Urogenital sinus. A method for reconstructing the vagina in girls with congenital dysfunction of the adrenal cortex when the proximal border of the urogenital sinus is located at or above the external urethral sphincter

The occurrence of congenital malformations of the female reproductive system can be quite easily explained if you know how the vagina and uterus develop. Each embryo has in the stage of differentiation both mesonephric ducts, from which the male genital tubules are formed, and paramesonephric or Müllerian ducts, giving rise to female organs.

In boys, a substance that inhibits the Müllerian structures inhibits the development of the paramesonephric ducts. In the absence of testicles, the mesonephric ducts regress and develop in a female pattern. The paramesonephric or Müllerian ducts develop from bilateral folds of the mesodermal epithelium.

Their proximal endings open into the abdominal cavity, being the forerunners of the fimbriae of the fallopian tube. They advance caudally, then travel ventrally to the mesonephric ducts and merge to form the uterovaginal anlage, which migrates caudally to the midline to adjoin the posterior wall of the urogenital sinus at 9 weeks of gestation.

The muscular wall of the future vagina develops from the surrounding mesenchyme. The junction of the Mullerian ducts with the urogenital sinus is called the sinus tubercle. From this point, paired endodermal synovaginal outgrowths develop between the urogenital sinus and the Müllerian ducts. The central part of this structure undergoes resorption, as a result of which the vaginal lumen appears, and the vaginal epithelium is formed from the peripherally located cells. This occurs at the 12th week of gestation.

The connection of the vaginal lumen with the urogenital sinus is the hymen, which persists until the late stages of fetal development, but in the end, normally, it usually breaks during the perinatal period.

Different opinions exist regarding the origin of the vaginal epithelium. Some researchers argue that the entire epithelium develops from the vaginal lamina. Others believe that the proximal sections of the epithelium represent the lower end of the merged Mullerian ducts, and the remaining sections are derivatives of the vaginal plate, that is, an outgrowth of the endoderm from the urogenital sinus. The dual origin more reliably explains the different levels of vaginal obstruction observed clinically.

From this point of view (dual origin), it can be assumed that low obstruction occurs as a result of a failed “breakthrough” between the urogenital sinus and the Müllerian ducts, and a higher obstruction can be explained by the lack of connection between the Müllerian ducts and the vaginal lamina. Duplications and unilateral agenesis or obstruction represent a lack of fusion between the Müllerian ducts or a unilateral developmental disorder.

The vaginal and cervical eggella secrete mucus in response to exposure to maternal estrogen. Vaginal obstruction leads to the retention of a significant amount of this secretion, which can stretch the vagina and uterus in the neonatal period. On the other hand, vaginal obstruction sometimes doesn't show up until the girl starts menstruating.

Vaginal obstruction. The most common cause of vaginal obstruction is the persistence of an imperforated hymen as a result of failed vaginal rupture through the sinus tubercle into the urogenital sinus. At the same time, in a newborn girl, a thin membrane may bulge between the labia, which often ruptures spontaneously, possibly as a result of the child's normal physical activity during the first week of life. After this, a small amount of scraps of thin epithelium remains attached to the normal hymenal ring. If the membrane remains unperforated, it can simply be incised with a few absorbable sutures to bring the epithelial duplication sheets closer together.

An imperforated hymen is sometimes not diagnosed until a girl in her teens has recurrent attacks of pain in the lower abdomen in the absence of menstruation. A similar clinical picture is sometimes repeated for several menstrual cycles, and only then does the presence of a tumor-like formation in the abdomen become apparent. The diagnosis is confirmed by ultrasound, which reveals the expansion of the vagina.

The bulging membrane, which prevents the release of the menstrual secret, is dissected (under anesthesia) with a cruciform incision. The dissected edges of the hymenal ring are sutured with absorbable sutures. Rarely, the obstruction may be more severe, with the membrane located higher than normal in the vagina. A combined vaginal, abdominal, and rectal examination reveals a normally developed lower vagina or partial urogenital sinus and a higher urethral orifice.

