Taiwanese Journal of Obstetrics & Gynecology 59 (2020) 183e188

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Taiwanese Journal of Obstetrics & Gynecology

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Review Article

Diagnosis and treatment of müllerian malformations

Itana de Mattos Pinto e Passos a, *, Renata Lopes Britto a, b
a
Center for Women's Health, University Hospital Professor Edgard Santos, Federal University of Bahia (UFBA), Salvador, Bahia, Brazil
b
Department of Gynecology and Obstetrics, Faculty of Medicine, Federal University of Bahia (UFBA), Salvador, Bahia, Brazil

a r t i c l e i n f o a b s t r a c t

Article history: Anomalies in the müllerian ducts are congenital alterations with more prevalence than it is imagined,
Accepted 5 December 2019 varying from 0.5 to 6.7% in the general population and up to 16.7% in women with recurrent miscarriage.
The main findings are primary amenorrhea, dysmenorrhea, pelvic pain, endometriosis, sexual difficulties
Keywords: and low self-esteem. The major impact on the quality of life in women stricken by these problems
Genital anomalies justifies this study, whose objective is to analyze their most important aspects such as etiopathogeny,
Müllerian malformations
classification, diagnostic methods and proposed treatments. The research was performed on the
Pelvic pain
Medline-PubMed database from 1904 to 2018. The American Fertility Society, European Society of Hu-
Primary amenorrhea
Vaginal agenesis
man Reproduction and Embryology, and the European Society of Gynaecological Endoscopy classify
malformations as: Class 1/U5bC4V4: agenesis or hypoplasia of uterus and vagina; Class 1/U5aC4V4:
cervical hypoplasia, associated with total or partial vaginal agenesis; Class 2/U4: unicornuate uterus;
Class 3/U3bC2V1 or Class3/U3bC2V2: uterus didelphys; Class 4/U3C0: bicornuate uterus; Class 5/U2:
septate uterus; Class 6: arcuate uterus; Class 7/U1: induced by diethylstilbestrol, represented by a T-
shaped uterus; and V3: transverse vaginal septum. The diagnostic methods are the two-dimensional or
three-dimensional ultrasound, MRI, hysterosalpingo-contrast-sonography, X-ray hysterosalpingography,
hysteroscopy and laparoscopy. Some müllerian malformations are healed with surgery and/or self-
dilatation. For vaginal agenesis, dilatation by Frank technique shows good results while malformations
with obstruction of the menstrual flow need to be rapidly treated by surgery.
© 2020 Taiwan Association of Obstetrics & Gynecology. Publishing services by Elsevier B.V. This is an
open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Müllerian malformations are anomalies that are originated dur-
ing the development of the paramesonephric ducts and are char-
acterized by failures in the fusion of these structures in the middle
line when they connect to the urogenital sinus. They occur due to
alterations in the formation of the upper vaginal lumen and the
uterine lumen, and also because of the non-absorption of the
septum in the fusion of the ducts. The forms vary from the very light
ones, generally asymptomatic but that can cause serious obstetrical
disorders, to the most serious cases such as vaginal and uterine
agenesis, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome
[1], or the vaginal obstructions caused by agenesis or septum, but
with a functioning uterus, which signal an emergency and should
have immediate intervention. Reviewing Saravelos et al. [2], a
prevalence of congenital uterine anomalies was found in 6.7% of the
general population, 7.3% in sterile women and 16.7% in women who
had recurrent miscarriage. Besides, it was also observed that the
* Corresponding author. Rua Augusto Viana, s/n, Canela, CEP, 40110-060, Salvador,
Bahia, Brazil. Fax: þ55 71 3283 8074.
E-mail address: itanapassos@gmail.com (I.M.P. Passos).
septate uterus is the most common anomaly among the sterile
women, while the arcuate uterus is the most common among those
who have habitual abortion. In another revision, Nahum [3] found
uterine anomalies in 0.5% of the general population, 0.17% in fertile
women and 3.5% in infertile ones. The great diversity of müllerian
malformations generates not only many diagnostic but also treat-
ment doubts and the majority of the publications refers to report of
isolated cases or small series of cases, thus reflecting peculiarities in
groups selected as emergencies due to obstruction of menstrual
flow, obstetric complications or a history of sterility [4].
A review of the literature was done by means of an electronic
search in the Medline-PubMed database in the website (www.ncbi.
nlm.nih.gov/pubmed) from 1904 to 2018 using the MeSH terms
“amenorrhea” or “cervix uteri” or “congenital abnormalities” or
“diagnostic imaging” or “female genital diseases” or “müllerian
ducts” or “urogenital abnormalities” or “uterus” or “vagina”. The
chosen publications described some type of classification, etiopa-
thogeny, diagnostic method or treatment of the pathology pre-
sented. Due to the small number of randomized studies, the method
was not a criterion for choice, but still the relevance of the study.

