How to diagnose and treat abnormal development of female genital organs in adolescence

  During the formation, differentiation and development of female reproductive organs, developmental abnormalities may occur in various parts of them. Some of these developmental abnormalities may lead to obstruction of the birth canal, and various acute illnesses may arise when menstrual blood is not discharged normally after the development of adolescent females. To deal with these problems, attention should also be paid to the presence of abnormal development of the urinary tract, because the female genital organs and the urinary organs are at the origin, and about 10% of those with abnormal development of the urinary tract are accompanied by abnormal development of the genital organs, and even by abnormal development of the digestive tract. It is important for the correct diagnosis of abnormal development of female genital organs in adolescence to reduce unnecessary open-heart surgery due to misdiagnosis. This article describes the diagnosis and treatment of various abnormal development of female genital organs during puberty.  1.Hymenal atresia (mi perforate hymen) Hymenal atresia, also known as imperforate hymen, is caused by the failure to cavitate the urogenital sinus tissue at the end of the vagina during development. The hymenal atresia isolates the vagina from the outside world and prevents the discharge of vaginal secretions or menstrual blood from the first menstrual flow, which accumulates in the vagina and sometimes the menstrual blood may flow backwards through the fallopian tubes to the abdominal cavity. If incision is not made in time, the repeated menstrual flow will increase the accumulation of blood and develop into blood in the uterine cavity, which may lead to atresia of the umbilical end of the fallopian tube due to blood adhesions.  In most patients, periodic lower abdominal cramps occur in adolescence and worsen progressively. In severe cases, it may cause anal or vaginal distension and frequent urination. On examination, the hymen is seen to be bulging, with a purplish-blue surface; on anal examination, a vaginal bulge can be found, which is convex to the rectum; and a pelvic mass can be found, and the hymen is more obviously bulging outward when the mass is pressed with a finger. Occasionally, young girls have been seen because of a large amount of mucus retention in the vagina and lower abdominal cramps, causing the hymen to bulge outward. Ultrasound examination of the pelvis reveals fluid accumulation in both the uterine cavity and the vagina. The diagnosis of hymenal atresia is not difficult.  Treatment is based on surgical incision as soon as possible. The hymen is first punctured with a thick needle to extract the old blood and then an “X” shaped incision is made to drain the accumulated blood.  2. Atresia of vagina is caused by the failure of the genitourinary sinuses to participate in the formation of the lower part of the vagina. The anatomical features of vaginal atresia are divided into two types as follows.  Type I vaginal atresia, in which the lower part of the vagina is atretic, while the upper part of the vagina, the cervix and the uterine body are normal.  Type II vaginal atresia, in which the vagina is completely atretic, is usually combined with cervical dysplasia, uterine hypoplasia or uterine malformation.  The symptoms are similar to those of hymenal atresia, but the difference is that the mucosal surface is normal in color and does not bulge outward. The hymenal atresia has similar symptoms to hymenal atresia, but differs from hymenal atresia in that the mucosal surface is normal in color and does not bulge outward. In type II vaginal atresia, endometrial secretion is abnormal, symptoms appear later, menstrual blood tends to reflux into the pelvis, and endometriosis often occurs. Magnetic resonance imaging and ultrasonography can help in the diagnosis.  Once the diagnosis is clear, it should be surgically removed as soon as possible. Surgery is performed to release the vaginal obstruction and allow the menstrual blood to drain freely. The vaginal mucosa is first punctured with a coarse needle to extract the accumulated blood and then the atretic segment of the vagina is incised to drain the accumulated blood, the cervix is routinely checked for normalcy, the excess atretic fibrous connective tissue is removed and the wound is covered with the free vaginal mucosa. A vaginal mold is placed and the vagina is dilated periodically after the surgery to prevent vaginal contracture.  The transverse vaginal septum is the result of a lack of penetration or partial penetration of the urogenital sinus at the caudal end of the convergence of the two paramedian tubes. Transverse vaginal septum is rarely associated with abnormalities of the urinary system and other organs. It can be located anywhere in the vagina, but is more common at the superior and middle junction. A transverse septum without a hole is called a complete septum (see Figure 4), while a small hole in the septum is called an incomplete septum. Clinically, the septum located at the upper part of the vagina is mostly incomplete, while the lower part of the vagina is mostly complete.  