Good. So this is a case of a twin, a girl who was born at 27 weeks, weighing, uh, just over 1 kg. Um, there was a prenatal diagnosis of a twin to twin transfusion syndrome, and she was the recipient twin. An ablation was performed at 22 weeks, um, and a stat C-section had to be done a few weeks later, uh, due to maternal septic shock. The mother had a premature rupture of membranes. It was thought initially that she might have had an amniotic fluid embolus, but actually it turned out to be E. coli sepsis for, uh, reasons that were unclear, and it was vertically transmitted to both, uh, neonates. Her rap guards were 3 at 1 minute and 1 at 5 minutes. Um, she was intubated at birth, and there were no other obvious congenital anomalies. The patient actually didn't have GI issues during the first couple of days, but on day three, because of, uh, iatrogenic manipulation of the, uh, umbilical venous line, she developed atrial flutter and significant, uh, tachycardia with some hemodynamic instability, and came here to our cardiologist to be cardioverted. At the time when she arrived, um, she was also noted to have, uh, quite significant abdominal distention, but was, uh, stable hemodynamically, uh, once the, the, uh, the, uh, cardioversion was complete. And uh this is a film of her abdomen as a pine and a cross table lateral, and you can obviously see a very large amount of, uh, free air, um, with a bit of an unusual shadow in the left upper quadrant that also looked like, uh, free air, but just, uh, almost like locculated. Um, so we took her to the operating room and, um, What we found was a, uh, this isn't a patient, this is actually another patient I had a couple of years before, but it was a similar type of perforation, except it was much worse. Uh, it was a longitudinal, uh, perforation along the greater curvature. The entire corpus of the stomach going into the fundus and cardia really was necrotic. It wasn't holding any stitches. We had more of what you can see here of, uh, essentially a stomach that was, um, that had completely necrotized with some margin at the cardia of what looked like a healthier tissue, and then a couple of centimeters of, um, of a good rum. Uh, so we really couldn't identify the proximal extent unless we were going to divide the left triangular ligament and fold the liver over, and, you know, we're talking about a 1 kg baby who's not super stable. Uh, so maybe I'll just stop here and ask people what would their options be in a situation like this. Yeah, it's a good point to stop, so. To you, so we've got subtotal gastric necrosis. You don't see the proximal extent, you said, correct? Yeah, no, so I could not see anything that looked like good tissue proximally. I mean, I would have had to do a lot more dissection, but the baby was already getting coagulopathic, and, you know, it was it was a difficult situation. Jack, any initial thoughts on this? Well, it's a tough one. I mean, it, what you want to do is, uh, is control the situation and, uh, get out of there as, you know, as quickly as you can. So, um, the stuff that's frankly, necrotic, I think you have to remove. If you can put a tie or a vessel loop or something around the upper part of it to try and control leakage of saliva, then that, then I would do that. Otherwise, I probably would just leave a drain into the upper abdomen. Um, and then, uh, staple or, or sew the distal end and put in a feeding judge in Austin. Would you put the drain, would you leave a drain next to this, or since you can't even find a proximal extent to it, put it actually inside the lumen and leave, uh, almost like a gastric. Uh, tube coming out through the wound. If you can, I guess if you can find the lumen, it's, it's going to amount to the same thing if you're removing the necrotic stomach and it's kind of disappearing up under the liver, which is, I think what Sharif is describing. I would just put the drain up under the liver, uh, you know, near the GE junction. I, I think it will likely drain whatever comes out. Yeah, I can't think of anything different to start. I mean, uh, because you don't know really what's caused the whole thing to cascade in the first place and where is it going, how far is it going to go? Any? Yeah, so, uh, go ahead, Sharif. All right, go ahead. Yeah, so I basically did a, a, a variant of that. We, we, we switched to damage control mode essentially, um. I'm trying to advance the slides. Oh, here, yeah, uh, guys, sorry. Uh, I need to go one slide back here. Uh, we'll take care of that for you, OK, if we can just go back to the initial slide, the next slide back, Mark. One more back. Again. One more. No, we need to go back again, yeah. So what, what I did is, uh, like Jack said, basically removed, uh, the part of the stomach that looked clearly necrotic, and then just before completely removing it, I, I put a Foley catheter up the, uh, the distal esophagus and just blew the balloon up, uh, 0.5 cc, and that