What is congenital absence of vagina?

What is congenital absence of vagina? Congenital absence of vagina is a kind of reproductive organ malformation. Most patients with congenital absence of vagina only have completely closed vaginal vestibule mucosa at the normal vaginal opening, without vaginal traces. Many people are not very familiar with this situation. Here is a detailed introduction to what is 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). Normal female chromosome karyotype, normal systemic growth and female secondary sexual characteristics, normal vulva, vaginal absence, uterine development (only bihorns remain), small fallopian tubes, and normal ovarian development and function. The most common patients are Rokitansky-Kustner-Hauser syndrome. Testicular feminization (androgen insensitivity syndrome) patients are relatively rare. Very few are true hermaphroditism or gonadal dysgenesis.

It is often accompanied by no deformity, and only slightly thickened strips of tissue are seen in the middle of the broad ligament in the normal uterine position. About 1/10 patients may have partial uterine development and functional endometrium. After puberty, due to menstrual blood retention, cyclical abdominal pain occurs, and there is no menstruation or it is not discovered until after marriage when seeking medical examination due to sexual intercourse difficulties.

Congenital absence of vagina can cause many complications, with or without uterine dysplasia. If the uterus is dysplastic, it may manifest as primary amenorrhea after puberty, with a small or deformed uterus; if the uterus is normally developed, it may present with primary amenorrhea accompanied by cyclical abdominal pain, intrauterine blood accumulation, uterine enlargement, and sexual dysfunction. Those with ovarian dysplasia may have incomplete development of secondary sexual characteristics, short stature, webbed neck, cubitus valgus, and other deformities.

Timing of treatment for congenital absence of vagina: After detailed examination, if the patient has a nearly normal uterus and normal ovarian function, surgery should be performed after the menstrual period to facilitate menstrual blood drainage and sexual intercourse. After treatment, there is hope for intrauterine pregnancy. If there is no uterus or primordial uterus and other infertile patients, it is best to perform artificial vaginoplasty before or after marriage to mainly solve the problem of sexual life.