Welcome to our next episode of Colorectal Quiz. I'm Liza Bokova, Colorectal Research Fellow at
Children's National, Washington DC. Today we're going to discuss a case of a malaria anomaly in a
patient with cloaca and we will start off with Dr. Levitt from Children's National.
It's another day, it's another topic. Welcome to the Colorectal Quiz.
I get calls and I know, Mark, you do as well about missed gynecologic issues in simple
anorectal malformation patients. That's our second host, Dr. Frischer,
Director of the Colorectal Center at Cincinnati Children's Hospital.
I'm proud to say that in both Jason and my places here, gynecology is part of the process. I want
to welcome Dr. Leslie Breach, my good friend for many years, who really is, in my view,
the first congenital gynecologist. Thank you and Dr. Frischer for inviting
your favorite gynecologist to join you. And then I'm very blessed to be working
with Veronica Gomez-Lobo, who is her counterpart on this side of the country in Washington DC,
who runs the GYN program here. We're really blessed to have you all include us in this
multidisciplinary care because in other countries, we just had a visiting professor from Korea. She
just sees the kids later in life when they have a problem in adolescence or when they're adults
and have questions about pregnancy and sexual activity. So today we're going to discuss a case
of a 12-year-old female with a history of cloaca, 2 cm common channel, with neurogenic bladder,
UTI, and hydronephrosis. She is also a status post-stethocord release, ASD and VSD repair.
She underwent a PSA or VUP with total urogenital mobilization, creation of a malone with assessment
of mullerian structures and colostomy closure. This story begins today when she presents for
her follow-up at puberty. She has a twin sister and her twin sister is showing signs of advancing
puberty. The patient we're talking about has not yet achieved her period. At what stage of the
patient's care are you making the assessment of their anatomy? During the original EUA to assess
where the fistula is, we perform a vaginoscopy. The vagina does not have a sphincter so you need to
squeeze the labia around the scope so that the vagina does fill up with water. And what are
you looking for? We look to see where the cervix is and if there's one cervix or two cervix. I think
before you even start to do that you really should grab the labia and pull them out so that the vagina
opens up just to assure that there isn't a septum that goes all the way out because sometimes you
can put the scope into one vagina and never realize there's a second vagina there. And then
if you are able to see a cervix in the midline, usually we feel like there might be just one
uterine structure but again you can have a second vagina that you might be missing and that's why
it's so important to look at the entrance of the vagina and assess the perineum very well.
What if you see a septum higher up and two cervixes? Then you know what the anatomy is. There's
two uteruses with two vaginas and two cervixes. But what if you see one cervix and one vagina?
And you don't really know what the anatomy is. We recently had a patient that we did a vaginoscopy
and the ultrasound showed a possibility of two vaginas. We did not find two vaginas.
Then we did the PSARP from below and we did not find two vaginas and then she came for her
post-operative evaluation and lo and behold we found the other vagina. And so anytime the baby
is under anesthesia she should be evaluated. So let us come back to the case we're discussing
today. This young lady had a vaginoscopy during initial repair. There was the visualization of a
single cervix. And how do you assess the anatomy after the initial repair? When they're in the OR
doing the colostomy closure for a cloaca we will do a vaginoscopy to check the repair but also
look in the abdomen to make an assessment of those reproductive structures. Particularly in cloaca
the majority of patients will have duplication. So just because you see the one don't be satisfied
be thinking is there another and how can I best assess for that? Is that at this moment or will
there be these opportunities that we're going to highlight in our conversation as we continue?
You know what that reminded me of? Leporotomy for a gunshot wound. You need to see an entry and an
exit. You only see one. You got to be confused. Where's the other one? At the time of the
assessment of the cloaca gynecology was there, was part of the endoscopy, was looking in to their
orifice that they worry about. So the endoscopy during the, let's go the newborn colostomy,
if they have a hydrocopos, the cloaca repair endoscopy for that to prep for the repair,
the repair itself, both the perineal view and an abdominal view. If you get that far in the cloaca
repair, the colostomy closure, and then if child needs any other intervention like a Malone,
which is done laparoscopically or their vesicostomy needs to be closed or their ureters need to be
re-implanted, the third collaborating partner here for these cloacas is obviously urology.
