Cystic Fibrosis (CF) is an autosomal recessive genetic disorder characterized by mutations in the cystic fibrosis transmembrane regulator gene. The pathophysiology is based on abnormal chloride secretion from columnar epithelial cells. As a result, patients with CF have symptoms related to their inability to hydrate secretions in the respiratory tract, pancreas, and intestine, among other organs. In the lung, thick, inspisated secretions give rise to chronic obstructive pulmonary disease characterized by severe pulmonary infections, culminating in respiratory failure. Subacute exacerbations of CF lung disease are treated with antibiotics and various forms of chest physiotherapy. When large areas of lung develop abscesses, necrosis, surgical treatment is often indicated. Options include lobectomy as a temporizing measure, and lung transplantation for end-stage CF lung disease. Here, we present an unusual case of a man with CF whose lung function had remained relatively good until adulthood. His left upper lobe became chronically infected and non-functional. Because his overall lung function was moderately preserved, an open left upper lobectomy was performed to prevent recurrences of subacute infections and subsequent damage to the left lung.
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Hi, I’m Chris Morse. I’m a thoracic surgeon at the Massachusetts General Hospital, and today we're going to do a left upper lobectomy for an adult patient with cystic fibrosis. He is a chronically infected and damage to left upper lobe likely from the cystic fibrosis and this is in turn leaving him with recurrent and chronic pulmonary infections beyond even what we would see with cystic fibrosis. He’s a little bit of an unusual case only because he's reached 50 to 55 years of age with the diagnosis of cystic fibrosis and has moderately preserved pulmonary function although he does the - the usual issues that go along with CF including recurrent pulmonary infections.
This will be a rather difficult operation I suspect because of his recurrent infections in the chest as I think his lung will likely be stuck to the chest wall to will require significant amount of work to achieve homeostasis, and we'll do something that is somewhat unusual for a routine lobectomy in that we will use some muscle from his chest wall to reinforce or buttress the bronchial closure because of his underlying plumber issues.
That's good - rotate towards me - Kelly clamp please - Kelly - that's good - Kelly. So we’re going to make a posterolateral thoracotomy in this patient, and the standard posterolateral thoracotomy starts halfway between the back of the scapula and the spinal processes - yeah please - and then one finger breadth off the tip of the scapula. As I mentioned before we started in this case, we're going to harvest muscle in the way to help buttress - knife please - to help buttress the closure because of the infectious issues in his chest, so we'll do that as we get going. Great, thank you. Knife. Pick up. Forcep. Blue towel please.
We're going to come through the latissimus muscle first. And this is the latissimus being divided here. And so this is the latissimus muscle almost completely divided. So we spare the serratus muscle, and we come down the inferior border of the serratus muscle and onto the chest while I'm going to mobilize along the inferior border of the serratus muscles so as to not have to divide it.
And we’re going to come into the chest over the fifth interspace so we have to cut some ribs to do that.
Harvest the muscle here, so score this and this. And as we talked about before starting the case, because of the infectious issues in his chest, we were planning to harvest a piece of muscle to help reinforce the closure of the airway - pick up this, got it - and that's what we're going to do now.
So we're now we're scoring the center of each rib which will allow us to harvest the periosteum off the rib. So now we're just peeling a periosteum off the rib. Going to the bottom.
So we just peeled it off the 6th rib, and now we're going to take out the 5th. We need to get into the plane and you keep it perpendicular when you carry it - perpendicular to the rib. As best you can, you stay perpendicular. Then you gotta make it feel right. Alright, hold the retractor for a second. Sometimes it can be a little sticky as we do this. Buzzer. Extended tip please. Pick up. Hold that there. Forcep please. Suction in another hand.
So the reason this is a little trickier is again cuz his pleura, the lining of the inside of the chest, is not totally normal again from a lot of repeat infection and…
So we’ve got part of the muscle harvested - maybe we get a little more of this. I think we're going to shingle the rib first. You can go up - up and down - pair of spinus along the bottom. Madison? We shingle the rib so that we can allow it - the chest to open without hopefully fracturing the ribs, and this is sort of a controlled break of the ribs. We’ll actually take a little piece of the rib out as we do this. Pick ups. This will be 6th rib for permanent pathology. Kocher. Bovie.
