VEPTR COMPLEX TITANIUM RIB PROCEDURE ON A THREE-YEAR-OLD CHILDREN S HOSPITAL, BOSTON, MA Broadcast November 10, :00: NARRATOR: Welcome

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1 VEPTR COMPLEX TITANIUM RIB PROCEDURE ON A THREE-YEAR-OLD CHILDREN S HOSPITAL, BOSTON, MA Broadcast November 10, :00: NARRATOR: Welcome to the live complete titanium rib procedure from Children s Hospital, Boston. Today s webcast will feature surgeons implanting a vertical expandable prosthetic titanium rib, or VEPTR, in a 3-year-old patient. Dr. John B. Emans, Director of the Division of Spinal Surgery at Children s Hospital Boston and Professor of Orthopedic Surgery at Harvard Medical School, will perform the surgery. The VEPTR device is used for the treatment of thoracic insufficiency syndrome, or TIS, and addresses both chest wall and spine deformity directly, while accommodating growth. You can send your questions to the OR at any time by clicking the MDirectAccess button on the screen. 00:01: JAMES KASSER MD: Good morning. My name is Dr. Kasser and I m the Chairman of Orthopedic Surgery at Children s Hospital in Boston. This morning we ll be demonstrating the titanium rib procedure that will be done by Dr. John Emans. Prior to the procedure, Dan Hedequist, a member of our department specializing in spine surgery, will be describing the procedure. This procedure was developed by Dr. Robert Campbell in San Antonio, Texas. Over the last 4 years, we ve done 40 of these procedures with great results. It s our goal to have you understand the indications and the techniques involved in this procedure. Dr. Hedequist? 00:01: DANIEL HEDEQUIST MD: Thanks, Dr. Kasser. Good morning. Dr. Emans and his team is, I think, almost ready for us in the operating room. We wanted to talk a little bit about the VEPTR device and a little bit about our patient before we get to the operating room in order to get the audience better oriented. So we ll talk about VEPTR as well as titanium rib. They re the same device. It stands for vertical expandable prosthetic titanium rib. You also hear the word being used today, expansion thoracostomy, and that is used along with VEPTR. That s a way that we can open the chest wall, expand the hemithorax of the chest, and allow for growth of the chest and growth of the lung. The VEPTR device was developed by Dr. Robert Campbell in San Antonio, through the SyntheSpine Corporation. On this slide you see it shows the VEPTR device and I m going to have Nick, our cameraman, pan in to me here. This is a spinal model. You can see the rib fusions that would be commonly used for a VEPTR device. This would be what s called a rib to rib device. There s a section which goes on a rib above and on a rib below, and this is a device which can expand over time and allow for growth of the chest wall and control of the spinal deformity. VEPTRs were developed for children with early onset spinal deformity. The problems with children with early onset spinal deformity is, with continued growth, they have continued curvature of the spine and inability of the thorax to grow and support normal lung growth. We call this thoracic insufficiency syndrome. In the past, thoracic insufficiency syndrome has been treated with early spinal arthrodesis when VEPTR hadn t been developed. The problem with early spinal arthrodesis in young children is as the spine continues to grow, we get chest growth. If you do a spine fusion on a young child, they no longer get growth over that area of the spine, no longer get growth of that side of the chest wall, leading to restrictive lung disease. This has been well studied at our institution and subsequently Dr. Campbell took information such as that and developed this procedure in order to control spine deformity in young children in order to allow lung growth and spinal growth at the same time. 00:04: So the VEPTR controls spine growth, controls chest growth, and allows expansion of their thoracic cavity. The indications for VEPTR that you ll see today, it s best seen for congenital spine deformities associated with multiple rib fusions. The goals of VEPTR surgery are to maximize spine growth and allow for maximal chest growth. This is

2 an example of a VEPTR device. The x-ray on the left side of the screen shows significant congenital scoliosis with rib fusions and a small left chest. After insertion of the VEPTR device, you can see the arrow showing expansion of the chest wall, allowing for chest growth and improvement in pulmonary function. Now, this is an example of a CT scan, both before the VEPTR device and after the VEPTR device. On the top right slide, we show the lung volume on the right side of a constricted hemithorax and a small lung. After expansion thoracostomy and VEPTR placement, you can see the increased growth of the right lung, leading to improvement in pulmonary function of the patient. 00:05: I want to talk a little bit about our patient today before we get to Dr. Emans. She is a 3-year-old patient, otherwise reasonably healthy. She has some kidney anomalies, which is common with congenital scoliosis. However, her cognitive function is normal and physically she runs around and plays without any problems. However, she has a significant spinal deformity. This was noted at 15o at birth and has progressed over the last 3 years to a 55o curvature. Right now, she has no respiratory problems, but our worry is as she continues to grow, her spine will continue to curve, leading to significant restriction in her right chest and resultant lung function problems. This is a CT scan, a 3-dimensional model showing the multiple congenital anomalies which she has. She also has, more importantly, multiple rib fusions. In patients with multiple rib fusions and congenital scoliosis, these can act as a tether for both chest growth