Fetal Interventions Part II: Lung Lesions

Somewhere else. Jack, you, you had mentioned that you don't, you guys haven't done fetal lobectomies, right? So, Oh, we're not hearing you. Not hearing me now I can hear you. Yes, uh, what fetal intervention have you done for thoracic? Uh, any, have you done, are you doing, uh, any thoracic intervention, not just for lung lesions, but are you doing tracheal plugs or, or anything like that, or exit, or what are you doing there? Uh, we do excess mostly for, um, uh, for neck masses, um, and we, uh, done quite a few shunts for macrocystic pecans, uh, with pretty good success and, you know, similar kind of numbers, uh. Test numbers, not certainly gross numbers as the, uh, doctor has done. Um, we've also had a couple of kids, um, like I mentioned before that have, uh, uh, bronchopulmonary sequestrations or, uh, hybrid lesions with a large feeding vessel where we've tried to occlude the vessel, uh, using a number of different techniques, uh, in the thinking that. Doing that might shrink the lesions, and we did one where we did a shunt and we occluded the vessel. So, uh, had mixed success with with those cases. We've only done a handful of them. How have you tried, have you tried to occlude the vessel, Jack? What, what technology have you used to occlude the vessel? Tried radio frequency ablation, which was a disaster, so I wouldn't recommend that predictably. Uh, the last one we used coils, uh, which were actually good initially, but then, uh, the fetus died about a week later for reasons that weren't clear to us. Um, and then we've also, uh, I guess we've used injection of, uh, alcohol as well earlier on. And my, my worry with alcohol injection, uh, which has actually been documented in a case that, uh, I was made aware of, is that it can go, and this was for a teratoma, um, but it can go through the shunt, right? So, it's not necessarily going to stop where you inject it. It can go through the vasculature and actually cause thrombosis in the systemic circular in the, uh, in this case, it, it, it was first noticed in the chambers of the heart. So thrombi circulating in the heart, and then they go out to various sites and so that's one risk. And the other, I think, is just, uh, you know, if, if it does go systemic and nobody's even studied this experimentally, which is amazing to me, you know, that you go and you inject alcohol in a fetus. And you haven't even studied in a sheep, you know, what happens when you inject alcohol in circulation, um. You know, what effect does that have on neural development and other other organ development in the fetus? It's just very worrisome to me. I, I don't think it's a good thing to do. I haven't done and the radio frequency ablation thing is very attractive, but because of the fluid content in the fetus, you simply can't control the heat. You know, the fetus is 90% water and. Uh, I put a radiofrequency ablation probe in one side of a teratoma after we resected it from a fetus and turned it on and watched, and suddenly the other side of the teratoma began to boil, and that's where the energy dispersed. And so it can go in any direction and cause collateral damage. And we have studied that in labs, and it can, it, it causes a lot of collateral damage in the thorax and other places, so. We, and, as I said, that was a disaster and we would never do that again. Yeah. I think just, it's important to keep things in perspective. I mean, probably 95% of the C cams I see now are prenatally diagnosed. And in, In the, you know, we're, we're talking about the end of the nth, um, the experience of CHOP or that you might have up in Toronto, but almost none of these lesions require prenatal prenatal intervention and in fact very few require. Intervention the day the child is born. And so it's just, I mean, so just for the audience out there to understand that the vast majority of these lesions do not fall in the realm of these, uh, what, what are very exciting and extreme treatments, but there probably are only one or two centers in the world that should even be thinking about these kind of interventions because the, the end is so small that, that, um, you don't, you don't want to think just because someone has a cyst on their. On a, on a prenatal, uh, sono or MRI that you need to put in a shunt or if there's a feeding vessel that you need to think about doing something about it. Cause the majority of these children can be delivered and, and don't even have fetal distress at that point, so. Uh, and that's been our experience here. We, uh, have had a few that we've referred to Cincinnati to Tim Crummer Holy, uh, because of, uh, the progression or the development of eye drops. Um, I've had patients where the eye drops went away and the patient didn't even, I couldn't even find the CTM after birth despite a CT. You know, a year later. Uh, so, that child was seen for fetal surgery. Fortunately, it regressed and nothing was done. Uh, so, we, we've had that experience as well. Yeah, and I, I completely agree that the majority of these lesions don't need fetal