Two blue towels please. Can we get a 30-30 on the cautery please? Forceps.
So this is the harvested intercostal muscle flop. It's a muscle that lies between the ribs. This is what we're going to use ultimately, hopefully to help reinforce the closure of the airway. We;ll probably the length that a little bit more, but it's a start. Can I have a Bell Ford retractor please?
So we got to take down a couple of adhesions - one right at the apex of the chest here, but you probably can't see as of yet. It's okay, can I have a duvall please - small? We’ll take the bend-to-beam - yep, top one - but don't - actually, sit tight for a second - it’s not as terribly stuck as I thought it might be - so have it - have it right there but don't open it yet.
So we need to - now we need to sort of carefully start taking down some of the adhesions in the chest, which are not nearly as bad as we thought they might be. One thing when we're working at least in the - in the anterior aspect of the chest, or the anterior aspect of the hi - of the hilum - of the center of the lung - is something called the phrenic nerve, which innervates the diaphragm. It’s a very important nerve in terms of respiration. So, make sure you see it, so I can talk about it. And especially in a guy who's got some - stay up on the lung - some intrinsic pulmonary disease, something we do not want to - stay up on the lung - something we don't want to injure, and does not appear that we did. It’s sort of right there, you can sort of see with the end of my suction. This is a phrenic nerve. It does get drawn slightly up into the center of the hilum there probably from the inflammatory changes at the hilum.
And looking at the chest, in the left chest, posteriorly to the hilum, to the center of the lung, this is the aorta, going down to provide blood to the distal part of the body. And we're going to start sort of a dissection around the hilum to free it up - free up all the pleura, and expose some of the structures we need to identify and divide to get this specimen out.
So we'll start by working posteriorly here. I'm retracting the lung towards me in - he was going to start to open up some of this pleura off the lung. The plane here that we like to achieve is staying right on the long and releasing the pleura. Natalie, is the Bovie on 30-30? Can you get him longer pickups please.
I would expect he'll have some enlarged lymph nodes in this area which will make this rather difficult. That’s point - tint it out. Right below where we are right now is the pulmonary artery so. And now, as we work posterior and superior, now we’re going to go anteriorly. Anteriorly, we have the - hold this up - superior pulmonary vein. So we try to open the pleura anteriorly to find that.
We’re just continuing to work the pleura circumferentially around this area. The top of the hilum is the pulmonary artery which we’ll try to avoid. Please have a 4-0 Prolene on an S H and a 4-0 on a BB and a sponge stick loaded at all times.
And doing a lobectomy, we’ll often take down the inferior pulmonary ligament. When we're doing a cancer operation, we look for lymph nodes in this area. In this operation, we’re taking this ligament down to allow the lower lobe to expand to help fill the rest of the chest after we remove the upper lobe, and the plane here is to stay right between the lung in the ligament. The top of the inferior pulmonary ligament is the inferior pulmonary veins - a mm towards the vein - I mean a mm towards the ligament. And it’s always nice to confirm that you have both an inferior and superior vein before you start to divide - stay out of the lung.
You can see how that allows the lung to come up, and as we get to the top, I’ll be looking for the vein - top of the ligament.
We've reached the - after taking down the ligament, we’ve reached the pulmonary vein on this side. I'm going to check our hemostasis here. And with that done, we’ll go back to work on a superior pulmonary vein now. I'll take a big Duvall now please. Forcep please.
I’d say these tissue planes are a little more stuck than they usually are - again, from chronic infections. 4-0 Prolene on a BB, backhand. Looks like a few days of bronchial - maybe. I’m sure - sure - sure it is, but I don’t really know. Okay. Forceps.
