Endoscopic & Surgical Interventions: Pancreatic Disease

So welcome back. Welcome back to managing the Diseased Pancreas and Children. We've had a great first two sessions covering acute pancreatitis, chronic pancreatitis. We're going to become a little bit more invasive, a little bit more interventional now, a little bit more surgical, and so we'll turn things over to, uh, Doctor Tom Lynn, who, uh, directs our, uh, endoscopy component of our pancreas care center, and he's going to start off with endoscopic and radiologic interventions as well as conventional surgical options toward the tail end. Great, thank you, Jamie. Um, as we see, we have, uh, 4 primary individuals part of, uh, this, uh, discussion along with the remainder of our panel, but, um, Doctor Milton Smith from UC, um, is joining us. Um, he's one of our adult, um, esteemed, uh, GI colleagues, um, uh, that, uh, is involved in, in the component of our, our patient care that too as well. So we'll start off with a, a case, um, and we'll get on to, uh, some of the details. And so this is an 11 year old female presents with intermittent back pain and chest pain for several months. Um, she has a, uh, A more chronic history of nausea and vomiting and had actually had a working diagnosis of cyclic vomiting syndrome. Um, Additional history included, um, 4 years prior, um, an inflammatory myofibroblastic peresophageal mass, uh, that was, uh, sampled and the histology, um, suggested that that. Particular finding but um had an unusual spontaneous reso resolution of that particular mass as well. Um her more recent labs upon presentation to us was a borderline elevation of her serum amylase and lipase um at that time with other uh uh biochemical abnormalities not being present. So, uh, here's her original imaging, um, related to that, uh, that inflammatory mass, and we'll have our, our, uh, colleague Doctor Trio kind of go over this. So I just wanted to, because this is an unusual case, I wanted to show the findings at the time of the original presentation, and what you have on the left there is a coronal, um, reformatted image from a CT scan performed with intravenous and oral contrast. The blue arrow indicates the stomach, um, which is filled with, uh, contrast material. And here pointing with the cursor, the yellow arrows indicates indicate the inflammatory mass that's surrounding the esophagus and the gastroesophageal junction. And because of the diagnosis of IMT, um, the patient had a FDG PET scan which is shown here on the right with the yellow arrow indicating abnormal FDG uptake in association with that inflammatory mass near the gastroesophageal junction. So by all imaging characteristics this would be very compatible with an IMT. This is the CT that the patient now has at the follow-up presentation. So again, as Doctor Linn suggested, the patient had a spontaneous resolution of their tumor, and they're now presenting with this CT scan, and clearly things have changed pretty dramatically. And what I'm what you have here is again a corona reformatted image here on the left side. From a CT scan, ACA reformatted image here on the right side again performed with intravenous and oral contrast. The yellow arrows indicate a large bilobed fluid collection that's similar in location to where her inflammatory mass was on the prior examination, though it does extend up through the diaphragmatic hiatus into the mediastinum. Shown on the image on the right here again, the fluid collection denoted by the yellow arrows, the pink arrow there indicates a hyper dense, um, dependently layering focus within the collection there. So whether this is blood clot or a solid component of whatever we're seeing here, the other important finding. Note here is on the coronal reformatted image, the white arrow here indicates extensive venous collaterals related to the spleen. So there's been a process that has compromised the splenic vein at a longer duration than simply this fluid collection that we're now seeing in the mediastinum and the upper abdomen. So these particular findings prompted our next evaluation via endoscopic ultrasound. So can we go back to the previous slide, um, first, and so if you look at the image on the right, it looks like although that's a fluid collection that there may be a solid component of that mass that's pressing on the posterior gastric wall there. And so given her prior history of IMT, we thought that it might be a good idea to try to use endoscopic ultrasound to see if we can identify and biopsy the solid component of the lesion. So at, uh, and we started out with just a regular, uh, gastroscopy, uh, showing here from the proximal stomach what looks like an extrinsic compression, uh, of the posterior gastric wall compatible with the lesion that we see on CT scan. And then here is a radial echo endoscope placed near the GE junction, uh, which really shows a large fluid collection and as best as I could tell at this study. With little to no solid component, you can see the left lobe of the liver on the left side of the screen. On the right side you could see, I saw a large anechoic structure that began in the lower chest and extended down to the upper abdomen, so not what I was expecting to see at all and really nothing to target with a needle for biopsy. So this further prompted an additional evaluation um from a radiologic standpoint. So what we have now on this slide is a series of images from an MRI in this patient, and the reason that we chose to go to MRI at this point was to better characterize that collection and to determine, you know, were there solid components or was it simply a complex collection on the left side, on the top there you have an axial fluid sensitive sequence, and on the lower is a T1 weighted sequence and the yellow. Arrows indicate this fluid collection and what you see on the T1 weighted image down here is that there's a fluid fluid layer within this collection with hyper dense material layering dependently, so either protinaceous fluid or bloody fluid within this collection. Again, the blue arrow on the upper image there indicates the stomach, which is fluid filled, and then we see these large venous collaterals. Related to splenno gastric shunts or or linogastric shunts as a result of a splenic vein compromise on the right here we have a coronal reformatted image again showing this fluid collection indicated by the yellow arrows with that dependent nodular component within the collection which again we had seen on the CT scan. Here again are those venous collaterals. The last image down here on the right, remember we were trying to. Determine were there solid components. So this was an axial post contrast image with the pink arrow again indicating that nodular focus showing no enhancement. So we really didn't see any solid components on MRI or enhancing components on