The Colorectal Quiz Episode 21: The History of Hirschsprung Disease

Hey there listeners, it's Amanda Jensen from Riley Children's Hospital. I'm here today with Dr. Mark Levitt from Children's National and Dr. Jason Frischer from Cincinnati Children's. Today we are going to talk about Hirschsprung's disease and the history and where it all started. Dr. Levitt, why don't you lead the way? I'd love to take a moment to go through the history of the leaders and the contributors to the field of Hirschsprung's disease. And we have some really cool personal insights into some of these individuals. And if you are on the Stay Current app, you can click on the link and look at the compilation of all the leaders in Hirschsprung's disease that made significant contributions. Dr. Levitt, why don't you tell us more? It is a photo of 14 individuals who have contributed to this wonderful field of Hirschsprung's disease. All right, let's start with the first person. So the first picture is Harold Hirschsprung's, who's the one who essentially figured out that a baby could be sick due to this problem, but he did not understand the pathology. However, of course, the name of the disease is named after him. And by the way, it is Hirschsprung disease, not apostrophe S. The next picture is Orvar Swenson. He's the one that really figured out the pathology. He went to the pathology lab and he was the one that defined the fact that there were no ganglion cells prior to that removal of the dilated colon was the treatment, which obviously was a mistake. It was the distal colon, the narrow colon that was the problem. Now, interestingly, he developed the first operation for Hirschsprung's disease, which is a full thickness rectal dissection, which is the kind of operation that Jason and I do. But there's still some folks out there doing Suaves. However, the Suaves are becoming more and more Swenson-like, making maybe a one centimeter cuff. And to quote Dan von Allman, those are basically Suavesons with a one centimeter cuff. But why was the Suave developed? And just to be complete, it really ought not to be called the Suave because Dr. Yancey was the first surgeon who described a submucosal dissection for Hirschsprung's disease. But he published his article in a journal that not a lot of people read. And Dr. Suave published his article a number of years later in a journal that more people read. So it really ought to be called the Yancey. But the reason why this was developed is because people said that the Swenson was a problem because patients were developing fecal and urinary incontinence or voiding dysfunction after the operation. And Dr. Swenson himself said and wrote a very nice paper saying, you guys are wrong. It's a good operation. You're doing it wrong. You're dissecting too wide. You're doing too wide of a rectal dissection. They didn't know that at that time. And the people that developed the new techniques, namely the Yanceys of the world, the Suaveists, and then of course, Duhamel, did everything in their power to avoid the full thickness rectal dissection to stay out of that rectal plane and avoid injury to the nerve erigentes. However, doing a proper Swenson right on the bowel wall, a la a PSARP right on the bowel wall. If you see fat, you can get closer. You can get closer to the rectal dissection. The nerves are in the fatty layer. If you're too wide, you'll injure them. Anyway, Swenson used to write me and Alberto Pena letters that said, thank you very much for promoting the Swenson. He was 105 when he died. Please tell everybody that it's a good operation. And I believe it is. And I hope many people have converted back to the Swenson. The next picture after Yancey is Duhamel, who obviously had a very interesting idea to leave the original rectum behind and do a pull through next to it and then mate the two lumens. We talked about that last time. I think at this point, it's really only appropriate for an ilio Duhamel, although I would still do an ilioanal. That's the subject of another podcast. And then there's rabine. You know about rabine? Rabine did a low anterior resection for Hirschsprungs, leaving about six centimeters behind. Amazingly, some of those patients did perfectly fine. The ganglionated bowel pooped through the six centimeters of A-ganglionated bowel. But that operation has gone to the wayside. In Europe, everyone knows what a rabine is. All right. And then Dr. Bolle. And Dr. Bolle has unique significance, both for Jason and for myself. Jason. He united us. Yeah. In a way. And he doesn't even know it. So Jason. Jason remembers. What is your connection with Dr. Bolle? Mine's a little different than yours. Yours is obviously a student-mentor relationship. Mine, more in a social dog relationship. My now past dog, Jeeves, who is an American Cocker Spaniel, and Dr. Bolle's dog, who is also an American Cocker Spaniel, were siblings. And so we were related through our pets. Mine was named Jeeves, and he was awesome. So I learned a lot about Dr. Bolle outside of the operating room. But Mark had a true mentor-mentee relationship. Well, Dr. Bolle is really responsible, frankly, for pushing me in the direction of pediatric colorectal surgery somewhat inadvertently. So he was at Albert Einstein Medical School when I was a medical student there. He worked at Weiler Hospital or Montefiore. I don't remember which of the two. He actually was the one who said that the suave technique, which was to leave the bowel hanging out and coming back at day seven to do a coloanal anastomosis, was unnecessary. So he was the first one to do the primary coloanal anastomosis of a suave. So the proper description is it is the suave technique with the bole modification, i.e. a suave bole. And at our medical school, you did not say suave without saying hyphen bole. Now, interestingly, when I thought about what I would do with my career as a third-year medical student, I lamented the fact that I really liked surgery, but I also really liked pediatrics. And I was very frustrated that I couldn't do both until a very senior fourth-year medical student said, you know, Mark, there is a field called pediatric surgery. You could do both. And I said, that's perfect. I'm going to do both. And I looked for an elective in pediatric surgery, and there was one with Dr. Boley available. However, the student, another student, had blocked the spot of the month that was ideal for me. Who was that student? Hung Bae Kim. That's right. The liver expert and transplanter up in Boston. We