Okay so now we’re going to work at the top of the hilum for a while now. Pick - pick - please tend to that - no, I’d… We’ll continue to mobilize this pleura - thickened pleura - off the artery here. And it continues to be a very delicate dissection because of the all the inflammation that is present here. Again, it’s very inflamed. So a pickups and a Bovie - or pickups in one hand a Bovie - a bunch of - sorry… There might be just a tiny bit more traction so we can see. I’ll take a Bovie please. The - I think you need to open a little bit of that up, so we can see - towards me.
So in attempting to get around the vein, we made a small rent or hole in the vein, and that’s why we just finished placing a couple stitches in to try and control while we do the rest of this operation - hopefully. Hold this up please.
So now we will continue to work around the vein, which has proven to be somewhat difficult today. Just trying to work behind the vein to find the inferior aspect of it. Forceps - another Duvall please. Right above me - stay close to me. My clamp back - take suction.
So that was a rather difficult approach around the superior pulmonary vein, and we're going to show it to you in just a second. The reason it was difficult was 1) we made a hole in it, and 2) there's a significant amount of inflammation - significant amount of inflammation around the vein itself. The tissue planes are not normal - again that's from chronic infection in his chest. Lying right behind the superior pulmonary vein in the chest is the bronchus, so we knew we had some space there that we can move inferior towards the center of the lung safely, but up near the top of the - of the hilum, or the center of a lung, is the pulmonary artery, which we had to try to avoid. I think in taking this vein at some point, we will significantly free up the hilum so that we’ll have better visibility of other structures, which hopefully we’ll see you in just a moment.
So we’re going to staple the - don't close it yet - we’re going to staple the pulmonary vein - superior pulmonary vein close. Like that - take it. A vascular staple load. Good. Scissors. So that’s going to give us much better exposure to the hilum now.
So now we’re going to do some very difficult work as we expected around the pulmonary artery, which we knew is going to be very difficult from the imaging beforehand because the information extended down right to the origin of some of the pulmonary artery. We’ll adjust to go to the top now. Yep, I got it. Cut it out - use your pickups. Lift up on the vein gently. We need to come off the bronchus slightly here.
So we've got that partially dissected out, going forward. Now we’re going to come to the back of the hilum, or the posterior hilum, and we're going to work on the artery back here. That white structure that you can see is the pulmonary artery that we're working on. And come back this way. K - find a branch.
Unlike some operations were we have the option of getting into the - into the Fisher or the center of the lung between the upper and the lower lobe, we do not have that option here - again because of the information. So we have to do this all from the - from the top, from the back, in the front, in the side. That’s what we’re working on. And that’s it. Can I have the Mets please? And the tissue - it won’t spread. Peanut please. Hold that Nick please. 30 curved tip chance that this is an apical branch of the pulmonary artery - I’ll take a peanut first please - peanut please - peanut.
This is a apical branch of the pulmonary artery that we’re trying to get around here. Can we have a loop? Twisted free side? I can’t see. And again, we're going to take this with a vascular staple load.
So we continue to work circumferentially around the center of the hilum - forcep, lower. And we have one large apical branch - slightly tricky to control. Okay so this is a big, very proximal apical branch. This is quite - do we have a leak somewhere? Kelly, does he have a leak somewhere? Am I hearing his airway? Maybe not. I thought I heard a leak. Oh yea, it’s coming out of the… Forcep. Hold this gently. Take the first loop out. Hold your breathing not please.
So this is the largest most proximal apical branch of the pulmonary artery we just now divided, and it puts us in a significantly better situation now to finish this operation. Pull up - forceps. Breath now. So we’ve taken the superior pulmonary vein. We’ve taken some branches of the pulmonary artery. No, no here. Now, we’re going to find a medial branch of the pulmonary artery, and right now, we're working on dividing his airway off of the pulmonary artery. And we’re just sort of taking tissue off of the airline.
So we’re worked a little in posterior. Now we’re going to go to anterior hilum to work on the airway a little bit here. Hold that up. Yeah, I’ll take it - thanks. Forceps.
