Anomalies in the development of female genital organs

Hereditary, exogenous, multifactorial factors lead to the appearance of abnormalities in the development of female genital organs. The occurrence of genital malformations is attributed to a critical period of intrauterine development . It is based on the lack of fusion of the caudal divisions of the para-mesonephric Muller ducts , deviations in the transformations of the urogenital sinus , as well as the pathological course of gonad organogenesis, which depends on the development of the primary kidney . These deviations account for 16% of all anomalies.

Anomalies in the development of the genital organs more often occur during the pathological course of pregnancy in the mother at different stages of pregnancy. These are early and late gestosis , infectious diseases , intoxication , endocrine disorders in the mother's body.

In addition, abnormalities in the development of female genital organs can occur under the influence of harmful environmental factors, occupational harmful effects in the mother, poisoning with toxic substances .

Along with genital abnormalities, in 40% of cases there are abnormalities of the urinary system ( unilateral agenesis of the kidney ), intestines ( atresia of the anus ), bones (congenital scoliosis ), as well as congenital heart defects .

1. Agenesis is the absence of an organ.
2. Aplasia is the absence of a part of the organ.
3. Atresia - the absence, underdevelopment or fouling of a channel or hole.
4. Heterotopy - the presence of cells of one kind or another in another organ.
5. Hyperplasia is an overgrowth of an organ.
6. Hypoplasia is a decrease in the organ.
7. Stenosis is a narrowing of the canal.

A complete absence of the uterus occurs with concomitant and other severe malformations:

Vaginal

The complete absence of the vagina ( aplasia vaginae ) is due to insufficient development of the lower sections of the muller passages. The uterus, fallopian tubes with aplasia are presented in the form of muscle and connective tissue cords - Mayer-Rokytansky-Kuster-Hauser syndrome . Aplasia of the vagina is accompanied by amenorrhea , sexual life is impossible.

Treatment . Vaginal aplasia is only treated surgically. To create an artificial vagina , a leather flap or a section of the sigmoid or rectum is used. Recently, an artificial vagina is created from the peritoneum of the posterior fornix.

Vaginal Atresia

Vaginal atresia is a complete fusion of the vagina. Symptoms and diagnosis are the same as with vaginal aplasia.

Ginatresia

Ginatresia is a violation of patency of the genital canal in the hymen ( atresia hymenalis ), vagina ( atresia vaginalis ), and uterus ( atresia uterina ).

Ginatresia occurs due to congenital defects of the Muller ducts, transmitted intrauterine infections (congenital atresia). Inflammatory processes transferred in childhood or adulthood ( diphtheria , sepsis ) can lead to scarring and closure of the lumen of one or another genital canal (acquired gynatresia).

Atresia of Hymen

Hymen atresia is detected only from the onset of puberty , when a lack of menstruation is detected. Blood released during menstruation accumulates in the vagina, stretching its walls (haematocolpos). The vagina overflowing with blood takes a tumor-like form, the upper pole of which, together with the uterus, is located above the plane of entry into the small pelvis .

With significant stretching of the vagina, symptoms of compression of the bladder and intestines, lower back pain, as well as cramping pains and a violation of the general condition on the days corresponding to menstruation can occur.

Diagnostics Recognizing atresia of the hymen is not difficult. On examination, a protrusion of the hymen of a bluish color (pressure and transmission of blood) is revealed. Rectoabdominal examination in the vagina reveals a tumor-like elastic formation, on top of which the uterus is located.

Treatment is reduced to a cruciform incision of the hymen and removal of the contents of the vagina. The operation is carried out in compliance with aseptic rules. In order to prevent repeated atresia, stitches should be applied to the edges of the incision.

Vaginal Atresia

Vaginal atresia can be localized in the upper, middle, or lower third of the vagina.

The main symptom is the absence of menstruation ( amenorrhea ). The accumulation of blood passes above the atresia, gradually filling and stretching the channel of the cervix and uterine cavity (haematometra). The uterus and tubes stretch, become thinner and take on an elastic consistency.

Uterine Atresia

With atresia of the uterus, menstrual blood lingers in the uterine cavity . The latter turns into a round, elastic form. Subsequently, blood enters the lumen of the tubes. When closing the abdominal openings of the fallopian tubes, a bilateral hematosalpinx occurs . The hematometer and hematosalpinx are dangerous, as they lead to rupture of the walls of the uterus or tube with the subsequent ingress of contents into the abdominal cavity , which can cause the occurrence of peritonitis .