https://doi.org/10.1016/j.tjog.2020.01.003
1028-4559/© 2020 Taiwan Association of Obstetrics & Gynecology. Publishing services by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://
creativecommons.org/licenses/by-nc-nd/4.0/).
184 I.M.P. Passos, R.L. Britto / Taiwanese Journal of Obstetrics & Gynecology 59 (2020) 183e188
Etiopathogeny
Sexual differentiation is a continuous process that starts with
the fertilization of the ovule by the sperm. In the female sex, in
normal conditions, the absence of the müllerian inhibitory factor
causes the degeneration of the mesonephric ducts and promotes
the development of paramesonephric ducts, which are originated
by the invagination of the coelom epithelium of the urogenital
crest. These structures are bilateral, suffer from stretching in
around the 9th week of pregnancy, and remain open and separated
in the upper segment thus originating the fallopian tubes. In the
lower segment, they unite to form the uterus and the upper 2/3 of
the vagina [1]. After the fusion, the septum between the para-
mesonephric ducts starts to be absorbed to form the uterovaginal
canal. Around the 12th week of pregnancy, the uterus assumes a
normal shape and its development is complete in the 22nd week
[5]. For the development of the vagina, it is necessary to have the
fusion of the urogenital sinus with the müllerian structures, which
gives the origin to the Müller tubercle. It induces the formation of
the vaginal plaque, whose canalization is complete in the 20th
week. The upper 4/5 of the vagina are of müllerian origin and the
lower 1/5 has its origin in the urogenital sinus. The epithelium of
the upper 1/3 of the vagina originates in the uterovaginal primor-
dium, the lower 2/3 in the urogenital sinus and the hymen is a sign
of the endodermal membrane [6]. Since the development of the
ovaries is independent of the uterovaginal canal and the uterine
tubes are developed from cells of a different origin from those of
the uterus and the vagina, these structures are not associated with
müllerian anomalies [1,7]. Due to the same mesodermal origin of
the genital and urinary tracts, any paramesonephric anomaly could
be associated with renal anomaly, which should always be inves-
tigated in these patients. Unilateral renal agenesis is a common
form. The congenital malformations of the female genital tract
occur when there is a failure in some stage of the embryogenesis
and the majority of them still have unknown etiology. Some studies
show the association with gene mutations, and the most cited are
those of HOXA 13 (Hand-foot-genital syndrome) [8] and HOXA 10,
expressed in the embryonic paramesonephric ducts [9]. Genital
anomalies induced by environmental agents such as diethylstil-
bestrol and thalidomide are also described in the literature. The
moment in which the failure occurs determines the type of mal-
formation and the earlier in the pregnancy it is, the more serious it
is. They vary from complete aplasia associated with urinary mal-
functions, if they occur between the 6th and 9th week, up to the
total or partial septation, rarely associated with urinary malfor-
mations if they occur between the 13th and 17th week.
Classification
Several classifications have been proposed since the 19th cen-
tury, based on embryology and on the development of müllerian
ducts, however, with imprecise terminology and failures in the
characterization of the anomalies. All of these intended to make the
diagnoses more accurate and enable comparisons between cases,
but without consensus in relation to their use. Among those, the
classification VCUAM (Vagina Cervix Uterus Adnex-associated
Malformation) [10] can be cited as well as that one proposed by
Acien and Acien [11]. Currently, the most used was the proposed by
Buttram and Gibbons [12], accepted and modified in 1988 by the
American Fertility Society (AFS), today the American Society of
Reproductive Medicine (ASRM) [13], which separates the anoma-
lies into seven classes (Table 1). The European Society of Human
Reproduction and Embryology (ESHRE) and the European Society
of Gynaecological Endoscopy (ESGE) [14] developed another clas-
sification based on anatomy, embryological origin, degree of
uterine deformity, cervical and vaginal anomalies (Table 2). It is
possible that no classification of müllerian anomalies can encom-
pass all these types of malformations, which could present them-
selves in many different ways. Based on AFS-ASRM [13] and ESHRE/
ESGE [14], the classification of anomalies is as follows (Fig. 1): Class
1 (AFS)/U5bC4V4 (ESHRE/ESGE): This class corresponds to agenesis
or hypoplasia of uterus and vagina, which in its extreme form is
known as the MRKH syndrome. The failure occurs at the start of the
development of the müllerian ducts, whose caudal extremity is
destined to merge and to originate the superior part of the vagina
and uterine cervix. It is a congenital anomaly, which presents itself
with agenesis or severe uterine hypoplasia and the absence of the
upper 2/3 of the vagina in patients with normal female karyotype
(46,XX) and development of secondary sexual characters compat-
ible with age [15]. The lower third of the vagina rarely passes 2 cm
in depth. Its prevalence is of 1/4500e5000 women [1,16] and,
despite being a rare disease, is considered the second most com-
mon cause of primary amenorrhea, right after hypogonadism [17].
MRKH syndrome is classified in two groups: typical (isolated ute-
rovaginal agenesis) and atypical (associated with extra genital
malformations of the kidneys, skeleton, auditory system and heart)
[15]. The patients present primary amenorrhea and incapacity for
vaginal coitus. The most present extra genital malformations are
renal, varying from 15 to 34% among several authors.
Class 1/U5aC4V4 (ESHRE/ESGE)
Congenital cervical atresia occurs in 1/80,000-100,0000 women
[18] and is associated with total or partial aplasia of the vagina and
renal anomalies. The uterus and tubes can be severely distended by
the menstrual blood that has no way to flow out, and in extreme
cases can lead to an acute hemorrhagic abdomen. It should be
diagnosed and treated early due to its significant morbidity and
mortality.
Class 2 (AFS)/U4 (ESHRE/ESGE)
Unicornuate uterus occurs when one of the müllerian ducts
does not migrate to its correct place and a failure in the unilateral
development occurs. This uterine horn can be unique, when there is
complete agenesis of one of the müllerian ducts (U4b), or accom-
panied by another rudimentary uterine horn, which could be of
three types: without cavity (U4b), cavitated or cavitated noncom-
municating horn (U4a). The endometrium in the latter undergoes
hormonal stimulation and its cavity progressively increases in
Table 1
Classification of müllerian duct anomalies according to the AFS [13].
Class 1 Segmental Vaginal
Hypoplasia/agenesis Cervical
Fundal
Tubal
Combinated
Class 2 Unicornuate Rudimentary horn with endometrial tissue
- Communicating with the main uterine cavity
- Not communicating with the main uterine
cavity
Rudimentary horn without endometrial tissue
No rudimentary horn
Class 3 Didelphys
Class 4 Bicornuate Complete
Parcial
Class 5 Septate Complete
Parcial
Class 6 Arcuate
Class 7 Diethylstilbestrol T-shaped
drug related
 I.M.P. Passos, R.L. Britto / Taiwanese Journal of Obstetrics & Gynecology 59 (2020) 183e188 185

