Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5824_Библиотеки_им_академика_М_И_Перельмана.pdf
8 The Normal Uterus
99
14. Gonen Y, Casper RF, Jacobson W, Blankier J.
Endometrial thickness and growth during ovarian
stimulation: a possible predictor of implantation in in
vitro fertilization. Fertil Steril. 1989;52(3):446–50.
15. Lenz S, Lindenberg S. Ultrasonic evaluation of endometrial growth in women with normal cycles during
spontaneous and stimulated cycles. Hum Reprod.
1990;5(4):377–81.
16. Santolaya-Forgas J. Physiology of the menstrual cycle
by ultrasonography. J Ultrasound Med. 1992;11(4):
139–42.
17. Bakos O, Lundkvist O, Bergh T. Transvaginal sonographic evaluation of endometrial growth and texture
in spontaneous ovulatory cycles-a descriptive study.
Hum Reprod. 1993;8(6):799–806.
18. Barker MA, Boehnlein LM, Kovacs P, Lindheim SR.
Follicular and luteal phase endometrial thickness and
echogenic pattern and pregnancy outcome in oocyte
donation cycles. J Assist Reprod Genet. 2009;26:
243–9.
19. McWilliams GD, Frattarelli JL. Changes in measured
endometrial thickness predict in vitro fertilization
success. Fertil Steril. 2007;88:74–81.
20. Amir W, Micha B, Ariel H, Liat LG, Jehoshua D,
Adrian S. Predicting factors for endometrial thickness
during treatment with assisted reproductive technology. Fertil Steril. 2007;87:799–804.
21. Richter KS, Bugge KR, Bromer JG, Levy MJ.
Relationship between endometrial thickness and
embryo implantation, based on 1,294 cycles of in
vitro fertilization with transfer of two blastocyst-stage
embryos. Fertil Steril. 2007;87:53–9.
22. Kovacs P, Matyas S, Boda K, Kaali SG. The effect of
endometrial thickness on IVF/ICSI outcome. Hum
Reprod. 2003;18:2337–41.
23. Noyes N, Hampton BS, Berkeley A, Licciardi F, Grifo
J, Krey L. Factors useful in predicting the success of
oocyte donation: a 3-year retrospective analysis.
Fertil Steril. 2001;76:92–7.
24. Sundstrom P. Establishment of a successful pregnancy
following in-vitro fertilization with an endometrial
thickness of on more than 4 mm. Hum Reprod.
1998;13:1550–2.
25. Insler V, Melmed H, Eichenbrenner I, Serr DM,
Lunenfeld B. The cervical score, a simple semiquantitative method for monitoring of the menstrual cycle.
Int J Gynaecol Obstet. 1972;10:223–8.
26. Moghissi KS. Postcoital test: physiologic basis, technique, and interpretation. Fertil Steril. 1976;27:
117–29.
27. Duijkers IJ, Klipping C. Ultrasonographic assessment
of endocervix and cervical mucus in ovulatory menstrual cycles. Eur J Obstet Gynecol Reprod Biol.
2000;93(1):13–7.
Congenital Uterine Anomalies
Beth W. Rackow
9
Abbreviations
2DUS Two-dimensional ultrasonography
3DUS Three-dimensional ultrasonography
CT Computed tomography
DES Diethylstilbestrol
HSG Hysterosalpingography
MA Müllerian anomalies
MR Magnetic resonance
MRI Magnetic resonance imaging
MRKH Mayer-Rokitansky-Küster-Hauser
syndrome
RPL Recurrent pregnancy loss
SIS Saline-infusion sonography
Introduction
Congenital anomalies of the female reproductive
tract or Müllerian anomalies (MA) may involve
the uterus, cervix, fallopian tubes, or vagina. Of
the Müllerian anomalies, uterine anomalies are
the most common; prevalence rates range from 3
to 8 % of fertile and infertile women [
true incidence of uterine anomalies in the general
population, cited as 0.5 % [
because reproduction is not always affected;
thus, some individuals are asymptomatic and
B. W. Rackow , MD
Department of Obstetrics and Gynecology ,
Columbia University Medical Center,
Center for Women’s Reproductive Care ,
1790 Broadway , New York , NY 10019 , USA
e-mail: bwr2113@columbia.edu
6 ], is hard to determine
1 – 5 ]. The
unidentifi ed, and accurate assessment and
diagnosis has not always occurred [ 4 , 5 , 7 ]. The
etiology of MA is poorly understood; the majority
of MA are infrequent and sporadic, although some
familial clustering occurs, and MA are generally
attributed to polygenic and multifactorial causes
[ 8 , 9 ]. This chapter will review the embryologic
development of the female reproductive tract,
classifi cation of congenital uterine anomalies,
gynecologic and obstetric presentations of congenital uterine anomalies, imaging techniques,
and management options for uterine anomalies.
