What is the best treatment for congenital absence of vagina?

During the differentiation and formation of sexual organs at about 7-10 weeks of embryonic period, after the two paramesonephric ducts merge, their tail ends are blocked or stagnant and fail to develop downward, resulting in congenital absence of vagina. It is often combined with congenital absence of uterus or rudimentary uterus, and occasionally a normally developed uterus. The ovaries originate from the genital ridge and are rarely affected at the same time. They usually develop and function normally. Congenital absence of vagina is one of the clinical manifestations of (R-K-H syndrome).

[Clinical manifestations] Primary amenorrhea, periodic lower abdominal pain, inability to have sexual intercourse after marriage, etc. Examination of female phenotypes showed normal vulva, only shallow pits in the hymen without vagina; no uterus or primordial uterus malformation, possibly with urinary system or bone malformation; ovarian function tests were within normal range.

[Diagnosis and differential diagnosis] The diagnosis is confirmed based on the patient's medical history, gynecological examination, H-ultrasound scan of the pelvic cavity and kidney area, endocrine test, chromosome karyotype analysis or PCR technology detection of sexual genes. At the same time, X-ray urography should be performed to determine whether there is any deformity, and bone radiographs should be taken to determine whether there is any deformity. It should be differentiated from secondary vaginal stenosis or atresia.

[Treatment] The timing and method of treatment are determined based on the patient's uterine development.

1. Timing of treatment

If the patient has a nearly normal uterus and normal ovarian function after a detailed examination, the 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 the patient has no uterus or a rudimentary uterus and is not likely to have children, it is best to perform artificial vaginoplasty before or after marriage to mainly solve the problem of sexual life.

(1) Vaginoplasty: There are many types of vaginoplasty, such as labia minora flap vaginoplasty, inner thigh flap vaginoplasty, sigmoid colon vaginoplasty, peritoneal vaginoplasty, etc. For specific methods, please refer to gynecological surgery. If pregnancy occurs after surgery, the patient should be closely monitored, and a cesarean section should be selected if the due date is close.

(2) Top-pressure method: For those who are infertile and have a partially sac-like vagina, local top-pressure method can be tried according to the patient's wishes. A round and blunt mold like the bottom of a test tube can be used for long-term self-pressure. Sexual intercourse can also be gradually deepened. If the patient is not satisfied, it also creates better conditions for surgery.