Case Presentations Part II: Cloaca and Complex ARMs 2015

The next case is a 12-month-old female with a history of a cloica. She also had a small lymphalocele at birth, which was closed, horseshoe kidney, left leg hypoplasia, and a clubbed foot. At birth, she had a sigmoid colostomy with a mucous fistula creation. She also was found to be malrotated and had a lab procedure done at that time. Importantly, the surgeon at that point did preserve the appendix, um, which is, you know, for long term, as we previously talked about, need for a metrofenephrine malone at the time of the colostomy creation. So she came to us on her EUA cystoscopy, vaginoscopy, found one perineal opening, um, had a very phallic looking structure. Common channel length was 6 centimeters. The length from the perineal opening to the bladder, um, sorry, from the uh cloica to the bladder. neck was 3 centimeters. Within the bladder at the base of the bladder were 3 openings. The right hemivagina was seen with an abnormal cervix. The middle opening was the rectum, and the left looked like a possible vagina, but we were not able to enter into it. This is her preoperative imaging of her um uh kidneys, which um did not show any hydronephrosis. Picture of her cloaiogram, you can see that this is her uh sigmoid colon here. Like we previously talked about, and that's why I asked Doctor Patel, um, the length of the common channel on radiology versus our measurements, um, uh, sometimes are very similar and sometimes just a little bit off. So we measured 6 in cystoscopy with this was 5 and then the additional almost became 6. Um, this is the picture of her bladder. So you can see a very large bladder. She had no previous drainage of any sort. She did not have hydrocopos at birth and so did not, um, have a vaginostomy tube. Sort of as we discussed before, you can see the end of her sacrum is right here. The end of the rectum is right here. So, at this point in time, um, operative approach, um, if the panel wants to weigh in, would you start with a laparotomy first? Would you start posterior sagittal, um, and counseling in the family for long-term fecal and urinary incontinence? So Down that end to my left. Go, I would start with laparotomy. I mean, just by looking at the contrast study, we can see that this rectum is reachable through the abdomen and it's not even advisable to search for it posterior sagittally. So as Doctor Pena mentioned in those high common channel cloacas, the common wall between the structures are, are not long common walls. They are actually easy to separate the structures, the rectum, if there is a vagina, the vagina, and the bladder. So there's some intraoperative pictures. You see, this is her very, very, very large bladder here. Here you can see, um, it's very small on my screen, um, but the structures that are coming off at the posterior aspect of the bladder. The two vaginas that we were able to identify were very tiny, and um Doctor Bree, do you want to sort of comment on how we assess the usability of that vagina, whether you want to preserve it or remove it? Right, so, um, when we entertain these, uh, larger procedures, we're all there in the operating room together, and it allows us to assess each of the, uh, systems together. In this patient, you can see there are 2 hemivaginas, 2 hemiuteri, and we'll make an assessment intraoperatively about the development of that Mullerian system. On each side we'll make an assessment of how well developed the uterine body is and in doing that I'll show you on some upcoming slides as well that you could do um what we call a saline perturbation. It's a way to sort of assess the patency of the reproductive tract. We do that in all of the cases when we're in the abdomen. We'll talk about how we can do that at other times as well, at other laparoscopic procedures and that sort of thing to assess the development of reproductive structures. I emphasized earlier how important it is to check out both sides and to assess the symmetry between those two sides. If one side is very well developed and the other side is very um atritic, it may be advantageous to retain the well developed side and perhaps remove a side that's very atritic and might be dangerous or risk at the time of menstruation and not helpful for future childbearing. We'll go over that a little bit later, but I think in this case we assess the uh development on each side and then we wanted to make an assessment of if the vagina or vaginas could reach the perineum and I think those are the pictures that Doctor Dickey is trying to demonstrate that the vaginas were not able to reach the perineum adequately. So this is one vagina here. This is the other vagina here. You could, they're two very separate ones, um, and this would not reach the perineum. So we needed to put a bridge of tissue to get it to the perineum. And what we used in this case was um colon. Um, for her, since on her contrast study, I don't know if you saw that she had really redundant, um, colon from her mucous fistula down. So we actually use the proximal, um, mucous fistula, um, as her sigmoid replace or her colonic interposition graft. Um, this is the proximal portion and it's put anti um peristaltic, just due to blood supply and the way that it would reach the perineum. So just schematically, um, what you do is hers, she had two, but the two vaginas are anastomosed onto the sigmoid replacement. Once again, you can see that that's sort of the completed anastomosis, and then it's brought down to the perineum. And sort of this is completion. Um, we ended up doing a formal vesicostomy on the patient, um, just due to her Uh, where she lived, her social situation, and how large her, her, her bladder was. Um, Doctor Vanderbrink, do you want to comment on why we did the vasostomy on that, this patient? Uh I think specifically one of my partners might have done that, but I do believe, as we alluded to before the break that the psychosocial factors, I um might have been uh observing earlier on in the operation because I think the size of the bladder was so significant that this was an interesting finding that was making it the way to the operating room. You got to go see the size of this bladder in this little girl, so. Um, I'm, I'm thinking that potentially letting this bladder maximally decompress to a functional vesicostomy may help, uh, her bladder from having some potential rebound recovery, um, but I think she's getting all of her urologic follow-up at an outside