Congenital vaginal agenesis, with a prevalence of 1 in 4000 females, occurs mainly as a feature of the Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome. Management of patients with MRKH syndrome includes, along with the proper psychological support, the creation of a neovagina to give them the opportunity to have a normal sexual life. Both surgical and non-surgical techniques have been suggested during the past century, for neovagina creation. Among them, the most widespread non-surgical techniques performed are the Frank technique and its modification by Ingram, while the surgical techniques include the McIndoe operation, the Vechietti procedure and its laparoscopic modification and the Williams vaginoplasty and its Creatsas modification.
The Creatsas vaginoplasty is a simple, fast and safe technique that has been performed in 178 patients in our Institution over the past 22 years and has provided excellent results in the vast majority of patients.
Introduction
Vaginal aplasia is a rare congenital anomaly of the female genital tract with an incidence of 1 in 4000 female births. The most common cause of this abnormality is the Mayer–Rokitansky–Küster–Hauser syndrome (MRKH syndrome). The aetiology of the syndrome is considered to be genetic, but it still remains unclear, though many candidate genes have been studied.
Women with this syndrome often present at adolescence with primary amenorrhoea, normally developed secondary sexual characteristics and normal 46,XX karyotype. Physical examination reveals normal external genitalia, and a more or less deep (2–7 cm) dimple at the introitus area. The uterus is absent or rudimentary, the fallopian tubes are normally developed and the ovaries have normal endocrine function. Other malformations associated with MRKH syndrome involve the upper urinary tract, the skeleton, the auditory system and the heart. In about 40% of the patients, upper urinary tract malformations are found and include pelvic kidney, horse-shoe kidney, unilateral renal agenesis and duplication of the renal pelvis and the ureter. Skeletal anomalies are found in 10–12% of patients and mainly involve the spine, including wedge, supernumerary or rudimentary vertebrae. Auditory defects have also been associated with MRKH syndrome. Conductive hearing impairment due to middle ear malformations (mainly stapedial ankylosis) is the most common disorder. Heart malformations such as pulmonary valve stenosis, Fallot’s tetralogy and aorto-pulmonary window have also been reported. Rare complications have also been reported in patients with MRKH syndrome and include the development of leiomyomas in the rudimentary uterine horns and the occurrence of symptomatic descending salpingitis.
Although vaginal aplasia can be diagnosed during a thorough physical examination of the female neonate, it is usually diagnosed during adolescence, when the adolescent and her family get perturbed by the absence of menstruation. Sometimes, the inability of the young girl to have a normal sexual intercourse leads her to seek medical advice. Diagnosis of the syndrome can be emotionally disturbing for the young girl, who realises her inability to have normal sexual intercourse and pregnancy, and may have significant consequences in self-image and sexual identity. Therefore, treating patients with vaginal aplasia requires, along with psychological support, the creation of a neovagina to give the patient the prospect of a normal sexual life.
Treatment of vaginal aplasia
Numerous procedures and techniques, both surgical and non-surgical, have been proposed over the years for the treatment of vaginal agenesis. The Frank technique and its modification by Ingram are the most well-known non-surgical techniques. The Frank technique involves the use of dilators, progressively increasing in diameter and length, which are placed in the vaginal dimple by the patient herself and are held in place for 20–30 min, 3 times a day. Ingram introduced a bicycle seat stool where the patient could sit and use her trunk weight to hold in place the vaginal dilators and create pressure to the vaginal dimple. Although this technique is associated with less morbidity than the operative ones, the use of vaginal dilators is not pleasant for the adolescent patient and, furthermore, it can be applied only when a vaginal dimple with a length of at least 3–4 cm is present. In patients with shallow vaginal dimples, surgical treatment is generally preferred.
The most widespread surgical techniques performed for the creation of a neovagina are the McIndoe operation, the Vechietti procedure and its laparoscopic modification and finally the Williams vaginoplasty and its Creatsas modification.
In the McIndoe operation, a mould covered with a full-, or a split-thickness skin graft is placed in a surgically created space between the patient’s rectum and urethra to create a neovagina. With this technique, the created neovagina is functional within 6–10 weeks, but the operation is associated with severe complications, such as urethra, bladder or rectum injury, haemorrhage, severe postoperative infection and creation of fistulas. Moreover, patient is obliged to use dilators postoperatively for about a year, even if she has regular sexual activity.
The Vecchietti procedure includes the placement of an acrylic olive in the introital dimple; the olive is dragged with sutures passing through the abdominal area (intra-.or extra-peritoneally) and secured to an apparatus located at the anterior abdominal wall. Traction from the inside elongates the dimple and, within a few days, the neovagina is formed. When this technique was described, back in 1965, laparotomy was mandatory to pass the sutures and place the traction apparatus in the abdominal wall. In 1992, the laparoscopic modification of the Vecchieti technique was introduced by Gauwerky et al., and since then, numerous variations, considering the securing and the course of the sutures, have been applied. This technique is considered to be safer than the McIndoe operation, but the regular stretching of the sutures, which is necessary for the creation of the neovagina, can be very painful and unpleasant. Moreover, the use of dilators is imperative, should the patient not have regular sexual intercourse to prevent the neovagina from shrinking.
