Dr. Farokh Demehri - The Pectus and Chest Wall Treatment Program: An Update

We'll just give us one more minute and then we'll get started. Okay great Dr. Fisherman you think it's okay to start? All right wonderful. So good morning everyone. I would like to again welcome you all to Circle Grand Rounds this morning. We have the pleasure today of having one of our very own Dr. Faroque de Miri joining us to come speak about an exciting and growing program here in the department. So most of us have the privilege of knowing and getting to work with Dr. de Miri who is an assistant professor of surgery of Harvard Medical School and a pediatric surgeon and intensivist here at Boston Children's Hospital. Dr. de Miri completed undergraduate education at Duke University followed by medical school at the University of Florida where he was actually valedictorian of his medical school class. He then completed General Surgery Residency at the University of Michigan. Following residency he came here to Boston Children's Hospital for his pediatric surgery fellowship. After being in attending for a year he then made the brave decision to do an additional year of training and critical care. That which he just completed. Dr. de Miri has received many accolades during his career thus far with the most impressive being his many teaching awards. He has renowned teaching abilities of which I know many of us both junior and senior have benefited from. Dr. de Miri has many clinical areas of expertise as well. One of these expert areas is that of chestwell abnormalities including chest oscobatum and keranodum. So we are honored that he has agreed to join us today to update us on the Pectus and Chestwell Treatment Program here at Boston Children's. So for Roke thank you so much for your time and your commitment to not just the department but also to our education. We look forward to your talk. Thank you. Jamie thank you so much. Those words mean a lot to me. I really appreciate it. Thank you everyone for having me this morning. Make sure everyone can hear me okay and it thumbs up. Great okay perfect thank you. But thank you I've been looking forward to this and hopefully we can spend some time talking about chest wall in our chest wall program. I'm going to share my screen. Okay. Okay everyone see my slides okay? Great another thumbs up excellent thank you. So today we're going to talk about our Pectus and Chestwell Treatment Program and hopefully just give a bit of an update on some of the things we've been working on. Our whole team is working on. I have no financial disclosures. I'm going to talk about a few devices throughout this but no financial relationship with any of them but the use of the devices that we use for these patients. Disclosures as Dr. Robinson mentioned this is not typical of the Grand Rounds where one might talk about their own career and accomplishments as far as research goes. I think this is more of a hopefully a preview of things to come. As you mentioned I only finished fell a three month ago but thanks to the work of our team and my and my partners on a lot of work. A college was have been made in development of our program. So I'm going to use this time to share the work of others here and elsewhere and mainly brag about our awesome team and then we'll discuss some of the best of what's sort of out there and what of those things we've incorporated into our practice in the past few years. We'll do a brief review of the chest full treatment program here at Boston Jolt is Hospital in the history of it here and then talk about what's new in fact is six of bottom specifically that's that's really what we've been most of our time talking about. Work up cryoblation and vacuum valve therapy some changes that have come recently and then things down the down the road including white light scanner and modified percent depth which will be more. So here is our practice and treatment practice and chest full treatment program it's about a year old as far as its current sort of iteration and here's the full team. I want to take a second to mention now and later who they are because they've been doing all the work. We have our nurse practitioner crew Kelly Jennifer Katelyn and Lindsay and in recent addition is Selena, our drug who's our program coordinator and then the positions who are seeing patients in the clinic are myself Dr. Jackson Dr. Mooney Dr. Little High and Dr. Papa Docuys. Now obviously this is a place one of my favorite places I work in here is the history and mentorship that that exists here and the treatment of chest wall here goes back quite some time and Dr. Shamberger of course has been one of my main mentors in this area and you know he's he's actually sat down on the yesterday and I was asking him about some of the history of test fall surgery here and it goes back to Dr. Pendren and particularly Dr. Welch. So Dr. Pendren bodice practice over from MGH and was doing some innovative things early on with with open repair and placing sort of intra majorlary struts called rebine struts to support the chest fall but the high volume practice really was Dr. Kenny Welch who's pictures also in the hallway and thanks Alex Kwenko for taking this patient send it to me yesterday. Dr. Welch was really known for his chest wall practice and served as Dr. Shamberger's main mentor and as we'll talk about later the open technique which is commonly referred to as the ravaged technique. Actually some of the attributes of that approach were innovated by Dr. Welch particularly the preservation of the paracondrial sheets to allow regrowth of the car liches and then Dr. Shamberger of course spent many years in research in this area and innovating as well in the open technique and we'll talk about some modifications he added to it including the more particular about the timing of the operation the use of a retro-sternal atkins strut and preserving the rectus insertion into the chest for the open technique as well. We'll talk a little bit more about that. I want to take a few minutes to really acknowledge sort of built this program from the beginning. Now as far as our team goes now one thing you know as with any of our programs and centers go I'm having people sort of driven to organize everything is really important and I really want to acknowledge up front Kelly who's stepped up to be our primary practice nurse practitioner and much of the sort of early work here that we're talking about she's helped to spirit. And with this program which like I said is over a year old with Kale and Boyd himself and others help me then officially a program with the website and such and you know build it and they will come. So in the first year since inception of the program past year 382 unique faces were seen that's an increase of 45% over the average of the previous several years. Interestingly about half and half X to bottom and care not even though the majority of patients typically are X to bottom and we're still doing work to improve access coordination of care and safe position. So our current clinics are being set in fourth Friday and the ideas optimized on sort of efficiencies we've seen visit stress x-rays trying to organize same visit brace copy valves for care dot in patients and same visit vacuum belt fittings which will talk to patients for interest and appropriate. So who are our patients these are the main two things that we see. Practice X to bottom practice care not and the classic story is a teenager who's