Colorectal Quiz Episode 17: Cloaca Part 1

All right, welcome to the colorectal quiz. This is Amanda Jensen from Cincinnati Children's. Hey everyone, this is Kira Ahmad from Nationwide Children's Hospital. Have you downloaded the new version of the Say Current Pediatric Surgery app? It's in the Google Play Store and in the Apple App Store. The reason I bring it up is that while you're listening to this episode, you can see all of the great images associated with what we're talking about. Today we have a very special episode for you. We are going to talk about cloaca and the newborn initial workup and management. This is part one of a three-part series. Today our special guest is Dr. Richard Wood, and he's from my home institution from Nationwide Children's Hospital. Along with Mark Leavitt from Children's National and Dr. Jason Frischer from Cincinnati Children's. Welcome back everyone to the colorectal quizzes. We're excited to have you and we have a special guest today. We have Richard Wood. He is a former partner of Dr. Leavitt. So maybe Mark, you want to tell us some interesting stories about Richard so our audience can get to know him a little bit better? We got lots of stories, but that's going to probably fill up the entire podcast. But I will tell you, Richard, I met Richard when he was a fellow training in Cape Town at Red Cross Children's Hospital. We met on a mission trip in Africa and he blew me away with his skills, talent, and humor. And I was blessed that I got to work with him at Nationwide Children's Hospital as a partner. We worked out a lot of challenges in cloacal malformations, which is the subject of this podcast. And I can't say enough about his talents, but in addition, the great collaboration with urology and gynecology, which is absolutely essential in understanding these complex kids. So Richard, thank you so much for joining us and for bringing these cases to discuss. Okay. Well, thanks very much for having me. So the first patient is a 31-week-old infant who was born one of a twin. On post-delivery day, one was noticed to have a single perineal orifice. The single perineal orifice should clue you into a cloaca. The vagina, urethra, and rectum are fused together inside, creating a single common channel. And on the basis of that, she had some workup, which basically showed that she had a, she did have a hydroculpus. As a reminder to everyone, hydroculpus is the distension of the vagina, which is caused by the accumulation of the fluid. She had grade five reflux on the left, a grade four reflux on the right on her renal ultrasound. She had a spinal ultrasound, which was normal, and a normal cardiac exam. And she didn't have a trachosophageal fistula. All right. The key point here is that a cloaca or an anal rectal malformation is associated with bacterial and needs to be worked up as such. All right, Amanda. What is bacterial association? As a review, V is vertebral anomalies. A is for imperforate anus. C is cardiovascular anomalies. T is tracheosophageal fistulas. E is esophageal atresia. And R is for renal or radial anomalies. And L is for limb defects. So to have a bacterial association, we need three or more anomalies. In general, what are some of the features that a perinatologist might see that should make them suspicious that a cloacal malformation is in utero? Yeah, I mean, I think that's a good question. Unfortunately, I would say the diagnostic yield in cloacas is still much lower than we would like it. But I think if children have, if little girls have a hydrocorpus, I think it's much easier for the perinatologist to be able to make that diagnosis on ultrasound. A hydrocorpus on a prenatal ultrasound should alert us to a possibility of a cloaca. There are other more subtle signs like abnormal kidneys. Perhaps a single kidney can alert them that there may be a bacterial situation going on. They do sometimes have an association with a two-vessel cord, and that's been associated with cloaca. But I think mostly they get much more clear on it if they do diagnose a hydrocorpus, and that's often been helpful. But as I said, the numbers of patients that are getting diagnosed antenatally is still pretty low from my reading and from my experience as well. I don't know if you guys have seen something different. Well, I was just realizing that this specific case would be really one that could potentially be prenatally diagnosed because there's a hydrocorpus, which they should be able to see, at least know that there's a pelvic mass, and the hydronephrosis they should also see. And if they know it's a female and there's a pelvic mass and something wrong with the kidneys, a smart perinatologist will say maybe a cloaca is about to be born, which could obviously influence where they should be born. And obviously this baby, I hope, was born somewhere where they could do some intervention radiologically and surgically. I mean, I must be honest, I have had a couple of those in the last five years where they've kind of had that exact configuration, and we have been able to counsel families and arrange for them to be born locally or closer to us. But I would