Double uterus

A double uterus is a very rare congenital disorder. We are talking about a malformation of the genital organ, which in the course of its development becomes paired, as a result of embryogenetic non-fusion of the Müllerian ducts. A double uterus has two separate cervixes and sometimes even a double vagina: each of the uterus is equipped with one horn connected to the fallopian tube, "looking" at the corresponding ovary.

Women with a double uterus do not always guess about their "peculiarities", since the anomaly may not manifest clinically, and pregnancy is quite likely. If the violation creates any problems with reproductive health, then they resort to surgery - the only way to correct the pathology.

Epidemiology

Double uterus refers to congenital defects in the development of the reproductive mechanism. Such a violation occurs as a result of the complete non-connection along the midline of the Müllerian ducts, which leads to the formation of two separate uterine organs with a vaginal septum. This anomaly is considered rare: the frequency of distribution ranges from 1: 1000 to 1: 30,000 (according to statistics collected in the United States of America, the prevalence is 1 in 3 thousand women).

Each of the formed uterus is connected to one fallopian tube and the corresponding ovary.

The onset of twin pregnancy, in which the fetus develops in a separate uterus, is a particularly rare case in patients diagnosed with a double uterus, with a frequency of 1: 1 million.

The onset of pregnancy with a double uterus is considered quite likely, provided there are no other reproductive disorders. According to statistics, infertility is observed in 12-30% of patients, and the frequency of miscarriage varies within 30-80%, with the frequency of premature births about 28 percent. It has not yet been clarified whether such indicators are only a consequence of an organ abnormality, or a double uterus is combined with morphofunctional insufficiency and a malfunction of the hypothalamic-pituitary-ovarian hormonal mechanism, as well as a disorder of the formation of the fetoplacental system.

Gestosis during pregnancy is found in about 10% of women with a double uterus, the abnormal position of the fetus was determined in 15-20% of cases. There is also a high incidence of surgical delivery (caesarean section) - in 45% of cases. Fetal hypotrophy was detected in 27%, low weight - in 15% of cases.

According to experts, women with an anomaly during pregnancy often have high blood pressure, and preeclampsia occurs 2 times more often than other pregnant women with a normal uterus.

Causes double uterus

The urogenital system as a whole is formed during the prenatal period from a single embryonic link, so any violation in it can become the primary cause of the formation of several defects at once. For example, a double uterus is often found in combination with other congenital diseases of the genitourinary system. A similar problem can appear in the following cases:

• with genetic disorders;
• in severe pregnancy (prolonged threatening miscarriage, gestosis, lack of nutrients, intrauterine infection of the fetus);
• prolonged intoxication during pregnancy (for example, medication, occupational, etc.);
• complex systemic diseases in the expectant mother.

In many cases, the true cause of the violation cannot be established. 

Hazel Johnson and the double uterus

Such an anomaly as a double uterus has always existed. However, close attention to the problem appeared after the publication of the story of Hazel Johnson, a woman from High Wycombe (UK), who was found to have a full duplication. Hazel took part in a popular program on the ITV channel, where she told the whole country about her peculiarity. She indicated, among other things, that she feels quite comfortable and practically does not suffer from the anomaly. The only problem is the beginning of the monthly cycle, which is harder than it should have been normal.

The doctors examined the patient and concluded that there was no need for surgical correction of the defect in Hazel. Nevertheless, if a woman wants to become pregnant, then she will need to be constantly monitored by specialists - in particular, due to the small size of the double internal genital organs. Delivery by cesarean section is recommended.

Risk factors

Particularly common risk factors are:

• exposure to occupational hazards during a woman's pregnancy;
• bad habits (alcohol consumption, smoking, drug addiction);
• viral infections during pregnancy (flu, toxoplasmosis, rubella);
• intoxicating effects of medicines.

There is evidence of a possible genetic predisposition to the formation of anomalies of the reproductive organs. So, it was found that the risk of developing a double uterus increased in families in which cases of other malformations were previously recorded - for example, kidney doubling, hypoplasia, etc.