The distended vagina is palpated over the site of obstruction. Ultrasound and computed tomography identify the thick membrane causing the obstruction and confirm the presence of a uterus and sagittal vaginal membrane. In such a situation, it is advisable to use nuclear magnetic resonance, especially due to the fact that clinically unsuspected doubling or atresia can be detected during this examination.

The choice of access (purely vaginal or abdominovaginal) to eliminate vaginal obstruction depends on the thickness of the membrane and the distance between the distal and proximal vagina.

Our experience suggests that a thorough physical examination of a patient under anesthesia provides more valuable and accurate information than standard radiological methods. True, transperineal sonography, measuring the distance between the distended vagina and the perineum using electronic meters, can be much more informative.

Highly located membranes no more than 1-2 cm thick can be reached from transvaginal access. The membrane is brought down by the sutures-holders and carefully dissected until the proximal vagina is opened. The edges of the mucosa are then sutured with separate sutures. After the operation, it is necessary to carry out bougienage until the vagina becomes large enough for free sexual intercourse. In cases where the membrane has a significant thickness, and with a large distance between the perineum and the proximal vagina, an abdomino-perineal access is necessary in order to be able to mobilize the proximal vagina well to connect it with the skin flaps of the perineum.

High obstruction of the vagina. The most difficult problem is those cases of vaginal obstruction when a newborn girl has a persistent full urogenital sinus and a high transverse septum. This situation may arise due to the fact that the Müllerian ducts simply did not connect to the urogenital sinus, or due to the lack of development of the uterovaginal plate (Fig. 64-1).

Rice. 64-1. Hydrometrocolpos in a newborn girl. The urogenital sinus is a continuation of the urethra; the vagina is very sharply stretched.

Clinically, this form of hydrometrocolpos is manifested by the presence of a huge tumor-like formation in the abdomen, often extending to the costal margin. The child may have respiratory distress due to the high standing of the diaphragm, which sometimes requires intubation and mechanical ventilation until the nature of the mass in the abdomen is clarified. The "tumor" is located anterior to the rectum, while the external genitalia are developed correctly.

Careful inspection with mirrors reveals the absence of the urethra. During catheterization of the urogenital sinus, urine is released, since the urethra is located at the top of the sinus. X-ray examination shows a sharp expansion of the vagina and uterus, displacement of the bladder anteriorly, ureters - laterally, bowel loops - up. Hydrometrocolpos can be a hereditary anomaly transmitted in an autosomal recessive manner.

Hydrometrocolpos is often combined with other congenital anomalies such as polydactyly, heart defects, urological anomalies. In the presence of a very high standing of the diaphragm, hydrometrocolpos requires emergency intervention. If the newborn child is in extremely serious condition, the vagina can be drained through a small median incision in the abdominal wall, after which the vaginal mucosa is sutured to the skin, thus forming a vaginostomy. Later, when the child's condition stabilizes, a radical intervention is carried out. However, in most cases, an operation of abdominovaginal reduction can be performed immediately (in the neonatal period).

Operation technique. The position of the child on the operating table is the same as for lithotomy. The abdomen, perineum and thighs are treated and covered with sterile linen. A catheter is inserted into the bladder through the urogenital sinus. The lower transverse incision of the abdominal wall provides full access to the distended vagina and uterus. The bladder is separated, using scissors and an electrocoagulator, from the anterior wall of the vagina until the vagina is exposed as low as possible. If there is a doubling of the uterus, then we can expect the presence of a septum dividing the vagina into two lateral halves. However, this septum is rarely complete.

After drainage and revision of the vagina from the inside, its back wall is lowered (“pushed”) towards the perineum, where an inverted U-shaped skin flap is formed immediately behind the urogenital sinus. With great care to avoid damage to the rectum and ureters, the posterior vaginal wall is mobilized so that it is freely, without tension, compared with the skin flap. The posterior wall of the vagina is opened, the mucosa is sutured to the perineal flap and to the posterior wall of the urogenital sinus. With this intervention, the urogenital sinus remains as the urethra, and the genital tract is completely separated from the urinary tract. While the child is under anesthesia, an examination should be performed to dilate the anastomosis.

