Congenital abnormaleties of the uterus

CONGENITAL ANOMALIES OF THE UTEUUS
PRESENTED BY:
OUSMANE BECHIR HASSABALLAH
IAN DONALD INTER SCHOOL FOLLOWSHIP OF ULTRASOUND
Batch 6
10/1012015

Introduction
 Uterine malformations result from partial or
complete failure of one of three mechanisms
either separately or combined - agenesis,
fusion, and resorption. Agenesis results in
either a complete absence of the uterus or a
unicornuate uterus; a failure to fusion gives
rise to uterine didelphys or a bicornuate uterus;
and a septated uterus is due to a failure of
resorption.

incidence
 The incidence of muellerian anomalies has
historically varied widely due to the different
populations studied, small sample sizes,
prospective versus retrospective study designs,
different classification systems, and the type of
test used to make the diagnosis . The need for a
standard classification of muellerian anomalies
was self-evident.
In the general population the incidence of uterine
anomalies are estimated to be 3-4 % (Sorensen , 1988).
This increases to 7.3 % in infertile patients ( Saravelos
2008),

Why is this important?
 Majority have no problem conceiving, but
have higher rates of:
– 1. Spontaneous Abortion
– 2. Premature Delivery
– 3. Infertility
– 4. Abnormal Fetal Lie
– 5. Dystocia at delivery
– 6. Dysmenorrhea, endometriosis
– 7. Cervical incompetence

Uterine Anomalies
Absence of Uterus Fusion anomalies

Müllerian duct anomaly classification
 The American Fertility Society (AFS)
classified muellerian anomalies according to
the major uterine anatomic types. The AFS
classes of muellerian anomalies are:
 Hypoplasia/agenesis
 Unicornuate
 Didelphys
 Bicornuate
 Septate
 Arcuate

Dr Ayush Goel and Dr Frank Gaillard et al
 The Müllerian duct anomaly classification is a
seven point system that can be used to describe a
number of embryonic Müllerian duct anomalies:
 class I: uterine agenesis/uterine hypoplasia
– a: vaginal (uterus: normal/ variety of
abnormal forms)
– b: cervical
– c: fundal
– d: tubal
– e: combined

 class II: unicornuate uterus/unicornis
unicollis, ~6-25%
– a: communicating contralateral rudimentary
horn contains endometrium
– b: non-communicating contralateral
rudimentary horn contains endometrium
– c: contralateral horn has no endometrial
cavity
– d: no horn
– class III: uterus didelphys, ~5-11%

 class IV: bicornuate uterus: next commonest
type, ~10-39%
– a: complete division, all the way down to
internal the os
– b: partial division, not extending to the os
 class V: septate uterus: commonest anomaly,
~34-55%
– a: complete division, all the way down to the
internal os
– b: incomplete division

 class VI: arcuate uterus, ~7%
 class VII: in utero Diethylstilbestrol (DES)
exposure: T shaped uterus

Classification into 4 groups:
 1. Agenesis of uterus/vagina: Rokitansky-
Kuster-Hauser Syndrome.
 2-Unilateral development :Unicornate
uterus
 3. Lateral Fusion defects (obstructive or
non-obstructive).
 4-Defects in Vertical Fusion (obstructive or
non-obstructive)

Uterine agenesis
 is the extreme of Mullerian duct anomalies
(Class I) where there is complete absence of
uterine tissue above the vagina.
 Epidemiology
 The uterine agenesis-hypoplasia spectrum
accounts for ~10-15% of all Müllerian duct
anomalies.
 Clinical presentation
 Clinical presentation is characterised by primary
amenorrhoea, with normal hormonal levels
guaranteed by fully functional gonads.

Pathology
 Complete absence of the Mullerian ducts is
termed Mayer-Rokitansky-Kuster-Hauser
(MRKH) syndrome which includes absence
of the vagina as well.
 Associations
 renal tract anomalies including renal
agenesis
 skeletal: vertebral anomalies

The uterus is small, measuring just 3.8 x 1.2 cm hypoplasia

Figure 2: Pelvic ultrasound longitudinal showing the urinary bladder and
absent uterus

Figure 3: Pelvic ultrasound showing an ectopic pelvic kidney indenting
on the urinary bladder superiorly

Figure 4: Intravenous urography showing pevicaeal system of the ectopic
kidney

Figure 5: Enhanced axial CT of the pelvis showing contrast in the urinary
bladder (and rectal contrast) but no uterus

Sagittal and transverse ultrasound images, show a markedly hypoplastic
uterus, measuring less than 4.8 x 1.6 x 2.7 cm in a middle-aged female
patient with primary infertility.