Risk factors during pregnancy are:

• acute respiratory viral infections in the first trimester of pregnancy;
• taking hormonal drugs or non-steroidal anti-inflammatory drugs, salicylates;
• gestosis.

Women at risk are required to conduct thorough prenatal diagnostics, the introduction of rational tactics of pregnancy management, and timely detection of violations.

Pathogenesis

In the pathogenetic aspect, several types of development of a double uterus are distinguished:

• Full doubling, in which there are both two uterine organs and two vaginas that are not connected to each other.
• Incomplete doubling, when there are two uterine organs and two vaginas, separated in a certain place by a muscular-fibrous membrane.
• Full-length doubling with one vagina, in which there are two uterine organs and two necks, but one vagina.
• Uterine doubling with one neck and vagina.
• Uterine doubling, in which there is one full-fledged organ and one rudimentary (underdeveloped).
• Bicornuate uterus, partially divided into two zones.
• Saddle uterus with a deformed bottom without organ division.
• One uterus, divided by a septum (partially or completely).

A two-horned reproductive organ with a partially overlapping inner membrane (the so-called uterus subseptis) is a division into two halves that communicate at the neck. Diaphragm sizes can vary. This defect is associated with insufficient reabsorption of the junction zone of the Müllerian ducts.

In the presence of a complete septal membrane (uterus septis), the entire internal uterine cavity is fenced off, two parts isolated from each other are formed - from the bottom to the internal pharynx.

The doubled (divided) body and the common neck (uterus bicollis unicollis) are a pair of separated uterine cavities that have a common cervical canal.

The duplicated body with atrophic or atretic changes in one horn must be distinguished from a congenital anomaly in the development of the uterine part and post-traumatic fusion of its cavity.

The formation of a two-horned uterus with an atretic internal cavity is due to the non-fusion of the Müllerian ducts and the fusion of their lumen. The reproductive organ itself has a powerful structure, or has a separate small cavity in the area of one horn. [1]

Symptoms double uterus

Most patients with a double uterus do not show any pathological signs: they have a normal and regular monthly cycle, however, sometimes menstrual bleeding is more profuse. With a full doubling of the organ and a double vagina, problems appear in the intimate sphere. [2]

In some women, manifestations are found a little later - in particular, disorders associated with conceiving and bearing a child may occur:

• threatening miscarriage throughout the gestation period;
• spontaneous termination of pregnancy at an early stage;
• late miscarriages;
• the formation of habitual miscarriage;
• premature birth;
• infertility.

With a rudimentary second uterine organ, problems may arise associated with a deterioration in the outflow of menstrual blood:

• pain in the lower abdomen, worse during menstruation;
• an increase in the abdomen, a feeling of pressure and bloating.

If the rudimentary part is connected to the neck, then the appearance of such symptoms is possible:

• bleeding a few days before menstruation and for several days after the end of menstruation;
• profuse menstrual bleeding. 

In such a situation, the risk of developing an ectopic pregnancy increases. In addition, the formation of genital endometriosis is possible, which is accompanied by the following symptoms:

• spotting in the middle of the monthly cycle;
• growing weakness, fatigue;
• algodismenorrhea;
• pelvic pain;
• problems with the onset of pregnancy;
• hypermenorrhea;
• irregular monthly cycle;
• pain and uncomfortable sensations during and after sexual intercourse.

Often, the patient does not even suspect that she has such a feature - a double uterus. A woman lives an ordinary life, gets married, becomes pregnant, gives birth to a child. In most cases, everything proceeds without any specific symptoms. Difficulties can appear if we are talking not only about a double uterus, but also about a double vagina.

In some patients, too abundant and especially uncomfortable periods are noted: such a violation may serve as a reason for contacting a doctor, where a developmental anomaly is found.

