Uterine Anomalies: Congenital and Acquired - SD

Introduction

I am Brian Bromley.
I'm a clinical associate professor of obstetrics
and gynecology at the Massachusetts General Hospital
and with an appointment in O-B-G-Y-N
and Radiology at the Brigham and Women's Hospital.
Today's lecture will be on malaria duct abnormalities,
both congenital and acquired,
and their impact on perinatal outcome.

The Role of 3D Sonography

The advent of 3D sonography
has been instrumental in our ability to detect
uterine congenital anomalies.
Those of us who are obstetricians
and radiologists involved in
reproductive medicine have known
that Malian duct anomalies are associated
with a wide variety of adverse perinatal outcome.
This includes things like recurrent miscarriage,
preterm labor and birth, intrauterine growth restriction,
mal presentation, incompetence, cervix,
and postpartum bleeding.
Interestingly, malaria duct anomalies typically don't
prevent conception and implantation.
A study by Heine and fert
and Stur in 1983 looked at 228 women
with malaria duct anomalies.
9.1% of them had primary infertility,
but most had other issues as the etiology
of their inability to conceive.

Prevalence and Challenges in Data

Now, any of the numeric data that we use, for outcome
or prevalence concerning Malian duct
anomalies is quite fuzzy.
And the reasons behind this is that most
of the major studies use widely different populations.
Some include fertile women, infertile women, women
with reproductive pregnancy losses,
all which affect the numbers on the other side.
There are many women with malaria duct anomalies
who are asymptomatic and don't present for medical care.
In general, in the general population,
however, approx there are the incidences of malaria
and anomalies is approximately 4.3%.
In the infertile population, this is 3.5%
and rises to 13% in women with recurrent pregnancy loss.
One of the better done studies looking at the frequency
of malaria and duct anomalies was done
by Simon Etal in 1991.
They looked at 679 fertile women
who had a laparoscopy
or laparotomy prior to a tubal ligation, followed
by a histo celling agram.
22 of 679 women,
or 3.2% were diagnosed as having a malian duct anomaly.
The anomalies that they saw were 90% septate uterus,
5% bico uterus, and 5% Delphi uterus.
Looking at other patient, populations, Opel Al
reported the distribution of anomalies
that was slot considerably different.
Um, they reported a 35% septate uterus
26% by corn uterus.
18% had argue uterus,
10% had unicorn uterus,
8% had uterus delphis,
and 3% had a genesis of the malaria system.

Classification Systems

Now there are different classes,
classification systems.
The most commonly used was a modification
of the original system by Butra
and Gibbons in 1979, where the American Society
of Reproductive Medicine identified seven classes
of uterine abnormalities.
And the classification is based on the degree
of failure from normal development, the symptomatology,
the treatment, and the prognosis
associated with these outcomes.
And many of you may be familiar
with this classification system, which is outlined here.

Embryology of Müllerian Ducts

Now briefly, embryologically, the
Malian ducts are paired structures that form
lateral to the ovaries.
The, inferior portion extend media,
coly and fuse together the
inferior duct then develop into the uterus, the cervix,
and the upper vagina.
This, the origin
of the lower vagina is distinctly
different, followed by the fusion.
There is resorption of the medial wall of the septum.
Notably the fallopian tubes are of different cellular origin
and are generally not involved with malaria duct anomalies.
And similarly, the ovaries arise from the mesenchyme
and epithelium of the gonadal ridge
and are not influenced by the formation of the meric
or perinephric ducts.

