Globalcast Transmission of Day 1, Pediatric Colorectal and Pelvic Reconstruction Conference, Nationwide Children s Hospital November 12, 2014

Transcription

1 Globalcast Transmission of Day 1, Pediatric Colorectal and Pelvic Reconstruction Conference, Nationwide Children s Hospital November 12, 2014 Dr. Ponsky: Just to give you the magnitude of this, when I look around this room it is rare to see something as specific as this many people watching and this is a fraction of who is watching right now, so we have about a couple hundred to... actually we ll never know the true number... but we have about two hundred unique people watching and unique computers watching us around the world. And, we have nearly 200 people in this room with us. One example is in Bejing, China, where there is an auditorium full of pediatric surgeons and pediatric specialists watching us. In Argentina there is a big auditorium full of surgeons watching. We have over 50 or so countries that are watching this right now on every continent. So, it s spectacular that you are able to share now with the rest of the world what s going on. Now this is interactive so if you all want we can give instructions so that you can log on and share the chat with all of the people that are participating right now and asking questions. I want to give a shout out to our colleagues joining via Globalcast. Actually in China, there is an audience of our friends and colleagues, and the catchment area for Ghanzhou Children s Hospital is one hundred million people. So, if we can make an impact there we are going to help a lot of children. We also have colleagues logging in from Argentina and from France which have groups of colleagues in rooms just like this following along. So, I hope everyone recognizes the significance of the video I just showed of a crocodile eating a wildebeast because from a surgeon s perspective it seems that we all want to personally be eaten by crocodiles before we possibly accept the fact that any possible complication can happen to us. We ve all sat in M & M conferences and said Ugh, that dehiscence would never happen to me. I use interrupted closures and I never get a wound infection. And I never have a problem with my colostomies. But the truth is we all know that s not the case. And what we are trying to convey over the

2 next few days is a lot of lessons learned. I can assure you that I have made every possible complication and have learned from them and have come up with a protocol to make it better for the next time. With us today we have a lot of experience in the room and that s why this unique group of faculty have been assembled so that we can hopefully avoid your making that same mistake and being personally eaten by the crocodile. So, today is ARM...the whole day is ARM. Many of our GI colleagues and Urology colleagues will be floating through starting tomorrow and the next day. This is really a session devoted to pediatric surgeons who care about anorectal malformations and, of course, we have some key Urology and Gynecology partners because there are a lot of those fields that we need to know about as pediatric surgeons and we have a specific session about that topic. Let s start with the management of the newborn. What I m going to show you now is a series of pictures of brand new babies and we are going to become experts in the next 15 or 20 minutes on the examination of the newborn. And this sounds very basic but we all know that there have been many mismanagement decisions based on an incorrect newborn exam. And I think we have to all become experts at this. It s really a key responsibility. First of all, I just wanted to tell you about this reference that I came across. This is a two thousand year old reference to the management of a baby born with an anorectal malformation so what you are supposed to do is take the infant without an anus, put some oil on the perineum, put them in the sun, and then where it s transparent you take a barley grain and slash across the opening and stool will come out. So I suppose that was the first anoplasty. Well, I m hoping we have evolved since then. This is not how we do it here. Right? Alright...Brand new baby, no prenatal suspicion of an anorectal malformation, 40 weeks gestation. So Jeff, what is this picture? Imagine we are at the art museum, what does the picture say to you? Dr. Avansino: So obviously this patient does not have an anal opening. This patient likely has some form of a rectal fistula to the urinary tract, either the urethra or the bladder neck. This patient s bottom is rather flat. He does not have a

