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The occurrence of all types of paramesonephric duct abnormalities in women is estimated around 0.4%.
A bicornuate uterus is estimated to occur in 0.1-0.5% of women in the U.S.
It is possible that this figure is an underestimate, since subtle abnormalities often go undetected. Some intersex individuals whose external genitalia are perceived as being male may nonetheless have a variably shaped uterus.
Pregnancies in a bicornuate uterus are usually considered high risk and require extra monitoring because of association with poor reproduction potential.
A bicornuate uterus is associated with increased adverse reproductive outcomes, such as:
- Recurrent pregnancy loss
- Preterm birth: The rate of preterm delivery is 15 to 25%. A pregnancy may not reach full term in a bicornuate uterus when the baby begins to grow in either of the uterine horns. A short cervical length seems to be a good predictor of preterm delivery in women with a bicornuate uterus.
- Malpresentation (breech birth or transverse presentation): a breech presentation occurs in 40-50% of pregnancies with a partial bicornuate uterus and not at all (0%) in a complete bicornuate uterus.
- Deformity: Offspring of mothers with a bicornuate uterus are at high risk for "deformities and disruptions" and "malformations."
Previously, a bicornuate uterus was thought to be associated with infertility, but recent studies have not confirmed such an association.
In the United States, uterus didelphys is reported to occur in 0.1–0.5% of women. It is difficult to know the exact occurrence of this anomaly, as it may go undetected in the absence of medical and reproductive complications.
A number of twin gestations have occurred where each uterus carried its pregnancy separately. A recent example occurred on February 26, 2009, when Sarah Reinfelder of Sault Ste. Marie, Michigan delivered two healthy, although seven weeks premature, infants by cesarean section at Marquette General Hospital. It is possible that the deliveries occur at different times, thus the delivery interval could be days or even weeks.
Most studies of uterine malformations are based on populations of women who have experienced a pregnancy loss and thus do not address the issue of the prevalence in the general population. A screening study by Woelfer et al. of women without a history of reproductive problems found that about 5% of women had an arcuate uterus when they defined an arcuate uterus any fundal protrusion into the cavity that had an apical angle of more than 90 degrees. Accordingly, it was the most common uterine anomaly, followed by septate uterus (3%) and bicornuate uterus (0.5%).
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%).
Most studies are based on populations of women who have experienced a pregnancy loss and thus do not address the issue of the prevalence in the general population. A screening study by Woelfer et al. of women without a history of reproductive problems found that about 3% of women had a uterine septation; the most common anomaly in their study was an arcuate uterus (5%), while 0.5% were found to have a bicornuate uterus. In contrast, in about 15% of patients with recurrent pregnancy loss anatomical problems are thought to be causative with the septate uterus as the most common finding.
The condition may not be known to the affected individual and not result in any reproductive problems; thus normal pregnancies occur. Indeed, there is no consensus on the relationship of the arcuate uterus and recurrent pregnancy loss. Accordingly, the condition may be a variation or a pathology.
One view maintains that the condition is associated with a higher risk for miscarriage, premature birth, and malpresentation. Thus a study that evaluated women with uterine bleeding by hysteroscopy found that 6.5% of subjects displayed the arcuate uterus and had evidence of reproductive impairments. A study based on hysterosalpingraphic detected arcuate lesions documented increased fetal loss and obstetrical complications as a risk for affected women. Woelfer found that the miscarriage risk is more pronounced in the second trimester. In contrast, a study utilizing 3-D ultrasonography to document the prevalence of the arcuate uterus in a gynecological population found no evidence of increased risk of reproductive loss; in this study 3.1% of women had an arcuate uterus making it the most common uterine anomaly; this prevalence was similar than in women undergoing sterilization and lower than in women with recurrent pregnancy loss.
AS has a reported incidence of 25% of D&Cs performed 1–4 weeks post-partum, up to 30.9% of D&Cs performed for missed miscarriages and 6.4% of D&Cs performed for incomplete miscarriages. In another study, 40% of patients who underwent repeated D&C for retained products of conception after missed miscarriage or retained placenta developed AS.
In the case of missed miscarriages, the time period between fetal demise and curettage may increase the likelihood of adhesion formation due to fibroblastic activity of the remaining tissue.
The risk of AS also increases with the number of procedures: one study estimated the risk to be 16% after one D&C and 32% after 3 or more D&Cs. However, a single curettage often underlies the condition.
In an attempts to estimate the prevalence of AS in the general population, it was found in 1.5% of women undergoing hysterosalpingography HSG, and between 5 and 39% of women with recurrent miscarriage.
