Treatment of vaginal and uterine malformations

The goal of treating malformations of the vagina and uterus 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.

Non-drug treatment of vaginal and uterine malformations

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.

To improve the elasticity of the tissues of the vaginal vestibule, colpoelongation is performed with the simultaneous use of Ovestin cream and Contractubex gel. The undeniable advantages of the method are its conservatism and the absence of the need to begin sexual activity immediately after its termination.

The duration of the first procedure is on average 20 minutes, subsequently it increases to 30-40 minutes. One course of colpoelongation is about 15-20 procedures, starting with one procedure per day with a transition after 1-2 days to two procedures. Usually 1-3 courses of colpoelongation are carried out with an interval of about 2 months.

In the vast majority of patients with vaginal and uterine aplasia, colpoelongation can achieve the formation of a well-stretched neovagina that can pass two transverse fingers to a depth of at least 10 cm. If conservative treatment is ineffective, surgery is indicated.

Surgical treatment of malformations of the vagina and uterus

In patients with vaginal and uterine aplasia, surgical colpopoiesis is used.

The first reports of attempts to perform this operation date back to the beginning of the 19th century, when G. Dupuitren attempted to create a channel in the rectovesical tissue using a sharp and blunt method in 1817. Before the introduction of endoscopic technologies, colpopoiesis was accompanied by an exceptionally high risk of intra- and postoperative complications.

To prevent the created rectourethral opening from becoming overgrown, they tried to use its long-term tamponade and dilation, insertion of prostheses into the created tunnel between the bladder and the rectum (Gagar dilators made of silver and stainless steel, a phantom with kombutek-2 and colacin, etc.). However, these procedures are extremely painful for patients and not effective enough. Later, numerous versions of colpopoiesis were performed with transplantation of skin flaps into the created tunnel. After such operations, cicatricial wrinkling of the neovagina and necrosis of the implanted skin flaps often formed.

In 1892, V.F. Snegirev performed colpopoiesis from the rectum, which was not widely used due to its high technical complexity and high frequency of intra- and postoperative complications (formation of rectovaginal and pararectal fistulas, strictures of the rectum). Later, methods of colpopoiesis from the small and large intestines were proposed.

Until now, some surgeons use sigmoid colpopoiesis, which includes among its advantages the possibility of performing this operation long before the onset of sexual activity when this type of defect is detected in childhood. The negative features of this type of colpopoiesis are its extreme trauma (the need to perform laparotomy, isolation and lowering of a section of the sigmoid colon), the occurrence of prolapse of the walls of the neovagina in a large number of operated patients, inflammatory complications, up to peritonitis, abscesses and intestinal obstruction, cicatricial narrowing of the entrance to the vagina, as a result of which abstinence from sexual activity. A psychotraumatic situation for patients is discharge from the genital tract with a characteristic intestinal odor and frequent prolapse of the vagina during sexual intercourse. When examining the external genitalia, a demarcation border of red color is clearly visualized at the level of the entrance to the vagina. One cannot but agree with the opinion of L.V. Adamyan et al. (1998) that this method of correction, performed not for vital indications, is traumatic, is accompanied by a high risk of complications both during surgery and in the postoperative period, and is currently of only historical interest.

In modern conditions, the "gold standard" of surgical colpopoiesis in patients with vaginal and uterine aplasia is colpopoiesis from the pelvic peritoneum with laparoscopic assistance. In 1984, N.D. Selezneva et al. first proposed colpopoiesis from the pelvic peritoneum with laparoscopic assistance using the "shining window" principle, the technique of which was improved in 1992 by L.V. Adamyan et al.

This surgical intervention is performed by two teams of surgeons: one performs the endoscopic stages, the second - the perineal stage.

Under endotracheal anesthesia, a diagnostic laparoscopy is performed, during which the condition of the pelvic organs, the mobility of the peritoneum of the vesicorectal cavity are assessed, and the number and location of the muscle ridges are identified. The manipulator marks this part of the peritoneum and moves it down, holding it constantly.

