Watch “Fetal Tumors: A Multidisciplinary Approach” to learn:
Risk factors for disease severity and death associated with fetal tumors
Which imaging studies provide the information to determine diagnosis, prognosis and therapeutic options
Factors that may require early aggressive interventions in fetal tumors
The various clinical presentations of fetal tumors
Indications for additional testing or hospitalization of maternal patients with a diagnosis of fetal tumor.
Refer to Fetal Treatment Center
Thanks for joining us today on our webinar and field tumors. My name is Maria Bremer and physician liaison with Benioff Children's hospitals. I'm gonna do some housekeeping items. You are muted so please if you have questions during the talk, go ahead and type them into the Q. And A. And the speakers will get to them at the end of the lecture. I'll do some quick introductions for our four presenters today. Dr Han Min lee. He is the director of the UCSF fetal treatment center and surgeon in chief of UCSF Benioff Children's Hospitals. He treats a wide variety of surgical conditions and fetuses, infants and Children. He's an internationally recognized leader, minimally invasive fetal and neonatal surgery as well as the treatment of life threatening birth defects. Also joining us today is Dr Anita Moon Grady who's the director of the UCSF fetal cardiovascular program with training in both pediatric cardiology and neonatal perinatal medicine to specialize in pediatric and fetal echocardiography and intra operative assessment during congenital cardiac surgery and fetal surgery chores closely with the UCSF Field treatment center and provide patient care focus primarily on echocardiography and offers field consultation services. We also have Mark Sergey, a radiologist specializing in abdominal imaging and ultrasound. He has clinical interest in obstetrics and gynecologic imaging as well as ultrasound guided procedures. Dr G's research interests include field development, maternal health during pregnancy and placenta across a spectrum disorder. Finally and dr Annalisa post will be kicking us off here in one moment is apparent. Atallah Gist with UCSF. Her research focuses on improving outcomes for patients with pregnancy complications including prick, Lancia chronic hypertension mono on. I'm new in the butcher, this mono amniotic twins and diabetic pregnancy. Welcome everyone. Go ahead dr post. Why don't you take the floor? Welcome. Thank you. Thank you Maria. So, yes, I'm Annalisa post one of the M. F. M. S. Or perry Nate ologists at UCSF and I work in the fetal treatment center a lot and I'm delighted to be doing this talk here with my colleagues. So we're going to do a kind of high level M. F. M. Overview and then turn it over to the pediatric and radiology specialists for some more detail. So fetal tumors are overall a rare diagnosis, fortunately it's really challenging to give an incidence because there are so many types and they are so rare. But overall the incidents and the reporting does seem to be increasing likely, just due to detection. You know, we're using ultrasound more ultrasound is better and so we're able to pick up things that in the past would not have been discovered until after birth. Overall, the diagnosis and the management is still very challenging. Um, it's hard to get a precise prenatal diagnosis during pregnancy. Um, some tumors have a very path, a demonic appearance where you know exactly what it is, but many times we have a differential that remains in place until delivery and until we're able to get histological diagnosis and of course these may be an incidental diagnosis in the third trimester, for example. And then we're scrambling to try and figure out what's going on and make a delivery plan after we detect these tumors. How do we manage them? So here is where we get immense benefit from having a multidisciplinary center, such as a fetal treatment center at UCSF for many other places where we can bring together all the specialties from the obstetrics side and the pediatric side to counsel these parents, we want them to understand what we think it is. We want them to understand the short and the long term prognosis. And after that, we always have a discussion of their full scope reproductive options, especially for earlier diagnoses and diagnoses that may have really severe outcomes for continuing pregnancies or even non continuing pregnancies. We of course offer genetic counseling, genetic assessment. Some tumors do have an associated genetic syndrome, but most of them are not associated with the genetic ideology. Prenatal treatment of these tumors. Dr lee is going to touch on more but it's rare. There's rare tumors that are actually amenable to some sort of treatment process during pregnancy. Much more important is focusing on the surveillance plan. How are we going to surveil this pregnancy and then the delivery planning and I'm going to go into a bit more detail in a moment but going back to prognosis. So, prognosis is of course extremely varied because of the varied nature of the tumors that we diagnose, but unfortunately often poor. one factor could be that there may be a malignancy. This may actually be a malignant tumor. But many tumors are benign but still associated with potentially poor outcomes. And one of the reasons for this is often mass effect. So these tumors can be very large or in very sensitive areas. A tumor