External genital deformities
Introduction
Introduction to external genital malformations External genital malformations include male external genital malformations and female external genital malformations. Male genital malformations include many, some congenital malformations only affect fertility, such as simple vas deferens, and some may affect sexual function, some deformities do not look as serious as phimosis, but improper treatment can also cause serious as a result of. Female external genital malformations are generally more common in female pseudohermaphroditism, congenital hymen atresia, congenital absence of vagina and other diseases. External genital malformation can have a great impact on fertility, and even if it is treated, it is a very small number of patients who can restore fertility. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: Infertility
Cause
Cause of external genital malformation
The cause of female external genital malformation:
1, congenital adrenal hyperplasia, cortical hormone synthesis in the absence of 21 hydroxylase or 11 hydroxylase, the emergence of female masculinity.
2, pregnant mothers have masculine tumors, such as ovarian cysts with excessive androgen secretion, luteal tumors or ovarian male cell tumors, or benign adrenal tumors. The mother showed masculine performance during pregnancy, and the fetus also had female genital external genital morphology.
3, the use of androgen drugs in the mother during pregnancy can also cause masculine performance of female fetuses.
Causes of male external genital malformations:
Male external genital malformations are congenital diseases. Abnormal gene expression caused by various factors.
Prevention
External genital malformation prevention
Some genital malformations are difficult to correct, but some patients, if found in time, can be surgically treated early in childhood. However, often because of the lack of knowledge in this area, and because of the special nature of the reproductive system, people are often ashamed to mention or fear to go to the hospital for examination. As a result, some patients missed the opportunity to treat and can only regret for the rest of their lives. At present, domestic premarital examinations are not universal, and if they are not carefully examined, they will also affect the stability of marriage and family. Therefore, advise parents not to neglect to carefully observe the growth and development of children. Once you find a problem, take your child to the hospital for examination. In general, some congenital malformations only affect fertility, while others may affect sexual function. Some malformations don't look serious, but improper handling can also have serious consequences. In general, the treatment of congenital malformations of the reproductive organs should be fine and should not be rough, and should not be taken sooner or later.
Complication
External genital malformation complications Complications, infertility
External genital malformations have a great impact on fertility, and common complications are infertility.
Symptom
Symptoms of external genital malformations Common symptoms Testicular hypoplasia No testicular vaginal atresia Penile abnormalities Gendery deformity Penis short vaginal adhesions or even atresia
Female external genital malformations:
1, the majority of congenital non-vaginal patients in the normal vaginal opening only the fully occluded vaginal vestibular mucosa, no vaginal marks. Some patients also have shallow depressions in the vestibule of the vagina, and each has a blind end vagina shorter than 3 cm. Often accompanied by no deformity, only a slightly thickened strip-like tissue is seen in the normal uterine position, located in the middle of the broad ligament. About 1/10 patients may have partial uterine body development, and have 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.
2, the external genital area is partially masculine, from the middle of the clitoris to the posterior part of the labia and the penis. The degree of masculinity depends on the amount of morning and evening and androgen when the embryo is exposed to high androgens.
3, the hymen is locked.
Male genital malformations:
1. Testicular malformation
(1) An abnormal number
1 There is no testicular disease, probably due to some factors in the embryonic development period, the testicular differentiation is incomplete, so the testicular atrophy and degeneration, but because there are some interstitial cells, the patient may have male external genitalia and appearance. Pay attention to the identification of bilateral cryptorchidism. This disease is characterized by elevated blood luteinizing hormone to chorionic gonadotropin, plasma testosterone levels are not elevated, and more with vas deferens epididymis.
2 single testicular, most do not require special treatment, the purpose of surgical examination is to find cryptorchidism that may exist in the abdominal cavity to prevent cancer.
More than 3 testes, which means 3 or more testicular size abnormalities.
(2) abnormal size
1 testicular due to hypoplasia and less than normal, more than isolated symptoms.
2 testicular due to hyperplasia, such as testicular teratoma.
3 congenital atrophy.