intervention. They do need fetal evaluation. Right. And, uh, I can't tell you how many lesions have been, uh, have come to us after a, uh, recommendation for termination. By people who don't really understand the natural history of these lesions, even when they're very large, they can regress and be asymptomatic at birth, or they can have very good survival rates with the kinds of interventions that we do. So I think it's important to understand the prenatal natural history, that people do understand that, you know, the garden variety postnatal cecam is very different than some prenatal secams that we see. And that there, there are places that you can refer these patients where there are options other than termination or fetal loss. What you describe as a disappearing secam, I think, is an incredibly unusual, if ever present lesion. And, uh, there are some things that look like scams in utero, like, uh, uh, segmental bronchial stenosis, things like that. They give you an echogenic microcystic appearance of the lung. But true cystic scams, uh, you know, we get, we see them all prenatally. We get CT scans on them all postnatally. They often regress. They often become very isoechogenic at the third trimester where you can't see them anymore. You can get a chest x-ray at birth and you don't see anything. But I can't think of a cystic ceca in utero that I got a CT scan, CT scan on, uh, a month after birth that wasn't still present and still prominent. So, I, I don't think true cystic secams disappear. They regress, but I don't think they disappear. And there are some things that can look like CA that we've got a better understanding of more recently that are usually in the bronchial stenosis atresia sequence that I think can be very minimally apparent or non-apparent after birth. We have about 4 minutes left and I want to, we have a slew of questions, so I want to see if we can do sort of rapid fire answering here. And then also Jean Martin. If you have any comments, uh, about, because I know you're doing a lot of this up at your center I wanna hear your, your input or comments or questions about what we've been discussing, just, uh, speak up. Yes, I wanted to speak up and actually talking about speaking up, if Jack can put up his level of the sound that we would hear him better. One question I agree with what Alan was just saying, but in that respect, I don't think we should call the CCA. We should say. Genital lung lesions and they're either macrocystic or they're hypeechoic or mixed, and this would avoid the confusion saying secams disappear because I agree with you, Alan, cams don't disappear if they are really cams, but CCA is a pathological diagnosis once a specimen is in the bucket. So that's one point I wanted to make. The other one was about the exit procedure. I think there's many tertiary centers who have the capacity to do an exit. I think it requires a huge team approach and somebody leading the team, but many people could do it. What's more important, I think, is the indication for exit because people start doing exits and then they want to do exit for all sorts of things. I'm aware of a case that. It looked like a huge hyperechoic lung lesion, mediastinal shift, very severe. They couldn't get an MRI done. The mom was too obese to fit in the machine, so they decided to do an exit, and I'm not sure why, because they just did an exit to intubate the baby. The baby did fine, ventilated well, actually extubated himself two days later, was fine, and it turned out to be that this baby had a congenitally absent. Lungs. So what looked like a CA with a medicinal shift was actually the only lung that there was. So thank God that they didn't do an exit to lobectomy procedure, then there would be no lung left. So I think you really have to be careful with the indications. Well, I think, I think what you're pointing out, Jean Martin, is that you really need good prenatal diagnosis. OK? Yes, and prenatal diagnosis is not equivalent in all centers or by all practitioners. And we very frequently, as I mentioned in my talk, get lung agenesis that have been diagnosed as microcystic secas elsewhere. So it really is an art, and you need people that are very good. If you're going to practice this kind of medicine and not refer to a fetal center, you need to have great confidence in your prenatal diagnosis or you make terrible mistakes just like you mentioned. Absolutely, and remember to first do no harm. So if you're not sure, better not to put the mom through an exit because it is more invasive than a regular C-section. Hey, uh, Jean Martin, quick little plug, can you tell us about the conference you're having up there. Pretty soon, right? Esophageal atresia conference, yeah, uh, October 8th and 9 in Montreal. OK, so Jack is coming and Steve and I hope that many of you can come as well. Many good invited speakers. So it's going to be focusing on esophageal atresia, uh, both the surgical tricks, but also the long term follow up. Perfect. Thank you. Yeah. Um, yeah, actually, yeah, Jean Martin, if you can get us, uh, type