The altered configuration of an organ with a double uterus can negatively affect the functionality of other nearby organs: a woman feels that something is wrong with her. Discomfort can be both physical (pain in the abdomen, feeling of fullness and pressure), and psychological (if the patient knows about her own characteristics). For many people, a double uterus is mistakenly associated with female inferiority, impairment, impossibility of motherhood and the creation of a family. The psychological mood of the patients is very important for conception: if you set yourself up for failure in advance, then conception may not take place (as, indeed, in women with an ordinary uterus). Problems with pregnancy also appear in the presence of concomitant disorders - for example, ovarian hypofunction, hormone deficiency, etc. A double uterus is a rare pathology, but even less often it happens that one or both uterus is underdeveloped. 

Double uterus and pregnancy

A double uterus in most cases is not an obstacle to conceiving a child - but only on condition that there are no defects on the part of other reproductive organs.

Having become pregnant, a woman may face such problems:

• spontaneous termination of pregnancy;
• premature delivery;
• atypical location of the fetus;
• profuse postpartum bleeding.

Most often, with a double uterus, only one of the uterine organs is fully ready for conception and gestation, and the second has slightly weaker characteristics - it can be attributed to rudimentary organs. It is noteworthy that with the development of pregnancy, this "rudiment" also begins to increase, which occurs until about the fifth month, which is associated with increased hormonal activity.

For most patients, a double uterus does not pose any danger, does not impair fertility, and does not require medical intervention. However, pregnancy should proceed under regular supervision by a physician in order to avoid complications and disturbances in the gestation process.

If spontaneous abortion occurs, then emergency curettage of both the first and second organs is performed. [3]

In isolated cases, pregnancy was noted in two uterus at the same time: in such situations, a woman first gave birth to one baby, and only a few weeks later - a second.

The need for medical termination of pregnancy is said in such cases:

• with improper attachment of the embryo (for example, to the inter-uterine septum);
• with hypoplasia of the uterine endometrium;
• with cervical insufficiency;
• when the embryo develops in a rudimentary organ unsuitable for implantation. [4]

Complications and consequences

The presence of a double uterus sometimes leads to diagnostic errors. As a result, the wrong treatment is prescribed, including unreasonable surgical interventions, such as appendectomy, tubectomy, removal of appendages, bougienage of the cervical canal and vagina.

Other unpleasant consequences can be:

• difficulties with intimacy (discomfort, etc.);
• accumulation of menstrual blood in the rudimentary part of the uterus;
• infectious processes (the formation of purulent cavities in the internal genital organs);
• difficulties with bearing a child (spontaneous abortion, premature birth);
• difficulty conceiving (infertility).

Diagnostics double uterus

The main diagnostic methods to detect a double uterus are as follows:

• ultrasound examination (preferably transvaginal);
• hysteroscopy, hysterosalpingography;
• Magnetic resonance imaging;
• laparoscopy.

The first stage of diagnosis usually includes more affordable procedures: ultrasound and magnetic resonance imaging. But endoscopy in the form of laparo and hysteroscopy is appropriate if it is necessary to combine the diagnosis and treatment of the disease. In this case, it is possible not only to detect the anomaly, but sometimes to remove the non-functioning rudimentary horn.

Instrumental diagnostics in the form of ultrasound and magnetic resonance imaging are considered the most informative, safe and affordable. They do not give radiation exposure to the body, but allow you to determine the exact anatomical changes in the reproductive organs. With full doubling during MRI diagnostics, two isolated uterine organs are visualized, from each of them there is a fallopian tube with an ovary, two isolated necks and two vaginas (the vaginal septum is complete). The two cervix and vagina have close wall contact. Both uterine organs and the vagina are separated from each other by the bladder and / or rectum, or touch each other by the walls. The double uterus can be completely anatomically and physiologically complete, or have an underdeveloped other half. Using T2-weighted viewing, it is possible to distinguish between layers of the uterus, depending on the intensity of the signal:

1. The central hyperintense layer corresponding to the endometrium and mucous tissue that lines the intrauterine cavity.
2. A narrow interlayer adjacent to the central layer, designated as a transition area.
3. The outer layer is the myometrium, which has an average signal intensity.