After miscarriage, a review estimated the prevalence of AS to be approximately 20% (95% confidence interval: 13% to 28%).
Women with the condition may be asymptomatic and unaware of having a uniconuate uterus; normal pregnancy may occur. In a review of the literature Reichman et al. analyzed the data on pregnancy outcome of 290 women with a unicornuate uterus. 175 women had conceived for a total of 468 pregnancies. They found that about 50% of patients delivered a live baby. The rates for ectopic pregnancy was 2.7%, for miscarriage 34%, and for preterm delivery 20%, while the intrauterine demise rate was 10%. Thus patients with a unicornuate uterus are at a higher risk for pregnancy loss and obstetrical complications.
Cervical agenesis is estimated to occur in 1 in 80,000 females. It is often associated with deformity of the vagina; one study found that 48% of patients with cervical agenesis had a normal, functional vagina, while the rest of the cases were accompanied by vaginal hypoplasia.
The extent of adhesion formation is critical. Mild to moderate adhesions can usually be treated with success. Extensive obliteration of the uterine cavity or fallopian tube openings (ostia) and deep endometrial or myometrial trauma may require several surgical interventions and/or hormone therapy or even be uncorrectable. If the uterine cavity is adhesion free but the ostia remain obliterated, IVF remains an option. If the uterus has been irreparably damaged, surrogacy or adoption may be the only options.
Depending on the degree of severity, AS may result in infertility, repeated miscarriages, pain from trapped blood, and future obstetric complications If left untreated, the obstruction of menstrual flow resulting from adhesions can lead to endometriosis in some cases.
Patients who carry a pregnancy even after treatment of IUA may have an increased risk of having abnormal placentation including placenta accreta where the placenta invades the uterus more deeply, leading to complications in placental separation after delivery. Premature delivery, second-trimester pregnancy loss, and uterine rupture are other reported complications. They may also develop incompetent cervix where the cervix can no longer support the growing weight of the fetus, the pressure causes the placenta to rupture and the mother goes into premature labour. Cerclage is a surgical stitch which helps support the cervix if needed.
Pregnancy and live birth rate has been reported to be related to the initial severity of the adhesions with 93, 78, and 57% pregnancies achieved after treatment of mild, moderate and severe adhesions, respectively and resulting in 81, 66, and 32% live birth rates, respectively. The overall pregnancy rate after adhesiolysis was 60% and the live birth rate was 38.9% according to one study.
Age is another factor contributing to fertility outcomes after treatment of AS. For women under 35 years of age treated for severe adhesions, pregnancy rates were 66.6% compared to 23.5% in women older than 35.
The uterus is normally formed during embryogenesis by the fusion of the two Müllerian ducts. If one of the ducts does not develop, only one Müllerian duct contributes to the uterine development. This uterus may or may not be connected to Müllerian structure on the opposite site if the Müllerian duct on that site undergoes some development. A unicornuate uterus has a single cervix and vagina.
Associated defects may affect the renal system, and less common, the skeleton.
The condition is much less common than these other uterine malformations: arcuate uterus, septate uterus, and bicornuate uterus. While the uterus didelphys is estimated to occur in 1/3,000 women, the unicornuate uterus appears to be even more infrequent with an estimated occurrence of about 1/4,000.
The uterus (womb) is normally held in place by a hammock of muscles and ligaments. Prolapse happens when the ligaments supporting the uterus become so weak that the uterus cannot stay in place and slips down from its normal position. These ligaments are the round ligament, uterosacral ligaments, broad ligament and the ovarian ligament. The uterosacral ligaments are by far the most important ligaments in preventing uterine prolapse.
The most common cause of uterine prolapse is trauma during childbirth, in particular multiple or difficult births. About 50% of women who have had children develop some form of pelvic organ prolapse in their lifetime. It is more common as women get older, particularly in those who have gone through menopause. This condition is surgically correctable.
The condition may not be known to the affected individual and not result in any reproductive problems; thus normal pregnancies may occur. However, it is associated with a higher risk for miscarriage, premature birth, and malpresentation. According to the classical study by Buttram there is a 60% risk of a spontaneous abortion, this being more common in the second than in the first trimester. However, there is no agreement on this number and other studies show a lower risk. Woelfer found that the miscarriage risk is more pronounced in the first trimester.
The condition is also associated with abnormalities of the renal system. Further, skeletal abnormalities have been linked to the condition.
