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Congenital absence of the vagina

  Congenital absence of the vagina is a congenital malformation, characterized by normal female karyotype, normal growth and development of secondary sexual characteristics, normal external genitalia, absence of the vagina, normal development of the uterus (only with bilobed remnants), small fallopian tubes, normal development and function of the ovaries, and Rokitansky-Kustner-Hauser syndrome patients with the most common features.

  Patients with testicular feminization (androgen insensitivity syndrome) are relatively rare. A few are true hermaphrodites or those with incomplete gonadal development. Congenital absence of the vagina is the result of incomplete development of the mesonephric duct during embryogenesis and requires vaginoplasty. During the process of human embryonic development, regardless of male or female, there are two pairs of longitudinal tubes, the mesonephric duct and the mesonephric ductal side. The longitudinal part of the mesonephric ductal side develops into the fallopian tube; the transverse part of the middle segment develops into the fundus and body of the uterus; the longitudinal part of the distal segment develops into the cervix and upper segment of the vagina. The most distal part of the two sides of the mesonephric ductal side merges and extends to the back of the urogenital sinus, forming a mesonephric nodule at the third month of embryogenesis. The two mesonephric nodules connect to form the lower segment of the vagina. Congenital absence of the vagina is due to abnormal development of the upper segment of the vagina, that is, the two sides of the mesonephric ductal side do not extend to the distal end after merging.

Contents

1. What are the causes of congenital absence of the vagina
2. What complications can congenital absence of the vagina easily lead to
3. What are the typical symptoms of congenital absence of the vagina
4. How to prevent congenital absence of the vagina
5. What laboratory tests need to be done for congenital absence of the vagina
6. Dietary taboos for patients with congenital absence of the vagina
7. Conventional methods of Western medicine for the treatment of congenital absence of the vagina

1. What are the causes of congenital absence of the vagina?

  Congenital absence of the vagina is caused by the failure of the distal part of the mesonephric duct to extend downward during embryonic development, so it often occurs with incomplete or undeveloped uterus, but the ovaries generally develop normally. Most reports indicate that the external genitalia of such patients are normal, with or without a hymen, and there is a shallow depression or a short shallow lower segment of the vagina. It is often not discovered before puberty, and is diagnosed when primary amenorrhea or sexual intercourse difficulties occur during puberty.

  This disease is not common, and the incidence has not been reported. There is also no obvious epidemic area. The etiology mainly includes the following aspects:

  1. Chromosomal abnormalities.

  2. Androgen insensitivity syndrome.

  3. Use of androgens, anticancer drugs, thalidomide, and other drugs during the early stages of pregnancy by the mother.

  4. Infection with certain viruses or toxoplasma during the early stages of pregnancy.

2. What complications can congenital absence of the vagina easily lead to?

  Complications of congenital absence of the vagina:

  1. With or without abnormal uterine development. If there is abnormal uterine development, it may manifest as primary amenorrhea after puberty, with a small or malformed uterus. If the uterus develops normally, primary amenorrhea accompanied by periodic abdominal pain, uterine hematometra, uterine enlargement, and sexual dysfunction may occur.

  2. Those with incomplete ovarian development have incomplete secondary sexual characteristics, such as short stature, webbed neck, and radial deformity of the elbows.

  We must grasp the complications of congenital absence of the vagina, as understanding these complications can help everyone actively avoid their occurrence. The aforementioned complications of congenital absence of the vagina are relatively common.

3. What are the typical symptoms of congenital absence of the vagina

  The vast majority of patients with congenital absence of the vagina have completely occluded vaginal vestibular mucosa at the normal vaginal orifice, without vaginal trace. Some patients also have shallow depressions in the vaginal vestibule, and a few have blind-ended vagina shorter than 3 cm. They often have no malformations, and only slightly thickened stripe-like tissue can be seen in the normal position of the uterus, located in the middle of the broad ligament. About 1/10 of patients may have partial development of the uterine body and functional endometrium. After puberty, due to menstrual retention, there may be periodic abdominal pain, amenorrhea, or until after marriage, due to difficulty in sexual intercourse, to seek medical examination and be found out.

