unique part of that is that it's all most of the time done within one day. And so the pregnant patient comes in or the parents come in and then they do all of this.

By the end of the day, they get to meet with all the experts and have as close as picture as possible by the end of that one day. And you can imagine these are people who are taking time off of work who have left their other kids at home or traveled sometimes nationally sometimes regionally, we have patients from Chicago. We have patients from other states here around Illinois. We have national and international patients. And you can imagine on one hand how much time they've taken to get some answers.

And on the other hand, how anxious they are to get questions that they need to make important decisions. And so that's the uniqueness. I think in my mind that CFH provides is that accurate, timely diagnosis and information.

Scott Webb: Yeah, accurate and timely. And it does seem like the multidisciplinary approach has become so common in medicine. And in preparing for this I wa s doing some research and I, I saw the topic and twin to twin transfusion syndrome. I have to admit that's a new one for me. So really gonna rely on your expertise here. What is that?

Dr. Amir Alhajjat: Yeah, twin twin transfusion syndrome is a condition that occurs generally in monochorionic diemiotic twins. Monochorionic diemotic twins are twins who have two separate sacks but share one placenta. And so these two twins have two separate vocal cords and separate shares of their placenta and almost all of these twins, even though they have their own share of the one placenta. They always have vessels that connected to and generally the vessels that connected to, cause a balance between the fluids of these babies.

Whatever fluid goes from one baby to the other, through these vessels that are connecting the two shares of the placenta the other baby gives back. And so there's generally a balance between the fluids of these babies. About 90% of the time. However, about 10% of the time, there's an imbalance. And so one baby is giving more fluid to the other baby than it's getting from the second baby. And so the baby that's giving the more fluid we call a donor and then the baby that is receiving more of the fluid is called the recipient.

And you can imagine how a change in that fluid balance can, cause trouble for that delicate, developing fetus. And so the fetus that has the less fluid in a way becomes dehydrated. And they have less fluid, they're making less urine. And so the fluid around the baby the amniotic fluid around the baby becomes less and less. And as they're become more dehydrated or have less fluid they're bladder becomes smaller because they're just not making enough urine to fill that bladder. And that baby ends up having something called oligo hydroneous, which is a condition where there's less fluid around that baby.

On the other hand, the baby it's getting more of the fluid from its twin starts to have more fluid. And because they're having more fluid, you can see they're making more pee and then their amniotic fluid is increasing. And so that's a condition called poly hydronous. And the first stage where the first indication of twin twin transfusion syndrome is when the recipient baby, the baby that's getting the more fluid has a lot more fluid around them. And we call that poly hydronous. And then the smaller baby or the baby that has less fluid has poly hydronous. And so that's when we start to diagnose twin twin transfusion syndrome.

And as the condition progresses, the baby who has the more fluid That fluid becomes a strain on their heart. And so their heart is working harder. It starts to have signs of working harder to mobilize all that fluid. Whereas a baby that has less fluid you start to not see the bladder in that baby, because of, like I said, the less amount of fluid. And when we start to see the poly hydrogneous on one and the oligo hydrogenous in the other, with the smaller baby, not having a bladder or the bladder's not visible that's to stage two.

Twin Twin transfusion syndrome. So things have progressed a little bit. And then we go to stage three and that has more hemodynamic problems with babies. And so the smaller babies starts to from all the fluid imbalance. We start to see changes in their umbilical cord flow. Which tells us that, that baby hemodynamically from a heart and flow perspective is doing worse. And the larger baby starts to show more of the signs of the heart strain and their heart working, harder and failing. And that's the stage three twin twin transfusion, which has some sub staging as well.

And then if it even progresses more, one or the other baby can have signs of heart failure which in the fetus we call high drops. And so they start to collect a lot of fluid in their cavities, around their heart and lung or in their belly. And that is what we call high drops and that's stage four. And this can even progress to death of one or both babies in what we call a stage five twin to twin transfusion syndrome.

Scott Webb: Yeah. So I'm with you so far. I think doctor on how you determine now, what do you do? How do you treat the pregnant patient, the mom to be and the babies?

Dr. Amir Alhajjat: Yeah, it all depends on the staging. And so when we start with stage one where one baby has more fluid. The other baby has less fluid but we see a bladder, the hearts look okay. And there's no other signs of strain on the babies. A lot of times with these babies, we can observe them. There's a fraction of them that either stays at stage one or regresses. But in our experience, the majority of them will progress and. But most times we watch those babies. Unless there are a couple of indications the amount of fluids, a lot around one of the babies and it's causing cervical changes.

In that scenario, we may offer an intervention, but generally stage one we watch and we have to watch them very closely. And so that means bringing them back for another evaluation sometimes within a couple of days or evaluating, twice a week sometimes just to catch that progression before it happens. And so that's the first method of treating these babies is just watching them. The second is a laser fleuroscopy. What we generally do for twin twin transfusion syndrome. And laser fleuroscopy is a fetal operation where we go into the sack of the larger baby or the baby that has the more fluid.

