Lecture of genital deformities

2021-03-19 12:00 AM

Genital stem cells appear on the wall of the yolk sac and then into the embryo along the mesenteric lining of the spine and form the genital crest before the kidney

Recall embryology

Genital differentiation is also a part of the urinary tract that allows for the interpretation and classification of genital malformations.

Week 3 - 17, after the differentiation of the urinary tract completely will begin to differentiate the genital tract. Including these two processes can be divided into 4 phases:

Urinary phase

The first phase contains only the urinary organs (weeks 3, 4 and 5):

Wolff tube forms and progresses towards the waste hole to create the secretory route of the primary kidney or the kidney.

Ureteral germ development, starting at the end of the Wolff tube directed from the renal stem or the posterior, will be the kidney later.

Urogenital phase

The second phase of both urinary and genital (weeks 6, 7, 8 and 9):

Perfecting the urinary tract by ascending and rotating the kidneys. Starting from week 9, the differentiation of the urinary tract is temporarily considered complete and cannot be directly interfered with by any malformation factors.

Create a Muller tube and progress to the urogenital sinuses.

The ovaries develop completely independent of the genital tract in terms of the fetus. Genital stem cells appear on the wall of the yolk sac and then into the embryo along the spinal mesenteric and form the pre-medial genital crest. At 8 weeks, the original genital cord was formed, which degenerates and is replaced by the fibrous organization that makes up the ovarian medulla. A new proliferation wave of the epithelium of the body cavity produces a new genital cord containing genital stem cells that turn into oocytes, which multiply rapidly. From month 3, the ovaries and fallopian tubes move into the subframe.

During the head-to-butt development phase, the Muller tube is divided into 3 phases:

The first segment will become the Fallope, parallel to the Wolff tube, outside the Wolff tube.

The segment on the inguinal ligament where the groin canal and the ovary are forming, the Muller tube tilted inward and crossed the Wolff tube in the abdomen. Then its middle part will be the uterine horn and later the uterus. The Wolff tube is parallel to it inward.

On the white line between two Muller tubes approaching each other, they do not stick together and become a vertical ladder at the end or segment of the uterus. Mr. Wolff and Muller tubes are always parallel, all four tubes are on the same plane that makes up the "genital cord".

This stage should be noted that the stage has differentiated both urinary and genital.

Muller tube bonding phase

The third, genital, sticky phase of two Muller tubes starts at weeks 10, 11,

This phase creates the morphology of the external genital tract. External genital organs arise from the genital tubers to form clitoris, genital folds, small lips, large lips.

Vaginal implantation is very rapid during the first day of week 10.

Adhesion of the uterine segment by gradually thickening from low to high within its triangular limit, the tap segment does not stick together.

Phase loss septum

The fourth stage, the loss of the adhesive septum of the Muller tube (weeks 13,14,15,16 and 17) the septum fades from the waist before the end of the sticky phase, low tachycardia, slow in the advanced stage. This phase creates a morphology in the genital tract.

Classification of genital malformations

Grouping genital malformations according to the stages of genital differentiation

Between weeks 3 and 6, the Muller tube is not available yet. One-keratinized uterine malformation accompanied by unilateral lack of a kidney is more common if the abnormality occurs early.

Week 6, Muller tube develops. If the malformation appears 6-9 weeks, the uterus causes a one-horny prosthetic uterus. Opposite uterine germs are present if the abnormality occurs late. If soon there will be related to the kidneys.

Weeks 10-13, two Muller tubes move closer to the mid lane. The form of the uterus involves a combination of these two tubes, causing a bipedal uterus.

After week 13, the malformation associated with a septum-loss phase produces a septum uterus. Urinary malformations cannot be seen in this phase.

Classification according to the location of genital organs

External genital malformations (vulva, hymen, perineal layer) :

Rarely, usually detected at birth. Paediatricians know better than gynaecologists.

Sticky lips: systematic detection at birth. If so, treat it immediately, using a separate instrument. When the age is detected, surgery must be performed separately.

The hymen does not puncture: should be detected at birth as if it were anus defects. In the new-born can cause painful mass in the subframe due to menstrual haemorrhage in the new-born, swelling hymen. Ultrasound allows to predict vaginal uterine fluid retention. It is necessary to incision the hymen. After puberty, the hymen does not puncture manifested by amenorrhea, abdominal pain, normal secondary genital features. Vaginal examination can be definitive.