In the Creatsas modification of the Williams vaginoplasty, a flap of the perineal skin is used to create the neovagina. The surgical steps are as follows: Under general anaesthesia, four Allis clamps are used to put the vulval tissues under stretch ( Image 1 ). The vulva is then inspected carefully for human papilloma virus (HPV) lesions, because sometimes young patients tend to change sexual partners trying to have successful sexual intercourse, prior to seeking medical advice, thus exposing themselves to the HPV. If lesions are present, diathermy is used to cauterise them. Afterwards, the hymen, which in many patients is present, is cut using diathermy on the 3rd, 6th and 9th hours to prevent haemorrhage during the first intercourse. A symmetrical U-shaped incision is then made in the vulva starting 4 cm lateral to the external urethral meatus at the medial side of the labia, extending down to the perineum and up to the other side of the vulva as shown on Image 2 . The tissues are then mobilised and a first layer of interrupted Vicryl ® or Dexon ® 2/0 sutures is put between the inner skin margins with the knots lying on the inside of the created neovagina ( Image 3 ). Absorbable sutures are also used to approximate the perineal muscles and the subcutaneous fat ( Image 4 ). The last step of the procedure is the stitching of the skin using the same material ( Image 5 ). It is important to observe symmetry during the procedure to achieve a proper aesthetic result.
Possible complications of the Creatsas vaginoplasty are haematoma, wound opening and trauma infection. To prevent the occurrence of a haematoma, careful haemostasis is essential during the operation. There is no need for the use of dilators to keep the neovagina open. Wound opening can be prevented by instructing the patient not to abduct her legs for a couple of days after the procedure. Typical wound hygiene and administration of three doses of wide-spectrum antibiotics postoperatively reduce significantly the risk of trauma infection. Another complication that may occur is hair growth at the neovagina area and it can be managed with laser treatment.
Treatment of vaginal aplasia
Numerous procedures and techniques, both surgical and non-surgical, have been proposed over the years for the treatment of vaginal agenesis. The Frank technique and its modification by Ingram are the most well-known non-surgical techniques. The Frank technique involves the use of dilators, progressively increasing in diameter and length, which are placed in the vaginal dimple by the patient herself and are held in place for 20–30 min, 3 times a day. Ingram introduced a bicycle seat stool where the patient could sit and use her trunk weight to hold in place the vaginal dilators and create pressure to the vaginal dimple. Although this technique is associated with less morbidity than the operative ones, the use of vaginal dilators is not pleasant for the adolescent patient and, furthermore, it can be applied only when a vaginal dimple with a length of at least 3–4 cm is present. In patients with shallow vaginal dimples, surgical treatment is generally preferred.
The most widespread surgical techniques performed for the creation of a neovagina are the McIndoe operation, the Vechietti procedure and its laparoscopic modification and finally the Williams vaginoplasty and its Creatsas modification.
In the McIndoe operation, a mould covered with a full-, or a split-thickness skin graft is placed in a surgically created space between the patient’s rectum and urethra to create a neovagina. With this technique, the created neovagina is functional within 6–10 weeks, but the operation is associated with severe complications, such as urethra, bladder or rectum injury, haemorrhage, severe postoperative infection and creation of fistulas. Moreover, patient is obliged to use dilators postoperatively for about a year, even if she has regular sexual activity.
The Vecchietti procedure includes the placement of an acrylic olive in the introital dimple; the olive is dragged with sutures passing through the abdominal area (intra-.or extra-peritoneally) and secured to an apparatus located at the anterior abdominal wall. Traction from the inside elongates the dimple and, within a few days, the neovagina is formed. When this technique was described, back in 1965, laparotomy was mandatory to pass the sutures and place the traction apparatus in the abdominal wall. In 1992, the laparoscopic modification of the Vecchieti technique was introduced by Gauwerky et al., and since then, numerous variations, considering the securing and the course of the sutures, have been applied. This technique is considered to be safer than the McIndoe operation, but the regular stretching of the sutures, which is necessary for the creation of the neovagina, can be very painful and unpleasant. Moreover, the use of dilators is imperative, should the patient not have regular sexual intercourse to prevent the neovagina from shrinking.
In the Creatsas modification of the Williams vaginoplasty, a flap of the perineal skin is used to create the neovagina. The surgical steps are as follows: Under general anaesthesia, four Allis clamps are used to put the vulval tissues under stretch ( Image 1 ). The vulva is then inspected carefully for human papilloma virus (HPV) lesions, because sometimes young patients tend to change sexual partners trying to have successful sexual intercourse, prior to seeking medical advice, thus exposing themselves to the HPV. If lesions are present, diathermy is used to cauterise them. Afterwards, the hymen, which in many patients is present, is cut using diathermy on the 3rd, 6th and 9th hours to prevent haemorrhage during the first intercourse. A symmetrical U-shaped incision is then made in the vulva starting 4 cm lateral to the external urethral meatus at the medial side of the labia, extending down to the perineum and up to the other side of the vulva as shown on Image 2 . The tissues are then mobilised and a first layer of interrupted Vicryl ® or Dexon ® 2/0 sutures is put between the inner skin margins with the knots lying on the inside of the created neovagina ( Image 3 ). Absorbable sutures are also used to approximate the perineal muscles and the subcutaneous fat ( Image 4 ). The last step of the procedure is the stitching of the skin using the same material ( Image 5 ). It is important to observe symmetry during the procedure to achieve a proper aesthetic result.
