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1857

WOMEN’S IMAGING
Müllerian Duct Anomalies: Role in
Fertility and Pregnancy

Mark D. Sugi, MD
Rubal Penna, DO Müllerian duct anomalies (MDAs) have important implications
Priyanka Jha, MBBS for the reproductive health of female patients. In patients with both
Liina Pōder, MD infertility and recurrent pregnancy loss, the incidence of MDAs is
Spencer C. Behr, MD as high as 25%. Congenital uterine anomalies are often only part
Jesse Courtier, MD of a complex set of congenital anomalies involving the cervix, va-
Evelyn Mok-Lin, MD gina, and urinary tract. Multiple classification systems for MDAs
Joseph T. Rabban, MD, MPH exist, each with different criteria that vary most for the diagnosis
Hailey H. Choi, MD of septate uterus. Recognizing the features that guide clinical man-
agement is essential for interpretation. Identification of an MDA
Abbreviations: ASRM =American Society for Re-
productive Medicine, CUME = Congenital Uter-
should prompt evaluation for associated urinary tract anomalies.
ine Malformation by Experts, ESGE = European In patients with infertility who seek to use assisted reproductive
Society for Gynecologic Endoscopy, ESHRE = technologies such as intrauterine insemination, recognition of
European Society of Human Reproduction and
Embryology, HSG = hysterosalpingography, MDAs may have an affect on reproductive success, particularly in
MDA = müllerian duct anomalies, MRKHS = patients who have an incomplete and clinically occult septum that
Mayer-Rokitansky-Küster-Hauser syndrome,
OHVIRA = obstructed hemivagina and ipsilateral
divides the cervix. Two-dimensional US is the first-line modality for
renal anomaly, 3D = three-dimensional evaluating the uterus and adnexa. Three-dimensional (3D) US or
RadioGraphics 2021; 41:1857–1875 MRI may help to visualize the external uterine fundal contour and
https://doi.org/10.1148/rg.2021210022 internal indentation of the endometrial cavity, which are two mor-
phologic characteristics that are keys to the diagnosis of congenital
Content Codes:
uterine anomalies. Hysterosalpingo contrast-enhanced US may be
From the Department of Radiology and Bio-
medical Imaging, Division of Abdominal Im-
performed in conjunction with 3D US to evaluate uterine morpho-
aging and Ultrasound (M.D.S., R.P., P.J., L.P., logic characteristics, the endometrial cavity, and tubal patency in
S.C.B., H.H.C.), Department of Radiology and a single examination. MRI helps to characterize rudimentary uteri
Biomedical Imaging, Division of Pediatric Ra-
diology (J.C.), Department of Obstetrics, Gy- in patients with müllerian hypoplasia and allows assessment for
necology, and Reproductive Sciences (E.M.L.), ectopic ureters, abnormally positioned ovaries, or associated deep
and Department of Pathology (J.T.R.), Univer-
sity of California, San Francisco, 505 Parnassus
infiltrative endometriosis.
Ave, 3rd Floor, M391, Box 0628, San Francisco,
CA 94143. Presented as an education exhibit Online supplemental material is available for this article.
at the 2020 RSNA Annual Meeting. Received ©
February 4, 2021; revision requested March 1 RSNA, 2021 • radiographics.rsna.org
and received April 13; accepted April 20. For
this journal-based SA-CME activity, the authors
M.D.S., S.C.B., and J.T.R. have provided disclo-
sures (see end of article); all other authors, the
editor, and the reviewers have disclosed no rel- Introduction
evant relationships. Address correspondence
to M.D.S. (e-mail: mark.sugi@ucsf.edu).
Müllerian duct anomalies (MDAs) occur in up to 7% of the general
©
population and in almost one-third of women with a renal anomaly
RSNA, 2021
(1,2). The prevalence of MDAs varies substantially according to the
population under study, and was reported to be as high as 25% in
SA-CME LEARNING OBJECTIVES women with a history of both infertility and miscarriage (3). In pedi-
After completing this journal-based SA-CME atric and adolescent patients with symptoms of outflow obstruction at
activity, participants will be able to:
menarche that are related to anomalies of müllerian duct hypoplasia,
„ Describe MDAs and their implications
the radiologist should delineate the anatomy of the female genito-
for fertility and pregnancy.
urinary tract (including atypically positioned ovaries) and search for
Discuss the differentiation of a septate
„
uterus from a bicornuate uterus accord- associated vertebral anomalies. In women with infertility or recurrent
ing to the updated major classification miscarriage and MDAs, imaging can help to identify candidates for
systems. interventions to improve reproductive potential or delineate associated
Identify anomalies such as obstructed
„
hemivagina and ipsilateral renal anomaly
and müllerian duct aplasia–renal agen-
esis–cervicothoracic somite association.
See www.rsna.org/education/search/RG.
1858  October Special Issue 2021 radiographics.rsna.org
TEACHING POINTS
„ According to the ASRM 2016 guidelines, a normal or arcuate
uterus is defined according to the depth of internal indenta-
tion of less than 1 cm and an angle of internal indentation
of greater than 90°. A septate uterus is defined according to
internal indentation of greater than 1.5 cm and less than 90°
A bicornuate uterus is defined according to external indenta-
tion of the fundal contour of greater than 1 cm.
„ A septate uterus is the most common congenital uterine
anomaly that is associated with two cervical canals at imaging
because of its prevalence in approximately one-half of all pa-
tients with MDAs. In this anomaly, a single cervix is divided by
a septum (septate cervix); however, it may be indistinguish-
able from a double cervix (true duplication) at imaging alone.
„ Most patients (92%) with MRKHS have unilateral or bilateral
rudimentary uteri, with varying sizes and degrees of differ-
entiation into three layers (ie, myometrium, junctional zone,
and endometrium). Although these patients generally have
normal ovarian function and secondary sexual characteristics,
the ovaries are abnormally located in 40% of patients be-
cause of unsuccessful descent of the gonads from their posi-
tion near the kidneys to the true pelvis. When rudimentary
uteri are present, they maintain a caudal relationship to the
ovaries.
„ Müllerian hypoplasia in combination with renal agenesis and
cervicothoracic somite anomalies has been described and is
known as the müllerian duct aplasia–renal agenesis–cervico-
thoracic somite association, or type II MRKHS. Patients with
type II MRKHS often have two to four anomalous vertebrae
located between the C5 and T1 spinal levels, renal agenesis
or ectopy, short adult stature, and musculoskeletal abnormali-
ties.
„ Obstructed hemivagina and ipsilateral renal anomaly (ie,
Herlyn-Werner-Wunderlich syndrome) is an uncommon mül-
lerian duct anomaly that is associated with a uterine anomaly
and renal dysplasia or renal agenesis. Any congenital uterine
anomaly should prompt evaluation of the urinary tract to as-
sess for renal anomalies, and specifically in women with an
obstructed hemivagina and ipsilateral renal anomaly, for ecto-
pic ureteral insertion into the obstructed hemivagina.
complications that may require surgical correction.
In this review, we highlight the principal updates
of the major classification systems of MDAs,
relevant imaging findings for clinical and surgical
management, and associated anomalies that merit
attention when an MDA is identified.
Imaging Modalities
Two-dimensional transabdominal US and endo-
vaginal US are the first-line modalities for evalu-
ation of the female reproductive tract, including
that of MDAs that involve the uterus, cervix, and
vagina (4). Three-dimensional (3D) US can be
helpful for assessing the external uterine fun-
dal contour and the internal indentation of the
endometrial cavity, which are best seen at mid-
coronal plane imaging of the uterus (5). MRI is
indicated for evaluation of complex anomalies or
when there is anatomic distortion due to deep
pelvic endometriosis, prior surgery, or trauma.
T2-weighted fast spin-echo MRI sequences in
orthogonal planes to the uterus and cervix allow
precise classification of anomalies and provide
additional information about the adnexa and
ovaries. In addition, volumetric 3D T2-weighted
MR images can be obtained, allowing for curved
planar reformations. Targeted axial or coronal
T2-weighted MRI of the abdomen allows as-
sessment for associated urinary tract anomalies.
Fat-saturated T1-weighted sequences can allow
identification of blood products in women with
hematometrocolpos or glandular components of
deep infiltrative endometriosis (6,7).
Hysterosalpingography (HSG) remains the
standard modality for evaluation of fallopian
tube patency in patients with infertility and may
prompt further evaluation for a suspected MDA
(Fig 1) (8). In certain circumstances, the uterine
fundal contour can be assessed by removing the
catheter and having the patient roll onto her side
on the table, allowing spilled contrast material
in the peritoneum to outline the uterine fundal
contour. The presence of a longitudinal vaginal
septum or a double or septate cervix and the rela-
tionship of the uterine cavities to one another can
narrow the differential diagnosis of an MDA, but
diagnosis ultimately relies on 3D US or MRI. If
two distinct ectocervices are present, cannulation
of each cervix is required for assessment of tubal
patency. Wide angles of divergence of the uterine
cavities (>105°) are more commonly seen with
fusion anomalies such as a bicornuate uterus;
however, the accuracy of the angle is dependent
on the obliquity of the fluoroscopic image (Fig
1). In practice, differentiation of a bicornuate
uterus from a septate uterus can be challenging at
HSG, with diagnostic accuracy of only 55% (9).
Hysterosalpingo contrast-enhanced (HyCoSy)
US combined with 3D US allows complete ana-
tomic and morphologic evaluation of the uterus
and patency of the fallopian tubes in a patient
with infertility or recurrent loss of pregnancy
(5). Similar to saline-infused sonohysterography,
this examination is best performed during the
proliferative phase, at days 5–10 of the menstrual
cycle, when the endometrium is trilaminar in
appearance and detection of endometrial abnor-
malities is optimal. First, a combination of gray-
scale and 3D US is performed for evaluation of
morphologic anomalies of the uterus. Next, saline
solution is infused to assess the uterine cavity for
synechiae, submucosal or intracavitary myomas,
and endometrial polyps (5). The final step of the
hysterosalpingo contrast-enhanced US examina-
tion is the US analog to HSG, in which either
saline solution or a diluted microbubble contrast
agent is injected to assess for spillage into the
peritoneal cavity to prove tubal patency. Limita-
RG  •  Volume 41  Number 6 Sugi et al  1859

