A uterine malformation is a type of female genital malformation 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 fal ...

(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 spe ...

. 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 menses ...

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, and pain, to normal functioning depending on the nature of the defect.

Types

The American Fertility Society (now American Society of Reproductive Medicine) 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 miss ...

(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

. ; Class II—

Unicornuate uterus A unicornuate uterus represents a uterine malformation where the uterus is formed from one only of the paired Müllerian ducts while the other Müllerian duct does not develop or only in a rudimentary fashion. The sometimes called ''hemi-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 Uterus didelphys (sometimes also ''uterus didelphis'') represents a uterine malformation where the uterus is present as a paired organ when the embryogenetic fusion of the Müllerian ducts fails to occur. As a result, there is a double uterus wi ...

, 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 A bicornuate uterus or bicornate uterus (from the Latin ''cornū'', meaning "horn"), is a type of mullerian anomaly in the human uterus, where there is a deep indentation at the fundus (top) of the uterus. Pathophysiology A 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 (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 biochemica ...

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 Des is a masculine given name, mostly a short form (hypocorism) of Desmond. People named Des include: People * Des Buckingham, English football manager * Des Corcoran, (1928–2004), Australian politician * Des Dillon (disambiguation), sever ...

uterus.

: The uterine cavity has a "

T-shape Many shapes have metaphorical names, i.e., their names are metaphors: these shapes are named after a most common object that has it. For example, "U-shape" is a shape that resembles the letter U, a bell-shaped curve has the shape of the vertical ...

" 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 ...

. An additional variation is the arcuate uterus 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 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 cavi ...

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 hymen ...

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 Uterus didelphys (sometimes also ''uterus didelphis'') represents a uterine malformation where the uterus is present as a paired organ when the embryogenetic fusion of the Müllerian ducts fails to occur. As a result, there is a double uterus wi ...

or a uterine septum.=

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 rec ...

, 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.Medlin ...

and/or hysteroscopy may be indicated. In some patients the

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%).

References

External links

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