3 well defined gluteal cleft, and as a result has a rectourethral bladder neck fistula, most likely. It s still early. So the baby has just popped out and is now in the NICU, and it s today. So for those of you I am going to pose the question in the form of an A or B, so A is: we operate today; B: we wait until tomorrow. Who wants to operate today; who wants to operate tomorrow? Okay. Does anyone want to speak about operating today? Those of you in the 16% group... Does anyone want to talk about the choice of operating today? Paola, do you want to talk about that thought process? Would you operate today? Dr. Midrio: No. To operate on the same day you might loose the chance to see if there is some meconium on the perineum. You can wait 20 to 24 hours. You really don t need to rush the operation on the day of the birth. You can wait a few hours. But what if tomorrow I am leaving for vacation and it s very inconvenient, and I can t get OR time, and the OR is offering me 2:00 PM today... Let s just do it, just do the colostomy today. Shall we? Well, I think the point that s being made is there is no rush. We shouldn t make a decision to do what we need to do on this patient today. As long as the baby s abdomen is soft and not distended, we have an opportunity to do a number of things which we ll talk about in a little while. I will tell you though this particular picture was done to be a bit provocative. There is no way there is going to be a fistula in the perineum on this patient. So, I agree with those of you who concluded that there is no perineal fistula happening here. There is most likely a rectourethral fistula as Jeff has said. I still would probably wait. We have other things we need to do today, like make sure there is no esophageal atresia, make sure we know what the kidneys look like, etc. But this is very likely going to be a colostomy and then a distal colostogram down the road. Let s do a poll of the audience again. Would anyone do a newborn repair? That would be A. Or colostomy, that would be B. If this kid looks the same tomorrow: newborn repair today or tomorrow rather... Colostomy would be B.

4 Dr. Ponsky: Marc, while we are waiting... Marcella from Argentina had a comment that she would manage this patient differently if it was based on a baby with Down s syndrome. I asked her why and she said because most of them have no fistula. Okay, so if this was a patient with Down s syndrome she would do a colostomy? Dr. Ponsky: So Marcella, why don t you answer that... Would you do?... I don t think it was in reference to... let me ask her what she is hinting at. But I think her point was what is the difference here, in patients... would it change your management if they had Down s syndrome with a less chance of having a fistula? I think that makes a good point. A Down s syndrome baby with a perineum looking like this... there is no perineal fistula in this child s future. I think a diversion probably makes a lot of sense. So, that s a good comment. Does anyone know, by the way, of patients with Down s, what percentage actually have no fistula? It s not all. It s certainly most. It s about 95% so you can predict no fistula but they certainly can have fistulas. Okay... Ivo de Blaauw of the Netherlands, here is another case, a newborn baby girl... Dr. de Blaauw: Yes, you see no anal opening and you can t really tell much more on this picture. On the type of malformation. You can see that the bottom s a bit flat. Just as in the other one, you don t see discoloring of where the anal opening should be but you should look into the labia to see whether there is a fistula or whether it s a cloaca.

5 Yes, I think that s wise. Although it has some cloaca features, you don t really know for sure until you do a careful look at the perineum. So, newborn repair tomorrow or colostomy? A or B. I promise you it will get more difficult. There is a fine line usually between courageous and crazy. And many people who are considered crazy are in retrospect courageous. Right? There are some plans for primary repairs out there. Does anyone want to defend their position? Although I find the audience responses in this very nice from an anonimity point of view, usually no one wants to claim their answer and there are usually a bunch of rebels who always pick the opposite answer... But we know who you are and, by the way, the fellows in the room, just so you know, your pads are linked to your program director s computers...so they are following along too. Okay. Sir Sutcliffe, representing the great country of the United Kingdom, so this is a brand new baby. What are your impressions? Dr. Sutcliffe: This is a brand new baby who is in a prone position with the appearance of an anal dimple with the suggestion of muscle and the scrotum doesn t look particularly bifid so the issue here is whether or not one can be confident that there is no fistula leading to the urinary tract or not. I would like to know how old this baby is? Brand new baby... just popped out. Ignore the fact that we re prepping for surgery. (laughter...) It was such a good picture of something I wanted to emphasize and that is the flatness of the bottom. Does everyone see that? I mean this is a pretty minimal muscular pelvis; so maybe Jack can say how you would guide the family when they ask you I hear you do a beautiful anoplasty but will my child s anus work in three years? What would you tell this family?