4. How to prevent congenital absence of the vagina

  Grasping the preventive methods for congenital absence of the vagina is the best way for everyone to stay away from the threat of the disease. Only by understanding the preventive methods for congenital absence of the vagina can everyone actively control this disease. So, what are the preventive methods for congenital absence of the vagina? The following is a professional explanation given by experts.

  When performing vaginoplasty for congenital absence of the vagina, venous pyelography should be performed before surgery to be vigilant about the existence of the aforementioned anomalies and avoid surgical injury. In addition, about 10% of patients with this disease have skeletal malformations, mainly including spinal bifida, sacral insufficiency, vertebral fusion, and non-segmented spine.

  Preventive methods for congenital absence of the vagina:

  1. Avoiding the use of androgens, anticancer drugs, thalidomide, etc. in the early pregnancy of the mother;

  2. Strengthening physical fitness in the early pregnancy to avoid infection with viruses or toxoplasmosis.

  The above-mentioned preventive methods for congenital absence of the vagina are relatively common. It is required that everyone, after analyzing the preventive methods for congenital absence of the vagina, must actively seek treatment for such diseases in regular hospitals to ensure everyone's health and safety.

5. What laboratory tests are needed for congenital absence of the vagina

  1. Gynecological examination: Fore庭凹陷 examination method (or called fore庭 pressure mark depth): In addition to observing the vulva morphology and development, gloves should be worn, and the index finger should be coated with paraffin oil and gently pressed on the fore庭 of the vagina, gradually pressing deeper to determine the depth of the fore庭 depression, which is significant for determining the choice of surgical method.

  2. Anorectal examination: Determine the presence of the uterus, uterine size, and whether there is tenderness. Generally, the primordial uterus on both sides of the pelvic wall is difficult to palpate, and only the trace uterus at the posterior aspect of the bladder can be palpated as a nodular small mass. Palpation of an enlarged uterus with tenderness is mostly due to uterine menstrual retention, which is a rare condition.

  3. Ultrasound examination: It is the most important and simplest auxiliary examination, which can determine the presence of vaginal air line, uterine size, ovarian size, and the existence and position of the kidneys.

  4. Other examinations: A few patients need to undergo radiographic contrast or CT scans to clarify abnormalities in the urinary system or skeletal spine and other aspects.

6. Dietary taboos for patients with congenital absence of the vagina

  Rokitansky-Kustner-Hauser syndrome patients with normal female karyotype, normal growth and development of female secondary sexual characteristics, normal vulva, absence of vagina, uterus development (only residual bicornuate), small fallopian tubes, and normal development and function of ovaries are the most common.

  Surgery is the preferred treatment method for many congenital absence of the vagina patients, but the key is to do a good job of postoperative care. Diet is the most important issue that patients should pay attention to after surgery.

  1. After the operation, patients should not eat too much indigestible food because the patient's body is weak, and rapid recovery is the most important thing. Therefore, food should be taken according to various nutritional combinations.

  2. Avoid eating cold, greasy, fried, salted, smoked foods, and also avoid eating spicy and刺激性 foods in large quantities.

  3. Avoid irritants. Two weeks after the surgery, although the recovery is good, the body's resistance is still weak during this period, and the risk of inflammation still exists, so it is necessary to avoid irritants such as mutton, fish and shrimp, beef, dog meat, etc.

7. Conventional Western Treatment Methods for Congenital Absence of the Vagina

  The current main treatment for congenital absence of the vagina is surgical reconstruction.

  If the tissue elasticity is poor and it is difficult to succeed, it has been basically abandoned and is rarely used now. The main method of surgical treatment is to separate the urethra-bladder and rectum to form an artificial cavity, and use different methods to find an appropriate cavity wound cover to reconstruct the vagina. In the past, the most commonly used method was the transplantation of thick free skin grafts from the patient's own body, but after the operation, the patient needs to use a rigid vaginal mold for a long time to expand the artificial vagina to prevent the stricture of the artificial cavity covered by the transplanted skin grafts, which increases the patient's pain and brings great inconvenience to work and life.

  Moreover, the characteristics of the skin and mucosal tissues are too different, which does not meet the physiological requirements and is its biggest shortcoming. Using the labial skin flap to shape the vagina often destroys the normal vulvar morphology and is often refused by patients. Using sigmoid colon or ileal segments to reconstruct increases the complexity of the surgery.

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