The female reproductive ducts can be born with abnormal development if they are disturbed by some intrinsic or extrinsic factors during embryonic development. They are often combined with urinary system anomalies.
I. Common congenital abnormalities of vaginal development and principles of management
Vaginal atresia: The atresia is located in the lower part of the vagina, which is about 2-3 cm long, with a normal vagina. There is no vaginal opening on examination, and the mucosa at the atresia is normal and does not bulge outward. Primary amenorrhea is present with periodic abdominal pain that worsens progressively after puberty. On anal examination, a vaginal mass protruding into the rectum is found, which is higher than the hymen, and the uterus is normally developed.
Once diagnosed, surgery should be performed as soon as possible. The atretic vagina is incised at the opening of the vagina, the mucosa of the middle part of the vagina with blood collection is partially freed, the mass is cut open and the blood collection is drained.
When incising the atretic vagina, care should be taken to avoid damaging the bladder and rectum, as indicated by a catheter or finger anal examination, which can be guided by first trying to draw the accumulated blood with a thick needle; if necessary, this should be done under ultrasound surveillance, where the needle can be seen to enter the cystic cavity of the accumulated blood and the atretic vagina is incised laterally in the direction of the needle. As far as possible, the free incised middle vaginal mucosa is pulled downward and the wound covering the lower segment is sutured to the outside mucosal margin, covering the incision to prevent adhesions.
Postoperative care is taken to prevent infection. To keep the vagina open, an open drainage tube of appropriate thickness and softness can be placed and kept for several days. If necessary, a small model should be placed in the vagina intermittently for 1-2 months, after 2 normal menstrual periods and a normal review. Avoid re-adhesion of the vaginal opening after several months.
Transvaginal diaphragm: This is caused by a failure to pass through the caudal end of both paramedian tubes after they meet with the urogenital sinus. The vaginal diaphragm can be located anywhere in the vagina, mostly in the upper 1/3 and lower 2/3 of the vagina, and is about 1 cm thick, mostly with a small hole at the center or side of the diaphragm through which menstruation can flow. The complete transverse septum is rare. It is often found during vaginal examination or childbirth due to menstrual discomfort, unsatisfactory sex life, and painful menstruation.
Surgery should be performed as soon as possible once the diagnosis is confirmed. The vaginal septum is cut radially in all directions until the base of the septum is loosened. Absorbable sutures are interrupted to close the rough surface or electrocoagulated to stop bleeding; the vagina is filled with oiled gauze to cover the incision to prevent adhesions; if necessary, a vaginal model is placed to avoid adhesions.
If the septum is thick, inflexible or high, elective cesarean section should be performed; if the septum is incomplete and floppy, radial incision can be made when the septum is thinner by the extension of the fetal head when the uterus is nearly fully opened. If the septum is complete and soft, the septum can be incised radially when the head of the fetus is stretched thin.
Longitudinal vaginal septum: It is caused by the septum not disappearing or not completely disappearing after the reunion of the two sides of the paramedian tube. There are complete and incomplete mediastinum. Complete mediastinum forms a double vagina, often combined with double cervix and double uterus. Sometimes the longitudinal septum is biased to one side to form an oblique septum, resulting in complete atresia of the vagina on that side, and may result in retention of menstrual blood to form a lateral vaginal mass.
If it does not interfere with sexual life or delivery, it can be left alone. If it interferes with the elimination of menstrual blood or intercourse, surgery should be performed after menstruation to cut the mediastinum down the middle. Absorbable sutures are used to intermittently close the rough surface or to stop the bleeding by electrocoagulation. If the mediastinum is found to interfere with the descent of the fetal first dew during delivery, early cesarean delivery should be performed. If the elasticity is good, the mediastinum can be cut immediately when the baby’s head descends, the vagina dilates and the mediastinum becomes thin. After the placenta is delivered, the vagina should be examined and, if necessary, sutured to stop the bleeding.
