History of Hirschsprung Disease

Good morning. Good afternoon. Good evening. Welcome to Evaluation and Management of Hirsch Crohn's Disease. This is now the 3rd time that we have done a show on pediatric colorectal disease in collaboration with Cincinnati Children's Hospital. But this time it's different. This time we're doing this live from Cincinnati Children's Hospital at the Center of Telehealth, and as you can see this, this is a remarkable center, and we're very excited to be here today. And today we're gonna be focusing on Hirschprung's disease and the host of the show is Doctor Andrea Bischoff and Doctor Alberto Pena who put together a fantastic agenda today. Just a couple of housekeeping events before we get started. This is, uh, we're always at the mercy of internet technology. If you have any problems, just restart your browser and things should work OK. This entire show is being recorded and in 48 hours you can watch this at your leisure and anytime you want by chapter, you can look it up and, and watch it before a case or however you wanted to do that. Um, we want to encourage the audience to participate, answer the polls. You're gonna chat, let us know what you think, ask questions. This is the whole purpose of this is interactive. We have an incredible agenda, but we want to hear from the audience. So without further ado, I'm gonna turn this over to the directors of the course, Doctor Andrea Bischoff. Thank you, Todd. On behalf of the entire faculty, the Alberto Pena Colorectal Center and Cincinnati Children's, I would like to welcome everyone to this academic event. The idea of discussing Hisprung today came to us when we were in the World Congress of Pediatric Surgery in Berlin. And we had a pre-congress course on colorectal, and one of the topics was on Hisprung, and we were really impressed on how diverse pediatric surgeons were managing Hiprung's disease all over the world. As soon as we finished the course, we went. Back to the hotel and we ran into Dr. Todd Ponsky and it immediately came to our mind that we should host a global cast with Hiprung disease. So please participate, please enjoy, and I will let Dr. Pena speak a few words of introduction before we actually begin. Good morning everybody. Uh, we are very excited with the members of the Colorectal Center at Cincinnati Children's Hospital, for being sharing with you this, uh, important events related with. Related with the treatment of uh Hirborne disease, I wonder. What would happen if Harold Hib could be here today? Contemplating these fantastic advances in technology and hearing, repeating and repeating his name are related with a condition that he described so many years ago. We are in depth with all those through history that contribute to the progress in the management of these serious condition that affects so many children. We are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition, and there is no question that there are many surgeons, very prominent surgeons all over the world that have contributed to improve the management of this condition. The purpose today, our purpose is to Remember those who work on this condition before us to honor them and then to have a um sharing with all of you to want, we want to learn from you, we want to hear what you are doing and we want to share what our experiences have been and also finally we were taking advantage of this fantastic technology, we want to. motivate and contaminate the young generation of pediatric surgeons to move forward to deal with the most serious challenge in this condition, which is the basic science approach to the problem. To solve the problem of enterocolitis and many other problems that affect the children with the Hip disease. Now we know that it's not only ganglion cells or no ganglion cells, it's a much more complex condition and it's the new generation of pediatric surgeons who will clarify this for the benefit of thousands of children. So welcome to this event and I feel very honored to be here. So we're going to start now with a history quiz just to know how much everyone remembers. I think if we don't know history, we are always at risk to repeat the same mistakes. So please start answering the polls. The first question is in which year Harald Hisprung presented a paper on constipation in newborns due to dilation and hypertrophy of the colon 1756, 1796, 1836, 1886, 1906. Everybody can vote and if you are in a room with many people, ask people to raise their hands. So everybody can stay awake and participating as much as possible. So before you give the answer, I want to tell you what the numbers are. So you ready for them yet or you want to wait? Yes, I'm ready. OK. So far 100% of the respondents are saying 1886. So I think they know everything. We should just stop the show. Very good, 1886 in the Society of Pediatrics in Berlin. And everybody should remember that Dr. Hiprung was actually a pediatrician, and he had many contributions in pediatric surgery, one unrelated with Hirsprung's disease. He developed the hydrostatic reduction of ileocolonic into susception. Now another question regarding early theories to explain the etiology of Hirprung disease, which one came first? Number 1, obstruction dilated colon was due to a mechanical blockage from redundant colon or rectal valves. Number 2, malformation, hypertrophic colon was the primary congenital defect. Number 3, spastic distal column contracted in spasm and cause functional obstruction. Everybody can vote and we will wait for your answer. So it's starting to change. We're going to wait a minute. There's a little bit of, uh, I want to make sure everyone has a chance. But so far, OK, we're getting a few different, we're getting a varied answer here. We got about so far about 50-60% are saying malformation, the hypertrophied colon was the primary problem. 