And then we visualize the placenta and we look at the two shares of the placenta between the two babies and figure out where those vessels that are connecting those two babies, where they are. Once we figure out where those vessels are, we use a laser fiber, really small laser fiber to pretty much coagulate or burn those vessels so that those babies are not allowed to share anymore. This is one of the situations where you don't want your babies to share.

Scott Webb: Okay. So there are times. Gotcha. Yeah.

Dr. Amir Alhajjat: Yeah, there are times where you don't want them to share. And so we burn the vessels that connect it to, and that way they're not allowed to share the fluids. And that in a way correct the condition. We use laser fleuroscopy or we call it SFLP for stage two and above generally twin twin transfusion syndrome and other conditions that affect twins, but the grand majority of our use of laser is for twin twin transfusion.

Scott Webb: I was gonna ask you so. does the procedure take, is it a big team in the OR maybe you can take us through that as well?

Dr. Amir Alhajjat: The real art and the procedures is figuring out when it needs to be done honestly. The procedure itself is a a simple procedure, you have to really balance the pros and cons or the indications and contraindications of an operation, because you know, you have to kinda really balance whether it's a good timing to go to the operating room or do it or not. Once we make that decision the procedure itself is relatively straightforward most of the time. So what we do is we usually take the mother back to the operating room within a day or two.

And then it's done under most of the times, local anesthesia, just like when you go to the dentist to have your molars taken out or your wisdom teeth taken out. It's just a Twilight, a little bit of sedation. In addition to local anesthetics, an injection of numbing medication into a certain area in the pregnant patient's abdominal wall. And then the laser fiber or the scope we use for this procedure is a really thin, probably a millimeter and just above a millimeter thick instrument. That's really long. We make an incision about one to two millimeters and we put this long scope into the recipient sac or the baby that has a lot of fluid.

And then thread that little laser that I told you would coagulate the vessels in between them. The operation itself, a lot of planning before. And so we spent quite a bit of time doing an ultrasound and figuring out the best place to go into and do this operation so thattakes quite a bit. The laser itself, the operation itself, takes somewhere between, 30 minutes to an hour and a half, depending on how hard it is to see the placenta. The team is a humongous team. It's a lot of people because you have two patients, you have the pregnant patient and you have the fetus.

And so you need people to take care of both of them. You need the fetal surgeon you need maternal fetal medicine doctor. You need the scrub nurses and the circulating nurses and a nurse for the mom and a nurse from our center to help us do the operation and multiple anesthesiologist. So sometimes it's a little alarming to the mothe r when she's placed in the operating room. But we try to remind them that it's just a big team because we have two patients. Most times the mother is under local anesthesia and the mother can be in and out of seeing the operation.

They can actually watch on the screen as we're looking at the placenta. The mom afterwards gets transferred to be observed on the floor usually, and that's just an overnight stay. She gets to eat and drink. We watch the mom and see how things are going. And then the next day we get an ultrasound to just to check on the fetuses see some heart rates, look at the fluids and then they go home the next.

Scott Webb: Yeah, well, it's really cool. Wondering about the outcomes, how does this procedure correct the condition? Are the outcomes really good for everybody involved? Maybe take us through that a bit.

Dr. Amir Alhajjat: It's important to understand where we're starting from. Because twin twin transfusion syndrome, untreated has an almost universal mortality. And so if it goes untreated, then most babies will pass. They won't be able to survive the hemo dynamic. The physiologic changes. When we do laser oscopy or S FLPs 95% of the time, at least one of the babies. Most times the recipient survives, 95% of the time. And over 80% of the time, both of them survive. You were talking about almost a universal mortality, universal death for the babies to over 80 to 90% survival of these babies. And that's, survival to going home from the nursery. Not just survival from the procedure itself, it's, long term.

Scott Webb: that's really amazing. As you say is uh, Certain mortality without the procedure. Wondering if your procedure the laser that you use in this procedure, you've explained to us here, is that kind of the gold standard? Is this kind of how everybody does it or is the way you all do it there is that unique in some ways? And if so, maybe you can explain that to us.

Dr. Amir Alhajjat: In a way it's unique in a way it's not unique. It's unique because not a lot of centers are than country are capable of providing this procedure. And so, it's just a handful of centers that can provide it. And that's why it's unique, but it's not unique because this is honestly the standard of care for twin twin transfusion syndrom.

Scott Webb: Wanted to dive in while I have you on the line here and talk a little bit just about you talk about your specialty. How'd you get where you are, the training that you've done and so on?

Dr. Amir Alhajjat: Yeah. So I am a a pediatric general surgeon and a fetal surgeon. Through my training, I did my medical school at at Jordan where I'm from it's the country of Jordan. And then you do general surgery training to start with and I did that at the University of Iowa. There's quite a bit of research that goes with it as well. And I did quite a bit of research in fetal therapy. You also have to do a pediatric surgery fellowship, which is, additional training to become a pediatric surgeon after you have already done training to do general surgery.

