Welcome back to another episode of the Colorectal Quiz.
I'm Shimon Jacobs, colorectal surgery fellow at Children's National in Washington, D.C.
In today's episode, we'll review a very intriguing case of a cloaca varian.
We have our usual host, Dr. Frischer and Dr. Levitt, who will introduce our special international
guest.
Carlos Reck is a very established pediatric colorectal surgeon in Vienna, Austria.
So, welcome, Carlos.
Thank you, Mark, for this great introduction.
Thank you, Jason, for having me.
Carlos, you want to tell us about a case that you brought for discussion?
So, this patient was originally here in Austria.
We have an organ screening around 20 to 24 weeks pregnancy where they saw that the child
had an intra-abdominal cyst.
So, she was referred for a prenatal MRI, which was done around 28 weeks.
If you're in the state-current app, open the first image where you can see two panels taken
from this fetal MRI and follow along as Dr. Reck describes the findings.
They still were able to see the structures, which appear to be a double cyst, differential
diagnosis, mesenteric cyst, differential diagnosis, ovarian cyst, differential diagnosis, hydrocholpus.
I think in a female with a large abdominal cyst, certainly with any associated urologic
issues, they should hopefully think about cloaca and any of the other bacterial anomalies.
Let's say the radius is missing or the sacrum is missing.
There should be suspicions that that cyst is associated with the hydrocholpus.
And yes, I thought definitely of the possibility of a cloaca, especially by seeing the location
of the cyst and this double configuration, which for me wouldn't make sense for, for
example, a mesenteric cyst or ovarian cyst.
In any case, she was referred to our hospital to give birth.
The child was born full term with a good size over three kilograms and didn't have any
trouble and the respiratory problems didn't require any type of resuscitation.
The second image shows the perineal exam.
Note the good technique for a female newborn exam, which retracts the labia majora upward
and outward to maximize visualization.
You can see there was only one perineal hole.
I just want to point out a couple things that orifice is large for a cloaca.
It's normal appearing.
Obviously there's no anal opening.
So I think that's an important finding that often means that the confluence of structures
is very low.
I find that if the single perineal orifice is very, very tiny and right at the clitoris,
that usually means a high confluence.
You can actually see that there is an abdominal mass protruding, which is something I could
feel on examination.
There wasn't an anus, but there was a good anal dimple and there was not a flat bottom,
but rather a good, well-developed child bottom.
I decided to take it to the OR to drain this hydrocopos where I was planning to do a cystoscopy
of this orifice.
I first went with a cystoscope.
Surprisingly, I wasn't able to get into any vagina.
Basically what you see is a direct entrance into the urethra and couldn't find any type
of UG sinus.
That's very interesting.
So you cystoscope the patient through the single perineal orifice and the only thing
you saw was essentially a urethra that entered a bladder.
No rectal or vaginal communication with that single channel.
Is that what I'm hearing?
Correct.
So that's a little crazy and I'm fascinated by the fact that this patient obviously had
a hydrocopos.
How does that happen?
How do you get a hydrocopos with no urine connected to it?
So that's where my whole diagnostic dilemma was and I couldn't explain it.
Yeah, well, so I thought the first thing is first.
So let me drain it and create a colostomy.
And I wanted to send some of this fluid to establish creatinine and see if there is urine
in there.
By the way, what did the fluid look like?
So when it came out, it was basically a little mucus looking fluid.
It wasn't infected.
It wasn't like urine smelling or anything.
It was just like a amber colored, dark, not completely transparent fluid, which was pretty
easy to drain.
I don't think it's urine because there's no communication, but obviously there's vaginal
mucus and the maternal hormones are affecting that.
And that's one of the explanations for lots of fluid.
Do we really know there's no contribution from urine?
I mean, could be an ectopic urinary connecting to one or both of the vaginas that we know
or that we don't know yet at this diagnostic moment.
And by the way, was there associated hydronephrosis?
So there was one unilateral, but it was only like grade two and on the other side it was
like grade three hydronephrosis.
So going back to the OR, after the cystoscopy, how did you deal with the hydrocopos?
So in the operating room, I decided to drain it.
I shortly was considering doing a vaginostomy, but I wanted to be less invasive and decided
to use this pigtail catheter and see if I can drain it with that so as to not create
a lot of abdominal scaring and intra-abdominal adhesions because I didn't know what was coming
next or what would be happening as far as next surgeries are concerned.
We talked about this at another podcast, the options of draining a hydrocopos.
You chose to use a pigtail catheter.
This is one that's large and one could consider a tubeless vaginostomy, just suturing it to
the abdominal wall, which is an option.
I recognize that you felt that that might ultimately tether the pull through, although
I haven't found that really is a concern and it's very easy to just take it down if it
is tethering and I haven't had it tethered.
There's plenty of vagina here.
So that's one way to sort of reduce the infection by not having an indwelling tube if the hydrocopos
is large and this one is above the umbilicus.
And what about the septum because you have two separate structures that need to drain
sometimes.
Well, I think the main factor is first, can you decide?
Of course, in this case, it was a very obviously and it was seen an ultrasound that there was
no septum and that there was a huge cyst.
And the second one is if you have a smaller one and you can see it's divided, I would
usually do them open and drain them openly or do a laparoscopy and then take a look at
it.
Again, in this case, I decided not to do it because of the size of it and I felt pretty
comfortable by seeing that there was no septum or ultrasound that I would be able to drain
it just with one catheter put in there.
How did you do that beautiful stoma?
There's no skin bridge.
Dr. Levitt is seeing image 3 in the state current app showing the abdomen post surgery.
Well, the way I do it, I usually make like a one and a half centimeter incision and then
with a bab cup or something, I go for the sigmoid colon and bring it up when I can identify
if I can see a white line, I will identify it as such and bring it bring up a loop and
then divide it outside.
