Mullerian agenesis (MA) is a rare congenital disorder of the female reproductive system characterized by the absence of the uterus, cervix, and/or upper 2/3 part of the vagina. MA is estimated to affect 1 in 4,000-5,000 women. It is also defined as Mayer-Rokitansky-Küstner-Hauser (MRKH) syndrome
1.
MA pathology includes a defect in the development of the caudal end of the paramesonephric ducts. Patients are typically first identified by a gynecologist at ages 14-15 years with the complaint of absence of menstrua-tion. Generally, these patients have normal ovaries, secondary sexual characteristics, normal chromosome number (46, XX), and external genitalia. Owing to the absence of the uterus, menstruation does not occur at an average age; however, ovulation occurs regularly2.
In these cases, it is necessary to construct a new vagina (neovagina) to help the patients in leading a a regular sexual life. More than 100 techniques have been defi-ned in relation to this procedure. The most applied techniques are given in figure 13,4.
The present study aimed to describe the experiences of the patients with pre- and post-procedural conditions who were admitted to our clinic with the complaint of MA and for neovagina reconstruction.
The patients diagnosed with MA and who underwent neovagina reconstruction procedures between 2016 and 2021 were included in the study. The written informed consent form was obtained from all patients. Pelvic examinations of the patients and transabdominal pelvic ultrasound was performed.
Karyotype analysis and pelvic MRI were ordered from all patients. The patients were given detailed informa-tion about neovagina techniques at the time of admis-sion. The detailed information on neovagina techniques was clearly explained to all patients at admission.
All of the patients had previously been diagnosed and had now applied for neovagina creation. One of t The neovagina techniques of the McIndoe procedure were used in 2 patients, the Frank method in 4 patients, and the laparoscopic modified Davydov procedure in 6 patients.
In the McIndoe procedure, urinary catheterization was performed in the lithotomy position under general anesthesia. A horizontal incision was made on the blind vagina, creating a vesicorectal space up to the Douglas peritoneum via sharp dissection, and hemostasis was achieved. A total thickness skin graft was harvested from the inguinal region by a plastic surgeon. The handmade mold was covered with the skin graft and placed in the vesicorectal space. The graft was fixed to the vaginal entrance in 4-6 places with 2.0 vicryl. On the postoperative fourth day, the mold was removed, the vagina was washed with physiological saline, and a new mold covered with a condom was inserted. The patient was advised to wear tight underwear and was taught mold care, removal, and reinsertion. On the postoperative seventh day, the urinary catheter was removed, and postoperative antibiotic treatment was administered for one week. Patients were recommen-ded to wear the mold continuously for one month and only at nights for the following two months. The pati-ent was called for regular checkups in months 1, 3, and 6. Sexual intercourse was recommended after the third3rd month. Patients were instructed to use the mold at night until the sixth month, then mold usage frequency was left to the patient's discretion.
The Frank technique is the process of creating a vesico-rectal cavity by invagination of the blind vagina with intermittent pressure. For this procedure, borosilicate glass rigid dilators in 3 sizes, i.e., 3×3, 3×5, and 3×8 were used. After the patient was taught how to use the dilator, she was advised to relax the perineal muscles and push them out of the introitus for 30 minutes twice daily. As the vagina lengthened, the patient was inst-ructed to use a larger-sized dilator. She was called for a check-up every two weeks.
In the modified Davydov procedure, under general anesthesia, an incision was made on the vagina in the lithotomy position, creating a vesicorectal space up to the Douglas peritoneum via sharp dissection. A gauze pad was placed after hemostasis. Then, laparoscopy was started. The bladder peritoneum and the Douglas peritoneum were released. An incision was made on the gauze pad and the vesicorectal space was opened. The bladder peritoneum was pulled through vagina and fixed to the anterior intraoitus, and the Douglas perito-neum to the posterior introitus at 2-4 places with 2.0 vicryl. The vaginal dome was formed by laparoscopi-cally suturing the bladder peritoneum, bilateral round ligament, and rectal peritoneum with a mesh string (2.0 Prolene, Ethicon, NJ, USA). A mold was placed in the vagina. During the postoperative 48th hour, the urinary catheter and the mold were removed. The patient was taught mold care and insertion and removal. It was recommended to wear the mold continuously for one month and only at nights for the following twomonths. In months 1 and 3, the patient was called for a regular checkup. Sexual intercourse was recommended on the third month. In the absence of regular intercourse twice a week, it was recommended to keep the mold inserted at night for six months. In the sixth month, the patient was called for a checkup.
Patient ages; vaginal length at admission, at the end of the procedure, using the Frank method, and at the pos-toperative sixth month in operative procedures; and frequency of sexual intercourse, complaints, and comp-lications were recorded.
The 12 patients who underwent neovagina reconstruc-tion with the diagnosis of Mullerian agenesis were aged 18-31 years (mean 24.4 years). The preoperative vaginal lengths of the patients were 1-5 cm (mean length 2.1 cm). The postoperative vaginal lengths of the patients were 7-10 cm (mean length 7.9 cm). All four patients who underwent the Frank method were single, one of them was divorced and one was planning to marry in four months. A patient who underwent the modified Davydov procedure was divorced and was planning her second marriage; all the other patients were married.
All patients had bilateral ovaries, and their karyotype was 46+XX. One patient had cross ectopia and fusion anomaly in the kidney. All patients used the administe-red molds regularly and came to their follow-up visits regularly. In the follow-ups, hair growth was observed in the vagina in both patients who underwent McIndoe. In a patient who underwent Davydov, 1 unit of erythrocyte suspension was given due to intraoperative bleeding during vesicorectal dissection. Fistula, stricture, and keloid scar infection were not observed in the ope-rated patients. Patient ages, pre- and post-operative vaginal lengths, kidney anomalies, sexual intercourse status, and complications are shown in table 1.