that's in the face, in the neck, in the throat, in the chest can clearly obstruct airway and make it difficult or impossible to ventilate after delivery. A mass in the pelvis can obstruct the urinary tract and lead potentially to renal dysplasia and dysfunction within the head. Of course a mass can actually replace brain tissue, compressed brain tissue lead to ventricular meagley within the chest. We can end up with cardiac compression. Cardiac tamponade. That dr Mcgrady is gonna talk about more and we can have other issues as well, compression of the esophagus leading to impaired swallowing, leading to poly hydra. Meows compression of the rectum. So really anywhere can can be in danger from these masses. And then another really key issue. Just go back to that one moment more is that there's a risk of high output cardiac states from large and vascular tumors which can lead to actually a high output cardiac failure and then to high drops, which is a really poor prognostic factor. So I'm going to very briefly go through masses, kind of head to toe and then we'll go into more detail in the future slides. So starting in the head, there's many different kinds of tumors that we can see in an intracranial location. Tara toma is the most common and this is a very poor prognosis. It tends to replace brain tissue can even erode into the skull, other tumors that we can see. Or astrocytes, toma, cranial ngoma, neuro ectodermal tumors. Um And the prognosis is often poor with intracranial lesions, but there are some rare exceptions moving into the face and neck. A tear atomic is again the most common diagnosis. This can arise from the actual face and neck itself on externally. Or there's a variation called epic notice that arises from the oral pharynx itself, which can obviously present severe difficulties with airway. Other masses in the face and neck that are common include lymph ngoma and hemangioma moving into the heart, which dr Mcgrady is going to go into much more detail on the most common tumors arrived in my oma. And this is strongly associated with the genetic condition of tuberous sclerosis. And then there's several other less common but really interesting and important tumors, including fiber, my oma, terra, toma, toma and hemangioma within the chest, outside the heart. We have other many other potential chest mass is the most common of course, being see pam congenital pulmonary aware malformation. Excuse me. Um and that will be discussed in more detail by dr lee and dr suki and then there's other masses as well that we can also see in the chest, within the abdomen. I'm not going to go through all of these lesions but there's cystic structures that we can see throughout that can arise from many different tissues, tissues of the excuse me, tumors of the liver, tumors, of the kidneys and tumors of the adrenal glands and I bolted neuroblastoma here because that's actually About the second most common fetal tumor, occurring in one and 10-100,000 verse, depending on who you ask. And finally, within the pelvis we have one of the most important tumors to recognize and discuss which is a sacred cock Singletary toma which can be a very large and potentially very vascular tumor that is considered one of the most common fetal tumors. So how do we surveil a pregnancy that's a continuing pregnancy affected by a tumor? I've already touched on one of the key factors which is surveilling for high drops. So as I mentioned, these some vascular lesions such as a sacred Territory. MMA do put the fetus at risk for this high output cardiac state which can lead to cardiac failure and to high drops. Also any obstruction of the lymphatic system such as a chest lesion or an actual lymphatic malformation can also lead to a state of high drops. And of course for these fetuses we are going to be surveilling their growth and their well being with ongoing antenatal testing plans and finally arriving at delivery planning again, it's critical to have multidisciplinary care and coordination between the specialties. We use our imaging to help plan what the fetus will need at delivery. We have availability of three D. M. R. I. Reconstruction at UCSF, which can be really helpful. Um and then of course we have to pick a timing a location and who needs to be present at delivery for a safe delivery. In some cases were considering an exit procedure. So an ex cetera inter partum treatment where a cesarean delivery is done in a very specialized fashion where the fetus remains on the utero placental circulation while we either secure the airway or in rare cases actually calculate for ECMO or in very rare cases even perform a surgical resection before finally delivering the fetus. So I'm going to briefly go through a case that illustrates a lot of these key points of pregnancy and delivery management. This patient was referred to us from an outside institution where a one centimeter neck mass was diagnosed actually at her 12 week ultrasound and it had already grown to four centimeters by 16 weeks. She underwent an amniocentesis had a normal micro ray and then by 24 weeks it was huge. So a 12 centimeter mass arising from the left neck. Also poly hydra hosts on ultrasound and M. R. I. The mass was suspicious for a face and neck terra toma with potential obliteration of the left oral pharynx and and facial structures. She was counseled by many providers there including AM FM. And anti about the