And one side I would do a little purse string and then bring it up through a second incision.
You know, it's a great trick and we've been doing many of our stomas in the neonates laparoscopically
now because I think avoiding that skin bridge is helpful from a wound care issue.
But yes, we have made sure or in short that we have emptied our distal limb doing irrigations
because as you know, and everyone knows, when you leave some meconium in there and you're
not doing your definitive repairs and colostomy closures for six months or so, that becomes
almost petrified and extremely difficult to evacuate and causes problems.
So yes, I usually also do laparoscopy.
In this case, I didn't because I didn't know this huge cyst, even if it was strained, how
I would be able with the laparoscope to look around it.
But at this point, I was only concerned in doing what's necessary to get all the this
was a newborn.
So I want to just try to be stabilized and to have all his functions functioning and
then go on to do further diagnosis.
So after the surgery, the patient is able to feed and eventually be discharged from
the NICU with the pigtail drain in place with plan for further diagnostic testing as an
outpatient.
Look at image four, showing an MRI at about one month out.
What's going on here, Dr. Rek?
Two days before this image, the child had lost the pigtail catheter, which is definitely
a disadvantage of having a catheter left and sending them home.
In any case, she didn't come for care and only came after two days because she had the
MRI, luckily, which was an MRI.
I mean, you can see that the hydrocoposal had failed again to this massive amount.
So I ended up draining it acutely, putting a new pigtail in there with my ultrasound.
The patient also had a distal colostogram done, shown in image five.
What do you think of this study?
There's not enough pressure buildup there, but you can see where the colon kind of ends.
So it wasn't a very high colon.
You can see it.
There was a good developed sacrum and the colon was at the PC line.
If you imagine a little more pressure in there.
But again, it was blind ending.
The line you can see with contrast was actually outside.
So this wasn't a fistula or anything.
I don't think this image is that bad.
It's reasonably well distended.
You can see the sacrum.
You can see the perineal marker.
Yeah, I mean, a little bit more distension just to be absolutely certain that there's
no fistula.
You have a good idea of where the rectum is that you can reach it from below.
So now you have the imaging, you know, obviously that there is a urethra.
You're happy with the location of the urethra and there's no connection to it.
So urethra is urethra.
Done.
Now you have to go posterior sagittal.
And then what did you find and what did you do?
I decided to go with the posterior sagittal approach because there was a low rectum and
I assumed that if there was a vagina, I would be able to find it from behind.
Image six shows an intraoperative photo during the cloaca repair.
The rectum is retracted upwards with the vicro sutures and the vagina is retracted with the
silk sutures.
And you can see I have put stitches around the vagina and brought it down or I'm putting
some tension on it that you can see on the picture.
And in this picture, you cannot see it, but by this time I had already opened the vagina
and seen inside.
So I was sure this is a vagina.
Any septum or how many services did you see?
Did it confirm what you saw on your imaging?
Yes, I didn't find any septum and I only found what appeared to be one services, but wasn't
very clear because of all this redundant tissue of the deflated structure.
So at this point, you can also see in the picture that the urethra was untouched.
I left it there.
The opening was left as it is.
And then I was able to bring the whole, I tried to separate the vagina from the bladder.
At this point, it was almost at the bladder neck that you can see below it and separated
as much as I could until I had no tension.
And then I had the rectum, the hydroculpus or vagina I would call and was able to do
the vaginoplasty and the anoplasty.
I was planning that if I need to do an interposition or something, I would go into the abdomen,
which luckily I didn't have to.
Yeah, a little bit of a black box, not having the usual connection points and
maybe doing a laparoscopy first would also have been the correct thing.
But again, I was afraid of having a lot of redundant tissue from this huge cyst and not
being really able to differentiate a lot with the abdomen being so small and not being able
to create a lot of space in there.
Yeah, I think that was good judgment.
I agree.
I like to light things up.
I would have the two important points relative to the hydroculpus, just to reaffirm what
Jason said is be aware there's a septum and you have to remove a little piece of the septum
and then make sure both sides are properly draining.
And then a contrast study through that tube or vaginostomy would be very valuable.
We would do it all together.
We would inject the distal rectum, the hydroculpus if present, and the bladder.
So all together is in a 3D cloacogram or a fluoro if you don't have the ability to rotate.
But I think cystoscoping the patient, putting a catheter in the bladder, putting a catheter
in the mucus fistula, putting a catheter in the hydroculpus, which you already have, and
then doing a fluoro study, particularly in the lateral projection, you would have had
very good data and been very confident that going in posterior sagittally, you would be
able to find both structures.
And then in this unique case, no work needs to be done on the urethra because the common
channel itself is the urethra.
Once you have absolutely confirmed, and it seems like you have successfully done that,
that there's no vaginal urethral fistula or rectovaginal fistula related to the rectum
part.
So very interesting case.
And I think, again, evidence that you got to know your anatomy.
I mean, at the end, I didn't know how to call this because calling it cloaca, we didn't
have a really common channel.
Yeah, I would call it.
I would definitely call it a cloaca, but I would say it's a variant.
You have a sink.
You have no anus and you have a single perineal orifice.
It's a cloaca.
The only thing that's particularly unique here is there's no vaginal or rectal connection
to the common channel.
This is a great anatomic case.
Phenomenal.
Carlos, any jokes from the overseas?
No, unfortunately, I don't have a joke.
I'm very bad at telling jokes.
Well, you know, it's still Halloween season, so a simple little discussion is I want to
say a knock knock joke.
Knock knock.
No, who's there?
Boo boo hoo.
Boo boo hoo hoo.
You don't have to cry about it.
Thank you for joining us, Carlos, and great to see you.
Great to see you all.
Thanks also a lot.
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