Bowel Management for Hirschsprung's Disease

But in addition, if we damage the mechanisms of bowel control, the patient is going to be fecally incontinent. All the operations designed to treat Hidbune disease. Emphasize the need, the, the importance of preserving the anal canal, preserving the anal canal meaning from 2 centimeters above depicting it lying down because the anal canal represents the most sensitive part of our body. We can recognize in that area the difference between gas, liquid, and solid. There is no other tissue in our body that can have that, that can do that. So if we damage the anal canal, the patient will have no sensation or poor sensation, and that means fecal incontinence. In addition, damaging the anal canal most likely represents the possibility that the sphincter was also damaged because the sphincter is located surrounding the anal canal. So that's why it's so important for us professors of pediatric surgery to be particularly obsessive about teaching our fellows and residents to preserve the anal canal in all these operations. We happen to have more reoperation for his spoon than primary his prunes to consider all the errors and problems that are provoking hispne disease and I have the next slide. Our sur surgical approach for Hib disease consists in doing a trans anal full thickness resection. In other words, you know that it's very popular to do a submucosal endorectal dissection. We prefer to do full thickness resection because we have learned in anorectal malformations that that if you stay very close to the bowel wall, you don't damage any structures in the pelvis. The end of rectal dissection was was uh designed for Hishpun disease but by Doctor Franco Suave because when Doctor Swenson published on his paper about the surgical treatment for Hishmun disease when people try to reproduce the operation of Doctor Swenson. Many problems occur. Many patients suffer from damage of important pelvic structures, nerves, neurogenic bladder, vagina, other neighbor structures. And in order to avoid that, not everybody could be so accurate as Doctor Swenson. Then Doctor Franco Suave created this endorectal dissection. But I believe that if you dissect the rectum close enough to the rectum, you respect everything and the patients don't have neurogenic bladder or any of those problems and we have done that many times. We do it all the time in the rectum. That's why we do a trans anal resection but full thickness rectum. And we go taking biopsies every 5 centimeters sending to pathology department but of course that you can do that provided you have an excellent pathology department. A big mistake and wrong generalization in pediatric surgery all over the world, including United States, is to believe that because there is a department of pathology, they, they, and because you have board certified pathologists they will be able to tell you the right answer in Hishbu disease that is grown. You need a pathologist that has experience with Hishman's disease and then. There are pathologists that have experience for frozen sections. There are pathologists that have no experience for frozen section. There are pathologists that have experiences with the hematoxyline neocene. So you have to, to, to find out what kind of experience your pathologist has. So this technique in which we are taking biopsies every 5 centimeters, send it to pathology for frozen section. Can only be implemented if your pathologist has experienced with frozen sections. Otherwise, otherwise you have to implement your own methodology treatment here you are seeing an image of the beginning of an operation for his bone disease. We use eight hooks that that separate the, the edges of the anus, and you can see they're depecting it line. So the distance between the skin and depicting a line, that's what is what we call anal canal that's is extremely important to preserve and to be sure that we preserve what we do after you see after you we put these hooks like you see there, we move those hooks deep into the to grab the pectinate line. At the next you see now those hooks are an independenting line. So now you are, you are seeing only rectal mucosa. All the anal canal is now folded and protected by definition. And then from there we go 2 centimeters deeper to put a series of stitches to in order to apply uniform traction to facilitate the dissection of the rectum. Remember, traction creates the plane. No traction, no plane, no plane, no good dissection. No good dissection. No good surgery. No good surgery. Complications. Complications. Bad reputation. Bad reputation. No patience. No patience, no money, no money, no wife. So you better put distinction traction. So you apply the traction and then start this is the dividing the rectum full thickness there. They want you to use the pointer and oh, I can, yeah, I can help with that next. Mark, can you give me the pointer? Yeah OK, oh, it's a video. It's a video, so you will see a very short video of the way