Incomplete transverse septum with foramen ovale is asymptomatic in the upper part of the vagina, but in the lower part, it can affect sexual intercourse and the descent of the fetal first dew during vaginal delivery. Complete transverse septum has primary amenorrhea with periodic abdominal pain that is progressively worse. On gynecologic examination, the vagina is short or only the blind end is visible, and the cervix and body of the uterus are palpable on anal examination. A complete transverse septum may be palpated as a mass in the area equivalent to the upper part of the transverse septum due to retention of menstrual blood.  Complete transverse septum is first punctured with a coarse needle, blood is drawn out and then incised, excess atretic fibrous connective tissue is removed, the cervix is routinely checked for normality, the vaginal mucosa above the transverse septum can be separated and pulled downward to cover the trauma of the transverse septum and sutured to the vaginal mucosa below the septum, and the vagina is periodically dilated or vaginal molds are placed after surgery to prevent contracture. If an incomplete septum is not diagnosed before, during delivery, if the septum is thin, the septum can be cut when the septum is compressed by the descending fetal first dewlap and then removed after delivery. After septotomy, attention should be paid to the healing of the wound and prevention of contracture of the septal stump.  The cause of oblique vaginal septum syn-drome, also known as Herlyn-Werner-Wunderlich syndrome (HWWS), is still unclear and may be due to a blind end of the paramedian tube that does not extend down to the genitourinary sinus. Vaginal strabismus is often associated with ipsilateral urogenital anomalies, most often with double uterine bodies, double cervix and renal agenesis on the side of the strabismus.  There are 3 types of vaginal oblique septum: type I is an imperforate septum: the uterus behind the septum is completely isolated from the outside world and from the other side of the uterus, and blood accumulates in the posterior cavity of the septum. Type II is perforated oblique septum: there is a small hole of several millimeters on the septum, and the uterus after the septum is isolated from the other side of the uterus, and the menstrual blood drips out through the small hole, and the drainage is poor. Type III is non-porous oblique septum combined with cervical fistula: there is a small fistula between the two sides of the cervix or between the posterior septum and the opposite side of the cervix, and menstrual blood can be drained through the other side of the cervix on one side of the septum, and the drainage is also poor.  The three types of vaginal oblique septum syndrome with nonporous oblique septum combined with cervical fistula generally have normal menstrual cycles, all three types have dysmenorrhea, type I is more severe, usually with pain in the lower abdomen on one side; type II has a small amount of brown vaginal discharge or old blood dripping between menstrual periods, and purulent discharge with odor; type III has a small amount of blood during prolonged periods, and may also have purulent discharge. Cystic masses can be palpated on one side of the vaginal vault or vaginal wall during gynecological examination. Type I masses are hard and enlarged uterus can be palpated when blood accumulates in the uterine cavity; Type II and III cystic masses are less tense and old blood flows out when pressed. Ultrasound examination shows blood accumulation in the uterine cavity on one side, the presence of a cyst next to the vagina and the absence of the ipsilateral kidney. Hysterosalpingography with iodine oil is useful for type III to understand the posterior compartment. Treatment:First puncture with a coarse needle, extract the accumulated blood and then incise, remove the excess atretic fibrous connective tissue, routinely check whether the cervix is normal, do maximum septal resection, edge electrocoagulation to stop bleeding or oil gauze roll compression for 24 to 48 hours, generally no vaginal model needs to be placed after surgery.  5. Congenital atresia (congenitalabnormalof the cervix ) Congenital atresia is an abnormal development of the cervix due to hypoplasia or arrested development of the caudal end of the paramedian tube, which can manifest as cervical agenesis, cervical atresia, and congenital cervical canal stenosis.  Congenital cervical atresia may present with periodic abdominal pain after puberty due to accumulation of blood in the uterine cavity if the patient has a functional endometrium, and menstrual blood may also flow back into the abdominal cavity via the fallopian tubes, causing pelvic endometriosis. Magnetic resonance imaging and ultrasonography can help in the diagnosis. The cervix can be surgically penetrated to create an artificial uterovaginal access, but the success rate is low, so direct hysterectomy is recommended; if the artificial uterovaginal access surgery fails, hysterectomy is recommended.