Unicornuate uterus
Dr Yuranga Weerakkody and Dr Natalie Yang et al.
 A unicornuate uterus or unicornis unicollis is a
type of Mullerian duct anomaly (class II). This
type can account for ~10% (range 6-13%) of
uterine anomalies and infertility is seen in
~12.5% (range 5-20%) of cases.
 Pathology
 There is failure of one müllerian duct to elongate
while the other develops normally. The
embryologic predominance of the unicornuate
uterus to be on the right has not been explained. It
may or may not have rudimentary horn.

 Sub-classification
 It can be classified into to the following types
according to the
 type a: with rudimentary horn the American
Fertility Society 3.
– a1: horn contains endometrium
 a1a: communicating contralateral
rudimentary horn contains endometrium
(10%)

 a1b: non-communicating contralateral
rudimentary horn contains
endometrium (22%)
– a2: contralateral horn has no endometrial
cavity (33%)
 type b: no horn (35%)

Associations
 renal abnormalities
– renal anomalies are more commonly
associated with a unicornuate uterus than
with other müllerian duct anomalies and are
present in 40% of cases, e.g. renal agenesis
– the renal anomaly is always ipsilateral to the
rudimentary horn
 cryptomenorrhea within endometrium
containing rudimentary horn that does not
communicate with the endometrial cavity
 primary infertility 4

 Ultrasound
 Can be diffcult to detect on 2D ultrasound.
The uterus may be seen tapering to one side.
 Hysterosalpingogram (HSG)
 MRI
Radiographic features

 Complications
 Of the Mullerian duct anomalies, a
unicornuate uterus is considered to have the
second worse obstetric outcome (worst with
a septate uterus).
 Spontaneous abortion rates are reported to
range from 41-62%. Reported premature
birth rates range from 10-20%. Fetal
survival rate is ~40% (range 38-57%).

Reconstructed 3-dimensional sonogram of a left unicornuate uterus in the
coronal plane showing the characteristic fusiform banana-shaped uterus
with a single left horn and a single endometrial cavity.

Unicornuate uterus after saline infusion

this ultrasound image (3D) is a coronal plane showing a unicornuate uterus.
The cavity (at "C") only goes to one fallopian tube (goes up to her right tube)

3 dimensional coronal image of a unicornuate uterus

Unicornuate uterus. A multiplanar display of the left unicornuate uterus is
shown. This diagnosis is difficult to establish with 2DUS because the uterus
may appear grossly normal or slightly laterally deviated. With 3DUS, the
diagnosis is confidently made because the coronal plane (C) shows clearly that
there is only a single cornual angle (arrow). The cervical canal has a normal
appearance. A, Axial view; B, sagittal view; C, coronal view.

Unicornuate Uterus Ultrasound Pregnant uterus – fetus

Uterus didelphys
Dr Jeremy Jones and Dr Frank Gaillard et al.
 is a type of Müllerian duct anomaly (class III)
where there is complete duplication of uterine horns
as well as duplication of the cervix, with no
communication between them.
 Epidemiology
 Didelphic uteri account for approximately ~8%
(range 5-11%) of Müllerian duct anomalies.
 Clinical presentation
 Many patients are asymptomatic although some
may occasionally experience dyspareunia as a result
of the vaginal septum.

Pathology
 It results from failed ductal fusion that occurs
between the 12th and 16th week of pregnancy and
is characterized by two symmetric, widely
divergent uterine horns and two cervices. The
uterine volume in each duplicated segment is
reduced. As with most uterine anatomical
anomalies, there is an increased incidence of
fertility issues, and Müllerian abnormalities in
general are over represented in infertile women.