potential for poor prognosis and referred to UCSF FTC. For another opinion. We saw her at 26 weeks and this is our imaging from that 26 week ultrasound you can see on the left there's an absolutely massive mass. So about 15 cm, very heterogeneous cystic and solid components. And that center image illustrates the relative size of the mass on the right to the fetal cranium on the left of that image and on the right are three d. ultrasound allows us to see the association between the mass itself and the structures of the face and neck. She next had the M. R. I. Which is on the next slide and the M. R. I. Again, you can see this massive massive lesion and this is an example of a three D. Reconstruction that again helps us to illustrate where the masses arising from and the characteristics of the mass itself. She decided to continue care at UCSF. And she had extensive repeated multidisciplinary counseling. Um She was counseled very frankly that there was a high high chance of significant morbidity even with best efforts and especially a lot of uncertainty regarding the ability to secure the airway. Even with what was suggested which was an exit procedure. We continue to monitor her closely, including that surveillance for high drops and cardiac output and those remained normal but she did have severe poly hydra meows and a lot of maternal discomfort, maternal Dystonia. So she did undergo an amnio reduction at 27 weeks and we were able to offer whole sequencing on that amniotic fluid at 29 weeks af I was back to 40. The tumor volume was massive at four leaders. So you can imagine how uncomfortable she was being 29 weeks pregnant with an A. F. 5 40 a four liter tumor. In addition, she was extremely uncomfortable with having preterm contractions. And so we began to develop a plan for a very early delivery because of this risk including a serious risk of preterm labor and an unplanned emergency delivery. At 30 weeks she underwent delivery with an exit procedure and she was able to access the fetus. Was able to be successfully intubated by E. N. T. Followed by delivery of the knee in eight and had lots of imaging over the next few days including this cT angiogram with three D. Rendering which helped with the surgical planning of the pediatric specialties. And then at day of life four the neonatal underwent excision of a massive left cervical terra toma. Will left hemi, thyroidectomy and radical neck dissection and complex closure with skin flaps. So a very complex surgery with multiple surgeons present the pathology as expected showed a predominantly mature terra toma with some focal immature terra toma elements and just in follow up she had negative whole exon sequencing. She's continuing to do um serial tumor markers because of the territory and the potential for malignant transformation. But those have been heading in the right direction. And this neo Nate is now one year old and actually doing quite well about to have its D tube removed. So at this point I'm going to turn it over to dr lee our pediatric surgeon and dr Mark. So G. R. Radiologist. Thank you. I thank you dr post hunt mainly here. Um And I just want to go through some of these um details from a surgical perspective and you'll hear some of these things repeatedly. The harmful effects from fatal masses can come from um increases cardiac output. And and my colleague dr Neiman Grady will go through this in detail also from mass effect by compression of adjacent structures. Uh And sometimes it's caused in utero damage as well as maldef elopement of certain organs. Uh And in the case just presented. Obviously airway was key amongst them. We also have to consider long term effects of function. And then although malignancy is not common, we do have to consider whether there is malignant potential in these next slide please. Um So the ones that do have malignancy risk or territo hmas, tara thomas is you probably know or categorized as mature um Which means not malignant um immature which means they could become malignant or frankly malignant. Uh And that's pretty uncommon um they can happen in multiple parts of the body but most commonly in the sacred area in the cervical area and sometimes also pericardial as well, plural pulmonary blast oma is a rare, rare tumor with about 300 cases in the world's literature um that arises from lung tissue. And then of course neuroblastoma which is from the ganglia neuroblastoma lineage. Um So um uh um as far as neck masses we think about uh lymph, oh vascular malformations in the neck rarely need exit procedures unless there's or a fair and jail disease within the the oral pharynx in in the trachea. And that can cause internal obstruction of the airway. Cervical territo Mazz can sometimes result in high drops. And we've once had to actually do a fetal resection because they were high drop IQ. But more often they can cause airway obstruction. Post natally. And an exodus often indicated for cervical terry thomas. And this is the most common indication for neck masses for exit goiter is endemic in some parts of the world and rarely as an indication for exit. I've never seen as as as as an indication for exit. As a matter of fact. Um So dr Sergey, do you want to go over this part of it? Um I just also wanted to highlight if you see this figure below this was actual, this is a picture of actual a three D. Reconstruction of that same neck mass that dr post showed. Um