Figure 1.  MDAs at hysterosalpingography (HSG) in three women, all of whom became pregnant after
HSG. (A, B) HSG image (A) in a 38-year-old woman with multiple prior miscarriages shows two uterine
cavities (arrows in A), each with its own cervical canal. Axial oblique T2-weighted MR image (B) shows a
septate uterus with minimal (<1 cm) external indentation of the fundus and deep internal indentation by
a fibromuscular septum that extends to the cervix (arrow in B). (C) HSG image in a 34-year-old woman
shows deep internal indentation and divergence of the uterine cavities, with an indentation angle slightly
less than 90°. Diagnosis of either a bicornuate or partial septate uterus depends on the uterine fundal con-
tour. (D) HSG image in a 34-year old woman shows cannulation of two separate cervical canals (arrows)
with separate uterine cavities. The degree of uterine and cervical separation suggests a uterus didelphys
configuration (compare with the complete septum dividing the uterus and cervix in A).

tions include operator dependence, large body
habitus, and distortion by multiple fibroids.
Embryology of Female Reproductive
Tract
In a female fetus, the mesonephric ducts regress
in the absence of testosterone, and the parame-
sonephric ducts (müllerian ducts) continue to
develop because of both the absence of antimül-
lerian hormone and the stimulation of maternal
hormones and the placenta. The müllerian ducts
form the basis for the uterine body, fallopian
tubes, cervix, and at least the upper two-thirds
of the vagina. Fusion of the müllerian ducts also
creates the broad ligament (ie, the peritoneal fold
that attaches the lateral aspects of the uterus to
the pelvic side walls) and creates the vesicouter-
ine and rectouterine spaces.
The caudal fused portion of the müllerian
ducts forms the uterovaginal primordium, which
ultimately makes contact with the urogenital
sinus and promotes formation of the vagina. The
different embryologic origins of the vagina were
first described in 1933 (10), and the vagina was
divided into upper and lower portions, arising
from the müllerian ducts and urogenital sinus,
respectively. Since then, advancements in immu-
nohistochemistry and recognition of the longi-
tudinal effects of diethylstilbestrol have shown
further complexity, and there was debate over
whether the vagina was derived from both mülle-
rian and urogenital sinus elements or solely from
the urogenital sinus (11).
Müllerian anomalies are most commonly
categorized as developmental anomalies (mül-
lerian hypoplasia, unicornuate uterus), fusion
anomalies (didelphys and bicornuate uteri), or
resorption anomalies (septate uterus). Longitu-
dinal and transverse vaginal septa are thought
to be the result of lateral and vertical fusion de-
fects, respectively. There are many case reports
of müllerian anomalies without classification,
1860  October Special Issue 2021 radiographics.rsna.org
including uterus didelphys with a normal cervix
and a normal uterus with a septate cervix and
vagina, suggesting variability in the degree of
superior fusion and inferior resorption during
embryogenesis (12).
The ovaries develop from mesothelial, mesen-
chymal, and primordial germ cells and are identi-
fiable by approximately the 10th week of gesta-
tion. In patients with müllerian hypoplasia, the
function of the ovaries is usually normal, which
reflects their separate embryologic origins. The
ovaries may be located superior to their expected
location owing to failure of descent.
Classification of MDAs
Since an article on imaging of MDAs by Behr et al
(13) was published in this journal in 2012, updates
to the classification systems have been published,
particularly those that address the diagnosis of a
septate uterus (14–20) (Table 1). Müllerian duct
anomalies are generally categorized into develop-
mental, resorption, and fusion anomalies, but in
practice, a single anomaly may be classified differ-
ently depending on the criteria of the classification
system applied (21,22) (Table 2, Fig E1).
The earliest classification system was proposed
by Eduard Kaufmann, a German pathologist,
and published in the American literature by an
obstetrician named Julius Jarcho in 1946 (23).
The American Fertility Society published their
initial classification system, which included seven
classes of anomalies, in 1988, including an arcu-
ate uterus (24). In 2013, the European Society of
Human Reproduction and Embryology and the
European Society for Gynecologic Endoscopy
(ESHRE/ESGE) published a comprehensive clas-
sification system (14) for congenital anomalies of
the female genital tract, which encompassed not
only uterine anomalies but also clinically signifi-
cant cervical and vaginal anomalies (Fig E2).
This classification system divides uterine anoma-
lies into six categories on the basis of uterine
wall thickness, which can be further subclassified
according to the coexistence of anomalies of the
cervix and vagina (Table 3) (15).
In 2016, the American Society for Reproduc-
tive Medicine (ASRM, previously the American
Fertility Society) published an updated guideline
for diagnosis of the uterine septum (17). The
guideline included criteria for defining a normal
or arcuate uterus, septate uterus, and bicornu-
ate uterus (Fig 2, Table 1). An arcuate uterus,
which was considered the mildest developmental
anomaly, was combined with the normal uterus in
these guidelines (24). Multiple additional classifi-
cation systems have been proposed, including an
embryologic and clinical classification by Acien et
al (25); however, the ASRM and ESHRE/ESGE
systems are the most widely used according to the
literature.
Septate Uterus
A septate uterus is thought to occur from failure
