DO WE NEED TO TREAT ANY OF UTERINE ABNORMALITY
IN FERTILITY SEEKING PATIENT ?
Muhammad Rusda
Department of Obstetrics and Gynecology School of Medicine, Universitas Sumatera Utara
Abstract
Uterine abnormality are resulting from failure in organogenesis, fusion, or in reabsorption.
Frequently, patients with mullerian anomalies are not presenting in childhood or adolescence,
but in the adulthood. Several radiology tools are available to assist in the diagnosis of
congenital anomalies of the woman reproductive tract. Because of the complexity of the
presentation, the diagnosis of uterine anomalies requires the use of more than one of imaging
method in 62% of cases. Therapy for uterine anomalies varies according to the specific type of
malformation found in each pasien. Surgical correction of uterine anomalies in asymptomatic
women with primary infertility remains controversial. Currently, surgery for uterine anomalies
indicated for women with pelvic pain, endometriosis, obstructive anomalies, and poor obstetric
outcomes such as RPL and premature delivery.
Keywords: uterine abnormality, mullerian anomalies, infertility
Introduction
The normal development of the female reproductive tract involves a complex integrative series
of events involving genetic, hormonal, and epigenetic factors leading to the normal
differentiation and development of the Mullerian, or paramesonephric, ducts, Wolffian, or
mesonephric, ducts, and urogenital sinus. Accordingly, any one of numerous alterations in this
process can lead to abnormalities of the uterus, cervix, fallopian tubes, or vagina. Depending on
the specific abnormality, affected women may experience gynecologic, fertility, or obstetrical
problems1.
Müllerian duct anomalies consist of a set of structural malformations resulting from abnormal
development of the paramesonephric or Müllerian ducts2. Uterine anomalies are the most
common of the mu¨ llerian anomalies, but the true incidence is not known since many women
are asymptomatic, and sensitive imaging modalities have only recently become available.
impaired ability to conceive. The proper management of infertile women with uterine anomalies
is controversial 3.
Physiopathology
Uterine malformations can be caused by failure in organogenesis, fusion, or in reabsorption.
Failure in organogenesis can results in agenesis, hypoplastic utery or unicornuate utery, while
failure in fusion can results in didephys, bicornuate atau arcuate utery. Failure in in reabsorption
can be manifested as uterine with partial or complete septum2.
Classification System
In 1979, Buttram and Gibbons proposed a classification system for mullerian anomalies based on
the type and degree of failure of normal development of the female genital tract. This rubric was
subsequently revised by the American Society for Reproductive Medicine in 1988. Although by
no means exhaustive, the utility of this classification system lies in its provision of a standardised
nomenclature, allowing for physicians to more accurately codify, and therefore treat, patients
with mullerian anomalies 4.
The AFS classification system has been widely accepted and is currently the most widely used
system. Two other proposals have also been published; in 2004, Acien et al. proposed a new
system based on the embryological origin of the anomalies, and 1 year later, Oppelt et al.
proposed the vagina, cervix, uterus, adnexae, and associated malformations (VCUAM) system.
Although it is too early for permanent conclusions, these systems have not been widely
accepted.5
The basis of the VCUAM system is the anatomy of the female genital tract. Each organ is
classified separately, similar to the tumor, node, and metastasis classification system for breast
tumors. This approach provides the opportunity to have a precise, detailed, and extremely
representative manner of classifi-cation; each type of anomaly could be described using this
system, and the clinician could have an accurate idea of each individual’s genital tract anatomy.
The main disadvantage of this system is that it is not simple or user-friendly. Patients are
classified only with the help of the classification system’s tables. ‘‘Translating’’ what a ‘‘V5b,
C2b, U4b, A0, MR’’ patient has (a woman with Mayer-Rokitansky-Kuster-Hauser syndrome) is
not easily done without the use of the appropriate tables. This system is not practical for
ESHRE/ESGE classification system has the following general characteristics:
(i) Anatomy is the basis for the systematic categorization of anomalies.
(ii)Deviations of uterine anatomy deriving from the same embryological origin are the basis for
the design of the main classes.
(iii)Anatomical variations of the main classes expressing different degrees of uterine deformity
and being clinically significant are the basis for the design of the main sub-classes.
(iv)Cervical and vaginal anomalies are classified in independent supplementary sub-classes.
Anomalies are sorted in the classes and sub-classes of the system according to increasing
severity of the anatomical deviation; the less severe variants are placed in the beginning, the
more deformed types at the end. For the sake of simplicity, an extremely detailed
subclassification is avoided: anatomical variations of uterine, cervical and vaginal anomalies are
grouped in sub-classes having as criterion the clinical significance of the abnormality 6.