we do this, uh, we the, the beginning of the operation is the crucial part, and, and 80% of the times through the transcendent approach you can reach the normal ganglionic bowel. Um, 20% of the time we have to go through the abdomen with laparoscopy or laparotomy depending on your preferences and your skills. They're loading up the video. So, in the meantime, I see that there is a question. You tell, oh, the video is ready? Yeah, it's playing. Go ahead. This slides show the normal rectum, normal anal canal and sphincter mechanism. The trans anal resection of the rectosigmoid must be performed dividing the rectum 2 centimeters above depicting at 9. The external appearance of these patients perineum shows a patulous anus and an extremely severe rash consecutive to fecal incontinence. This slide shows the anus of a patient that had a technically deficient transanal resection of the rectosigmoid. The resection unfortunately included the entire anal canal and the pull through bowel was anastomos to the perianal skin. The patient suffered from permanent fecal incontinence. We have done 125 trans anal resections. This video will show what we consider the essential steps of the operation to preserve the continent's mechanism. We strongly recommend the use of a lone star retractor. The 8 hooks are placed circumferentially at the end of cutaneous junction. Depicting line is exposed. The next step consists in replacing the hooks of the retractor, taking rectal mucosa in order to hide and protect the pectinate line. A series of fine sutures are placed in a circumferential manner higher than 2 centimeters above the pectinate line. Applying uniform traction on the sutures, a circumferential incision of the rectal wall is started. The dissection of the rectum is performed, remaining in intimate contact with the rectal wall, dividing and burning all the extrinsic blood supply of the rectum. Remaining in intimate contact with the rectal wall prevents damage to important nerves and pelvic structures. In order to do this, all the fat tissue must be dissected away from the rectum. Once we have reached the desired level of resection, a two layer anastomosis is created between the pull through bowel and the rectal wall. Here you can see the first couple of stitches of the outside layer. The sutures are placed by quadrants followed by stitches in between. This is the anterior stitch. And the lateral stitch. The outside layer has been finished. The rectal wall is divided and the inner layer suture line is started again by quadrants. This is a lateral stitch. This is the posterior stage. The anterior one. And the last lateral stitch. Then the sutures in between the quadrants are placed. The hooks after these are removed following this principle, the sensation area of the anal canal and the sphincter mechanism are preserved, which guarantees bowel control. Yeah. Well, I hope you, uh, were able to see the important details to to preserve the anal canal and for as part of our. As part of our protocol of study of patients that come to our clinic, patients who were born with Houston disease were operated for Houston disease, the patients are 457 years old and fecally incontinent. We have a specific protocol for them. We order a contrast enema to see if the patient belongs to a constipated patient with fecal incontinence or hypermotility non-dilated colon with fecal incontinence. And in addition, we put the patient to sleep to see if the patient has an intact anal canal or the anal canal has been destroyed. And we have an enormous number of patients with a completely damaged anal canal. When we see that there is no anal canal, when the surgeons pull the bowel and suture it basically to the skin, you can say that the patient is going to have fecal incontinence for life. It's going to be, is going to receive bowel management for life. If you see the anal canal intact and the patient is very constipated, then you have a reason to believe that perhaps actually the patient is suffering from overflow pseudo incontinence and with laxative with the right amount of laxative, the patient may behave like a continent patient. If the patient has tendency to diarrhea, have constant very liquid stools. And intact anal canal by giving constipating diet and Imodium, chances are that the patient will be able to control. And in addition, we give them 3 meals per day and a special fiber to make the stool bulky. But if the patient has no anal canal, don't waste your time with laxatives and all that, because the patient will be incontinent, most likely needs bowel management and the bowel management, the type of bowel management will be discussed, uh, later today. Um Next night. Next month? Yeah. So the complications and the complications and sequela in patients with his spoon disease, I personally like to divide it into preventable, non-preventable, which is enterocolitis. We don't know why patients have enterocolitis. It's a kind of a mystery we are looking forward to. The new