and spinal growth, leading to spinal curvature, and thus our patient has developed a 55o curve through age 3. So the goal of the operation today is to expand her chest wall, allow for continued growth of her spine and her chest wall, and prevent an early fusion which would lead to restrictive lung disease. So our goal of the operation is shown schematically here. The slide on the left would show multiple rib fusions and a small left hemithorax. If you do expansion thoracostomy, which is opening the chest wall, placing a VEPTR device, allowing for expansion of the left side of the chest, improved lung growth, and control of the spinal deformity at the same time. So our goal with VEPTR insertion and expansion thoracostomy today is control of the spinal deformity, but more importantly, control of her lung growth and improvement of her lung function over time, which would be inhibited by an early spinal arthrodesis. 00:07: So, Dr. Emans, we re going to flip to you. I have a slide up showing that the patient is in the lateral decubitus position. If you could just orient the audience to where you re at and go ahead with the procedure. Thank you. 00:07: JOHN EMANS MD: Good morning. Thank you for coming. We ve gotten a little bit of a head start here, but I think it would be worth orienting everyone. We re lucky to have a good team here today. We have Eileen Coyle, a very experienced scrub nurse; Travis Metheny, our fellow in pediatric orthopedic surgery; Dr. McCann; and her entire anesthesia team. 00:07: The patient, if we can pan back here so that we can see the entire patient, the patient is resting in the lateral decubitus position. We start out with the patient in a lateral position. We have pelvis down here. Here s the scapula, marked up here. These lines going down here are the line of the spine. We ve done a modified thoracotomy skin incision here. The goal of placement of the skin incision is to allow us to get at the rib fusions and to get at the thoracic constriction, but also allow us subsequently to use this same incision for our subsequent every 6 month lengthenings. If we were to do a rib to spine device, we d make a separate skin incision down here on the spine. So what we ve done thus far is we ve made the skin incision and then we ve made an incision in the latissimus. I don t know whether you can see it on this young patient s original clinical photograph, but she s very deficient in her latissimus dorsi muscle and deficient in her rhomboids. So we ve incised and marked the latissimus distally. We ve tried to do so a little bit farther down than the skin incision so we have a fairly long muscle flap and a regular sized skin flap. There were no rhomboid muscles. She is missing her rhomboid muscles, so we didn t have to take down the rhomboid muscles, but normally you d take those down and mark them. Then we ve taken great care to take the paraspinals and to reflect the paraspinals posteriorly, extraperiosteally, until we get to the point where we can actually feel the transverse processes. The paraspinal muscles are then used as a muscular cover to cover over the device at a later time. So then we re pretty well set here with our exposure. You can look up into here. We have enough view up in here so that we can feel all the way up to the first rib and count ribs. We can see the scalenes coming in here. Her scalenes are underdeveloped. As Dr. Hederquist mentioned, she has some right upper extremity anomalies. We can see her

3 scalenes here and we ve again pulled back far enough to get to the tips of the transverse processes, although there is no spine exposure, per se, in this procedure. So we have a reasonable view to go with here. 00:10: Now, before we ve done all this, the patient has had SSEP and motor evoked potential monitoring established. This patient has upper extremity anomalies and we re actually not able to get upper extremity motors and upper extremity sensories, so we re going to be careful to make sure that at the close of the procedure, the patient still has upper extremity motion, since there not rarely have been brachial plexus palsies following this procedure. We have tried to identify where we are relative to the plan Dr. Hedequist showed you on the 3D CT. We had planned to go to the equivalent of the 10th rib, but upon opening it here, we feel that the 10th rib is really too mobile. If we push down on the 10th rib, it s a little bit too floating, so we ve changed the plan to go up one rib farther than we talked about. We re going to go up to the rib that would be the equivalent of 9 for our lowest anchor point, because it s more stable. Then, up above here, the anatomy is quite confusing, but we re going to end up putting the superior cradle on the equivalent of the 4th rib. We like to do thoracostomies in the most constricted area of the chest and we ve chosen these two partially congenitally fused intervals. We saw on the 3-dimensional CT that this entire area is quite constricted, so I think the first thing we re going to do is go ahead and do the thoracostomy. 00:11: JAMES KASSER MD: John, can you tell me how you pick the bottom rib, where the fusion is and then how you pick the bottom rib, where you re going to put your hook? 00:11: JOHN EMANS MD: I ll tell you what, how about if I just do it here and Dr. Hedequist tells you that? 00:11: JAMES KASSER MD: Sure. That would be good. 00:11: JOHN EMANS MD: We re going to find a little separation here in the cleft of these ribs and then we re going to proceed again with the Bovey to try to find this interval. We ve cheated here a little bit and gone ahead and marked where we think we should be here, but the position for the lowest distraction device for the rib cradle needs to be on a place that is secure and strong, but still allow some correction relative to the spine. So we re trying to separate this cleft between the partially congenitally fused ribs and I think we re being fortunate here in that this is partially open. It s not totally fused. Dr. Hedequist, do you want to talk about how to choose levels? 