A girl who has undergone this intervention should undergo a full gynecological examination at puberty. We observed two patients with a congenital anomaly of the vagina who developed tubo-ovarian abscesses in adolescence. There are reports in the literature of successful pregnancies and successful deliveries by caesarean section in women who had had a similar operation in childhood.

Doubling of the vagina. Violation (absence) of the process of fusion of the Müllerian ducts can be the cause of the development of two uteruses and a vagina divided by a septum into two parts. Obstruction of one of the halves of the vagina with stretching by its secret leads to the appearance of a tumor-like formation, which is sometimes detected already in the neonatal period. However, most often the correct diagnosis is established when, with the onset of menstrual age, the girl develops abdominal pain.

In typical cases, the girl menstruates normally, but there are pains associated with obstruction of half of the vagina. This anomaly is often associated with unilateral renal agenesis. Examination reveals an open vagina, cervix, and paravaginally located formation. Radiological examination shows a stretched half of the vagina with a "own" uterus and fallopian tube. During the operation, the distended vagina is emptied by aspirating the contents with a syringe through a needle. The wall (septum) between the two vaginas is excised, the mucosa is sutured. As a result of this intervention, a single vagina with two necks is formed.

Vaginal agenesis. The absence of a vagina, with or without a uterus, combined with a number of anomalies of the Müllerian ducts and renal agenesis, is called Mayer-Rokitansky-Küster syndrome. In the classic version of this syndrome, the ovaries are normal, while the uterus and fallopian tubes are absent or vestigial. A significant frequency of family cases indicates a single gene lesion, which is not limited to the implementation of the pathology only in girls. The syndrome can also occur in boys in the form of renal agenesis and the absence of the vas deferens.

Tarry and colleagues proposed a classification of various variants of this anomaly.

The absence of a vagina may not be diagnosed until the age when menstruation should be expected. When examining a child, a depression is visible between the labia minora. Radiological examination of the abdominal cavity allows you to determine the presence of the uterus and proximal vagina, as well as the urinary tract (Fig. 64-2).

Rice. 64-2. Ah, a 13-year-old girl. The stretched vagina was located 6 cm from the perineum.
B, Arrows show a septum within the uterus, indicating no fusion of the superior Müllerian ducts.

If a uterus is present, a vagina must be created in order to drain the menstrual fluid that accumulates in the proximal remnants of the vagina between the bladder and rectum. When developing a treatment plan, it is important to take into account the attitude (degree of concern) of the child and parents regarding future sexual and reproductive functions.

The timing of colpopoiesis surgery depends on a number of psychological factors. In particular, intervention may be deferred until such time as the girl in question has specific circumstances suggesting that she will have sexual intercourse. Alternatively, it is possible (and seems to be preferable) to perform the operation in early adolescence, regardless of the specific circumstances.

Unfortunately, there is no operation that can be called perfect. For total vaginal reconstruction, one of the most acceptable and often successful interventions is the use of a split skin graft or the Mclndoe operation.

This intervention is preferred in the Mauo Clinic, which has extensive experience in the treatment of patients with this pathology. One report from this clinic indicates that out of 47 patients, 85% achieved results that can be called satisfactory. Unfortunately, patients required prolonged bougienage to prevent stenosis. There are reports in the literature of the development of squamous cell carcinoma after vaginal reconstruction using split skin grafts.

The position of the patient on the operating table as in lithotomy. A catheter is inserted into the bladder (and left there). In preparation for surgery, it is necessary to clean the intestines in order to prevent contamination of the perineum in the postoperative period. The incision is made 1.5 cm behind the urethra and 2 cm short of the anus. The tissues are prepared in a blunt way, creating a cavity along the midline. Hemostasis must be absolutely complete, since the occurrence of a hematoma may prevent the taking of a skin graft.

