The most common doubling options:
• doubling of the uterus and vagina;
• doubling of the uterus and vagina with partial aplasia of one vagina;
• two-horned uterus;
• uterus with an additional closed functioning horn;
• saddle uterus;
• uterus with a septum (complete or incomplete).
Clinical symptoms. With a complete doubling of the uterus and vagina complaints there are no patients, and the malformation is detected by chance with an ultrasound scan or in connection with any surgical intervention. In patients with complete doubling of the uterus and vagina and partial aplasia of one of them, the menstrual blood accumulates in a confined space, and therefore, on menstruation days, 3-6 months after the onset of menarche, severe pain in the lower abdomen (dysmenorrhea) occurs, which are not arrested by painkillers and antispasmodic drugs. Sometimes in a partition between vaginas there is a fistular opening; in these cases, the menstrual blood partially falls into a full-fledged vagina, but most of it lingers in a confined space. Patients who have a fistulous opening complain of purulent discharge from the genital tract due to the addition of a secondary infection.
With a two-horned uterus with an additional closed functioning horn, adolescent girls complain of cramping pain in the lower abdomen during menstruation, and pain arises with the onset of menarche and is explained by the formation of hematometers. The saddle-shaped uterus, as well as the uterus with a septum, as a rule, has no clinical symptoms in the adolescent period.
Diagnosis of doubling of the uterus and vagina with impaired outflow of menstrual blood often causes difficulties. Frequent diagnostic errors lead to unreasonable surgical interventions, even radical, – appendectomy, removal of appendages, removal of one of the queens, etc.
An objective examination of the external genitals in patients with a doubling of the uterus and vagina is not changed. With a complete doubling of the uterus and vagina, vaginoscopy allows you to identify in each vagina on the cervix. If one vagina is partially aplased, then only one full-fledged vagina is available for inspection, in the dome of which one cervix is visualized. In the hematocolpos of a partially aplastic vagina, one of the walls of a full-fledged vagina (left or right) protrudes into its lumen. Sometimes with a large hematocolpus, its lower pole reaches the hymen. Rectal and abdominal examination does not always allow to clearly define the two uterus. The appendages are usually not defined. The hematocolpus of the partially aplassed vagina is palpated as a formation of a tight-elastic consistency, the immobile, slightly painful, lower pole of which may be 1–5 cm from the anus. In patients with a fistulous course during a rectal-abdominal examination, purulent discharges usually increase sharply.
Ultrasound of the pelvic organs gives reliable information in typical cases of doubling the internal genitalia, allows you to determine the size of the uterus, the amount of hematocolpos. Be sure to conduct ultrasound of the abdominal cavity and retroperitoneal space in the kidney, as a complete doubling of the uterus with partial aplasia of one of the vagina is usually accompanied by agenesis of one kidney. In the case of atypical forms of doubling, the most valuable information that determines the choice of surgical intervention is provided by an MRI scan, and the study should be carried out in the frontal projection. In some cases, hysteroscopy and laparoscopy should be performed in order to clarify the nature of the anomaly during doubling.
Treatment. Surgical treatment of adolescent girls with a doubling of the internal genitalia is indicated only when the outflow of menstrual blood is disturbed (for example, with a complete doubling of the uterus and vagina with partial aplasia of one of them). Surgical treatment consists in creating an anastomosis between the hematocolpos and the unchanged vagina by excision of the intervaginal septum, preferably the maximum. With an additional closed functioning uterus, its removal is shown. In case of a septum in the uterus, the question of surgical correction is resolved only in case of a violation of the reproductive function.
Aplasia of the uterus and vagina
Congenital absence of the uterus and vagina (Rokitansky-Kuster-Meier syndrome) is a rare developmental disorder. The frequency of aplasia of the uterus and vagina ranges from 1 in 5000 to 1 in 20 000 of newborn girls.
Clinical symptoms. The main complaint with which 15-16-year-old girls go to a doctor is the absence of menstruation. At an older age, patients complain about the impossibility of sex life.
Diagnostics. The physique of the surveyed is usually correct, physical and sexual development corresponds to the age norm. Quite well developed secondary sexual characteristics. External genitals, as gynecological examination shows, are developed correctly. Vaginal sounding allows detecting a 1-1.5 cm long hygienic cavity. At a rectal abdominal examination, the uterus is not detected in a typical place, and the cord is palpable. The appendages are not defined.
Ultrasound reveals the ovaries and the absence of the uterus. Sometimes, the patients are found to have various malformations of the urinary system (aplasia of the kidney, lumbar or pelvic dystopia of the kidney, doubling of the cup and pelvis system).
Treatment. Surgical treatment of aplasia of the uterus and vagina is to create a neovagina. As with aplasia of the vagina and cervix, peritoneal or sigmoid colpopoiesis is performed.