As additional studies, the patient is prescribed laboratory tests:

• general blood analysis;
• general urine analysis;
• coagulogram;
• biochemical blood test (urea, creatinine, total protein, glucose);
• hormonal research.

The need for treatment is determined, first of all, by the presence of complaints from the patient. The doctor needs to get information about the quality of a woman's sex life, whether there have been attempts to get pregnant, whether there have been other problems with the urogenital system (diseases, surgery, abortion, miscarriages, etc.). The quality of menstrual function is necessarily analyzed, the following questions are clarified:

• the period of the onset of the first menstruation (at what age);
• regularity of the monthly cycle;
• profuse bleeding;
• soreness at the beginning of menstruation;
• cycle time;
• the presence of vaginal discharge in the middle of the cycle.

In addition, an examination is performed in a gynecological chair, a two-handed vaginal examination (it is necessary for palpation to determine the size of the internal genital organs, their ratio, the condition of the ligaments, the mobility of the appendages, pain, etc.). [5]

Differential diagnosis

Today there are many modern methods of visualization of internal organs. But, despite this, the diagnosis of a double uterus can be difficult, which entails an erroneous recognition of pathology. According to statistics, incorrect diagnoses and, accordingly, incorrect prescription of therapy for a double uterus leads to unjustified surgical interventions in about 30% of cases. To avoid this, experts recommend, if organs are suspected of doubling, perform mandatory MRI, which allows more accurate differentiation of diseases and provides much more information about the existing pathology.

The greatest difficulties in differential diagnosis arise with such types of uterine anomalies as full doubling, two-hornedness, the presence of a septum and a saddle uterus.

Hysteroscopy and hysterosalpingography can suggest an anomaly. But these methods can not always be used - first of all, because of their invasiveness: such procedures are not used in relation to children and young girls who have not previously had sexual intercourse. In addition, hysteroscopy and hysterosalpingography provide a picture of only the internal contours of the organ cavity, and this information is not enough for differential diagnosis. The outer contour can be seen with laparoscopy, but this method is also invasive. [6]

Among the non-invasive techniques for reliable interpretation of pathology, ultrasound and magnetic resonance imaging are used, which make it possible to evaluate both the internal and external uterine contours. Since it is more optimal to carry out transvaginal ultrasound, this procedure will be contraindicated in relation to children and girls before the onset of sexual activity. Therefore, preference is often given to MRI, with the analysis of the configuration in the T2-weighted image in the standard plane (coronal, drawn along the axis of the uterine body). For further differentiation, the interstitial parts of the fallopian tubes are used as control points to draw a line between them.

Treatment double uterus

There is no need for treatment if the double uterus does not cause problems in reproductive, sexual and menstrual functions, does not have complications and disorders from other organs. A special approach to treatment is required if the anomaly is accompanied by the accumulation of menstrual blood in the cavities of the genital organs. This pathology is characterized by severe pain, especially with the onset of a new monthly cycle. Some women develop infectious complications in the form of the formation of purulent inflammatory foci.

Surgical treatment is necessary if the patient has problems in the sexual aspect, impaired conception and gestation. The type and complexity of the intervention depends on the specific defect and its degree. In this case, the operation is the only way to correct the pathology. Surgeons usually use minimally invasive techniques, including laser technology and coagulation. One of the most common operations is hysteroresectoscopy, during which the doctor removes the septum that divides the organ into two parts.

In general, they have the following indications for surgical intervention:

• an abnormal structure of the vagina that interferes with normal sex life;
• closed rudimentary second uterus;
• an underdeveloped second uterus, with a high degree of probability of developing an ectopic pregnancy in it;
• habitual miscarriages;
• uterine septum;
• a combination of a double uterus and other urogynecological anomalies and pathologies.