of resorption of the uterine septum at approxi-
mately the 20th week of gestation. The composi-
tion of the uterine septum is variable, ranging
from a highly vascular muscular septum to a less
vascularized fibrous septum, with implications for
pregnancy (Fig 3). Specifically, the more vascu-
larized muscular septum may result in changes to
uterine motility and thus cause preterm delivery
or miscarriage, while the less vascularized fibrous
septum may interfere with implantation (26).
A septate uterus is the most common MDA,
accounting for 55% of cases, and is associated
with high rates of both first- and second-trimester
miscarriage, preterm delivery, and fetal malpre-
sentation (27–29). The literature does not support
a direct relationship between a septate uterus and
infertility; however, there is a lack of consensus
on the treatment of patients with infertility and a
septate uterus (28,30). Current evidence for ef-
ficacy of metroplasty for infertility is limited owing
to nonuniform definition, diagnosis, and surgical
approaches for a uterine septum (31).
There are key differences between the ASRM
and ESHRE/ESGE classification systems in the
definition of a septate uterus. In both classifica-
tion systems, a horizontal line of reference known
as the interstitial or interostial line is established
in the mid-coronal plane of the uterus, connect-
ing the uterine ostia of the fallopian tubes (16).
The external indentation is defined as the depth
from a line that connects the apex of the right
and left uterine horns with the lowest point of the
indentation of the uterine fundus in the midline,
whereas the internal indentation is defined as the
depth from the interstitial line to the lowest point
of the indentation of the uterine cavities. Accord-
ing to the ASRM 2016 guidelines, a normal or
arcuate uterus is defined according to the depth
of internal indentation of less than 1 cm and an
angle of internal indentation of greater than 90°.
A septate uterus is defined according to inter-
nal indentation of greater than 1.5 cm and less
than 90° (Fig 4). A bicornuate uterus is defined
according to external indentation of the fundal
contour of greater than 1 cm (17) (Fig 2).
In the ESHGRE/ESGE classification system,
external and internal indentation criteria are
defined relative to the uterine wall thickness on
a midcoronal plane image as the distance be-
tween the interostial line and the external uterine
contour (Fig 5). When a coronal plane image
is not available, the mean of the anterior and
posterior wall thickness on a mid sagittal plane
RG  •  Volume 41  Number 6 Sugi et al  1861

Table 1: Classification of MDAs according to ASRM and CUME Guidelines

Class, Diagnosis, and Feature Imaging Feature Description and Criteria (US and MRI)
Normal or arcuate ASRM makes no distinction between normal and arcuate
 Fundus Convex
  Internal indentation* Indentation depth <1 cm, angle of divergence >90°†
 Cervix Single
 Vagina Single
Resorption anomaly class V: septate
 Fundus Convex or flat; minimal external indentation <1 cm‡
  Internal indentation Muscular or fibromuscular septum
Complete division to the internal cervical os
Partial division above the internal cervical os
   According to ASRM classification >1.5 cm, <90°
   According to CUME classification ≥1.0 cm, <140°
 Cervix Single, septate, or double
 Vagina Single, longitudinal, or transverse septum
Fusion anomaly, classes III–IV
 Bicornuate
  Fundus External indentation >1 cm‡
  Internal indentation Muscular or fibromuscular septum, complete (division to the internal
cervical os) or partial
  Cervix Single (unicollis) or septate (bicollis), with septum of variable length
  Vagina Single, longitudinal, or transverse septum
 Didelphys Two separate uterine cavities with double cervix
  Uterine bodies May be separated or partially fused with deep external indentation
  Endometrium No communication between the endometrial cavities
  Cervix Double
  Vagina Longitudinal or oblique septum (eg, OHVIRA)
Developmental anomaly, classes I-II
 Unicornuate
  Uterine body Elongated (banana-shaped) and deviated to the right or left pelvis
Isolated (35%) or with rudimentary horn (noncavitary, 33%; cavitary
noncommunicating, 22%; cavitary communicating, 10%)
  Cervix Single
  Vagina Single
  Associations Renal agenesis on the same side as absent or rudimentary horn
  Hypoplasia or agenesis MRKHS or vaginal agenesis
  Uterine body Unilateral or bilateral rudimentary horns (92%), or absent horns
Rudimentary horns connected by low-signal-intensity fibrous bands
(48%)
  Endometrium Variable differentiation into three layers(myometrium, junctional zone,
and endometrium)
  Cervix Absent or replaced by triangular soft tissue
  Vagina Hypoplastic or absent, rectovaginal septum may be visible
  Ovaries Abnormal location (40%), cephalad relationship to rudimentary uteri
Sources.—References 14–20.
Note.—Percentages are of patients with the anomaly who have the feature. ASRM = American Society for Re-
productive Medicine Classification System, CUME = Congenital Uterine Malformation by Experts Classifica-
tion, MRKHS = Mayer-Rokitansky-Küster-Hauser Syndrome.
* Internal indentation is the depth from the interstitial line to the lowest point of the indentation of the uterine
cavities.
†
Angle of indentation or divergence is the angle formed by the apex of the endometrial cavity and the interstitial
line.
‡
External indentation is the depth from the apex of the uterine horns to the indentation of the uterine fundus
at the midline. Inclusion of uteri with minimal external fundal indentation as septate is controversial, and this
uterine shape has been described as a combined fusion and resorption anomaly.
1862  October Special Issue 2021 radiographics.rsna.org

