Welcome back to the final podcast coverage of IFMSS 2019 with your host, Doctor Beth Remey, fetal surgeon at Cincinnati Children's Hospital, and myself, Ray Hankey. Today we cover a variety of clinical research findings discussed in Sales, Switzerland. We start off with a conversation with Doctor Steven Fenton, director of the Fetal Center and Pediatric surgeon at Primary Children's Hospital in Salt Lake City, Utah, as he discusses the impacts of a CDH protocol change. We've been treating CDH for a long time in Salt Lake City and uh our ECMO program was established in 2003, uh, and in 2007, we had our first protocol for CDH care implemented. Uh, in 2016, that underwent a change, uh, mainly, uh, brought on by our neonatologist. And the biggest change in the protocol was what I like to say to parents, actually, when I'm doing prenatal counseling, and that is, for the 1st 72 hours, we're going to put the baby in a corner. So, what I mean by putting baby in a corner is the child, uh, our delivery ORs are, is right next to uh what we would term our birthing NICU. So there is a NICU unit, it's managed by the same neonatologists and pediatric surgeons that definitively care for the child. But we put them in a, in a, a, a place of the NICU that's quiet. We immediately intubate them and put them on a high frequency oscillator, and mainly we decrease the overall mean airway pressure that that we do it on. We also try and Minimize stimulation, so we put them on a bed that can actually be used for transport as well. The the child doesn't have to be moved from one bed to another if they're being transported. We give them sedation. We don't do an echo within the 1st 24 hours, especially if they already have a prenatal echo. With our oxygen management, we actually wean oxygen according to the preductal SATs, and we do not do any post ductal SAT monitoring. We do do some ABGs from the umbilical line, which would be post ductal if, if needed, but we don't really uh look at it routinely um from a postductal SAT. And we actually start our oxygen resuscitation at uh FIO2 of 0.4 instead of 1. and then finally, we really uh have tried to, I would say, only use inhaled nitric oxide. Uh, and or other ionodilators or oppressor agents for what I would, uh, term clinically significant hypoperfusion. So what mean airway pressure are you titrating to, or do you have a max that you're working with? No, but when we look back at it, I mean, there have been some studies that have come out that have compared an oscillator and conventional vent settings, and what we found is that our mean airway pressures were higher. So, the median of our mean airway pressures was about 13, and since implementing this change, it's actually now 11. So it has been reduced. And the reason we do that is we feel like that that actually increased pressure is compromising gas exchange in the child as well as the vascularization within the lungs, which could lead to increasing pulmonary hypertension or exacerbation of it. And are you applying this protocol to all prenatally diagnosed CDH, or do you stratify this based on severity? We're, we're applying it to all. So, uh, I mean, interestingly, and honestly, I, I brought this up with our neonatologists because That we, they had made the change. They're the ones that do the majority of the, of the care for the kids in the NICU, obviously, the non-surgical care. But what I noticed is that our ECMO rate had really decreased. So, I arrived at Primary Children's in about 2014, and, uh, you know, we were doing ECMO quite regularly for our CDH kids, and I noticed that we weren't doing it that much. And the first thing we found is that we dropped our ECMO utilization rate across the board from about 37% down to 13%. And that, and that held true when we look at, let's say the prenatal predictor of liver position. So whether the liver was up in the chest or down in the abdomen, it was still a significant decrease in our utilization of ec. We also found that our overall survival rate increased, and it increased from 74% up to 89% overall, and that was a significant change. It was also significantly improved for our liver down kids. The liver up kids, uh, again, the survival was increased, but it was not found to be statistically significant. And then, uh, of course, uh, with decreased utilization and increased overall survival, we found that we had an increased survival without the use of ECMO. And I think ECMO is a great adjuvant in taking care of these very sick kids, but it is not without consequence. And so, if we can find ways to continue to increase survivability without having to worry about the consequences of, of an ECMO run, it's going to be, you know, good overall for these kids. And the small number of patients that did go on ECMO, were those patients always from the most severe prenatal prediction group or not necessarily? I mean, not necessarily. So, uh, we did have, we, you know, since 2016 with, with the creation of the Utah Fetal Center, we're directing and coordinating the prenatal care of these kids. Uh, I would say better than we did before. And so, we are getting like MRIs now, uh, uh, on almost all kids. I think 97% versus 24% before the fetal center and So, we have a small denominator. I mean, since 2016, uh, isolated left CDH there were 53 kids in the study after exclusion, and, and so the denominator is relatively small. But when we looked at MRI, uh, total fetal lung volume, observed to expected less than 15%. And then 15 to 25%, 25 to 35%, greater than 35%, we did see some uh variability in overall ECMO use, and the less than 15% kids, you would expect them to have a 100% um ECMO utilization. But we actually only had a 38% ECMO utilization, and some of that is probably due to other, the kids having other anomalies, not necessarily fitting what we would have for criteria of, of being placed on ECMO, meaning PAO2 never rose above 50 and we could never get their CO2 levels below 100. And so, we do have criteria when the child is born. We want to give every child a chance, but we, we realize that there are some kids that the gas exchange will probably never happen, and so, to place that child on ECMO and have them be on ECMO for several weeks and then