00:12: DANIEL HEDEQUIST MD: I think superiorly, as far as where we want to put the rib cradle, we d always like to get as high as possible. Usually for the device proximally, we re going to choose the second rib, if possible. If you go higher than the second rib, that s fairly high in the chest and even near your neck area, where we worry about impingement on the brachial plexus and even the brachial artery. Placement of a device below that, the inferior rib cradle, we would want to find a rib that had enough size in order to handle the device, as well as enough mobility in order to distract against this. I m trying to put a slide up here which shows us a little bit about how we have chosen our levels for a thoracostomy here. If you could talk a little bit, Dr. Emans, about any pitfalls as far as medial dissection during thoracostomies and any trouble you can get in with your medial dissection. 00:14: JOHN EMANS MD: Well, we emphasize making sure that the medial dissection to separate the fused ribs goes far enough to truly make them separate, but if you go too far medially, there have been instances of encountering the segmental vessels and there may be, just as in this girl, with her multiple hemivertebrae, there are segmental defects in the spine also and one can enter a foramen without intending to. 00:14: DANIEL HEDEQUIST MD: Dr. Emans, have you decided to place two opening wedge thoracostomies or one, or is that something you re going to decide intraoperatively? 00:14: JOHN EMANS MD: We re going to do two. Probably you should not do any more than three. The limiting factor is that if you do too many, you end up with ribs that are not necessarily viable. 00:15: DANIEL HEDEQUIST MD: Have you had any problems with creating a large area of rib bed which is devascularized and causing problems afterwards?

4 00:15: JOHN EMANS MD: I ve not seen that as a problem, but I know it has occurred. I m fortunate to have had Dr. Campbell s pre-experience to help keep us out of trouble. We ve been doing this now since, I think, 1999, so we ve had a reasonable amount of experience. We re just separating down here more and more toward the medial part of the rib and we ll try to now push these apart with a lamina spreader and try to make this thoracostomy complete. You can, if you wish, do this and leave the pleura intact. It often ends up being simpler and better to simply incise the pleura, rather than to try to preserve the pleura. If the pleural defect is quite large, then it is best to reconstruct the pleura, but if it s a moderate pleural defect and the lung is not emerging from it or a corner of a lobe is not getting caught, then it s probably reasonable to leave it without reconstruction. 00:17: DANIEL HEDEQUIST MD: Dr. Emans, I have multiple questions, but two people have ed the same question. It has to do with the risk of paralysis with stretching the chest versus stretching the spine. Have you found a lower incidence with expansion thoracostomy versus distraction of the spine itself? 00:17: JOHN EMANS MD: Well, the standard VEPTR titanium rib procedure does not include any spine operation. It s very tempting to talk about excising hemivertebrae or doing rib osteotomies or some other relatively invasive procedure for the spine. The danger of that is that this is such a powerful procedure that you can overstretch the spine. I know that there have been a couple instances oföexcuse me, let me just digress here for a second. I think it has kind of proved to be easier to just incise the pleura here. 00:18: DANIEL HEDEQUIST MD: Dr. Emans, when you need to excise the pleura, does that change the postoperative management with chest tubes in a child? 00:18: JOHN EMANS MD: No. Either way, you need a chest tube because there will be the potential for a continued hemothorax. So the rib osteotomy and the rib separation needs to go far enough forward so that you can ensure that it will open up anteriorly. So I don t know whether the camera shows that well enough, but I ve gone quite far forward here so that I can feel that it is all the way out to the costochondral junction, so it s plenty far forward and free forward. Then we ll go work on the back part here. Knock on wood, we ve not had any neurologic issues with this procedure, vis a vis the spine. We had one significant brachial plexus palsy associated with a large correction. Since that time, we are very careful to monitor the upper extremities during the procedure and we re very aware of the whole possibility of a brachial plexus palsy. In this child, with an already abnormal upper extremity, that would be my concern, that without recognizing it, we can get a brachial plexus palsy. So here we re following down. 00:20: DANIEL HEDEQUIST MD: Dr. Emans, can you orient the viewer to how close you are to the spine itself? 00:20: JOHN EMANS MD: The spinous processes are here at my fingertip. The transverse processes are here at my fingertip. So we are right there. We re right down at the rib heads and right down at the costotransverse articulation. This needs to be separated enough so that there can be a lot of correction, but not so much that we avulse the ribs. I m pushing pretty hard on the ribs. Now, if Travis looks down here, he can see the vertebral bodies. I don t know whether the camera can, but he s looking just as he would with an anterior approach. See anything interesting, Travis? 00:21: TRAVIS METHENY: No, but you can definitely see the vertebral bodies. 00:21: JOHN EMANS MD: So we ll just follow this down to the level of the rib head and make sure that it is quite free. So this expansion thoracostomy now opens up quite easily and I think that s sufficient for this. 00:21: DANIEL HEDEQUIST MD: Dr Emans, intraoperatively, how do you decide if you need to do another expansion or what you ve done is sufficient? 00:21:54.000

