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Welcome back to another episode of the Colorectal Quiz.

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I'm Shimon Jacobs, colorectal surgery fellow at Children's National in Washington, D.C.

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In today's episode, we'll review a very intriguing case of a cloaca varian.

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We have our usual host, Dr. Frischer and Dr. Levitt, who will introduce our special international

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guest.

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Carlos Reck is a very established pediatric colorectal surgeon in Vienna, Austria.

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So, welcome, Carlos.

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Thank you, Mark, for this great introduction.

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Thank you, Jason, for having me.

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Carlos, you want to tell us about a case that you brought for discussion?

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So, this patient was originally here in Austria.

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We have an organ screening around 20 to 24 weeks pregnancy where they saw that the child

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had an intra-abdominal cyst.

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So, she was referred for a prenatal MRI, which was done around 28 weeks.

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If you're in the state-current app, open the first image where you can see two panels taken

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from this fetal MRI and follow along as Dr. Reck describes the findings.

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They still were able to see the structures, which appear to be a double cyst, differential

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diagnosis, mesenteric cyst, differential diagnosis, ovarian cyst, differential diagnosis, hydrocholpus.

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I think in a female with a large abdominal cyst, certainly with any associated urologic

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issues, they should hopefully think about cloaca and any of the other bacterial anomalies.

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Let's say the radius is missing or the sacrum is missing.

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There should be suspicions that that cyst is associated with the hydrocholpus.

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And yes, I thought definitely of the possibility of a cloaca, especially by seeing the location

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of the cyst and this double configuration, which for me wouldn't make sense for, for

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example, a mesenteric cyst or ovarian cyst.

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In any case, she was referred to our hospital to give birth.

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The child was born full term with a good size over three kilograms and didn't have any

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trouble and the respiratory problems didn't require any type of resuscitation.

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The second image shows the perineal exam.

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Note the good technique for a female newborn exam, which retracts the labia majora upward

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and outward to maximize visualization.

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You can see there was only one perineal hole.

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I just want to point out a couple things that orifice is large for a cloaca.

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It's normal appearing.

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Obviously there's no anal opening.

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So I think that's an important finding that often means that the confluence of structures

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is very low.

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I find that if the single perineal orifice is very, very tiny and right at the clitoris,

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that usually means a high confluence.

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You can actually see that there is an abdominal mass protruding, which is something I could

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feel on examination.

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There wasn't an anus, but there was a good anal dimple and there was not a flat bottom,

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but rather a good, well-developed child bottom.

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I decided to take it to the OR to drain this hydrocopos where I was planning to do a cystoscopy

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of this orifice.

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I first went with a cystoscope.

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Surprisingly, I wasn't able to get into any vagina.

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Basically what you see is a direct entrance into the urethra and couldn't find any type

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of UG sinus.

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That's very interesting.

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So you cystoscope the patient through the single perineal orifice and the only thing

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you saw was essentially a urethra that entered a bladder.

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No rectal or vaginal communication with that single channel.

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Is that what I'm hearing?

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Correct.

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So that's a little crazy and I'm fascinated by the fact that this patient obviously had

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a hydrocopos.

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How does that happen?

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How do you get a hydrocopos with no urine connected to it?

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So that's where my whole diagnostic dilemma was and I couldn't explain it.

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Yeah, well, so I thought the first thing is first.

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So let me drain it and create a colostomy.

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And I wanted to send some of this fluid to establish creatinine and see if there is urine

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in there.

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By the way, what did the fluid look like?

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So when it came out, it was basically a little mucus looking fluid.

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It wasn't infected.

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It wasn't like urine smelling or anything.

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It was just like a amber colored, dark, not completely transparent fluid, which was pretty

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easy to drain.

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I don't think it's urine because there's no communication, but obviously there's vaginal

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mucus and the maternal hormones are affecting that.

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And that's one of the explanations for lots of fluid.

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Do we really know there's no contribution from urine?

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I mean, could be an ectopic urinary connecting to one or both of the vaginas that we know

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or that we don't know yet at this diagnostic moment.

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And by the way, was there associated hydronephrosis?