have to start the process of how are we going to get them off ECO. and withdrawal, you know, withdrawal of care. Some of those kids, we, we talk to parents extensively and kind of fit some, uh, put in some criteria as far as when we would use ECMO or when we won't. And if they don't fall within those criteria, then we have a wonderful palliative team that that works with the parents. And so some of those kids. Even though you would expect 100%, ECMO weren't placed on that. And, you know, in the long run, that, that might have affected overall survival for that group because the MRI less than 15% group did have a 50% survival. But when you look at survival, we're looking at survival, uh, you know, you can say you're looking at 30 day survival, or you're looking at survival at discharge, and those children may be discharged, you know, 9 months later, but, but won't necessarily survive the year. And, you know, again, I'm not trying to say that we don't want to give that, those children hope, but I think what this has helped us do is one, reduce our overall ECMO use, but then to kind of help stratify a little bit better as far as when, you know, ECMO will really be useful in their care. One final question. Previous reports of, you know, similar protocols, uh, after birth have been very dependent. On individual providers, and that's been heavily criticized. Was your protocol only driven by select providers, or was it generally used throughout the entire neonatology and surgical faculty? Yeah, I mean, that, that's the, I guess it's the nice thing partly is that the NICUs are closed units and our kids are born at the university, which is connected basically to the children's hospital. The neonatal providers there and at the children's hospitals are the same providers, and They implemented this as a group. So it's not a single provider who's answering all the phone calls and directing it is the group, and I mean, you know, I, I don't even know how many neonatologists we have now. We've had, um, you know, hired a lot of, um, new people from elsewhere and, and, um, new, uh, new graduates, but they all adhere to the protocol. I think some of the hardest, uh, aspects of implementing the protocol was actually changing the culture. I mean, Well, we always got echoes on these kids within the 1st 24 hours, and we always transport them immediately to, you know, the primary children's NICU, and we, we always look at the post ductal sat monitoring, and that's how we adjust our oxygenation. Well, I mean, it's hard to break those habits and to change and to make a change, but Then when you do it, and people realize, you know, that the outcomes are better, um, they really kind of grasp onto it. And so, now, when I go see, I know, when I look up on the monitor, there's only one, you know, SAT recording up there. There's no longer two SAT recordings. And our amazing, um, you know, NICU nurses who are obviously very involved and, and very dedicated to the care of these kids, I mean, they have, they have grasped it, and I think that's what's helped to move it forward. Well, thank you for sharing your data. You're welcome. Decreasing ECMO rates and improved survival sounds pretty promising. With a bit more sobering findings, Doctor Uchenna Kennedy tells us about outcomes in patients with giant omphalocele. I am a pediatric surgeon at the Children's Hospital in Zurich, um, but I did my fellowship in general pediatric surgery at CHPP in Philadelphia. This is also where I conducted the research. It is a retrospective study on outcomes in patients born prematurely with tantumphiles. We looked at patients that were born before 34 weeks gestational age versus those born after 34 weeks and compared overall outcomes. Our most significant findings include that there were significant differences in respiratory outcomes. Such as the need for neonatal intubation that was significantly higher in the preterm group and also the requirement for a tracheostomy and ventilator dependence at discharge, which was not statistically significant but was at a high rate of 31%. In the preterm group, there was a very high rate of neonatal death of 26% versus 6% in the term group, which is definitely significant and compares to overall death in children born at much earlier gestational ages in the overall preterm population. How did you guys choose 34 weeks as your cutoff in terms of Pre-term versus term, since technically 35 weeks is also preterm. We thought that 34 weeks is a good cut off considering fetal lung maturity that is significantly better or after 34 weeks, so we thought that probably the respiratory outcomes would depend on that cut off. Back up to the chest, but for a different reason. Doctor Oliulinka Olutoye, surgeon in chief at Nationwide Children's Hospital, discusses prenatal differentiation of pure esophageal atresia versus an EATEF combination. So, for families that are presented with a fetus with the diagnosis of oesophageal atresia prenatally, the next challenge in counseling is how can we assure them or reassure them of what the, the projected outcome would be. And so being able to distinguish between pure resia and dysto fistula was something we thought we should look at to see how effective or accurate we are in our prediction. The prenatal diagnosis of osophageal atresia has improved over time. With three main items that would suggest the presence of oesophageal atresia. One is polyhydramus, uh, the other is the, uh, a small stomach, and the third is, uh, the presence of a distended proximal osophageal pouch. So, all this will suggest an esophageal atresia but doesn't necessarily tell you whether it's a pure atrichia or has a distal fistula. And so our hypothesis was that size of the stomach helped distinguish those that have a pure atrisia from those that have a distal fistula. Since on postnatal radiographs, when we take a uh an abdominal or a babygram. You see, air in the stomach would confirm that there is a distal fistula and that can we use fluid in the stomach prenatally to ascertain those that have a distal fistula or not. So, we reviewed a series of patients and, and classified them by the size of the stomach, those that had an absent stomach where you couldn't even see any fluid