Congenital absence of vagina: Bilateral paramedian duct hypoplasia, absence of vagina combined with absence of uterus or primordial uterus, normal ovaries and primary amenorrhea. The vulva and secondary sexual characteristics are normally developed, and the external vaginal opening is only a shallow depression. The diagnosis should be confirmed with a pelvic ureterogram as 15% of cases have a urinary tract malformation. In addition, it should be distinguished from androgen insensitivity syndrome, which has a chromosome of 46XY and elevated blood testosterone to male level, because the vulva tissue is insensitive to androgens, resulting in a female vulva, but no vagina and no uterus; it should also be distinguished from male primary glandular dysplasia, which has a chromosome of 46XY, with a cord-like testis, no androgen secretion, and no degeneration of the paramedian duct, and a female phenotype, which may have no vagina and no uterus. Sometimes there is a dysplastic uterus.
For those who wish to marry, various vaginoplasties are performed depending on the development of the vulva. It is usually performed 6 months before marriage. Prior to surgery, a systemic system, pelvic ultrasound, hormone measurement, chromosomal examination, pyelogram and, if necessary, laparoscopy should be performed to find out the presence or absence of a uterus and its degree of development, gonadal morphology, urinary system anomalies, and whether there is hermaphroditism. If the chromosome is 46XY, male gonadectomy should be performed in addition to artificial vaginal surgery. Because of the abnormal development of the testicles and the high temperature in the abdominal cavity, there is a risk of malignant transformation.
II. Several vaginoplasty procedures and their advantages and disadvantages
(i) Frank urogenital sinus pure dilatation method (parietal pressure method).
Indications: 1. congenital absence of vagina, but with short and shallow vaginal recesses, well developed vulva and flaccid tissues. 2. acquired vaginal stenosis with residual distal part of vagina. 3. absence of uterus or only primordial uterus. 4. performed in late pubertal development or before marriage.
Method and procedure: At the beginning of 2-3 months, a 1cm diameter round tubular model is used to press backward and inward against the external vaginal opening for at least half an hour 2-3 times a day in order to create a 4-5cm deep invagination. Then a 2-3 cm diameter model is used to press the vaginal shaft in the direction of the vagina (inward and forward) to a depth of 6-7 cm; after that, a longer and thicker (4-5 cm diameter) device is used to expand and press. In six months to a year, a vagina about 9 cm deep can be formed and can be. Pay attention to the direction of the pressure to avoid damaging the urethral opening and rectum.
Advantages and disadvantages: the method is simple, effective and more satisfactory for sexual life; however, it is not easy to insist on carrying out successfully.
(ii) Williams vulvovaginoplasty.
Indications: Same as (i).
Operation method and procedure: 1. Anesthesia, body position and disinfection are the same as non-porous hymenotomy. 2. Infiltrate the labia majora and posterior union with saline (0.2ml:20ml) diluted with vasoconstrictors (such as epinephrine or vasopressants). 3. Make a horseshoe-shaped incision in the posterior union and both labia, with the upper end reaching the level of the urethra and 4-5cm wide on each side; the incision is deep to the superficial perineal muscle layer. 4. The medial cutaneous edges of the labia will be placed in the midline and intermittently sutured from the bottom up with absorbable 00 thread. Intermittent sutures are made to connect the muscle layer and subcutaneous tissues at the midline. 6. Intermittent sutures are made to close the skin edge with non-absorbable sutures. 7. A new vagina with a depth of about 7-8 cm and a capacity of 2 fingers is formed.
Caution: 1. keep the urinary catheter in place for 48 hours. 2. give antibiotics to prevent infection. 3. keep the vulva clean and apply local heat or physical therapy. 4. remove the sutures in one week. 5. after the wound has healed, dilate the new vagina twice a day with a suitable dilator. 6. 6. In case of wound infection and dehiscence, it will take 3 months before discretionary treatment.
Advantages and disadvantages: The method is simple. However, the postoperative vagina is short and shallow and needs further top pressure expansion; in individual cases, the labia are involved in the vagina and sexual life is unsatisfactory.