seemed to lodge well. In the esophagus. They also left a Penrose drain right next, uh, you know, basically under the left lobe of the liver. And then again, there was about a 2.5 centimeter remnant of, um, Of rum, and I use that as essentially an ostomy tube. Put an ostomy tube in there and uh to to drain that. And there were also. Um, you know, the small bowel was like wet toilet paper. It was really difficult to handle, even though with utmost care, just from exteriorizing and putting the small bowel back, there were multiple hematomas in the wall that then several of them went on to perforate, so we had to do several enterographies to repair multiple areas in the small bowel. Of course, by the time we finished, the baby was Uh, was quite coagulopathic, and, you know, we we weren't sure if we're even going to make it out of the operating room, but we did get all this done. And uh the post-op course, I actually just read the chart again yesterday, and I, I wrote this in the chart because I told the parents that, you know, we've taken some heroic steps, but I think this is about as far as we'll go, um, and I said no further procedures planned as high likelihood of futility. But the baby actually, within about 12 hours, turned around amazingly. I mean, uh, she actually became quite hemodynamically stable. Um, she wasn't needing any pressors, was making good urine output, and, uh, looking, uh, looking extremely, extremely good. And then on the 3rd post-op day, we started to get bilious drainage from the Penrose drain. And on the 4th post-op day, she distended again and became somewhat difficult to ventilate. And once again, we saw. A lot of free air, but because the baby had actually done reasonably well, I sort of ate my words and, and, um, you know, in discussion with the family and the neonatologist felt that she deserved a trip back to the operating room. I thought maybe the gastric remnant had simply necrosed, or maybe I was not controlling the distal esophagus well, and I'm getting a leak from there, but, um, I, you know, we, we had to do something because this baby actually was fine and then suddenly deteriorated again. So, the second laparotomy was another surprise. Actually, um, there was no problem with the distal esophagus, and there was no problem with the remnants of the stomach. But now we had, um, new intestinal perforations, and they were not the areas where we had done the repair. Um, there was a significant necrosis in the proximal jejunum for about 3 centimeters. And it was just about 5 centimeters from the ligament of trite. It was very hard to bring up as a stoma, so we actually ended up doing a resection of primary anastomosis of that. And then in the distal ileum, there was what looked like a spontaneous perforation. Um, and there we just, uh, we just debrided that and repaired it, and left our gastrostomy and, uh, esophagostomy tubes all, uh, intact. Um, and she turned around once again very quickly, um, did, uh, extremely well. In fact, uh, even despite her prematurity, was only requiring CPAP after about 7 days of mechanical ventilation. So at 2 weeks post-op, we did contrast studies from above and below, and you can see that the esophagus here essentially became like an retic, uh, esophagus, uh, with a segment below. The diaphragm, and interestingly, even though we had in this little filling defect there is the balloon of the Foley, but there was really no leak around the Foley at all, and it, it coincided with the fact that we weren't seeing anything from the Penrose drain. So the esophagus had essentially Uh, fibrosed around the catheter. And then distally, uh, we put contrast in the stomach remnant, and everything went through. Uh, the resections that we had done had healed fine. There was no stenosis. Everything within a short period, uh, traversed the, uh, the bowel. Uh, so, uh, following this, um, we essentially removed the esophageal tube. We advanced it gradually and took it out. We started feeding through the gastric remnant, and that actually went well initially, but it was limited, limited by the gastric capacity. So we would get to about 34 ccs and essentially that was all the baby needed at the beginning. But as she got bigger, she needed more, and we would just start to get leak around the G tube right away, and it would, you know, it would not go well through the pyloris. So then we took her back to Floro and through the gastrostomy we advanced a gastrodujunal feeding tube. And that was successful in actually getting her off TPN within a relatively short period, giving her all the feeds through her, um, uh, small intestine, and we started to give her small amounts of sham fees that were just being suctioned through the repoggo, uh, in the esophagus. And then, um, the baby grew and uh was uh really progressing very well. There was no neurological issues. Uh, she really had no other anomalies, and in fact, had transferred out of the NICU, um, to the, to the