So they may need to go to the OR for something. And then if all of that's done and you have a
pretty good idea, they turn age 12 and now you got to make sure once again that things are okay and
intervene if necessary. It is known that cloaca patients are at risk of menstrual obstruction in
the future. How can we assess this risk in a particular patient? We'll also try to attempt
test menstruation where we'll do what we call saline perturbation or to put some salt water
through the system to do the first period to assess that things flow well. We would just put
dye at the entrance of the cervix and see if it would come out on the tubes. This procedure
has been stumbled on over the years to ensure that there wasn't a risk in the future of an
obstructive phenomenon. Perturbation can basically be performed either in a retrograde fashion or in
an endograde one. Could you please tell us more about it? We at GLAAD have done something called
chromo perturbation to be able to assess the patency of the fallopian tubes using what we
would call sort of a retrograde perturbation. So at the cervical area to put dye through the system
and to be able to see that all is open. So a modification of that when in the abdomen uses
an opportunity to an endograde perturbation by using a small feeding tube in the distal aspect
of the fallopian tube and then in an endograde fashion instill the saline to ensure that that
side of the mullerian system was completely open. Are there any risks associated with this procedure?
A gentle perturbation carries relatively low risk. The other thing that can be done is a
histroscopy. So if you have a small enough cystoscopy equipment, sometimes you can go
into the cervix and look at the uterine cavity and that gives you an idea because you would see
whether there's one or two osteo. And should we be looking at gynecologic anatomy in other patients
with anorectal malformations like those with vestibular or perineal fistula? Yes, there is
an increase in the association of mullerian anomalies in all patients with anorectal
malformations. The closer the vagina and rectum are together, the higher the chances that there
is anomaly. So cloaca is the highest association of mullerian anomalies followed then by recto
vestibular and then the least being perineal fistula, which is still slightly above the
rest of the population. In this particular case, the patient had a cloaco repair, a PSA or VUP,
all from below. So there was no investigation of the abdomen because there was no opportunity to
do so. But I think then you alluded to the fact that at the time of the colostomy closure,
you've had an opportunity to look inside the abdomen and luckily you were working with a surgeon
that said, hey, gynecology buddy, we're looking into the abdomen. Would you like to join me?
We should be very careful to not have a mullerian black box because you don't want a 12 year old
that's in trouble. How do we avoid that black box from a workup standpoint? This is a case that was
managed by an interdisciplinary team that had a gynecologist assessing these uterine bodies,
but you will see along the way, it still wasn't a hundred percent clear until the blossoming of
puberty. So it can take longitudinal assessments over time and the blessing of the pixie dust of
puberty to make it clear to us. The one thing you did do here is you knew of the possibility,
checked, and then you intervened. Jason, you remember Fred Reichman used to say,
it's much easier to stay out of trouble than to get out of trouble. So what opportunities have we
got to open the black box of mullerian anomalies? You might get a clue prenatally. Sometimes you see
hemi vaginas, even on a prenatal ultrasound or MRI. We've had situations where the ultrasound said
there were two and we can't find two. And then we said, we better keep looking because there should
be two. I want to make a point too about what's happening hormonally. So when babies are first
born, they're under the influence of mom's hormones and then they go through a mini puberty. And that's
a great time to look at the mullerian structures because they get really, really small after that.
Once the hormones are gone, the reason that this declares itself in puberty is because the hormones
kick in again. But again, an ultrasound after birth is very valuable because the mullerian
structures are more visible. You could get some really helpful imaging during the neonatal period
because of the hormonal exposure from the mother's hormones. And then you lose that opportunity
really basically till puberty where imaging is a little tougher and maybe MRI is a little more
helpful, but then they have to get sedated. We all want to be cost saving, but we could get the
ultrasound at couple days or a week of life, but we're really not going to use that information now
so we don't need to get it, but we might've lost an opportunity there. So-
Do we ever need an MRI in a three or four year old to make a mullerian assessment?
I can't tell you how many girls I've had who've been told they don't have a uterus when they're
seven years old, who then at 13 get a period. So do not diagnose an absent uterus when somebody's
seven years old. Ultrasound is extremely good and MRI is extremely bad because the structures are
very tiny. All you really want to know is are anything dilated. And at that age, there's nothing
really dilated except to the point that you just made is in the very early stage when they're still
under a mom's hormonal effects. I think there's still a lot of MRIs being done that aren't
necessary. Of course, if they have symptoms and they're 12 years old, then MRI is an awesome
modality to see what's dilated. This little girl had a malone created at age four.