(3) Abnormal position
1 Testicular fusion refers to the fusion of the testis in both the abdominal cavity or the scrotum. It is easy to be mistaken for cryptorchidism or single testicular disease combined with renal malformation.
2 cryptorchidism refers to a deformed state in which the bilateral or unilateral testes do not fall into the scrotum after 2 months of birth.
3 ectopic testicular finger testicular deviation from the normal way in the process of development decline, did not enter the scrotum and is located in the pubis, penis, thigh, perineum, groin and other parts.
4 abnormal attachment of testicular epididymis can make sperm through the production of obstacles to cause infertility, the two are not well connected, it is easy to occur testicular torsion and even tissue necrosis due to insufficiency of blood supply.
2, vas deferens malformation
Can be divided into congenital loss and ureteral phase traffic and repeated vas deferens and other situations.
If other abnormalities are not complicated, only the simple vas deferens is absent, the patient's sexual desire and sexual function are normal, and the only symptom is infertility. The clinical manifestations are azoospermia but serum serum levels are normal. Testicular biopsy can confirm the possibility of surgical treatment of the vas deferens stump with the epididymis if possible.
3, abnormal penis
Can be divided into size anomalies and location abnormalities and other malformations at the same time.
(1) complete penis loss
More combined with urethral malformation, treatment is quite difficult, the past practice of penileplasty, the effect is not good, it is best to remove the testis, for urethral vaginoplasty, to maintain female sexual characteristics with estrogen after puberty.
(2) latent penis
Due to short development, it is covered by fat in the pubic scrotum and pubis. As the fat is reduced during development, the penis is exposed, and the penis can be "liberated" by plastic surgery to return to normal.
(3) congenital penile twist
When the penis is twisted, the direction of the urethra changes. A few people may have symptoms such as occult pain during erection. This disease is also easy to combine with abnormal erection.
(4) Double penis can be arranged in parallel or in tandem.
(5) The penis is too big or too small
When it is rare, it can be used for truncation of orthopedics. Too small a small cause should be properly treated for the cause.
(6) phimosis
It can account for more than 25% of boys, but adult phimosis is significantly less than that of adolescents. It can be divided into physiological phimosis pseudo-phimosis (foreskin is too long) true phimosis and incarcerated phimosis.
(7) Upper and lower urethra
The latter is more common in the former and can affect erectile function.
4, prostate congenital gland congenital malformation
(1) Prostatic hypoplasia
More with other sexual organ dysplasia combined with the presence of prostate cysts can be complicated by dysuria.
(2) seminal vesicles lacking infertility
Preliminary judgment can be made from the extremely low level of refined berry sugar and the small amount of semen, and it is diagnosed according to vas deferens seminal stenosis.
Examine
Examination of external genital malformations
an examination:
External genital malformations generally do not require special examination, just a diagnosis can be diagnosed.
Diagnosis
Diagnosis and diagnosis of external genital malformation
diagnosis:
1, medical history and physical examination
The patient should first be asked if the mother had a history of high-efficiency progesterone or danazol in the first trimester, whether there was a similar history of malformation in the family, and detailed physical examination. Pay attention to the size of the penis, the position of the urethra, whether there is vaginal and uterus, rectum, abdominal examination and uterus indicate multiple female pseudohermaphroditism, but the possibility of true hermaphroditism should be excluded. If the groin and gonads in the groin, labia majora or scrotum are without exception, testicular tissue, but true hermaphroditism cannot be ruled out.
2, laboratory inspection
The karyotype is 46, XX, the blood estrogen is low, the blood androgen is high, and the urinary 17-ketone and 17-hydroxyprogesterone are high, which is congenital adrenal hyperplasia. The karyotype is 46, XY, the blood FSH value is normal, the LH value is increased, the blood testosterone is in the normal male range, and the estrogen is higher than the normal male but lower than the normal female value, and the androgen insensitivity syndrome.
3, gonad biopsy
For true hermaphroditism, gonad biopsy is often performed by laparoscopic or laparotomy to confirm the diagnosis.