6 Dr. Langer: Well, I don t think you can be 100% sure but the flat bottom babies usually have a high fistula and they usually do not have normal continence. But, I would also want to look at the sacrum and see if it s normal because that s another predictor of continence. Jack brings up a very good point and we are going to have a whole section on that but I agree. I think every anorectal malformation baby needs to be thought of in the context of: (1) What is the malformation? (2) What is the quality of the sacrum? And, (3) what is the quality of the spine? And, we have called that the ARM continence index. And if you have all three of those factors in mind I predict as we run our data we will be able to predict continence. So, for example, a flat bottom, a poor sacrum and a tethered cord in the newborn period one could say bowel management with enemas in three or four years. Don t try to potty train this child. You likely will not succeed. As opposed to perineal fistula, good quality sacrum, good quality spine is a patient that you can be a little bit more confident telling that family the child will have continence. And I think of every single patient in that way. I can tell you that when I review submitted articles about ARM and continence, almost uniformly the sacrum is not mentioned, the spine is not mentioned and patients are just globbed together as a group of equivalent patients and we all know that that is not true. The bladder neck patient is very different than a perineal fistula patient. Here is another newborn baby. This is rare footage. I actually believe I have a video of this. Let me see if I can find it. Where is that video? Anyone see it? This is unbelieveable. Watch this. Never seen before. You can turn the sound down. Watch this. Has anyone ever seen a video of that? I bet not. So, that is a patient with a rectourethral fistula, actually in the process of voiding meconium. The scrotum is covered by meconium. So this is a patient that we know has a rectourethral fistula. Everyone can obviously see that. Jeff. Newborn baby.

7 Dr. Avansino: So again, just looking at this child we can see a bead of meconium at the perineum. This patient also again may be it s just the way he is positioned with his legs pulled back but kind of... appears to have a rather flat bottom... again it s hard to tell What about this is not a normal anus? Dr. Avansino: It is off center. So the whole is not in the center of the sphincter. Do you agree with that? So we want... you have to look at this and see that there is a pinkish elipse. Right? And that hole needs to be in the center of it, and if it s not, it s not in the right place. But, what is you opinion about whether someone described this as an anterior ectopic anus? Ivo, how does that make you feel? Dr. de Blaauw: In a male, yes there is a lot of discussion about what s an ectopic, anterior ectopic anus but if you really want to define it, I think you should define it as being not in a normal position but it has to be circumferentially surrounded by the muscle complex. We have some of those to show you. So this is a hole that s too small and it s not in the right place. Does everyone agree? So there is no doubt that this patient needs some improvement surgically on that opening, so let s poll the audience and it s now... this baby was born today and we have booked the OR for tomorrow. Tomorrow comes, it s an obvious perineal fistula and we call the OR. It s going to be an anoplasty... or it s a urethral fistula and we call the OR and say actually we are just going to do a colostomy but at least we have held the time. So, for this case, choice A: anoplasty; choice B: colostomy tomorrow. Baby just born today. Okay. Very good. So, Jonathan...

8 Dr. Sutcliffe: My thoughts are, if there is a degree of anatomic abnormality it would probably make me tend towards doing a colostomy and do things a little bit down the line. Probably want to get an ultrasound of the perineum just to make sure... it looks like there s possibly a bit of fullness just on the right on the sides and you might be able to feel whether there is a lipoma which wouldn t necessarily alter things but I would want to know that before doing any anal repair and that business around the bottom of the scrotum, it does look a little bit abnormal. I just want to know that the renal tract was okay and there s no bladder outlet problem. Okay. Sebastian King has traveled all the way from Australia via Toronto for this question. Dr. King: What about option C of dilatation and wait and then do a primary repair? Very good. Alright. Let s change the polling. Let s assume this is a patient with... everything is fine except for a perineal fistula. Perineal fistula, healthy baby. Choices are A: anoplasty tomorrow, B: dilation and anoplasty scheduled electively in the next six months. This will breed some controversy. There will be a 50 /50 split is my prediction. Actually, I predict 30 / 70, 30 /70. Then we can talk about the advantages and disadvantages of both approaches. Alright. So we re pretty split, so a lot of people would do an anoplasty tomorrow and some people will do a dilation and a delayed repair. Jack you want to give us your thoughts on that choice? Again, this is a straight forward perineal fistula, no other issues. Dr. Langer: The first question for me is whether the child is pooping fine through it the way it is. Some perineal fistulas they actually can poop just fine as a newborn, you don t even need to dilate them, and I don t dilate them. I would calibrate but I wait until they are three to six months of age. I