And so at age four, you can imagine there is no hormone stimulation. Let us hear what was
happening in the OR. If you are listening from the Stay Current app, you can pull up the photos
that are going to be described. So when we took a look, look at the first image in the Stay Current
app. We saw a beautiful image on the right that shows the right-sided malarion structure. You see
the fallopian tube and right ovary. But did you see some adhesions, particularly on the left,
likely from the previous colostomy? But you see there's a malarion structure on the left.
It doesn't seem to progress as far down in the pelvis, but definitely see the tube and ovary
there under some adhesion. So when I was there, we couldn't really make a good assessment. And we
tried again to do our saline perturbation at that time. But I think the question becomes, are we
traumatizing the distal fallopian tube under the prepubertal state? And so we said, great,
we have more information. We know there's only one cervix, but we see there's these two bodies,
and are they communicating or not? Then she returns to us, right, 12 to 13. Her sister as well,
it's puberty. And she was getting a Botox procedure. We thought, great, another chance to take a look.
And Veronica mentioned earlier the role of something called hysteroscopy, taking a scope
and going into the uterus, which most pediatric surgeons, I would not recommend be doing that.
But in this situation, being a gynecologist, the patient puberty stimulated, we put a small
scope into the uterus itself and did what's called hysteroscopy because we wanted to find out,
was there any connection that we could learn between those two malarion structures?
Have a look at the second picture illustrating the hysteroscopy findings.
We see the cervix, which is deviated slightly to the patient's right at the top of the vagina.
And we go through the cervix and into the uterine body, we could only see one osteo or opening into
the fallopian tube. So that small intervention at the time of her Botox procedure told us right
there that those two uterine bodies are not communicating into that one cervix. There is
one tube opening. That means there is a unicornuit uterus on one side. So that other side is not
communicating. And if there is endometrium within that non-communicating horn or part,
that's a problem. So that's why then we said, hey, let's get the post-fibral MRI.
Look at the MRI pictures. You can see in the pelvis, the right-sided
malarion structure, which is really a right unicornuit uterus, which is gorgeous. It has
endometrium, the most beautiful looking cervix I've seen in a while on that right side. So we
know that side is the one that's communicating to the vagina. But over on the left, there is a
non-communicating muscle structure with the dark color and the lighting up white endometrium,
which means that's a brewing period that has no way out. Look at the axial images.
You can see the same thing on these coronal images, again, showing the right-sided
unicornuit structure. And you can see an arrow with the image pointing to a less well-formed,
no cervix seen structure on the left. That is the danger structure. With that information,
then we spoke to family about the idea that there is a unicornuit uterus, one side open,
patent will allow the menstrual flow to come when it would happen. But that there was this left-sided
non-communicating malarion remnant, this malarion horn that we call it, this uterine horn, or what
I call a shack, because we don't want to keep a shack around. We want a house with a nice front
door leading down the sidewalk of the vagina and out of the firm. We need that for patient flow.
This shack needed to be removed. Interestingly enough, remember, this is a young lady who has a
wonderfully functional malone through her umbilicus. And that is typically where a lot of
times we would do the approach. But this is a left upper quadrant insertion of the scope. And she had
a laparoscopic resection of that left malarion remnant. Have a look at the fourth photo in the
attachments. We kept this beautiful right-sided unicornuit uterus, but now these mature beautiful
fallopian tube with fimbria and a beautiful ovary. And then we look on the left. Look at the next
intraoperative photo. And we see this non-communicating left-sided uterine remnant.
The tube is a little bit also adhesed, but you see the ovary above it. And there's another view
that you can see it's a little more prominent. You see that it's kind of protruding because it
has that endometrium that's not going to be able to get out and go anywhere. So we're going to want
to resect that, but preserve that beautiful ovary you see over there on the left. The blood supply,
we don't want to compromise that, but we also were able to keep this beautiful functional appendice
of malone, which we can see in the last photo. And you prevented her from having severe pain
because these uterine horns, it's so much worse than the other obstructive anomalies. They get
severe pain. And because these can be very small, sometimes people have a hard time identifying them.