 The chance of seeing a pregnancy to term is
significantly reduced, down to only 20%,
with a third of pregnancies ending in
abortion and over half in premature
deliveries. Only 40% of pregnancies
resulted in living children 2.
 Along with unicornuate uterus, uterus
didelphys has the greatest impact on
reproductive performance reference required.

 Associations
 renal agenesis
 vaginal septum which can include transverse
vaginal septum
– there is a vaginal septum in 75% of cases, and
obstruction to one horn is possible from occasional
transverse septae.
 Radiographic features
 Classically shows two widely spaced uterine corpora,
each with a single Fallopian tube. Separate divergent
uterine horns with large fundal cleft (as distinct from
a septate uterus)

 Hysterosalpingogram (HSG)
 Pelvic ultrasound
 Separate divergent uterine horns are
identified with a large fundal cleft.
Endometrial cavities are uniformly separate,
with no evidence of communication. Two
separate cervices need to be documented.
 MRI

2-dimensional image of a didelphic uterus with a
gestational sac (arrow) in the left horn.

Ultrasound image showing duplication of uterus corpus characterizes this
Mullerian Duct Anomaly. Didelphys uterus (Type III) or Complete
bicornuate (Type IVa) Ultrasound scan.

 Complications
 infertility
 unilateral hydrocolpos / haematocolpos (if a
vaginal septum is present)
 endometriosis

Bicornuate uterus
Dr Frank Gaillard et al.
 A bicornuate uterus is a type of uterine
duplication anomaly. It can be classified as
a class IV Mullerian duct anomaly.
 Epidemiology
 Overall, congenital uterine anomalies occur in
~1.5% of females (range 0.1-3%). Bicornuate
uteri are thought to represent ~25% (range 10-
39%) of Mullerian duct anomalies.

 Clinical presentation
 In most cases, a bicornuate uterus is
incidentally discovered when the pelvis is
imaged. The most common symptomatic
presentation is with early pregnancy loss
and cervical incompetence 6 .
 Pathology
 A bicornuate uterus results from an
abnormal development of the
paramesonephric ducts. There is a partial
failure of fusion of the ducts, resulting in a
uterus divided into two horns.

 Associations
 associated longitudinal vaginal septum may be
present in ~25% of cases
 as with other Mullerian duct anomalies,
abnormalities of the renal tract may also be
present
 Subtypes
 Bicornuate uterus is divided according to the
involvement of the cervical canal:
 bicornuate bicollis - two cervical canals; central
myometrium extends to external cervical os

 bicornuate unicollis - one cervical canal;
central myometrium extends to internal
cervical os

 General
 The preferred methods of imaging uterine
anomalies are ultrasound,
hysterosalpingogram or MRI. The external
uterine contour is concave or heart-shaped,
and the uterine horns are widely divergent.
The fundal cleft is typically more than 1cm
deep and the intercornual distance is
widened.

 The uterus is seen as comprising of caudally
fused symmetric uterine cavities with some
degree of communication between the two
cavities (usually at the uterine isthmus). Although
not a specific finding, the angle between the
horns of the bicornuate uterus is usually more
than 105°.
 Fluoroscopy - hysterosalpinogram (HSG)
 A divided uterus can be seen, but it is difficult to
differentiate between septate and bicornuate
anomalies since the uterine fundal contour is not
visible .
 MRI

 Treatment and prognosis
 Surgical intervention is usually not indicated in
absence of reproductive difficulties.
 In women with a history of recurrent pregnancy
loss and in whom no other infertility issues have
been identified a Strassman metroplasty can be
considered.
 In patients with cervical incompetence
placement of a cervical cerclage may increase
fetal survival rates 9. Indeed the association
between cervical incompetence and bicornuate
uterus is so high, that prophylactic cerclage may
be appropriate in some instances.

 Practical points
 Septate uterus increases the risk of early
pregnancy loss and hysteroscopic
intervention to resect the septum is
sometimes pursued. In this situation,
differentiation between a septate uterus or a
bicornuate uterus is critical. outcome.

