Malformations of the vagina and uterus

Malformations of internal genital organs are a congenital disorder of the shape and structure of the uterus and vagina.

Synonyms: anomalies or malformations of the development of the vagina and uterus.

ICD-10 code

• Q51 Congenital malformations (malformations) of the body and cervix.
• Q51.0 Aging and aplasia of the uterus, including congenital absence of the uterus.
• Q51.1 Doubling the uterus body with doubling the cervix and vagina.
• Q51.2 Other duplications of the uterus.
• Q51.3 Double-breasted uterus.
• Q51.4 The one-horned womb.
• Q51.5 Cervical aegesis and aplasia (congenital absence of the cervix).
• Q51.8 Other congenital anomalies of the body and cervix (hypoplasia of the body and cervix).
• Q51.9 Congenital anomalies of the body and cervix of unspecified genesis.
• Q52 Other congenital malformations (malformations) of female genital organs.
• Q52.0 Congenital absence of the vagina.
• Q52.1 Doubling the vagina (divided by a vaginal septum), with the exception of doubling the vagina with doubling of the body and cervix, marked Q51.1.
• Q52.3 The hymen, completely covering the entrance to the vagina.
• Q52.8 Other specified anomalies of female genital organs.
• Q52.9 Congenital anomalies of female genital organs of unspecified genesis.

Epidemiology of uterine bleeding in the pubertal period

Malformations of female genital organs account for 4% of all congenital anomalies of development and are found in 3.2% of women of reproductive age. According to E.A. Bogdanova (2000), among girls with severe gynecological pathology, anomalies in the development of the vagina and uterus are revealed in 6.5%. The developmental defects of the urino-genital system occupy the fourth place (9.7%) in the structure of all the anomalies of modern human development. In the last 5 years there has been a 10-fold increase in the frequency of malformation of the genital organs in girls. Most often in the adolescent period, girls detect atresia of the hymen, aplasia of the lower parts of the vagina and doubling of the vagina and uterus with partial or complete aplasia of one of the vaginas, as well as aplasia of the uterus and vagina (Rokitansky-Küster-Mayer syndrome) and defects associated with impaired outflow of menstrual blood. The incidence of aplasia of the vagina and uterus is 1 case per 4000-5000 newborn girls.

[1], [2]

Causes and pathogenesis of malformations of the vagina and uterus

Until now, it has not been established exactly what exactly is the origin of the developmental defects of the uterus and vagina. However, the role of hereditary factors, the biological inferiority of cells that form sexual organs, the effects of harmful physical, chemical and biological agents is undoubtedly important.

The emergence of various forms of malformations of the uterus and vagina depends on the pathological effect of teratogenic factors or the realization of hereditary traits in the process of embryogenesis.

What causes malformations of the vagina and uterus?

[3], [4], [5], [6], [7], [8]

Symptoms of malformations of the vagina and uterus

In the pubertal period, vaginal and uterine malformations are manifested, in which primary amenorrhea and / or pain syndrome are observed: vaginal and uterine aplasia, hymen atresia, aplasia of the whole or part of the vagina with the functioning uterus.

In girls with aplasia of the vagina and uterus, a characteristic complaint is the absence of menstruation, in the future - the impossibility of sexual activity. In some cases, in the presence of a functioning rudimentary uterus from one or both sides in the small pelvis, cyclic pain in the abdomen may occur.

Patients with atresia of the hymen at puberty age complain of cyclical pains, a feeling of heaviness in the lower abdomen, sometimes difficulty in urinating. In the literature, there are many examples of violations of the function of neighboring organs in girls with atemia of the hymen and the formation of large hematocolpos.

Symptoms of malformations of the vagina and uterus

Classification of malformations of the vagina and uterus

Currently, there are many classifications of malformations of the vagina and uterus, based on differences in embryogenesis of internal genital organs.

In the practice of gynecologists of children and adolescents, the classification of E.A. Bogdanova and GN. Alimbaeva (1991), which deals with vices, clinically manifested in puberty.

• Class I - atresia of the hymen (variants of the structure of the hymen).
• Class II - complete or incomplete aplasia of the vagina and uterus:
  • complete aplasia of the uterus and vagina (Rokitansky-Küster-Meyer-Hauser syndrome);
  • complete aplasia of the vagina and cervix with a functioning uterus;
  • complete vaginal aplasia with the functioning uterus;
  • partial vaginal aplasia to the middle or upper third with a functioning uterus.
• Class III - defects associated with lack of fusion or incomplete fusion of paired embryonic genital ducts:
  • complete doubling of the uterus and vagina;
  • doubling of the body and cervix in the presence of one vagina;
  • doubling of the uterus body with one cervix and one vagina (saddle uterus, or bicornic uterus, or uterus with full or incomplete internal septum, or uterus with rudimentary functioning closed horn).
• Class IV - defects associated with a combination of doubling and aplasia of paired embryonic genital ducts:
  • doubling of the uterus and vagina with partial aplasia of one vagina;
  • doubling of the uterus and vagina with complete aplasia of both vaginas;
  • doubling of the uterus and vagina with partial aplasia of both vaginas; o doubling the uterus and vagina with complete aplasia of the entire duct on one side (unicorn uterus).

[9], [10], [11], [12]

Screening uterine bleeding puberty period

Women with occupational hazards and bad habits (alcoholism, smoking), and women who have transferred viral infections during pregnancy from 8 to 16 weeks should be included in the risk group for the birth of girls with malformations. And girls born of such women are subject to screening.

Diagnosis of malformations of the vagina and uterus

Gradual diagnosis includes a thorough examination of the anamnesis, gynecological examination (vaginoscopy and recto-abdominal examination). Ultrasound and MRI of the pelvic organs and kidneys, endoscopic methods.

Diagnosis of malformations of the uterus and vagina presents significant difficulties. According to the research, inadequate surgical treatment before admission to a specialized hospital is performed in 37% of girls with malformations of the genitals with a violation of outflow of menstrual blood, and unreasonable operations or conservative treatment - for every fourth patient with aplasia of the vagina and uterus.

Diagnosis of malformations of the vagina and uterus

[13], [14], [15]

Treatment of malformations of the vagina and uterus

The goal of the treatment is the creation of an artificial vagina in patients with aplasia of the vagina and uterus or outflow of menstrual blood in patients with delay.

Indications for hospitalization - the patient's consent to a conservative or surgical correction of the development of the uterus and vagina.

Medicamentous treatment for malformations of the uterus and vagina is not used.

The so-called bloodless colpopoiesis is used only in patients with aplasia of the vagina and uterus by using colpelotongators. When carrying out colpelongation according to Sherstnev, the artificial vagina is formed by stretching the mucous vestibule of the vagina and deepening the existing or formed "fossa" in the vulva with the help of a tread (colpoelonator). The degree of pressure of the apparatus on the tissue of the patient is regulated by a special screw taking into account its own sensations. The patient conducts the procedure independently under the supervision of medical personnel.

Treatment of malformations of the vagina and uterus

Forecast uterine bleeding pubertal period

With timely access to a gynecologist in a qualified gynecological department, equipped with modern diagnostic and surgical equipment, the prognosis of the course of the disease is favorable. Patients with aplasia of the vagina and uterus in the conditions of development of methods of assisted reproduction have the opportunity to use the services of surrogate mothers under the program of in vitro fertilization and embryo transfer.

[16], [17], [18], [19]