In addition, European Society of Human Reproduction and Embryology (ESHRE) and the
European Society for Gynaecological Endoscopy (ESGE), recognizing the clinical significance
of female genital anomalies, have established a common working group under the name
CONUTA (CONgenital UTerine Anomalies), with the goal of developing a new updated
classification system 6.
Epidemiology
In recent study, canalization defects, namely subseptate or septate uteri, have a prevalence of
2.3% in the unselected population when optimal tests. This condition, however, significantly
more common in women with miscarriage 7
Chan YY et al in their systematic review found that, in general/fertile population, the prevalence
of hypoplastic utery is 0,1%, unicornuate 0,1 %, didelphys 0,1%, bicornuate 0,3%, septate 1,3%,
arcuate 2,4%, and T-shaped uterine 0,003%. The most prevalence is arcuate uterine. Meanwhile,
in infertile population, the prevalence of hypoplastic utery is 0,6%, unicornuate 0,4 %, didelphys
0,2%, bicornuate 0,8%, septate 3,9%, and arcuate 2,1%. Septate uterine is the most common in
infertile women compared with other uterine anomalies. They also found that arcuate uterine is
the most prevalence in the recurrent miscarriage population (12% from 1937 cases of uterine
Diagnosis
Women with recurrent miscarriage have a high prevalence of congenital uterine anomalies and
should be thoroughly investigated. HSG and/or 2D US can be used as an initial screening tool.
Combined hysteroscopy and laparoscopy, SHG and 3D US can be used for a definitive
diagnosis. However, The accuracy and practicality of MRI remains unclear 8.
In one recent study on the accuracy of 3D ultrasound in diagnosis and classification of congenital
uterine anomalies, it is showed that ultrasound diagnosis proved inaccurate in two cases (total
230 cases), including an arcuate and a bicornuate uterus that at endoscopy were, respectively,
classified as a subseptate and a complete septate uterus 9. However, Bermejo C et al suggest that
there is a high degree of concordance between 3D ultrasound and MRI in the diagnosis of uterine
malformations, the relationship between cavity and fundus being visualized equally well with
both techniques. 3D ultrasound should be complemented by careful gynecological exploration in
order to identify any alterations in the cervix 10.
Treatment
Currently, surgery for mullerian anomalies is indicated for women with pelvic pain,
endometriosis, obstructive anomalies, and poor obstetric outcomes such as RPL and preterm
delivery. Prior to performing surgery, it is important to exclude extrauterine factors which may
cause pregnancy loss. The goals of surgery include treatment of pelvic pain, restoration of pelvic
anatomy and uterine architecture, and preservation of fertility. Inherent developmental
abnormalities, however, such as abnormal myometrium or altered vascularization, may
permanently impair uterine function 3.
Unicornuate uterus. women with unicornuate uterus should be managed expectantly with adherence to standard indications for cerclage placement. Additionally, removal of a functional
rudimentary horn is recommended as treatment for pelvic pain and endometriosis, and to prevent
conception in an obstructed horn 3,11,12.
Uterine didelphys. Uterine reconstruction with the Strassman metroplasty should be considered. The Strassman metroplasty achieves unification of two endometrial cavities in a divided uterus
(bicornuate or didelphys), and is associated with a live birth rate greater than 80%. Several
experts believe, however, that existing data do not support repair of a didelphic uterus to improve
pregnancy outcome. In contrast, incision of the longitudinal vaginal septum is indicated for an
Bicornuate uterus. The Strassman metroplasty should be reserved for select women based on poor reproductive outcomes. Furthermore, the bicornuate uterus is associated with a high
incidence of cervical incompetence (38%). Although studies have identified improvements in
fetal survival rates and decreased preterm delivery rates with a cervical cerclage, expectant
management and appropriate adherence to standard indications for cerclage placement are
warranted3. Sinha R et al. reported a case of bicornuate uterus managed by laparoscopic
metroplasty. Comparison of the reproductive outcome after this procedure and the abdominal
Strassman’s metroplasty requires more study. They suggest that laparoscopic metroplasty could
form a viable alternative 13.