Diagnosis Using 3D Ultrasound

3D. Ultrasound has been monumental in our ability
to diagnose uterine anomalies
because we can get a volume acquisition from a series
of 2D images and display them in any plane that we want.
The coronal plane is the most important plane to
identify malaria abnormalities
because it shows the triangular shaped endometrial cavity
as well as the serosal surface of the fundus of the uterus.
The technique for obtaining the cor, the coronal view,
was described by Dr.
Abu Hamad and has been termed the Z technique.
This is an example of how it would be done.
You can see an image A, that the endometrium is aligned
so that it is parallel to the horizontal axis.
The reference point and positional
dot is placed on the endometrium.
Then one goes to the B coronal plane
and you can rotate it again around the Z axis up
and down the, towards the fundus of the uterus.
Again, with your reference point in the middle
of the endometrium, this allows you
to have a perfect coronal view
of the uterus in the sea plane.
And here you can see that you can
identify the triangular shaped endometrium
and the surface contour of the uterus.
In its convex appearance,
one can also use a rendering modality to
evaluate the uterus.
Here we are decreasing the size of the uterus,
again in image A, aligning the
endometrium parallel to the horizontal plane
going into the B plane, looking at the transverse image,
aligning that, and there's our rendered view
of the uterus in the coronal plane.
We can scroll in and out and look at the outer contour.
We can look at the endometrium
and its normal triangular shape,
and this is a normal uterus.
Similarly, we can look at a uterus
with a congenital anomaly.
Here we enlarge the image in screen A,
we are putting our reference.in the middle
of the endometrium.
Now we're moving our reference.in the transverse view
to the middle where the defect is
back to image A,
where we're again realigning the endometrium so
that it is parallel to the horizontal axis.
This gives us our coronal view.
We rotate the coronal view around the Z axis so
that it shows itself in its traditional orientation
and can scroll back
and forth through the volume, looking at the cornea, looking
where the cervix might be identified.
Now if we wanna render the image, we go up
to an the render mode,
and again, we align on the A image, our endometrium so
that it's parallel to the horizontal axis.
We move our green line to the area
of interest,
then we proceed to be where we rotate
our transverse view of the uterus so that again,
our endometrium is horizontally aligned with the
X axis
back to the A plane to perfect the endometrial orientation.
And there we have our rendered image in the D box,
which we can turn into its traditional orientation.
And again, we can scroll through the volume.
Looking at the cornua,
We can evaluate the outer surface
of the uterus showing that it is convex
and evaluate the endometrial lining
as it splays towards the fundus.
We can go on to measure the uterine septum
using our calc package.
The calipers go across the base of the septum
and down to its deepest depth,
and our measurements are there in the right
hand, lower quadrant.
Now this is instrumental.
Many people will say that they can tell on 2D
that there is splaying of the endometrium.
This is obviously a bate or a septate uterus.
But I would say that both these images at the top
look fairly identical.
And if you look at the coronal view,
they're very clearly different.
The left image is a mild indentation that you would see
in an arcuate uterus.
While in this image, you can see a deep septum
extending down into the lower uterine segment.
Similarly, these two coronal images
are fairly identical,
but if you do the coronal plane, you can see that this
outer contour is indented characteristic
of a bico uterus,
while the outer contour in this image is convex
with a broad based septum in a septate uterus.

Diagnostic Criteria for Specific Anomalies

Now, to go over the different criteria for diagnosis,
a septate uterus results because of a defect in canalization
or resorption of the midline septum
between the two malaria ducks.
The external contour is convex flat
or mildly concave,
and there are two separate endometrial cavities.
You may have a complete septum as in the image on the left,
or you may have a partial septum
as in the image on the right.
These are 3D reconstructed rendered images
of a complete septate uterus.
The outer contour is convex.
There is a broad base tissue bridge extending down from the
fundus all the way down to the cervix.
Same thing, convex septum, broad-based tissue
all the way down to the cervix
septum may be complete or incomplete.
These are examples of incomplete septal uterus.
Again, the outer contour of the fundus is convex.
There splaying of the endometrium with a tissue bridge
that extends partially down towards the cervix
and in a different patient, another image
of a septate uterus with your convex fundus,
your splayed endometrium
with the tissue bridge extending only partially
down the uterus.