Table 2. Algorithm for Evaluation of MDAs

Step Details Likely Anomaly

Step 1 Is the uterus of normal size and well developed?
  Yes. Proceed to Step 2. ...
  No. Evaluate for müllerian agenesis/hypoplasia or unicornuate ...
uterus:
   Absent or rudimentary uterus Müllerian agenesis/hypoplasia
    Assess for rudimentary uteri (present in 92% of patients); if
present, assess for cavitation.
    Evaluate for vaginal agenesis or hypoplasia
    Assess for abnormal position of the ovaries (present in 40% of
patients)
   Deviated and elongated (banana-shaped) Unicornuate uterus
    Search for rudimentary horn; if present, assess for cavitation
  and communication with the unicornuate uterus.
Step 2 Evaluate the uterine fundal contour:
External indentation ≤1 cm. Proceed to Step 3. ...
External indentation >1 cm. Proceed to Step 4. Bicornuate uterus or uterus
didelphys
Step 3 Evaluate the internal indentation and cervix:
Internal indentation <1.0 cm Normal or arcuate uterus
Internal indentation ≥1 cm to ≤1.5 cm “Gray zone”* or septate uterus†
Internal indentation >1.5 cm Septate uterus
Evaluate the cervix: single or septate. Proceed to Step 6.
Step 4 Do the endometrial cavities communicate and/or is there a single
cervix?
Yes. Proceed to Step 6. Bicornuate uterus

No. Proceed to Step 5. ...

Step 5 Is there a double cervix or complete septate cervix?
Yes. May be indistinguishable at imaging. Uterus didelphys ‡ or bicornuate
bicollis uterus§
No. Incomplete septum dividing the cervix. Proceed to Step 6. Bicornuate uterus
Step 6 Evaluate for associated vaginal septa and renal anomalies
Sources.—References 17,19, 21, and 22.
Note.—Diagnostic criteria adapted from the 2016 ASRM and CUME guidelines. Mean intercervical distance
is the distance between the inner margins of the cervical canals of a double or septate cervix measured in the
transverse plane.
*According to ASRM criteria.
†
According to CUME criteria.
‡
A mean intercervical distance of 12.1 mm is reported to be associated with a didelphys uterus.
§
A mean intercervical distance of 5.4 mm is reported to be associated with a bicornuate bicollis uterus.

image is used as an alternative (15). According
to ESHRE/ESGE criteria, a septate uterus is any
uterus with a “normal outline and an internal
indentation at the fundal midline exceeding 50%
of the uterine wall thickness” (14). A completely
septate uterus is characterized by a septum ex-
tending to the internal cervical os, and a partial
septate (ie, subseptate) uterus is characterized by
an incomplete septum that terminates above the
internal cervical os (Fig 4).
In practice, a number of uterine anomalies
have combined features. Specifically, a uterus
with an internal indentation of greater than 1.5
cm, which meets the criteria for a septate uterus
according to the ASRM, may have minimal
external indentation of the fundus of less than
or equal to 1 cm (Fig 6). This configuration has
been variably described as a combined resorp-
tion and fusion anomaly or “hybrid” septate
uterus (17,18). A similar entity with mixed
features is termed a bicorporeal septate uterus
(class U3c) in the EHSRE/ESGE classification
system. In both the ASRM and ESHRE/ESGE
classification systems, a septate uterus may be
associated with a septum extending through the
cervix and/or vagina.
RG  •  Volume 41  Number 6 Sugi et al  1863

Table 3: ESHRE/ESGE Classification of Female Genital Tract Anomalies

Type of Anomaly and Diagnosis US Criteria

Normal uterus (class U0)
 Fundus Normal outline or external indentation <50% of uterine wall thickness
  Internal indentation Straight or curved interostial line with internal indentation <50% of
myometrial thickness
 Cervix Normal
 Vagina Normal
Resorption anomaly: septate uterus
(class U2)
 Fundus Normal outline or external indentation <50% of uterine wall thickness
  Internal indentation Internal indentation >50% uterine wall thickness
 Partial Septum dividing the cavity above the internal cervical os
 Complete Septum extending to the internal cervical os
 Cervix Single or septate
 Vagina Single or septate (longitudinal nonobstructing, longitudinal obstructing,
or transverse)
Fusion anomaly: bicorporeal uterus External indentation >50% of uterine wall thickness
(class U3)*
  Partial bicorporeal Indentation dividing the body above the cervix
  Complete bicorporeal Indentation dividing the body to the cervix
  Bicorporeal septate uterus Both fusion and resorption defects with midline external indentation
>150% of uterine wall thickness
 Cervix Single, septate, double, or unilateral aplasia (eg, OHVIRA)
 Vagina Single or septate (longitudinal nonobstructing, longitudinal obstructing,
or transverse)
Developmental anomaly
  Dysmorphic uterus (class U1) Small uterus with normal outline but abnormal shape of uterine cavity,
excluding septa
  T-shaped Narrow uterine cavity, with thickened lateral walls and normal corpus-
to-cervix ratio
  Infantilis Narrow uterine cavity, with normal lateral wall thickness and abnormal
corpus-to-cervix ratio
  Other External indentation <50% of uterine wall thickness
  Hemiuterus (class U4) Unilateral formed uterus, subdivided by a functional rudimentary cavity
  Aplastic uterus (class U5) Absence of any fully developed uterine cavity
Rudimentary horns may manifest with or without a functional cavity
Unclassified (class U6) Ectopic müllerian tissue anomalies
Sources.—References 16 and 17.
Note.—ESHRE = European Society of Human Reproduction and Embryology, ESGE = European Society for
Gynecologic Endoscopy.
*Both bicornuate and didelphys uteri are categorized as bicorporeal.

An important change introduced in the
EHRE/ESGE classification system was the reclas-
sification of more than one-half of the women
who were classified as having an arcuate uterus
according to the 1988 American Fertility Soci-
ety criteria as having a partially septate uterus
(32). An expert panel has suggested that use
of the ESHRE/ESGE guidelines likely results
in overestimation of the prevalence of septate
uteri, while use of the ASRM guidelines likely
results in underestimation, leaving a substantial
portion of uteri in a “gray zone.” To rectify this,
the panel proposed a new set of classification
criteria known as the Congenital Uterine Mal-
formation by Experts (CUME) in 2017, which
recommended decreasing the threshold depth of
internal indentation to less than or equal to 10
mm for diagnosis of a septate uterus (19). These
seemingly minor changes to classification criteria
can have a substantial effect on public health.
Authors of one study (28) noted that patients
who received a diagnosis from the same group
of observers showed a prevalence of septate uteri
of 5% when the ASRM criteria were used, 12%
1864  October Special Issue 2021 radiographics.rsna.org

Figure 2.  MR images (with corresponding illustrations, top) show classification of normal or arcuate (A), septate (B), and bicornu-
ate (C) uteri on the basis of ASRM 2016 criteria, which apply for both complete septate and partial septate uteri. (A) Axial oblique
T2-weighted MR image of a normal or arcuate uterus in a 33-year-old woman shows a depth of internal indentation (solid line) of
less than 1 cm (double-headed arrow) and an angle of indentation of greater than 90°. (B) Axial T2-weighted MR image of a septate
uterus in a 31-year-old woman shows an internal indentation (solid line) of greater than 1.5 cm (double-headed arrow), an angle of
indentation of less than 90°, and a flat external uterine contour (dashed line). (C) Axial oblique T2-weighted MR image of a bicornu-
ate uterus in a 37-year-old transgender man shows external uterine fundal indentation (dashed line) of greater than 1 cm (dashed
double-headed arrow).