Septate uterus. Hysteroscopic metroplasty has been demonstrated to significantly improve the live birth and miscarriage rates to approximately 80 and 15%, respectively, and is recommended
when the uterine septum is implicated in RPL, second trimester loss, malpresentation, or preterm
delivery. The hysteroscopic approach is preferred due to its safety, simplicity, and excellent
posttreatment results. Concomitant laparoscopy enables evaluation of the pelvis and external
uterine contour, and guides the extent of septum resection. Traditionally the cervical portion of a
complete septum is left intact due to the risk of cervical incompetence, but a recent small
randomized study demonstrated that resection of the cervical septum is associated with a less
complicated surgical procedure and equivalent reproductive outcomes. Postoperative formation
of intrauterine synechiae is rare, and routine use of an intrauterine balloon catheter, estradiol
supplementation, or antibiotics have not been shown to be necessary. A follow-up examination
should be performed 1–2 months after the procedure; ultrasonography, HSG, and hysteroscopy
are reasonable approaches 3.
In recent study, there is 63.5% pregnancy rate and a 50.2% live-birth rate after hysteroscopic
metroplasty of septate uterus. Although no prospective randomized studies have been performed
with an adequate number of patients to demonstrate the efficacy of treatment vs no treatment, the
overall success reported indicates its efficacy and reaffirms the place of minimally invasive
treatment such as hysteroscopic metroplasty as the criterion standard and method of choice for
treatment of this septate uterus 14.
A prospective randomized study compared Small-diameter hysteroscopy with Versapoint versus
resectoscopy with a unipolar knife for the treatment of septate uterus. Small-diameter
resectoscopy with unipolar electrode regarding reproductive outcome and is associated with:
shorter operating time and lower complication rate. Hysteroscopic metroplasty is now the
reccomended treatment for septate uteri due to its relative simplicity, low morbidity and
excellent reproductive outcome 15.
Arcuate uterus. Compared with women with a normal uterus,women with an arcuate uterus have a higher proportion of second trimester losses and preterm labor. Reconstructive procedures on
an arcuate uterus, however, do not improve pregnancy outcomes 3,11.
Diethlylstilbestrol exposure. Due to uterine and cervical anomalies, pregnant women with a history of DES exposure are at risk of cervical incompetence; options include expectant
management, bedrest, and prophylactic or emergent cerclage placement. Studies have shown a
term delivery rate greater than 70% in DES-exposed women without and with cerclage (both
prophylactic and emergent procedures). Cerclage placement may benefit DES-exposed women,
thus appropriate candidates should be considered based on standard indications for cerclage
placement 3,16.
Reproductive Outcomes
A recent systematic review showed that arcuate uterus were associated with increased rates of
second-trimester miscarriage and fetal malpresentation at delivery. Arcuate uterus are
specifically associated with second-trimester miscarriage. Canalization defects were associated
with reduced clinical pregnancy rates and increased rates of first-trimester miscarriage, preterm
birth and fetal malpresentation. In addition, unification defects were associated with increased
rates of preterm birth and fetal malpresentation. All uterine anomalies increase the chance of
fetal malpresentation at delivery 7.
Several studies cite profound rates of adverse obstetrical outcomes associated with uterine septa,
with foetal survival rates of 6–28% and a rate of spontaneous miscarriage as high as 60% or
greater. 4
Unicornuate uterus, with its compromised uterine mass and sometimes associated uterine horns,
brings with it considerable reproductive hurdles. Unicornuate uterus has been implicated in
IUGR, miscarriage, malpresentation, preterm labour and cervical incompetence.57 In our recent
review of 290 patients with unicornuate uterus, 175 patients conceived to carry a total of 468
pregnancies. In sum, 2.7% of pregnancies were ectopic, 24.3% ended in first trimester abortion
delivery and term births were 10.5%, 20.1% and 44.0%, respectively, with a total live-birth rate
of 49.9% 4,17.
Obstetrical outcomes are generally reported to be better in cases of bicornuate uterus than in
unicornuate uterus, perhaps given the significant variation in bicornuate uterine anatomy,
subtypes of which involve a partially fused central uterine cavity. A 36% abortion rate and 23%
preterm delivery rate have been quoted, but may still overstate the extent of obstetric
compromise given selection bias and escaped detection of many asymptomatic patients 11.
Uterine didelphys has a relatively good prognosis for achieving pregnancy; an infertility rate of
13% has been reported from a study of 49 women with a mean follow-up of 9 years. A review of
114 patients with untreated uterine didelphys who had a total of 152 pregnancies exhibited a
mean miscarriage rate of 32.9% and preterm delivery rate of 28.9%, with a live-birth rate quoted
as 56.6% 11.
Research has suggested that congenital absence of the uterus and vagina are not commonly
transmitted to offspring in MRKH surrogate pregnancies. Uterine transplantation remains a
hypothetical treatment of the future, with only one failed human attempt documented in the
literature. Animal models for such transplantation, however, have enjoyed some degree of
success and continue to be researched 4.
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