Septate Uterus: Outcomes and Treatment

3D ultrasound has been, as I mentioned
before, incredibly important in the diagnosis
of uterine anomalies.
Al have reported a sensitivity of a hundred percent
with a specificity of 80%.
Sonohysterography adds to our ability
to be certain about our diagnosis.
We can clearly see the endometrial cavities,
which if the endometrium is not thick, may not be quite
as apparent on normal 3D.
Ultrasound and algo
colleagues have reported a sensitivity in a specificity
of a hundred percent using sonohysterography.
Now, a variety
of adverse reproductive outcomes have been reported.
With the septate uterus, this is a coronal view
of a septate uterus.
As you can see, a small gestational sac
within one of the horns.
This is a transverse view of the same, patient again
with a small gestational sack.
And one of the horns and a little yolk sack
septate uterine have the highest risk
of adverse reproductive outcome.
And this may be due to a variety of reasons.
Two of the hypotheses have been abnormal.
Vascularity to the uterine septum or abnormal endometrium.
Gram is did a large review of reproductive outcome
with septate uterus.
And my point is not to have you memorize the exact outcomes
because the data is very fuzzy.
As I mentioned before, a lot
of the studies are done on different populations.
There are different ways to classify the anomalies,
different diagnostic methods by which the data was acquired
and so on and so forth.
However, the important point is to realize that the risk of
pregnancy loss from spontaneous abortion is as high
as 28%.
14.8% will deliver preterm,
and 56% will have a term delivery of these women.
With septate uterus, only 66% had living children.
Now, hysteroscopic metro plasty has been shown
to be useful in reproving improving reproductive outcome.
This is a different study by Homer at Al,
hence the different numbers
where pre hysteroscopic metro plasty,
the miscarriage rate was close to 90%.
The disparate numbers being due
to different populations studied
and the term delivery rate was quite low at less than 10%
after the resection,
the term delivery rate was way up in the high 88%
range, and the miscarriage rate was
more in the mid 15 20% range.
The goal in the surgical management is
to restore normal uterine architecture
and preserve fertility.
I'm grateful to Dr. Laos in Jordan
for providing me these images of histo, scopic,
resection of a septum.
You can see this is looking into the septate uterus.
You can see the septum right here and it is taken away.
And this is the post, septate removal images.
Now a less than one centimeter residual septum
is considered an optimal resection.

Bicornuate Uterus

Moving on to bi cornea uterine,
these occur when there's a failure of the malian ducts
to completely fuse, and there are different types.
There's the bi coordinate uterus, which has one cervix,
which has been termed unicos
and the bi coordinate uterus with two services,
which has been termed
bico Arbitrarily.
The definition of a bico uterus has been a fundal
indentation of greater than one centimeter.
Here you can see a coronal view outlining the fundus
and a big dip between the two horns
of a bico uterus.
One endometrium is splayed this way
and one endometrium is splayed.
That way we can measure the degree of indentation.
Another method of classifying a bico uterus is a
fundal indentation below the inters osteo line,
or less than five millimeters above.
This is another way to measure this type of bico uterus.
This is the transverse image of that uterus.
In comparing, this is a septate uterus, we talked about the
convex outer fundus,
and you can see that there is a lot of tissue on top
of the inter, a line drawn between the inter oste points.
This is an example of a bi cornal bico uterus.
This is the coronal plane.
You can see the fundal indentation splayed endometrium,
and you can follow them right in their two separate
endocervical canals.
This is a 3D representation of a patient with a bico uterus.
You can see the fundal indentation,
and in this case, the patient has an IUD in the middle
of the bi cornea uterus.
Now bi coordinate uterus is the most common
of the mullerian duct anomalies to be associated
with cervical incompetence.
The reproductive outcome was amalgamated by al.
They reported on 261 women with bico uterine
resulting in 627 pregnancies.
Again, the rate of miscarriage is very high at 36%, 23%
of pregnancies delivered preterm,
only 40% delivered at term,
and 55% of women had a live birth.

Unicornuate Uterus

A unicorn U uterus occurs when there's failure of one
of the mullerian ducks to elongate,
and this is seen on the right side more than the left.
This is the netter drawing of a unicorn uterus
on the left and on the right you can see the 3D coronal view
of a unicorn uterus.
Now the unicorn uterus has been divided into
several categories.
It can be isolated.
There can be a communicating horn, as you can see here,
a non communicating horn where there's no communication
between this vestigial horn and the main horn.
You may or may not have an endometrial cavity,
and as I mentioned,
occasionally they are isolated.
This is a standard 2D ultrasound of a uterus
where this is the transverse view of the fundus.
This is the sagittal view,
and I think years ago we would've passed this
as a normal appearing uterus.
However, if you throw on your 3D
and you do a 3D reconstruction, you can see
that this is a very abnormal shaped uterus,
and in fact is a unicorn uterus.
This is another example of a unicorn uterus on sagittal
standard sonography and with the 3D coronal plane
and another 3D image of the reconstruction
of a unicorn uterus,
this is an example of a unicorn uterus
with a rudimentary horn.
You can see the unicorn main horn here
and the small rudimentary horn
with some communicating endometrium.
This is a unicorn uterus with a
non communicating horn with endometrium.
This is theor side.
This is the non communicating horn with a little bit
of endometrium up here.
This is an example of a Ute unicorn uterus
where a 3D reconstruction has been done.
The rendered image here shows
that you have a unicorn uterus with a fused horn,
but no endometrium within that fused horn.
Now, reproductive outcome for women with unicorn,
uterine is poor as well.
Again, a third of these patients,
a little over a third will have miscarriages
and pregnancy losses.
16% will deliver preterm
and only 44% will deliver at term.
There are 54% live births in this population.
Now, it is generally accepted that removal
of a non communicating rudimentary horn
with functional endometrium is in fact a reasonable thing.
It reduces dysmenorrhea and endometriosis
and the risk of ectopic in and of itself,
however, it does not impact on reproductive outcome for the
one part of the horn that is present.
Importantly, however,
if one does get pregnant in the obstructed horn,
there's an 89%, risk of uterine rupture,
and that is an obstetrical catastrophe.
Now, unicorn uterus
or any of these uterine abnormalities may have some
of the more standard, issues that we see
with regular uterus,
and this is a unicorn uterus with a large endo
cervical endometrial polyp.