afraid to take their shirt off in locker room. We're not going to get too much into the path physiology and things that is one that sort of just lay the picture this is the type of patient we're going to help today. So practice X to bottom on the left funnel shapes sunken sternum care and autumn. For chest and other name for it for protruding sternum. Sometimes we have these mixed effects. Okay this on the left is an interesting one which for those who might see these patients want this to be one that you remember because you might be might think oh this is a pectus care and autumn but this is one that would not typically respond well to bracing therapy and this is actually what's called pectus arqueo water or a contravenuvial defect where actually it's it's protrusion up top at the maneuverium and then a depression down low at the sternum and on the side actually an x-ray sometimes it looks like a little comma but this is one that typically does require operative repair and class that actually open up here but also that's a mixed effect one kind of the other one on the right is protrusion on one side and depression on the other and so sometimes you have to be creative in the way that we approach these patients. So what types of chest wall deformities are as Dr. Schaimberg or Dr. Mooney I know with what's my recipe for deformities because it's true that actually when we say these patients have deformities it these are sensitive patients who are teenagers and the truth is the the line between you know what's the deformity and what's you know along the spectrum of growth is hard to draw and so as my mentors have taught me try to use other words such as confirmation or shape of the chest when when talking to patients but the main things we see are exyvato care at autumn rarely slipping roots syndrome sternal cleft and pole and syndrome we're gonna spend pretty much all the time talking about exyvato this is a main operative thing that's the thing. So let's quickly walk through case and get some of the innovative things we're doing. So what symptoms my this patient have well generally it's well tolerated and actually most common is notice early life at infancy but the patients the family's really bring bring it to physician attention later in puberty as he goes with the growth spurs. Symptoms are typically mild but can be more significant. Jordan subbreath is the classic thing exercise intolerance chest pain as with what many think we're pain is the reason you're operating should raise a little bit of red flag but sometimes you do have pain actually with it but the main thing is short and subbreath. But and the most important thing is a psychological presentation often these patients are withdrawn self conscious avoid gym their teas they avoid sports and they're bothered by it and as I tell the resident some you know the most important question asking in the clinic for the patient is does this bother you right if you have a patient who doesn't mind it and a family doesn't mind it and that's a very different story and one that may not be appropriate for an operative repair compared to one who's really the lifestyle is significantly impacted by it. But then I guess to the question isn't just a cosmetic problem and it is a significant one I'll say that these patients are often the most appreciative patients to have you know those will do a middle-of-night X-lap on a baby who's dying and then a elective operation on a teenager and these patients are are sometimes multiple fold more appreciative because it really changed the life in a different way. But there are physiologic impacts the physiologic impact is debated Dr. Amber and others have done work over the years looking trying to understand through some really intricate tests the physiologic impact. In the it's it's still a debated thing the bottom line is that there is good evidence of reduced cardiac and pulmonary function with aerobic exercise primarily with severe defects at the extremes of aerobic activity. So when we see this patient what are the tests that we want to do? Okay so the goals of pre-operative imaging are to understand the anatomy for operative planning and to get some objective measurements of physiologic compromise. So what we'll usually do in our clinics we'll get a full history physical exam we'll consider an echocardiogram and we'll talk about that but the real reason is to screen for Marfans or other connective tissue disorders and to look for aerobic problems. We're going to chest-liquid or CT at certain discretion really to see to make sure we understand the anatomy as well as we need to without exposing the patient to unnecessary radiation if we don't have to. Cardi-pulmonary exercise testing, PFT is an allergy testing. So that echo usually what we may see would be in perestropling increase with that cardiomyeliprolaptic is a common finding and then recently we've been doing this called positional upright echo. Having the patient actually sit up rather than letting it back sit up and then forward where we might see that the heart as it sits against the sternum we then see a right retricle outflow tract obstruction, which explains by some patient positional symptoms and as I mentioned rule out aerodilation. And the reason is important one of our partners in the program is Dr. Ron Lackrow who's the director of the Cardi-Vase and Genetics Clinic here and you actually gave a great talk with us recently. We invited him at Dr. Mooney's suggestion to discuss connective tissue disorders and actually you know the incidence is quite high so some of the things with the connective tissue workup that we look for is scoliosis, Marfans syndrome, 37% of patients have family history and then as Dr. Lackrow reviewed with us, there's been evidence actually that incidence is pretty significant. So in a recent study from Cohen childrens of a 241 patients who presented with Pectis all of whom were for connect tissue workup as part of the study, 5.3% had Marfans syndrome that's compared to 0.3% of the general population so much higher. And this is much higher in patients by the combined or complex Pectis issues and the overall incidence of cardiac anomalies was 35%. Now those in Marfans 84% have cardiac anomalies so with that in collaboration with him our practice is shifted to referring all patients who are severe enough for any kind of intervention or evaluation by his team and then also those who screen in on exam and history. Sometimes their patients who are mild may not be interested in intervention but still based on flexibility scores and family history will still sometimes refer them for this evaluation because obviously as I mentioned Pectis issues are generally not life threatening however we don't want to miss this as a first presentation of a connected tissue disorder which could actually be life threatening down the road so that's why we want to not miss this. The remainder of the workup will do exercise testing and includes cardiac pulmonary exercise, test and metabolic testing where we're looking for auction consumption, V.CO2 as CO2 production and often these will be reduced again with exercise. And then PFTs typical findings would be reduced FVV1 and FVC, your force biocompatity, an unchanged total lung capacity. And as we document these sort of findings preoperatively then the question comes up is will this get better if we fix it? And this is another area of interesting debate because the data is kind of variable. And