Congenital absence of vagina: who has more to do with it, the parents or not?

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 method 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 commonly used method was the patient's own medium-thickness free skin graft, 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 transplanted skin graft 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 with labia flaps destroys the normal vulva shape and is often rejected by patients. Reconstruction with sigmoid colon or ileum segments increases the complexity of the operation. Covering with amniotic membrane or pelvic peritoneum 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 local anatomy of the patient's vulva 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.