5 JOHN EMANS MD: Well, I think that most of that is probably decided preoperatively. We could look at her constricted chest and see that we needed a fair amount of expansion of this hemithorax, so we planned ahead of time two expansion thoracostomies. 00:22: JAMES KASSER MD: Dan, do you have the preoperative imaging that you could show these rib fusions that John is taking down? 00:22: DANIEL HEDEQUIST MD: Yes, I believe there s a slide up. The dotted lines there should show the locations of the planned thoracostomies. 00:22: JOHN EMANS MD: This is two fused ribs above where the last entry point was. We ll see if we can find the remnant of a vertebral space here. On the CT, it looks as though these levels are completely fused. I don t know whether the camera shows that there is actually a little bit of a cleft here that we re following. The goal of the way that the rib separations are made is to try to make it so that you get an appropriate amount of expansion, but not so much that the intervening rib is nonviable. 00:23: DANIEL HEDEQUIST MD: Dr. Emans, if you re unable to find the cleft, will you continue the work at the same level or will you go a level above or below to try to find an area to get into? 00:23: JOHN EMANS MD: No, you can create a cleft. If there should have been a cleft there, you just burr down through the bone. We re lucky here in that there s a cleft here that you see we can tease apart. What s interesting also is that the interior of the periosteum and pleura is very thick here, so this is an anomalous condition, but it s almost as if the anomaly is anomalous scar. Along with the rib fusions goes, of course, other anomalies. As we ve seen, the latissimus dorsi is largely absent here. 00:24: DANIEL HEDEQUIST MD: Is it your sense if there s no rib fusions in these children, even the area between two ribs that s not fused or constricted, and there s scar tissue? 00:24: JOHN EMANS MD: Yes. The tissue that I ve just gone through is much denser than a normal pleura plus periosteum. 00:24: DANIEL HEDEQUIST MD: So, for the audience, this would be an area between two ribs. He s not cutting bone itself right now. He s cutting through scar tissue. 00:25: JOHN EMANS MD: This shows up as a fusion on the CT, but actually it s really not a fusion. 00:25: DANIEL HEDEQUIST MD: Dr. Emans, I have an question regarding the viewer notices it s quite an operation on the chest wall itself. Can you talk a little bit about the pulmonary workup of the patients in the department and their role in the preoperative evaluation? 00:25: JOHN EMANS MD: Well, we and all the other centers that do this try very hard to have a multidisciplinary team. It usually takes a generalist interested in children with special needs to sort through many of the issues. It sometimes takes a thoracic surgeon and it always takes the involvement of a pulmonologist to help us decide the issues involved. Some of the issues that are to be decided include when to operate. In other words, should you wait until the child is substantially bigger or when should you do this? The earlier you do it, the better chance you give the lungs to develop. The longer you wait, the larger the child gets and, in that sense, the easier the operation gets. So now we ve gotten a little bit anterior to the most constricted part of the chest here. This part of the chest is now opening up rather nicely without too much effort here. This is a modified Cloward retractor that s used to help spread the chest wall. It helps spread the expansion thoracostomy. We don t want to devitalize this intervening rib segment, but we don t want this to also still be stuck together in front and I think we are now free enough that the effect of the expansion thoracostomy goes all the way to the costochondral junction in front. Let s look in the back here, make sure that this is open also, and then we ll put in some devices. 00:28:02.000

6 DANIEL HEDEQUIST MD: Dr. Emans, I have another question from the viewers. Will the original device have to be replaced? I know we haven t put the device in yet, but also during that time, will you have to repeat the expansion thoracostomy? 00:28: JOHN EMANS MD: Well, in general, the plan is to do repeatödan, you asked where we are. My finger is on the tip of the transverse process, so we are all the way to the costovertebral junction anteriorly here. Travis can look down and see that, or he could put his finger down there and feel the anterior vertebral bodies. We want this to be free, but not detached. So you asked about taking the devices out? I m sorry, what did you ask? 00:28: DANIEL HEDEQUIST MD: We had a question of will the original device have to be replaced? More importantly, during replacement of the device, do they have to have the chest wall expanded again through thoracostomies or just through lengthening of the device itself? 00:29: JOHN EMANS MD: Well, the plan would be to continue to expand the existing thoracostomies. We re going to put in a rib to rib device, but we may eventually switch that after a few years to a rib to spine device. Right now, a rib to spine device would be pretty prominent in this child because it s young and I think we can actually control it without a rib to spine device. The larger the device is to start with, the more length you get, so if this is a #6, and we re going to do well to get this in, this has got perhaps cm of expansion and then you re out of length and have to take out the center portion of the device and replace it with a longer device. You can keep doing that without having to do the expansion thoracostomy. Now, we have 3 of our 40 patients whose ribs have re-fused and stuck together, and 2 of those 3 have already had a repeat procedure in which the ribs are separated again to allow continued expansion. So the answer to your question is it s not our plan to have to do repeat expansion thoracostomies, but it has certainly occurred. 