Embryology of the Female Reproductive Tract
While genetic sex is determined at the time of
fertilization, male or female phenotype is not
defi ned until after the sixth week of development.
Early in embryologic development, both the
Wolffi an, (mesonephric) and Müllerian (paramesonephric) ducts are present. The paired Wolffi an
ducts connect the embryologic kidney (mesonephros) to the cloaca between 5 and 10 weeks of
gestation; development of the functional kidney
(metanephros) is stimulated by an outgrowth of
the Wolffi an duct, the ureteric bud. Müllerian
duct development occurs concomitant with the
development of the urinary tract, and kidney and
ureteral anomalies are associated with MA; renal
anomalies include agenesis, ectopic location, or
abnormal anatomy [
opment begins at the same time as Müllerian duct
10 ]. Although gonadal devel-
L.A. Stadtmauer, I. Tur-Kaspa (eds.), Ultrasound Imaging in Reproductive Medicine,
DOI 10.1007/978-1-4614-9182-8_9, © Springer Science+Business Media New York 2014
101
102
B.W. Rackow
development, at 6 weeks of gestation, the two
processes are separate and distinct; females with
MA usually have normal ovaries and steroid
hormone production.
Normal development of the female tract
involves a complex series of events, and failure of
any part of this process can result in a Müllerian
anomaly. Paired Müllerian ducts arise from coelomic epithelium along the lateral walls of the
urogenital ridge, and these solid ducts are present
by week 6 of development. In the absence of
Müllerian-inhibiting substance released from the
male gonad, the Müllerian ducts proliferate while
the Wolffi an ducts regress. The Müllerian ducts
elongate caudally and cross the Wolffi an ducts
medially, and midline fusion of the ducts forms
the primitive uterovaginal structure. By week 10
of development, fusion occurs between the
caudal end of the joined Müllerian ducts and the
urogenital sinus. Subsequently, the unifi ed
Müllerian ducts undergo internal canalization
which results in two lumens divided by a midline
septum. Resorption of the septum commonly
occurs in a caudal to cranial direction. The fused
caudal portion of the Müllerian ducts becomes
the uterus, cervix, and upper vagina, and the
unfused cranial portion becomes the fallopian
tubes. Uterine development is completed by
week 20 of development.
The lower vagina has a separate embryologic
origin. At week 10, when the fused Müllerian
ducts connect with the urogenital sinus, the sinovaginal bulbs develop and proliferate toward the
caudal end of the uterovaginal canal, forming a
solid vaginal plate that elongates with time. The
central cells of the vaginal plate degenerate in a
caudal to cranial direction, forming a hollow
structure. Vaginal development is also complete
by week 20 of development. The hymenal membrane originates from the sinus tubercle and separates the vaginal lumen from the urogenital
sinus. The central epithelial cells usually degenerate prior to birth, achieving a patent structure
with a thin fold of mucus membrane at the
introitus.
Although the caudal-to-cranial direction
of Müllerian duct fusion and septal resorption
is the traditional theory of female reproductive
tract development, unusual MA have been
documented that are exceptions to this order of
progression. Examples include a complete septate
uterus with a double cervix and vaginal septum,
and a normal uterus and cervix with an isolated
longitudinal vaginal septum [ 11 – 14 ]. Hence,
medial fusion of the Müllerian ducts can proceed
in a caudal or cranial direction or both [ 15 ].