This is actually a physical laser printed model and this is very helpful in planning our exit procedure. Yeah. Thanks dr lee. Yes. In our institution we we complement our ultrasound imaging with fetal M. R. And we have subspecialty um reads from both the neuro radiology side as well as from the body side. So as you saw in the last couple of slides and from dr post as well um just some images from some more fetal M. R. S. This is it case on the top image you can see there's a saddle image of a fetus who had a trans spatial lymphatic malformation. So again the type of mass that for which you potentially be concerned about airway compromise and having these available Antonioli and before delivery can be really useful for for surgical planning as well as for potentially planning for a procedure like an exit. So we use this as a complementary modality and Um and as Dr. Lee said um the three D. Reconstructions can be really helpful as well. Um So the major consideration is obviously airway for territo Mazz. Um The long term considerations for lymphoma vascular malformations can really be significant in that it's hard to completely resect and they can uh invade into structures both in the neck, face and chest. So really careful counseling with the families particularly for the lymph vascular malformations although there they're not as threatening in terms of airway obstruction as the territo Mazz is really critical. Uh Chess masses. We think about a variety of chess masses. There's both intra and extra low bar sequestration uh and forgot duplication cysts. Uh These rarely uh cause high drops. And really the basis of these is just to treat them post natally. See pam's however, can cause high drops and those with um CVR see pam volume ratio of greater than 1.6 have a 50% plus risk of hi drops. Uh And when we see high drops, we used to often do open fetal surgery, but that's been replaced by minimally invasive or noninvasive treatments as macro cystic shunts are treated with shunts and very effective at relieving high drops. And about 20 years ago we came up with the use of maternal maternal steroids for micro cystic lesions. And our hypothesis is that what happens is is that micro cystic or solid c pam's are immature lung tissue. And that the steroids just like when we give steroids to mature lung tissue for preterm delivery, causes maturation of this immature lung tissue and causes evolution. Uh And uh really quite effectively treats it. The one thing to keep in mind is plural plummeted blast oma. Again, very rare, but if it's in multi multiple lobes and very big, then we should consider plural pulmonary blast oma. Um dr Sergey. I don't know if you want to go over this. M. R. I here. Yeah, sure on this, on this image and also on the next slide. This is just showing a saddle em. Our image of the fetus in the second trimester and the finding here is somewhat subtle, but I'll show a couple of images on subsequent slides that really highlight the use of em are. So in this case you might be able to appreciate where the arrow is pointing that there's some increased signal intensity in the portion of the lung anterior relative to the portion posterior lee. Um So some of these types of findings aren't always um they're not always apparent Sana graphically. So an ultrasound but one can look pretty normal. But the difference is due to a Dema or um in this case there was some extrinsic compression on the lung due to large pulmonary arteries that resulted in an electrolysis. Post natally was evident on the prenatal M. R. So these kind of subtle changes in signal abnormality can also be very useful um and are well depicted by M. R. Um So as I said that the first fetal treatment for hydro pixie pants was actually history to me uh and fetal thoracotomy described by Mike Harrison, my senior partner in Lancet um now over 30 years ago. But as I said, macro cystic lesions are treated with oracle amniotic shunt with 70 plus percent survival. First described by Doug Wilson who was at chop there almost 20 years ago. Um Microcystis prasad lesions. However, we described the use of maternal steroids and very effective With high drops probably 85% survival. And this is a picture of open fetal surgery for a lumpectomy for a lung lesion. Next slide please. Um so if you click again the cumulative centers experience, I'm sorry that uh that's okay. Um we can just go onto this slide. Just some of our initial experience shows um survival to discharge within the steroid group was 83% compared to 56% with open fetal surgery. Mean gestational age of delivery was was greater with steroids than with open fetal surgery. And resolution of high drops was better with steroids and with open fetal surgery and only one out of 12 of the patients with steroids needed ventilator after delivery as opposed to virtually all of them with open fetal surgery. And so we have just um um shown as several other groups have followed us that steroid therapy for high dropkick microcystis C pans is a much better treatment than open fetal surgery. And I I don't even remember the last time we did open fetal surgery from hydro optic micro cystic, see pan next slide fruits. Um So pluripotent blast omagh's the characteristics are there in multiple lobes there really large often separated and have cystic and solid elements. The key is to check the dicer one mutation. Post natally. This can be done from blood samples. It's