Bicornuate uterus (with pregnancy in one horn)

Surface rendering of a bicornuate uterus. The serosal indentation
(arrow) is evident

 Bicornuate uterus with unilateral pregnancy

Septate uterus
Dr Matt A. Morgan and Dr Natalie Yang et al.
 A septate uterus is a common type of congenital
uterine anomaly, and it may lead to an increased rate
of pregnancy loss. The main imaging differential
diagnoses are arcuate uterus and bicornuate uterus.
 Epidemiology
 It is considered the commonest uterine anomaly
(accounts for ~55% of such anomalies). It is classified
as a class V Mullerian duct anomaly. Septate uterus
is the most common anomaly associated with
reproductive failure (67%).

 Pathology
 Septate uterus is considered a type of uterine
duplication anomaly. It results from partial or
complete failure of resorption of the uterovaginal
septum after fusion of the para-mesonephric
ducts. The septum is usually fibrous but can also
have varying muscular components.
 Subtypes
 a partial septum (sub-septate uterus) involves the
endometrial canal but not the cervix
 a septum is considered "complete" if it extends to
either the internal or external cervical os 10
 septum extends into the vagina: septate uterus and
vagina

 Associations
 As with other uterine anomalies, concurrent
renal anomalies may be associated
 General
 the external uterine fundal contour may be
convex, flat, or mildly (< 1 cm) concave
 acute angle <75 degrees between uterine
cavities
 endometrial canals are completely separated by
tissue iso-echoic to myometrium with
extension into endocervical canal

 Ultrasound
 The echogenic endometrial stripe is separated at the
fundus by the intermediate echogenicity septum.
The septum extends to the cervix in a complete
septate uterus. The external uterine contour must
demonstrate a convex, flat, or mildly concave
(ideally <1cm) configuration and may best be
appreciated on coronal images of the uterus.
 Colour Doppler
 May show vascularity in the septum in 70% of
cases; and if present may be associated with a higher
rate of obstetric complications

 Fluoroscopy - Hysterosalpingogram
 Accuracy of hysterosalpingogram alone is
only 55% for differentiation of septate uterus
from bicornuate uterus.
 Pelvic MRI
 MRI is considered the current imaging
modality of choice.

 Complications
 90% miscarriage rate
– a patient with a septate uterus does not
usually not have difficulty conceiving, but
a septate uterus is associated with the
highest rate of pregnancy loss of the
Müllerian duct anomalies

 Treatment and prognosis
 The distinction between septate uterus and bicornuate
uterus has important management implications. In
septate uterus, but not in bicornuate uterus, the
septum can be shaved off during hysteroscopy
(metroplasty) to form a single uterine cavity without
perforating the uterus 4.
 Reproductive outcome has been shown to improve
after resection of the septum, with reported decreases
in the spontaneous abortion rate from 88% to 5.9%
after hysteroscopic metroplasty

 Differential diagnosis
 Considerations a hysterosalpingogram include
 bicornuate uterus: the shape of the external
uterine contour is crucial to differentiate a
septate uterus from a bicornuate uterus, because
widely different clinical and interventional
approaches are assigned to each anomaly.
 On ultrasound or MRI images also consider
 arcuate uterus: small myometrial indentation in
the fundus with normal fundal contour.
 thick intra-uterine adhesion band

Septate Uterus
 Most COMMON anomaly 55%
 May be complete/ incomplete
•25 % early abortions
•5 - 7% late abortions & Premature labors

3-dimensional coronal view of a septated uterus (a) distance
between ostia (+. .+); (b) length of septum (x . . x).

(12 weeks) : Longitudinal section of the fetus showing its location belong the
uterine septum within the amniotic cavity that had spread to the both uterine
horns.

The fetal head situated in the right uterine horn

Transverse plane through the uterus. The uterine septum is hypoechoic and
hypovascular. At this level, the placenta (PL) begins to spread on the posterior
wall (retroverted uterus)

Donald School Journal of Ultrasound in Obstetrics and Gynecology, July-September
2011;5(3):243-256
 Figs A to C: Septate uterus seen on 3D VCI-C (A) septal vessels on 3D
power Doppler (B) and 3D SIS with coronal plane (C)

Arcuate uterus
Dr Yuranga Weerakkody and Dr Natalie Yang et al.
 An arcuate uterus is a mildly variant shape of
the uterus. It is technically one of the Müllerian
duct anomalies, but is often classified as a
normal variant. It is the uterine anomaly that is
least commonly associated with reproductive
failure. Arcuate uterus can be characterized with
ultrasound or MRI.