you know once a child reaches that a lessons and your parent early studies showed that actually that it was very hard to detect improvement in those objective studies after a pair. However with interestingly with the sort of increasing use of the minimally invasive technique there have been more research on that after bar removal. Okay so we'll talk about we leave the bar in for about three to four years. There is a mild increase in the objective measurement of FVV1 and cardiac output. And that might have to do with the preservation of chest wall flexibility with the minimally invasive technique. However the most important improvement is in the psychosocial improvement. And Dr. Schamberger and partners are a really nice multi-center study in pediatrics. I'm really showing how this dramatically had improvement in quality of life scores after a pair. So that's really the real drive for doing not surgical pair of these patients. So let's talk about some scores. How bad is it when we see our patient? So there's a few indices for the residents, the one number that we can know is the classical one which is called the HALR index. We'll talk about a couple others, the correction index which is becoming which is also really helpful. And then one that's down the pipeline called modified percent depth. Okay so these are the three measures based on cross-section limiting. So the HALR index is the one that is actually most commonly used and the one that actually insures companies still requires the document. And it's a measure on CT scan classically of the lateral distance across the chest wall from side to side. Right. And then the distance distance two which is the distance from the sternum to the spine. Okay so the wider from side to side and narrower from front to back the patient is the higher the HALR index is going to be. So here on the left we have a still significant pectus of 3.5 HALR index and get us high up into 20s and 30s for extremely severe ones to see on the right there. We always need to do a CT. We will actually calculate our problem characteristics right. However many insurance companies will not accept that. So depending on the complexity case we'll still get a CT often. The cutoff typically we're calling it a severe pectus is over 3.25 and that's the severity to consider the whole is 2.5. I can do any sound. The problems with the HALR index though were that it doesn't account for overall chest shape. Okay some patients as you know are sort of can take shape chest other people are barrel chested. Okay very round chest. HALR index is a little bit a challenging way of patients who are at the extremes of chest shape. You see a patient on the far left there is actually normal doesn't have you see the sternum no pectus next to bottom. Whereas the HALR index is 3.7 and that's because there are a pain cake shape chest. Whereas the patient on the right actually has significant pectus next to bottom with the real depression of the sternum as you can see there on the front of the scan but their HALR index is normal 2.15 and again it's because of how round that patient's chest is. So it's not perfect for saying you know in the end when a surgeon looks at the patient says yeah you have real pectus next of bottom. The LARWAY is still the most important thing. So with that Dr. St. Peter in his group described let a decade ago this thing called the Crescent Index which is really measuring basically how much you could correct it if you repair it. Okay it's the distance from the deepest point of the sternum to the spine and then the distance from the most anterior proportion of the ribcage to that same line and then you say what percent's correction can we achieve. And typically with this a correction index of greater than 10 percent is considered significant or severe and they looked at this and found that it had a better discrimination between normal chest and pectus chest than the HALR index. So this is a measure that I like to you know we talk about using one needed and clinic to help distinguish those patients who you know the HALR index doesn't really match what we're seeing. Now the modified percent depth I want to mention this because it's something that we're going to be participating in a study for. So this is essentially a non-radiation and non-imaging based measure external measurement of that correction index I just talked about. Okay it's actually pretty low tech and simple it's using a $37 external caliper that's used in orthopedic for like measuring spine and greatest and dissociative stuff and measuring from the outside similar measurements. Okay the distance from the deepest point in this sternum to the back right this the balance external and then the distance from the most anterior part of the ribcage to that same point again trying to predict how much improvement we could get and similarly a modified percent depth of 10 percent or greater is very sensitive to specific or severe pectus at the bottom and the idea is that this maybe something can be used without any radiation exposure to measure how severe these patients are. And so thanks to the work of our team Nicole Wynn who just set down her position as our research coordinator and then Kelly and Jennifer and others here we've just got IRB through and so we're going to be participating in a multi-center study with the Eastern Piatty Surgery Network to track the outcomes of our patients using this and use a picture of them for the caliper is able to use them. So let's talk about surgery. All right so the options now so we've done our work up we've measured how severe our patients affected this about amazing and now our job is to counsel them through their options. Okay so the options include open repair, minimally invasive repair affected this bottom we're commonly called the NUS back to embellishment or no intervention and I mentioned that here because before we get too much into the operative porous site you know it is out of the patient come to see as many of them rightfully choose no intervention and that is that is okay I think this is where getting a good understanding of patients social psychosocial physiologic predispositions is real important because some patients are best in a managed with no injury. But the patient needs appropriate for repair there's two main options the welcher open repair and then the NUS procedure minimally invasive repair. How we think about who is best for which surgeon preference is a part of it and then things that will lean more towards an open repair would be asymmetric defect a mixed defect someone wants to talk about particularly when you have that perfectis arqueo bottom. Some things about it does involve a larger incision longer operative time. The minimally invasive repair is really most straightforward in a symmetric defect it has small incisions typically shorter operative time. The difference in pain between the two I think is changed recently which we'll talk about and then as I mentioned there may be a benefit to improve test law flexibility long term minimally invasive repair. These are some of the big picture things one other thing with the open repair is for patients who have cardiac anomalies who may need a sternotomy in the next three to four years another reason to get the echo is because if they're going to need the bar out to get a sternotomy in a couple years it may be best to do the open repair because that strut only means a few six months. So here's the open repair and