00:30: DANIEL HEDEQUIST MD: Looking back at those patients, is there any way to avoid that? 00:30: JOHN EMANS MD: Well, when we ve had to make actual bone cuts, we do a lot of coating of the bone with bone wax to try to make it as non-osteogenic as possible so that the fragments won t grow back together. So what I m going to do now is I m going to try to put the superior cradle in. In our original plan, we had planned to be up one notch farther than we are here. I m on the first rib here, so there s first, the equivalent of second, and then this is kind of a combined third and fourth rib. We re going to try to go aroundöwe re going to go underneath this, actually, in our expansion thoracostomy and over the top of the next rib up, so we re going to have kind of 3 and 4 in a claw type grip, I hope. So let s take a superior rib cradle. This is the device that rests up against the rib and there s no problem putting it in. That s an easy placement. When you don t have that, as we ll see down here, when you don t have an actual expansion thoracostomy to put it in, you make a small incision between the ribs and thread it in. The goal of that is to go extraperiosteally so that it doesn t choke off the nutrition to the rib. 00:32: DANIEL HEDEQUIST MD: Dr. Emans, in smaller patients, are the ribs too small and in that case do you put one device around two ribs? 00:32: JOHN EMANS MD: Well, you have the option to encircle more than one rib. I m going to go above this rib right here with a small incision. Then we re going to just dissect down over the top of the rib here and between pleura and rib so that there will be a little bit of an opening here. 00:32: DANIEL HEDEQUIST MD: I m not sure if you mentioned it, but one thing you ve always stressed is to be as medial as possible to the spine, is that correct? 00:32: JOHN EMANS MD: That is correct. 00:32: DANIEL HEDEQUIST MD: Is that for a mechanical advantage? 00:32: JOHN EMANS MD: That s for a mechanical advantage and for safety. That is actually the second rib right there, so if we do that, we re going to be quite cephalad, probably a little more cephalad than we d like to be, so let s

7 instead put it into this cleft here. Although we ve done a wonderful job of planning out exactly what we re going to do preoperatively, like so much surgery, the plan can change. So our superior cradle end half is going to go in this little cleft. This is quite simply encircling this rib, so that s our upper rib. We re trying to be just as medial as we can. The tip of the transverse process is right there. 00:33: DANIEL HEDEQUIST MD: Dr. Emans, the superior rib cradle and inferior rib cradles, are there size differences? Does one size fit all? 00:34: JOHN EMANS MD: There are size differences and there are a number of size options. You can see that this device is relatively large compared to her overall size. 00:34: DANIEL HEDEQUIST MD: There s been an regarding what is the youngest patient you ve performed this operation on? 00:34: JOHN EMANS MD: The FDA has approved the device for children over six months of age and the youngest I ve done, I think, is nine months. It has a lot to do with the size of the patient also. If the patient s much too small, it s simply not doable. On the other hand, the children who are not growing well and not doing well are the children you d also like to have a device in quite quickly so that they can begin to grow. So now we need to mate these together, something like this, so it s up against the rib here. Then Travis will come in there and then tap that in and that s the cradle lock. This is our upper attachment up here. Once that s in, we can go ahead and feel comfortable about trying to stretch out and expand for the expansion thoracostomy. 00:35: DANIEL HEDEQUIST MD: Dr. Emans, I have a model here with the VEPTR. If they could just flash to this real quick, where he is in the procedure is this is superior. This would be the head of the patient. This would be down low. These are the medial ribs and this is the cradle that he s talking about. It encircles a rib and is locked into place. So that s the part of the procedure, in case someone showed up late or if having a little bit of difficulty orienting, so we can probably flash back to Dr. Emans. Dr. Emans, could you quickly orient people again to what s medial, lateral, head and spine? 00:36: JOHN EMANS MD: Sure. Head is here. Spine is here. Scapula is here. Superior cradle is here. Inferior cradle is right down here. Now we re going to do the same thing as we did up above, but we re going to do it in a reversed fashion. I have my finger right here on this little stitch on the transverse process, corresponding to this rib, and this rib is about a 9th rib equivalent. We reach around this and make sure that the pleura and the lung are out of the way. 00:37: DANIEL HEDEQUIST MD: I ve put a slide up, Dr. Emans. Your option, if your inferior rib either isn t strong enough or is too small, would be to convert this or just go to a rib to spine device. Would that be correct? 00:37: JOHN EMANS MD: That would be right. If your upper rib is not strong enough and doesn t hold up to this, then there are fewer options. There s not a good sort of option available for that. So we re going to encircle this one rib. Now the question is what size device can we get in here? We d like to have as much expansion as possible. There are a couple of motivations behind that. We d like to have a device that is as long as possible so we can get as many lengthenings as possible, so it has as much lengthening potential. We d also like her to correct as much the first time around, since we seem to do best with maximum correction the first time around. 