an autism all dominant mutation mostly seen germline and it's a very complicated tumor to treat um treatment requires surgery plus chemotherapy. So if you have these characteristics of multi lobe are large cept ated cystic too solid. This really needs referral to a center that has experienced with these. Um And uh really a challenging diagnosis but fortunately very very rare. Next slide please. So dr Susan. Yeah this is just a click again. Just these are just a couple of slides showing again back to that long signal abnormality. So one other thing that we do with them are depending on the type of mass. Sometimes it's not necessarily a mass that we're looking at. We have a lot of congenital diaphragmatic hernia patients for instance with a large amount of either liver and or bowel up in the chest. And so we routinely calculate lung volumes for a lot of these patients and M. R. Allows us to do that quite accurately through measuring both lungs entirely instead of just using a trace method or rather just a single measurement. And if you continue to click forward and just onto the next slide I think that I would just want images showing some normal lungs for third try comparison. So again these can be somewhat subtle on M. O. R. But the differences in signal abnormality can often be indicative of an underlying mass or for example a CPM. Um That may be less conspicuous Sana graphically so they're complementary modalities I think next slide please. So this case the one that was just shown on em are actually just turned out to be a right middle lobe collapse add elected system. The setting of a if you had tetralogy of follow with absent pulmonary valve. So this fetus had dilation of the pulmonary arteries bilaterally, which actually caused some degree of extrinsic compression on the error way, which resulted in this right middle lobe collapse, which you can see post natally on the X ray here and on the subsequent cT as well. You get this segmental, add electrolysis. So pretty interesting finding um not quite a mass but a really nice correlate between the prenatal imaging and the post natal radiographs and cT. Um so as far as abdominal masses um really um no, you know, there isn't an indication for fetal imaging in terms of needing to do fetal surgery but often um uh so a little bit of, you know sort of word of this differently but often obviously families and clinicians want to know what it is and so um we often do get M. R. I. Um Perhaps a couple of things to know are larger variances may need surgery shortly after birth to minimize the chances of ovarian torsion and the cut off size that we use about five centimeters. Several studies have shown that if they're bigger than five centimeters then there's a higher risk of ovarian torsion. So we'll do a cyst ectomy shortly after birth. And we can do this laparoscopically and really minimally invasively. And then the other thing to really keep in mind is if you have a political system this needs to be excised uh to decrease. Um not only the risk of biliary tract obstruction but also malignancy. And we usually do the surgery around one year of age. Pretty complicated operation. But we can do these laparoscopically as well. Dr yeah so as dr really said these may not necessarily change the prenatal course. Um But determining what they are at least characterizing the lesion and and giving um you know the sense of what this could potentially be. And some prognosis can be very helpful. So this is a case of something benign that this is just a hemorrhagic ovarian cyst which on the corona or kind of oblique T. Two image M. R. I. You can see that there's a cystic lesion. Um They're very subtle cept ations that you can see which I think you can appreciate better on the ultrasound image. On the bottom left. The very classic particular pattern of echoes. This turns out just to be a hemorrhagic cyst actually better characterized on ultrasound in this case. Although some additional sequences also show some of the associated blood products and hemorrhagic cyst on the MRI. But again not something necessarily it will change the prenatal course but helps for post natal planning in this case this was fairly small probably a couple of centimeters. So um as uh analisa said dr post said that sacred cox's territo mazar, one of the more common fetal tumors. Still very very uncommon. Probably about 10 per year in the state of California for instance. And we usually see about, I would say we usually see about almost half of those at UCSF. Um Prognostic factors include size, tumor fetal ratio when they're cystic, much better prognosis when they're solid, better prognosis when they're mostly external versus internal. And dr moon Grady will talk about the combined cardiac output. High drop results in demise unless treated. Treatment without high drops is post natal resection, which is most of the fetuses um with high, excuse me. This should say. Treatment with high drops Less than 28 weeks. Consider fetal surgery, including all the things that means to the pregnant woman At 28-32 weeks. Consider both fetal surgery and early delivery and after 32 weeks early delivery. Um so dr mark. Yeah, just another image here we have a lot of, although it's rarely a fairly rare tumor, I think we've seen quite a few of these um Ter tomas before and often we're asked to characterize whether there's an interest pelvic component. Um and how much of it