Classifi cation of Müllerian
Anomalies
Müllerian anomalies are commonly classifi ed
into three categories: agenesis and hypoplasia,
lateral fusion defects, and vertical fusion defects.
Reproductive tract abnormalities due to in utero
exposure to diethylstilbestrol (DES) comprise a
fourth group of anomalies. Agenesis and hypo-
plasia can occur for a portion of or an entire
Müllerian duct, or for both ducts, affecting one or
multiple Müllerian structures. Lateral fusion
defects are the most common category of
Müllerian defects and originate due to failure of
migration of one or both ducts, midline fusion of
the ducts, or absorption of the midline septum
between the ducts. A range of anomalies can
occur including symmetric or asymmetric and
nonobstructed or obstructed Müllerian structures.
Vertical fusion defects occur due to disordered
fusion of the Müllerian ducts with the urogenital
sinus or abnormal vaginal canalization and may
present with menstrual fl ow obstruction.
Although there is no universally accepted
standard classifi cation for Müllerian anomalies,
the American Fertility Society classifi cation system from 1988 is commonly utilized and provides a standardized nomenclature to describe
anomalies (Fig. 9.1 ) [ 4 , 16 ]. This classifi cation
system focuses on the major categories of uterine
anomalies and describes them based on their
embryologic etiology. Hypoplasia/agenesis (category I) and unicornuate (category II) denote
anomalies with developmental failure of one or
both Müllerian ducts; didelphys (category III)
and bicornuate (category IV) describe anomalies
involving a varying degree of failure of midline
fusion; septate (category V) and arcuate (category
9 Congenital Uterine Anomalies
103
Fig. 9.1 Classifi cation of uterine anomalies according to the American Fertility Society [ 16 ]. DES, diethylstilbestrol
(Reprinted Bermejo et al. [
VI) identify anomalies with some degree of
failure of resorption of the midline septum. DES
drug-related anomalies (category VII) are a
separate category of anomalies and will not be
discussed in this chapter. With this classifi cation
system, associated anomalies of the vagina,
cervix, fallopian tubes, and urinary system must
be documented separately. Two additional issues
with this classifi cation system are the inability to
fully describe a uterine anomaly when multiple
abnormalities are present (i.e., septate uterus with
duplicated cervix) and the lack of specifi c diagnostic criteria to enable differentiation between
bicornuate, septate, and arcuate uteri [ 4 , 11 , 18 ,
I Hypoplasia/agenesis II Unicornuate III Didelphus
(b) Non-
Communicating
(a) Complete
VII DES drug relatedVI ArcuateV Septate
(c) Fundal (d) Tubal (e) Combined
(a) Complete
(b) Partial
17 ]. With permission from John Wiley & Sons, Inc.)
(a) Communicating(a) Vaginal (b) Cervical
(c) No Cavity (d) No horn
fallopian tube, and the contralateral side may
have a variety of confi gurations: agenesis or a
rudimentary horn in 74 % [ 6 ]. The rudimentary
horn can be noncommunicating (70 to 90 %) or
communicating with the unicornuate uterus and
may contain functional endometrium [ 20 ].
Women with a rudimentary uterine horn containing functional endometrium may present with
cyclic or chronic pain, endometriosis, or a horn
gestation [ 20 ]. Nonfunctional rudimentary horns
are usually asymptomatic. Lastly, the unicornuate uterus is associated with a 40 % incidence of
renal anomalies, usually ipsilateral to the anomalous side [ 20 – 22 ].
IV Bicornuate
(b) Partial
19 ]. Hence, complex anomalies need to be
described according to the component parts.
Uterus Didelphys
Overview of the Uterine Anomalies
Unicornuate Uterus
The unicornuate uterus arises due to agenesis or
hypoplasia of one of the two Müllerian ducts.
The unicornuate uterus is a functional uterus
with a normal-appearing cervix and a single
The uterus didelphys results from complete
failure of lateral fusion of the two Müllerian
ducts; duplication of the Müllerian structures is
the result. Anatomically, these women have two
unicornuate uteri, two separate endometrial cavities, and two cervices. In the majority of women
with a uterus didelphys, vaginal duplication
also occurs and a longitudinal vaginal septum is
104
B.W. Rackow
present. Additionally, this anomaly can present
with an obstructed hemivagina and associated
ipsilateral renal anomaly [ 23 , 24 ].
