Congenital malformations of the genital organs make up from 3 to 7% of gynecological diseases in children and adolescents. These defects occur in the process of embryogenesis due to exposure to genetic, endocrine, exogenous and other harmful factors. Normally, the proximal Mullerian ducts do not merge and form the fallopian tubes, and the distal ducts merge to form the uterus and the proximal part of the vagina. The distal part of the vagina is formed as a result of complex interactions between the caudal part of the fused Muller ducts, the urogenital sinus and the cloaca. Depending on which area did not occur such a merger and sewage, there is one or another malformation. Anomalies of the reproductive system are often combined with malformations of the urinary tract.
There are the following forms of malformations:
• obstruction of the vagina and cervix;
• doubling of the uterus and vagina;
• aplasia of the uterus and vagina.
By obstruction of the vagina and cervix is meant aplasia (the absence of part or all of the organ) or atresia (septum less than 2 cm in length), which prevents the flow of menstrual blood and in the future – sexual life. The hole in the septum determines the fistulous form of atresia. The most common forms of obstruction of the vagina and cervix.
Classification of obstruction of the vagina and cervix:
• atresia of the hymen;
• atresia of the vagina (full, fistulous);
• aplasia of the vagina;
• upper division;
• upper and middle sections;
• middle department;
• middle and lower divisions;
• total;
• atresia of the cervical canal;
• aplasia of the cervix.
Clinical symptoms of congenital obstruction of the vagina and cervix, regardless of the form, usually appear with the onset of menstruation and the formation of a hematocolpus (accumulation of blood in the vagina) or hematometers (accumulation of blood in the uterus). Defect is extremely rarely diagnosed before the onset of menstruation due to the absence of complaints in little girls. However, in babies, as a result of stimulation of the vaginal and cervical glands by maternal estrogens, filling and stretching of the vagina with mucus is possible with the formation of a mucocposis (fluorocolpos). Mukokolpos has no characteristic symptoms, is difficult to diagnose and is detected by chance, when examining a child about anxiety associated with urination, abdominal mass formation, and sometimes due to associated developmental defects. With a significant stretching of the vagina, obstruction and hydronephrotic transformation of the upper urinary tract are possible. In adolescence, obstruction of the vagina and cervix leads to menstrual stretching of the genital tract with acute, cyclically recurring abdominal pain, with which the girls go to surgical clinics. When hematocolpos pains are aching; hematometer manifests as spastic pain, sometimes with loss of consciousness.
The clinical symptoms of congenital atresia of the cervical canal and cervical aplasia are the same as in the absence of the vagina: a hematometer is formed, menstrual blood reflux through the fallopian tubes into the abdominal cavity is possible. As a result of menstrual blood reflux, symptoms of peritoneal irritation join spastic pains in the lower abdomen.
Fistulae (incomplete) forms of vaginal atresia in both early and adolescence are often accompanied by ascending infection and the formation of pyocolpos. Fistula atresia with pyocolpos is accompanied by occasional purulent secretions, the cause of which is difficult to determine. Quite often, emptying of the pyocolpos occurs during a rectal-abdominal examination or spontaneously against the background of anti-inflammatory therapy. Undrained pyocolpos is rarely isolated. As a rule, pyometalus, pyosalpinx and pus reflux into the abdominal cavity develop rapidly. Then the clinical symptoms of “acute abdomen” appear, the patient’s general condition worsens, the temperature reaches febrile values. Rising infection sometimes develops so quickly that even emergency colpotomy does not save the patient from peritonitis.
Diagnostics. A standard examination for suspected vaginal and cervical obstruction includes anamnesis, an assessment of physical and sexual development, an examination of the external genital organs, general clinical and laboratory studies, bacteriological and bacterioscopic examination of secretions from the genital tract, rectal-abdominal examination, sensing of the vagina, ultrasound of the genital tract, and urinary systems. As a rule, these studies are sufficient to accurately determine the variant of the defect and select the method of surgical treatment.