thank you Dr. Shamberger for these illustrations in this light of what I'm going to go over is a technique that again is maybe called a ravage here we call the Welch and Shamberger technique based on the technolot of patients that they contributed and we'll swap through this briefly. So this is typically done with either a midline or inframarine incision and you start by lifting something for a flaps as illustrated for you to get down to the chest wall and then the first and most time consuming part of the operation is doing the sub-paracondral resection of the cost of cartilage and here's where initially this is reported by ravaged it to to remove the whole cartilage. Dr. Wells really emphasized preserving the paracondrial sheath in order to improve the growth of the cartilage's long term and here you can see illustration of how that's done and making an incision along the anterior border of the paracondrium and then excise the actual body of the cartilage and here's more illustrations of that and Dr. Shamberger in doing these operations with him you'd always emphasize maintaining the growth plates on either side so that growth can have a special chance of ongoing afterwards. And here's some illustration of some of the interpretive photographs of the same thing. So again that launch utilization on the front and here we see using the wellchelle paracial elevators to get underneath and around the body of the cartilage and then excising it leaving the paracondral sheath. And here when all said and done after excising all those you can see the sternum in the midline and those white circles are the cut ends of the medial aspect of each fuzzle cartilage. And then there's the adcun strut underneath it and the next step after exdoing your subparacondal exisions is to do the wedge osteoonyms. So this allows you to actually pop the sternum forward and so with this technique you use a we use a dremel tool to excise the anterior plate of the sternum just inferior to the medial brim usually between ribs two and three and then and here's some pictures in the operating them cauterizing and then drilling and then using a taliflet by doing a pretty forceful maneuver to cause a green stick fracture of the posterior plate of the sternum in order to lift it up and then passing a retro sternum at constrict which is illustrated here which we then fix to the cut edge or the bony aspects of the ribs lateral and here's photograph of that behind the sternum and here's a post-op x-ray of this front in good position with you look at the lateral view of the correction of that sternum and this strut because of this one time improvement everything we've already sort of disconnected the sternum from the lateral aspects the strut doesn't need to be in that long about six months typically unless they have connected to the support you may want to leave it a little longer and the use of this strut and the preservation of the rectus sheets as I mentioned down here actually not potentially not disconnecting the rectus from the sternum or two of the other technomatocations that are chambered against this technique so with the open repair I want to mention one of the things which is timing as here's a five-year-old boy with a chest wall deformity or confirmation configuration and the question comes in is pretty severe you know so should we just fix this now while the kids chest walls still flexible and for years answer was yes Dr. Welch and others would repair these patients when they were young and flexible four or five six years old and it's actually much easier for those who were there during that time operating and and made a much more straightforward operation the problem by the way this is the effect is our two automated I don't be noticed but the problem is that you can have what's called acquired restrictive thoracic district okay so for some group of these patients who were appeared early before adolescents what would happen is the cost when you excise those causal cartilages the bony ribs with fuse immediately to the sternum or to the scar plate of the sternum and as they grow their chest when grow and they'd end up with acquired junes syndrome essentially where you have this fixed small in this case four-year-old or five-year-old rib cage and a grown person and they can have significant cardiac sorry cardiac respiratory compromised mainly restrictive lung disease and this is a patient that came to see me early after entry practice who was 30 I want to say 35 years old who had his open repair you can see his incision an inframarine incision when he was four years old and he had severe restrictive lung disease and you can see his chest is fused this all this middle portion this is actually his rib cage here and his fuse medially and he has no flexibility in his chest wall and he could barely go up flat as theirs and use them in his mid-30s he's someone I refer to an adult surgeon who had sort of made a career a made a practice or career of re-operating on these patients in adulthood with acquired research facilities from open repairs when they were young so and this is something that again Dr. Shambert or I think contributed to this field and in pushing back their repairs to when their patients are in adolescence we're just still something that we could be practiced so let's talk about the minimally invasive repair now so this was first described the injuries by Dr. Donald Nuss from a children's hospital King Daughters in Virginia in 1999 and he'd been doing these for many years and presented his series of at a p-naximity and and really changed the thing to field in some ways and in how we approach these patients and Dr. Shambert and Dr. Lilahai made the decision actually to have two sort of surgical approaches offered here and so Dr. Lilahai Dr. Moni Dr. Jassy Dr. Papadakis went and learned the minimally invasive technique so we could have one group who were doing offering minimally invasive in that Dr. Shambert continued to do the open approach and I learned how to do this operation from Dr. Lilahai and others here and and so we'll talk through briefly how that is done so the minimally invasive approach is done under general anesthesia and we typical mark three land marks the central mark is the deepest point of the funnel okay we'll talk about funnel depth with vacuum belt in a minute but basically is the deepest part on the sternum and then you mark where the anterior most portions of the ribcage are okay and the idea is that we can we make all those marks at the same level when we're done okay we're creating a suspension bridge between the two lateral marks suspending that middle one in between them that's the way it kind of thing we do that with a metal bar this place for a sternum one classically this is done actually initially without orthoscopy um you know the stories are of of you know like or not some others just doing it under palpation and looking at the EKG monitor um obviously now we do it with visualization to improve safety of this and we'll talk about a few other technical modifications that we've incorporated recently to really make this as safe as possible but number one is use of orthoscopy so we insert a thoric scope in the right chest and then use an introducer to dissect a tunnel for the input and here's sort of an overview of how that's done you want to pass you prick your tunnel between the heart and the sternum and again entering the chest immediately just the side of the sternum and then pop