MRI that would suggest a tumoral component. This at this point looks like a complex fluid collection, perhaps with a retractile clot or some other debris layering out there dependently. Great. So the next stage of her evaluation, um, based on her collective imaging, including the initial endoscopic ultrasound, uh, there was a higher suspicion that this represented a, a, a pseudocyst or some variant thereof. Um, her, uh, additional testing prompted, despite a lack of, uh, significant history for a definitive pancreatitis, prompted hereditary pancreatitis genetic testing, and she turned out to be PRSS one positive. So going back to a 2nd, moving on to a 2nd endoscopic ultrasound. So the patient, um, remained symptomatic, and the interesting thing about this patient to me was the fact that she'd never had, at least as far as we could tell, an acute episode of acute pancreatitis, but based upon her symptoms and what we thought we were seeing, uh, we decided to proceed with, uh, endoscopic drainage of the collection. And so here we are again at another EUS session. Uh, again, gastroscope retroflects in the mid-stomach showing extrinsic compression of the posterior gastric wall. And this time we're looking at a linear EUS image showing again a large anechoic structure. If you look carefully, you can see some debris within it. Uh, it's got a thick wall and no solid components, and, uh, we thought this was in good position to go ahead and proceed with, uh, drainage of the collection. And so we've accessed it here with a 19 gauge FNA needle. And, uh, secure the access by calling on fluoroscopic guidance a a standard ERCP guide wire with within the collection. You can see how the looping around there within the cyst. Ah, and then, uh, dilate the tract between the stomach and the cyst using a 10 millimeter balloon. And then followed that by a placement of 4, 10 French double pigtail catheters. So, um, after that, uh, that cyst gastrostomy from, uh, Doctor Smith, um, she had follow-up imaging, uh, two months later. So this is a follow up CT scan in the patient after the endoscopic drainage procedure, and this is an axial image performed with intravenous contrast. There's no oral contrast on board at this point, and what we see on the single axial image, these hyper dense linear components are. The pigtail drain the drain that was placed from the stomach here, this component extending into what was previously the fluid collection. There remains some abnormal soft tissue related to that collapsed fluid collection, but clearly there's been a dramatic decrease in size of the collection on this CT scan. Again in the upper left upper quadrant here we see the venous collaterals related to the splenic fleeing compromise in this patient. Great, um, so at the time of this follow-up, um, imaging, um, she, her symptoms had significantly improved. So 3 months later, So this is another follow up CT scan in this patient and to further document resolution of collection, I believe is the reason it was performed, but there are some unexpected findings on this exam compared to the prior. So we don't see those drainage catheters on the images I've selected here again, the yellow. indicates the abnormal soft tissue at the level of the gastroesophageal junction extending up into the mediastinum, and this appears to have increased somewhat from before and there's now a little bit more fluid along the gastric cardia and fundus, and there's a small left pleural effusion, so not the progression we like to see on imaging, at least in terms of what we would expect to see with the expected resolution of that collection. I think before, before we head on, uh, Milton, maybe you could comment a little bit on, you know, management. Once you have these drains in, what's, you know, when do you take them out, you know, how long does that cyst gastrostomy stay open when you approach it by EUS, etc. Right, good, thank you. So we generally leave the catheters in for about 1 month to 6 weeks and repeat imaging as was done here. The other thing that we like to do before taking the catheter. Cathets out is to evaluate the status of the pancreatic duct to see if there's any evidence of communication or ductal disruption or stricture, something that you need to know about and treat to to be confident that if you take the catheters out, that the fluid's not going to recollect. Now, in her case, the 4 catheters migrated out spontaneously, and the patient was asymptomatic. And so then the question is, well, do you really need the pancreatogram? Or do you wait and see how she does? And so we, we chose the latter route. Um, but at this 3 month, um, follow up, um, time period with the, um, pleural effusion, she actually, um, had a recurrence of her particular symptoms. So moving on to our first poll question, what's the next, next best course of management for this pseudocyst, uh, slash fluid collection recurrence? Uh, number 1, repeat endoscopic ultrasound with cyst gastrostomy, percutaneous drainage, ERCP, surgical management, or some other type of intervention not listed, and we'll come back to this. And so our approach for her, um, because of the recurrence was actually to go forward with ERCP. And so this first image, fluoroscopic image of her pan pancreatogram, you see the, um, the duct within the head of her pancreas filling, um, slightly, uh, it is dilated and as, uh, with additional contrast injection. Fills up to beyond the genuine starting into the body of her pancreas. In additional contrast injection, we see immediate extravasation of the contrast that is going cranially as we see from the yellow arrows and moving up the uh the the flu fluoroscopic machine we see how much um um uh north um the uh the contrast, um, extravad towards. uh, this prompted, um, endoscopic intervention including a pancreatic sphincterotomy and two endoscopic. Uh, pancreatic stent placements, we see them circled here with single pigtails, um, extending into the duodenum as well as radio opaque markers that are within the duct itself with hopes that this would end up resolving her pancreatic duct leak. Um, ideally we would have wanted to attempt to bridge the defect, um, to, to maximize our success of resolving the, the leak, but that was, uh, was not possible in this situation. So, um, uh, her symptoms did improve from that initial ERCP, and here's her follow-up ERCP five weeks later, uh, documenting that she indeed had a positive favorable response to the, the, uh, endoscopic intervention via ERCP with res apparent resolution of her, of her, um, ductal leak, which was a very, uh, um, positive, and, uh, the family was very happy with that. So coming back to our poll question, um, if we can pull up those, the poll results. So ERCP was the number one answer. Great. And number 2 was repeat EU US with cyst gastrostomy. OK. There was a what