9 wouldn t do that on a regular basis if they are pooping. This one is unlikely going to stool normally through this so what I was taught and what I generally practice is to dilate for three to six months. I just think that you can do a better job with your anoplasty if they are somewhat bigger. I think those are the key points. As Jack said, you know you want to make sure they are stooling properly. These are the patients that I have seen a couple that have actually perforated in the sigmoid because they didn t get the stool out properly as someone was managing them by waiting. So, you have to be very careful particularly if this is a premie but I think the dilation or calibration, gentle dilation strategy is fine. You don t want to injure the tract because that makes the repair much more bloody and more annoying. There is no rush. However, if you feel confident and its relaxed time and you have OR time and you want to do it, there is nothing wrong with doing a newborn repair. Also the tissues are a little different. It s a little more moist. It s a little more fluid filled and we all know what it s like to operate on a newborn. And sometimes Jack is right. It s nice to wait. My only suggestion would be, well, let s ask this as a question; How long should you wait? Question from the audience: If you wait for six months wouldn t you have a dilated colon? Aha, so that s what we are getting at. So let s say we ve decided to dilate and delay our repair. The choices are three to five months or eight to 12 months. A, or B. I think you are bringing up a very important technical point. So, does anyone want to defend the late anoplasty position. I predict that that person will remain annonimous. Alright. Let s talk about that because I... this is a very confounding problem because not infrequently these patient s diagnosis is delayed. They are sent home in the newborn period. No one noticed that there was a problem and this is particularly relevant in females. And, in six months they show up at the pediatrician and the problem is constipation. We ve all heard this story. They go see someone else or a gastroenterologist and say wait a minute, something is wrong with this perineum and they end up in a surgeon s office at about eight or nine months. We ve all been there. What is the technical challenge then for the surgeon in that situation. So Jeff, you want to...

10 Dr. Avansino: So in that situation you obviously have a dilated rectum and pulling a dilated rectum into the anal canal is going to be difficult so that that patient will either need a tappering or you need to try to decompress him and get a better rectum down and pull though. I also think that the fistula tract is more inflammed. It s just a much more annoying case then if you do it then as compared to when it is done earlier. They haven t been dilated. They haven t gotten inflammed and then I would also add another factor. It s not as pleasant to dilate them postoperatively because one year olds are stronger than most of us in this room. As you all have seen. Whereas it s really quite pleasant to dilate a four month old. It s not nearly as big of a challenge for the family. So, I would advocate if you are going to delay the repair which is perfectly reasonable, try to get it done in the first three to six months. I think that is going to limit how much frustration you will have with that case. Of course, if you get the referred patient at one year of age you don t have much of a choice. Dr. Langer: And I think that the key cut off point is where they go from being breast fed or formula fed to when they are started on solid foods because there is a very important change in the character of the stool at that point and that s when they start to get that dilatation of the rectum. Usually around six months. Good. Great points. Alright. Jonathan. Dr. Sutcliffe: I once had a patient that became very unstable in transit having lost the airway and the ph was 6.7 for a fairly long period of time so I didn t even think this child would survive but actually got through the first week really well. Wasn t well enough to go to theater for a stoma and I looked and there was a membrane that you could see meconium through at the level of the dentate so we perforated it we started dilatations and since then there has been some spontaneous stooling and absolutely no distention. So my

11 question is when we get to four months of age and this child is still able to withstand dilatations obviously without an EUA would you consider a repair mandatory and do you think it is possible just to leave it at that? That s a very interesting discussion. So, my response to that is that an anus is defined as a properly sized hole in the center of the sphincter that is mucosal lined. If you can confidently state that you have that scenario I don t think that patient needs surgery. My concern is that the patient has sort of fistulous tissue that will stay constricted or in need of dilation and in the mean time the child is growing and the anal opening doesn t grow with the child. But in a male, you don t have the perineal body concern like you do in a female. That scenario has occurred and then it looks like an anus. But I think if the anus is mucosal lined, is supple, accepts a dilator easily and you are confident that it is in the center of the sphincter, it s hard to improve on that scenario. Jack do you agree? Dr. Langer: Yes. I agree. I have often dilated kids with these membranes or with anal stenosis and there s probably going to be a discussion with that in a little while but I tend to dilate where some of my colleagues operate. Dr. Ponsky: Marc, in China they want to know about the use of perineal MRI to determine the sphincter location. I m going to defer to my Radiology colleagues. MRI to define the perineum. The muscle complex. How good is MRI in the very, very distal aspect of the sphincter... where ever the rectal muscle fits. Dr. Bates: We use MRI usually in the patients who are coming back after anoplasty and are having problems. And in that scenario it s pretty good in showing that the muscle complex is surrounding the rectum or not. In the primary situation, clinical exam should suffice.