institution. Can I ask you a question, Belinda? Yeah. Instead of using the mucous fistula as the vaginal replacement, why could you not use uh the distal rectum and just turn it back on itself, leaving its blood supply completely intact. We've done that in a few cases and uh it seems a little simpler. You know, we considered both of them, but the way that the blood supply and how things reached it actually reached better using the proximal mucous fish slug because then if we took a segment for the rectum, then the actual remaining segment had difficulty reaching down to the perineum because it was so high up. Um, the, the, when we have used rectum as vaginal replacement, I think it's important to note that um those are the patients where you believe that they won't be fecally continent, um, because if you use the rectum and take away that reservoir, you may change a patient who is potentially fecally continent into somebody who's not going to be fecally continent. So with her, it probably didn't matter because she had a tethered cord, she had a short sacrum, um, that we could have used the distal rectum or, or another portion of the colon. Let me, let me, uh, re-emphasize what you just said, Belinda. That's a very important point because, um. In the first cases that I uh operated, not, not exactly cloacas, but patients with vestibular fistulas and absent vagina, I, I thought I had a brilliant idea of using the rectum that was already located behind the urethra and leave it as a vagina and take up the rectum down and, um, and then following those patients through the years I found that the bowel control was less than optimal in those cases. In addition, those patients have usually bad sacrum and tetecord, but anyway, what we learned is that the preservation of the rectum in patients with good functional prognosis, patients with good sacrum, not tete cord, and a good malformation is extremely important to preserve the rectum. So we use the rectum. With many, many vaginal replacement with rectum, but as, as you mentioned, it, if, if we have the, uh, we know that the patient will have not, no bowel, will have no bowel control and therefore it's not gonna make a difference for her to have the rectum and not the rectum. The rectum is a natural reservoir and if we remove it in patients with an rectum malformation, most likely the patient is gonna be incontinent. Because they cannot tolerate the, the constant passing of stool that occurs when you connect colon down to the perineum. Doctor Pena, we have one of your 3D animations, so I'm gonna push play and be up on the screen for you. There you have the case of a a very long common channel and, and you look at, look at the, the anatomy you see two little hemi vaginas connected into the trigon or the bladder neck, the rectum also there and the ureter is also there, so these are easier to separate through the abdomen rather than from below. Once you separate them, you, you can bring the vaginas together, but very soon you, you may find out that the vaginas are located very high in the pelvis and there's, it's not possible to bring them down. And at that point you have to decide a vaginal replacement. We have been using more and more the descending colon. We have tried small bowel, rectum, and colon, but we have been using more descending colon because the arcades are very nice in order to preserve the blood supply. So you can see the piece of bowel doing a partial vaginal replacement in this particular case and now you see the rectum placed in the right location and the, the uh vaginal, the partial vaginal replacement in front with a piece of colon. So, um, you know, these are the, we sort of talk about laparoscopy and whether laparoscopy is indicated in any of these procedures and a lot of the reports and literature reviews we've done, um, show that many of the repairs previously that say they've done laparoscopically is really the rectal repair of the, um, cloaca. So, you know, the addition, this is the case that um Doctor Calvill first presented, the prenatal diagnosis of the anal rectum malformation with a possible cloica. Um, This is what the baby looked like when she was born. So she had a little bit of vestigial tail. Um, you can note that this is why they called her ambiguous genitalia. Um, very phallic looking, um. Structure here with almost a scrotal looking sac um apparent. Um, Doctor Pena, you have, you have mentioned before, whenever you see some structure like this, what is it's indication of a very we, we have seen approximately 65. Those cases that came to us operated originally, I mean had a colostom in another institution and at birth because the baby has similar anatomy, in other words, the presence of uh something that looked like a phalluss, the diagnosis of intersex was made and and then the patient was extensively uh studied by, you know, endocrinologists, geneticists and urologists and so forth only to conclude that they were chromosomally normal females. So they came here and. We treat the the actually those all those patients were cloacas. I must say we have never seen a patient with an intersex and a cloaca. So if you see a patient with a pseudohaus like that, a single perineal orifice, the way to differentiate that from uh adrenal hyperplasia is by palpating. Painting that structure in a real adrenal hyperplasia you will be able to palpate corpora, real, real penis there, whereas in these patients what you that that structure that looks like a penis actually is just folded skin and we as a matter of fact we use that folded skin to facilitate the reconstruction of the of the vagina so this is something that um. Should everybody should be alerted about because the the the families of the patients agonized during the 2 or 3 weeks that the doctors are trying to make the diagnosis of intersex. And, and, you know, even though we as the center see this baby prenatally, we saw this baby postlatally in the NICU, um, unfortunately, our neonatologists thought they knew better and sent off chromosomes on this child, which, of course, she had a prenatal diagnosis of being XX. So even in our institution, there is more education to be done. So, um, for her, we actually approached her colostomy, a mucous fistula, and uh, uh, she had hydrocopos, um, with the laparoscope. And so, Uh, you know, we were able to um perform it through sort of two incisions really. We had the colostomy, the mucous fistula, and our vaginostomy tube, um, which drained her hydrocopus.