Uterine Anomalies - OB-GYN

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Uterine Anomalies - OB-GYN New York |
Gynecologists in New York
UTERINE ANOMALIES AND ASSOCIATED CAUSES,
Nathan Fox, MD (Digital)
By MFM on September 6, 2023
Congenital abnormalities of the uterus, or congenital müllerian anomalies, are

malformations of the uterus that develop during the embryonic stage of fetal growth.
Some examples of these anomalies include uterus didelphys, arcuate uterus,
unicornuate uterus, bicornuate uterus, and septate uterus. When an individual is in
their mother’s womb, the uterus begins to form in two separate halves. These two
tube-shaped structures, called the müllerian ducts, later fuse together to create one
uterus. For some, the connection of these two ducts is incomplete, or fuses in a way
that results in an abnormal uterus.
The development of the female genital system is a complex embryological process. Congenital
malformations of the female genital tract may occur isolated or in combination with urologic defects.
They are a heterogeneous group of congenital anomalies that result from a failure in one of the three
development phases of the paramesonephric or Müller ducts:
• Organogenesis phase: One or both Müllerian ducts are not fully developed, leading to anomalies, such
as agenesis, bilateral or unilateral uterine aplasia (unicornuate uterus).
• Fusion phase: The process during which the distal segments of the Müllerian ducts fuse to form the
uterus, cervix and upper third of the vagina, is called the lateral fusion. A failure, abnormal fusion or
incomplete fusion result in a uterus didelphus or bicornuate uterus.
• The septum resorption phase: After the fusion, the central septum is reabsorbed leading to just one
endometrial cavity and just one cervix. If reabsorption does not take place, it results in septate uterus
that may or may not reach into the vagina
Associated symptoms of uterine malformation
Many women with these abnormalities are asymptomatic while others experience a variety and degree
of manifestations that can occur at any age, and generally depend on the type of anomaly and of
reproductive age. The following are the most common:
• Amenorrhea in Müllerian agenesis.
• Dismenorrhea in obstructive anomalies.
• Menstrual bleeding in communicating uteri.
• Repeated misacarriage and obstetrics complications in cases of fusion and reabsorption anomalies
(infertility, fetal malposition, ectopic pregnancy).
• Pelvic tumors caused by menstrual remains and endometriosis.
• Associated urinary and skeletal malformations

d. In 1983, the American Fertility Society (AFS), now American Society for Reproductive Medicine
(ASRM), proposed to qualify the above described classification with very few modifications in 198819
(Fig. 3). Seven groups are included in this classification of the lates 1980’s. It is useful but incomplete
because other associated anomalies are not mentioned (e.g. communicating uteri, etc.). Group I:
Hypoplasia/Agenesis
Group I: Hypoplasia/Agenesis
• Vaginal agenesis: It is a very rare malformation with a incidence of 1:10000 cases. The external
genitalia, fallopian tubes and ovaries are normal. The cervix is hypoplastic or absent, and the uterine
cavity may be normal or show any abnormalities. Ultrasound scan allows us to differentiate from
Rockitansky´s syndrome which has uterine agenesis. Also, testicular feminization can be excluded as no
ovarian tissue are visible.
• Cervix agenesis with uterine cavity and functional endometrium, is exceptional. Over 50% other
Müllerian anomalies associated (most common bicornuate uterus).
• Fundus agenesis is associated with primary amenorrhea and/or infertility. Ultrasound shows
rudimentary or absent uterine cavity in a woman with normal external genitalia, normal or hypoplastic
cervix with normal ovaries (Fig. 4).
• Fallopian tubes agenesis
Group II: Unicornuated Uterus
It is the result from the development of just one of the Müller’s duct while the other is not developed or
it may be partially developed and connected or not to the uterus. Three-dimensional ultrasound shows
uterine endometrial cavity with its volume decreased, and let us check if the authentic rudimentary horn
has functional endometrium and if they are communicated or not. They frequently are associated to
kidney agenesis. Therefore, the different possibilities are the following: • Unicornuate uterus with
rudimentary horn communicated and functional endometrium (IIa), • Unicornuate uterus with
rudimentary horn but not communicated with functional endometrium (IIb), (Fig. 5). Unicornuated
uterus with no functionalrudimentary horn (IIc), • Unicornuated uterus (IId
Group III: Didelphys Uterus
It is the result of the failure of both ducts fusion midline. Each hemiuterus develops complete but
independently leading to duplication of the genital system. This anomaly is usually associated with the
presence of a longitudinal vaginal septum in 75% of cases. Three-dimensional reveals two hemiuteri and
two cervix. Uterine bodies are presented as a mirror image, with a deep fundal separation
Group IV: Bicornuated Uterus
This type derives from a failure of the fusion of both Müller’s duct at the fundus. We can see two
endometrial cavities that get joined near the cervix, typically presenting a ‘notch’ at this level between
the two uterine horns. Depending on the slit extends partially (IVb) or completely (IVa), two varieties are
covered with different reproductive prognosis. Three-dimensional ultrasound has allowed differentiate
from septal uterus without performing a diagnostic laparoscopy.
Group V: Septate Uterus