(iii) Vaginoplasty —- biofilm method (amniotic membrane method):
Indications:1. congenital absence of vagina. 2. vulvovaginal dysplasia. 3. absence of uterus or only primordial uterus.
Procedure: 1. Lumbar or epidural anesthesia. 2. Take a truncated bladder position and disinfect the vulva and vestibular area. 3. Enter the needle at the mucosa between the urethral opening and the anterior border of the perineum and inject saline plus epinephrine (0.2:20 ml), either under the guidance of an anal finger or/and a metal catheter. 4. Make a transverse incision here and make a blunt separation of the finger to a depth of 9 cm and to accommodate 2-3 fingers. 5. Put a penis sleeve on the slightly open speculum, then cover the outside of the penis sleeve with the prepared amniotic membrane, gently place it into the created cavity and fill the speculum with the gauze ball.6. Withdraw the speculum and intermittently suture the external vaginal opening to prevent the gauze from coming out of the artificial vagina.7. After 10 days remove the sutures, remove the penis sleeve and gauze and douche and replace the vaginal model daily.
Amniotic membrane preparation: Take fresh amniotic membrane (about 28 x 20 cm), wash it in saline, put it in 100 ml saline containing 200,000 penicillin and 1 g streptomycin, soak it for 2 hours and it is ready for use. Patients need to do penicillin and streptomycin skin test before this.
Precautions: 1. 3 days before surgery, less residue diet, oral antimicrobials, preoperative clean enema. 2. Intraoperative attention to avoid injury to the bladder and rectum, if the injury is found intraoperatively, it should be repaired correctly in time. If found postoperatively, a three-stage operation should be done for enterostomy, fistula repair, and return of intestinal fistula.3. Place a catheter for 10 days after surgery to keep the vulva clean.4. Instruct the patient to change the vaginal model by himself after discharge from the hospital. Improperly sized or placed models can cause vaginal compression necrosis and produce bladder, urethra or rectal fistula. 5. 3-4 months after surgery, coitus is possible, and sexual intercourse can increase good results. If you are not married for a while, the vaginal model can be placed at night and removed during the day.
Advantages and disadvantages: the surgical method is relatively simple and easy to perform; no need to take the skin of other parts of the body. However, the drainage time is long and the epithelium in the artificial vagina is slow to evolve.
(iv) Artificial vagina – flap grafting method (punctiform skin grafting method)
Indications: Same as (iii).
Operation method: 1, do the same vaginal cavity as before. 2, use electric section knife to cut a thin half layer of skin slice from the back of the hip, hip or upper inner thigh, 0.3mm thick, 14cm long and about 6cm wide, use dotted skin slice slicer to make it into a mesh, the skin slice can be drawn into 20x9cm square, put into penicillin 200,000 units of warm saline to keep. The patient needs to do penicillin skin test before this. 3. The skin slice is intermittently sutured into a tube with 00 gauge absorbable suture, placed over a slightly open speculum with a condom on the surface and gently inserted into the newly made vaginal cavity. 4. The speculum is filled with gauze strips and removed. 5. The outer edge of the skin slice is intermittently sutured with 00 gauge absorbable suture to the outer cut edge of the artificial vaginal opening. 6. The outer opening of the vaginal cavity is intermittently sutured to avoid the artificial vaginal The gauze strip is dislodged.
The advantages of the punctiform skin slice method are as follows: (1) a smaller amount of skin is required; (2) secretions can be drained in the space between the punctiform skin slice with less rejection; (3) the possibility of narrowing is eliminated; (4) the vagina is completely epithelialized after a few weeks.
Precautions: Same as (c).
Advantages and disadvantages: the epithelialization in the artificial vagina is faster than the release of amniotic membrane. However, it leaves a scar at the site of skin removal; the slipperiness in the vagina is not enough. In individual cases, hyperpigmentation, hair growth and even vaginal cancer occur at the grafted skin.
