Vaginal and uterine malformations

Malformations of the internal genital organs are congenital disorders 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 anomalies (malformations) of the body and cervix of the uterus.
• Q51.0 Agenesis and aplasia of uterus, including congenital absence of uterus.
• Q51.1 Duplication of the body of the uterus with duplication of the cervix and vagina.
• Q51.2 Other duplications of uterus.
• Q51.3 Bicornuate uterus.
• Q51.4 Unicornuate uterus.
• Q51.5 Agenesis and aplasia of the cervix (congenital absence of the cervix).
• Q51.8 Other congenital malformations of body and cervix uteri (hypoplasia of body and cervix uteri).
• Q51.9 Congenital malformations of body and cervix uteri, unspecified genesis.
• Q52 Other congenital anomalies (malformations) of female genital organs.
• Q52.0 Congenital absence of vagina.
• Q52.1 Duplication of vagina (septate vagina), except duplication of vagina with duplication of body and cervix uteri, specified under Q51.1.
• Q52.3 The hymen, which completely covers the entrance to the vagina.
• Q52.8 Other specified congenital malformations of female genital organs.
• Q52.9 Congenital malformations of female genital organs of unspecified genesis.

Epidemiology of uterine bleeding in puberty

Defects in the development of female genital organs account for 4% of all congenital developmental anomalies and occur in 3.2% of women of reproductive age. According to E.A. Bogdanova (2000), among girls with pronounced gynecological pathology, 6.5% have abnormalities in the development of the vagina and uterus. Defects in the development of the genitourinary system occupy fourth place (9.7%) in the structure of all developmental anomalies of modern man. In the last 5 years, a 10-fold increase in the frequency of defects in the development of genital organs in girls has been noted. The most common abnormalities in girls during adolescence are atresia of the hymen, aplasia of the lower vagina, and duplication 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 menstrual blood flow. The incidence of aplasia of the vagina and uterus is 1 case per 4,000-5,000 newborn girls.

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Causes and pathogenesis of malformations of the vagina and uterus

To date, it has not been established exactly what exactly underlies the occurrence of malformations of the uterus and vagina. However, the role of hereditary factors, biological inferiority of cells that form the genitals, and the impact of harmful physical, chemical, and biological agents is undeniable.

The occurrence of various forms of malformations of the uterus and vagina depends on the pathological influence of teratogenic factors or the implementation of hereditary traits in the process of embryogenesis.

What causes vaginal and uterine malformations?

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Symptoms of vaginal and uterine malformations

During puberty, malformations of the vagina and uterus appear, in which primary amenorrhea and/or pain syndrome are observed: aplasia of the vagina and uterus, atresia of the hymen, aplasia of all or part of the vagina with a functioning uterus.

Girls with vaginal and uterine aplasia have a characteristic complaint - absence of menstruation, and later - impossibility of sexual activity. In some cases, in the presence of a functioning rudimentary uterus on one or both sides of the small pelvis, cyclical pains in the lower abdomen may occur.

Patients with atresia of the hymen in puberty complain of cyclic pain, a feeling of heaviness in the lower abdomen, and sometimes difficulty urinating. The literature provides many examples of dysfunction of adjacent organs in girls with atresia of the hymen and the formation of a large hematocolpos.

Symptoms of vaginal and uterine malformations

Classification of malformations of the vagina and uterus

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

In the practice of gynecologists of children and adolescents, the classification of E.A. Bogdanova and G.N. Alimbaeva (1991) is most often used, which examines defects that clinically manifest themselves in puberty.

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

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Screening for uterine bleeding during puberty

The risk group for the birth of girls with developmental defects should include women who have occupational hazards and bad habits (alcoholism, smoking), and women who have had viral infections during pregnancy from 8 to 16 weeks. And girls born to such women are subject to screening.

Diagnosis of malformations of the vagina and uterus

Step-by-step diagnostics include a thorough study of the anamnesis, gynecological examination (vaginoscopy and rectoabdominal examination), ultrasound and MRI of the pelvic organs and kidneys, endoscopic methods.

Diagnosis of uterine and vaginal malformations presents significant difficulties. According to research data, inadequate surgical treatment before admission to a specialized hospital is performed in 37% of girls with genital malformations with impaired menstrual blood flow, and unjustified operations or conservative treatment - in every fourth patient with vaginal and uterine aplasia.

Diagnosis of malformations of the vagina and uterus

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Treatment of malformations of the vagina and uterus

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

Indication for hospitalization is the patient's consent to conservative or surgical correction of a developmental defect of the uterus and vagina.

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

The so-called bloodless colpopoiesis is used only in patients with vaginal and uterine aplasia by using colpoelongators. When performing colpoelongation according to Sherstnev, an artificial vagina is formed by stretching the mucous membrane of the vaginal vestibule and deepening the existing or formed during the procedure "pit" in the vulva area using a protector (colpoelongator). The patient regulates the degree of pressure of the device on the tissue with a special screw, taking into account her own sensations. The patient performs the procedure independently under the supervision of medical personnel.

Treatment of malformations of the vagina and uterus

Prognosis of uterine bleeding during puberty

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

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