Table 2 only one cervix. Depending on the degree of deficiency of the

Classification of female genital tract anomalies according to the ESHRE/ESGE [14]. fusion, the bicornuate uterus can be complete, when the cavities
Uterine anomaly are separated up to the internal orifice of the cervix and are not
Main Class Sub-class
linked, or partial, when there is some linkage. It represents around
U0 e Normal uterus 10% of the müllerian malformations, is asymptomatic in the ma-
U1 e Dysmorphic T-shaped jority of cases and can cause miscarriage or premature birth [22].
uterus Infantilis
Others
Class 5 (AFS)/U2 (ESHRE/ESGE)
U2 e Septate uterus Parcial
Complete
U3 e Bicorporeal Parcial Septate uterus is the result of the deficit in the reabsorption of
uterus Complete the median septum after the fusion of the müllerian structures.
Bicorporeal septate
Depending on the moment when the failure occurs, the septum can
U4 e Hemi-uterus With rudimentary cavity (communicating or
not horn)
be complete or partial and the external contour of the uterus is
Without rudimentary cavity (horn without always normal. The structure of the septum can be muscular or
cavity/no horn) fibrous and this diagnostic is extremely important for therapeutic
U5 e Aplastic With rudimentary cavity (bi- or unilateral horn) approach. It represents 55% of the malformations and is associated
Without rudimentary cavity (bi- or unilateral
with recurrent miscarriage and premature birth. It is one of the
uterine remnants/aplasia)
U6 e Unclassified malformations with the worst results in relation to reproduction
malformations [22,23].
Cervical/vaginal
anomaly Class 6 (AFS)
C0 e Normal cervix
C1 e Septate cervix Arcuate uterus is also considered a variant of the normal and it
C2 e Double normal only presents a discrete curvature on its bottom, with no clinical
cervix translation [13]. Eventually it can be the cause of reproductive
C3 e Unilateral cervical aplasia
alteration when no other problem is detected. It occurs due to the
C4 e Cervical aplasia
V0 e Normal vagina
failure in the final stage of reabsorption of the intermüllerian
V1 e Longitudinal non-obstructing vaginal septum septum and does not need intervention.
V2 e Longitudinal obstructing vaginal septum
V3 e Transverse vaginal septum and/or imperforate hymen Class 7 (AFS)/U1 (ESHRE/ESGE)
V4 e Vaginal aplasia