generation of pediatric surgeons with the knowledge and dedication to research, basic science research to tell us what's wrong with these patients that developed this, they, they grow bad bacteria in the colon and they have this terrible complication that may kill patients. Um, then there is something there or constipation, I believe that is partially, partially, uh, preventable because we have learned that in fishbone disease we are obligated to resect the, the ganglionic segment of bowel but also to. Resect the normal ganglionic dilated piece of bowel because if you do a pull through with a very dilated piece of bowel that piece of bowel dilated doesn't have a normal peristalsis and the patient will suffer from constipation. But even following these rules, some patients have constipation. I don't know why, and that's why I call this partially preventable and then becomes the preventable preventable complications, complications that should never occur. The complications that are our responsibility, which is dehesence stenosis. Retraction, fistula formation, fistulas to the vagina to the urinary tract, all those are technical errors from the surgeon, so that should never happen. We should, uh, we should train our fellows to avoid that kind of problems. And finally, fecal incontinence because of destruction of the anal canal. If you look into the literature about. About the publications for his disease you will not find many publications discussing this subject because we don't like to talk about that but it's extremely important. So, as you know, enterocolitis, I don't have to say much about that, but you have seen these patients with Hirschp disease. They suffer after an operation, after a technically good operation, the patients suffer from abdominal distention, bacterial proliferation, sometimes, specifically, they develop, um, C. difficile and release of toxins and death, so. We are very obsessive about the prevention and treatment of the uh of this complication. We are very proactive in the management of this. So we, when we do a pull through in a patient with Hodgkin disease, most of our patients, we do it without a colostomy, but we keep the patient. In the hospital taking X-ray films, looking at the radiologic image of the bowel, and we don't hesitate to start irrigations of the rectum to as soon as we suspect that the patient is developing enterocolitis. We don't want to wait until the baby is extremely sick. And we teach the mother how to do rectal irrigation because rectal irrigation is the most valuable maneuver saving lives maneuver in patients with Hisb disease and all mothers should learn how to do rectal irrigation. You will be surprised how many institutions, very academic, famous institutions, confuse the, the, the, and they don't know the. Difference between enemas and irrigations and they use both terms in an indistinct way and that's wrong because if you give enemas to a patient with enterocolitis you may make making the patient worse in fact you may produce a perforation of the bowel the bowel is very sick so irrigation we you will see a video that illustrates what irrigation means for us. And our nurses are experts in doing that and we teach the mothers and then the mothers learn when the baby needs an irrigation very early. They don't wait until the baby is very sick. The child is not eating well, looks a little distended, makes a lot of noise in the bowel. The mother does the irrigation and the patient feels much better. So we are very, and we also give metronidazole in addition to the irrigation. So specifically when a patient has a huge operation in our institution. We discharge the patient with indications, proactive indications, prophylactic, 3 per day and and Flagyl, and a month later, the patient comes back to the clinic. We take an X-ray film. If the gas pattern in the abdomen looks normal, if the child is eating and growing normally, we decrease the number of irrigations to 2 per day and decrease the amount of Flagyl 50%. And then a month later it comes back, another X-ray film. The baby's doing well. Again, we decrease the number of irrigations to one per day and decrease the amount of Flagyl. And by doing that, we have, we have not lost one patient from enterocolitis. Want to see the video. Yeah, before we play the video, let me just make one thing clear to everyone. Um, I know that. With our new technology now, the faculty is a little confused. All the faculty out there, um, we have not brought your cameras in until we're ready for the panel discussion. So in about 5 minutes, we're gonna be bringing all of you in, uh, to talk and share what's going on and answer questions. So we're bringing all your cameras in. We can see you, but the rest of the world cannot see you yet. So, uh, as soon as we're ready for the panel discussion, right now, we're gonna roll the video. If your child has been diagnosed with entercolitis, we recommend that irrigation should be done 3 times a day and more often