A 0.015 inch (0.38 mm) thick graft is taken from the inner thigh. The graft must be large enough to fit over a 10 cm mold and 7 cm in circumference. Either a specially made tissue expander or a condom filled with plastic foam can be used as a mold. The graft is sutured around the mold and placed in the cavity created by the preparation.

The skin of the perineum is sutured over the mold to hold it in place. After about 10 days, the mold is removed and the cavity is examined (under anesthesia). Dilation is necessary to prevent stricture. This is done by the patient herself by inserting the form used in the reconstruction into the newly created vagina, or, if this is an adult patient, then with the help of regular intercourse. Promotes a more normal epithelization of the vagina and topical application of an estrogen cream. As an alternative to the described method, local skin flaps from the perineum are used to line the cavity above the mold.

Whenever possible, I prefer to use local skin flaps formed from the labia minora. These flaps have some advantages over skin grafts, as they are more sensitive to estrogen stimulation, thus epithelialization of the vagina takes place in a more natural way, and besides, when they are used, the tendency to stricture is less pronounced. To increase the size of local skin flaps, tissue expanders can be used.

Various methods are also used to create an artificial vagina from intestinal segments, most often from the sigmoid colon. This may be the best method in cases where it is necessary to create an anastomosis with the cervix to ensure the flow of menstrual flow. Yet, for optimal results, the lower vagina must be created from local skin flaps, which eliminates such a complication as prolapse of the intestinal mucosa.

K.U. Ashcraft, T.M. Holder

URINARY SINUS URINARY SINUS

(sinus urogenitalis), a cavity into which the excretory ducts of the sexual flow into and will allocate, systems in many. vertebrates. M. s. opens into the cloaca (shark and whole-headed fish, turtles, cloacal mammals), and in its absence - outward (cyclostomes, multi-feathered, ganoid, some male bony fish, viviparous mammals). In female placental mammals (rodents, ungulates, primates) M. s. forms the vestibule of the vagina, separated from the vagina of virgins, the hymen. At males of viviparous mammals from M. of page. the top develops, the department of the urination. channel.

.(Source: "Biological Encyclopedic Dictionary." Chief editor M. S. Gilyarov; Editorial staff: A. A. Babaev, G. G. Vinberg, G. A. Zavarzin and others - 2nd ed., corrected . - M .: Sov. Encyclopedia, 1986.)

See what "URINARY SINUS" is in other dictionaries:

- (sinus urogenitalis) see Genitourinary sinus ... Big Medical Dictionary

The reservoir into which the excretory ducts of the reproductive and excretory systems flow in many vertebrates. M. s. opens into the cloaca (See Cloaca) (in shark and whole-headed fish, turtles, cloacal mammals), and with its reduction outward ...

See Genitourinary System...

See Genitourinary System... Encyclopedic Dictionary F.A. Brockhaus and I.A. Efron

The embryonic structure is the anterior part of the cloaca, from which the body and the apex of the bladder and urethra subsequently develop. Part of this structure may also be part of the vagina. Source: Medical Dictionary... medical terms

SINUS URINARY- (urinogenital sinus) the embryonic structure of the anterior cloaca, from which the body and the apex of the bladder and urethra subsequently develop. Part of this structure may also be part of the vagina... Explanatory Dictionary of Medicine

URINARY ORGANS- URINARY ORGANS, Excretory organs, quite closely connected in most vertebrates, together with the genital organs, into one genitourinary, or urogenital system. In invertebrates. animals usually do not have such a connection; they have urinary organs ... ... Big Medical Encyclopedia

"Lamprey" redirects here; see also other meanings. ? Lampreys ... Wikipedia

Organs of sexual reproduction (See Sexual reproduction). Sexual organs of animals. To P. about. include the sex glands Gonads (testes and ovaries), genital ducts (vas deferens and oviducts), the so-called. additional education and ... ... Great Soviet Encyclopedia