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Table 1: Neovagina procedures we performed and pre-postoperative conditions of the patients. |
The main reason for neovagina creation in cases of MA is to make it possible for these patients to engage in sexual intercourse5,6.
The fact that many techniques are described confirms that no single technique is a perfect answer to this complex problem. Ideally, the creation of a neovagina should be simple, safe, and most importantly, allow satisfactory sexual intercourse7. The timing of the surgery depends on the patient’s anatomical condition and the presence or absence of functional endometrial tissue. Opinion differs depending on when this correc-tion should be introduced.
The Frank method is the most common nonoperative technique. The back of the blind vagina is loose fib-roareolar tissue and can be stretched easily. This tech-nique is based on the principle of increasing the vaginal length and width via the daily self-administration of rigid vaginal dilators. Treatment should only be started when the patient is mature enough and expresses a desire to try because the patient may stop dilating or not accept this method from the beginning due to pain and fear8,9.
We applied the Frank method to 4 patients who accepted the procedure. All were single and one patient planned to marry in four months. All patients could tolerate the dilatation and consequently opted for surgi-cal intervention. We think that the preference for this method by single individuals is influenced by the need to use the vaginal mold for a long time in operative techniques, in the absence of regular sexual intercourse.
Other dilatation methods described in the literature are dilatation with dilators mounted to a bicycle stool (Ingram)10 and dilatation with coitus (d’Alberton)11. D’Alberton reported a 95% success rate for neovagina dilated via coitus. Complications of this method are urethral coit and vaginal prolapse. Two of the patients who applied to us had dilatation with coit during their divorced marriages. They had vaginal lengths of 4 and 5 cm when they applied. Since one of them was plan-ning for a second marriage, she preferred operative vaginoplasty. Dilatation was performed on the other patient using the Frank method.
The American College of Obstetricians and Gynecolo-gists (ACOG) recommends dilatation as a first-line treatment because of the generally good results and low risk of complications12.
Operative vaginoplasty can be performed with various techniques when dilatation fails or at the request of the patient.
Bainster and McIndoe first described the McIndoe technique in 193813. This technique has been the preferred method for many clinicians. The low compli-cation rate and relative simplicity, as well as the redu-ced surgical risk as it does not require a transabdominal approach, are the advantages of this technique. However, it has disadvantages such as scar tissue for-mation in the grafted area, keloid formation, stricture formation, risk of infection, and hair growth in the vagina. Squamous cell carcinoma has been reported in neovagina7,14.
Hair growth was observed in the vagina in the two patients who underwent the McIndoe procedure. However, it has been reported that this troubling condi-tion subsided over time due to follicle atrophy and cutaneous metaplasia15,16.
In this technique, As with most surgical procedures, the first operation is probably the most successful in this technique. Compared with secondary operations performed after unsuccessful surgery, it is relatively easy to create a suitable area and protect it after the operation with a cooperative patient in the first operation 7. Therefore, regular and adequatepostoperative dilatation performed by the patient is the most critical-factor affecting the operational success. We use a boro-silicate glass rigid dilator for postoperative dilatation. Molds made of soft materials can also be used after surgery. However, there are not enough studies in the literature comparing the results of soft and hard molds. Regardless of which mold is used, regular and effective use under the supervision of a doctor is required14.
The method that does not require dilatation after sur-gery is vaginoplasty, in which intestinal grafts are used. However, this method prevents it from being the first choice due to the need for laparotomy and the risks of severe infection, intestinal stenosis, dehiscence, and fistula formation. In addition, there is a risk of vaginal discharge with intense mucus content in the vagina and rarely malignancy in this method14.
Another surgical approach to treat vaginal agenesis is to create a new vagina using a peritoneal flap. Davydov first used this approach in 196917.
This approach can be done laparoscopically or via laparotomy. However, there is a risk of damage to the bladder and/or ureter, peritonitis, and vesicovaginal fistula formation18. One patient for whom we app-lied this technique, was given a 1U erythrocyte suspen-sion due to excessive bleeding during vesicorectal dissection.
In the postoperative vagina examination of 51 patients who underwent the modified Davydov procedure, a positive Schiller test was observed after the sixth month, adequate mucosal thickness and differentiation and glycogen storage were observed in light microscopy in biopsies, and an ultrastructural surface appearance close to normal was observed in electron microscopy19.
It is noteworthy that six patients to whom we applied the modified Davydov procedure had sufficient vaginal length, did not have dyspareunia, and the technique was easy to apply. This technique also requires the use of a postoperative dilator.
Another method applied laparoscopically is the Vac-hietti procedure. This method is based on the traction of the threads attached to the bead (olive) placed in the vaginal dome, through the abdominal route and a tension device5. Disadvantages are the long hospital stay, long-term bladder catheterization, the tension set used, and the high cost of the operation due to the length of hospital stay. In addition, the traction of the vaginal dome can be very painful and may not be tole-rated by the patient. A postoperative dilator is also necessary for this method14,20.
As a result, most of the literature consists of non-comparative single-center case series. Therefore, the best treatment of vaginal agenesis in terms of outcome and complication rate remains controversial. Neovagi-na techniques applied to optimize sexual life in women with Mullerian agenesis should be individualized ac-cording to the patient. Non-operative methods should be the first method to be recommended as they provide information about the use of dilators and the procedure. The McIndoe procedure is simple and effective met-hod, but it should be kept in mind that hair may grow in the vagina, and the use of postoperative molds is essential for the formation of an appropriate vagina length in both McIndoe and Davydov procedures.
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