12 You have to ask yourself what are the goals of treatment. And the goals of treatment are to get an anus that is supposed to do what anus do and if you achieve that you are done. The dilation process is sometimes very gratifying in a sick baby and then they release the meconium and their belly decompresses and then you don t have to rush to do anything, and that happens I think that s sort of a trick you need to learn how to do is how to get the meconium out gently without injuring the urethra. But remember in these cases that hole you dilated is not in the center of the sphincters. So, here is another similar case with a little bit of variation to a theme. So, let s poll the audience. Would you consider a newborn repair tomorrow or delayed repair but a colostomy now. A or B. Newborn repair or colostomy. Dr. Ivo de Blaauw: Yes, I would consider this a perineal fistula to the scrotum which are generally maybe in more than 90% low type of malformations and you ll find the rectum very low you can do a primary repair, neonatal repair. You should be prepared that you may find that there is a fistula in the perineum. There is no urethral fistula, but the rectum may be a little higher in some cases. Would you do any additional testing in this patient to feel confident about which scenario you are dealing with? Dr. De Blaauw: I would actually, you can do a cross table x-ray. You sometimes see the fistula by air and you can see the rectum at 1 or 2 cm. away from the perineum. You can do an ultrasound if you have good radiologists but this is debateable... you need a very good radiologist who doesn t push too much, who is qualified to do it.

13 If you re going to add a radiologic study as part of this patient s evaluation, would you do A: cross table lateral film, B: ultrasound at your institution currently? So this is the cross table lateral film. Does everyone know what an invertogram is? You literally hold the baby upside down, they vomit everywhere. Instead, the new way is to just put them up on a bump in prone position you can get very good data. We have some pictures of that. Dr. de Blaauw: And you should wait at least hours before taking this film. So, A: x-ray, B: perineal ultrasound. I am just curious to know what the group thinks. Let me tell you, if this slide and that question came up when we were in Europe the percentage would be heavily favored towards ultrasound. But, I am curious to know what this group would say. Here we tend to irradiate our children more frequently. I think the issue is that for an ultrasound you need a good radiologist, you need to make sure not to push. Maybe, Dr. Bates or Dr. Kraus or Dr. Adler, our three partner radiologists in the audience surrounded by a bunch of surgeons can make a comment. Dr. Kraus: Good morning. Well, I think with ultrasound the baby has to be quiet. If they are screaming bloody murder and valsalving you may get a false sense that the position of the rectum is lower than you think. Dr. Bates: The other thing too is that you have an opening in the perineum and what the cross table or the prone invertagram is supposed to do is supposed to tell you after a long period of time and high pressure in the rectum that you have a distended rectum and if you have a hole in the rectum then the air is going to escape there and its going to make you have the false sense that the rectum is higher than it really is.

14 Dr. Adler: I think in this scenario that by far the majority of the kids have a low rectum so the goal of your radiological testing here is for those very rare cases where it s a higher rectum and then you ve got a very thin little tract down to the perineum. But that scenario is extremely rare. I think if you are approaching these malformations from a posterior incision your number one responsibility is to know what will be the first structure you encounter. If you can confidently say that the first structure that you encounter is rectum then you are safe. If you don t have that information, you need more data, and the best way to get that data is a colostomy and a distal colostagram but in a patient like this and we will show you some cross table films you can know where the rectum is. Dr. Ponsky: Question from Tripoli, Libya. What is the value of an exam under anesthesia. Could that help change your plan in the operating room? I think that s a good question. What would an exam under anesthesia look like in a baby like this? They are very comparable cases so this patient to me looks like there ought to be a hole there eventually that somehow meconium can get out of... so I would call this a perineal fistula and I would call this a perineal fistula as well. Isn t it pretty. One is black and one is white. One is meconium and the other is mucous but it s the same anatomic situation. An examination under anesthesia I think in this particular patient with an electrical stimulator would show contractions here with no opening except there must be a rectum very close by and an examination under anesthesia here would show this discoloration to be the center of the sphincter and that to be the beginning or the end of the fistula. Question from the Audience: What do you do with the mucous in the raphe? Do you open it?