Vulvar malformation:

Male and female prosthetics: the external genitals are more or less the same with men while having ovaries.

Male and female prosthetics: the patient has testicles but has the same sexual characteristics as female (female testicles).

Vaginal abnormalities:

No congenital vaginal defects due to lack of development of Muller's vaginal section, vulva is normal, vestibule slightly recessed, uterus is barely present (Rokitansky's syndrome) but may be normal, even still vigorous activity causes menstrual blood stasis in the uterus.

Transvaginal septum: usually 1/3 above or in the middle of the vagina. The septum can be punctured, so it doesn't cause menstrual bleeding or pain during sex. If no perforation causes menstrual stasis, causing primary amenorrhea. Septum surgical treatment.

Septum along the vagina: septum can be complete or partial, often associated with uterine abnormalities (1/6 cases of uterine malformation) or urinary tract. There may be no symptoms, be found accidentally during routine gynaecological examination, or cause pain during intercourse, obstetric problems, fluid retention in case of vaginal bladder. Treatment with septum removal surgery.

Bow cabinet abnormality:

Classification of Musset:

An undeveloped uterus (no uterus).

The wombs.

The uterus has a septum.

The uterus information.

Infertility uterus: a very small uterus in a woman of sexually active age, diagnosed at the time of examination for infertility, consecutive miscarriages. When taking a baby's basilar uterus, 40mm of quadrature / body of uterus reversed. The uterus may be narrow or narrow.

Ovarian abnormalities:

Most developmental abnormalities:

No complete or near complete ovaries (Turner syndrome).

There is no one ovary associated with no ovary and no kidney on the opposite side.

Excess ovaries.

Abnormal ovarian position: may be located in the lumbar, pelvic fossa or groin.

The testicular ovarian-type abnormal structure is responsible for androgynous conditions.

Tubular abnormality:

Does not have a whole two oviduct.

Does not have a fallopian tube, half uterine body, or an ovary on the same side.

Excess of the oviduct, excess of the tap.

Classification according to clinical

Painless secondary amenorrhea: Rokitansky syndrome, without a cervix and waist.

Painful secondary amenorrhea: narrow vagina. Septum across the vagina, without a cervix.

Unilateral dysmenorrhea: a bipedal uterus, cervix, uterus with an unbalanced septum, a one-horny pseudo uterus.

Recurrent preterm pregnancy loss: the uterus has a septum, the uterus with two horns.

Pain during intercourse: vaginal septum, hymen abnormality.

Discovered by chance if the clinical picture is not clear, slowly.

Diagnose

The reason to see a doctor

Out of pregnancy, genital malformation can be detected for the following reasons:

Primary amenorrhea.

Primary dysmenorrhea cannot find the cause, the treatment does not work.

Infertility.

Continuous premature birth.

Pain during intercourse.

Vaginal septum.

Vaginal wall malformation: bulging.

No vagina.

The cervix.

There is no cervix.

During pregnancy it is possible to diagnose an malformation when:

Recurrent preterm birth miscarriage.

Constantly abnormal pregnancy.

Accidents in the vegetable basket: bleeding or confined vegetables.

Serious complications are rare: internal bleeding caused by rupture of the underdeveloped uterine horn, pseudo-monk, or acute abdominal syndrome due to uterine torsion (no contraceptive ligaments).

Uterine malformations and reproductive dysfunction:

Infertility due to malformations, uterine minimization.

Pregnancy causes miscarriage, premature birth due to partial deformities such as uterine cleft palate, premature rupture of amniotic fluid due to abnormal position.

Labor: abnormal birth, mechanical difficulty delivery, caesarean section and procedure.

Vegetable flakes: bleeding, vegetables left.

Test

In theory, there are many diagnostic tests but indicated for each specific case:

Hysteroscopy: assesses uterine condition but does not allow to confirm the type of malformation.

Uterine dysplasia is often associated with malformations. The obstetric prognosis is very poor.

This test also shows the condition of the oviduct and cervix.

Hysteroscopy: allows to probe the uterus but has not yet accurately diagnosed the malformation in all cases. However, it is a necessary test. Allows treatment of the uterine septum.

Ultrasound: allows to evaluate the outside and in the uterus, accessory condition.