00:39: DANIEL HEDEQUIST MD: Dr. Emans, there s a question about nerves being attached to the ribs or neurovascular bundles being underneath the ribs. How do you avoid prolonged chest wall pain or neuralgias secondary to the device? Is that something that s been a problem? 00:39: JOHN EMANS MD: It just doesn t seem to be an issue. There s a little offset built into the superior cradle, so if you see the device here, there are little prongs that are intended to contact the rib. If that is the case, it ll tend to keep the cradle offset from the rib, so if this is your typical rib cross sectionöremember, on the inferior edge of the rib, there s a little bit of an indent where the bundle lives. So it goes like that and pretty much keeps it away

8 from the neurovascular bundle. Now, with time these tend to drift through the ribs. I think during that time, the neurovascular bundle probably has to adjust to a new position. What we don t get, though, is we don t get complaints from kids about their chest being numb or neurovascular complaints. So there are a couple of different ways to get the device in at this stage. None of them are easy because you d like to have the longest device possible. What Eileen has here is the rib sleeve. It s the source of the expansion length and we re going to put it on here and then, if we re really good and really lucky, and I m not sure we re going to be this long, we can get that to go in. I m just not sure we re going to be able to do that. 00:42: DANIEL HEDEQUIST MD: Dr. Emans, I was going to show the model again to reorient people where you are. He has a superior rib cradle in. He has an inferior rib cradle in. He s putting in the sleeve now. The sleeve, once attached, will allow expansion of the chest and then they can lock it into place. So we have a cradle in the top, cradle in the bottom, and right now he s trying to put the sleeve in, so we can flash back to Dr. Emans again. 00:42: JOHN EMANS MD: If you overdo this, I think you can result in some significant trouble. Let s see if we get a little time and a little stretch here, if we can get it to go on. 00:42: DANIEL HEDEQUIST MD: Have you used any temporary distraction devices? 00:42: JOHN EMANS MD: Yes. Travis has the Cloward there that s doing the temporary distraction. So we ll just let it stretch here for a minute and hope that it goes in. 00:43: DANIEL HEDEQUIST MD: We have an , Dr. Emans, regarding is there an option to put the rib to pelvis, which I m going to put a slide up that shows that. Can you give a quick indication of when you would want to go to the pelvis, rather than into the lumbar spine or into the rib itself? I know that s a long question. 00:43: JOHN EMANS MD: The use of the pelvic device is often in neuromuscular children with a collapsing defect or in children who don t have any bone in the lower spine, like a spina bifida patient, or in a big long curve. For most ambulatory, healthy children, it s probably better to end up in the spine than in the pelvis because the pelvis devices, I think, tend to drift and loosen with time. So, Dan, I think we ve been lucky here. We got in a fairly big device, namely a 6, and you can see that this keeps it reasonably well expanded. It s reasonably secure and will probably expand. 00:44: DANIEL HEDEQUIST MD: Dr. Emans, how do you decide when too much expansion is too much and when to stop intraoperatively? 00:44: JOHN EMANS MD: That s a good question, Dan. You tell me. We re much bigger and stronger than this child, so I m sure we can keep spreading if we really wanted to, but this is under a little bit of tension and we probably should settle for about like this and then quit there. 00:45: DANIEL HEDEQUIST MD: Do you use x-ray in the operating room? Is it experience? 00:45: JOHN EMANS MD: Before we leave, we ll get an x-ray, but I think here we re going to go just by feel. Now, when it comes time to distract this and do the periodic lengthening, the way this would be lengthened is to make a small incision over this, then take the distraction device, put the distraction device in, and spread and elongate the device. There s another distraction device that you can use, so that distraction device also goes in, spreads, and distracts. 00:46: DANIEL HEDEQUIST MD: I ve put up a slide which shows the small incision for the lengthenings. Sounds like a lengthening every 4-9 months. In general, is that a day surgery procedure, an overnight procedure? 00:46: JOHN EMANS MD: For healthy children, it could be a day surgery procedure. This is a relatively uncommon procedure, so many of our patients come from far away and the prospect of day surgery just doesn t work. 00:46:44.000