The second team of surgeons begins the perineal stage of the operation. The perineal skin is dissected along the lower edge of the labia minora at a distance of 3-3.5 cm in the transverse direction between the rectum and the urinary bladder at the level of the posterior commissure. A channel is created in a strictly horizontal direction, without changing the angle, using a sharp and predominantly blunt method. This is the most important stage of the operation due to the possibility of injury to the urinary bladder and rectum. The channel is created to the pelvic peritoneum.

The next important stage of the operation is the identification of the peritoneum, which is carried out using a laparoscope by illuminating (diaphanoscopy) the parietal peritoneum from the abdominal cavity and bringing it in with soft forceps or a manipulator. The peritoneum is grasped in the tunnel with clamps and dissected with scissors. The edges of the peritoneal incision are lowered and sutured with separate vicryl sutures to the edges of the skin incision, forming the entrance to the vagina.

The final stage of the operation is the formation of the dome of the neovagina, which is performed through a laparoscope. Purse-string sutures are placed on the peritoneum of the urinary bladder, muscle ridges (rudiments of the uterus) and the peritoneum of the lateral walls of the small pelvis and sigmoid colon. The dome of the neovagina is created at a distance of 10-12 cm from the skin incision of the perineum.

On the 1-2 day, a gauze tampon with Vaseline oil or Levomekol is inserted into the neovagina. Sexual activity can begin 3-4 weeks after the operation, and regular sexual intercourse or artificial bougienage to maintain the lumen of the neovagina is a mandatory condition for preventing its walls from adhering.

Studies of remote results have shown that almost all patients are satisfied with their sexual life. During a gynecological examination, there is no visible border between the vaginal vestibule and the created neovagina, the length is 11-12 cm, the elasticity and capacity of the vagina are quite sufficient. Moderate folding and minor mucous discharge of the vagina are noted.

In case of an incomplete rudimentary but functioning uterus and pain syndrome, usually caused by endometriosis (according to MRI and subsequent histological examination), their removal from the pelvic peritoneum is performed simultaneously with colpopoiesis. Removal of functioning muscle cords/cords is possible in case of severe pain syndrome in young patients without colpopoiesis. Colpopoiesis is performed at the second stage of treatment: surgical (from the pelvic peritoneum before the onset of sexual activity) or conservative (colpoelongation according to Sherstnev).

Similar treatment tactics are the only justified method of correcting vaginal aplasia in patients with a rudimentary functioning uterus. To select a method of surgical correction, it is necessary to have a clear idea of the anatomical and functional adequacy of the uterus. A functioning uterus with aplasia of the cervix or cervical canal is a rudimentary, underdeveloped organ that is unable to fully perform its reproductive function, and there is no need to preserve the defective uterus at any cost. All attempts to preserve the organ and create an anastomosis between the uterus and the vestibule of the vagina using sigmoid or peritoneal colpopoiesis were unsuccessful due to the development of severe postoperative infectious complications that required repeated operations. Under modern conditions, extirpation of a functioning rudimentary uterus in vaginal aplasia can be performed laparoscopically.

Stages of extirpation of a functioning rudimentary uterus using laparoscopic access:

• diagnostic laparoscopy (pelvic revision, hysterotomy, opening and emptying of the hematometra, retrograde hysteroscopy confirming the absence of continuation of the uterine cavity into the lumen of the cervical canal);
• creation of a canal to the functioning rudimentary uterus and pelvic peritoneum using perineal access:
• extirpation of a functioning rudimentary uterus using laparoscopic access (intersection of the uterine ligaments, fallopian tubes, proper ovarian ligaments, opening of the vesicouterine fold, intersection of the uterine vessels, excision of the uterus);
• colpopoiesis from the pelvic peritoneum for patients ready to start sexual activity; for patients not planning sexual contacts, after surgery and healing of sutures, colpoelongation can be performed.

In a certain number of operated patients with vaginal aplasia and rudimentary uterus, histological examination of the removed specimen reveals a non-functioning endometrium, and adenomyosis and numerous endometrioid heterotopias are detected in the thickness of the rudimentary uterus, which, apparently, is the cause of severe pain syndrome.