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So there was one unilateral, but it was only like grade two and on the other side it was

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like grade three hydronephrosis.

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So going back to the OR, after the cystoscopy, how did you deal with the hydrocopos?

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So in the operating room, I decided to drain it.

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I shortly was considering doing a vaginostomy, but I wanted to be less invasive and decided

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to use this pigtail catheter and see if I can drain it with that so as to not create

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a lot of abdominal scaring and intra-abdominal adhesions because I didn't know what was coming

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next or what would be happening as far as next surgeries are concerned.

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We talked about this at another podcast, the options of draining a hydrocopos.

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You chose to use a pigtail catheter.

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This is one that's large and one could consider a tubeless vaginostomy, just suturing it to

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the abdominal wall, which is an option.

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I recognize that you felt that that might ultimately tether the pull through, although

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I haven't found that really is a concern and it's very easy to just take it down if it

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is tethering and I haven't had it tethered.

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There's plenty of vagina here.

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So that's one way to sort of reduce the infection by not having an indwelling tube if the hydrocopos

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is large and this one is above the umbilicus.

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And what about the septum because you have two separate structures that need to drain

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sometimes.

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Well, I think the main factor is first, can you decide?

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Of course, in this case, it was a very obviously and it was seen an ultrasound that there was

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no septum and that there was a huge cyst.

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And the second one is if you have a smaller one and you can see it's divided, I would

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usually do them open and drain them openly or do a laparoscopy and then take a look at

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it.

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Again, in this case, I decided not to do it because of the size of it and I felt pretty

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comfortable by seeing that there was no septum or ultrasound that I would be able to drain

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it just with one catheter put in there.

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How did you do that beautiful stoma?

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There's no skin bridge.

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Dr. Levitt is seeing image 3 in the state current app showing the abdomen post surgery.

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Well, the way I do it, I usually make like a one and a half centimeter incision and then

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with a bab cup or something, I go for the sigmoid colon and bring it up when I can identify

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if I can see a white line, I will identify it as such and bring it bring up a loop and

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then divide it outside.

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And one side I would do a little purse string and then bring it up through a second incision.

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You know, it's a great trick and we've been doing many of our stomas in the neonates laparoscopically

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now because I think avoiding that skin bridge is helpful from a wound care issue.

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But yes, we have made sure or in short that we have emptied our distal limb doing irrigations

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because as you know, and everyone knows, when you leave some meconium in there and you're

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not doing your definitive repairs and colostomy closures for six months or so, that becomes

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almost petrified and extremely difficult to evacuate and causes problems.

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So yes, I usually also do laparoscopy.

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In this case, I didn't because I didn't know this huge cyst, even if it was strained, how

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I would be able with the laparoscope to look around it.

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But at this point, I was only concerned in doing what's necessary to get all the this

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was a newborn.

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So I want to just try to be stabilized and to have all his functions functioning and

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then go on to do further diagnosis.

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So after the surgery, the patient is able to feed and eventually be discharged from

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the NICU with the pigtail drain in place with plan for further diagnostic testing as an

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outpatient.

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Look at image four, showing an MRI at about one month out.

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What's going on here, Dr. Rek?

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Two days before this image, the child had lost the pigtail catheter, which is definitely

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a disadvantage of having a catheter left and sending them home.

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In any case, she didn't come for care and only came after two days because she had the

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MRI, luckily, which was an MRI.

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I mean, you can see that the hydrocoposal had failed again to this massive amount.

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So I ended up draining it acutely, putting a new pigtail in there with my ultrasound.

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The patient also had a distal colostogram done, shown in image five.

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What do you think of this study?

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There's not enough pressure buildup there, but you can see where the colon kind of ends.

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So it wasn't a very high colon.

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You can see it.

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There was a good developed sacrum and the colon was at the PC line.

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If you imagine a little more pressure in there.

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But again, it was blind ending.

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The line you can see with contrast was actually outside.

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So this wasn't a fistula or anything.

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I don't think this image is that bad.

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It's reasonably well distended.

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You can see the sacrum.

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You can see the perineal marker.

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Yeah, I mean, a little bit more distension just to be absolutely certain that there's

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no fistula.