Laparoscopy: allows to confirm the diagnosis of uterine malformation, shows the type of malformation and also damage coordination to have a correct prognosis in fertility: endometriosis. However, this test is not systematic. It is necessary in the case of a unicorn uterus to determine what type of malformation is and the uterus has a septum that is not clearly diagnosed under ultrasound.

UIV: This test has lost its role since the ultrasound can see the fovea, diagnose the kidney misplaced, the kidney failure. In cases of minimized renal failure, UIV is still necessary to determine the anatomical status and remaining kidney function.

Testing is necessary in case of early malformations according to differentiation of uterine hypoplasia, Rotansky syndrome, bicamolar uterus, cervix.

In other forms (bicamolar uterus, uterus with septum) this test is not necessary because there is no coordinated urinary malformation.

Test indications:

In addition to laparoscopy to confirm the type of malformation, ultrasound is a convenient test that is essential in a diagnostic test for vaginal uterine malformation.

Hysteroscopy is not indicated in the absence of vagina, does not allow differential diagnosis of the unicorn and monaural uterus. It allows to diagnose a double uterus but does not distinguish between a bicamolar or septum uterus.

Hysteroscopy also has a lot of consideration, although nowadays with technical advances, it is possible to cut the uterine septum when it is done.

Ultrasound allows to distinguish all types of malformed uterus (theoretically). Allows confirmation of the Rokitansky syndrome diagnosis and avoid unnecessary laparoscopy. It also allows for the diagnosis of true monocular and monophilic uterus, which is usually diagnosed by laparoscopy and distinguishes the twin uterus (septum or bicamen).

However, it is not easy, it must be guaranteed sharpness by an experienced doctor, modern machines.

There is no uterus

An undeveloped uterus is an uterus that is not formed due to a lack of one or two Muller tubes, often with disorders of differentiation of the genitals to obey 3-8 fetuses.

Classification of the uterus is not bilateral, complete or incomplete, and is not unilateral.

The uterus does not develop completely on both sides

This form does not exist because it does not have bilateral kidneys.

The uterus is not completely bilateral (also known as Rokitansky - Kuster - Hauser syndrome)

This type of uterus and vagina does not develop because there is no growth of a Muller tube in the vagina.

Urinary abnormalities (lost place or no kidney development): common (33%). The clinical picture is primary amenorrhea.

Suspected diagnosis in the absence of a vagina. Diagnosis is confirmed based on ultrasound; The uterus is not seen behind the bladder. The uterine position only found relics of the fibrous strip. See normal ovaries. Lumbar fossa sometimes shows no or unilateral stray kidney. A definitive laparoscopy is confusing but is unlikely to be necessary because definitive trauma can be based on ultrasound. The only treatment method is vaginal imaging that allows for almost normal sex.

In a few cases there was a hollow in the vestibule, dilated, causing the vestibular to deepen enough to allow intercourse (Frank method).

In other cases, surgery is required. It is possible to perform a simple dissection procedure between the bladder and rectum and then skin or peritoneum graft, in which case it is possible to mechanically separate by placing an oval object to induce relaxation (Vecchietti procedure); making a colonic prosthesis when the above surgeries fail. Postoperative incubation is essential to ensure surgical results in case of separation.

The procedure is only conducted when there is a need to have sex.

The uterus does not fully develop to one side

Because there is no single-sided Muller tube. It is a true single-horned uterus. Hysteroscopy showed that half of the uterus was deviated to one side, like vocal fusion. There is only one uterine horn, the two sides are symmetrical convex connecting with the oviduct. Obstructive hysteroscopy fixes the tube of the uterus. When only one ovary opening confirms the unicorn uterus.

Ultrasound in the uterus cut along the uterus is very skewed to one side, the mark is very tapered, for the image of the semicircle. When transect, cannot see the opposite side can think of a body of the uterus.

Laparoscopy confirmed the diagnosis. When a true single-horned uterus can be diagnosed, a laparoscopy is not required.

The uterus does not develop completely on one side

One-horned pseudo uterus is different from the anterior body by having relics of the uterus, opposite lateral appendages. Connected to the quadrilateral one horn by a circular wire.

Hysteroscopy shows an image of the unicorn uterus.

Hysteroscopy also shows a single-horn uterus. The uterine cavity is long, with only one batch of fallopian tubes.