you want to comment on that, Milton? I mean, it didn't seem like there was a giant collection at that point to try to approach it in that fashion. Exactly, clearly leaking, but not an organized collection like we saw before that you could drain endoscopically. And 20% said surgery, so it's distal or I mean it's in the tail of the pancreas, so why not go in there and take out, take it off right, you know, proximal to the leak and and stop the leak. Well, you remember this, this, this child has a genetic etiology and so the concept that, uh, you know, this patient may end up potentially needing a total pancreatectomy in the future, uh, is there, um, it, it, you know, her, her chronic pain may progress and. Uh, taking out the tail for this leak and tossing it in, in Pat's hands at this point, um, uh, would compromise eyelet yield in the future. So, so we elected to go the non-surgical route in this circumstance. When you, um, pass the side viewing scope, are you able to pretty well evaluate your, if you have a previous cyst gastrostomy to evaluate its patency, or do you put in a regular scope first, then take it out and then put in a side viewing scope? Well, you would expect it's been several months now that the cyst gastrostomy is probably healed up, closed, right? Exactly. So if there's a persistent leak, it, it, it, you hope it wouldn't close, right? Correct. So but you think it does anyways, even if there's because. If it's a controlled fistula, if it's a fistula and there's stuff coming through it, shouldn't it stay open, or does it close even? Um, it's usually not brisk enough to keep it open. I mean, a few days after those catheters come out, the fistula closes right? And so, so it's 410 French catheters is what was in there, and I, I would imagine, we don't know for sure, I would imagine they probably migrated out one at a time until she eventually lost all of them. And it gets back to the principle that in general when you, when you drain these cysts through the GI tract, you want to know what's going on with the pancreatic duct. And you don't have to necessarily do them at the same time. Sometimes I do, uh, do the ERCP first and then in the same session, uh, drain the cavity, but it just makes the procedure longer. So our original plan was to bring her back and then before taking the catheters out, do a pancreatogram to make sure that this isn't happening. So this is a good place to actually move on to um uh some discussion on, uh, inflammatory pancreatic fluid collections and, and so what we've learned, uh, based on, uh, uh, data and, uh, expert opinion from our adult colleagues, our adult GI colleagues is that from the revised Atlanta. Classification from 2012, um, that they have classified inflammatory pancreatic fluid collections into four categories acute peripancreatic fluid collections, pancreatic pseudocysts, acute necrotic collections, and walled off necrosis as it relates to acute peripancreatic, um. Fluid collection fluid collections, we do understand and know that they tend to develop early in the course of acute pancreatitis. It's rare for them to become infected, and up to 40% of acute pancreatitis patients, regardless of. will develop some form of a fluid collection, but also knowing that the majority of those fluid collections will resolve and spontaneously resolve, um, but then even less, actually less than 5% will potentially evolve into a pseudocyst. As it relates to pancreatic pseudocysts, um, most commonly they arise from the previously mentioned peripancreatic, uh, fluid collections and, and, um, uh, more so are formally defined, uh, when they reach, uh, a time threshold of 4. Weeks or greater, uh, they do contain a high amylase, uh, rich content, low fluid. Um, there's little to no internal debris, um, and, uh, they oftentimes by the time that they are characterized as pseudocysts have a well defined non-epithelialized wall. As it as it relates to management of pseudocysts, um, the questions that are important to ask oneself is, um. Uh, Especially if there's a thought of being, uh, invasive and, and, uh, and, uh, interventional, um, is the patient symptomatic? Uh, is there infection of are there suggestions of infection of the pseudocyst, and are there also suggestion that the, the, the fluid collection slash pseudocyst is enlarging and all those questions, um, factor into uh the next management therapy if there is a decision to go forward with that. So for pseudocyst drainage, um there are different approaches that are available and so we know that percutaneous utilizing our interventional radiology colleagues, um, as we have touched upon, endoscopic ultrasound and endoscopic management, um, also utilizing ERCP, so either a transmural via. EU US transplant papillary via ERCP or even a combination of both. And then thirdly, surgical intervention for drainage of a pseudocyst is also available modality as well, but I wanted to emphasize that management intervention and the choice of the intervention. can be very much based on the local expertise that the facility has or or the local area has as well as the presence or severity of patients' comorbidities. Yeah. Uh, from an endoscopic standpoint, uh, important things to factor in in terms of the, the feasibility and the likelihood of success. Um, the anatomic relation to the stomach and duodenum in order to, uh, uh, produce that transmural connection, um, is there a ductal communication is an important factor to also consider, cyst contents as well as the size of the cysts is an additional factor to consider at the time of endoscopic management. In this example, as well as one of the earlier sessions that we had that documented a pancreatic duct leak, uh, there are different manifestations that we can expect from such leaks from an internal standpoint, they can develop, develop into a peripancreatic fluid collection. Subsequently, possibly developing further into a pseudocyst, pancreatic ascites, pleural effusion, as this case demonstrated that we presented, and a pancreaticoenteric biliary or bronchial fistula can also be an internal manifestation. From an external standpoint, there is a potential for a pancreatico cutaneous fistula that might develop from a pancreatic duct leak. So how best to manage such leaks and fistulas, um, it definitely does, um, benefit from having a multidisciplinary team management approach, um, as we had mentioned before, transpapillary pancreatic stent, uh, placement has a greater likelihood of excessive success. You're capable of bridging the defects, but there are certain situations that can still resolve, certain leaks that can still resolve despite not being able to successfully bridge the defect within the pancreatic duct. Um, when considering whether or not to intervene and, and go forward with, uh, endoscopic, uh, intervention, whether it be EUS or