Uterus Didelphys

The uterine delphis occurs because of complete failure.
Failure fusion.
This is a transabdominal scan of uterus delphis.
You can see one horn here
and one horn here again, a larger image,
one horn, the other horn,
Grazi etal looked at 114 women with uterus delphis
and having 152 pregnancies.
And again, the reproductive outcome is similarly poor.
Here's an image of a uterus delphis with
a normal appearing left horn right here,
and a right horn that has a fetus within it.
And later on you can see there's your left horn,
which is normal appearing
and your right pregnant horn with the amniotic fluid.
Now, 20 to 40% of women
with malaria duct anomalies will have an associated renal
anomaly in an obstructed horn.
Many of the times this is an ipsilateral renal agenesis.
Other types of abnormalities
that might be seen are horseshoe kidney duplicating,
collecting systems, pelvic kidney and ectopic ureters.
So it's always crucial in a person who's diagnosed
with a malaria duct anomaly
to perform a formal evaluation on the kidneys.

Arcuate Uterus

Now, the AQ at uterus is a somewhat controversial finding.
Some people have classified it as a septate
or bi corn uterus.
Other people have considered a variant of normal.
The outcome data is widely disparate.
Here's a 3D ultrasound showing the coronal view
with a convex contour
and a broad based tissue band
that extends just minimally down the endometrial canal.
The outcome for pregnancy
for an arcuate uterus has been very variable.
In this study by grames, the outcome was quite poor, as in
with other anomalies.
However, the live birth range looking at other studies,
has been as good as 83%.
And it may be that many of women with this type
of relatively mild anomaly go undiagnosed,
and we're not really aware of what their outcome
their better outcomes may be.

Acquired Anomalies from Diethylstilbestrol (DES) Exposure

Ethyl sterile is a synthetic estrogen that was introduced,
into clinical practice between 1949
and 1971,
and was used for a variety of poor obstetrical events such
as miscarriage, bleeding, et cetera.
Women exposed to this in utero have
a variety of uterine abnormalities,
most commonly the T-shaped uterus,
and this is an example of a coronal view on its side
of a T-shaped uterus.
You can see the T-shaped endometrium extending
down to the cervix.
And this is a 3D representation of a woman
with a T-shaped uterus.
You can see a normal convex outer fundus
and the T-shaped endometrium