Bicornuate Uterus
Incomplete lateral fusion of the Müllerian ducts
at the fundus results in a bicornuate uterus.
Commonly, a single cervix and two endometrial
cavities are present. Variability exists in the extent
of separation between the two cavities, with maximal separation extending down to the internal
cervical os (complete bicornuate). A fundal indentation of at least 1 cm has been found to be reliable for differentiating a bicornuate from a septate
uterus [ 11 , 25 – 28 ]. Although a normal vagina is
commonly present, a longitudinal vaginal septum
can occur with the bicornuate uterus [ 14 ].
Septate Uterus
The septate uterus occurs due to a defect in
resorption of the midline division between the
two fused Müllerian ducts, and a fi bromuscular
septum remains. The degree of septation can vary
from complete, extending from the uterine fundus
through the cervix, to partial, in which a portion
of the caudal aspect of the septum is resorbed.
Since the Müllerian ducts are completely fused,
a normal external fundal contour is present despite
a complete or partial division of the endometrial
cavity. A longitudinal vaginal septum is a common fi nding with a complete septate uterus and
can also occur with a partial septate uterus [ 14 ].
Endometriosis is also associated with septate uteri
and has been documented in 30 % of fertile and
infertile women with septate uteri [ 29 , 30 ].
Arcuate Uterus
The arcuate uterus demonstrates a slight, rounded
midline septum with a broad fundus and sometimes has a small indentation at the fundus. It
has been characterized as a variant of normal
uterine anatomy or a uterus with a small partial
septum [ 16 ], or a bicornuate uterus. Appropriate
imaging to defi ne uterine anatomy is essential so
as not to misclassify a uterus as arcuate instead of
partial septate or bicornuate, which have different
reproductive implications.
Müllerian Agenesis
The most extreme of the Müllerian anomalies is
Müllerian agenesis, otherwise known as MayerRokitansky- Küster-Hauser (MRKH) syndrome,
which occurs due to agenesis or hypoplasia of the
Müllerian ducts and affects approximately 1 in
5,000 females [ 31 ]. Müllerian agenesis involves
congenital absence of the vagina and variable
uterine development that ranges from agenesis to
hypoplastic and rudimentary structures. One
study demonstrated that in females with MRKH,
87 % had Müllerian remnants, 26 % of the remnants were cavitated and contained endometrial
mucosa, 7 % had a Müllerian remnant measuring
>4 cm, and 30 % had anomalies of the urinary
tract [ 32 ]. Along with urologic anomalies,
Müllerian agenesis is associated with other extragenital anomalies involving skeletal, cardiac, and
auditory systems and digits and palate [ 33 , 34 ].
Clinical Presentation of Congenital Uterine Anomalies
Although many females with congenital uterine
anomalies are asymptomatic and a late diagnosis may occur during evaluation of infertility [ 35 , 36 ], it is important to recognize several
gynecologic and obstetric signs and symptoms
that may indicate a uterine disorder (Table 9.1 ).
Müllerian agenesis presents with primary amenorrhea. Women with an obstructive anomaly
may report cyclic or noncyclic pelvic pain and
dysmenorrhea, and these symptoms can begin
several months after menarche or into adulthood. Obstructive uterine anomalies are associated with hematometra, retrograde menstruation,
and endometriosis [ 21 , 37 ]. Endometriosis is
a common fi nding in women with obstructive and nonobstructive Müllerian anomalies
9 Congenital Uterine Anomalies
105
Table 9.1 Clinical presentation of uterine anomalies
Gynecology Obstetrics
Pelvic pain, cyclic or
noncyclic
Dysmenorrhea Cervical incompetence
Primary amenorrhea with
pain
Primary amenorrhea
without pain
Hematometra Placental abruption
Abnormal uterine bleeding Intrauterine fetal demise
Dyspareunia Malpresentation
Pregnancy loss: fi rst and
second trimester
Preterm labor and
delivery
Intrauterine growth
restriction
Cesarean delivery
Pregnancy-induced
hypertension (related to
renal abnormalities)
Pregnancy in rudimentary
uterine horn
and is a known etiology of infertility [ 29 , 37 ].