15 Yes, I do take that off and it just heals. I mean I wouldn t leave this here. Just unroof the meconium. From the Audience: Marc, I just wanted to see if you want to talk about the dangers of not doing any repair at all. Sure. Why don t we talk about that right now. How do you approach a patient like this in the safest possible way? I want to just make one point about the technical. We are going to show some cases, both today and tomorrow, and the goal of showing those cases is not only to show some technical tricks that you might like but also specifically what to do to avoid trouble because in this field there is a lot of trouble and it s relatively easy if you know how to avoid it and how to stay out of trouble. One of my professors liked to say it s a lot easier to stay out of trouble then to get out of trouble. Dr. Shaul: One of the things that I noticed about my junior partners is that they are very hesitant to do an anoplasty in some babies even though there may be some meconium on the perineum. They ll want to do a colostomy which surprised me and so I said call me and I ll come do the anoplasty with you. We are talking about a male with a perineal fistula. But the truth is that when you are considering doing a newborn anoplasty in a male with a rectoperineal fistula the urinary tract is incredibly close and so the way I avoid that is that I don t do a circumferential mobilization in a newborn male like this. I do what we might call a cut back and essentially in this baby shown in the previous slide you can unroof where the meconium beading is. You have to mobilize the posterior rectal wall but you have to be very careful if you start mobilizing the anterior rectal wall because it s amazing how close the urethra is and I know of some excellent surgeons who have transected or injured the urethra thinking they were doing something as inoccently as a newborn perineal anoplasty and I think one of the ways to stay out of trouble is to not put the baby in a prone position.

16 Okay. Let s poll the audience. Who would do this anoplasty A: prone, B: lithotomy? With the prone position there are some unique advantages. I personally prefer the prone position because the difficult part of the dissection is always the anterior part and I like to look down not up on the hardest part. And that s also true for Hirschsprung s. I like to do transanorectal dissections prone because the hardest part is the anterior and that s where the vagina or the urethra are. But, there are a couple of points to be made here. One is what Don is saying is to simply fill this space with rectal tissue and I thinks that s a very nice option. Essentially a glorified cutback provided that this is the beginning of the sphincter. That option is not going to work if the hole is not in the center of the sphincter. Then you really need to transpose that hole into the center of the sphincter. And then you really have to do an anterior dissection but in this particular case you have that option and you can avoid some of the anterior dissection. Jack? Dr. Langer: Are we allowed to disagree with you Marc? You were invited here to disagree. Dr. Langer: So. I disagree with your contention that you need to move the front of it back so that it s all within the sphincter. I believe that if you just enlarge that opening and bring the back part of it...i think this is what Don is saying also. That s what I said. I said if the fistula were here... Dr. Langer: No, I m saying even if the fistula is further forward I would just do a cutback. I would do a suture, not like the old cutback where you put a scissors in there and just cut it but I would just extend the rectum back to the back part of the sphincter. I would leave the front part in it s abnormal position because I think there are several advantages to that. One is that

17 you are avoiding that anterior dangerous dissection and the second is that it doesn t tend to stricture down afterwards as much and you don t really need to dilate them on a daily basis because you only have a partial circumference suture line. I think those are good points. In a female we don t have this luxury because you need to gain a bit of a perineal body. In a male it s a little bit different. My only concern with that idea is if you re going to leave an anal opening outside of the sphincter what I have seen is patients later in life who have had that done when they try to close the sphincter they can t completely close the hole and they have a little anterior leakage, particularly during atheletics or if they have loose stool. So my goal is to try to get the hole completely surrounded by a sphincter that can close the anal opening and hold in the stool. That s my only hesitation with your idea. If you had a fistula way up here, when they squeeze this sphincter they are not going to squeeze the stool in the anterior portion so I think that when I finish, I want to make sure that that principle has been achieved. Maybe Paola can comment. Paola wrote a very nice article about two years ago on the older female that was operated on, meaning a patient who either did not have a proper repair or never was touched and later in life had some trouble. Dr. Midrio: The problems seem to start when they approach the age of sexual activity. It s all disturbances in the mechanics and we have these adults who became incontinent because of sexual activity and they basically had a rectal fistula, an untouched fistula, and they didn t know they had it. She was handling the situation but when she had a partner she became incontinent. She could handle this for 20 years in her life and then after we repaired her she said now I feel the muscles push and she really realized when she was constricting the muscles and when or not. Let s now get into the female discussion. So why don t we say this for this photo, A: this is a normal female. B: this is a perineal fistula. It s not so simple. I would suggest that probably one of the most common clinic visits is: is this normal or not question from a pediatrician or a gastroenterologist in a female in particular. So A: normal female, or B: perineal fistula.