- (or reproductive organs) organs that are directly related to reproduction. From this point of view, the stolon, which serves for asexual R. (see), is also an organ of R. But usually, under the name of organs of R., only organs of the sexual R are understood ... Encyclopedic Dictionary F.A. Brockhaus and I.A. Efron

Surgical treatment of newborns with indeterminate sex is one of the most "mysterious" problems of pediatric urology, requiring an urgent comprehensive solution by a team of specialists, which should include neonatologists, children's, and. An urgent determination of the genetic sex and biochemical disorders is necessary, after which a plan, timing and types of surgical correction are outlined. Surgical treatment of patients with intersex disorders aims to achieve the most normal appearance of the genitals and good function to ensure normal sexual intercourse.

Female pseudohermaphroditism due to congenital adrenal hyperplasia (CAH) is the most common cause of indeterminate genitalia in newborns. This pathology is inherited in an autosomal recessive manner and in 75% is combined with life-threatening metabolic (salt-salting) disorders. Exposure of the female fetus to 46,XX adrenal androgens results in varying degrees of virilization of the external genitalia and distal vagina, although the Müllerian progenitors of the internal genitalia, fallopian tubes, uterus, and proximal vagina develop normally in the absence of the Müllerian inhibitory substance. The prognosis for these patients, who develop as girls after appropriate surgical reconstruction, is excellent, with normal pubertal development, with all female characteristics, normal sexual activity and reproductive capabilities. The success of reconstruction depends on the accuracy of determining the anatomical structure of the genitals, where the main attention should be paid to the localization of the opening of the vagina in the urogenital sinus and its relationship with the pelvic floor and the external sphincter mechanism. The listed data can be obtained using voiding cystourethrography (VCUG), CT or MRI and panendoscopy. Most often, the anatomical structure of the genitals is determined by a deficiency of 21-hydroxylase and is represented by moderate or severe virilization of the clitoris with localization of the entrance to the vagina on the "seminal tubercle" or even lower.

Feminizing plastic surgery of the genital organs has 5 main goals:

ensure the normal localization of the entrance to the vagina on the perineum,
create a normal view ("wet") of this input,
completely separate the vagina from the urinary tract,
remove the phallic erectile tissue, preserving the head with its innervation, sensitivity and blood supply,
avoid urinary tract complications such as infection and urinary incontinence.

In order to achieve these goals, we are dealing with two main "problems" - an enlarged clitoris and vagina. can be done in stages or in one step. In recent years, several methods of simultaneous feminizing plastic surgery of the genital organs have been described. Our method and experience is based on the principle of bringing down the vagina, mobilization of the enbloc urogenital sinus through the perineum, clitoroplasty reduction and reconstruction of the vaginal inlet using remnants of the urogenital sinus mucosa with phallic skin.

The timing of genital reconstruction in the presence of indeterminate sex remains the subject of very heated debate. Psychiatrists and medical ethicists have raised the question of the irreparable damage that parents cause by “assigning” a gender to a child before he himself began to feel that he belonged to one or the other sex. Fortunately, these debates are largely irrelevant to our decision making in cases of 46,XX AHN, since these patients are clearly female. Until recently, almost everyone recommended that girls with CAH, especially those with high vaginal entry into the urogenital sinus, undergo reconstruction in two stages: first, reduce the clitoris as early as possible in order to avoid anxiety of parents and people around the child, and then, in more late age, to carry out vaginoplasty to prevent the development of vaginal stenosis. This complication (vaginal stenosis) can be avoided by using the operations described by Passerini-Glazel, Gonzales, Rink and Farkas, since these methods involve the use of the skin of the penis and foreskin, as well as the urogenital sinus, instead of perineal and other skin flaps. Therefore, early one-stage surgery has the great advantage of using the remnants of the skin of the penis and foreskin for reconstruction, especially in those girls who have a pronounced (maximum) virilization. This fine plastic material is simply discarded if the reconstruction of the clitoris is performed in the first place as a separate independent operation. De Jong and Boemers believe that one-stage intervention should be performed in the neonatal period. Prenatal hyperstimulation by maternal and placental estrogens, which continues during the first 3-4 weeks of life, causes the vagina to enlarge, due to mucus secretion, and hypertrophy of its wall, facilitating the operation of lowering the vagina. We currently prefer single-stage surgery at 3-4 months of age, which we describe below.

