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A uterine malformation is a type of
female genital malformation A female genital disease is a condition that affects the female reproductive system. Classification by type of disease Malformation Malformations can be congenital. They are classified by location of the malformation, such as uterine malforma ...
resulting from an abnormal development of the
Müllerian duct Paramesonephric ducts (or Müllerian ducts) are paired ducts of the embryo that run down the lateral sides of the genital ridge and terminate at the sinus tubercle in the primitive urogenital sinus. In the female, they will develop to form the fa ...
(s) during
embryogenesis An embryo is an initial stage of development of a multicellular organism. In organisms that reproduce sexually, embryonic development is the part of the life cycle that begins just after fertilization of the female egg cell by the male sperm ...
. Symptoms range from
amenorrhea Amenorrhea is the absence of a menstrual period in a woman of reproductive age. Physiological states of amenorrhoea are seen, most commonly, during pregnancy and lactation (breastfeeding). Outside the reproductive years, there is absence of mense ...
,
infertility Infertility is the inability of a person, animal or plant to reproduce by natural means. It is usually not the natural state of a healthy adult, except notably among certain eusocial species (mostly haplodiploid insects). It is the normal st ...
,
recurrent pregnancy loss Recurrent miscarriage is three or more consecutive pregnancy losses. In contrast, infertility is the inability to conceive. In many cases the cause of RPL is unknown. After three or more losses, a thorough evaluation is recommended by American So ...
, and pain, to normal functioning depending on the nature of the defect.


Types

The American Fertility Society (now
American Society of Reproductive Medicine The American Society for Reproductive Medicine (ASRM) is a nonprofit, multidisciplinary organization for advancement of the science and practice of reproductive medicine. The society has its headquarters in Washington, D.C and its administrati ...
) Classification distinguishes: ; Class I—
Müllerian agenesis Müllerian agenesis, also known as Müllerian aplasia, vaginal agenesis, or Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome), is a congenital malformation characterized by a failure of the Müllerian ducts to develop, resulting in a mis ...
(absent uterus). : This condition is represented by the hypoplasia or the agenesis (total absence) of the different parts of the uterus: :* Vaginal hypoplasia or agenesis :* Cervical hypoplasia or agenesis :* Fundal hypoplasia or agenesis (absence or hypoplasia of the fundus of the uterus) :* Tubal hypoplasia or agenesis (absence or hypoplasia of the Fallopian tubes) :* Combined hypoplasia the agenesis of different part of the uterus :This condition is also called Mayer-Rokitansky-Kuster-Hauser syndrome. The patient with MRKH syndrome will have primary
amenorrhea Amenorrhea is the absence of a menstrual period in a woman of reproductive age. Physiological states of amenorrhoea are seen, most commonly, during pregnancy and lactation (breastfeeding). Outside the reproductive years, there is absence of mense ...
. ; Class II— Unicornuate uterus (a one-sided uterus). : Rare condition in which a partial or complete lack of one Müllerian duct is present. The uterus has a typical "banana shape" on imaging systems. This condition can be sub-classified in: :* Communicating: a rudimentary uterine horn is formed and it presents a cavity that communicates with the body of the uterus :* Non-communicating: a rudimentary uterine horn is formed and it presents a cavity that does not communicate with the body of the uterus :* No cavity: a rudimentary uterine horn is present without any cavity :* No horn: there is no rudimentary uterine horn, just the functioning one is present ; Class III— Uterus didelphys, also uterus didelphis (double uterus). : Both Müllerian ducts develop but fail to fuse, thus the patient has a "double uterus". This may be a condition with a double cervix and a vaginal partition (v.i.), or the lower Müllerian system fused into its unpaired condition. Se
Triplet-birth with Uterus didelphys
for a case of a woman having spontaneous birth in both wombs with twins. ; Class IV— Bicornuate uterus (uterus with two horns). : Only the upper part of that part of the Müllerian system that forms the uterus fails to fuse, thus the caudal part of the uterus is normal, the cranial part is bifurcated. The uterus is "heart-shaped". This condition can be complete or partial. ; Class V—
Septated uterus A uterine septum is a form of a congenital malformation where the uterine cavity is partitioned by a longitudinal septum; the outside of the uterus has a normal typical shape. The wedge-like partition may involve only the superior part of the cav ...
(uterine septum or partition). : The two Müllerian ducts have fused, but the partition between them is still present, splitting the system into two parts. With a complete septum the vagina, cervix and the uterus can be partitioned. Usually the septum affects only the cranial part of the uterus. A uterine septum is the most common uterine malformation and a cause for
miscarriage Miscarriage, also known in medical terms as a spontaneous abortion and pregnancy loss, is the death of an embryo or fetus before it is able to survive independently. Miscarriage before 6 weeks of gestation is defined by ESHRE as biochemical ...
s. It is diagnosed by medical image techniques, i.e. ultrasound or an MRI. MRI is considered the preferred modality due to its multiplanar capabilities as well as its ability to evaluate the uterine contour, junctional zone, and other pelvic anatomy. A hysterosalpingogram is not considered as useful due to the inability of the technique to evaluate the exterior contour of the uterus and distinguish between a bicornuate and septate uterus. This condition can be complete or partial :A uterine septum can be corrected by hysteroscopic surgery. ; Class VI— DES uterus. : The uterine cavity has a " T-shape" as a result of fetal exposure to
diethylstilbestrol Diethylstilbestrol (DES), also known as stilbestrol or stilboestrol, is a nonsteroidal estrogen medication, which is presently rarely used. In the past, it was widely used for a variety of indications, including pregnancy support for those with a ...
. An additional variation is the
arcuate uterus The arcuate uterus is a form of a uterine anomaly or variation where the uterine cavity displays a concave contour towards the fundus. Normally the uterine cavity is straight or convex towards the fundus on anterior-posterior imaging, but in the a ...
where there is a concave dimple in the uterine fundus within the cavity. The distinction between an arcuate uterus and a
septate uterus A uterine septum is a form of a Congenital disorder, congenital uterine malformation, malformation where the uterus, uterine cavity is partitioned by a longitudinal septum; the outside of the uterus has a normal typical shape. The wedge-like parti ...
is not standardized. A rudimentary uterus is a uterine remnant not connected to cervix and vagina and may be found on the other side of a unicornuate uterus. Patients with uterine abnormalities may have associated renal abnormalities including unilateral renal agenesis.