Ultrasound allows to confirm when the coupe cuts across, seeing a u on the opposite side. In the event that the horn has an opening into the uterus, a hose-tightening is necessary to prevent the fetus developing in the horn from developing as the sperm moves into the pregnant abdomen.

The single-horned uterus is divided into 3 bodies:

Type 1: The opposite uterus is just a fibrous relic.

Type 2: miniature, closed uterus.

Type 3: an open, underdeveloped uterus with a fallopian tube, risk of pregnancy.

The uterus hose

Late differentiation disorder between 10-13 weeks of the fetus, when Muller tube approaches the middle line to form a bipedal uterus, it is possible to completely create a bicamolar uterus, two cervix or not completely create a uterus. two horns, one womb.

Two-horned uterus, two cervix

Two-horned uterus, two cervix have one-sided amenorrhea because the cervix is ​​not growing and the opening of the cervix is ​​poured into the vagina.

Clinically suspected diagnosis in young girls, primary dysmenorrhea accompanied by a tumor in the subframe followed by in the abdomen.

Ultrasound is the most important test, as it is difficult to do a physical examination because it is hindered by a vaginal tumor. Ultrasound results showed that there is a large uterus containing fluid in the abdomen, sound drum, and below there is a fluid bag that is half of the vagina.

On a coupe, cutting the opposite half of the uterus is normal.

Always see a kidney that does not develop inside the vagina.

Hysteroscopy showed limited results, showed a single-horned uterus, after which the uterus was congested with unenhanced menstrual bleeding.

Hysteroscopy is not necessary for diagnosis. The visible picture is the unicorn uterus. Laparoscopy clearly showed a subframe tumor and in the abdomen was a vaginal hematoma, the cervix had a picture of a big head with two small ears, which were 2 uterus (Musset). In addition, there are often endometriosis lesions of the endometrium due to reflux of blood. That is one of the factors in obstetric prognosis. Treatment for this type of deformity is surgery, a vaginal beak to drain the blood of the uterus with blood stasis or a removal of the uterus that is congested and the vaginal pouch.

Uterus 2 horns, 2 necks of the quadrangular Clinically sometimes there is a vaginal septum. Hysteroscopy confirmed completely two uterus, but could not determine any type. It can be seen that two separate quadrangles separated from each other by partitions. This is also difficult to differentiate in hysteroscopy.

The ultrasonic cross-sectional coupe identifies these two structures by the axis of the two uterine chambers. There is also a cavity behind the bladder between the uterus. This constant is especially evident when the ligament between the back of the bladder and the front of the rectum is the ligament of the rectal bladder. Laparoscopy confirms the diagnosis but is not necessary.

Two-horned uterus, a quartet

No symptoms, just discovered by accident on a uterus or ultrasound.

Laparoscopy, laparoscopy with predetermined ultrasound. In case of recurrent late miscarriage, a uterus may be indicated.

The uterus has a septum

Uterine malformation is most common (40%). The uterus has septum when late differentiation disorder (weeks 13-18). Never accompanied by urinary abnormality. Stage malformation that loses the septum of the uterus half together in the midline. The septum can be complete, partial or partial in the cervix or body of the uterus.

A scan of the uterus looks like a two-horned uterus. Hysteroscopy confirmed septum. However, the two tests above do not allow to confirm the malformation accurately: the bipedal uterus or the uterus with septum. An ultrasound is the most needed test. When making the coupe cut across, it is clear that the two chambers are separated by the middle line, the sound characteristics, the parallel axis (gun barrel image).

Laparoscopy confirms the diagnosis when the uterine appearance is normal but slightly broad at the base of the uterus.

In the case of recurrent miscarriages, preterm delivery is performed by scissors or YAG laser resection of the uterus. During the guided procedure under ultrasound. An angioplasty (Bret-Palmer procedure) is indicated to cut the septum in two planes, using four pieces to expand the uterine cavity. Currently not using this procedure because of progress in endoscopy.

The uterus is connected

This case is very rare, is an unusual result of a septum loss. Correct diagnosis is based on a hysteroscopic scan of the ovary when a vertical X-shaped image is visible.

Complete septum of the uterus.

Two-horned uterus, two necks with a tight vagina.

Two-neck uterus, body septum.