is inside or outside of the fetus. So this is another M. R. Just showing this predominantly intra pelvic cystic terra toma. Um just nicely characterized separate from the bladder here. And just this picture is a picture of open fetal surgery and you see the terra toma on the inferior part of the picture and the fetus. We just try to deliver only the part of the fetus that we need. And this red rubber catheter you see going in the rectum so that we protect the rectum during the surgery. Next slide please. This is just a complementary couple of images of this is a separate patient with a similar sacred cox. It'll tear a toma. This feed has had both an intra pelvic and extra pelvic component um which you can see on the ultrasound nicely. Some of this more eca genic solid appearing material and this multi lock related cystic appearance that extends below on this actual image. And then on the M. R. This is a sad little view of the same thing and you can see sort of the cystic component, posterior lee and some of these more grayish solid components entirely. So again, we kind of use this these complementary modalities to characterize the size and also the type of mass. The solid and cystic components of it. So just a few things which dr post alluded to the maternal considerations. It's a classical hysterectomy for fetal resection and patients cannot have trial of labor be backs after C. Section. There is a high risk of uterine rupture. If they do, we learned a lot of these lessons from our fetal myeloma legacy all the moms trial. Uh and part of the counseling should be the possibility or desire for future pregnancies. Next slide please. So summary most tumors can be treated effectively post natally ultrasound and M. R. I. Are used in adjunct and can be helpful to identify those tumors that need fetal treatment. Fetal treatment can result in survival for some high dropkick fetuses that otherwise would have demise. Um And fetal treatment also in um is means exit when appropriate, particularly for the neck masses. It really requires a multidisciplinary approach for best outcomes and I'll stop there and turn it over to my learned colleague Dr Moon Grady. So I'm gonna take over the slides now. I am Anita Mcgrady um a pediatric cardiologist in case you missed the initial part of the introductions. I'm going to talk about masses that are interesting to me which are actually all of the ones that have already been discussed now. Why would a fetal cardiologist be interested in anything that's outside of the heart? Well, we'll get into that. The cardiac tumors I want to discuss first though, just as a brief overview, um these are very rare. Many are diagnosed in the neonatal period and the true fetal incidents of these tumors. It is Largely unknown. Although estimated at about 19 per 14,000. The U. S. As was already mentioned are primarily rhabdo myo hmas with terra toma is coming in second. My brahmas maxima's hemangioma is. And then a handful of others that are all worthy of case reports. Although most of these are histological e benign there or they can be associated with pericardial effusions, compromised blood flow, arrhythmias, myocardial dysfunction and eventually fetal hydra. It's this is an example of a patient with fetal my oma. These are very easy to recognize their homogeneous echo, bright and often multiple. They can be inter kava, terry intramural. They can involve the myocardial um or the atria um the ventricles or the atria there often well circumscribed but they can be podunk elated and inter cafeteria and they can also be sort of massive and almost appear to be pericardial. About half of them are asymptomatic but in the others there can be some obstruction to inflow and outflow that depend on the size and location of the they can lead to high drops if this is the case, as is shown in this example where there is mitral regurgitation, an extremely large but also on the right side of the heart. We see that there is no obstruction to inflow. So uh just the size does not tell you the whole story and um it's really necessary to add color Doppler and to actually assess the cardiac output and the venus Doppler, we have occasionally recommended premature delivery if the fetus is viable but usually we just observe if they do develop high drops. However, that may be an indication for some of the novel field therapies that have been recently described. This is case report in the new England journal using maternal administration of surliness to shrink the tumors in a fetus that was high drop IQ. And I would just caution that although these drugs are available, they are not necessarily benign to the mother. And so multidisciplinary approach to potentially treating a maternal fetal dad with this type of problem would be indicated and certainly would be what we would do at our center and should be reserved only for non viable gestational age. With hydra apps, terra toma Z. R. As I said, the next in line, as far as as frequency that that we see them. We see maybe one or two of these every couple of years. They're extremely rare but pretty easy to see in the fetus as I'll show you they developed from the pericardium and attached to the roots of the pulmonary and aortic valves. And so they almost always look like they're arising just above the the right ventricle, in between the ventricle and the atrium. As shown in this example, they very, very often have large