18 Alright, so no one thinks it s normal. So what about this picture, Jeff. Is this abnormal? In what way is this abnormal? Dr. Avansino: So first I think... as you pointed out... you have to understand what the normal findings are. So the anus has to be in the center of the muscle complex and has to be appropriately sized and there has to be adequacy of the perineal body in a female. So in this picture, looking at it, I mean the anal opening obviously looks very small. It s actually smaller than the vaginal introitus. It s also just behind the vestibule of the vagina so it would be anterior to the expected position. So, the small anus, and the fact that it s anterior, and then you get a sense that the anal muscle complex is actually posterior to it, so given those three criteria that would suggest that it s a perineal fistula as opposed to being normal and it s outside the vestibule so that s how you distinguish it from a vestibular fistula. So if we go back to the definition of an anus, that hole you see here is too small and it s not in the center of the sphincter which is here. Alright, so let s see what the panel would do for this baby. So who does A: newborn anoplasty tomorrow, B: dilation with delayed repair in the next six months. Assuming a healthy baby. A: newborn anoplasty tomorrow. B: gentle dilations and repair in the next six months. Dr. Sutcliffe: I would concur with the audience. I think that I would also do an MR in the time that I was doing the dilatations because I want to know that there is no tethered cord or presacral mass. I am assuming that we have the capacity to make sure that the child is decompressing, that there s nursing support once the child s gone home so if they run into problems that we know. That s what I d do. And Jack, ala your discussion in the male, how is this scenario different?

19 Dr. Langer: I would dilate it if possible. If the child is evalcuating well with the dilatations then I would come back in three to six months and the anoplasty that I would do at that time would be the posterior kind that I talked about. So, from a technical point of view you would just enlarge this hole? Dr. Langer: I would, I would not touch the front of it. So, let s ask this. A: you would just fill this space with more anus; B: you would mobilize this structure and put it in the center here and then close where the fistula currently is. So, A: I would call that essentially a cutback, Or, B: mobilize this, put it in the center of the sphincter and close that space. The big issue is the perineal body. How much perineal body do we need, and we will talk about that in the gynecology session. And I can tell you this is a big controversial point. What to do? From a technical point of view you don t have any anterior dissection. However, from a functional point of view you want to be absolutely certain to put the anal opening into the center of the sphinter, and for that I believe you need to do a formal movement of the anus. Dr. Langer: A transposition, I think most people would call it that. Transposing of the hole. Dr. Langer: What I have found as I have followed these kids into teenage years is that what looks like a very small perineal body here actually gets much bigger.

20 I think that is a really, really important point. You have to get a sense of which perineal bodies are going to be long enough and which are going to be inadequate. Dr. Georgeson. Dr. Georgeson: In 2005 I sat next to Dr. Stevens at the meeting in Krickenbeck, Germany, and I had a running loud commentary about everything. Because he couldn t hear well and so he also spoke loudly... but anyway, his contention to me as we sat there with this kind of an issue was that they all tend to migrate backwards so that the cutback is fine. That that will grow over time. Yes, I think it s a very important technical point. I think though, you need some perineal body. I m not sure. Does Geri Hewitt, my gynecology partner, have a comment about the perineal body in the future. Dr. Hewitt: From a gynecology standpoint, we would advocate to build as good of a perineal body as you could. Not only to support separation of the reproductive organs from the GI tract but also in terms of sexual functioning and also in terms of any possibility of a vaginal delivery. So from a gynecological standpoint we would advocate building a perineal body. And then the question becomes how long is long enough for the perineal body and I don t think there is an answer for that. But, you do bring up a very interesting question. Let s poll the audience. You have a beautiful repair either way. Everything has healed beautifully. Twenty years from now the patient tells you that they are pregnant and they want to have a delivery. You, as the surgeon, would advise A: normal vaginal delivery; B: mandatory Caesarean section. Now, I will tell you there is no right answer to this, but I m curious to know what everyone s opinion is. That s quite a mixed answer. Anyone on the panel want to comment?