ERCP, um, there are important things to consider and also kind of discuss with the family as well. Um, so one of the risks for, as we know both from ERCP and the US. There is an elevated risk for developing pancreatitis or exacerbating someone's pancreatitis from the intervention while you're trying to be helpful. There's clearly a risk for introducing infection of a sterile of a fluid collection that is initially sterile and then introducing infection within that sterile fluid collection. Um, and then there is a particular, uh, concerns for possibly developing, uh, ductal changes, uh, that might include a pancreatic duct stricture from having a, uh, a temporary endoprosthesis, um, within the duct, um, due to that being a foreign body and potentially resulting in an inflammatory process, uh, that would be introductile and leading to a stricture. As it relates to endoscopic ultrasound, um, we do know that endoscopic ultrasound, um, is one of the more, uh, newer modalities that we have for, uh, for adult intervention and even more so in pediatric intervention, and it provides high resolution imaging of the pancreatic parenchyma as well as the ductal structure. Structures both biliary and pancreatic, um, it has both diagnostic and the therapeutic capabilities, and these, these, uh, interventions have been clearly well established and, and beneficial in adults, but the information and available literature that we have as it relates to pediatrics in this intervention is rather limited. So why are they, why is this limited, uh, in terms of its role in, uh, uh, the use of EUS in pediatrics? And so there's some obvious reasons, um. As we mentioned, there's limited data. Part of it is the fact that there's a limited number of pediatric indications that we currently understand. Um, there is also insufficient inadequate awareness of the potential capabilities and and the utility of EU US for various disease processes. And there's also a very limited number of pediatric, um, gastroenterologists, uh, endoscopists who, um, have, uh, attained the appropriate training to actually perform EUS safely and effectively. But this, this information is actually changing, I think, for the good, the greater good, and so these are a couple of studies that have recently come out related to the experience at specific centers utilizing endoscopic ultrasound for specific pancreatic diseases in children and from their personal. Experiences at these specific centers there has been shown to be very good favorable benefits from this intervention and so we're learning more and as time goes by hopefully there'll be greater evidence on on the safety profile and the the uh the best utilization of this modality within children. So from some of the studies that we have, um, in particular, one of the studies I presented on a previous slide, um, their diagnostic and therapeutic indications, um, I have listed here, and a lot of it, if not much of it overlaps with what we've learned and what we know from our adult colleagues who have utilized endoscopic ultrasound, um, in their, uh, in their patient pathology. So moving on to the next case, um, this is a nine year old female who also has a PRSS1 um hereditary pancreatitis mutation, um, has came to us with already established chronic pancreatitis and she was referred to our pancreas care center for primarily management of her chronic pain. So these are axial CT images in this patient, again, in this case performed with intravenous but without oral contrast, and what you can see are several different findings. So the yellow arrows indicate fluid, peripancreatic fluid. So Doctor Lynn was talking about acute peripancreatic collections, but also ascites here. Down in the abdomen. Other findings to note that the pancreas has a discrete focus here of absent or clear hypo enhancement within the body or tail region of the pancreas. And remember, when you're looking for necrosis, that's that's what you're looking for is you're looking for foci of absent or hypo enhancement of the pancreas that may be sites of necrosis that then. Port the risk for developing chronic duct leaks. The other finding I'm indicating here with the White Ararrow is that the peritoneum is actually thickened and a little bit hyper enhancing here. So there's a peritonitic component related to this patient's inflammatory process. This is a subsequent MRCP performed in this patient about a month later, and you can see there's been a dramatic increase. In the volume of ascites in the abdomen here in this patient, um, all that bright signal here on the fluid sensitive sequence, displacing bowel loops centrally surrounding the liver and the spleen is pancreatic ascites. Blue arrow here again indicates this defect within the body or tail of the pancreas that now actually. A more walled off component outside of the pancreas, so it's beginning to look a little bit more like a pseudocyst and one thing I wanted to briefly mention about what Dr. Lynn had talked about with regard to the Atlanta criteria, that's a classification scheme that's been described for adult patients, and they specifically call out in there that this. You know, they did not, we're not considering pediatric patients, and so we're largely extrapolating those criteria to the pediatric population. It seems to hold true. It seems to bear out in terms of defining acute fluid collections, necrosis, pseudocysts, etc. But again, that is a largely that is an adult consensus classification scheme, right? Important points. Um, so moving on to our, our, our for our next poll question, what's the next best course of management for this particular patient? So ERICP with intervention, percutaneous drainage, distal pancreat distal pancreatectomy with surgical intervention, or is she actually a more appropriate candidate for TPIAT? So, uh, we, uh, went forward with an ERCP, and here's your scalp image showing, uh, no particular abnormalities, and then, um, uh, a cannulation of her biliary tree, nothing abnormal there, and then we see our guide wire going into the pancreatic duct, uh, and then contrast injection as we see from the yellow arrow, she has a, a blush of. Contrast extravasation towards the the tail of her pancreas that that somewhat somewhat supported our high suspicion that she had a pancreatic duct leak. So this led to, and we can see more contrast being non-focal and being more dispersed as it's extravasd outside of the pancreatic duct. And then finally, um, uh, a, uh, pancreatic ducts stent placement along with a, a pancreatic sphincterotomy, um, because of the, the depth, um, of the leak for her, um, it would, it was challenging to actually attempt to. Bridge that defect, um, and we were hopeful similar to our previous patient that we presented that despite the lack of um stent bridging of her pancreatic duct leak defect, um, we would