Abnormal bleeding can also occur with uterine
anomalies and has been associated with septate
uteri [ 29 ]. Furthermore, vaginal anomalies may
occur in conjunction with uterine anomalies, and
abnormal bleeding may be due to a partial or
microperforate vaginal obstruction or a longitudinal vaginal septum. A nonobstructive vaginal
anomaly such as a longitudinal vaginal septum,
which is associated with septate and didelphys
uteri, may be a woman’s fi rst presentation with
a uterine anomaly; associated symptoms include
diffi culty with tampon insertion, bleeding around
one tampon (two are required), and dyspareunia.
Hence, if a vaginal anomaly is identifi ed, then
uterine imaging is warranted [ 14 ].
In obstetrics, congenital uterine anomalies are associated with a higher rate of poor
obstetric outcomes: recurrent pregnancy loss
(RPL), fi rst and second trimester pregnancy
loss, intrauterine growth restriction, preterm
labor and delivery, placental abruption, malpresentation, and intrauterine fetal demise [ 1 ,
7 , 21 , 38 , 39 ]. Among women with RPL, the
incidence of uterine anomalies is highly variable and ranges from 6 to 38 %, but based on
meta-analyses is likely closer to 12 to 16 % and
is as high as 25 % in women with second trimester pregnancy loss [ 3 – 5 , 40 ]. Uterine dys-
function may occur due to diminished cavity
size, insuffi cient musculature, impaired ability
to distend, abnormal myometrial and cervical
function, inadequate vascularity, or abnormal
endometrial development [ 1 , 3 , 8 , 22 , 41 – 46 ].
Due to higher rates of malpresentation, an
increased rate of cesarean delivery can be seen
with uterine anomalies. Additional obstetric
complications such as cervical incompetence
[ 47 ], pregnancy-induced hypertension (due to
renal anomalies), and antepartum and postpartum bleeding are also associated with congenital uterine anomalies. Lastly, pregnancy may
occur in an obstructed or rudimentary uterine
horn. These pregnancies are surgical emergencies due to an 89 % rate of rupture and the
related morbidity and mortality [ 20 ].
Imaging of Congenital Uterine Anomalies
Initial testing to evaluate pelvic anatomy,
especially in infertile women, may include
hysterosalpingography (HSG) and two-dimensional ultrasonography (2DUS). While these
modalities are useful for the initial assessment
of uterine anomalies, additional testing may
be warranted such as saline-infusion ultrasonography (SIS), magnetic resonance imaging
(MRI), and the increasingly common technique of three- dimensional ultrasonography
(3DUS). The benefi t of 3DUS and MRI is the
ability to simultaneously assess the uterine fundus and cavity [ 17 ]. However, there are inher-
ent strengths and limitations to each imaging
technique; thus, a combination of several techniques may be necessary to evaluate a uterine
anomaly. Although surgical evaluation (i.e.,
laparoscopy, hysteroscopy, laparotomy) has
been considered the gold standard for evaluation of complex Müllerian anomalies [ 18 , 41 ],
with readily available diagnostic imaging, surgery is infrequently necessary for evaluation
and diagnosis of anomalies. Surgical intervention with hysteroscopy and/or laparoscopy may
only be necessary when the uterine anomaly
is amenable to surgery and the intervention is
clinically necessary [ 4 , 48 , 49 ]. This discussion
106
B.W. Rackow
will review all available imaging techniques
and will focus on the evolving technique of 3-D
ultrasonography.