say the vast majority, that's unfortunately not the case. But I think you're right. This is a great example of one that perhaps could. Unfortunately, in a large majority of patients with cloaca, the diagnosis is made at birth. All right. So this baby pops out in the delivery room. And if you have your Stay Current app, you can see what the perineum looks like. And maybe, Richard, you could just comment on what this perineum looks like to you and also the fact that some neonatologists might conclude that this is, quote, ambiguous genitalia and do some workup for endocrine problems, which I suppose this baby doesn't need, right? Yeah, Mark, that's correct. So I think the first thing you've got to do is do a really good exam with good lighting. Dr. Wood, what did you see on your physical exam in this patient? I think if we look at this child, you really can't see all that much. But, you know, if we just distract the labia a little bit, we'll be able to see a lot better. We can also see that there's a perineal groove behind the labia structures. And it does appear to be some dimpling, which could represent a muscle complex. So I would say that it's not a completely flat perineum, which I think is something that I look at. But I think if you distract the labia, you can see that there's really more of a clitoral hood than real labia minora. And then a single perineal orifice between sort of posterior to the clitoral hood and the sort of underdeveloped labia minora. And that would fit with our clinical diagnosis of a cloacal malformation. A single perineal orifice, high, high suspicion for cloaca. And in a child with a cloacal malformation who does not have an anus, there really isn't an indication to investigate for ambiguae stenitalia or disorders of sexual differentiation. Because we know that these children with cloacas are female and they don't need that workup. So although they can have pretty prominent clitoral hood and labial tissue, more prominent than one might normally expect, we don't believe that they need a workup for karyotyping and stuff. You see this in the NICU or transferred into your institution. What are the next steps? What do we need to do urgently? What do we have time to do in the future? I think you've got a couple of things to sort out. That's most notably, you've got to make sure that they can decompress their kidneys and drain urine. And you're really trying to work out if there's a hydrocorpus, which this child did have. And then, well, make the diagnosis, sorry, obviously first. And then you've got to make sure there's nothing going on that's going to make going to the OR to do a colostomy dangerous. So you probably want to get a cardiac assessment and look for a TEF, which you can do with a nasogastric tube, a chest x-ray, and an echo. And I think you probably need to think about doing those up front. And then I would always add a pelvic and renal ultrasound in these patients to diagnose the hydrocorpus if it's not obvious clinically and work out whether their kidneys are obstructed or not. So again, prior to going to the OR, the initial workup should consist of an NG tube and a chest x-ray, as well as a cardiac echo and a pelvic and renal ultrasound. These studies combined assess for TEF, cardiac anomalies, hydrocorpus, and hydronephrosis. If they've got bilateral hydronephrosis and a hydrocorpus, you're going to need to manage that hydrocorpus as part of the initial treatment. How do you do that? You know, traditionally that was always in the form of vaginostomy. We've moved heavily away from doing vaginostomies and much more with doing CIC through the common channel. So how do you perform CIC? So we would pass a tube through the common channel, drain fluid, and then get an ultrasound and check that the tube is in fact in the hydrocorpus or the vaginas or vagina. And then see that we can decompress it and then try and do that recurrently. And you can use the time prior to doing the colostomy to work out, can you drain the hydrocorpus? And if you can effectively drain it, then we've chosen to just do colostomy these days and then continue to try and drain it postoperatively. If you don't manage, you can always go back. But I know there's obviously a different school that believes, you know, formal vaginostomy is the way to go. That used to be our practice, but I would say we've largely moved away from that. I really want to emphasize, Richard, what you just said, because I really think that that dogma is no longer valid. You know, obviously you and I learned on a number of these cases, and I really give Seattle Children's a lot of credit here, because they were the ones that said, Paul McGarrian and Jeff Evan Sino and Caitlin Smith, et cetera, they were the ones that were really saying you can drain a lot of these hydrocolpi, I guess, is that a word, perineally. And I was convinced because I had been doing hydrocorpus drainages with tubes or sutured every single time I saw one that was problematic like this. And amazingly, a lot of them can be drained perineally. And if you know the anatomy of the urethral takeoff or to the bladder neck, you're more