Treatment of congenital absence of vagina

Congenital vaginal embryos are obstructed by internal or external factors during development, or may be caused by abnormal development of the paramesonephric duct due to gene mutations (may have a family history). The most common patients are Rokitansky-Kustner-Hauser syndrome, which is characterized by normal female chromosome karyotype, normal systemic growth and development of female secondary sexual characteristics, normal vulva, vaginal absence, uterine development (only bihorn remnants), small fallopian tubes, and normal ovarian development and function. Testicular feminization (androgen insensitivity syndrome) patients are relatively rare. Very few are true hermaphroditism or gonadal dysgenesis.

The principle of treatment for congenital absence of vagina is to reconstruct the vagina. There are various methods for artificial vaginal formation, including non-surgical treatment, which is to use the means of top pressure to gradually push the closed vestibular mucosa in the normal vaginal position toward the head end along the vaginal axis to form an artificial cavity. This method requires a long treatment time and the artificial vagina formed is short. If the tissue elasticity is poor, it is difficult to succeed. It is now basically abandoned and rarely used. Surgical treatment mainly separates the urethra, bladder and rectum to form an artificial cavity, and uses different methods to find an appropriate cavity wound covering to reconstruct the vagina. In previous years, the most common method used was the patient's own medium-thickness free skin grafting method, but after surgery, a hard vaginal mold needs to be used for a long time to expand the artificial vagina to prevent the artificial cavity covered by the grafted skin from contracting, which increases the patient's pain and brings great inconvenience to work and life. Moreover, the difference in the characteristics of skin and mucosal tissues is too great, and it does not meet physiological requirements, which is its biggest disadvantage. Vaginoplasty using labia skin flaps destroys the normal vulva morphology and is often rejected by patients. Reconstruction using sigmoid colon or ileum segments increases the complexity of the operation. Using amniotic membrane or pelvic peritoneum to cover also has its own disadvantages. Therefore, although there are many methods, there is still no very ideal shaping surgery. The choice should be mainly based on the patient's vulvar local anatomy and other specific clinical conditions. In recent years, with the progress of microsurgery, vascularized myocutaneous flaps have been used to cover the cavity, opening up a new way for this operation. Its advantages and disadvantages need to be promoted before a conclusion can be drawn.

When the paramesonephric duct is seriously dysplastic, there is often incomplete renal rotation, low migration of the kidney, or migration to the pelvic cavity, forming pelvic kidney, horseshoe kidney, transboundary renal ectopia (two kidneys are located on the same side of the body), renal agenesis, renal insufficiency and other urinary tract malformations. Literature reports that renal malformations account for more than 30% of RKH syndrome. Therefore, when vaginoplasty is performed on cases of congenital absence of vagina, intravenous pyelography should be performed before the operation to be alert to the presence of the above malformations and avoid surgical injuries. In addition, patients with this disease also have skeletal deformities, accounting for about 10%, including spina bifida, sacral occult fracture, vertebral fusion and spinal non-segmentation.