Figure 3.  Variable imaging appearance of the uterine septum. (A) Coronal T2-weighted MR image
in a 34-year-old woman with a complete septate uterus shows a flat external uterine fundal contour
with a deep muscular septum (arrowhead) and a fibromuscular septum extending to the level of the
cervix (arrow). (B) Coronal T2-weighted MR image in a 32-year-old woman with a partial septate
uterus and a singleton fetus at 28 weeks of gestation shows a fibrous septum extending partially into
the uterine cavity (arrow). The placenta is in the right lateral uterus (arrowhead).

when the CUME criteria were used, and 31%
when the ESHRE/ESGE criteria were used.
Bicornuate and Didelphys Uteri
Bicornuate and didelphic uteri are the result of
fusion anomalies. The ASRM defines a bicor-
nuate uterus as an external fundal indentation
of greater than 1 cm, without further specifica-
tion regarding the degree of myometrial fusion.
Uterus didelphys, in comparison, is defined as
two separate uterine bodies with duplication
of the cervix (Fig 7). In this category of fusion
RG  •  Volume 41  Number 6 Sugi et al  1865

Figure 4.  MR images (with corresponding illustrations, top) of MDAs related to failure of resorption (A, B) compared with a nor-
mal uterus (C). (A) Axial oblique T2-weighted MR image of a septate uterus in a 34-year-old woman shows a convex uterine fundal
contour (dashed line), internal indentation of greater than 1.5 cm (double-headed arrow) from the interostial line (solid line) to the
apex of the indentation of the endometrial cavity, and a fibrous septum extending to the level of the internal cervical os (arrow).
(B) Axial T2-weighted MR image of a partial septate uterus in a 36-year-old woman shows a convex uterine fundal contour (dashed
line) and internal indentation of greater than 1.5 cm (double-headed arrow). (C) Axial T2-weighted MR image of a normal or arcuate
uterus in a 35-year-old woman shows a convex uterine fundal contour (dashed line) without significant internal indentation (<1 cm).
Criteria and illustrations adapted from the ASRM 2016 guidelines (28).

Figure 5.  Midcoronal three-dimensional (3D) US image of a normal uterus in

a 25-year-old woman shows the measurements adopted by the ESHRE/ESGE
classification system. The uterine wall thickness (vertical dotted line 1) is the
distance between the interostial line (solid line spanning the uterine ostia of the
fallopian tubes) and uterine fundus (horizontal dotted line above the solid line).
Internal indentation (vertical dotted line 2) is the distance from the interostial
line to the apex of the endometrial indentation (dotted line below the solid
line). Note the centrally located intrauterine device (arrowhead).

both bicornuate and didelphys uteri, recognizing

that these entities fall within the range of fusion
anomalies. A bicorporeal uterus is defined accord-
ing to external indentation of the uterine fundal
contour of greater than 50% of the thickness of
the uterine wall, which may partially or com-
pletely divide the uterine corpus to the level of
the cervix.

anomalies, any degree of communication be- Double and Septate Cervices

tween the endometrial cavities with an external
fundal indentation of greater than 1 cm suggests
a bicornuate uterus, as does a single cervix (Table
1). The term bicollis has been used to describe
further a bicornuate uterus with a septum that
either partially or completely divides the cervix,
which is an anomaly that may be indistinguish-
able from uterus didelphys.
The ESHRE/ESGE classification system
employs the umbrella term bicorporeal to include
The nomenclature for cervical anomalies is in-
consistently defined in the literature. Two cervical
canals can result from two types of anomalies: a
double cervix, which we define as duplication of
a normal cervix, and a septate cervix, in which
a septum partially or completely divides a single
cervix into two cervical canals (Fig 8). ESHRE/
ESGE classification uses similar nomenclature,
defining subclasses of cervical anomalies as a
septate cervix or a double normal cervix, which is
1866  October Special Issue 2021 radiographics.rsna.org

Figure 6.  Septate uterus with minimal external indentation of the uterine fundal contour. This mül-
lerian duct anomaly has been described as a combined anomaly, with features of both fusion and
resorption anomalies. Axial T2-weighted MR images in a 40-year-old woman show slight external in-
dentation of the uterine fundal contour of less than 1 cm (dashed line in A) and internal indentation
of greater than 1.5 cm (double-headed arrow in A) by a fibromuscular septum extending to the level
of the cervix (arrow in B). The patient underwent hysteroscopic resection of the midline septum,
which was found at surgery to extend to the level of the internal cervical os.

Figure 7.  MR images (with corresponding illustrations, top) of MDAs of absent or incomplete fusion
according to the 2016 ASRM criteria. (A) Coronal T2-weighted MR image of a uterus didelphys in a
26-year-old woman shows two separate unicornuate uterine cavities (arrows) and a double cervix (ar-
rowheads). (B) Coronal T2-weighted MR image of a bicornuate uterus in a 31-year-old woman shows
external indentation of the uterine fundal contour that is slightly larger than 1 cm, with a septum
extending to the level of the internal cervical os (arrowhead).

the presence of two distinct externally rounded
cervices, which is found at physical examination
with a speculum (14). A common misconcep-
tion is that the presence of two cervical canals
implies a diagnosis of uterus didelphys, which, in
part, stems from diagnostic ambiguity in differ-
entiating a double cervix from a septate cervix
at imaging (Fig 8) (21). The mean intercervical
distance (ie, measured as the distance between
the inner margins of the cervical canals in the
transverse plane of a double or septate cervix)
has been shown to be more than twice as wide in
patients with uterus didelphys (12.1 mm) than
in either bicornuate or septate uteri (5.4 mm and
RG  •  Volume 41  Number 6 Sugi et al  1867

Figure 8.  Double cervix versus septate cervix that were associated with different congenital uterine anomalies in three patients.
(A) Axial T2-weighted MR image of a septate cervix in a 30-year-old woman shows a longitudinal septum dividing the cervix (arrow-
head). (B) Coronal T2-weighted MR image of a double cervix in a 29-year-old woman with uterus didelphys shows rounded cervical
canals (arrowheads). Note that a double cervix and a septate cervix may be indistinguishable at imaging. (C) Axial T2-weighted MR
image of plicae palmatae (endocervical folds) in a 31-year-old woman with a septate uterus shows normal longitudinal folds of the
cervix (arrows), which can mimic a cervical septum.