likely to get into the vagina, frankly, than into the bladder. So cathing is usually not that bad of a problem. The thing that you have to check, which you mentioned, is on ultrasound, did you successfully get in where you wanted to get in? So I have to pause, you guys, because this is, if I look at my textbooks behind me, this is not written in the chapters written by people on this podcast of what to do for cloacas and hydrocorpus. So this is huge. This is why, this is real time. And we all know a book chapter takes five years to, three to five years to get published. So what is the modern way to drain a hydrocorpus? Let's go over that one more time because this is new. And the last 20 years of every edition of a textbook does not say this. Jason, I will say it's in the seminars paper. That's a seminars, that's a journal article. But no textbook has this yet. All right. The seminars paper he's referring to is organizing the care of a patient with a cloacal malformation. Key steps in decision making for the pre, intra, and postoperative repair. This is in the seminars of pediatric surgery. Click on the link associated with this podcast. Let's go over it again because it's huge and great. And I was with Mark when I learned this in Seattle and we were very hesitant when we saw this being performed. But it makes sense. So tell me what happens when it goes wrong. How do we know, how do we follow it? How long do we follow it to ensure that we're properly draining? Because we definitely don't want to do any damage. So I would just say what we've found to be helpful is pass the tube initially and make sure you can pass it easily. Leave it in and get an ultrasound and confirm that. When do you get that ultrasound? Straight away at the bedside within the first 24 hours. So when you see the baby, pass the tube, get a bedside ultrasound and confirm that you got into the hydrocopos. And then confirm that you're decompressing it. Let me. And then we leave it in for. Before you continue, I just want to emphasize that point because I have actually seen it done live. And Jason, Steve Kraus, phenomenal radiologist, he showed me live that while you're doing an ultrasound, if you put the catheter into the hydrocopos, not only do you drain all that urine that's been accumulated in there, but as soon as you do that drainage, guess what? The bladder fills, which is exactly the physiology that's so problematic about the hydrocopos, because the hydrocopos is obstructing the ureters. And this is another point I really want to make. A vesicostomy is the wrong move here. In almost every cloaca, a vesicostomy is not necessary, but the hydrocopos needs to be drained. The new thing here, as Richard mentioned, is drainage by perineal catheterization. Dr. Leavitt, what do you think about just doing CACs? Once you drain the hydrocopos, the ureters now are no longer compressed at the trigone. They can successfully empty into the bladder. And then the bladder either empties out the common channel or back into the hydrocopos, which then you drain with a sequential perineal catheterization. Let me ask some questions, because again, huge. How often do you have the family catheterized? How long do you, in the NICU, check to make sure that the catheterization, the perineal catheterization, is accomplishing the job at hand, draining the hydrocopos and relieving the bladder obstruction? And when do you say it's okay, this is safe? And what happens, because many of these patients have vaginal septums and are complete vaginal septums, and what happens in those patients? Or are those their failures? No, they're not. So Jason, I think important with all of these is you've got to follow their kidneys. Because the most important thing is if you've got a hydrocopos with completely normal kidneys, it doesn't matter. The important thing is that you're decompressing their kidneys. All right. So how will we know that we're adequately draining this hydrocopos? We would do an initial drainage with a bedside ultrasound, check that we drained the hydrocopos. We'd probably leave the catheter in for a little bit to make sure it continues to drain. Then we would try and catheter for, you know, we usually do three times a day initially. You can get down to twice a day when the family start doing it. And then you just follow with serial ultrasounds every, we usually do two to three days initially, and then stretch it out to a week, especially a little 31 week like this. They're going to be in the NICU for a while. So you've got plenty of time to sort it out, make sure it's effective before they're going home, because they're not going home for a couple of weeks. Now, if it's a full-term baby, that's a different story. You're going to have to get yourself organized a little faster. But we have generally found if you ultrasound every couple of days, you'll be sure that you're decompressing the kidneys nicely. And then once they go home, we've just done a monthly ultrasound to make sure that they're continuing to do well. And then if they're doing really well and you want to stretch it out a little bit, you can to maybe every six weeks or