So we're trying to define the - the airway. I'm going to follow this, so take this - take it right here. Take this issue down right here. This is just soft tissue, inflammatory tissue, nodal tissue, that’s around the airway. And this tissue here. Get this tissue out, there’s going to be a lingering-regular pulmonary branch in there somewhere. It’s going to be right there, so make sure you can see everything - your Bovie’s in place.
So we’re just sort of again feeling all this inflammatory tissue around the center of the lung - take that down towards the airway, just up here. Push up a little bit. So again, an incredibly difficult dissection around the left upper lobe airway as a result of his chronic pulmonary infections. A lot of what we deal with here is not only just infectious issues, but nodal tissue that's enlarged as well, and so we’re... One of the easiest ways to figure out where normal structures are is to remove the nodes - the - especially when they are enlarged - both in this, infectious situations and cancer situations. This is a node. Cauterize my pickups. Lift up with the sound towards you. Divide the tissue. 3-0 silk tie and a passthrough please.
Sometimes when you have again a big infectious situation in the chest, the arteries along the airways, the bronchial arteries, can you be massively enlarged and inflamed. Lift up towards you and I’ll retract in a second. I think that’s a little bit what we're dealing with right here. So we’re going to control that with a tie. Last thing you want to do is store up some bleeding later - bleeding cause of this situation - there we go. Gentle - Jesus. Okay. Pickups.
So we’re going to continue to work around this airway, sweeping some of this nodal stuff up has been difficult - peanut please - difficult to somewhat sort out. Okay, coming back towards - Duvall - coming back towards the center of the lung, we’ll look for this airway and dissect - dissect it out again on this side. It’s a terrific view of it. Release that node - bring it up here more. Don’t pull in, pull up. Good. So we’re going to take a feel of it, and see if we can feel for airway there. Oh, I don’t know. Pull that tissue out.
So now we’ll run the - the I believe is the left upper bronchus, and we’ll confirm that in a couple different ways. And so what we’ll do next is dissect this out a little more thoroughly now that we think we're around it. Now lift the other side. So flipping along back to expose the anterior aspect of the hilum. Hold this tissue down. Hold that tissue down there. With some of these nodes or nodes we’re seeing from the other side. And as we discussed, in a difficult dissection, it’s easy to - easier - to take some of the nodes out to find the anatomy.
So again, we’re just trying to clean up the bronchus so that we can dissect it. Looks good. Maybe that’s the left upper lobe bronchus. Stapler. So now we’re going to divide the airway. We’ll use a thicker stapler than we had in the past. Just going to inspect this from a couple different angles - hold this here. Take it out. Hold that there - pull this guy out. So, Taylor, we’re going to give her breath to his side in a moment - the left side - I’ll tell you when. Close.
So part of our way of confirming that we're in the proper spot is to - is to clamp the bronchus that we're going to divide and then ventilate this side to prove that the rest of the lung comes up - that we have just the left upper lobe bronchus. Can you give us a breath to the left side to 15. Or 20. Good, so you can see that lower lobe start to expand. Okay, you can let it down. You happy with that? You can let it down and isolate it again.
So now we feel comfortable that we've got just a left upper lobe bronchus done, and with that, we will divide this - ahuh, nice and slow. Okay, great. So with that complete, we’ve done all - almost all the major structures of the - of the left upper lobe. We’ve done the bronchus, we’ve done the artery, we’ve done the vein. And so now we just need to figure out how to get the rest of the specimen out. You stick the artery with the Fisher? I don’t know if I see the artery, but it’s just… As you just pointed out, we need to find one more branch of the pulmonary artery, which we’re going to do right now.
So again we're going to just make sure we have all the vessels divided that we want. Forceps. The tissue is off - I’d pick that up towards me. Ring clamp for a second please. Ring clamp. Cuatery. We’re going to divide on the stapler. Stapler. 45 black. Take this first bite here.