if needed. If your child has entercolitis symptoms of fever, belly distention, not stooling, vomiting, explosive diarrhea, foul smelling stool or gas, you should irrigate first, then seek medical attention immediately. In order to perform a colonic irrigation, you will need to gather some supplies. You will need normal saline. A few bath towels or washcloths. Two basins or small tubs. A 60 mL syringe with a catheter tip. Lubrication that is water-soluble. A silicone catheter you will need a 16 French catheter if your child is under 1 year of age or a 24 French catheter if your child is over 1 year of age. Start by warming the normal saline. It is suggested to sit the bottle of saline in a sink of warm water. It is very important to test the temperature of the saline on your wrist to ensure that it is not too hot. Pour the warm saline into one of your basins. Lubricate the appropriate size catheter and gently insert it into the rectum. Pause and allow for any stool or gas to run out the end of the catheter into the basin. As you advance the catheter, allow for any other pockets of stool or gas to empty. If gently pushed, the catheter should follow the curve of the colon. It is important not to force or advance the catheter further than the wide divider port of the catheter. Note that the best position is to have your child laying on their back with their knees bent to their chest. Draw up 20 mLs of warm saline into your catheter tip syringe. Connect the syringe into the end of the catheter and inject the saline into the catheter. Disconnect the syringe from the end of the catheter and allow for the saline solution to drip into an empty basin. You will repeat this process by advancing the catheter about 1 inch each time before injecting 20 mLs of warm saline. It is important that between each installation of saline to allow for the solution to drain from the catheter into the basin. If the amount that is draining out is not equal to or more than the amount of saline that you put in, continue to move the catheter in and out while twisting to drain the pockets of saline, gas, and stool. If you feel this has not allowed the colon to drain well, you can attach the syringe to the catheter and pull back very gently on the syringe. It is important not to pull on the syringe if you feel resistance. Repeat this process until the fluid that is draining back out from the catheter is clear. Once the return fluid is clear, remove the catheter from the rectum. Wash your supplies with soap and water and allow to dry. These supplies can be reused. OK, so I just wanna draw attention to something that we feel is very important that we teach our families for the irrigation um when our parents suspect um signs of entercolitis or there's distention. And they are concerned that their child needs to seek care, we always instruct the parent to give an irrigation first in the home and then go to seek attention. We often have patients who show up in an emergency room or a care office and there is not supplies there for them to perform the irrigation. And then the care is delayed and the child could be sitting for, for a long time to hours without irrigations getting very sick. So we always instruct the parents to give the irrigations before seeking care and to even take their supplies with them. Hold on. Sorry guys. OK, now it's it. Next. Go ahead. Next is. The next slide, OK. Yeah. Now, the um the um patients with fecal incontinence for Hibs disease suffer very much about severe diaper rash, particularly if they belong to the group of patients with tendency to diarrhea. And the, the, we surgeons usually don't discuss the problem of diaper rash. We let that, let that problem for the mothers and the nurses. But it's is, is, uh, the severe diaper rash like the one that you are seeing in your screen. Produces a very bad quality of life, makes the babies really suffer a lot as well as the mothers. And next. Now, this particular patient that you are seeing here has a completely damaged anal canal. Mark, yeah, um, someone keeps restarting the OK. It's a completely damaged anal canal and severe diaper rash that it's a diaper chronic diaper rash that develops areas of granulation equivalent to a second degree burn next. Here you see a patient that had an operation for his spoon and you don't see a pectinate line that means that the suture, the, the surgeon sutured the colonic mucosa to the skin basically next. Next. And these are the, the steps of a next. Now we're I'm gonna say a few words about another condition called total colonic ganglionosis. In this picture you can see um Doctor Lester Martin who made a contribution in the treatment for total colonic ganglionosis. It turns out that Doctor Lester Martin worked in at Cincinnati Children's Hospital. He trained at Boston Children's Hospital and become the pioneer of pediatric surgery in Cincinnati. So he's still um he's retired but he visits us once in a while so we had the opportunity to take this picture