Figure 9.  Incomplete septum dividing a single cervix in a 37-year-old transgender man with a bicornuate uterus (U3b/C1 according
to ESHRE/ESGE classification). (A, B) Axial T2-weighted MR images show a rounded single cervix with a longitudinal septum (arrow
in A) that only partially divides the cervix, with a single external cervical os (arrowhead in B). (C) Coronal T2-weighted MR image
shows the incomplete septum (arrow) to better advantage. At physical examination with a speculum, the single external cervical os
did not indicate the presence of a septate cervix. Attempts at intrauterine insemination before referral were unsuccessful. Intrauterine
insemination was subsequently performed under real-time transabdominal US guidance and the patient became pregnant.

5.4 mm, respectively) (21). A septate uterus is the
most common congenital uterine anomaly that
is associated with two cervical canals at imaging
because of its prevalence in approximately one-
half of all patients with MDAs. In this anomaly,
a single cervix is divided by a septum (septate
cervix); however, it may be indistinguishable
from a double cervix (true duplication) at imag-
ing alone (21). In bicornuate and septate uteri,
the cervical septum may incompletely divide the
cervix, resulting in the appearance of a single ex-
ternal cervical os, despite division at the level of
the upper cervix (Fig 9). Septate uteri with true
cervical duplication have been reported, under-
scoring the complexity of uterine and cervical
anomalies (29,33).
T2-weighted fast spin-echo MRI perpendicular
to the long axis of the cervix is useful for char-
acterizing cervical morphologic characteristics.
In cross-section from inner to outer, the cervix
is characterized by a high-signal-intensity central
stripe (ie, mucus, cervical mucosa), a low-signal-
intensity inner zone of fibromuscular stroma con-
tiguous with the junctional zone of the uterus, and
an intermediate-intensity outer zone of fibromus-
cular stroma (34). Occasionally, low-signal-inten-
sity endocervical folds called plicae palmatae may
be seen in the midline of the cervix and should
not be confused with a cervical septum (Fig 8);
these folds can be described as longitudinal ridges,
with only partial attachment to the fibrous cervi-
cal stroma (35). Endovaginal US can also be used
in conjunction with injection of saline solution
into the vagina for evaluation of the ectocervix. At
HSG, the presence of two ectocervices usually im-
plies a uterine anomaly that requires separate can-
nulation of each cervical canal to assess for tubal
patency of the bilateral fallopian tubes (Fig 1).
Müllerian Hypoplasia and Vaginal
Anomalies
Müllerian agenesis, also known as Mayer-Rok-
itansky-Küster-Hauser syndrome (MRKHS),
refers to a group of female genital tract anomalies
1868  October Special Issue 2021 radiographics.rsna.org

Figure 10.  Developmental MDAs. (A) 3D US image (with corresponding illustration, top)
of a unicornuate uterus in a 32-year-old woman that was acquired during saline solution–
infused sonohysterography shows a single fluid-filled uterine cavity (arrow). (B) Axial T2-
weighted MR image (with corresponding illustration, top) in a 36-year-old woman shows
a unicornuate left uterus (solid arrow) and an atretic noncommunicating right uterine horn
(dashed arrow). Scrollable images from this case are available at https://bit.ly/unicornuate.

Figure 11.  Unilateral left renal agenesis with ipsilateral absent uterine horn in a 61-year-old woman with
a right unicornuate uterus. Coronal (A) and axial (B) T2-weighted MR images show a solitary right kidney
(arrowhead in A) and a right unicornuate uterus that deviated to the right hemipelvis (arrow in A and B).
An isolated unicornuate uterus is present in 35% of patients with this developmental anomaly, most of
whom have some form of a rudimentary horn.

caused by interrupted development of the mül-
lerian ducts, resulting in agenesis or hypoplasia
of the uterus and vagina. The term itself is a
misnomer, because most of these patients have
a rudimentary uterus and vaginal atresia; thus
hypoplasia is a more accurate description. The
condition affects approximately 1 in 4500 female
neonates, who typically present in adolescence
with primary amenorrhea (36).
Abnormal or failed development of one of the
paired müllerian ducts results in a unicornuate
uterus, which is associated with a rudimentary
uterine horn in almost two-thirds of cases. When
present, the rudimentary horn can be noncavi-
RG  •  Volume 41  Number 6 Sugi et al  1869
tary (33%), cavitary noncommunicating (22%),
or cavitary communicating (10%) (Fig 10) (37).
A unicornuate uterus is deviated to one side of
the pelvis, with an elongated shape that is often
described as similar to that of a banana (Fig 10).
It is associated with renal agenesis ipsilateral to
the side of the absent or rudimentary horn in ap-
proximately 40% of cases (Fig 11) (38).
Transvaginal US is the initial imaging modality
for patients with suspected müllerian hypoplasia.
When an anomaly is identified, MRI may allow
further characterization of rudimentary uteri and
evaluation for atypically positioned ovaries. Most
patients (92%) with MRKHS have unilateral or
Figure 12.  (A–C) MRKHS with
rudimentary uterine horns and
vaginal agenesis in an 18-year-old
woman. Axial T2-weighted MR im-
age (A) shows fat in the expected
location of the lower vagina (ar-
row). Coronal T2-weighted MR
image (B) shows bilateral rudi-
mentary uterine horns (arrows).
Axial T2-weighted MR image (C)
shows low-signal-intensity fibrous
bands (arrows), extending from
the rudimentary horns to the mid-
line, which is a common feature
in these patients. (D) Coronal T2-
weighted MR image in a 30-year-
old woman with MRKHS shows bi-
lateral pelvic kidneys (arrowheads)
and abnormal positioning of the
left ovary in the left paracolic gut-
ter (arrow). (E) Sagittal short-tau
inversion-recovery MR image of
the cervical spine shows a seg-
mentation anomaly and fusion in-
volving the C5–C7 vertebral bod-
ies (arrowheads) in a 48-year-old
woman with müllerian duct apla-
sia–renal agenesis–cervicothoracic
somite association.
bilateral rudimentary uteri, with varying sizes and
degrees of differentiation into three layers (ie,
myometrium, junctional zone, and endometrium).
Although these patients generally have normal
ovarian function and secondary sexual character-
istics, the ovaries are abnormally located in 40%
of patients because of unsuccessful descent of the
gonads from their position near the kidneys to the
true pelvis. When rudimentary uteri are present,
they maintain a caudal relationship to the ovaries
(36). At T2-weighted MRI, fibrous bands of low
signal intensity characteristically extend from the
rudimentary uteri in the lateral pelvis and con-
verge to an area of triangular soft tissue at the
midline, which can have variable signal intensity
at T2-weighted MRI but is usually isointense
in comparison with the uterine buds (Fig 12)
(39,40). Endometrial tissue can be present in the
cavitated rudimentary uteri and is more often seen
when the rudimentary uterus is unilateral (41).
Müllerian hypoplasia in combination with renal
agenesis and cervicothoracic somite anomalies has
been described (42) and is known as the mül-
lerian duct aplasia–renal agenesis–cervicothoracic
somite association, or type II MRKHS (Fig 12).
Patients with type II MRKHS often have two to
four anomalous vertebrae located between the C5
and T1 spinal levels, renal agenesis or ectopy, short
adult stature, and musculoskeletal abnormalities.
1870  October Special Issue 2021 radiographics.rsna.org