And that's because. pediatric patients are unique. They're not just small adults. We all know that and so you have to do additional training to be able to do pediatric surgery and then additional training to do fetal surgery. And I did my pediatric surgery training at Phoenix Children's in Mayo Clinic, Arizona, and then my fetal surgery training at Cincinnati Children's in Cincinnati. And that combined training will allow you to be able to operate on babies and fetuses.

Scott Webb: I was just thinking you went from the University of Jordan to Iowa. That's a big culture Isn't it?

Dr. Amir Alhajjat: Especially in the weather. A lot of changes in the weather right there.

Scott Webb: Wondering, you as an expert where do you turn? Do you turn to other members of the multidisciplinary team that you work with? When you have questions, where do you turn to get the answers?

Dr. Amir Alhajjat: We have tons of expertise just here at Lurie Children's that is, really close and everybody's available almost all the time to help if you need a question or have some concern or you need help with managing a patient or fetus or a baby or a child. THere's experts in almost every field from a fetal perspective. I work closely with Dr. Aman Shaman, the director for the Chicago Fetal Health, who is, renowned in fetal surgery he's been doing this for a couple of decades, at least now.

And so I turn to him for almost all the cases, we discuss almost every case and frequently patients who will see us counsel together and operate together and stuff like that. I definitely turn to him quite a bit. If it's in the field, if it's in another field, then I see a lot of experts here that are always happy to help.

Scott Webb: We talked about the mortality rate, if nothing is done for these fetuses what were things like? What was the mortality rate like before this sort of standard of care, this procedure was adopted as the gold standard?

Dr. Amir Alhajjat: Yeah, it was almost universal mortality. We did mention there is one way of also treating twin twin transfusion syndrome. That's through taking off fluid off of the baby that has the, the extra fluid and that is a treat modality sometimes when the laser or laser is not an option in certain circumstances. But as you can imagine, that' s just putting a bandaid really on the pathophysiology, because instead of actually targeting the connections that caused the disease. You're just taking fluid off to relieve some of that strain on the recipient. And you can you imagine that doesn't treat it all the time or much as laser does.

And so that was done in the past and then people started to do lasers and lasers have advanced quite a bit since they were first discovered 20 or 30 years ago where the equipment now is much more. Mill invasive and smaller, the incisions are smaller. The lasers are much better. The visualization, the cameras and the resolution of the cameras and the screens that we have now allow us to see even the smallest vessels that connect these babies. And so it's progressed quite a bit.

And hopefully in the future we only even have to do surgeries even though I am a surgeon and I love doing surgeries am always striving to find ways to not operate on babies or children whenever we can. And just. Fix things with medicines part of the research that's going on right now in a lot of different places, one of them Chicago's for fetal health is figuring out the markers for twin twin transfusion and what we can learn from amniotic fluid and the mom's plasma in terms of diagnosing the severity of the disease.

And hopefully in the future, we'll be able to even find a medication that reverses it without having to do an operation. So obviously within research and hopefully something that we can do next 10 or 20 years.

Scott Webb: Yeah well, so much education and great information today. And I just love speaking with the experts and hearing your excitement and your compassion, especially about the future. As we wrap up here, doctor, what gives you hope for pregnant patients with fetal complications?

Dr. Amir Alhajjat: It's very important to see the kind of trajectory in fetal medicine and fetal therap. 10, 20, 30 years ago, we couldn't do anything for these fetuses. And throughout the last 20 or 30 years, things have progressed tremendously. Five or 10 years ago, ultrasounds and MRIs and echoes were available, but weren't as good as they are right now. We have much more therapies now, than we did five or 10 years ago or 20 years ago.

And so what gives me hope is knowing the trajectory that we've been on and knowing that in five or 10 years, what we are doing now will be obsolete and we will have much better therapies and much better understanding and much better. Insight into the fetus and how to treat the fetus than we do right now. So I think we're on a great trajectory for growth and hopefully better for the patients.

Scott Webb: Yeah well, it gives you so much hope and optimism, about the future and hopefully we'll have you back on. We'll have you back on in five years or maybe three years, as you say all happening so fast. And it seems to be happening in real time. So we'll have you back on and we'll look back at lasers and laugh about, oh, remember when we were still using lasers, right.

Dr. Amir Alhajjat: It's remarkable.

Scott Webb: That's the perfect word. We're gonna end with that. Remarkable. Thanks so much. You stay well.

Dr. Amir Alhajjat: Thank you very much. Thank you.

Scott Webb: And visit Luriechildrens.org/fetal for more information or to make an appointment. And if you found this podcast helpful, please share on your social channels and check out the full podcast library for additional topics of interest. This is Precision: Perspectives on Children's Surgery, the podcast from Lurie Children's Hospital. I'm Scott Webb. Stay well.