regular floor. Uh, and so we started to think about a reconstruction plan. So I was wondering how people would suggest, uh, to reconstruct this. I got the answer. You call David, you get his balloon, and then you, cause you're, because wherever you do, you're gonna get a stricture your distal esophagus. Um, so is it gonna, you know, gastroesophagogastrostomy, esophagojeinnostomy, um, any other suggestions from the, from the faculty here, Kathy? Uh, it's a tough one. I, is there any way you can stretch the antrum to get the trim up there? I mean, did you feel that the distal esophagus would serve as a conduit, or did you think you were gonna need to remove some of the esophagus? It was hard to tell from looking at the. Mm, yeah, so I mean we did get another contrast and um. And the contrast did show, I'll show it to you in a minute, but it did show a very small amount of antrum, about 1 centimeter, that seemed to have survived. So we did have the GE junction and a small segment, not sorry, not antrum, but cardia, very small segments of cardia that appeared to have survived. So, so you have two, you have some proximal stomach and some distal stomach, right? Um. I think I would probably try to put those two ends of stomach together, but then if that didn't work, use some kind of jujunal conduit. Yeah. Any other suggestions? We have, we have a suggestion, uh, from Doctor Peterson, who says a Hunt Lawrence's pouch. Yeah, so, so I think Cathy brought up a, a good point whether the stomach could still be salvaged, and I thought about that, but I would have ended up with a stomach of about, um, you know, 4 cubic centimeters, and I, I, I think I would have just made the patient, the patient with microgastria, with all the attendant problems of reflux and, and other issues. So I. I didn't think that I was going to go that route. The Hunt Lawrence pouch sounded like a good plan. It's certainly been described for microgastria. We're all colored by our experiences. I've only taken care of two patients with with those that I hadn't done myself, and they really didn't do well. They were always having trouble emptying. There was significant stasis. Uh, they just were not progressing well. So, um, what was interesting is, as I was thinking about this, Our friends from Michigan actually published a case report, and I think, uh, that's why individual case reports, my, my mentor John Masan says pediatric surgery is a collection of case reports, and I think this is really illustrates that. Um, and they had published a case of microgastria that they treated with essentially what we would do for an adult with gastric cancer, a rule on why. Esophago or fundo or cardioinnostomy. And so, um, I decided to take that route for this, uh, little girl. We did this study just a few days before the reconstruction, and you can see that there was a small segment of stomach left, um, you can see it again here. Um, the esophagus actually otherwise was perfectly fine. It distended nicely. There were no strictures. Everything, um, in the esophagus looked fine. So we did a reconstruction at 5 months of age. She, uh, was 55 weeks post-conception age at the time, weighing 5 kg, and essentially we did the Roan Y fundugenostomy using a segment 15 centimeters distal to a ligament trite and a 25 centimeter limb that just seemed to be how it wants to sit. Uh, we left the gastrostomy tube in place. And, uh, the recovery, uh, was, uh, was, uh, quite uneventful. And on post-op day number 6, we did a study, uh, through the esophagus, and the emptying looked quite good. Um, and so we started combination feeds, uh, by mouth and, um, and back through the gastrostomy. Um, this girl has done amazingly well. Um, she is on a combination of oral and G2 feeds. She essentially takes a normal diet for her age. She's now 2.5 years old. Um, uh, she was discharged 52 days after the reconstruction, stayed in the hospital a total of 207 days. Uh, she's now on full oral feeds, as I mentioned, and just gets, uh, gastroscopy feeds at night to supplement her calories, but We're anticipating discontinuing that very early in the new year because uh she really is holding her own very well with minimal, um, supplementation. And, um, one of our newspapers here publishes Miracle Miracle Children's every year, and this is actually her, uh, at the last Christmas edition. Um, and she is bigger than her twin who did not have any of those problems. That's fantastic. Great save, Sharif. Yeah, it's interesting, you know, you get, uh, you may have, she won't have to worry about her getting an obesity surgery later. So, we may find out that what, what age they fail at because, uh, can you eat through a, through, uh, it is, it gets a gastric, gastric bypass. Yeah, yeah, great case. Um, unless someone has any comments that we're about 15 minutes behind, I was going to move ahead, but if someone wanted to make a comment. OK.
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