so, just to make sure that the kidneys are continuing to drain nicely. Because I think that's one of the dangers, even with the vaginostomy tubes prior to this, is people put in a vaginostomy tube and then they just assume that everything's fine. But actually, even with a vaginostomy tube, you've got to keep checking and making sure that the job I think it's doing, it's doing. Because if it's not keeping the kidneys decompressed, then it's not doing its job. So I think the message to everyone is, however you drain the hydroculpus, your job is to continue checking the kidneys are decompressed. Richard, could you just give us a bullet point of the newborn management, just to summarize what we just talked about? Yeah, so I think a good exam to make the diagnosis with good light. You don't need to do an endocrine workup if you diagnose a cloaca. If you've diagnosed a patient with a cloacal malformation, I think renal and pelvic ultrasounds, as well as tests to make sure the patient's safe for anesthesia, are key steps. If there's a hydroculpus, we think it should be drained and believe you can start off by trying to drain that by clean intermittent catheterization. And then obviously, you're going to set the patient up for a colostomy within the first 24 to 48 hours. All right, to summarize, diagnoses, bacterial workup, drain the hydroculpus, and stoma. And we haven't really dealt with the colostomy. I don't know if you've dealt with that in another session. And how do you do the stoma? I would advocate for doing your colostomy as proximally as you can in this situation to make sure that you have enough length for distal work. So I'd probably do it at the descending sigmoid junction rather than sort of any lower down sigmoid. Quick question for you, because I think a lot of us, a lot of pediatric surgeons out there, see these patients. Let's say either they were unable to do a perineal drain or drain with a perineal catheterization, or it's just not in their algorithm right now. Now, discussion about how you make the colostomy, meaning I know you said descending sigmoid junction. Do you use any laparoscopy? And how do you do a vaginostomy tube if you needed to do one? Yeah, great. So if the patient's not distended, I really do like to use the laparoscope for these newborn colostomy formation. I think there are a number of advantages. A, you get a really good view of the anatomy, which I think in a cloaca patient is very helpful to get upfront insight into what's going on in their pelvis, even if they don't have a hydrocorpus. I think you can pick your spot very nicely for the colostomy. And in this case, I would mobilize the sort of lateral attachments of the descending lower down to give myself the opportunity to use the real corner of the descending sigmoid junction to give myself plenty of length. I think the other big advantage with doing the laparoscopy is that you don't have to have that wound between your two stomas if you're using a divided stoma. So we like to bring the bowel up through the site that's going to be the mucous fistula, staple it, wash out the distal by cutting off a corner until it's completely clean, and then make a separate incision to pass up just the proximal bowel to make a stoma, but with no incision around it. So you have a clean piece of skin with just a circle for the working stoma. And then you can close that mucous fistula site somewhat and just have your mucous fistula in the corner. And that allows you to have a wound that's easy to heal and bag, but you've gotten good anatomical information while you're in the pelvis. How would you create a vaginostomy if you absolutely have to do it? If you do choose to do it, I think it depends slightly on whether the patient has a septum or not. I think if the patient has a septum, I would advocate for opening the anterior wall of the hydrocorpus vagina and removing a small portion of the septum so I can drain both sides through one hole. So remember, prior to creating a vaginostomy, check if the patient has a septum or not, because we may have to remove a part of the septum to adequately drain the vagina. If they had a single vaginal structure, I think you could use your laparoscope and you could pass a tube in if you wanted to do that, or you could just bring up a single hole if you wanted to do a tubeless setup. And I think both of those are okay. If you use tubes, just know that they can become encrusted and colonized. So there is some advantage to the non-tube version. But I think you just got to judge the anatomy. If you can get it up easily, maybe tubeless is a better option. If you're struggling to get it to reach the abdominal wall, then perhaps a tube option is slightly better. Let's say you needed to do this with an open technique. Is there a certain incision you would use over another? Do you go midline versus our standard like oblique, left lateral oblique incision for making stomas in these cases? I do the colostomy just like Richard described. But if you have a massive hydroculpos, then you basically should do a lower midline incision. You have to get above the hydroculpos. And the hydroculpos