Preparation for surgery consists in complete emptying of the intestine, adequate steroid therapy and stabilization of the biochemical status.

Genital plasty surgery

The operation begins with a mandatory panendoscopy, which is performed with a pediatric cystoscope. Examine the bladder and determine the place where the vagina flows into the urogenital sinus. Once this site is located, it is almost always possible to pass the cystoscope into the vagina. At this stage, the telescope is removed and a silicone Foley catheter is inserted through the tube of the device into the vagina. The balloon is inflated to a volume of 2-3 ml. The tube of the apparatus is removed and the Foley catheter is occluded at the distal end of the urogenital sinus to prevent balloon deflation. The distal end of the catheter is cut to allow the device sheath to be completely removed. The catheter is then clamped again. In some cases, in the presence of a narrow opening of the vagina, a Fogarty catheter can be used, while the cystoscope is inserted only to the place where the vagina enters the sinus, but not into the vaginal cavity. In addition, an 8 or 10F catheter is inserted into the bladder in the usual way.

The patient is placed in a lithotomy position with the hips widely separated so that the perineum can be opened as much as possible. The operation begins with a skin incision around the entire circumference around the enlarged clitoris and the opening of the urogenital sinus. This incision is extended into two vertical incisions along the dorsal and ventral surfaces of the clitoris. From the ventral side, the incision is continued into a U-shaped to connect the incision circumferentially just below the head and end proximally with the formation of an inverted (inverted) wide U-shaped flap. On both sides, this flap goes to the ischial tuberosities. Such an incision provides good access to the anatomical structures of the genitals and to the entire perineum with the urogenital sinus.

The urogenital sinus is separated completely from the cavernous and spongy bodies of the penis. Isolation is continued, using a needle electrocautery, between the urogenital sinus and the rectum, which is retracted dorsally, in a layer located laterally between the urogenital sinus and the legs of the cavernous bodies and further below and behind the branches of the pubic bones, until the entire urogenital sinus is released en block and brought out. Then the back wall of the vagina is released and the vagina is brought out to the perineum without tension. The presence of a balloon in the vagina, which is periodically palpated during the operation, facilitates the extraction of the vagina and makes this stage safe.

At this stage, clitoroplasty is performed using the method described by S. Kogan: the proximal ends of the previously isolated legs of the cavernous bodies are tied with two ligatures. Buck's fascia is dissected along the ventral-lateral surface of the penis in order to preserve the blood supply and sensitivity of the clitoral head. Buck's fascia is separated from the erectile tissue, which is again ligated just below the glans, and then all of the erectile tissue is removed.

Two mucocutaneous flaps, formed from the skin of the penis and foreskin, are completely separated from the cavernous bodies and taken aside.

If the head looks too big, it should be reduced by removing the tissue in the center, leaving as much tissue as possible on the sides, since this area is most sensitive. The head is reconstructed with 6/0 sutures. The head of the clitoris is then placed under the pubic arch and sutured in place with two or three 4/0 absorbable sutures.

The posterior surface of the urogenital sinus is moved without tension as close to the perineum as possible. Bulbocavernous muscles are dissected along the midline and retracted laterally, thus opening the posterior wall of the vagina, which is widely dissected behind the balloon between traction and hemostatic sutures.

After dissection of the posterior wall of the vagina, the places of its confluence with the urogenital sinus and urethra are visible. This connection is sutured with separate 5/0 absorbable sutures from the inside of the vagina. A Foley catheter inserted into the bladder "protects" the urethra.

An appropriately long urogenital sinus is retained as a tube and sutured directly below the glans clitoris to retain this part of the sinus as the urethra. The rest of the sinus is dissected in the midline from the dorsal side, creating a mucous plate.