Induced by diethylstilbestrol, represented by a T-shaped uterus

volume due to the retention of menstrual blood, which has no way
of flowing out. This causes pain and an increase in the abdominal
volume. Patients with a unicornuate uterus have a bad reproductive
prognosis and can have obstetrical complications such as mis-
carriages, restriction of intrauterine growth, premature birth labor,
etc. The presence of only one uterine artery and the small contri-
bution of the contralateral arterioles compromises the blood irri-
gation, reducing the muscular mass of the organ. Unicornuate
uterus represents 0.3e4% of the uterine anomalies, occurs in 1/
5400 women and 74e90% are associated with rudimentary horn
[19,20].
Class 3 (AFS)/U3bC2V1 or U3bC2V2 (ESHRE/ESGE)
Uterus didelphys occurs when there is complete failure of
approximation and fusion of the müllerian ducts, nonetheless their
development continues individually, giving origin to two uterine
cavities, two cervices and two vaginas separated by a longitudinal
septum and with normal menstrual flow (U3bC2V1). The septum
can also be oblique, obstruct one of the vaginas and cause men-
strual flow retention of that hemi-uterus causing pelvic and lumbar
pain, vaginal discomfort, hematocolpos, hematometra and hema-
tosalpinx (U3bC2V2). When, added to this, there is ipsilateral renal
agenesis, it is called the Herlyn-Werner-Wunderlich (HWW) syn-
drome and represents 3e4% of the müllerian malformations. The
obstetric prognosis is good and there are reported cases of preg-
nancies with twins with a fetus in each uterus [21].
Class 4 (AFS)/U3C0 (ESHRE/ESGE)
Bicornuate uterus occurs when there is a failure in the fusion of
the two müllerian structures that results in two uterine horns and
detected in daughters of women who used this drug during preg-
nancy. The uterine cavity is irregular and hypoplastic; there are
poor chances for pregnancy and high risk of miscarriage or ectopic
pregnancy [24]. Diethylstilbestrol was discontinued in 1971, and,
for this reason, this is an increasingly rare anomaly, which tends to
disappear.
V3 (ESHRE/ESGE)
Transverse vaginal septum results from the failure of canaliza-
tion of the vaginal plaque at the point where the urogenital sinus
meets the müllerian duct and it is not associated with other mal-
formations. It can be perforated or not, with variations of thickness
and localization along the vagina. Women with a perforated
septum take more time to have a diagnosis because they
menstruate normally and there are few symptoms. The thickness
and localization are extremely important to define the treatment:
the lowest, the thinnest and the perforated ones have better results,
while the highest and the thickest ones have great chances of
complications such as rectovaginal fistula and hysterectomy. Its
occurrence is estimated to be between 1/2100 and 1/72,000
women [25].
Diagnostic methods
The gynecological exam is always important, although in many
cases of genital malformations it cannot be done completely or will
not be elucidating. In cases of primary amenorrhea, the external
genitalia should be rigorously inspected to evaluate the distal
vaginal third. The initial diagnostic method is the two-dimensional
ultrasound (US2D), but also used are three-dimensional ultrasound
(US3D), MRI, hysterosalpingo-contrast-sonography, X-ray hystero-
salpingography, video-hysteroscopy and video-laparoscopy.
186 I.M.P. Passos, R.L. Britto / Taiwanese Journal of Obstetrics & Gynecology 59 (2020) 183e188
Fig. 1. Classification of müllerian duct anomalies according to the AFS and correlation with the ESHRE/ESGE classification.
The main advantages and disadvantages of these exams follow
below:
US2D is the initial method because it is simple, non-invasive,
and low cost, is usually available and provides good information;
however, it is highly dependent on the experience of the examiner
[26e28].
US3D has good reproducibility, high level of agreement among
different observers, provides additional and more reliable images
and allows for the evaluation of the cervix and the vagina; however,
it is less available and requires more specialized training than the
US2D [26e29].
MRI is considered the gold standard, offers objective and reliable
tridimensional information about all the genital and peritoneal