Unfortunately, girls with vaginal aplasia (partial or complete) and a functioning uterus with symptoms of "acute abdomen" are often given an incorrect diagnosis (acute appendicitis, etc.). As a result, appendectomy, diagnostic laparotomy or laparoscopy, removal or resection of the uterine appendages, erroneous and harmful dissection of the apparent atretic hymen, etc. are performed. Carrying out surgical interventions in the amount of puncture and drainage of hematocolpos, including subsequent bougienage of the aplastic part of the vagina, is unacceptable. This not only does not eliminate the cause of the disease, but also complicates the further implementation of adequate correction due to the development of an infectious process in the abdominal cavity (piocolpos, pyometra, etc.) and cicatricial deformation of the vagina.

Currently, the optimal method for correcting incomplete vaginal aplasia with a functioning uterus is vaginoplasty using the sliding flap method. In order to reduce the risk of surgery, objectively assess the condition of the uterus and appendages, and, if necessary, correct concomitant gynecological pathology, vaginoplasty should preferably be performed with laparoscopic assistance. In addition, the creation of pneumoperitoneum helps to shift the lower edge of the hematocolpos downwards, which significantly facilitates the operation even if it is insufficiently filled.

Stages of vaginoplasty using the sliding flap method.

• Cruciate dissection of the vulva with mobilization of flaps over a length of 2-3 cm.
• Creation of a tunnel in the retrovaginal tissue to the lower pole of the hematocolpos. This stage of the operation is the most complex and responsible due to the risk of injury to the bladder and rectum, which are closely associated with the aplastic part of the vagina.
• Mobilization of the lower pole of the hematocolpos over a length of 2-3 cm from the underlying tissues.
• X-shaped incision of the lower pole of the hematocolpos (at an angle of 45" relative to the straight cross-shaped incision).
• Puncture and emptying of the hematocolpos, washing the vagina with an antiseptic solution, visualization of the cervix.
• The edges of the vulva and the lower edge of the emptied hematocolpos are connected in a wedge-in-groove manner (the principle of gear teeth).

After the operation, a loose tampon soaked in Vaseline oil is inserted, followed by daily sanitation of the vagina and repeated insertion of the tampon for 2-3 days.

In case of a functioning closed horn of the uterus, the rudimentary uterus and hematosalpinx are removed through a laparoscope. To reduce trauma to the main uterus in situations where the rudimentary uterus is intimately connected to the main uterus, L.V. Adamyan and M.A. Strizhakova (2003) developed a method of surgical correction of a closed functioning horn located in the thickness of the main uterus. Laparoscopy, retrograde hysteroresectoscopy and resection of the endometrium of the closed functioning horn of the uterus are performed.

Surgical treatment of a double uterus and vagina with partial aplasia of one of them consists of dissecting the wall of the closed vagina and creating a communication between it and the functioning vagina measuring 2x2.5 cm under laparoscopic control.

• Vaginal stage:
  • opening of hematocolpos;
  • emptying the hematocolpos;
  • washing the vagina with an antiseptic solution;
  • excision of the closed vaginal wall (creation of an "oval window").
• Laparoscopic stage:
  • clarification of the relative position of the uteruses, the condition of the ovaries, and the fallopian tubes;
  • control of emptying of the hematocolpos;
  • emptying of the hematosalpinx;
  • detection and coagulation of endometriosis foci;
  • abdominal cavity sanitization.

In girls with atresia of the hymen, an X-shaped incision is made under local anesthesia and the hematocolpos is emptied.

Approximate periods of incapacity for work

The disease does not cause permanent disability. Possible periods of disability - 10-30 days are determined by the rate of recovery after operations.

Further management

In patients with vaginal and uterine aplasia, it is advisable to repeat courses of colpoelongation 2-3 times a year in the absence of a permanent sexual partner to prevent neovaginal stricture after surgical colpopoiesis.

For the purpose of timely diagnosis of cicatricial changes in the vagina after surgical correction of the vagina and uterus, dispensary observation with examination once every 6 months up to 18 years is indicated.

Information for patients

The absence of independent menstruation at the age of 15 years and older, cyclical pain in the lower abdomen that increases in intensity and menarche are indications for consultation with a gynecologist of childhood and adolescence for timely detection of malformations of the uterus and vagina. In case of severe pain during the first sexual intercourse or impossibility of sexual activity, attempts at sexual intercourse should be stopped in order to avoid penetrating mutilating ruptures of the perineum and urethra in patients with vaginal aplasia.

Forecast

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.