(v) Artificial vagina: Schubert-Schmid method, (colonic transfer method)
Indications: As before.
The lower abdominal incision is made to investigate the development of the internal genital organs. 2. The sigmoid colon is raised, its blood supply and mesentery are properly treated, and a 15-cm-long intestinal tube is cut and wrapped in wet gauze. 3. The preserved sigmoid colon is anastomosed end to end. 4. The vaginal cavity is made (as before). 5. The pelvic floor peritoneum is incised in the same way as the vaginal cavity, and the free segment of intestine is inserted into the artificial vaginal cavity. 6. The distal end of the intestine is intermittently sutured to the perineal incision. 7. The intestinal cavity of the new vagina is filled with oiled gauze rolls and removed in 7-10 days. 1-2 months later sexual intercourse can be started.
Caution: 1. Avoid poor healing of the intestinal anastomosis. 2. The rate of necrosis of the excised sigmoid segment is about 1%. 3. The rest is the same as before.
Advantages and disadvantages: The vagina has sufficient depth and width, no need to carry a model after surgery and has a certain degree of slipperiness. However, the new vagina sometimes has an odor and the open abdomen leaves a scar; the procedure is relatively complex, long and requires surgical cooperation. The procedure is relatively complicated, long and requires surgical cooperation. It is not used at present.
(vi) Live flap vaginoplasty
Indications: Same as before.
The flap is made into an artificial vaginal space (as before). 2. 4×12 cm flaps are taken from each side of the groin to the external opening of the artificial vagina (the superficial branches of the external pubic artery can be located with a doppler ultrasound scan), the distal end is free, and the flap is interrupted with absorbable sutures to form a cylinder. 4. The speculum is filled with gauze strips and removed in 10-14 days. 5. The incision after skin removal is closed with interrupted sutures with silk thread. 6.
Precautions: same as before.
Advantages and disadvantages: live skin flap, fast wound healing. However, the vagina is dry and the slipperiness is unsatisfactory.
(vii) Vechitti vaginoplasty
Indications: Same as before.
Operation method: 1. transverse incision in the lower abdomen to explore the development and extent of the internal reproductive organs. 2. transverse incision of the recto-uterine sunken peritoneum 5-6 cm posterior to the traced uterus and slightly separated to the pelvic wall on both sides; blunt separation from the cysto-rectal space to the vaginal vestibule 1 cm, allowing at least two fingers width. 3. 2.5 cm diameter plastic ball threaded with nylon thread on both heels. 4. thick perforating needle with eyelet from the pelvic floor peritoneum The two heels of nylon thread attached to the plastic ball are brought into the separated tunnel and then the two anterior abdominal walls are threaded separately outside the peritoneum. 5. The pelvic floor peritoneal incision is closed. The layers of the abdominal wall are closed. 6. A metal fixator is placed on the abdomen, and the two heels of the threads pierced out of the abdominal wall are tied to the ends of the fixator, and the two threads are tightened by twisting the spiral. Generally, the button can be lifted 2-4 cm after surgery.
Note: After the surgery, the stretched threads are adjusted every 1-2 days so that the threads can be lifted 1cm and the new vagina can be formed about 10cm after 10 days, then the stretched threads and plastic balls are removed. Then the vaginal model is placed for 2-3 months and you can get married.
Advantages and disadvantages: the vagina is formed quickly and the sex life is more satisfactory. However, the procedure is more complicated and leaves a scar on the abdomen.
(viii) Biofilm artificial vagina (Davidov peritoneal method)
A combined laparoscopic vaginal surgery with the peritoneum forming the inner wall of the vagina
Indications: as before.