be successful, equally successful as we had been on the previous patient um and so we'll come back to this poll question um and look at our results briefly. OK, ECP with stent. Yes. Right, so majority, um, um, went with, um, the intervention that we decided, um, uh, on going forward with, uh, unfortunately, um, her ERCP did not turn out to be very effective. Her, uh, her stent, um, spontaneously migrated despite having an intraductal flap, and, um, uh, she continued to have, uh, large fluid collections, um, within her, uh, abdominal cavity. And so, um, uh, through percutaneous taps she would be, um, uh, drained, um, uh, uh, serially, um, oftentimes, um, draining as much as 2 L, um, from her, uh, intraabdominal cavity, um, and ultimately there was a decision to place a peritoneal drain. And this is a limited CT image in this patient following that peritoneal drain placement by interventional radiology. I believe the arrow here indicates the pigtail portion of that drain, which lies in the peritoneum here in the left upper quadrant, and you can see the dramatic response that has been possible with this peritoneal drain in terms of draining that extensive ascites in the abdomen. So her drain was able to resolve the ascites. However, she developed the new findings of a mediastinal pseudocyst two months later, despite the fact that the drain was effective in eliminating the fluid collection. Can I just recap, so your stent fell out. So you have a persistent leak. Yes, you have a drain and persistent free leak into the drain, right? So now you've got a perineal drain in. Mhm. Um, there's no reason to believe that this leak's going to go away because it hasn't gone away. How long has it been now? Two months started in November, October, yeah, it's been 4 months, a few months, 23 months, OK, yeah, the goal here, uh, you know, as you might suspect, is, you know, she had had, and, and we didn't talk about it too much, but she had had multiple admissions for, um, uh, acute pancreatitis episodes in the years prior. PRSS1 mutation, you know, the ultimate goal here was to get her safely to be able to undergo TPIAT. Uh, the question is, is, you know, when is safe, you know, we had an active leak that was, uh, resulting in free ascites, very inflammatory, really can create a pretty hostile abdomen, and so everything that we're presenting here truly was to be able to get it, get, get us to a point where we could safely take the pancreas out. so we weren't totally clear on that. Got it, OK. And so that CT showed we were able to get control of the ascites by placing a peritoneal drain. Unfortunately, the patient then subsequently developed this process which if this is in similar location to you remember she had that defect in the body tail of the pancreas that was the source of what we thought was the free leak. So now she has what looks like a much more walled off process, so she has this multilobulated fluid. Here on a fluid sensitive MR coronal image here extending all the way up through the diaphragmatic hiatus and up into the mediastinum. In fact, our normal MR does not include up into the chest, and we've lost the, the most cranial extent of this collection. Similar to our prior patient, there is some material within this collection that I've indicated here by the, the pink arrow, um, that on the, uh, T1 weighted, um, sequence shows, ah, hyperintensity. So this is a, a retracted clot, ah, within that collection as well. So I'll, I'll interject here, um, before we go on to the, to the management is that this was, um, uh, a surprising finding on her cross sectional imaging. We're literally 2 weeks away from her scheduled OR date and, uh, her symptoms had not been, her symptoms had not been tremendously bad, and we said, well, you know, we should just make sure that, you know, from a cross sectional imaging perspective things are not too hostile. So hence the MR and then the surprise and. Right, and so, um, she, uh, had a, uh, a, uh, drain placement from our interventional radiology colleagues, um, as we see from these particular images for the sake of time, we'll move on, on to her next imaging that was, uh, a week after her drain placement. So this was a tough, uh, we don't unfortunately we don't have one of our interventional radiologists, uh, here, but, uh, very challenging, um, through the back just adjacent to the spine just above. The kidney. Wow, small window, probably traversed pleura too as our IR colleagues feel, but, um, that, that saved the day. That saved the day and, uh, you know, we waited things out another 6 weeks and that resolved it. She drained pancreatic juice from that drain for 6 weeks. But the entire inflammatory mass, you know, that had, had all resolved by then, and it probably was pretty hostile. But if you weren't able to do that, would you have considered trying laparoscopically, placing it in through up through the hiatus, almost like a Nissan approach, right, because that's really where you could have just lay a drain up there. That's probably like I was thinking, how would you. That would have been maybe, but my guess is it would have been stuck down and really inflamed in there. Yeah, I know, clearly, clearly we anticipated a lot of hostility. The other question that comes up here, this collection was very similar in location to the prior collection and so why did, you know, someone might be wondering why did we choose the percutaneous approach as to the trans gastric approach, right? You have another collection that's right up against the stomach, and yet here you went to a more complicated. Procedure where they had to go paraspinal and get into this. So this was this well formed with a wall around this was, yeah, at this point, yeah, so you know the the decision making there was from the standpoint that you know we had a planned total pancreatectomy coming up as opposed to the first case where, you know, we felt that she might do fine with draining, you know, the initial patient's pseudocyst and she might have a resolution of her symptoms at that point, but this patient. Um, you know, this patient was heading toward TPIT, and so we felt that introducing a, um, a connection between stomach and pseudocyst might create, you know, a lot more scarring in the retroperitoneum and make it a little bit more challenging. So that's why we took this percutaneous approach in this circumstance. That's pretty impressive, uh, perk drain place. Yeah, our IR docs are, can we go back to the slides? Shout out to the IR docs. So these are just limited CT images showing the drain they're indicated with the pink arrow on the coronal reformatted image and the dramatic improvement in the collection and inflammatory process. The blue arrow on the left hand image indicates the persistent defect in the pancreas that's the source of all this. And then another follow-up