9 DANIEL HEDEQUIST MD: Dr. Emans, I m going to have them flash back to the spine model again here. So where we re at, the superior rib cradle is in. The inferior rib cradle is in. The sleeve is in. He s expanded this and then a lock will be placed in order to hold the chest expanded. If you don t have a lock, the device obviously will collapse back, so he will do his expansion, like that. Oops, I just broke the ribs, John, and then he ll lock that. We should go back to the webcast, Dr. Emans. 00:47: JOHN EMANS MD: Better on your spine model. 00:47: DANIEL HEDEQUIST MD: I was going to say, I m happy that happened on the model, rather than the patient. 00:47: JOHN EMANS MD: It s a good question though, have there been rib fractures? There have been rib fractures. We have had 3 episodes of rib fracture. Two of them were recognized around the time of lengthening and it wasn t a problem. We just chose a different rib. In one of them, it was a major problem and still is a problem because it was the last fixation that we had available. In this child also, there s really not much else of a place to go to up here if we avulse or break these ribs, so I m being not over-ambitious here. I think in fact we re going to end up getting like 1 cm here, extra. Dan, can you see that distracting? Do you see how this is working? We re not distracted here. Now we re distracted. You can see it begin to spread the thoracostomies apart farther. So that would be what happens during your periodic lengthenings, is to go like that. We have a pleurotomy, pleural incision, here and here. You can see that the lung is behaving itself. It s staying inside, so we re not going to need to do a pleural reconstruction. If there were a corner of the lingula coming out and bothering us, we could instead reconstruct the pleura here with a bioabsorbable membrane. That s nice, I suppose, because it keeps more of a barrier between her muscles and the lung, but it s also more foreign body and it s not clear to me whether that makes more scar or less scar to put in the pleural reconstruction. So unless there s a clear indication for it, like this gap is more than, say, 2.5 or 3 cm, or there s a little corner of lung coming out, we don t feel compelled to do that. So I ve been just gently stretching here a little bit. I could stretch much harder, or if I really wanted a hard stretch, I could ask Travis, who s really strong, but I don t want to do that. I don t want to have anything come apart here, so I think we will settle for this as our final length. So this distraction lock is not settled into its hole properly. Now it is. 00:50: DANIEL HEDEQUIST MD: Dr. Emans, for families out there, would device failure be a common or relatively uncommon thing? 00:50: JOHN EMANS MD: There have been some device breakages in the lumbar extensions. Maybe you could show it on your thing what a lumbar extension looks like. To my knowledge, there s never been, in all of Dr. Campbell s experience and the group in general, there s never been a failure of the device itself, so the device is very strong. The problems that are encountered are the issues associated with repetitive surgery, scarring associated with this, and that kind of difficulty, not the device, per se. Now, this is pretty secure. This is strong enough for me to take and put the patient where I wish to put the patient. It ll rock a little bit, but it s not about to fall out of place. It s not about to come out of place. So it s quite secure. If this intervening rib were anteriorly placed, you could also do a procedure in which you take and pull this rib up toward the device and sew it into place on the device. That s a whole separate operation for hypoplastic thorax, in which you cut through the ribs, bring the whole ribs out as a section, out to a bowed device. Dr. Campbell is done a great number of those for Jeune syndrome and other hypoplastic thoraces. So we re going to basically be finished here. I m going to run a chest tube. 00:52: DANIEL HEDEQUIST MD: Can you tell the audience a little bit about what the expected course would be in the hospital for the patient and then maybe over the next year or two, lengthenings and device exchange? 00:52: JOHN EMANS MD: You see that the lung is actually smaller than the chest, so when the anesthesiologist gives a big breath, the lung fills up to the chest, but the lung when we came occupied a much smaller place. Now it has a bigger chest to breath in, so it tends to fall away. Over time, it will accommodate to the new position. Next we ll put in a chest tube, but the next real issue here is getting this closed. That sounds silly, but it isn t so silly because suddenly we ve made the chest much bigger and you can see that it doesn t easily pull down to close, so we re going to have to work at pulling her muscles down a little big. We ll pull her arm down a little bit. This is

10 the origin, presumably, of the brachial plexus palsies. So if I put my finger up in here, where the brachial plexus goes, and pull down on the scapula, down on the clavicle, and down on the whole arm, there s just less room for the brachial plexus on this side. One of the ways to help urge this to close is to take and put a stitch around the inferior corner of the scapula and use it as a traction stitch to take some of the tension off the soft tissues, but this is why we desperately wish children to have lots of nutrition, so we have plenty of stuff to close here. 00:53: DANIEL HEDEQUIST MD: Children who have had a previous thoracotomy for other procedures, is there a role for plastic surgery to see the patient, a role for tissue expanders prior to expansion? 00:54: JOHN EMANS MD: We, and Dr. Campbell also, have been very disappointed in tissue expanders. Tissue expanders have a great role in lots of things, but here what you need is not just cutaneous coverage, but you need coverage by skin, subcutaneous fat, and muscle, so if you are going to do expansion, it needs to be submuscular expansion and that tends to cause a lot of scarring and a lot of trouble, so the times that he has tried that, he s been very disappointed with it. It would be better to ahead of time create flaps on a prospective basis. 00:54: DANIEL HEDEQUIST MD: Dr. Emans, I have a slide up that shows a little bit how you protect the device in the immediate postoperative period. Can you talk about the hospital stay and the post-op rehabilitation? 