When you do take out one of those atretic systems and you preserve the ovary,
what do you do to the tube? So if somebody doesn't need their tube and you're there,
they can just be taken out. Not only are you preventing ectopic pregnancies, but you're
preventing ovarian cancer. So more recently it's come out that what we used to think was ovarian
cancer, serious cystadenocarcinoma, is actually coming from the tube. How you remove a fallopian
tube and save an ovary because that is no picnic and these structures are relatively small.
So the main blood supply are the ovarian vessels. So you want to be protecting those at all costs,
but there are definitely many vessels that you find in the basal cell pinks. So we're going to
want to be close to the fallopian tube itself and you want to progress down to the uterine body,
stay close to the fallopian tube. But the ovarian vessels are the highest priority.
If the tube is here, the ovary is going to be underneath it and the blood supply is going to be
coming from the side. And so if you're above the ovary, close to the tube on the mesocell pinks,
then you can remove the tube fairly easily. Oftentimes in patients with renal anomalies
on the side of the malarion anomaly, we use stents that can be placed to help protect also the ureter,
which can be in that admixture of near the ovarian vessels, then going down into the pelvis.
And so it's important also to consider the blood supply to ovary as well as the ureter.
This patient happened to have two normal kidneys, but oftentimes we'll find these remnants and these
anomalies on the side of the pelvic kidney or absinthe kidney. As we've progressed to doing some
of the work laparoscopically, reproductive endocrinology colleagues remind us to be very
gentle and careful with the fallopian tubes, not to want to cause any trauma that could be subsequently
associated with hydrocephalic pancreas or scarring of the tube.
And what is the reproductive potential of this patient?
With this uterine horn, they should have normal reproductive potential. She's got this beautiful
tube that doesn't seem to be damaged by her previous surgery, but there is a higher rate of premature deliveries.
And here are some take home points.
We emphasize the importance of suspecting malarion anomalies in patients with anorectal malformation.
An early delineation of that anatomy is extremely important, particularly prior to the onset of
puberty so that we don't run into those emergent and more catastrophic situations where we have an
obstruction. But as you progress along the journey of caring for your patient with an ARM, there are
some pit stops that you can make along the way in those opportunities. The original repair,
colostomy creation, colostomy closure, other surgeries like creation of a Malone. But let's
say that those other opportunities aren't utilized, then there are imaging techniques. And we have
emphasized that maybe a post pubertal MRI might have a role, but really those pre-pubertal MRIs
are not as helpful. And if there is imaging, it's early on under mom's hormonal stimulation and
seems like we favorite the ultrasound as a team. Some of these cases are really a longitudinal
progression of deciphering that anatomy and having sort of red flag patients where you suspect that
there could be something more and all you see is one cervix. If you keep an eye out for this,
you're preventing this child a lot of pain. The different abnormalities of the malarion system
are associated with different issues with pregnancy and the families will want to know that too.
Our nurse Debbie did a project that talked a lot about how the awareness for parents very early on
in their care of their child with an anorectal malformation was very reassuring to understand
the reproductive anatomy, but they really signed on as a partner to say at puberty is the time we
really have to know. Any patient with an anorectal malformation has to go through a process we have
and probably many others have similar where we will not take that patient to the operating room
or schedule an appointment in clinic without our gynecology and neurology seeing if they want that
opportunity to either be in the operating room or have a clinic visit. That helps to avoid an extra
anesthetic for these patients. It is important to remember that malarion anomalies are associated
not only with cloicas. 10% of vestibular fistulas have an absent vagina. Well, you may see more
complicated situations. This hasn't been evaluated perfectly well with a huge sample size, but the
numbers that I use is 5% of vestibulars have a septum and 1% which might be high have distal
vaginal atresia and from before perineal it's much much more uncommon. You can have a septum in a
perineal and you can have distal vaginal atresia as well. But you will not be complete as a pediatric
surgeon without a gynecologist. Leslie, you complete me. Jason, thank you for suggesting this phenomenal
topic. Thank you, Leslie Breach from Cincinnati Children's and Veronica Goma's logo from
Children's National Hospital, our two partners. Awesome, thank you so much. Thank you everyone.
Thank you for joining us this week. This is Lisa Bokova from Children's National.
We hope you enjoyed it. Tune in with us next time.