It is the result of the reabsorption failure of the septum and may be partial (Vb) or complete (Va).
Septate uterus is the most common Müllerian malformation. Using 3D US can be differentiate septate
versus bicornuate uterus.22-24 A careful evaluation of the uterine fundus is preceptive. Septate uteri
show a flat or lineal fundus, whereas bicornuate shows a typical indentation (Fig. 15). This type is the
most common Müllerian malformation, twice as much as bicornuate uterus. The sub-septate variant
(Vb) is present in 85% of cases. Herein, we show a set of complete septum images (type Va), the septum
reaching cervix and vagina (Figs 15 to 18).
Complete septate uterus: Note, in the frontal plane, the lineal uterine fundus (yellow line) and the
septum reaching the external os (yellow arrows). To the right: 3D view using 'magic cut' software, the
septum is signaled (red arrow). Complete septate uterus with two cervix (yellow arrows): To the left TUI
mode. To the right 3D magic cut
Group VI: Arquate Uterus Represents a minor anomaly, caused by the failure of terminal fusion of the
Müllerian ducts. Initially, it was considered a variety of uterine septum. The ASRM classification labeled
it an own group, given its clinical features, totally different from the mentioned uterus. It is considered a
variant of normality, as it is not associated with an increased incidence of obstetric and reproductive
disorders. But today has generated much controversy, as some would be associated to bad reproductive
prognosis (Figs 24 and 25). Three-dimensional shows a linear fundus with an arched shape endometrial
cavity (Figs 24 and 25). Medical literature,25-28 describes that this variety and the uterine hypoplasia
represents two thirds of all uterine anomalies.
Group VII: Related with Dietilestilbestrol Uterus
Nonsteroidal estrogen DES was used in the management of various obstetric complications, including
first trimester bleeding, recurrent miscarriage, pre-eclampsia, premature delivery, etc.
This type of uterus typically shows a T shape morphology of the uterine cavity, as a consequence of the
uterine constriction produced by hypertrophy of the myometrium adjacent to atrophic areas.
Hypotrophic uterus, vaginal adenosis and cervical abnormalities are also usually described in this type of
Müllerian malformation
MÜLLERIAN MALFORMATIONS NOT INCLUDED IN THE AFS CLASSIFICATION Communicans Uteri

Although they are known since many years ago, they are very rare. This group is formed by uteri with
independent cavities wich are communicated in a particular point.

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INTRODUCTION

Congenital abnormalities of the uterus, or congenital müllerian anomalies, are

Associated symptoms of uterine malformation

• Amenorrhea in Müllerian agenesis.

• Dismenorrhea in obstructive anomalies.

• Menstrual bleeding in communicating uteri.

• Pelvic tumors caused by menstrual remains and endometriosis.

• Associated urinary and skeletal malformations

• Fallopian tubes agenesis

Group II: Unicornuated Uterus

Group III: Didelphys Uterus

Group IV: Bicornuated Uterus

Group V: Septate Uterus

Group VII: Related with Dietilestilbestrol Uterus

MÜLLERIAN MALFORMATIONS NOT INCLUDED IN THE AFS CLASSIFICATION Communicans Uteri

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