Figure 13.  Normal vaginal anatomy and congenital vaginal septa in four patients. (A, B) Axial T2-
weighted MR images in two 26-year-old women show the normal H-shaped (arrow in A) and W-shaped
(arrow in B) cross-sectional anatomy of the lower vagina. (C) Axial T2-weighted MR image in a 32-year-
old woman with a complete septate uterus shows two vaginal canals (arrows) that are separated by a
longitudinal septum. (D) Coronal oblique T2-weighted MR image shows hematometrocolpos with layer-
ing blood products of different ages (arrows) in the lower uterus and upper vagina of a 12-year-old girl
with a high transverse vaginal septum, which was confirmed and excised at surgery.

The degree of vaginal hypoplasia is variable
in patients with MRKHS. The vagina is col-
lapsed under normal physiologic conditions,
with greater rigidity in the lateral walls than in
the anterior and posterior walls, which leads to a
typical H or W cross-sectional appearance of the
lower vagina at CT and MRI (Fig 13) (43). In
many cases, the vagina (including the distal one-
third) may be completely absent at imaging, with
fat replacing the expected location of the vagina.
The rectovaginal septum, which appears as a hy-
pointense linear band that is located between the
bladder and the rectum at T2-weighted MRI, is
often seen in patients with MRKHS, even in the
absence of vaginal tissue (44).
In addition to vaginal agenesis, other con-
genital vaginal anomalies include an imperforate
hymen and vaginal septa. A congenital imperfo-
rate hymen is a condition in which the epithelial-
ized connective tissue of the hymen fails to open,
leading to fluid accumulation in the vagina and
uterus (ie, hydrometrocolpos), which can be de-
tected in the fetus or neonate at US or MRI as a
pear-shaped cystic structure, with layering debris
between the bladder and the rectum (45).
Vaginal septa may be longitudinal or trans-
verse, and obstructing or nonobstructing. The
longitudinal vaginal septum is the result of a lat-
eral fusion anomaly and has been further classi-
fied according to its extent (partial or complete),
its central or eccentric position in the vagina, and
its association with the cervix and vaginal open-
ing (normal, stenotic, or persistent hymen) (46).
At T2-weighted MRI, the longitudinal vaginal
septum appears as a thin hypointense band ex-
tending through the vagina, the length of which
is best appreciated with coronal or sagittal views.
Vaginal gel may be used for improved visualiza-
tion in selected patients. A longitudinal vaginal
septum is associated with most cases of uterus
didelphys (70%–100% in the literature); however,
a septate uterus is the most common uterine
anomaly that is associated with a longitudinal
vaginal septum because of its higher overall prev-
alence (21,47). Transverse vaginal septa are the
result of vertical fusion anomalies and are often
isolated, without associated uterine anomalies.
Patients with a transverse vaginal septum may
present with primary amenorrhea in adolescence
or with abdominal pain caused by obstruction
RG  •  Volume 41  Number 6 Sugi et al  1871
and hematometrocolpos (Fig 13). MRI allows
evaluation of the level and extent of the septum.
Neither type of vaginal septum is diagnostic for
a congenital uterine anomaly, including uterus
didelphys; however, the presence of a longitu-
dinal vaginal septum may suggest the presence
of a septate or didelphic uterus. The ESHRE/
ESGE classification system describes coexistent
vaginal anomalies as a subcategory, according to
the orientation of the septum and whether it is
obstructing (14). Indications for surgical inter-
vention include dyspareunia, labor dystocia, and
unexplained infertility, which could be related to
the limitations of sperm reaching only one-half of
the uterine cavity in patients with associated uter-
ine anomalies. However, the incidence of primary
infertility is generally not higher in women with a
longitudinal vaginal septum than in those without
this anomaly (48).
Obstructed Hemivagina and
Ipsilateral Renal Anomaly
Obstructed hemivagina and ipsilateral renal
anomaly (OHVIRA) (ie, Herlyn-Werner-Wun-
derlich syndrome) is an uncommon müllerian
duct anomaly that is associated with a uterine
anomaly and renal dysplasia or renal agenesis
(49–51). Any congenital uterine anomaly should
prompt evaluation of the urinary tract to assess
for renal anomalies, and specifically in women
with an OHVIRA, for ectopic ureteral insertion
into the obstructed hemivagina. Patients typi-
cally present after menarche with cyclical ab-
dominal pain or dysmenorrhea, because menses
causes recurrent bleeding into the obstructed
hemivagina.
Uncommonly, patients with this condition
present later in life because of the presence of a
small septal defect that allows incomplete ob-
struction of the hemivagina (Fig 14). Three dif-
ferent categories of this syndrome, with variable
clinical and imaging manifestations, have been
described. Type I is an obstructed hemivagina
without communication to the ipsilateral uterine
cavity. Type II is a partially obstructed hemiva-
gina with a small opening. Type III is a hemi-
vaginal septum with a cervical fistula (52,53).
Endometriosis occurs in approximately 17% of
these patients, possibly because of recurrent ret-
rograde menstrual flow (54). Treatment typically
involves vaginoscopic resection of the oblique
vaginal septum.
US is used for initial imaging evaluation, with
3D reconstruction of the uterus to assess for
morphologic abnormalities, most commonly, a
didelphic uterus. US findings include hematocol-
pos, pyocolpos, or hematometrocolpos, which is
asymmetric and occurs slightly more commonly
on the right side (54). MRI allows further charac-
terization of the course and integrity of the vagi-
nal septum that divides the obstructed from the
unobstructed hemivagina, and T2-weighted MRI
may show a linear hyperintense structure, which
represents an ectopic ureter, inserting into the
vagina in up to 40% of women with this anomaly
(55) (Fig E3).
Implications for Fertility and
Pregnancy
MDAs collectively are associated with higher rates
of preterm delivery (<37 weeks), preterm prema-
ture rupture of membranes, fetal malpresentation
at delivery, and perinatal mortality (56,57). The
incidence of preterm birth increases according to
the type of uterine anomaly, ranging from septate
(31%), bicornuate (39%), unicornuate (43%),
to didelphys (56%) anomalies (Fig E4) (58). In
comparison, the relative risks of first- and second-
trimester miscarriage are highest in patients with a
septate uterus (57). There is insufficient evidence
to suggest that MDAs are associated with infertil-
ity. The reported prevalence of MDAs in women
with infertility (and not miscarriage) is comparable
to that of the general population (17,59).
Limited studies have been performed to assess
the effect of MDAs on the success of assisted
reproductive technologies, including intrauterine
insemination and in-vitro fertilization. The imple-
mentation of intrauterine insemination is highly
variable across practices, with nonstandardized
techniques performed at each stage of the process
(60). US guidance is not routinely used because it
does not affect pregnancy rates. However, physical
examination alone may result in underdiagnosis of
anomalies such as incomplete cervical septa that
do not reach the external os (61). Further studies
are needed to assess the effects of these techniques
in patients with MDAs.
Clinical Management and
Reproductive Outcomes
Patients with müllerian hypoplasia usually pres-
ent with primary amenorrhea and have normal
ovarian function; thus in-vitro fertilization with
a gestational surrogate or uterine transplanta-
tion are potential options for reproduction. An
equally important aspect of the treatment of
MRKHS is vaginoplasty for restoration of sexual
function. Female adolescent patients with ob-
structive müllerian anomalies (most commonly,
OHVIRA) often present to clinical attention
because of pain at the onset of menarche. Vagi-
noscopic incision of the oblique vaginal septum
has been shown to be a successful surgical op-
tion for the treatment of OHVIRA (53). Failure
to recognize an ectopic ureter inserting into the
1872  October Special Issue 2021 radiographics.rsna.org