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You have a good idea of where the rectum is that you can reach it from below.

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So now you have the imaging, you know, obviously that there is a urethra.

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You're happy with the location of the urethra and there's no connection to it.

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So urethra is urethra.

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Done.

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Now you have to go posterior sagittal.

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And then what did you find and what did you do?

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I decided to go with the posterior sagittal approach because there was a low rectum and

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I assumed that if there was a vagina, I would be able to find it from behind.

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Image six shows an intraoperative photo during the cloaca repair.

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The rectum is retracted upwards with the vicro sutures and the vagina is retracted with the

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silk sutures.

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And you can see I have put stitches around the vagina and brought it down or I'm putting

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some tension on it that you can see on the picture.

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And in this picture, you cannot see it, but by this time I had already opened the vagina

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and seen inside.

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So I was sure this is a vagina.

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Any septum or how many services did you see?

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Did it confirm what you saw on your imaging?

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Yes, I didn't find any septum and I only found what appeared to be one services, but wasn't

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very clear because of all this redundant tissue of the deflated structure.

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So at this point, you can also see in the picture that the urethra was untouched.

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I left it there.

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The opening was left as it is.

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And then I was able to bring the whole, I tried to separate the vagina from the bladder.

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At this point, it was almost at the bladder neck that you can see below it and separated

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as much as I could until I had no tension.

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And then I had the rectum, the hydroculpus or vagina I would call and was able to do

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the vaginoplasty and the anoplasty.

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I was planning that if I need to do an interposition or something, I would go into the abdomen,

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which luckily I didn't have to.

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Yeah, a little bit of a black box, not having the usual connection points and

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maybe doing a laparoscopy first would also have been the correct thing.

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But again, I was afraid of having a lot of redundant tissue from this huge cyst and not

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being really able to differentiate a lot with the abdomen being so small and not being able

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to create a lot of space in there.

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Yeah, I think that was good judgment.

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I agree.

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I like to light things up.

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I would have the two important points relative to the hydroculpus, just to reaffirm what

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Jason said is be aware there's a septum and you have to remove a little piece of the septum

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and then make sure both sides are properly draining.

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And then a contrast study through that tube or vaginostomy would be very valuable.

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We would do it all together.

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We would inject the distal rectum, the hydroculpus if present, and the bladder.

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So all together is in a 3D cloacogram or a fluoro if you don't have the ability to rotate.

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But I think cystoscoping the patient, putting a catheter in the bladder, putting a catheter

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in the mucus fistula, putting a catheter in the hydroculpus, which you already have, and

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then doing a fluoro study, particularly in the lateral projection, you would have had

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very good data and been very confident that going in posterior sagittally, you would be

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able to find both structures.

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And then in this unique case, no work needs to be done on the urethra because the common

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channel itself is the urethra.

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Once you have absolutely confirmed, and it seems like you have successfully done that,

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that there's no vaginal urethral fistula or rectovaginal fistula related to the rectum

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part.

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So very interesting case.

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And I think, again, evidence that you got to know your anatomy.

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I mean, at the end, I didn't know how to call this because calling it cloaca, we didn't

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have a really common channel.

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Yeah, I would call it.

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I would definitely call it a cloaca, but I would say it's a variant.

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You have a sink.

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You have no anus and you have a single perineal orifice.

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It's a cloaca.

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The only thing that's particularly unique here is there's no vaginal or rectal connection

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to the common channel.

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This is a great anatomic case.

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Phenomenal.

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Carlos, any jokes from the overseas?

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No, unfortunately, I don't have a joke.

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I'm very bad at telling jokes.

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Well, you know, it's still Halloween season, so a simple little discussion is I want to

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say a knock knock joke.

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Knock knock.

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No, who's there?

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Boo boo hoo.

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Boo boo hoo hoo.

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You don't have to cry about it.

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Thank you for joining us, Carlos, and great to see you.

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Great to see you all.

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Thanks also a lot.

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Thank you for tuning into the episode of the colorectal quiz.

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Check out the Stay Current app for more episodes and other great pediatric surgery content

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because now it should be free.