The mucous plate of the dorsally dissected urogenital sinus is sutured to two lateral flaps of the skin of the penis and foreskin, previously separated from the penis. As a result, a wide plate is formed, consisting of a mucous membrane in the center and at the top, while its lateral parts are formed from the skin of the penis, soft, "hairless" and extensible.

The mucocutaneous plate is turned down, the mucosa at its base is sutured to the ventral wall of the vagina, and the lateral edges are sutured to the lateral wall of the vagina, resulting in an anastomosis of the mucosa with the mucosa around the vaginal opening. The apex of the preformed "inverted" U-shaped perineal flap is sutured to the posterior dorsal angle of the vagina. The skin of the penis is sutured around the entire circumference, creating large and small labia.

A tampon with a xeroform is inserted into the vagina, a pressure bandage is applied to the wound. Both the swab and bandage are removed after 48-72 hours. The urethral catheter is left for 7 days.

Conclusion

Since 1991, we have operated on 67 patients with simultaneous feminizing plastic surgery of the genital organs according to the method described above. In two cases, during the operation, there was a slight damage to the wall of the rectum, sutures were immediately applied and there were no further consequences and complications. Wound infection in the gluteal region developed in 3 cases, in one of them a hypertrophic scar formed as a result. In one girl, the clitoris was completely lost due to a violation of the blood supply. Two cases developed recurrent clitoromegaly associated with inadequate androgen suppression. The cosmetic results are very good. In a small number of patients who have already reached puberty, we have found a wide mucosal entrance to the vagina, without fibrosis. None of our patients have yet had sexual intercourse, so we have no information on the difficulties in these contacts, sexual satisfaction, fertility and psychological aspects of sexual relations.

It is important to emphasize that the method described above is mainly indicated and can be used in most children with CAH, with 21-hydroxylase deficiency and with vaginal entry into the urogenital sinus at the level of the pelvic floor or even lower (in the region of the genital tubercle). With a low confluence of the vagina into the sinus, it is enough to simply move it backwards or use small perineal flaps, as described by S. Fortunoff et al. In rare cases of very high vaginal openings above the pelvic floor at the bladder neck, perineal access may be inadequate. In these cases, Passerini-Glazel offers an abdominal transvesical approach with a transtrigonal incision of the bladder wall. This method was originally described by J. Monfort as access to the prostate uterus. In such rare complex cases, the method of choice and the best method is the posterior transanorectal approach according to Turnip.

The article was prepared and edited by: surgeon

This pathology is characterized by deviations in the structure and functioning of the genital organs. The origin of the disease has not yet been established, however, doctors believe that the syndrome develops due to excessive production of androgens by the adrenal cortex. The disease can be caused by various tumors or congenital glandular hyperplasia.

What is adrenogenital syndrome

Congenital adrenal hyperplasia is the most common type of verifying pathology. Adrenogenital syndrome is a disease that is known to world medicine as Apert-Gamet syndrome. Its development is associated with increased production of androgens and a pronounced decrease in the level of cortisol and aldosterone, which is caused by congenital dysfunction of the adrenal cortex.

The consequences of deviation can be serious for a newborn, since the adrenal cortex is responsible for the production of a huge amount of hormones that regulate the functioning of most body systems. As a result of pathology in the body of a child (this can be observed in both boys and girls), there are too many male hormones and very few female ones.

Inheritance type

Each form of the disease is associated with genetic disorders: as a rule, anomalies are hereditary in nature and pass from both parents to the child. More rare are cases when the type of inheritance of adrenogenital syndrome is sporadic - it occurs suddenly during the formation of an egg or sperm. Inheritance of adrenogenital syndrome occurs in an autosomal recessive way (when both parents are carriers of the pathological gene). Sometimes the disease affects children in healthy families.