back out on the other side and then pass your bar now one of the technical modifications to improve safety here um that we've incorporated is sternal elevation um for all cases okay um I'll show you why but the these are this is the device that we use called um the one on the right is called the rule track that's the one that we use and you can also use a Thompson Retractor but the idea is to pre elevate the sternum to really optimize your visualization um and here's why that helped these are pictures from think my last case that are pulled but you can see here how the sternum how the view is from right floor orthoscopy the four sternal elevation you mean here's the heart here's some fat around the franics and and here's maybe some leftover diamonds but you really can't see this this thing is pushing down on the heart and the heart is way into the left chest it is very typical and so what you the classic way of doing this with the dissector is to actually kind of blindly push down that bat and this and this is just keep grinding against the sternum and hopefully you get across without pinching the pair the pericardium and the um the the life threatening complications that this have classic and because you pinch that pericardium is across and so by lifting the sternum ahead of time you have a nice much bigger window now one of the things that I've if I contribute anything is is um you know I it always kind of bound to me using this big metal thing to do with or with orcoscopic dissection that wasn't really made for orcoscopy and so when sort of doing these with with with the group um decide to put in some instruments in through the lateral incision in order to actually do a little medisthenal dissection in order to everything under visualization and carefully pull down the pericardium so no 100% the pericardium is nowhere near that sternum make that tunnel ahead of time under good orcoscopic vision and then just pass the bar across so you're not ending so there's no guesswork at all and I'll show you um and so this is a big jameer Daniel doing this dissecting here making a nice big window here so that when the bar is passed um there's no stress after the tunnel is made we pass after the the tunnel is made we pass the um dissector in the tunneler across and then exchange it for the the bar and so here's the retro throttle um uh nuss bar and once we pass it across then we flip it around and when you flip it around again what it does it pushes the sternum forward so it's suspended as a suspension bridge between those two anterior points of the ribcage and then affix it to the chest wall with um sutures typically with with um pericondrial um or uh pericostal stitches around the ribs on both sides and here's some pictures of how this fits with the uh we'll typically do a stabilizer on one side but not everyone knows but we will now one of the other things that we've been doing more recently is using more than one bar and this has to do with the patients that we're seeing with seeing more older patients and um and so patients where more rigid chest walls it's important to seriously consider putting in one bar a more than one bar to decrease the chance of bar displacement okay and it is just basic you know engineering right if you have one strut holding up the roof and that roof is heavy and fighting back that's a lot of work for that one strut to do right and so instead just like if you were lifting up something heavy you'd want to help so to have more than one bar will help distribute that force decrease the chance of one of one of those bars moving and also maybe decrease pain because you're not having as much force pushing back against the ribcage and one spot and so actually I'm one of the uh pecking meetings Dr. Nusson speaking he said you know he said I've never regreted replacing two bars um and so something we think about on our older patients especially if they're rigid chest walls and so um our post op chessics where after placement this is one with this stabilizer on the right and so I would know this is one that Dr. Jack saked it because he plays them on the right and Dr. Lula I play some on the left but um this is what it typically looks like um and here's some typical results this is one of my early cases um I mean pending and and you can see here um really nice result push it forward and little sensations on the right on the sides and um one of the things you know we talked down about ahead of time is it's not unusual to have a little bit of an over correction which actually Dr. Nuss is a big reporter enough um because what'll happen is it'll flatten out and then when you remove it it prevents recurrence but that's what these are the costo um uh conural sorry these are where the um ribs insert on the sternum here a little bit anterior and use of the cost of margins here where sometimes you can accentuate the rib flare um as well um but that's the minimum invasive repair okay so now we'll use the rest of our time talking about some of the um innovations and things that we've been trying to incorporate I mean our practice the big thing that I've had a lot of questions about is um are use of cryobletes okay so one of the things that when we um council patients were the minimum invasive repair typically was that yes smaller incisions but more pain okay and that had been our experience for a long time um is you know think a bit as um as uh you know putting your braces on and then crank them cranking them on to correct your teeth completely 100% the first day right that's what this is with the Nuss repair and so it can be a very painful and some our typical length of state was about a week and that wasn't just us that was nationwide um for the Nuss repair um that's even with with epidurals um and um now with new radjunks multi-modal pain therapy um the length of state is decreasing ours is now around three days um and I'll show you there's state of that you know we we we have reason to keep striving to improve that um because it can be done for it so the the the basic sort of technical thing that we've been doing is um using uh cryoblation okay so here's Mr. Freeze from I think the most underrated Batman movie um and um the cryo probe um is um pictured on the right here and it's um been actually the first describe use to be used in um uh uh cryoblation for atrial fibrillation um and but the same concept was then implemented uh for this and for other thoracic procedures it was first used actually an adult for economy to decrease the transphosphorycotomy syndrome um but um over the years since about 2015-2016 the first ascribans of his use in pediatric Nuss procedure um and the idea is that um by freezing the nerve all right which I'll show you pictures in the video of it causes ax axona axana mesis um which is exonal nerve damage that does not completely sever or doesn't damage the surrounding endon nerve sheet and it does this um by something called the jewel compsin effect so it's actually kind of neat it plugs right into the nitric um the the nitrous oxide supply for anesthesia um that's in the room and it uses that nitrous oxide which is the cryogen gas um of choice here um and what it does it allows it to rapidly expand in this metal tip and that expansion that rapid expansion causes cooling and then it then exhausts back out and that's important because we do this only in the rooms that have exhaust for for nitrous um but that's how it works and the idea is that it rapidly goes about