is very adherent to the anterior abdominal wall and very inflamed. And if you do your standard left lower quadrant incision for those who are not doing laparoscopy, you will have a lot of trouble getting north superior to the hydroculpos. So in that case, I will do a lower midline incision, get above the hydroculpos, open into the dome, take out a bit of the septum as Richard described, close it, obviously put in a tube. Now a tube will drain both sides. Or if it's that large, suture it to the abdominal wall, just like you would a vesicostomy or a gastrostomy. And that's a really nice way to deal with this because then you don't have to have an indwelling tube, which is a nidus for infection. And then I would divide the stomas, just like Richard described, and then bring them out in the left lower quadrant. And of course, in that circumstance, you don't need a skin bridge. The one thing I did want to mention relative to the newborn is, Richard, you talked about knowing it's a cloaque on physical exam. And I just want to emphasize the reason why you knew it was a cloaque on physical exam is because there was a single peroneal orifice and imperferent anus. There's no anal opening. As opposed to the circumstance where there is a completely normal anus and a peroneal orifice, i.e. a urogenal sinus. That's a different ballgame. That is a urogenal sinus, and that is a patient that definitely needs an endocrine workup, checking of electrolytes, and making sure that it's not one of the CAH situations. But if there is no anus, it is a cloaca. It is not ambiguous genitalia. It is a cloaca. It is a female. And you go on from there. So I just want to make sure that's included because there will be a newborn that shows up that looks a lot like this that you see in the picture, but actually has an anus, and that's a urogenal sinus situation. And by the way, those patients can have hydroculpose, too, and hydronephrosis. So similar management, just obviously no anus or colostomy that needs to be dealt with. Once again, single peroneal orifice with no anal opening is a cloaca and does not need an endocrine workup, whereas a peroneal opening with a normal anus is a ugesinus and does need an endocrine workup. Great points. So, Richard, let's move along here and say we're able to catheterize through the perineum. You laparoscopically make a colostomy, and the patient is discharged from the NICU. Next, what do we do? Yeah, so next, I think you want to follow the patient up carefully, like we said. Make sure their kidneys are well decompressed and that they're growing well. You probably want to have gotten based on kidney functions testing done when they were with you, and you might want to follow that up depending on how things looked. And then, obviously, just for those first several months, you just want to make sure that the parents are managing with the stoma as well and that the baby is growing and thriving. I think those are your key aspects. And we would try and follow those children up relatively closely initially. And then, once they're kind of in a stable pattern, then we can think about what the sort of timing seems like as a good time to consider getting definitive imaging and working out on a reconstructive plan. But I think your key is to make sure the patient's growing and thriving first, because if you've got the urine drained effectively and the stool drained effectively, they really should be thriving unless there's other underlying issues going on, which you should be looking for if the patient's not doing as well as you would like. And we are out of time for today. So, to summarize, in a patient with suspected cloaca, do a good perineal exam to make the diagnosis, and you do not need an endocrine workup in a patient with a cloaca. An appropriate testing preoperatively for these patients includes a renal and pelvic ultrasound along with an echo and assessment for a TEF. If there is hydrocopals, appropriately drain, either with a clean intermittent calf or with a vaginostomy. In patients with cloaca, protect the kidneys. And you want to set up these patients for a colostomy in the first 24 to 48 hours. And there's a couple of different techniques that we talked about and tricks regarding colostomy and vaginostomy if needed. And then the big takeaway is making sure that the baby is growing and thriving before you plan for definitive imaging. Next week, we'll plan to discuss that definitive imaging as well as operative planning. All right, Dr. Frischer, let's hear that colorectal joke of the day. Well, I've got one. Do you remember, I think it might have even been our first podcast. I asked about, did you hear about the movie about constipation? Yes, and I think you told us that it's not out yet. It hasn't come out yet. I'm so glad you remember. Well, listen to this. The sequel came out. Did you hear about the sequel, Diarrhea? No. It leaked, so they had to release it early. Contribility care, my friends. All right, that's a wrap. This is Amanda Jensen with Cincinnati Children's. Remember, knowledge should be free. A WB-8-1-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0-0