Operation method: 1. make vaginal cavity (as before). 2. wipe into the laparoscope, explore the pelvic organs, inject 50 ml of saline (with epinephrine 0.1 mg) in the front and back of the traced uterus, cut the recto-uterine sunken peritoneum transversely here and extend it to 5-6 cm wide on both sides, separate the anterior bladder-uterine reflex peritoneum and the posterior recto-uterine reflex peritoneum 6×10 cm each. if If there is a primordial uterus, it is excised and then the anterior and posterior lobes of the peritoneum are freed more clearly.4. Cut through the vaginal apex, sew four No. 4 sutures on each of the freed peritoneal margins, and pull the sutures to pull the peritoneum into the vaginal cavity.5. Fix the peritoneal margins to the outer edge of the artificial vagina with interrupted absorbable sutures.6. Suture the peritoneum of the rectal uterine reflex and the bladder uterine reflex at the apex of the cavity to form the vaginal apex.7. Place the vaginal model and keep it for 7-10 days.8. the vaginal model for 7-10 days, then douche and replace it daily.
Caution: 1. The closed suture of the vaginal apex should be complete to avoid penetration of the vagina with the abdominal cavity. 2. If the peritoneum is difficult to free downward pulling tension, the round ligament can be cut or part of the pelvic funnel ligament peritoneum can be cut. 3. Early coitus is performed one month after surgery.
Advantages and disadvantages: no wound on the abdomen, quick exhaustion and recovery after surgery, easily accepted by unmarried young women. The peritoneum is slippery, the drainage time is short, and epithelialization in the new vagina is rapid. However, the procedure is more complicated and requires skills in vaginal surgery and laparoscopic surgery.
III. Surgical approach to an anovaginal functioning uterus
Very few congenital anovaginas still have a normally developed uterus, so they have periodic abdominal pain due to menstrual flow leading to accumulation of blood in the uterine cavity by the time of puberty. A bulging hymen is not visible at the vulvar opening. An enlarged and painful uterus can be detected on anal examination. Once diagnosed, surgery should be performed promptly to anastomose the ectocervix to the formed proximal vagina, while draining the uterine cavity and preserving the uterine fertility. In cases of combined cervical atresia, only rarely can the atretic cervix be incised or excised and the cervical or endometrial cavity successfully anastomosed to the vagina proximally. If the uterus cannot be preserved, it should be removed.
Procedure: 1. Laparoscopy to determine the type and extent of uterine malformation. 2. Excision of the uterus if a vestigial angle is found. 3. Making a new vaginal gap between the rectum and the lower urinary tract (as before), which is joined to the uterus from the tip of the vagina. 4. If a normal patent cervix can be exposed, the accumulated blood is drained, and the new vaginal gap is covered with amniotic membrane or semilaminar skin sheets and a drainable vaginal model is placed. 5. If cervical atresia is found, the uterine cavity and cervix are inspected with a probe from the abdominal cavity and an attempt is made to dilate the atretic cervix or to remove the cervix, the upper part of the new vaginal space is connected to the cervical or endometrial cavity and a drainable vaginal model is placed.6. In these cases, the uterine cavity should be regularly probed postoperatively to avoid adhesions.7. The uterus should be removed.
We encountered a case of a 16-year-old girl with primary dysmenorrhea for several months who was found to have congenital absence of vagina and accumulation of blood in the uterine cavity. First, she underwent vaginoplasty in an outside hospital, which failed to communicate with the uterus. The following month, she came to our hospital with severe dysmenorrhea, which could be tolerated only with continuous epidural anesthesia. She underwent vaginoplasty and cervical dissection via combined vaginal and open surgery, but after several menstrual periods, the cervix was adherent again. The second time, the vaginal part of the vagina and the cervix were opened vaginally and a special intrauterine device with a thick tail was placed to prevent further adhesions. It has been six months since the operation and menstruation has been uneventful. Although the spacious vault and columnar cervix are not visible, the vagina and the apical epithelium are smooth. The thick caudal filament of the IUD is normal. There was also a case of a 14-year-old girl with severe dysmenorrhea, diagnosed with congenital anovagina, myometriosis and absence of cervix. First laparoscopy revealed only a spherically enlarged uterus with surface congestion, purple-blue nodules, and adhesions, and confirmed the diagnosis of myometriosis and absence of cervix. Vaginal surgery was to be performed before marriage.