negative 60 degree Celsius and that causes um the axon to be sever while preserving the epinural and endonural sheet um and it takes about two minutes per free star cycle um and I'll show you how the looks interoperatively um now this is not something that we you know created or invented where um this is actually something that we're sort of from following uh the literature um trail and there's been you know a well-to-literature in the past four or five years um on this from uh several centers so um it is something that patients are actually coming requesting um and but I'll talk about you know some of the discussions that we have with patients about the risk benefits of it um but the first descriptions were in um 2015-2016 and pediatrics and then as you can see just through these titles I'm not going to go through all these but um the initial findings were that it decreased help um the length of stay and decrease opioid requirements um and then as time has gone on people try to using it for the opener pair it's not commonly used that way but then the length of stay afterwards has gone from three days to in the last year study showing next status charge and then most recently outpatient must and it's not just the cryo all right it's it's multimodal pain therapy we'll talk about we spent a couple minutes talking about probably the um the one paper that I want to discuss as far as showing this initially um so here was the first randomized trial of of cryoanel geyser versus epidural and they showed um 10 patients and patients a decrease length of stay from mean of five days to three days just adding epidural sorry cryo instead of an epidural and with that decrease opioid use um and that's not just because of short length of stay and then in this series of all pediatric patients no short term or long term or apathy type pain in that series and it takes out a 10-hour turn in sensation for weeks um the same group actually um a release of a thought of an important paper um that we're going to try to follow up on um answering the question in the key question which has been our hesitation to incorporate this but just are you going to cause long term neuropathy in this patient I mean if you're severing the axon issues and relying on valerian you know on on regeneration of these nerves it's something you're going to go right or in that you end up with neuropathy so to answer that question they looked at their patients 43 um who had the nested cryo and of the patients who were young meaning 21 years and younger um none of them experienced neuropathy pain all right um of the pains to were older older in 21 years and these are pretty old mean age of 28 years old okay um about 23% of them well three of them but 23% had neuropathic symptoms and that was according to a subjective lead assessment of neuropathic symptoms and so we have counsel our patients who were older you know approaching 21 years older that you know the risk benefit is that they may have neuropathy and we counsel younger ones that evidence shows that probably won't happen but we can't guarantee it won't but that is part of the informed consent also interestingly those who are younger have quicker return of sensation so what's cool when they wake up and say interesting they're numb I mean they're stressed out usually it kicks in about 24 hours later the next morning um but um the younger patients had returned in about three months whereas older patients were between one had about 10 months and I don't know that's just because it's a longer distance to regenerate I assume that's why but regardless that's also that's something I understand um so the next step since um it's been so fun you're collaborating with people with um people here so uh Dr. Anjali Poka who's um when we're reading seisologists in part of the pain division here um she's been I'm helping us out with the practice program in part of it and um so we've been discussing um looking at this more objectively and so when the midst of um planning a study looking at a multimodal pain management protocol and then doing formal objective sensation measurement study using something called quantitative sensory testing so all our patients who are having cryo done or having a a nuston will be seeing her and her team preoperatively and then postoperatively um if they if they consent for the study to really measure what their um uh neurologic outcome is in an objective way so here's the nothing bolts of how we do it all right the fellow is no some of the residents know it's um uh the way we do it is as we do lung isolation that's one thing we have to add to do prior oblation to protect the lungs and the skin um the probe gets very cold and there have been reports of um delayed pneumothoruses and skin to her burn if we're not careful protecting the um the lungs of the skin um so with that we do lung isolation and then bilateral thoracoscopy so we see what we're doing and then um apply the probe three to four centimeters away from the spine you want to avoid the parasympathetic chain or the sympathetic chain um and then we'll typically do five levels in each side three through seven and um you see this work there we go so here's here's how the probe works so we will insert it through the lateral incision where the bars can be passed anyways and then apply it to the rib um to the intercoxal space you can it's important to apply pressure you really want to push it against that intercoxal bundle and you see as it starts freezing icicles start to form on it um and it's a two-minute cycle and um it'll give us a little audio um feedback on when it's met the negative six degree temperature and then once two minutes are up then there'll be another signal when it's completed the two minutes and then you have to hold it there and wait until it thaws and it'll give you another another sign when it's thawed um and you'll see the icicle disappear and that's when you want to remove it because you can remove it too soon you can actually disrupt the nerve and the nerve sheath if you're still frozen to the nerve so that's that's really the nuts and bolts of it you want to hold it on there protect everything finish your full free cycle and your 15 second thaw cycle and then release it and go to the next rib space again use the ribs breathe through seven as you get more inferiorly you have to be a little bit thoughtful and creative how you bend it thing actually is is flexible so you can get angles in here i'm also very the full two minutes and here's a view of what it looks like on the left side um again you know put a camera on the left side and uh and we do the same thing and here here we saved on and so um that's how we do finalization um i think it'd be interesting to look at you know to think about how this could be done for other things just so everyone knows it's it's only it is FDA approved for the nuts procedure in particular so anything other than that would be off label um here's final results of a typical nut is a two bar you see a little two scars on the side um and then we'll spend two minutes now just talking about a couple of the things you've been doing this is to this vacuum valve this is my son Zubin um he doesn't have to affect his ex about him but when we got the samples from Germany uh he was the perfect<|ml|><|en|> from Europe um and um this this is the same series of bells that are available um and you can see other different shapes that have been chested our nurse practitioner is really