21 Dr. de Blaauw: I would definitely say C-section. Definitely say C-section. C-section from the Netherlands. No C-section in Italy. Seattle? Dr. Avansino: They could become incontinent with a perineal body tear. I think that the risk is too high. That s what I think. Although some cases can have a vaginal delivery, I am sure. But I wouldn t take the risk. That s my point. From the Audience: I think I would say vaginal delivery but with a lateral episiotomy if they need an episiotomy if it s not going well. And that s an education issue. The patient has to understand that issue and has to talk with their obstetrician about it. I agree. And I actually think that it s an important connection and we re working very hard on making that connection between Geri Hewitt and the folks that might actually deliver these babies and making sure they understand what is an anorectal malformation and what is a reconstructed perineal body. Because I can tell you most of them probably don t have that in their mind at three o clock in the morning when they are trying to deliver a baby. That s a delivery that need to be properly planned and discussed with a pediatric gynecologist who knows about these kinds of repairs. Dr. Midrio: Marc, can I... If we do like Jack is suggesting like a cutback, I am against a cutback so, but one positive aspect is that you don t throw away any rectal tissue or minimal tissue because some one says that you still have some sensory fibers there so one potential advantage is you save more of a, sort of a dentate line.

22 We will have more opportunity to talk about that today because we have a comparable case in the operating room today. We will discuss some of these issues. Okay. Anyone have a comment on this newborn female? What is the diagnosis. I can tell you that this case was described as an imperforate anus with urogenital sinus. It s true. So that s a lot of words and I ve got a sixletter word that says the same thing. A cloaca. So this is a single perineal orifice but we need to make a very important distinction and I am being a little bit provocative with this slide because you don t really see the anus. So, for those of you who are bothered by that you are right because if you had the exact same appearance with a normal anus, that s a different situation. That s urogenital sinus that happens with or without virilization. However, if there s no anus at all, that s a cloaca. By definition, such a patient does not need an endocrine evaluation. Their electrolytes will be normal. However, if there is a normal anus, that s a whole different story. Shumyle, do you want to make a quick comment about that distinction? When you get called to the Neonatal Unit about a urogenital sinus and the anus is in fact normal, what s different about that compared to a cloaca? Dr. Alam: Well, I mean that would be consistent with a disorder of sexual development and the concern would be adrenal hyperplasia so its a relatively emergent workup. The incidence of adrenal hyperplasia in the anorectal malformation subgroup is almost zero. Cloaca is an anatomic problem. This is not an endocrinologic problem. These children all have normal ovaries and they do not need a workup but many times these patients are evaluated for ambiguous genitalia. In the female section I have some more pictures of these situations. What would you call this? Fourchette fistula. Vestibular fourchette or perineal. Well, okay, so it is a little more controversial. So there is a hole here. It s too small. It s too anteriorly located. Jack, would you do a formal cutback in this case?

23 Dr. Langer: For me the difference between a perineal fistula and a vestibular fistula is whether there is a perineal body. Correct. Dr. Langer: In this case there is no perineal body so this is a vestibular fistula and I would do a minimal PSARP but I would normally do this without an ostomy and that s controversial. Most of my colleagues would do it with a colostomy. So, does everyone understand that distinction? Alright. So, let s ask that question. Who would do a newborn PSARP? Let s put it this way. Who would do newborn PSARP or a delayed PSARP with no diversion. A. or B: colostomy with a repair sometime later. And, everyone recognizes the difference here. There is basically no perineal body here. We ve got to do something about that. Alright. And by the way, both answers are correct. I think it s based on the clinical experience of the surgeon. I personally would feel comfortable doing an undiverted repair but I can assure you, you will have less perineal complications if all of your vestibulars are diverted but then you get the exchange of having all of the colostomy complications. And the colostomy closure. So, I think if you get comfortable with this procedure, it s definitely one that s doable in a primary way without a diversion and perhaps an intervening step is to do a primary repair with diversion at the same time, and then do a colostomy closure down the road. But I think the days of colostomy repair, colostomy closure in three stages, are probably not needed.