 Pathology
 An arcuate uterus is
characterized by a mild
indentation of the
endometrium at the
uterine fundus. It occurs
due to a failure of
complete resorption of
the uterovaginal septum.
It is the most common
Mullarian duct anomaly,
affecting 3.9% of the
general population .

 General features include:
 normal fundal contour
 no division of uterine horns
 smooth indentation of fundal endometrial
canal - the depth of indentation is usually
considered to be <1 cm
 increased transverse diameter of uterine
cavity

 Pelvic ultrasound
 A normal external uterine
contour is noted, with a
broad smooth indentation
on the fundal segment of
the endometrium. No
division of the uterine
horns.
 Hysterosalpingogram
 MRI

 Differential diagnosis
 septate uterus
– arcuate uterus and septate uterus exist on a
spectrum from least to most resorption of
the uterovaginal septum, respectively
 bicornuate uterus
– arcuate uterus can be distinguished from
a bicornuate uterus on the basis of its complete
fundal unification (i.e. the arcuate uterus has a
normal or slightly indented external fundal contour,
whereas the bicornuate has a more marked fundal
indentation, no more than 5 mm above the level of
the uterine horns)

Figs A to E: Arcuate uterus seen on 2D scan (A) SIS (B) 3D reconstructed
coronal plane during SIS (C) 3D VCI-C rendering (D) and 3D inversion mode
(E)
Donald School Journal of Ultrasound in Obstetrics and Gynecology, July-
September 2011;5(3):243-256

RKH Syndrome: Diagnosis
 Expected Menarche
 Difficult to differentiate from imperforate
hymen
 No uterus on exam, U/S, MRI,
Laparoscopy, IVP
 Confused with Androgen Resistance
Syndrome with shallow pouch and no
uterus.
 Determine karyotype.

Abnormalities of the
ovaries:
 1) agenesis or complete absence.
 2) Gonadal dysgenesis "streak
gonads" as in Turner syndrome.
 3) Failure of descent into the pelvis.
 4) Ovotestis “true hermaphrodite”
In which combined ovarian and
testicular tissues seen.

conclusion
All uterine anomalies negatively affect the live
birth rate and result in a higher frequency of
obstetrical complications. Depending upon
the type of muellerian anomaly, cervical
incompetence, spontaneous miscarriage,
preterm delivery, breech presentation,
abnormal fetal lie and intrauterine growth
restriction are all increased to a variable
degree 1,6,7 .

3DUS enables the measurement of the length
of a uterine septum & depth of fundal cleft.
In addition 3D ultrasound, in diagnosing
uterine congenital malformations, has been
found to be a reproducible method
(Salim 2003)

 While transvaginal sonography is an excellent
screening examination for uterine anomalies, it is
not as effective as 3D ultrasound in distinguishing
specific malformations. For example, Jurkovic et al
20 reported a 100% sensitivity and specificity for
the three-dimensional ultrasound detection of
uterine anomalies in contrast to 100% sensitivity
and 95% specificity for two-dimensional
ultrasound. However, the positive predictive value
of three-dimensional and two-dimensional
ultrasound for muellerian anomalies was 100% and
50%, respectively

 MRI has been the "gold standard" for categorizing
uterine anomalies because of its 98% - 100% accuracy .
While ultrasound will remain the primary modality
utilized to evaluate muellerian anomalies, MRI can
offer additional diagnostic information in patients with
equivocal ultrasound findings. As a result, laparoscopy
or open surgery are no longer required to make a
definitive diagnosis of a uterine anomaly .

At 6TH week gestation
Paramesonephric
or Mullerian Duct
develops lateral to
the Mesonephric
”wolffian “ Duct

The middle and caudal parts of
the Mullerian ducts undergoes
medial migration and fusion.
 The cranial 1/3 → tubes.
 The middle 1/3 → uterus and
cervix.
 Caudal 1/3 → upper 3/4 of
vagina.

Congenital abnormaleties of the uterus

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