"Double vagina"

As the
vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hy ...
is largely derived from the Müllerian ducts, lack of fusion of the two ducts can lead to the formation of a vaginal duplication and lack of absorption of the wall between the two ducts will leave a residual
septum In biology, a septum (Latin for ''something that encloses''; plural septa) is a wall, dividing a cavity or structure into smaller ones. A cavity or structure divided in this way may be referred to as septate. Examples Human anatomy * Interat ...
, leading to a "double vagina". This condition may be associated with a uterus didelphys or a
uterine septum A uterine septum is a form of a congenital malformation where the uterine cavity is partitioned by a longitudinal septum; the outside of the uterus has a normal typical shape. The wedge-like partition may involve only the superior part of the cav ...
.=


Diagnosis

Besides a physical examination, the physician will need imaging techniques to determine the character of the malformation:
gynecologic ultrasonography Gynecologic ultrasonography or gynecologic sonography refers to the application of medical ultrasonography to the female pelvic organs (specifically the uterus, the ovaries, and the fallopian tubes) as well as the bladder, the adnexa, and the re ...
, pelvic MRI, or
hysterosalpingography Hysterosalpingography (HSG), also known as uterosalpingography, is a radiologic procedure to investigate the shape of the uterine cavity and the shape and patency of the Fallopian tubes. It is a special x-ray using dye to look at the womb (ute ...
. A hysterosalpingogram is not considered as useful due to the inability of the technique to evaluate the exterior contour of the uterus and distinguish between a bicornuate and septate uterus. In addition,
laparoscopy Laparoscopy () is an operation performed in the abdomen or pelvis using small incisions (usually 0.5–1.5 cm) with the aid of a camera. The laparoscope aids diagnosis or therapeutic interventions with a few small cuts in the abdomen.Medl ...
and/or
hysteroscopy Hysteroscopy is the inspection of the uterine cavity by endoscopy with access through the cervix. It allows for the diagnosis of intrauterine pathology and serves as a method for surgical intervention (operative hysteroscopy). Hysteroscope A hyst ...
may be indicated. In some patients the
vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hy ...
l development may be affected.


Treatment

Surgical intervention depends on the extent of the individual problem. With a didelphic uterus surgery is not usually recommended. A uterine septum can be resected in a simple out-patient procedure that combines laparoscopy and hysteroscopy. This procedure greatly decreases the rate of miscarriage for women with this anomaly.


Prevalence

The prevalence of uterine malformation is estimated to be 6.7% in the general population, slightly higher (7.3%) in the infertility population, and significantly higher in a population of women with a history of recurrent miscarriages (16%).


See also

* – where some malformations in humans are "normal"


References


External links


BBC NEWS - Triplets for woman with two wombs
{{DEFAULTSORT:Uterine Malformation Congenital disorders of female genital organs