congenital absence of vagina
Introduction
Congenital no vagina introduction Congenital absence of vagina to normal female chromosome karyotype, systemic growth and secondary sexual development of women, normal vulva, vaginal loss, uterine development (only double angle residual), small fallopian tube, ovarian development and normal function are often characterized Patients with Rokitansky-Kustner-Hauser syndrome are the most common, and testicular feminization (androgen insensitivity syndrome) is rare. Very few are true hermaphroditism or gonadal dysplasia. Most congenital vaginal patients have only a completely occluded vaginal vestibular mucosa in the normal vaginal opening, no vaginal marks, and some patients have shallow depressions in the vestibule of the vagina, and some have a blind end vagina shorter than 3 cm. Often accompanied by no deformity, in the normal uterus, only see a slightly thickened case-like tissue, located in the middle of the broad ligament, about 1 / 10 patients may have part of the uterus development, and functional endometrium, After puberty, due to menstrual blood retention, periodic abdominal pain, no menstruation or until after marriage due to difficulty in sexual intercourse diagnosis and found. basic knowledge The proportion of sickness: 0.00001% Susceptible population: newborn Mode of infection: non-infectious Complications: abnormal uterine development amenorrhea
Cause
Congenital absence of vaginal causes
Congenital vaginal etiology mainly has the following aspects:
Chromosomal abnormalities (30%):
A chromosome is a basic substance that constitutes a nucleus and is a carrier of a gene. Chromosome abnormalities are also called chromosomal dysgenesis. American Chinese Jiang Youxing (1956) found 46 human chromosomes, and Caspersson et al. (1970) first published human chromosomes.
Androgen insensitivity syndrome (20%):
The androgen receptor gene is located near the centromere of the long arm of the human X chromosome and contains 8 exons encoding 910 amino acids with two zinc finger regions in between and an androgen binding region at the C-terminus. Gene mutations in certain parts can lead to androgen insensitivity syndrome or testicular feminization. Clinical manifestations of individuals with karyotype XY develop into seemingly normal but fertile women, and the testes usually stay in the abdominal cavity. There is no spermatogenic process.
Drug impact (10%):
Mothers use androgen, anticancer drugs, and stop the reaction in the early pregnancy.
Infected with certain viruses or toxoplasma during the first trimester.
Prevention
Congenital absence of vaginal prevention
1, the mother avoids the use of androgen, anticancer drugs, and reaction stops in the early pregnancy.
2, enhance physical fitness in early pregnancy, to avoid infection with viruses or toxoplasma.
Complication
Congenital absence of vaginal complications Complications uterine dysplasia
1, with or without uterine dysplasia, if the uterus is abnormal, after puberty, the primary amenorrhea, uterus young or deformed, if the uterus is normal, is the emergence of primary amenorrhea with periodic abdominal pain, uterine hemorrhage, Increased uterus, sexual life disorders.
2, with ovarian hypoplasia, secondary sexual development is not complete, short stature, neck, elbow valgus and other deformities.
Symptom
Congenital absence of vaginal symptoms Common symptoms Sexual intercourse difficulties Abdominal pain Abdominal hemorrhage
Most congenital vaginal patients have only a completely occluded vaginal vestibular mucosa in the normal vaginal opening, no vaginal marks, and some patients have shallow depressions in the vestibule of the vagina, and some have a blind end vagina shorter than 3 cm. Often accompanied by no deformity, in the normal uterus, only see a slightly thickened case-like tissue, located in the middle of the broad ligament, about 1 / 10 patients may have part of the uterus development, and functional endometrium, After puberty, due to menstrual blood retention, periodic abdominal pain, no menstruation or until after marriage due to difficulty in sexual intercourse diagnosis and found.
Examine
Congenital non-vaginal examination
Abdominal plain film: The density of organs in the abdominal cavity is soft tissue density, lacking natural contrast. However, when an organ develops calcification due to disease, or has X-rayed foreign bodies, stones, or free gas in the abdominal cavity, and the gas or liquid in the intestinal lumen increases or the intestine expands, density will appear. The difference is shown on the photo.
Abdominal CT. The plain scan CT is generally a cross-sectional scan, and the auditory sputum line is mostly used as a baseline, and the scan is continuously performed upward or downward. Contrast agents commonly used in enhanced CT scans are 60% diatrizoate, 1.5 to 2.0 ml per kilogram of body weight. Anyone with a history of allergies and heart and kidney failure is banned from 60% diatrizoate.
Diagnosis
Congenital vaginal diagnosis
Diagnose based on
1. Prepuberty is often neglected. If you check carefully, with or without hymen, there is a shallow depression or short shallow vagina in the mouth of the hymen.
2. With or without uterine dysplasia, if the uterus is abnormal, puberty is characterized by primary amenorrhea, uterus young or deformed, if the uterus develops normally, there is primary amenorrhea with periodic abdominal pain, uterine hemorrhage, The uterus is enlarged.
3. Sexual life disorders.
4. With ovarian hypoplasia, secondary sexual development is incomplete, short stature, neck, elbow valgus and other deformities.
It should be differentiated from primary amenorrhea and hymen atresia.