CT, um, as we continue to watch this collection resolve, you see the drain again indicated by the pink arrow and the inflammatory process extending, um, up toward the chest, no longer into the chest, um, but around the, um, gastroesophageal junction continues to, um, resolve and decrease. Great. And as, uh, Doctor Nathan was suggesting, um, this, um, uh, effectively allowed her pseudocyst to resolve, um. And allow the inflammatory process to resolve to the point where she underwent her TPIAT soon thereafter and then has actually had a very good outcome. Yeah. So the, uh, next case that we'll talk about. Uh, is a 10 year old female that, uh, presented, uh, she's about 8 years ago or so, um, with, uh, uh, symptoms of chronic pancreatitis, evidence of chronic pancreatitis, uh, secondary to a known PRSS 1 mutation, so another PRSS1 mutation. She had been managed, uh, with serial ERCPs and stent placements at the outside hospital for management of what ended up, uh, being a dominant, uh, uh, pancreatic head stricture. Uh, and she had actually quite good response to, uh, stenting with, uh, a relief of her pancreatitis, uh, episodes for quite some time, uh, but then came to us, uh, after she had really become refractory, uh, to the interventions, uh, and the stenting, the repeated stenting, and part of this was, uh, because, uh, there ended up being, uh, frequent, uh, stent occlusions, uh, and so it became a, a, a real challenge to, to manage it. So this was imaging. Go ahead, Andrew, and this is a collage of the images that were performed in this patient showing also to some degree demonstrating the correspondence between MR and ultimately ERCP. The big advantage of ERCP, it allows you to intervene. The advantage of MR, it's noninvasive and you can get quite good correspondence between the two. But on the on the left here, a coronal reformatted in an axial. a coronal acquired and then an axial fluid sensitive MR sequence in this patient shows dilation of the pancreatic duct indicated by that yellow area. The pancreatic parenchyma is atrophic. There's not much parenchyma surrounding this duct, so we have imaging findings here that would be compatible with chronic pancreatitis. ERCP images here on the right hand side have again indicated the very abnormal pancreatic duct, which is dilated, irregular, multiple sacculations. With the yellow arrow, the blue arrow indicates a stricture, so the very dilated duct comes down into the head down toward the papilla, and we see this very tight, narrowed segment of the duct with proximal dilatation, and subsequently a stent was placed across that area of narrowing indicated here by the white arrow, resulting in some decompression right away of the contrast that was injected into the duct. And so for this case we'll have a poll question. What's the next best course of management for this patient's, uh, process, uh, lateral pancreatic geinostomy or pus or modified puste. 2, a whipple. 3, a duodenum preserving pancreatic head resection. Or number 4, total pancreatectomy with eyelid autotransplantation. So we'll head on to what we did. So based on the concept that she had done very well for a number of years dealing with this isolated stricture in the head, uh, with stenting, uh, and based on the fact that she still had a predominantly, uh, a dominant stricture in the head of the pancreas, uh, we elected to, uh, offer, uh, duty and preserving pancreatic head resection, uh, uh, the burn procedure, uh, and so these are not from her operation, uh, but from, uh, a similar operations, uh, from a colleague. Uh, and you'll see here that we have, uh, stay sutures, silk stay sutures, uh, in the, uh, head of the pancreas, anterior aspect of the head of the pancreas, uh, that then allows us. Sorry, that then, uh, allows us to, uh, uh, gain upward traction, uh, on the, uh, uh, area of the head of the pancreas, uh, and then a variety of techniques can be utilized to go through parenchyma, whether it's cautery, uza, etc. uh, so that you can effectively get down to that area of stricture, uh, and excise it. And you'll see here in the next slide, uh, this is a Cusa technique of, of, uh, uh, taking dissection into the head of the pancreas and basically coring out the head of the pancreas, uh, and getting into that pancreatic duct. So you're effectively, uh, opening up that pancreatic duct, uh, to this cavity. And then at that point, uh, we will, uh, sew up a run, uh, basically, uh, anastomosis to the, to the cavity and, and such. Why is, why was, uh, Pousteau not a good answer? So, um, you know this was a dominant stricture in the head of the pancreas. It was not particularly a kind of a chain of lakes uh appearance. We know that this patient has a PRSS1 mutation. So again, a patient that may end up needing a total pancreatectomy in the future. Uh, and we know that there is certainly data that puste patients who have undergone a puste that is then followed by a total pancreatectomy actually they have compromised eyelet yield. OK, and so that was, so thinking if that wasn't a consideration, uh, the, the islet cell transplant later on, um, if you have a stricture at the head. Wouldn't it still backflow out through the puste or no? Yeah, the, the challenge is, how far up toward the head can you get that adequate drainage. Uh, and so some of the failures with pustes, and I'll show it here in a second, is that you, you just don't get enough, um, um, uh, adequate length of your drainage, and if you don't, those patients will fail. And so there's actually a pretty high failure rate. Except under pretty specific circumstances, I'll go through that a little bit in subsequent slides. If I can just comment on your point, Todd, it's actually when we really are considering the patient and just to clarify for all the audience too, when we're considering that this patient is really complex enough and had enough impaired quality of life, and we'll show that in the last section to be considered for a total pancreatectomy. We probably would not be going the route of a limited surgical resection and then waiting on the total this patient clearly had evidence that she responded to stenting. She responded to interventions to that particular stricture for a very long time. She was adolescent. It was 8 years ago, you know, before some of the more recent data now from Minnesota, of course, with the outcomes in children over a series of 75 patients that suggests, you know, good outcomes after TPIT. So you know, uh, based on our hesitancy a bit at that point, a number of, a number of years ago, we were, uh, uh, not pushing ahead with TPIT at that point in this age. Yeah, if you saw this same patient today with this same anatomy in 2016, you might reconsider, might reconsider, might reconsider, yeah, but it's still not a bad option here in this, in this circumstance with an