Acquired Müllerian Anomalies

Fibroids

now acquired.
Malaria anomalies are things like fibroids or polyps
or maybe uterine signia.
And for the presence of fibroids, these occur in about 20
to 40% of women during their reproductive years.
However, it really depends on
what population you're studied.
They are seen in 2.7%
of women having a second trimester ultrasound.
They've been identified in 12.6%
of women undergoing IVF,
and in as many as 25% of women who have
been undergoing IVF with a donor egg.
This is a sagittal image through a uterus.
This is the endometrium,
and you can see this small hypoechoic area protruding into
the endometrium characteristic of a submucosal fibroid.
I'm gonna limit my con my comments to submucosal fibroids
because subserosal fibroids have no known
effect on fecundity.
Intramural fibroids are highly controversial,
and if they do have an impact,
it's probably not an enormous impact,
but that remains to be definitively assessed.
Submucosal fibroids are thought
to decrease implantation rates,
lower clinical pregnancy rates,
and are associated with a higher risk of miscarriage.
This is a coronal view of a uterus
and with a large submucosal fibroid extending into the
endometrial cavity.
Re A 3D reconstruction shows the
rendered image at the time of a
sonohysterography of a fibroid.
This is the fluid and this is your
essentially completely submucosal fibroid.
This is what that type
of fibroid might look like on hysteroscopy.
Again, I wanna thank Dr.
Laos for providing me this scopic image.
It is felt in general that surgical removal
of submucosal fibroids are associated
with a higher implantation rate.
Cassini at al in a study in 2006 looked at a group of women
who had had a greater than one year, plight of infertility and had fibroids.
They looked at spontaneous conception after surgical removal
or non-surgical, or no surgery in these women,
and they found that women who had surgical removal
of their fibroids, a spontaneous conception was achieved 41%
of the time as opposed to 21% in women
who had not had a surgical removal of their fibroid.

Endometrial Polyps

Endometrial polyps are a different, subject.
End endometrial polyps occur as an overgrowth
of the endometrial tissue.
You can see this projection into the endometrial canal shown
on a sonohysterography with a feeder vessel characteristic
of an endometrial polyp.
Endometrial polyps are seen in between 16
and 26% of patients with unexplained infertility
and between 0.6
and 5% of patients with recurrent pregnancy loss.
Perez Medina et al looked at 215 women with
endometrial polyps,
and there were some
of them underwent hysteroscopic polypectomy.
Others underwent hysteroscopic, evaluation
and biopsy of the polyp, and they looked at pregnancy rates
after four cycles of IUI, there were a total
of 93 pregnancies in the group, 64
having occurred in the women who had had the resection
of their polyp and 29 where the polyp was left alone
with the exception of the biopsy.
See, interestingly
enough, 65% actually conceived
before the planned IUI,
so there may be a slightly increased risk,
slightly increased likelihood
of pregnancy if the polyp is removed.
Having said that, this is also controversial
because lasal have shown using IVF
or Ixi that there is no difference in pregnancy rate
or miscarriage rate when the polyps are less
than two centimeters.

Intrauterine Adhesions

Moving on to intrauterine adhesions.
Intrauterine adhesions are seen in 1.5%
of the general population,
and they're seen in between seven to 30%
of women undergoing hysteroscopy after a miscarriage
and subsequent curettage.
Most often we see these kind of intrauterine adhesions
after either a postpartum curettage
or a post aboral curettage.
This is a coronal view of an image of a woman
with intrauterine adhesions.
You can see the endometrial cavity is a little bit more echo
bright like you would normally see,
and then you can see these strandy pieces of tissue
crossing the endometrial cavity in no specific alignment.
This is characteristic of uterine sinia.
Now the classification of uterine
Signia is available from the American Fertility Society,
now called the American Reproductive Society,
and is based on the extent of cavity involvement, the type
of adhesion and the menstrual pattern that the woman has.
This is a woman who had had two DNCs
after a retained placenta
and had an abnormal bleeding pattern.
This is the sonohysterography of her uterus,
and you can see that there is a
large structure extending across her uterus,
with no particular orientation,
which would suggest either a septum
or any other congenital anomaly.
This, again, is a transverse view of a uterus
and a woman with a signia.
You can see the little tissue bridge extending across the
uterus in this transverse view,
Schenker etal looked at 292 women
with intrauterine adhesions.
45% became pregnant.
However, there was a high risk of miscarriage
and a high risk of preterm delivery.
Now, adhesion removal has been associated
with an improved pregnancy outcome Valley et al,
and the American Journal, reported on 187 women having 143 pregnancies.
These women all had intrauterine adhesions.
Here you can see a sonohysterography again with a woman
with adhesions.
This is the transverse view of the fundus of the uterus,
and below it is the sagittal view.
Here it is of the uterus,
and you can see this hypo coac, tissue bridge extending,
and here it is up here.
The removal is associated with a much better
reproductive outcome than that his,
than historical controls.
Now, the pregnancy rate does depend on the type of adhesions
and the degree of cavity involvement.
This is a hysteroscopic image of what some
of these adhesions might look like.
The live birth rate is 81%
after removal of mild filmy adhesions and not so great
after dense adhesions.

Thank you.