Figure 14.  OHVIRA, also known as Herlyn-

Werner-Wunderlich syndrome, in a 24-year-old
woman with right renal agenesis. (A, B) Coro-
nal T2-weighted MR images show two separate
uterine cavities (arrows in A), which is compat-
ible with uterus didelphys. An oblique longitudi-
nal vaginal septum (arrowheads in B) results in
obstruction of the right hemivagina (∗ in B). (C,
D) Axial T2-weighted MR images show the lon-
gitudinal vaginal septum (arrow in C) separating
the obstructed right hemivagina (∗ in C), which
contains a small defect inferiorly (arrowhead in D)
that allows for partial decompression. The patient
presented several years after menarche. (E) Illus-
tration shows an OHVIRA with a right dysplastic
kidney. (Illustration courtesy of Rubal Penna, DO.)

obstructed hemivagina may result in persistent

symptoms of abnormal vaginal discharge. Care-
ful evaluation with MRI and/or a technetium
99m 2,3-dimercaptosuccinic acid renal exami-
nation is recommended to search for an ectopic
dysplastic kidney. When symptoms persist and
the ectopic kidney is not located at imaging,
laparoscopy can be diagnostic and simultane-
ously therapeutic (51).
A meta-analysis (62) of retrospective studies
comparing pregnancy outcomes after hystero-
scopic metroplasty for a septate uterus overall
showed markedly better outcomes for women
with multiple recurrent miscarriages (≥3) who
underwent surgery; however, the use of hystero-
scopic metroplasty for infertility is controversial.
A randomized controlled trial (63) compar-
ing hysteroscopic metroplasty with expectant
management in women with septate uterus and
subfertility, miscarriage, or preterm birth showed
no improvements in reproductive outcomes with
surgery. The term metroplasty refers to reconstruc-
tion of the uterus. Surgery for the uterine septum
is usually performed with hysteroscopy by incis-
ing the septum from its apex in a cephalad direc-
tion, or from one cornua to the other, depending
on the width of the septum. Laparoscopy may
RG  •  Volume 41  Number 6 Sugi et al  1873
Figure 15.  Endometriosis and adenomyosis that are distorting a septate uterus in a 30-year-old woman. (A) Coronal T2-weighted
MR image shows a globular heterogeneous uterus with deep internal indentation of the endometrial cavity (arrowhead). (B) Sagit-
tal T2-weighted MR image shows distortion of the posterior uterus by adenomyosis and an endometriotic implant that is invading
the posterior myometrium (arrow), which is tethered to the adjacent ovary (arrowhead). (C) At hysteroscopy, a large midline
fibrous septum (arrowheads) was incised.
also be used in combination with hysteroscopy to
guide incision of the septum or when unification
of the uterine bodies is warranted in patients with
a bicornuate uterus, with the use of the modified
Strassman technique (64). A “second look” hys-
teroscopic examination may be indicated when
intrauterine synechiae (adhesions) are suspected
postoperatively. Saline solution–infused sono-
hysterography is also highly sensitive (90%) and
specific (95%) for the detection of intrauterine
synechiae in these patients and may be used to
assess the shape of the uterine cavity after surgery
(65). At saline solution–infused sonohysterogra-
phy, adhesions appear as linear echogenic bands
extending across the endometrial cavity.
Endometriosis
The higher incidence of endometriosis in women
with obstructive MDAs is well established. Rates
of endometriosis in patients with OHVIRA have
been reported to be up to 17% (54). In compari-
son, 11% of women in a general population cohort
received a diagnosis of endometriosis at MRI (66).
The relationship between nonobstructive MDAs
and endometriosis is less clear. Endometriosis
has been reported in a patient with MRKHS and
complete uterine agenesis, supporting the alter-
native hypothesis of coelomic metaplasia, which
describes transformation of the germinal epithe-
lium (which lines the peritoneum and surface of
the ovary) into endometrial cells (67).
MRI is the primary imaging modality for deep
infiltrative endometriosis and for evaluation of pa-
tients with complex cases in whom endometriotic
implants cause distortion of an existing congenital
uterine anomaly (Fig 15). Transvaginal US per-
formed by experienced operators allows detec-
tion of both deep retrocervical and rectosigmoid
endometriosis (68). A thorough US evaluation of
a patient with suspected MDA and endometrio-
sis should include 3D rendering of the uterus in
a coronal plane and examination for the "sliding
sign" to assess for deep infiltrative endometriosis
with rectal involvement, in which the posterior
uterus is tethered to the rectum (69).
Conclusion
MDAs are embryologic anomalies that may
be associated with congenital urinary tract or
cervicothoracic somite anomalies. The relation-
ship between MDAs and recurrent miscarriage
is well established; however, the association of
MDAs with infertility remains unclear. Systems
of classification of MDAs have been updated to
include more detailed categorization of cervical
and vaginal anomalies and have evolved in paral-
lel with advancing techniques for hysteroscopic
metroplasty and assisted reproductive technology.
The role of metroplasty remains controver-
sial. Imaging is critical in the workup of adoles-
cents with primary amenorrhea and adults with
infertility and/or recurrent miscarriage, because
recognition of these anomalies and their associa-
tions allows tailored management to optimize
reproductive health.
Disclosures of Conflicts of Interest.—M.D.S. Activities related
to the present article: disclosed no relevant relationships. Activi-
ties not related to the present article: consultancy for Nextrast,
lectures for the University of California at San Francisco, and
book royalties from Elsevier. Other activities: disclosed no rel-
evant relationships. S.C.B. Activities related to the present article:
disclosed no relevant relationships. Activities not related to the
present article: board membership for AAA and Novartis. Other
activities: disclosed no relevant relationships. J.T.R. Activities
related to the present article: disclosed no relevant relationships.
Activities not related to the present article: spouse receives salary
and stock options from Merck. Other activities: disclosed no
relevant relationships.
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