great about sizing these for our patients now but here's um uh some some some of our patients of results after repair for an appropriately selected patients and i'm sure that we've had similar results on some of our patients um this probably the the biggest study or the the the main thing that we've based our practice on is um out of King's daughters um where they um reported their experience with this and really what they found was um an excellent outcome in about 20 to 40 percent of patients um and um the best outcomes were in patients who were young under 11 years old mild defects less than 1.5 centimeters depth we still have flexible chest walls and we'll test that in the clinic we'll have them valve salveancy can you push your chest out young kids when you do this young bear it down their sternal push forward and that really um uh argues that they'll do well with the vacuum belt and then pains for compliant will wear it and we ask them to wear it two hours twice a day um and if they can do that for a year um they they they have a good chance of a correction um then the quick story about why we started doing actually an adaptation early on who um had a very mild practice but was totally destroyed by and he hated it but he was too minor to get fixed um and then you know he was disappointed went home and then his his dad called me and and happened to mention that he was a he was a an FBI agent oh boy all right i'm in trouble and he said i really need you to fix my son's chest and i go okay um and so um uh but then actually you know how to talk to him and and and then do that conversation you know so well why don't we try this up this this vacuum belt that you've heard about and so um reached out to the company in in Europe and they sent us the samples and then use of course to use it and he wasn't a typical patient he was 18 and sort of read your chest not not an ideal patient but actually had complete correction um of course he was super dedicated to get it done well out of that grew our vacuum belt practice and that which is now thankfully um our nurse practitioner group has really spearheaded this and has been driving that um so in our practice we've been using it for patients who are not surgical candidates who are halloween x less than 3.2 patients who are too young for repair but old enough to to comply patients with flexible chest walls patients who are not interested in surgery and unfortunately insurance companies don't cover it and they have to we don't it's only made by um the device we use is made in Germany um and so what we do is we recommend we give them a script and then they actually send that to the company Germany and they do it on their own but it's an out of cost out of pocket cost about $700 so there was a socioeconomic factor unfortunately so kelly was kind enough to um compile our initial results over the first year and we've had 54 patients fitted since July 2020 more more in the recent six months or so um and you can see our the ages are usually in teen years that's based on um our referral pattern we're trying actually encourage patients come a little bit younger in that like eight to eleven range that's sort of the sweet spot if if vacuum bell is is is an option um why do range of deaths but we've been doing the vacuum belt for the CACC the more minor ones you know less than three halloween x and um the preliminary results of the 25 patients we follow up on um we've had two with complete correction in the first year um 84 percent had had improvement not complete correction you can still detect there's a little bit depression there but the patients are very satisfied with the outcome and and in the first batch um but with improved funnel death and patient satisfaction um and then in three months um had an average of a half centimeter reduction in the depth of the external depression as long as they're conflying and worrying um two hours twice a day so obviously not for everyone um and we're still learning which patients going to be best suited with or not we have incorporated two sort of our list of options patients some patients really want that others are you know they're not appropriate for it um next steps are to understand or is it a durable correction doesn't involve skeletal correction or soft tissue and can we better track the progress in non-option and with that i'll spend a minute just mentioning um another research study that um uh dr. Nina Scalise who's our one of our innovation fellows is spearheading is really is is to look at other non non invasive ways to measure improvement and so what we're going to uh what um she's going to start looking at is using what's called a white light scanner as a non-reading method to measure improvement in these patients and so as i mentioned earlier you know there's CT scan x-ray there's this very imprecise depth funnel depth measurement to see how bad affected it but our own peace shop actually has a thing called a rodent white light scanner and thanks to dr. Mooney another for pointing this out and connecting us because um what this is is it's pretty cool it's a non-radiating led um camera that you just rotate around the patient and you get a nice 3d picture of the patient's chest wall um and um things about three minutes and we've been collaborating with the own peace shop who've been great with us um and we've always started scanning our first few patients as part of the study and um and you can see here sort of the typical output from the um from the report and with this then we can obtain essentially how our index measures um and subjective measurements of how the chest improves or doesn't improve with therapy and so um there's something that walls be in the pipeline and so we're going to measure this um at before after at three six and twelve months um so that's the end of our time um uh and I wanted to just really thank everyone um he's part of our group and everyone's screen who's helped make this possible to date and um and ala ed a kiela boy who's really um helped sort of get the approval for us to be a program and collecting data um and the rest of our team where I mentioned so thank you all very much for all that's terrific um you commented you just finished your fellowship but you're like uh quintuple border or something by now so it's not uh you never really go back to fellowship uh so thank you for your kind continue to commitment to our programs and this is like a perfect example of uh focus expertise and and you give a beautiful description of the history uh going going back um well before your time and um uh you know the contributions made by doctors well to shamburger really were um monumental uh in the treatment of these patients uh and the transformation that's happened over time which um Dr shamburger supported the entire time of moving towards the minimally invasive approach while uh maintaining Boston Children's is one of the only places with significant expertise in the open management of some of the more complex and asymmetric deformities which is still necessary and that's a um that's a skill set that's being lost internationally as the minima invasive techniques have taken over her ponderance of care but but a focus program like um you and the others are building will allow those um very specific techniques to remain available for those uh percent of the patients that will need them and like we see in all of our other such specialty programs um when you do where things commonly you