isolated stricture, OK. So, um, uh, just to finish up, I know time is running out, but just, uh, some of the concepts of surgical goals of management of chronic pancreatitis, uh, and so, you know, certainly. We think about surgical options, non-TPIT options. Again, all surgical options get considered only after maximum medical therapies and endoscopic approaches have failed to relieve pain and address specific complications. Approximately 50% of chronic pancreatitic patients will eventually require surgery. Those indications are obstructive indications, so bile duct obstructions, duodenal obstructions, certainly pseudocysts, as Tom described already. Suspicion of malignancy, less so of course in the pediatric population, but really the most common indication is debilitating pain that fails to respond to medical and endoscopic treatment options. Certainly, uh, we want to preserve as much pancreatic tissue as possible to to preserve exocrine and endocrine function and resolve any adjacent complications. There are really few studies that have examined optimal timing for surgical conventional surgical options for chronic pancreatitis. Some have felt that earlier surgery may be beneficial, you know, because it basically avoids delay and progressive destruction from the inflammatory component. Uh, but you know, honestly, most because surgery, uh, uh, surgical approaches are not all uniformly successful, uh, others have really advocated a watch and wait, approach. So there's really no clear consensus on timing of surgery for, for chronic pancreatitis. There's also no single surgical procedure recommended, of course, for all patients with chronic pancreatitis. Uh, we try to, uh, select, uh, procedures based on severity of pain. You know, what are the pancreatic duct abnormalities look like, dilated duct, not dilated duct, etc. Where is the tissue? Where is the pancreas most diseased? Uh, is there a dominant, uh, head of pancreas, disease? And again, that's where we, uh, uh, consider some of these duodenum preserving pancreatic head resections with dominant disease in the head of the pancreas. Uh, are there any local complications, uh, pseudocysts, etc. And what's the overall condition of the patient. So really this, uh, boils down to a number of surgical procedures, bypass procedures, uh, so-called drainage procedures or puss. We have partial pancreatic resections, uh, whether it be distal pancreatectomies, uh, or Whipple procedures, uh, and then there's the combination of drainage and partial resection, so the burn, uh, the beggar, and the fry, uh, and then of course, uh, the total pancreatectomy is an option. Uh, so in the interest of time I'm going to just move ahead, uh, just a little bit. To pancreatic pseudocysts, uh, we really only consider surgery if symptoms are persistent, enlarging cysts, or if complications occur, and endoscopic and interventional approaches have failed. So those can be open cyst gastrostomies or cyst geostomies. But then really the treatment of pain. So when we're going beyond just an isolated stricture, an isolated duodenal obstruction, and patients have really chronic pain. Uh, then, uh, the question is, what is the appropriate option to offer. So we really have to assess the morphology of their disease again. What does the duct look like, uh, or, you know, is there, uh, dominant disease in the head of the pancreas? Uh, so this is a picture, of course, of the Puste, uh, that, uh, whereby the pancreatic duct is opened lengthwise and a ruin wide duodenal limb is sewn longitudinally to, to that open duct, uh, and it's, it's straightforward, uh, relatively low complications. It does preserve pancreatic tissue. There is short term relief, and this is largely from adult studies, in 75%, but greater than 50% of patients do have recurrent pain. And that largely is related to inability to decompress the duct fully or continued inflammation in that head of that pancreas. And so we do consider puste's typically not in the setting of hereditary pancreatitis for isolated large duct disease or this chain of lakes appearance without an inflammatory mass in the head of the pancreas. What do we do in the setting of enlarged head? So this has sometimes been termed the pacemaker of disease. We don't see it as much in children. We see it more in the adult population, uh, whereby patients do present with dominant head of pancreas disease, and there really are several approaches for this disease variant. There have been some randomized controlled trials in adults, particularly out of Europe, but really few reports in children, and those two approaches are either Whipple or. Duty and preserving pancreatic heterosections as our patient underwent, and so clearly a whipple eliminates the obstructive head mass, but you're really offering, particularly if you're considering for children, a pretty substantial operation, multiple anastomosis, and certainly you're sacrificing non-deceased organs, bile duct duodenum, that require reconstruction. There is relatively high morbidity due to leaks. There is good pain relief at 4 to 6 years. Again, largely this is adult data, but there is late endocrine and exocrine dysfunction in a large percentage of patients, 50% of patients. So really this is uncommonly considered in children with a head of pancreas predominant disease. So what we'll finish on here is the duodenum preserving pancreatic head resections which are so-called drainage slash partial resections, uh, and then, and so the rationale here is you achieve benefit of head resection and decompression of the duct, but you preserve the bile duct and GI continuity. Uh, with the considerations that, uh, we have, Bger procedure was the, uh, one of the earlier, uh, operations whereby the, uh, head of the pancreas is cord out. You do leave a little rim of pancreas along the duodenum. You do actually divide the pancreas, uh, over the portal vein. And then you reconstruct, uh, with the duojunal limb, a suture to the distal pancreas as well as to that, uh, cavity in the head of the pancreas. So you have two anastomoses. Uh, there is good pain relief again, uh, adult, uh, data, 80 to 85% of patients well maintained at 5 years. Uh, it does preserve function, uh, with minimal endocrine and exocrine insufficiency compared to a whipple. But what has evolved from the bear is the burn, which is really a technical simplification. Uh, the head of resect the extent of head resection is about the same, but you don't, it does not require dissection over the portal vein, so a little bit, uh, uh, uh, more simplified from a technical perspective, uh, and it does require only a single anastomosis to this cavity with the ruin jujunal limb. Uh, if you do have an associated biliary stricture, you can go ahead and open that biliary duct into the cavity as well. Uh, good pain relief, uh, 85 to 90% equivalent