Rarely, a sharply tilted uterus is due to a disease such as endometriosis, an infection or prior surgery. Although this may make it more challenging for the sperm to reach the egg, conception can still occur. A tipped uterus will usually right itself during the 10th to 12th week of pregnancy. Rarely (1 in 3000 to 8000 pregnancies) a tipped uterus will cause painful and difficult urination, and can cause severe urinary retention. Treatment for this condition (called "incarcerated uterus") includes manual anteversion of the uterus, and usually requires intermittent or continuous catheter drainage of the bladder until the problem is rectified or spontaneously resolves by the natural enlargement of the uterus, which brings it out of the tipped position. In addition to manual anteversion and bladder drainage, treatment of urinary retention due to retroverted uterus can require the use of a pessary, or even surgery, but often is as simple as having the pregnant mother sleep on her stomach for a day or two, to allow the retroverted uterus to move forward.
If a uterus does not right itself, it may be labeled "persistent".
Uterine prolapse is a form of female genital prolapse. It is also called pelvic organ prolapse or prolapse of the uterus (womb).
Risk factors for uterine prolapse include pregnancy, childbirth, chronic increases in intra-abdominal pressure such as lifting, coughing or straining, connective tissue conditions, and damage to or weakness of the muscles.
Treatment may be conservative or surgical and should be based upon patient symptoms and preference.
Hematometra develops when the uterus becomes distended with blood secondary to obstruction or atresia of the lower reproductive tract—the uterus, cervix or vagina—which would otherwise provide an outflow for menstrual blood. It is most commonly caused by congenital abnormalities, including imperforate hymen, transverse vaginal septum or vaginal hypoplasia. Other causes are acquired, such as cervical stenosis, intrauterine adhesions, endometrial cancer, and cervical cancer.
Additionally, hematometra may develop as a complication of uterine or cervical surgery such as endometrial ablation, where scar tissue in the endometrium can "wall off" sections of endometrial glands and stroma causing blood to accumulate in the uterine cavity. It can also develop after abortion, as well as after childbirth. It can also develop after female genital mutilation.
Diets high in fruits and vegetables tend to lower the risk of developing fibroids. Fruits, especially citrus, have a greater protective benefit than vegetables. Normal dietary levels of vitamin D is shown to reduce the risk of developing fibroids. No protective benefit has been found with the consumption of folate, whole grains, soy products, or fiber. No association between the consumption of fat, eggs, dairy products has been shown to increase the risk of fibroids.
In most cases, a retroverted uterus is genetic and is perfectly normal but there are other factors that can cause the uterus to be retroverted. Some cases are caused by pelvic surgery, pelvic adhesions, endometriosis, fibroids, pelvic inflammatory disease, or the labor of childbirth.
If untreated, the accumulation of menstrual fluid in the uterus caused by cervical agenesis can lead to hematocolpos, hematosalpinx, endometriosis, endometrioma and pelvic adhesions.
As the vagina 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, leading to a "double vagina". This condition may be associated with a uterus didelphys or a uterine septum. Since the condition is internal and usually asymptomatic, a person may not be aware of having a "double vagina." If necessary, the partition can be surgically corrected, however, there is no valid medical reason for such a procedure.
Fraser syndrome is a disorder that affects the development of the child prior to birth. Infants born with Fraser syndrome often have eyes that are malformed and completely covered by skin. Also the child is born with fingers and toes that are fused together along with abnormalities within the urine tract. As this disorder relates to vaginal atresia, infants born with Fraser syndrome are also born with malformations in their genitals.
The prognosis for vaginal atresia is one that is complicated. There are variations in patients' anatomic findings as well as an absence in consistent surgical techniques which makes it difficult to give a prognosis for this condition. Along with other conditions that give rise to an abnormal perineum (i.e. ambiguous genitalia and other various abnormalities that range from cloaca to urogenital sinus), individuals with vaginal atresia often report reconstruction as an outcome of treatment. Due to this, it is difficult to compare outcomes between individuals with vaginal atresia.
The cause of this condition is not completely understood. Potential risk factors may include defects in the egg, abnormalities within the uterus, or nutritional deficiencies. Women under 20 or over 40 years of age have a higher risk. Other risk factors include diets low in protein, folic acid, and carotene. The diploid set of sperm-only DNA means that all chromosomes have sperm-patterned methylation suppression of genes. This leads to overgrowth of the syncytiotrophoblast whereas dual egg-patterned methylation leads to a devotion of resources to the embryo, with an underdeveloped syncytiotrophoblast. This is considered to be the result of evolutionary competition with male genes driving for high investment into the fetus versus female genes driving for resource restriction to maximise the number of children.