Hysterosalpingography
A common procedure for evaluation of tubal
patency in women with infertility, HSG can also
provide information about the contour of the uterine cavity. In a woman with a uterine anomaly, the
HSG may identify patent canals and any complex
communications, but is unable to adequately evaluate the external uterine contour and, hence, cannot reliably differentiate between uterine anomalies
[ 4 , 11 , 35 ]. When a uterine anomaly is identifi ed,
assessment of the external uterine contour can be
achieved with 2DUS, 3DUS, and/or SIS. In one
study, HSG correctly diagnosed 55 % of septate
and bicornuate uteri, and the addition of ultrasonography improved this result to 90 % [ 50 ]. Since
the HSG involves exposure to ionizing radiation,
in young women with desired fertility, this test
should only be ordered when clinically indicated.
Two-Dimensional Ultrasonography
Two-dimensional transabdominal or transvaginal
ultrasonography is a common initial technique
for assessing pelvic structures. It effectively visualizes the uterine structure and endometrial contour, can detect a pelvic mass or hematometra,
confi rms the presence of ovaries, and can be used
to evaluate the kidneys. When 2DUS is performed in the secretory phase of the menstrual
cycle, better visualization of the endometrium
and internal uterine contour can be achieved [ 51 ,
52 ]. A compilation of 2DUS studies for uterine
anomalies noted a pattern of low sensitivity and
high specifi city; although 2DUS can only identify about half of the uterine anomalies present,
the diagnosis of an anomaly is highly likely to be
correct [ 4 ]. When indicated, saline infusion
sonography can be employed to further assess the
internal and external uterine contours and can
accurately diagnose uterine anomalies as well as
identify other intracavitary abnormalities such as
polyps, myomas, or adhesions [ 4 , 49 , 53 ].
Pelvic Magnetic Resonance Imaging
Pelvic MRI is a sensitive and specifi c imaging
modality for evaluating Müllerian anomalies [ 11 ,
54 ]. MRI provides detailed delineation of inter-
nal and external uterine contours, can differentiate between a myometrial and fi brous uterine
division, can differentiate between a septate cervix and duplicated cervix, can diagnose vaginal
anomalies, and can identify if a rudimentary uterine horn contains functional endometrium [ 11 ,
17 ]. Furthermore, MRI can also assess renal mor-
phology and location. Although costly, this noninvasive imaging modality is less expensive than
surgery [ 18 ]. Pelvic MRI may not be necessary
for every patient with a uterine anomaly and may
be best utilized for the evaluation of complex
Müllerian anomalies [ 17 , 36 ].
A number of studies have evaluated the effi cacy of MRI to assess surgically confi rmed uterine anomalies [ 18 , 55 – 58 ]. A range of sensitivity
(29 to 100 %) and specifi city (33 to 100 %) and
positive predictive value (83 to 100 %) and negative predictive value (25 to 100 %) was identifi ed.
The ability of MRI to detect and correctly diagnose a uterine anomaly can be limited by the
availability of technically adequate images which
may be infl uenced by the MRI machine and software utilized and requires image interpretation
by a practitioner with experience in the diagnosis
of uterine anomalies [ 18 , 49 ].
Three-Dimensional Ultrasonography
Three-dimensional ultrasonography (3DUS) is a
relatively new imaging technique that provides
detailed and highly accurate views of pelvic anatomy; it constructs three-dimensional volumes
from a series of two-dimensional images [ 18 ,
27 ]. After the volume is created, it can be stored
and any section of a structure can be examined.
With uterine anomalies, the ability to visualize
the coronal section of the uterus is invaluable
for assessing the architecture of the endometrial
cavity and the uterine fundus (Fig. 9.2 ) [ 17 , 27 ,
48 , 59 , 60 ]. Therefore, by evaluating the inter-
nal and external uterine contours, 3DUS is able
to reliably differentiate between various uterine
9 Congenital Uterine Anomalies
abcd e
107
fgh
Fig. 9.2 Three-dimensional rendered coronal ultrasound
images demonstrating different uterine anomalies using
the American Fertility Society classifi cation [
mal uterus; ( b ) unicornuate uterus; ( c ) didelphic uterus;
( d ) complete bicornuate uterus; ( e ) partial bicornuate
Table 9.2 Three-dimensional ultrasound criteria for classifi cation of congenital uterine anomalies
Uterine morphology
Normal Straight or convex Uniformly convex or with indentation
Arcuate Concave fundal indentation with central point
Partial septate Presence of septum (does not extend to
Complete septate Presence of septum that completely divides
Bicornuate Two well-formed uterine cornua Fundal indentation >10 mm dividing the two
Unicornuate uterus Single well- formed uterine cavity with a
Adapted from Refs. [
Fundal contour
of indentation at obtuse angle (>90°)
cervix) with central point of septum at an
acute angle (<90 %)
cavity from fundus to cervix
single interstitial portion of fallopian tube and
concave fundal contour
19 , 61 ]
16 ]: ( a ) nor-
uterus; ( f ) complete septate uterus; ( g ) partial septate
uterus; ( h ) arcuate uterus; ( i ) uterus with diethylstilbestrol
(DES) drug-related malformations (Reprinted Bermejo
17 ]. With permission from John Wiley & Sons, Inc.)