pain and quality of life outcomes versus the bigger procedure. Again, this is in adults. There's no data in children. Uh, and just to finish up with the fry procedure, uh, this, uh, is typically, um, uh, a, um, truly a combination, uh, drainage, uh, and a head resection whereby, uh, you core out the head of the pancreas again for the dominant, uh, disease, uh, and then you also open the entire rest of that, uh, main pancreatic duct longitudinally, so sort of add, adding a puste, uh, type approach, uh, and then placing that run Y duodenal limb over this entire opening. Reconstruction, as you can see here. And so there is a little bit of data, uh, sorry, the slides are a little bit, um. There's a little bit of data of, uh, utilization of the Fry procedure, uh, in children. Uh, again, exocrine and endocrine function in the short term, uh, is maintained. These are very small studies, uh, one out of Utah, uh, uh, several years ago, or a number of years ago, uh, as well as, um, another study below that's a little bit larger, uh, but really, uh, we don't have any good long term, uh, follow up data on, uh, outcomes, uh, uh, in children. And so really the consideration that we make and when we consider conventional surgical options is, you know, what is the likelihood this patient may need a total pancreatectomy in the future and if we think that there is not a high likelihood. And again, you know, how do you determine that? Very challenging, but if there's not a high likelihood and they failed medical and surgical or excuse me, medical and endoscopic approaches, uh, then we do consider some of these conventional operations. But we do have to remember that there is initial pain relief in in. Most of these, uh, operations, uh, but really over the long term, again this is adult data, uh, and we don't have any in children, uh, about 50% of patients have recurrence of pain in, in the future and so this is where we, uh, uh, think about, uh, uh, rescue with a total pancreatectomy, uh, down the line. So with that I breeze through the rest of the the surgical details, a lot of data, a lot of information, um, but really I think the, the crux of the matter is, is that there are surgical options that are non-TPIAT for inappropriately selected patients, but again in the context of failure of endoscopic and, and, uh, interventional radiologic approaches. When you do the, do the, when you do the burn procedure, do you put a stent in the bile duct while you're doing the operation so you can. No, no, we don't, we, we, we can utilize endoscopic ultrasound, so we'll often be able to find that duct with endoscopic ultrasound, uh, excuse me, not endoscopic ultrasound, um, intraoperative ultrasound, right, right. And so, uh, and sometimes you can find that duct simply by sticking a needle in it. So if you need to guide yourself in terms of where you're going, you can find the duct by sticking a needle into it under ultrasound in the OR, OK. That was a great, the audience actually, so maybe we can read it. Um, it's more surgical. So from Jerry Snadoff, by my opinion, Berger Beger procedure is more appropriate for the developing. Organism of the children than a whipple. Can we also though add that throw in the mix of that genetics is involved in about 70% of those chronic pancreatitis patients. So whatever we're trying to cure with a limited resection, you're not going to change the genetic makeup of that pancreas. Absolutely agree. So that's just something to keep in mind when you're dealing with kids. Absolutely. Yeah, so I, I, I completely agree with the comment, um, is that, um, Whipple is, is not something that we typically offer, and it's certainly offered in the adult population. Milton, you, uh, so it would be reserved in pediatrics. I mean we're worried about cancer, if you were about a tumor, that's different, you know, the likelihood of cancer in a child, in a child is exceedingly low. So then, so, um, in our opinion, little role for Whipple, right. I don't know if Milton, if you want to comment on, uh, make a comment on the adult population, uh, since you spend a lot of your time, we do, uh, whipples depending upon the particular patient and their anatomy. I would say that we do more combination drainage slash core up ahead of the pancreas type of procedures, fry, uh, than whipple, but occasionally in head predominant disease when you're especially if you're concerned about cancer, then, uh, a whipple might be the more appropriate thing. So I, I always like to totally simplify, and this may be something we just can't oversimplify, but can you fly through in like one minute. The, the bagger, the fry, the whipple, the pusto, what when they apply. So basically you're looking at, I'll just start off, a whipple is when it's mostly in adults when you have a suspicious lesion that you really need to resect the head. Little role in the children. Pusteau has got to be across the, the length of the or chain of lace appearance, right? So if you have a predominant head of head, head of the pancreas stricture. Uh, you know, you could core that out and lay a duodenal limb up there, right, and, and, and it'll drain, but if you have the chain of lakes appearance, right, you really have to filet open the entire length of the pancreatic. By that you mean intervening strictures and dilation. Thank you, chain of lakes. Thank you. You sort of breeze through that, but going all the way to the head, all the way, yep, then Cousteau may be a good option for that patient, correct, uh, in the absence of here of genetic etiology, right? If exactly right, let's let's, let's start with that because if, if like you said, if we think that we're going to be doing a total pancreatectomy. This is all the whole thing changes, right? Is that what you're talking about it's all transplant. We are, um, um, less likely to consider these resectional and drainage procedures in a patient that has genetic etiology of pancreatitis. OK, that's in children. OK, that's the quote I'll tweet right now. OK, good. Uh, all right, this, this was a good review. Um, I, you know, it's hard to, so the fry, the fry would be in a, in a patient. Why do that? So if, you know, if you have, you know, inflammatory mass on the head of the small inflammatory mass in the head of the pancreas, OK, you want to core out that pacemaker, but you may have strictures along the rest of that duct as well. So it's sort of a, uh, you know, kind of beyond the hybrid, yeah, OK, that was great. That was a great summary. So with that, I think we'll close the session and we'll move on very shortly here to total pancreatectomy. That'll be our last session. So 5 minute break, Mark, one last comment. OK, no, we just, we just did that one. Is there another one? Yeah, OK, very good. 5 minute break and uh we'll come right back. Thank you. That was a great session.