In case of disturbances in the outlet of menstrual blood, the vaginal walls are dissected, contact is formed between the "working" and the closed cavity, the focus of accumulation is opened and drained, and vaginal sanitation is performed. During laparoscopy, the uterine localization is examined, a procedure is performed to empty the focus, and the abdominal cavity is sanitized.

Vaginal aplasia is an indication for the use of colpoelogation (bougienage) and colpopoiesis (artificial formation of the vaginal canal from the tissue of the uterine-rectal serous bursa).

If a woman has a thin intrauterine septum, then it is optimal to perform the Tompkins operation, which allows to achieve the formation of a good uterine cavity. [7]

An incomplete but dense septum is an indication for Jones surgery. In order to create a single cavity in the organ, the surgeon performs a partial wedge-like excision of the membrane, after which he dissects the rest of its parts. As a result, a sufficient volume of the uterine cavity is created while maintaining the endomyometrium.

A bicornuate uterus with an underestimated horny fusion is an indication for Strassmann's operation, and in case of an overestimated or medium fusion, the uterine body is dissected under the zone of the confluence of the horns, with further opening of the cavities of the first and second horns. This method of intervention facilitates recovery and reduces the trauma of the operation.

With full doubling, a two-stage operation is performed, consisting of the following stages:

• dissection of the vaginal septum and the formation of a single uterine cervix;
• plastic surgery (metroplasty).

The development of isthmic-cervical insufficiency can become a complication of such an operation.

Prevention

There is no specific prevention of the development of a double uterus. You can limit the risk of an anomaly if you carefully prepare for pregnancy and follow all the doctor's recommendations during the entire period of gestation.

Experts talk about the following preventive measures:

• visit a gynecologist regularly (twice a year);
• plan and prepare in a timely manner for pregnancy (be fully examined, treat existing chronic diseases, infections);
• prevent the development of unwanted pregnancy, exclude abortion;
• timely register for pregnancy with an obstetrician-gynecologist (earlier than 13 weeks of gestation);
• during pregnancy, visit a doctor regularly (optimally - once every 7-14 days, if indicated - more often), follow his recommendations;
• give up bad habits, eat normally and fully, do not self-medicate, avoid intoxication.

It is ideal if the couple seeks a doctor's advice at the stage of pregnancy planning. In such a situation, the doctor has the opportunity to conduct the necessary examinations, draw up an individual scheme of preventive measures necessary to create optimal conditions for the maturation of the egg, its implantation and the development of the embryo.

Forecast

Women with different types of double uterus often have concomitant gynecological and extragenital pathologies that complicate the prognosis of the anomaly. Experts talk about a relatively high incidence of infertility and miscarriage.

The most prognostically unfavorable type of pathology is considered to be a two-horned uterus with the confluence of the horns in the middle and in the lower third, as well as the presence of an intrauterine septum. With these defects, the greatest likelihood of infertility, recurrent miscarriage and premature placental abruption is noted. With a rudimentary second uterus, an ectopic pregnancy often develops, a delay in fetal development or an abnormal position of the fetus is found. The birth of premature and low birthweight babies requires emergency resuscitation and a long recovery period.

With an intrauterine septum, a two-horned or saddle organ, the risk of developing isthmic-cervical insufficiency in pregnant women increases.

To improve the prognosis, early detection of anomalies, dispensary observation of patients during the planning of conception and throughout pregnancy is recommended. In order to detect probable combined defects in the development of the urogenital system, all women with a double uterus should undergo an ultrasound of the kidneys. During pregnancy, it is recommended to place the expectant mother in a hospital during periods of critical periods: from 8 to 12 weeks, from 16 to 18 weeks, from 26 to 28 weeks.

There is no need to perform curettage of the second non-pregnant organ after childbirth. On the fourth day, an ultrasound scan is performed: if a hematometer is detected, a vacuum aspiration procedure is performed. [8]

Nevertheless, for many patients, the double uterus does not become an obstacle, either for a full sexual life, or for the conception and birth of a baby. However, careful gynecological observation during pregnancy should still be mandatory.