et al. [
External contour
<10 mm
Uniformly convex or with indentation
<10 mm
Uniformly convex or with indentation
<10 mm
Uniformly convex or with indentation
<10 mm
cornua
Fundal indentation >10 mm dividing the two
cornua if a rudimentary horn is present
i
anomalies and can assess the often subtle differences between septate and bicornuate uteri
[ 17 , 18 , 27 , 28 , 59 , 61 ]. However, distortion by
leiomyomas may make uterine assessment more
challenging [ 7 , 18 , 59 ]. This modality is less
expensive and less time consuming than surgery
or pelvic MRI, is less invasive than surgery, and
may be better tolerated [ 17 , 18 , 52 ]. Although
the American Fertility Society classifi cation for
uterine anomalies (Fig. 9.1 ) does not provide
dimensions or measurements to enable differentiation of uterine anomalies based on ultrasound
fi ndings, a modifi cation of the AFS criteria based
on 3DUS landmarks has been utilized to facilitate
the diagnosis of uterine anomalies (Table 9.2 ,
Fig. 9.3 ) [ 11 , 16 , 18 , 19 , 48 , 61 ].
When compared to HSG and 2DUS, 3DUS
demonstrates high sensitivity and specifi city
108
ab
B.W. Rackow
cd
Fig. 9.3 Three-dimensional rendered coronal ultrasound
images demonstrating ultrasound criteria for classifi cation of congenital uterine anomalies. ( a ) Bicornuate
uterus: two divergent cornua are noted, divided by a sagittal cleft >10 mm ( arrow ). ( b ) Complete septate uterus:
a normal external uterine contour is present, and a septum
divides the endometrial cavity and extends to the cervix.
for the identifi cation of a normal uterus (98 and
100 %), arcuate uterus (100 and 100 %), or major
uterine anomaly (100 and 100 %) [ 59 ]. In com-
parison, 2DUS has lower sensitivity and specifi city for the diagnosis of a normal uterus (88 and
94 %) or arcuate uterus (67 and 94 %), but is
similarly accurate with major uterine anomalies
( c ) Arcuate uterus: a normal external uterine contour is
identifi ed with a concave fundal indentation of the endometrial cavity at an obtuse angle. ( d ) Partial septate
uterus: a normal external uterine contour is present, the
septum does not extend to the cervix, and the central point
of the fundal indentation demonstrates an acute angle
(Reprinted Ghi et al. [
48 ]. With permission from Elsevier)
(100 and 95 %). Hence, 2DUS may be best
utilized as a screening test for uterine anomalies,
with 3DUS as the defi nitive diagnostic test [ 59 ].
Several studies investigated the accuracy of
3DUS for the evaluation and diagnosis of uterine
anomalies and confi rmed the radiologic fi ndings
at surgery (laparoscopy and/or hysteroscopy).
9 Congenital Uterine Anomalies
109
In one study, 3DUS assessment of the uterine
fundus correlated 91.6 % with laparoscopic fi ndings, and evaluation of the uterine cavity correlated 100 % with hysterosalpingography [ 62 ].
Wu et al. compared 3DUS with laparoscopy for
the detection of uterine anomalies, and 3DUS
demonstrated 100 % sensitivity and specifi city
and correctly diagnosed 92 % (11/12) of septate
uteri and 100 % (3/3) of bicornuate uteri [ 28 ].