pericardial effusions but will almost always have normal doctor stenosis. Doppler. So as I said, they developed from the pericardium attached to the roots of the pulmonary and aortic valves. Their heterogeneous with cystic and solid components and can have even calcifications in them and with the pericardial and pleural effusion is very easy to recognize. So once you've seen one of these, hopefully you would not miss it in the future. We also look at fetal well being because the infusion is secondary to the tumor and not necessarily a reflection of hydra apps. We need to monitor fetal well being. And this is where the cardiologist starts to become very uh interested in um in the monitoring of these patients as they may develop evidence of high central venous pressure prior to developing overt high drops. However, the converse is uh is not true. If they have hydra, they don't necessarily have to have abnormal Doppler. And so in this fotis who rapidly developed high drop the different fetus from the one that I showed um this developed within a few days of of the initial referral and still had normal doctor stenosis Doppler. So really uh case for looking at the whole picture and not just anyone uh feature prognostic lee. The best measure seems to be combined ventricular output. This is a paper from Children's hospital of philadelphia that reported fixed fetuses with pericardial terra toma and all of the terra Thomaz were characterized by rapid growth with gradually declining combined ventricular output. So, tumor size in black and combined ventricular output in red and normal is about 500 cc's per kilo per minute. So we can see that these are all uh later in gestation falling below 300 cc's per kilo per minute. And that's probably a combination of mass effect and decreased cardiac output and insufficient oxygen delivery to the tissues that is leading to the fatal high drops in these patients. So the argument is to intervene just before you start to see high drops, but certainly as uh as the cardiac output drops, they need much more intensive monitoring and we can do fetal surgery to remove them. But uh that has only been successful in a very small number of patients nationwide. Fibromyalgia are rare, so rare that I don't actually have a picture of a fetus with fibromyalgia. But on the differential diagnosis of rhabdo myo oma is if the rab if something looks like a ragdoll, my oma, but it is single. Sometimes we entertain a diagnosis of fibromyalgia and this is where post natal M. R. I. Is helpful. Mix omagh's are also quite rare in childhood. Much more common in adults. And our polyp oid masses that are soft, friable and can be seen as shown in these still frames um to uh sort of prolapse into the ventricle and then back up into the atrium with the cardiac cycle. So a freely mobile mass in the atrium should uh make one think of soma and Haman gee. Almost finally, also very rare. And this one that I'm showing sort of mimicked a pericardial terra toma but without the pericardial effusion. Pericardial and myocardial hemangioma is can have effusions associated with them. Um And they are non homogeneous. So as I said, they can they may mimic a pericardial tera toma. So um uh again, vote for post natal MRI. What about the extra cardiac masses that have been so nicely discussed by my colleagues. Um Inter thoracic masses also interest me, even if they aren't directly attached to the heart. We have C. Pans and um and a handful of others. I will just talk for a moment about C pans as dr lee told us they come in different flavors. This is a multi uh sorry, a micro cystic. So this is a homogeneous echo bright uh tumor. And these respond quite well to steroids. We look at the size of the tur. Um But we also look at cardiac size and cardiac outputs in these patients. This is a much more ominous looking mass. Not only is it bigger but it is not my closest IQ. If there's a micro cystic and macro cystic components to it. And the ratio of the heart to the total size of the thorax Is very small, with normal being about a third. And this is about measuring about a little over 1/10 of the entire thorax. So When the heart size gets below about 20% of the thorax and the outputs are low. That is when we start to be concerned about evolution of high drops in these in these chest masses that are extra cardiac, they may or may not be associated with doctor stenosis. Doppler abnormalities. So again, a multi pronged approach to the valuation of the thermodynamics is necessary. The physiology of this I think is just fascinating. And was studied um many, many years ago by Mike Harrison in a land model where they put in a tissue expander in the chest and blew it up and see how quickly the high drops evolved after a certain tissue expander volume. And as soon as you uh let down the expander that high drops resolve. So this is a direct effect of tamponade physiology on the heart, leading to low cardiac output and poor oxygen delivery to the tissues. And then this is the data that I mentioned about DPM uh size and thoracic size cardio thoracic area ratio in high dropkick versus non hydro optic with a cut off being around 20%. We also see in flow Doppler abnormalities in these patients when they begin to evolve. High drops also signifying that there's tamponade physiology. If I croak oxygen territo Mazz, the other mass that uh we see a lot of in our cardiac program. And I find very