become you become the experts and the patients will come uh from far and wide so thank you for for carrying the mantle and uh um yeah you show the pictures of the um people senior to you who taught you how to do this that you've taken on leadership of the program and that that's the way the transformation into the department happens and you also gave appropriate credit to those who are actually doing the work um and there's practitioners, coordinators um Kayla etc so thank thank you for that. I'm sure this time I have a bunch of questions but I'm opening up to others to ask questions or thank comments you Dr. Sanford. Well I just want to commend you for the great way you've seen this opportunity to push the management of kids with just all of my affirmations forward I applaud you and I think fact and bell is a is a great addition uh in your armamentarium that you can offer patients either have mild depressions which we've struggled obtaining insurance coverage for any sort of intervention and also for the younger kids and I think that suggests it is where you're going to get the most improvement and I think through the years I've seen the pediatricians continue to refer the kids when they're pretty young so I think you'll have a good population or cohort of the children and what you can utilize the the back of bell and hopefully avoid any required for surgical intervention so so great job and as Dr. Fisherman says getting a cohort together and starting a program to address a problem is the way to make the biggest impact on kids with that problem so great work thank you Dr. Sanford other comments or questions if I really enjoyed the talk and learned a lot I'm wondering in what context are you seeing referrals for repair failures and reoperative cases and what perhaps it's a case by case thing but what's your general approach great question um to be honest have have seen few of those um mentioned one and I had this had some who are failed or complications from open repair elsewhere and I think that comes from Dr. Lambert Dr. Lilaheiz referral referral base um and those are complex um where I had a couple with for the retained hardware after um sort of misadventures at other places um and um you know in the handful that that have had a very low threshold to incorporate to actually involve other expertise particularly cardiac surgery and orthopedic surgery um because those are very complex situations where we're talking about potentially a reop sternotomy or or a retro-stermal approach um have not had at least so far um failed you know nusses from elsewhere we're doing a reop nuss um there are other centers that have reported series of that um and those are the ones that are notoriously you know have the higher rate of of misadventures you know cardiac injury particularly because the hardest part um but um that's a great question and and I think as as as we as this grows you may see more of those patients but right now it's been just a handful well nobody else is gonna pop in on a few questions um everybody has seen and can remember the extreme pain that these patients have postoperively where they have an open repair or a mineral we have repaired some of the most intense pain we see in pediatric surgery on a routine basis and and you demonstrated uh significant improvement with techniques and we had used epidurals for a long time and as you pointed out that wasn't really enough and required to stay in the hospital you mentioned with the older patients the um neuropathic pain that develops in a small percentage um after co-opulation um and there's neuropathic pain and there's neuropathic pain yeah are they you know short lasting sort of minor um sort of numbness itching you know or are these people really um in misery great question and I think that's uh you know as you know as you said pain is a complex beast so in that in that series which is really the only one out there really specifically looking at this neuropathic pain question the three patients who had who are older who had neuropathic pain on that assessment two of them were mild um where they didn't need any particular intervention both and all three of them were treated with gabapentin and two of them got better with it but there was one patient who was getting you know um injections for chronic pain afterwards um so so whether that was due to the cryo or not I think you can't didn't die I mean it started after um so um it does give pause um is one patient who's been reported but um regardless I think it's something to um to to think about the vast majority though other than that one that I found a larger um uh it's been very mild um but enough that at least on the assessment of their report and the typical thing is tingling sensation and then with gabapentin it's gotten better some who started using cryo um start profilically treating every patient gabapentin pre-op and then keeping a post-op we decided not to do that um mainly because of the side effects of gabapentin and then you know again speaking to other centers who would do you for longer many have moved away from that because of side effects it turns out that if you keep in your back pocket for the rare patient who might need it then at least you have that as a therapy you can imagine that might come up let's a quick question sure if I guess presumably if they did have a long lasting severe neuropathic pain you could treat that because it's peripheral you could treat that with nerve ablation if you had to would that's exactly right I mean in fact the first I meant the treatment this the correlation was first used for neuropathic pain right um but um yeah and you mentioned the the the the socioeconomic impact of the um payers not covering uh a valve um that's a one-time expense or order to change size to bells or a time of treatment well good good question we we haven't had any patients have had to change size um uh generally I mean depending on when they're intervened on we say they might need to when they're older but it's usually been a one-time thing because their correction happens pretty quickly within you know within three to six months at most a year if it were to happen at all um but yeah since typically one time it's okay but the insurance companies do pay for the visits correct and then okay so let's talk an offline we can probably work out a charitable way of dealing with that I don't want those patients not have access to that so thank you yeah I mean in general one thing of you know in chat with chess wall in general I think another interesting question which has really been explored is you know the notice or the referral base tends to be um those of with means um and we loved one day try to understand are there patients who are not of means who are not being even referred for the transfer on you bet I noticed where a time Jamie I want to thank yeah for your um tacking this this this program I know that um Dr. Schemberger was was thrilled when he recruited you to to take on his his leadership and and pass on the torch and and the fact that you've been amongst all the other things you're doing willing to take us on and to drive forward to new programs and show how you can build a team with with others is really spectacular so thanks for your great work we'll look forward to an update in a couple years thank you all and particularly I want to mention thank you Dr. Lilla Hy who's been holding my hand through all this um so thank you Dr. Lilla have a good day everyone