The treatment is mainly surgical. The operation is carried out carefully, observing the rules of asepsis. With atresia of the vagina, excision of the overgrown space is performed. In the presence of extensive atresia, excision is completed with plastic surgery .

1. Doubling of the uterus and vagina occurs due to a violation of the process of connecting those sections of the Muller ducts from which the uterus and vagina form during normal embryogenesis .
2. Uterus didelphys - the presence of two independent genital organs of two uterus (each has one tube and one ovary ) of two necks, two vaginas. The uterus and vagina are located separately, between them are the bladder and rectum. Two halves can be developed satisfactorily or unevenly: a complete or partial absence of a cavity in one or both halves is possible. Pregnancy can occur in turn in each uterus. This type of anomaly does not require treatment.
3. Uterus duplex et vagina duplex - the presence of two queens, two necks and two vaginas. But, unlike the first form, both uterus are connected in a limited area, often in the cervical region, with a fibro-muscular septum. One of the queens is inferior in size and in functional terms. On a less developed uterus, there may be atresia of the uterine pharynx .

   With partial atresia of one vagina, blood accumulation is possible - haematocolpos lateralis. If there is a blockage in the upper part of the vagina, then infection with accumulated blood and the formation of an abscess in the atresized vagina are possible. If there is a cavity in the rudimentary horn of the uterus and aplasia of the vagina, menstrual blood accumulates and a haematometra is formed.

   A fertilized egg can enter the rudimentary horn from the fallopian tube.

   Pregnancy in the rudimentary horn proceeds as an ectopic pregnancy and is subject to surgical treatment.

4. Uterus bicornis - a two - horned uterus arises from the confluence of the paramesonephral passages. As a result, a common vagina is observed, and other organs are bifurcated. As a rule, organs on one side are less pronounced than on the other.

With a two-horned uterus, there may be two necks - uterus bicollis. In this case, the vagina has the usual structure or it may have a partial septum.

Sometimes with a two-horned uterus there can be one neck, which is formed from the complete fusion of both halves - uterus bicornis uncollis. Almost complete fusion of both horns is possible except for the bottom, where a saddle-shaped depression is formed - the saddle - shaped uterus (uterus arcuatus). In the saddle uterus there may be a septum that extends to the entire cavity, or a partial membrane in the area of ​​the bottom or cervix.

With the satisfactory development of one uterine horn and a pronounced rudimentary state of the other, a unicorn uterus, uterus unicornus, is formed.

The clinical picture . Doubling of the uterus and vagina can be asymptomatic. With a sufficiently satisfactory development of both or even one uterus, menstrual and sexual functions are not violated.

Pregnancy can occur in one or the other cavity of the uterus, the normal course of childbirth and the postpartum period is possible. If different degrees of doubling are combined with underdevelopment of the ovaries and uterus, there are symptoms characteristic of developmental delay (violation of menstrual, sexual and reproductive functions). Often there are spontaneous miscarriages , labor weakness, bleeding in the postpartum period. Hematokolpos and hematometer are accompanied by pain, fever. Palpation of the abdomen reveals a painless, shifting tumor.

Diagnostics Recognizing the doubling of the uterus and vagina in most cases is not difficult, this is done using conventional examination methods ( bimanual , examination using a mirror , sounding, ultrasound ). If necessary, metrosalpingography , laparoscopy are used.

Treatment . Doubling of the uterus and vagina is asymptomatic and does not require treatment.

In the presence of a septum in the vagina, which prevents the birth of the fetus , it is dissected.

In the presence of symptoms of delayed development of the genitals, cyclic hormone therapy is prescribed.

If blood accumulates in an atresized vagina or in a rudimentary horn, surgical treatment is required. If there are abnormalities of the uterus, surgical correction is performed - a metroplasty operation.

• V.L. Grishchenko, M.O. Shcherbina. Gіnekologіya - Pіdruchnik . - 2007.
• Grishchenko V., Vasilevskaya L.N., Scherbina N.A. Gynecology - A textbook for specialized universities. - Phoenix, 2009 .-- 604 p. - ISBN 978-5-222-14846-4 .
• Children's gynecology. - Medical News Agency, 2007. - 480 p. - ISBN 5-89481-497-9 .