00:54: JOHN EMANS MD: We re going to run out of time here, so we won t see putting in a chest tube, but we ll lay in a chest tube along the back of the chest and then we ll close. We ll try to make sure that the skin is not under tension. The latissimus, as small as it is, will be at a little bit different level than the skin incision itself. We re going to bring the paraspinals that we ve worked very hard to preserve, we re going to bring the paraspinals up and over to cover the device, provide soft tissue coverage for the device. Then we will make a big foam pad arrangement so that when the child is lying on her back in the hospital in the first several days, she doesn t lie on the device because the device itself can be quite prominent. 00:55: You asked about postoperative rehabilitation. She ll probably spend 2 days, or maybe 3 days, intubated. The reason is not that she s terribly sick. You can see we haven t really lost a lot of blood, but by the time you give her enough pain medicine to control her pain, you also pretty well depress her respirations. It hurts to move this side of the chest, so her respiration will be inhibited. On the lesser ones, some of them have been extubated immediately, but on this one probably it ll be two days. That will probably be a little bit safer. 00:56: DANIEL HEDEQUIST MD: We have an from a viewer. Will she be able to walk afterwards and will she need a brace? 00:56: JOHN EMANS MD: I think braces are a bad idea, in general, because they inhibit the movement of the chest and this device is strong enough so you don t need a brace. We let kids walk. We let kids, once they re over all this, they can do any sports they want to do. I suppose if somebody really wanted to play tackle football, we d probably say no, but we don t restrict their activity. Dan, with the little bit of time we have left here, just to get a perspective of how large this is relative to the patient, here s the scapula. Here s the spine again. We ve covered the device. We ll pull this over and cover it over here with the paraspinal muscles. We re going to pull the latissimus down and we ll end up with a cosmetically reasonably satisfactory incision. Someday she s probably going to need a spine fusion and that would end up being a separate incision along here, so we ve stayed away from the spine and not done any spinal procedure. 00:57: DANIEL HEDEQUIST MD: Another . Can the device be seen through the skin or, more importantly, can you feel the device through the skin? 00:57: JOHN EMANS MD: In thin children, you can always feel the device through the skin. After many operations, I would say in the most thin children, yes, you can actually see the bump of the device and the line of the device. Again, I think it s important to emphasize why we re doing this. We re doing this for children who very early on have a very serious anomaly, anomaly of the spinal curve and chest wall abnormality. If you didn t do this and

11 did more conventional treatments, like a spine fusion, you d end up stopping the spine growth at this relatively short distance. If you were to control this child with a conventional spine fusion, it would take a spine fusion from about T3 probably to about T9, so T3 to T9 would end up being, if it got fused here, it would stop growing and would be like 8 cm long. Alright, now if you flip over here to Travis, if this girl is going to be as big as Travis, is she going to be okay at the end of growth with a chest that s only 7-8 cm long? I would submit that avoiding early fusion for serious spine problems is our major goal for this. It s also sometimes handled by growing rods also. 00:59: JAMES KASSER MD: John, it might be worth stating that not too many years ago, the standard procedure was to fuse the spine over this number of segments. 00:59: JOHN EMANS MD: I think that probably is still the standard procedure. This girlöi don t know, Dan, whether you can go back to one of the slides of her, but remember, she went from almost no curve at birth to now, at age 3, having 55o of curve, so if we do nothing, she ll have a very major curve by the end of growth. If we do a spine fusion, it probably would stop her spine curving. With the ribs stuck together, though, I m not sure that she wouldn t continue to pull over into the spine fusion. Our series and Dr. Campbell s series has a number of children that have had prior spine fusions which failed to control the curve and were then controlled by subsequence expansion thoracostomy and VEPTR procedure. So I appreciate the opportunity to try to show this to everybody. We ll just finish closing up here and I think things will go along nicely. We ll make sure that she moves her arms after the end of the procedure. Thank you all very much. 01:00: JAMES KASSER MD: I hope you ve enjoyed our presentation today and it gives you some idea of what the possible role for this procedure is in people with complex spinal deformities. We re grateful for the work of Bob Campbell in San Antonio in developing the procedure that has led us to embrace it in these children. Drs. Emans and Hedequist, as well as our Division of Spine Surgery, is, I think, quite excited about the early results in this procedure. Thank you. 01:00: DANIEL HEDEQUIST MD: Yeah, I think with that being said, the most important thing is a thorough preoperative evaluation with a pulmonologist, the pediatrician, appropriate imaging studies, and the postoperative course of these patients can be pretty complex, so a good ICU team and a good critical care medicine team will help avoid complications. I think for the right patient, it s an excellent treatment, instead of an early arthrodesis expansion of the chest to control both the chest deformity and spine deformity in the right patient. That s it. 01:01: NARRATOR: Thank you for watching the vertical expandable prosthetic titanium rib procedure from Children s Hospital, Boston. To obtain more information, to make an appointment, or to receive a physician referral, please click the buttons on the screen.