20, 30%'s changing a lot. 30% about the same spastic colon, 20% obstruction. Very good. So the real answer is malformation, but we must remember that all those theories were actually wrong because everybody at that time was obsessed that the dilated portion was. A diseased one. So all those theorists were trying to explain why the dilated portion was the cause of the disease and not the consequence of the disease, but the one that came first was number 2, malformation. Now who proposed colostomy or rectal tube and irrigation as possible treatments for Hichsprung's disease? Option 1, Robert Grass. Option 2, William Ladd. Option 3, Over Swensen. Option 4, Franz Koe. Option 5, William Osler. Everybody can vote. And Todd is gonna give us what's the poll looking like. It's moving along. I don't wanna go too quick because it keeps changing every second here. It's all over the place, all over the place. Very good. There's only one person, actually, one person that that has, that doesn't have a representative answer. The only person that nobody chose was Franz Ko, but every other person has been chosen. So let's see, Robert Gross, 40%, William Osler. is about 20%. Swenson It's changing so much. 25% and Ladd is 16%. Very good. And the answer actually is Doctor William Osler. He was one of the four founding professors of Johns Hopkins, and many people know him as being the creator of the residency. And now we're going to start again and we're going to have the polls again. So make sure you vote. Everybody raise their hands if you're in a big room. In which year the finding of no ganglion cells in the narrow rectal sigmoid was finally recognized 1946, 1926, 1906, 1896, or 1876. Everybody can vote. Do we have the polls now? Yes, it should be. Yeah 10. So. How do you polls have to be. Just use 123 And the answer is actually number 1, 1946 Over Swensen. Actually, others have recognized that there were no ganglion cells in the distal portion, but again, they didn't think that this was the cause of the disease. They thought that this was an Acquired condition. So even Dr. Dalale before in 1920 mentioned that, but it was only Dr. Over Swensen, the one that identified this as the cause of the disease, not something that was happening afterwards. The next question is, all these treatments were used for Hichsprung prior to 1946 except anal sphincter dilation, rectosigmoid myotomy, spinal anesthesia, lumbar sympathectomy, electric enemas, resection of the distal non-dilated portion. Everybody can vote, and we're going to wait for your answer. Looks like most people are saying option choice 6. Very good. Some are saying some are interesting though, Andrea. Some are saying 5 electric enemas. Have you ever heard of electric enemas? I have not. Is this some new thing, Dr. Pena? Do you want to comment on what is an electric enema. An electric enema was described in 1908 or around those years, and what happened is that somebody came up with the idea of passing a tube through the rectum to give an enema with saline solution, but inside the tube was an electrode, and the other electrode was in the in the abdomen. So by giving about 40 milliamps of interrupted the cycling current, they facilitated the expel of the saline solution from the colon, not much different from what some people are proposing nowadays. They are are going back to the time of believing that the electrical stimulation may improve. It's just a different way to do it. We don't know actually how much that works from the therapeutic point of view or actually to produce pleasure because enemas have been used for centuries for other non-medical purposes. So the answer here, actually, the only treatment that was not used is number 6, the resection of the distal non-ilated portion. Now when did barium enema technique become the standard diagnostic test for Hirschsprung's disease? Option 1, 1938. Option 2, 1948. Option 3, 1958. Option 4, 1968. Option 5, 1988. Everybody can vote and we're going to look at the results of your poll. So the results are coming in. It's a varied response here all over the place. We don't have, uh, we got, looks like 40% are saying choice two. And the answer is Choice 2, 1948. Dr. Swenson was also in this publication, and it's very interesting. At the end, I will comment on what was the rationale of Dr. Swenson to come up with the mechanism of the disease and also the treatment for it. When was rectal biopsy introduced as a diagnostic tool for his Sprung's disease. Option 1, 1915. Option 2, 1935. Option 3, 1955. Option 4, 1975. Option 5, 1995. Everybody can vote. OK, I think we're back with my screen, so we're going to move to the next question. Who proposed punch biopsies for newborns that required no closure nor anesthesia? Option 1, Wever Swenson? Option 2, Gerardi, Option 3, Dobbins and Bill. Option 4. Schendling option 5, Bodian. Everybody can vote. Mark, can you put that poll back up again I had it up. And we're going to wait for your answer. Mark, I'll do it. Just uh leave my poles up and I'll take them down. Yeah, thanks. Everybody can vote. And currently I see mixed results. And the answer is number 4, bearish handling. He was the one that proposed the punch biopsy. Now who performed the first Barry Shandling from? I have no idea. Is that, was that a, is that a, uh, something in the United States? We don't know, huh? Barry Shandling work in Canada, Canadian, Canadian, yes. So who performed the first successful operation for total colonic e ganglionosis? Option 1, Lester Martin. Option 2, Ken Kimura. Option 3, Rein. Option 4, Kayswater. Option 5, Syndergaard. So I suspect most people will get this one wrong. I'm just curious to see what's the answer. And I see many people voting in number one, Dr. Lester Martin. Dr. Lester Martin actually was the chief of pediatric surgery here at Cincinnati Children's, and every year he comes here to grand rounds at least once a year. And some people are saying Ken Kimura and some people are saying rein, and actually nobody got it right. The answer is Dr. Sandegaard, and he is from Sweden. And it was in 1953, and he did a colon resection with an ileo anal anastomosis. So that ends our history session, and I want to end with this picture because I think it represents very well the history of the treatment of Hirschsprung's disease. Everybody for such a long time was looking in the same direction, thinking that the dilated bowel was the cause of the disease, and then someone finally stood up and decided to look into the other direction and question maybe we are not moving in the right direction. And that was the person that changed history and finally came up with the right treatment of Hisprung disease, and I think Dr. Swenson was this person. And if you read his articles on how he explained how it all came to his mind, it's completely logical. So he said that he noticed that when the patient had a colostomy, the obstruction was relieved, and at that time they were just doing the colostomy and then closing the colostomy, and then the disease was back again. So when the patient had a colostomy, he decided to scope from the distal, from the rectum, and he saw that there was actually no true obstruction. So he excluded this option. Then he decided with a probe to put a probe on the proximal stoma, and he saw that there was normal peristalsis, and he put a probe in the distal one, and he saw that there was no peristalsis on that one. And then finally he decided to do a contrast study and he started to observe this non-dilated followed by a dilated portion, and that's why he concluded that the distal portion was the diseased one. So I think Dr. Swenson should be, we should all be very proud of him and how he stood up out of a crowd and found a solution for this problem. So were you impressed with the audience? I was. They were very good. They were very good. So congratulations.