anatomy, except for the tubes; it can be used in all cases, including
obstructive malformations. It is more expensive and less available
than the US and needs a qualified professional to interpret the re-
sults [26,30e32].
Hysterosalpingo-contrast-sonography is a minimally invasive
method, low cost, and provides good information about the cervix
and uterine cavity but is highly dependent on the examiner and the
distention of the uterine cavity can modify its internal contours
generating false negative images [26,33].
X-ray hysterosalpingography provides information only about
the uterine cavity and tubes and is used more in cases of infertility.
It is an invasive, painful exam and does not evaluate the external
contour, does not differentiate the septate uterus from the bicorn-
uate one, does not diagnose the noncommunicating uterine horn
and cannot be used in vaginal and cervical obstructions [26,34].
Hysteroscopy is minimally invasive and provides reliable infor-
mation about the vagina, cervical canal and uterine cavity, although
it does not evaluate the external contours or the thickness of the
uterine wall and does not differentiate the septate uterus from the
bicornuate one [26].
Laparoscopy evaluates the external contour of the uterus and
the peritoneal structures, but it is an invasive exam, does not
evaluate the thickness of the uterine wall and completely depends
on the experience and subjective evaluation of the examiner [26].
Treatment
Some müllerian malformations are healed with surgery and the
success of the treatment depends on accurate diagnosis and the
choice of the best technique [35].
Vaginal agenesis can be treated with dilatation or vaginoplasty
and since 2002 the ACOG has recommended dilatation as the first
choice given the high rate of success and lack of complications [36].
The most common method is the Frank technique [37,38], which
consists of daily self-application of rigid vaginal dilatators with
progressive increase in the length and width, for 30e120 min, for
many months. Other dilatation techniques described are those of
Ingram [39], with a dilatator affixed to the seat of a bicycle and
dilatation through coitus, according to d’Alberton [40]. The treat-
ment should only be started when the patient is mature enough
and expresses the desire to try, since it is not a widely accepted
method; it is also difficult to manage and comes with a lot of
anguish and fear. The patient also must know that is just in order to
make normal vaginal coitus possible. When the dilatation is un-
successful, vaginoplasty could be done by various techniques [41],
the recommended one being that in which the surgical team has
more experience. The most used is that of McIndoe [42], which
utilizes an autologous skin flap in latex mold placed after the
tunneling of the vagina. Other described techniques are Davydov's
[43], through laparoscopy and using peritoneum to recover the
vagina walls, Baldwin's [44], through laparotomy and using a
segment of the intestine, and Vecchietti's [45], through laparoscopy
and pulling the vaginal dimple. The best anatomical results with the
lowest rates of complications are in patients that perform dilatation
through coitus. The McIndoe vaginoplasty has the highest rate of
complications, the most common of which are the shrinkage and
stenosis of the neovagina [46]. The choice for the best treatment for
vaginal agenesis continues to be controversial, principally due to
the lack of randomized and comparative studies. The rarity of the
pathology makes the surgical teams always use the same technique.
Congenital cervical and vaginal atresia is controversial in terms
of treatment, since there are no guidelines or randomized studies
 I.M.P. Passos, R.L. Britto / Taiwanese Journal of Obstetrics & Gynecology 59 (2020) 183e188
that point to the best surgery. It is a gynecological urgency with
various risks and hysterectomy is the principal option for treatment
according to several authors, principally due to its innumerous
benefits [47]. Zhang Y et al. [18] reported on four patients diagnosed
with cervical and vaginal atresia who underwent robotic assisted
reconstruction of cervix and vagina by SIS graft during the year of
2015. All of them recovered after the surgery and had regular
menstruations without periodic pelvic pain. Average follow-up was