a little text at the thing about a link on how people can learn more about it, uh, if there's a, a way they can learn more, that'd be great. Um, I'll do that. We have a, uh, Phil Hammond, your question we're gonna talk about in the next session which we're getting to momentarily about which CCMs you res you resect, um, or actually, I'm sorry, lung lesions you resect, um. Michele Torre uh says, can you clarify to me the role of the pleural amniotic shunt? Yes, uh, uh, we, we use it very, uh, specifically for macrocystic se cams that have, uh, evidence of hydrops. And so by high drops we mean fluid in, uh, our skin edema. Um, it has to be more than just ascites. So, pure ascites is not necessarily hydrops. It can be simply related to the mediastinal shift and, uh, hepatic pulmonary or hepatic venous return. Uh, so, we require a pleural effusion, a pericardial effusion, and skin or scalp edema. And that's when we use, uh, Uh, pleuro amniotic shunts. We avoid putting them in prophylactically, um. Unless we will tap large macrocystic lesions just prior to delivery sometimes to improve ventilation, but we don't generally put in a shunt unless the lesion is causing evidence of high drops. Uh, OK. And then Kurt Koon says, uh, a question. Basically, when should we refer to a fetal center? When you're not comfortable managing the patient, OK? So, I, I think, and, and, you know, obviously, it, it depends on the, the wishes of the patient, but they should certainly be offered the option of referral to a fetal center if, uh, they've got a, a very large lesion, if they're requiring very frequent, uh, monitoring, if you don't have adequate, uh, uh, Uh, radiologic or maternal fetal medical support to do good prenatal diagnosis and to follow these lesions closely. Um, and if you don't have the ability to intervene if it, uh, develops eye drops. So, I think, you know, those are, are the reasons I would refer a patient. If you can't fix it and you're worried about it, then, uh, that would be the time to send it to a fetal center. All right. Uh, I think that's actually a pretty common question and I'm glad someone asked it. Uh, what CVR would be considered safe antenatally before intervening? Would be considered safe antenatally before intervening. Well, I mentioned that the CVR is just a, a estimator of size. And so, the physiology is really what you're looking at. And the CVR is valuable for prediction of, uh, adverse physiology. So, uh, we use a cutoff of 1.6. As I mentioned, it's very, very predictive with microcystic lesions. So, if you have a less than 1.6 on presentation of a CCAM, Um, Then the likelihood it'll evolve hydrops is about 3%, 3 or less than 5%, and that's a pretty good predictive capacity, and that's held true, uh, both in the prospective study that uh Tim Crummelholm published when he was a CHP, uh, on through our ongoing experience. So it's very unusual to have. A CVR of less than 1.6 and a microcystic lesion, uh, evolve into high drops. The corollary is also true. Greater than 1.6, uh, they need to be watched very closely, and there's a much higher likelihood that they will evolve into high drops. And macrocystic lesions are a little bit of a wild card because the cystic component can, uh, grow very rapidly. And so, uh, they can be, uh, worrisome even if they're, uh, less than 1.6. Uh, when would you plan a fetal MRI? We get fetal MRI's routinely for, uh, but, you know, that's what we do. So, um, so all of our, uh, lung lesions get an MRI prenatally. And, you know, I think MRIs are often helpful in defining the anatomy, um, and, uh, You know, just the, the general size of lesions for me because I can read them easier than ultrasound. Um, but I don't think, uh, they're essential, you know, for all centers to use. So, I think MRIs, uh, can clarify, uh, abnormalities if there's ambiguity on the ultrasound, if there's confusion about the diagnosis. Um, you know, those are the reasons that if you don't routinely get MRIs, then I would get one. Yeah, I would just, uh, I mean, in a center that's not quite as geared towards the fetal aspect of it, we, you know, unusual cases, we'll get fetal MRIs, but most of the time we just follow these patients with the fetal ultrasound and then evaluate the patient once they're, they're born. Um, honestly, part of it's just a, I would love to have fetal MRIs on it. It's just a practical and cost issue that it's, it doesn't change the management in the prenatal portion. Um, very often, um, except for in these extreme cases, so. Well, you know, MRI is better for some things and not as good for other things. And so, it depends on the fetal anomaly you're talking about. If you're a fetal referral center and people send you patients, uh, that are, uh, difficult for one reason or another, I think you want to get all the information that you can. And an MRI often, uh, you know, contributes in one way or another to our understanding of the lesion, so. Uh, is there a registry like we have for ECMO for fetal surgery and, uh, or for, uh, the exit procedure? No. Get on it. Yeah, so, uh.