A study of 3,850 infertile women who underwent
uterine evaluation with 3DUS and hysteroscopy
identifi ed 689 (17.9 %) with septate uteri, and
3DUS demonstrated 99.27 % sensitivity and
100 % specifi city for diagnosing a septate uterus
[ 7 ]. Another recent study investigated 254 nul-
liparous women with recurrent pregnancy loss,
and 3DUS fi ndings were confi rmed by offi ce
hysteroscopy (for normal uteri) or laparoscopy/
hysteroscopy if a uterine anomaly was identifi ed
[ 48 ]. Fifty-four subjects (19 %) were diagnosed
with a uterine anomaly, and 3DUS correctly
identifi ed 52 (92.3 %) of the anomalies; two partial septate uteri were misclassifi ed as bicornuate and arcuate. When 3DUS and 2DUS were
compared for the diagnosis of uterine anomalies
during different phases of the menstrual cycle,
both modalities had higher sensitivity and specifi city during the luteal phase, but 3DUS demonstrated greater sensitivity and specifi city in both
the follicular and luteal phases, and the diagnostic accuracy of 3DUS was comparable to HSG,
hysteroscopy, and laparoscopy [ 52 ]. Lastly, the
reproducibility of the interpretation of 3DUS
volumes to diagnose uterine anomalies has been
established [ 61 ].
Few studies have compared the diagnosis of
uterine anomalies by 3DUS versus pelvic MRI.
Bermejo et al. determined that in women with
uterine anomalies, 3DUS and MRI demonstrate a
high degree of concordance, with a kappa index
of 0.880 (95 % CI, 0.77 to 0.99) [ 17 ].
Discrepancies occurred in the diagnosis of 4 of
65 anomalies; 3DUS misclassifi ed one bicornuate uterus as uterus didelphys, and 3 septate uteri
as bicornuate uteri. In contrast, Faivre et al.
investigated women with suspected septate and
bicornuate uteri; all 31 uterine anomalies were
confi rmed by hysteroscopy and/or laparoscopy
[ 49 ]. 3DUS correctly identifi ed 31/31 uterine
anomalies, and pelvic MRI correctly identifi ed
24/31 uterine anomalies; fi ve septate uteri were
misclassifi ed as bicornuate uteri, and 2 partial
septate uteri as complete septate uteri. These discrepancies were attributed to the lack of a coronal
uterine image and lack of familiarity with the
evaluation of uterine anomalies.
3DUS has been demonstrated to be at least as
accurate as pelvic MRI for diagnosing uterine
anomalies. However, 3DUS is not a widely available imaging modality and requires a high level
of practitioner skill and experience to achieve
high diagnostic accuracy [ 17 , 18 , 57 ]. Although
these studies have promising results, it must be
emphasized that they were performed by practitioners with expertise in the performance and
interpretation of 3DUS and in the diagnosis of
uterine anomalies.
Urinary Tract Imaging
Lastly, since urinary tract anomalies are associated with Müllerian anomalies, imaging of the
urinary tract needs to be considered when a uterine anomaly is identifi ed. Upper urinary tract
anomalies include renal agenesis, horseshoe or
pelvic kidney, duplication of the collecting system, or an ectopic ureter [ 10 ]. Renal anomalies
most commonly occur with unicornuate and
didelphic uteri and with Müllerian agenesis and
are infrequently identifi ed with bicornuate, septate, and arcuate uteri [ 63 ]. If an obstructive
Müllerian anomaly is identifi ed such as a unicornuate uterus with a rudimentary uterine horn or
uterus didelphys with an obstructed hemivagina,
renal anomalies including renal agenesis are
commonly identifi ed ipsilateral to the obstruction. In more than 50 % of cases, renal agenesis is
predictive of an obstructive Müllerian anomaly
[ 20 ].
Options for urinary tract imaging include
renal ultrasound, intravenous pyelogram, computed tomography (CT) scan, or magnetic resonance (MR) urogram. In women diagnosed with
a Müllerian anomaly, the kidneys should be evaluated with ultrasonography, and further imaging