So we’re going to start dividing the Fisher, which I think is a little bit of an unusual step only in the sense that it will help expose a little more stuff in this very thickened area of the lung. So this is the Fisher, the delineation between the left upper and lower lobe bronchus. Take a 60. Good close. Let me just see something here. Close. Close? Close. It’s closed. Take it. And we’ll take one more - 60 please. Close. That it. 45 black - actually 60 black.
So it’s a very damaged left upper lobe, and see that it's kind of beaten up now, but it's definitely smaller, more consolidated and certainly a result of his chronic pulmonary infections. As we talked about, we took the superior pulmonary vein. We took several individual apical branches of the pulmonary artery. Then we worked hard to get around the left upper lobe bronchus and divided that all with staplers, different varying degrees of height - thinner for the vascular staple loads and thicker for the bronchus. This is the bronchial staple line right here, right in front of my grasper - this white structure here. These are some lymph nodes that are adjacent to it. This is a vein cuff it looks like here. I can't really see the bronchial artery. Staple. Staples in here right now - maybe one right there. Anyways so left upper lobe for permanent pathology. Warm irrigation please.
So we irrigate out the chest. Where are you now? This is irrigation now. I don't think so. Sponge though. Forceps. Retract that.
So next we're going to, with the specimen out, and this not being a cancer operation, so we're not going to do a comprehensive lymph node harvest, we’re going to work on reinforcing that staple line. After we we make sure - coagulate made. Good work.
So we're going to now secure that muscle flap over the top of the airway. Long 4-0 silk please. So we’ll do this in four points around the airway. One of the top, one at the bottom. We’ll do the heel first. Maybe 2 - come up towards me. More like - don't we need the flap? We don’t have enough flap. No pull it through. Come back towards you now. Like that. I'm going to go across the back of it, so stop. We're going to go this way. Peanut. So you - let’s go ahead and lay this way, so you go this - under - you pick this direction right cuz you're going on this side. Pickups to me please. Trying to go back towards the PA? That’s so, but…
Cut and a snap please. Stitch. And now we do one on either side of the airway. Forcep. Scythe. Where’s the scythe? So this is the scythe towards me. Thank you. Snap. Stitch. Okay.
So, we’re going to put a couple chest tubes in now. Knife. Knife. Schmidt. Tunnel 1 posterior, tunnel 1 anterior, forehand. Small square hole please. Bring the lung up please. Can you bring the lung up please? Can you bring the lung up? Can we bring the lung up? Can you inflate the lung please? Forcep.
The lung is reinflating now - the left lower lobe. If you can ventilate both sides now, it’d be great. And ultimately this will expand I think to fill the chest. Another breathe, just to get some Alexis out. Can you give us another breath and hold please? Can we have another breath and hold to like 20? That’s good, great. Thanks. Sucks.
And so with the chest tubes in place, we’ll close the chest in sort of the opposite way we came into it, by closing the ribs. I put stitches around the ribs to pull them together. Travel. We’re only putting two in. Cut. The - switch. You take some of the flex out of the bed for us please? That’s good, thank you. Zero please.
So now we're going to close the serratus layer that we had opened additionally. We’ll try to put a number of layers in so it's watertight and airtight - back to the corner, don’t forget the lot. So again, just closing the serratus, closing latissimus, which we’ll close in two layers, and tie these two layers to themselves. Can you roll the table away from me please, and bring it down a little bit. Here’s the nadal bed.
So that was a left upper lobectomy as we talked about for a rather difficult situation. Guy who has CF, but as an adult, with fairly preserved pulmonary function, but had a very damaged left upper lobe from recurrent pulmonary infections. And this damaged lobe was never going to heal despite antibiotic therapy, and I think it contributed even further pulmonary issues than his baseline CF does. So because of that, we chose to proceed with a left upper lobectomy given his pulmonary function was adequate to remove it. The things that made this operation more difficult than a typical lobectomy, for a lung cancer for example, were just the dense inflammatory changes at the hilum which is not unexpected given his - his CF diagnosis and his recurrent infections.