at our colorectal center and he is a very nice person. We have a great deal of respect for him. The idea of Doctor Martin uh talking about total coronic ganglinosis, the problem, as you know, in total coronica ganglionnosis is that we resect the entire colon and therefore these patients will have diarrhea for life. Then, um, in order to decrease the number of bowel movements in order to absorb water, in order to try to form solid stools, he came up with the idea of preserving part of the ganglionic bowel and take normal ganglionic bowel down and make a lateral lateral anastomosis between the the ganglionic and the normal ganglionic. Using taking advantage of the peristalsis of the normal ganglionic bowel and taking advantage of the water absorption capacity of the ganglionic segment, he will create a, a, a um a pouch or, or reservoir that would be able to form more solid stool. The idea was very good. Then Doctor Kimura used the same principle but using the right color. And so it's very ingenious to do that, but Mother Nature doesn't follow our plans. So every time we try to, to cheat on Mother Nature we learn that things are more complex than what we thought. So what happens is that when, when you take, when you retain stool in patients with his disease, when you have, um, holding the stool, it produces bacteria proliferation, inflammatory changes that produce secretory diarrhea. So we don't like the operation originally done by Doctor Lester Martin and Doctor Kimura or any other kind of patches of uh of patches because we have been in charge of resecting that and those patients don't do well. Uh, total coronica ganglionosis is a very serious condition, and we prefer to do an ileo direct ileoprocto anastomosis preserved in the anal canal with emphasis in the anal canal, but we have a, a, a very peculiar way to manage these patients that not everybody likes, but we are convinced that it is the best way to manage these patients. Regardless of the age of the patient, when the patient comes to us, we do a total colectomy, ileoprocto anastomosis, ileorectal anastomosis above depicinate line, and a protective ileostomy, and we leave the patient with the protective ileostomy until the patient is toilet trained for urine, which means usually more than 3 years old. So we are living in the era of competitions. Pediatric surgeons compete. If you operate on a patient, uh, 1 year old, I tried to do it 6 months. Another guy tried to do it 3 months, another 2 months, another newborn, another in utero, but we sometimes we don't take very much into consideration the quality of life of the patient. So we believe that if you close the ileostomy, uh, as you do this procedure in a, in a baby, the baby will have a terrible diaper rash even if you preserve the anal canal because the baby doesn't make any effort to hold the stool, will be passing liquid stool constantly, and the diaper rash that these patients, uh, have are the worst type that you have seen, whereas what we do is to wait until the patient is 3 years of age and comes to the clinic. And we and demonstrates us that he's toilet trained for urine. The child talks. He tells the mother, I want to use the toilet. He's accustomed to be clean in the underwear. And another condition is that she must accept rectal irrigations because patients with total coronic ganglionnosis have a very high incidence of enterocolitis. So if the patient doesn't tolerate rectal irrigations every time the mother is trying to do that in a child. That is 3 years old is gonna be a big drama in the family. So the, the, we instruct the mother to, to practice the, uh, simulate irrigations at home with a very soft catheter so the child understand that doesn't hurt. And so when the, the mother should come to the clinic with a toilet trained child for urine. And tolerating erectile irrigation, then we close the ileostomy and guess what? If you preserve the anal canal, within 3 days, the patient is totally trained for a stool. Parents sometimes don't like the idea to give a baby with an ileostomy for 3 years, and doctors don't like that idea, but actually the patient is very happy and we are trying to benefit the patient, not the doctors and not the parents. Next slide. Got it. So look at the kind this this patient total chronic ganglionosis, they did a pull through newborn baby and in addition they destroy destroyed the anal canal and the patient came like that a year or two later and is really suffering a lot next. So in our protocol, as I said, total colectomy, straight ileoprocton anastomosis, protective ileostomy next. And then a ileostomy closure only when the patient is totally trained for urine and accepts rectal irrigations. The patients without colon cannot have enemas, a small bowel um. Uh, the, um. The small bowel absorbs nutrients and there is no way to clean the small bowel and stop it from moving in between enemas like we do with the colon. Next. Um-hum. Hm. It looks like that's the last slide. That's it. So,