interesting. They have been talked about there's a small mass that grew quite a bit in this fetus who reached a viable gestational age without developing high drops. And so was delivered electively. It was noted by my colleague norman Silverman and again, Mike Harrison peter Callan years ago that these patients who had a secret coaxial tara thomas had a very very high cardiac cardiac output Combined ventricular output. As I said around 500 is normal. So all of these uh measurements is actually only three different fetuses but all very, very high triple the normal and developed high drops due to high cardiac output, high output failure. So we wanted to look at the fetuses who were not yet hydra pick to see if we could um devise any sort of scoring system for those who were likely to develop high drops. And so we were lucky to have a large natural history uh experience here at UCSF and indeed Found that with high combined ventricular output over 500 that they tended to have poorer outcome. That has shown in black. And we also looked at a variety of either combined score which is the cardiovascular profile score or the individual components cardiothoracic ratio, aortic and mitral valve sizes and tv valve regurgitation and some diastolic indices and the ones that were significant between the group that had a good outcome for poor outcome. Again, this is without fetal therapy. Just natural history were those shown in red And so the conclusion from from this natural history study that in all fetuses with a poor outcome, there was at least one of the findings of enlarged heart, high cardiac output regurgitation or um mitral or aortic Z score um suggesting that there was mild dilation of the chambers and no fetuses with good outcomes exhibited any of these findings. So this now is pretty standard for us to help risk stratify and patients with all kinds of high output failure. Um extrapolating this sacred custom terra toma data to two other fatal to us. And so with the patient that's been discussed, the giant cervical terra toma. We saw her at 28 weeks, valve sizes were normal, but the cardiothoracic ratio was a little bit elevated. She had no significant regurgitation. And this is our calculation of the combined ventricular output which was um not elevated, it was 520 mph, which was um since we didn't have a fetal weight indexed to gestational age. And this is a graph that tool that we've developed for um for Showing what the output is versus gestational age. So she was at 50th%ile, so mildly enlarged heart, nothing else going on and cardiac output normal at 28 weeks. And that was evidence that high drops was not impending in in this fetus. And as you saw we got several more weeks now, if were concerned or sea elevations, we might see them weekly to um to see if the uh cardiac outputs are tracking with gestational age or becoming abnormal. Um As far as high drops, I just have one or two more slides to uh to show how we are now using this comprehensive fetal echo assessment. Uh This was a paper that crystal darian worked with us as a resident I believe, I believe. Um looking retrospectively at 167 fetuses that we saw for a variety of indications and there was a clear demarcation between high output high cardiothoracic ratio legions and the low ctr. Low output lesions. So uh given that we then divided them into the high versus low and generated a cardiovascular profile score uh which takes into account many of the variables that I've already mentioned. And as it turned out in the high output failure patients, the cardiovascular profile score was predictive of survival. It was not predictive of survival in the low cTR. But a lot of these are ones that had um that had intervention. And so uh it interestingly though the non survivors had pretty good cardiovascular profile scores suggesting that it's um more uh the compression from the mass than actual over cardiac failure. So to summarize the fetal masses and cardiovascular function we incorporate a common basic evaluation of baseline outputs function, filling venus Doppler is cardiothoracic ratio inflow Dobler's and cardiac output for risk stratification for high output failure and for chess masses. See cam. See pam and pericardial territo Mazz. We look for signs of cardiac compression and tamponade leading to low cardiac output failure. Most thoracic masses do not cause compromise. Most sacred cock singletary toma is due. Um And then uh I had prepared a few slides on inter operative human dynamic assessment that we also do. But I think in the interest of time we will skip those. So thank you. And I think that this is time for questions. I also want to turn over to Aaron to talk a little bit more about our fetal treatment center. Good afternoon. My name is ERin Matsuda. I'm the patient care and service line director for the federal treatment center in both san Francisco and Oakland. First off, thank you to dr post dr lee Dr Sergey. Dr Mcgrady for taking the time to come and present to us today. Thank you to all of you for calling in and attending um If you've missed this, if you want to hear this again in a couple of weeks we'll have this posted on our med connection website for U. C. S. F. In addition on the slide is how to refer and how to contact us. If you have any questions for any of our wonderful providers who presented today, um you can reach out to these phone numbers as well as for patients to refer.