in the stomach, those that had a small amount of fluid in the stomach and those that had a normal sized stomach. And the summary of it really is, even though we had a significant portion with polyhydramnos and those that had the proximal esophageal pouch, for those that had prenatal diagnosis, the smallness of the stomach did not correlate with whether there was a distal fistula or not, because while almost all those that A pure resia had a small stomach. There was also a significant portion, about 43% of those that had a distal fistula that also had a small stomach. So, seeing a small stomach in and of itself does not confirm or does not discriminate between a pure atrisia and, and those with check oesophageal fistula. So we need to still be Very broad in our counseling because we don't have enough discriminatory information in the prenatal diagnosis. And were those findings all ultrasound based? So in our series, we, there were a combination of ultrasound and MRI. So even with MRI we still were not able to distinguish those. So my takeaway here is that how the stomach looks on prenatal imaging still doesn't tell you if a TE fistula is present. So we wrap up our conversation today with a discussion with Nina Bentz, pediatric surgical research fellow at Milwaukee Medical College of Wisconsin. She tells us about their latest research on mothers prenatally diagnosed with fetal anomalies. Prior data has shown that there's a higher rates of uh anxiety and depression in patients that have that diagnosis prenatally. And we don't do any of that screening and there's actually no guidelines for screening in that population at all. So it was a survey-based study where we enrolled patients that were seen in our fetal concern center for their first visit. If patients agreed to be part of the study, then they completed a series of 3 surveys that assessed 5 validated measures of things like depression, anxiety, PTSD, and resilience, which is basically like your ability to. Cope in a stressful situation. The first survey was done before they met with the pediatric surgeon, and then following their consultation with the surgeon, they would complete the second survey to assess if there was any change based on their visit with the surgeon. And then following delivery they would complete the 3rd survey. What we found was that over 1/3 of patients in our study screened positive for depression, anxiety, and PTSD, and that only 25% to 33% of the Those patients had prior histories of psychiatric diagnoses. We also found that there was overall lower scores for resilience in our population, though with these results, it really shows that we need to be better about screening these patients and then finding ways to support them and offer resources. We've now got grant funding to enroll many more patients and then to not only look for the rates of mental health disorders, but for risk factors for who should be screened, and then also looking at what's called allostatic load, which is like the biophysical cumulative measure of how much stress they've had over a lifetime to see if that correlates also with distress during pregnancy in these patients that are diagnosed with fetal anomalies. Did it matter if it was a like lethal anomaly versus a fixable anomaly? Were you able to do any sort of subset? Sure, yeah, that's a great question. We did look at um the specific anomalies, diagnoses that both the surgeon would evaluate based on whatever the diagnosis was, and then the mother would evaluate as well on the same scales we could compare them. The majority of our diagnoses were screened in kind of the mid severity range and so part of this going forward we will also be looking to see whether maternal severity ratings correlate with physician severity ratings and whether the rates of mental health disorders correlate with that as well, right? And so you had the mother surveyed at 3 different time points. So, was there Progression or change or did you look at that? Also a very good question. So the pre uh consult to post-consult surveys, so basically just looking at the difference based on their consultation with the surgeon, there was no difference. A lot of our patients are still kind of in the pre-delivery phase and so we haven't completed most of the post delivery surveys yet, so that data is not available, but I think, yeah, that will be. Definitely interesting to see as we get more of those available. Yeah, it's a great idea to look at. I know, you know, in our fetal center we have a social worker sort of screen all the patients for, you know, risk factors for some of these things. A lot of it's, you know, social stability and how likely are they to be able to cope with some of the things they might be suggesting, particularly if it's a surgical intervention, but I think it would be interesting to see over the long term. Does it matter what the diagnosis is, so you can really kind of fine tune because I would think personally it would be different if you're telling the mother that the baby has a, you know, lethal anomaly that is going to result in them dying shortly after birth versus a gastroschisis where you anticipate that there's going to be surgeries, there's going to be challenges, but that 90% of the time they're taking the baby home. Exactly. That's why we're looking. Looking at both physician and maternal ratings because there's some data actually in the trauma world that shows that there's not really a correlation between disease severity or injury severity and patients development of PTSD and things like that. So, I think that is going to be really important to look at too, whether that bears on our data. There's more work to be done here, but as you can tell, exciting progress is being made in many areas of fetal medicine. Have any questions or thoughts about the topics discussed here in the podcast? Please share them on the Stay Current app, Twitter or Facebook. If you haven't had a chance to yet, check out the first two IFMSS 2019 podcast on clinical translation and clinical trials and technical and educational innovations. This chapter is created and edited by Todd Ponsky, Alex Cassar, Alex Gibbons, and myself, Ray Hankey. Remember, knowledge should be free.
Comments