An objective examination of the vestibule of the vagina and the hymen usually look. Even with total aplasia of the vagina, his vestibule is preserved, and only with atresia of the virginal membrane, its appearance is different from the usual one. When pressing on the anterior abdominal wall above the lap, the hymen erupts in the form of a cyanotic dome as a result of the hematocolpos. The upper pole of the vagina can reach the level of the navel, while the uterus is located high above the entrance to the small pelvis.
The results of bacteriological and bacterioscopic studies of secretions from the genital tract provide the basis for adequate antibiotic therapy.
A roundly elastic, slightly painful and sedentary formation palpable during rectal and abdominal examination, which pushes the uterus upwards, is usually a hematocolposus. The pressure on the hematocolum through the rectum is transmitted to the uterus and is felt on palpation above the womb. The displaceability of the hematocolposa is limited; this is what distinguishes it from an ovarian cyst.
Vaginal sounding allows you to determine the depth of the latter and is carried out simultaneously with rectal-abdominal examination. Measuring the distance from the top of the dome of the vagina to the bottom of the hematocolpos helps to accurately determine the diastasis between the vaginal parts, assess the stocks of plastic material and outline the plan of operation. The distal part of the vagina is often represented by a single vestibule and has a depth of about 1–2 cm. Less commonly, the recess behind the hymen can be less than 1 cm.
Ultrasound can reliably establish the level of obstruction of the vagina or cervix only with hematocolpos and (or) hematometer. Important parameters for this are not only their size, but also the distance from the bottom of the hematocolpos to the skin of the perineum. For small hematocolpos or its absence, an MRI of the pelvic organs should be used. In some cases, the most informative are laparoscopy and vaginography.
Special preparation for MRI is not required. The study is conducted in the position of the patient on the back in the frontal, sagittal and axial projections. In atresia and aplasia of the vagina to clarify the anatomical structure of the most informative sagittal projection, allowing you to accurately determine the diastasis between the sections of the vagina, the dimensions of hematometers and hematocolpos, to assess the condition of the cervix.
Ultrasound and MRI data for a vaginal fistula may vary depending on the time of the examination and the degree of vaginal filling. Erroneous interpretation of MRI data is possible when emptying pyokolpos.
Compared with ultrasound and MRI in the diagnosis of fistulous forms of vaginal atresia, vaginography is more informative, which is associated with the introduction of a contrast agent into the vaginal cavity above the site of obstruction with subsequent x-rays.
Diagnosis of congenital obstruction of the vagina often causes difficulties, which leads to an erroneous diagnosis and the wrong tactics of the patient. The disease often manifests unexpectedly, begins with acute abdominal pain, urinary retention or the appearance of a tumor-like formation in the abdominal cavity, which cause an unwarranted laparotomy. Many teenage girls with congenital obstruction of the vagina are first subjected to appendectomy due to suspected acute appendicitis, and only then a correct diagnosis is established. This is due to severe abdominal pain, often leaving the surgeon no doubt about the need for emergency surgery. To avoid unjustified appendectomy allows ultrasound, which is detected hematocolpos.
Severe consequences can result from incorrect diagnosis of mucocolpos in children. Suspicion of a tumor or cyst of the abdominal cavity provokes the surgeon to perform a broad laparotomy with the removal of the upper section of the vagina and cervix.
Acute urinary retention or an admixture of pus in the urine (pyuria), which occurs during fistulous pyocolpos, requires a urological examination.
In connection with acute pains in the lower abdomen with high obstruction of the vagina, hematocolpos are often opened and its contents evacuated without subsequent vaginoplasty. Such an intervention is associated with a significant risk of damage to the urethra, bladder and rectum and can lead to the development of pyocolpos, pyometras, as well as peritonitis. There is no emergency indication for emptying the hematocolpos. The female genital tract is characterized by large adaptive capacities, and analgesic therapy (baralgin4, maxigan4) is sufficient to alleviate or relieve pain syndrome. Surgery can be performed only by highly qualified specialists, preferably in patients in the intermenstrual period.