were medical students together. And he and I were friends. And I said, Hung Bae, can I do the elective with you? And we'll be two students. It was a fourth-year elective. No big deal. He said, sure. But there's also another pediatric surgery elective in the book. Some guy named Pena. Why don't you see if he can take a student? So I called their office, and they said, yes, we can take a student for that month. And that was 30 years ago. And I did that rotation with Alberto Pena, who obviously influenced my career in a very positive way, basically because Dr. Boley's elective was blocked by Hung Bae Kim. And by the way, Hung Bae is the person that figured out the step procedure and is a brilliant mind. And I thank him for somehow getting me to Dr. Pena's elective. The next picture is Dr. Henry So. Henry So was a pediatric surgeon in the Philippines, and he was the first surgeon to do a primary pull-through. A trans-abdominal, by the way, the Suaves and the Swenson's and the Duomels were all trans-abdominal laparotibes at this point. And he's the first person to do everything with no preceding stoma. And the reason why he did that was because patients who were home with stomas in the Philippines were not cared for. There was such a social stigma against stomas that the babies were basically left to die by their families. Out of desperation, he just did the whole operation once and for all for Hirschsprungs. And he was the first person to do that. He then went to New York at Long Island Jewish Hospital, Schneider Children's Hospital, and joined the Becker group. And I was a medical student there. So I actually got to know Dr. So. He's a lovely, lovely man. Dr. Martin. Jason, do you have any insight into Dr. Martin? He's the next picture. Yes. I mean, we both know Dr. Martin, and we both have had the privilege of being in our conference room at Cincinnati Children's, where he attended many of the conferences that we both attended. But Dr. Martin was the first surgeon in chief and pediatric surgeon at Cincinnati Children's Hospital, and clearly a leader in the field. But he has some real colorectal establishment, not just in Hirschsprungs disease, but all related to Hirschsprungs disease. One, Dr. Martin developed the Martin procedure, which is a sort of an expansion of the Duamel procedure of leaving a long, just a longer aganglionic segment of rectum and pulling through ganglionated bowel for a long segment of Hirschsprungs disease. It's a super duper Duhamel. Right. It's a juiced up Duhamel. But Mark, you know, he has a tremendous contribution to ulcer of colitis. Yes. That's his biggest contribution for sure. So his contribution and published in 1977, so before the J pouch, he's the one who took basically the endorectal pull through that we all learn as pediatric surgeons for Hirschsprungs disease and transferred that technique to the surgical treatment of ulcerative colitis. And so he did the total proctocolectomy and use the endorectal techniques we use in Hirschsprungs disease and delivered that to the ulcerative colitis patient population and did a ilioanal anastomosis for ulcerative colitis. And he was the first person to describe that way before the J pouch then came along and sort of modified that technique. But the transanal dissection, the suave plane dissection is the same concept as the mucosectomy in ulcerative colitis. And Jason and I both suffered through, I mean, I'm sorry, we're trained. Trained. Trained. And how to do, we were trained by the same people, some fantastic, fantastic surgeons at Mount Sinai where we both, we both were trained. I met Jason when I was the chief resident and Jason was the medical student checking out the program who just happened to show up on a day that I was giving the grand rounds of surgery. And my topic was what? Pediatric colorectal surgery. Go figure. Here we are. Oh my God. Plus decades later. Yeah. That was in, I think, 1998, 798, probably 98. It was, it was a while ago. All right. Let's finish this list. Helen Noblet is the one who figured out the suction rectal biopsy. She's from Melbourne, Australia. Uh, Keith Jorgensen, a dear friend and a elegant surgeon who made many contributions to MIS, of course, but in particular to Hirschsprung's disease, he's the one that did the laparoscopic version of the Suave. Interestingly, in his original description, which by the way, Tom Inge is on that original paper. Our buddy, Tom Cincinnati, now at Denver. Um, they talked about leaving a five centimeter cuff, which nowadays would be way too much of a cuff. Of course, he talked about splitting it and we can, we've talked about all the problems with the cuff. And then of course, Jack Langer, our dear friend who approached trans anally. So he was able to do this operation trans anally. And around the same time, so did Luis de la Torre to do a trans anal resection of the rectosigmoid with or without laparoscopy with or without laparotomy. But the trans anal component, it's truly amazing what you can do. And there are obviously some places around the world that are doing trans anal only. I do that in certain circumstances. And we learned that from these two gentlemen. And then our final photo is my dear friend and, uh, Dan Teitelbaum who passed away way too early from a brain tumor who did an incredible amount of work in Hirschsprung's disease and particularly a significant amount of research in enterocolitis. And we miss you dearly. So this is the, the hit parade of all the contributors to Hirschsprung's disease. I think that's the who's who of Hirschsprung's right there. Yeah. Awesome. It's an awesome slide. And actually I remember putting up this slide at one of our courses and have this beautiful picture of this slide is in the background and the panel in the front at that panel sat Keith, Jack, Luis, and Dan. And they were sitting there in real life and behind them was this slide. Pretty cool. I invited Dr. Hirschsprung's, but he could not make it. Pretty awesome. Well, I have a feeling we're going to have way more discussions about Hirschsprung disease because just because, because I think it's a fascinating disease that there are so many intricacies. You just mentioned dentorocolitis. We could talk forever about that. So I look forward to many future podcasts. All right. And that wraps up episode 21, the history of Hirschsprung's disease for our colorectal quiz. This is Amanda Jensen from Riley Children's. Remember knowledge should be free. 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