12 months, average vagina length was 8.9 ± 0.3 cm, vagina width
was 2.9 ± 0.1 cm and there was no re-admissions.
Unicornuate uterus only needs surgical correction if there is a
cavitated noncommunicating rudimentary uterine horn, which
needs resection due to the pain that results from the impediment of
menstrual flow. In some cases, its muscular mass is reduced,
causing isthmus-cervical incompetence and there might be the
necessity of cerclage in a future pregnancy [19].
Uterus didelphys has a good reproductive prognosis and only
needs intervention in cases of HWW syndrome, since one of the
vaginas is obliterated and the septum between them needs to be
resected to drain the hematocolpos and hematometra, as well as it
allows for the outflow of normal menstrual flow [21].
Bicornuate uterus is not a cause of difficulty conceiving, but
rather a recurrent miscarriage in the 2nd trimester of pregnancy
and premature birth. When no other cause is identified, Strass-
man's metroplasty can be recommended, with good results and a
90% rate of full pregnancy [48].
Septate uterus is the most common müllerian malformation
with the worst prognosis for reproduction, associated with mis-
carriages in the first and second trimesters, although the treatment
is relatively easy. The chosen treatment is hysteroscopy metro-
plasty, with good results [23].
Transverse vaginal septum should be treated with surgical
resection and anastomosis of the proximal and distal vaginas. The
choice of the technique depends on its localization and thickness,
which is diagnosed in a physical exam, US and MRI, and it can be
vaginal or laparoscopic. The lowest, the thinnest and the perforated
ones have the best results, and the main complications are stenosis,
re-obstruction, dyspareunia and psychological difficulties. Vaginal
dilatation is generally recommended after surgery to improve the
result [25,49].
Discussion
The various müllerian anomalies present themselves in diverse
forms and in different phases of a woman's life. A careful anamnesis
is always the first step and a thorough clinical exam plays a funda-
mental role for any decision about the best diagnostic methods and
choice of the best treatment. The two principal classifications used
currently, that of AFS-ASRM [13] and of ESHRE/ESGE [14], encompass
the majority of cases, although there are others and there is still not a
consensus. There are isolated cases of müllerian malformations that
do not fit into any of the classifications.
Women with more impacting symptoms such as primary
amenorrhea, incapacity for vaginal coitus or pelvic pain, due to
obstruction of the menstrual flow, are diagnosed earlier because
they seek assistance earlier. However, those women experiencing
problems related to reproduction, such as recurrent pregnancy loss,
premature birth and infertility, many times only have diagnosis of
genital malformation during the investigation of these symptoms.
Diagnosis through images is indispensable, US2D is used a lot
for the first exam and various authors consider MRI as the gold
standard.
The clinical presentation and treatment for müllerian malfor-
mations are directly related to the anatomy of the defect. Malfor-
mations that obstruct the menstrual flow should be treated rapidly
187
doing a suitable surgical procedure for each case. Patients with
vaginal agenesis may undergo vaginoplasty or vaginal dilatation
through diverse techniques. In these cases, psychological support is
extremely important and the procedure should only be done after
the patient is ready for it.
Due to the frequent association between müllerian and urinary
anomalies, the finding of any of the types should lead to the
investigation of the other. Since it is a rare pathology, the majority
of studies found in the literature is composed of case reports, small
series of cases, comparative studies and many authors just report
the experience of their service. Studies involving genetics and
embryology, as well as randomized studies, are necessary so that
one can understand and treat, in an increasingly better fashion,
women who are born having to deal with the fact that they are
different.
Declaration of Competing Interest
The authors have no conflicts of interest relevant to this article.
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