Adrenogenital syndrome (AGS) is characterized by the following patterns that affect the likelihood of a child being affected by it:

if the parents are healthy, but both are carriers of the StAR deficiency gene, there is a risk that the newborn will suffer from congenital adrenal hyperplasia;
if a woman or a man was diagnosed with a syndrome, and the second partner has normal genetics, then all the children in their family will be healthy, but they will become carriers of the disease;
if one of the parents is sick, and the second is a carrier of adrenogenetic pathology, then half of the children in this family will be sick, and the other half will carry a mutation in the body;
if both parents have the disease, all their children will have similar abnormalities.

Forms

Androgenetic disease is conditionally divided into three types - viril simple, salt-losing and post-pubertal (non-classical). Varieties have serious differences, so each patient requires a detailed diagnosis. How are the forms of adrenogenital syndrome manifested:

Viril form. It is characterized by the absence of signs of adrenal insufficiency. The remaining symptoms of AGS are present in full. This type of pathology is extremely rarely diagnosed in newborns, more often in adolescents (boys and girls).
Salting type. Diagnosed exclusively in infants during the first weeks/months of life. In girls, pseudohermaphroditism is observed (the external genitalia are similar to male, and the internal ones are female). In boys, the salt-losing syndrome is expressed as follows: the penis has a disproportionately large size relative to the body, and the skin of the scrotum has a specific pigmentation.
Non-classical look. The pathology is characterized by the presence of unclear symptoms and the absence of pronounced adrenal dysfunction, which greatly complicates the diagnosis of AGS.

Adrenogenital syndrome - causes

Congenital adrenal dysfunction is explained only by the manifestation of a hereditary disease, therefore, it is impossible to acquire or become infected with such a pathology during life. As a rule, the syndrome manifests itself in newborn babies, but rarely AGS is diagnosed in young people under the age of 35 years. At the same time, factors such as taking potent drugs, increased background radiation, and side effects from hormonal contraceptives can activate the pathology mechanism.

Whatever the incentive for the development of the disease, the causes of adrenogenital syndrome are hereditary. The forecast looks something like this:

if at least 1 parent is healthy in the family, the child is likely to be born without pathology;
a couple where one carrier and the other is sick with AGS in 75% of cases will have a sick child;
carriers of the gene have a 25% risk of having a sick child.

Symptoms

AGS is not a deadly disease, but some of its symptoms cause serious psychological inconvenience to a person and often lead to a nervous breakdown. When diagnosing a pathology in a newborn, parents have the time and opportunity to help the child with social adaptation, and if the disease is detected at school age or later, the situation may get out of control.

It is possible to establish the presence of AGS only after a molecular genetic analysis. Symptoms of adrenogenital syndrome that indicate the need for diagnosis are:

non-standard pigmentation of the skin of the child;
steady increase in blood pressure;
low growth inappropriate for the age of the child (due to the rapid end of the production of the corresponding hormone, growth stops early);
periodic convulsions;
digestive problems: vomiting, diarrhea, severe gas formation;
in girls, the labia, the clitoris are underdeveloped or, on the contrary, are enlarged;
in boys, the external genitalia are disproportionately large;
girls with AGS have problems with menstruation, conceiving a child (infertility often accompanies the disease), bearing a fetus;
in female patients, male-type genital hair growth often occurs, in addition, growth of mustaches and beards is observed.

Adrenogenital syndrome in newborns

The disease can be detected at an early stage in newborns, which is associated with neonatal screening on the 4th day after birth. During the test, a drop of blood from the baby's heel is applied to the test strip: if the reaction is positive, the child is transferred to an endocrinological dispensary and re-diagnosed. Once the diagnosis is confirmed, AGS treatment begins. If adrenogenital syndrome in newborns is detected early, then therapy is easy, in cases of late detection of adrenogenetic pathology, the complexity of treatment increases.

Boys

The disease in male children develops, as a rule, from the age of two or three. There is an enhanced physical development: the genitals increase, active hair growth occurs, erections begin to appear. At the same time, the testicles lag behind in growth, and in the future they completely stop developing. As in girls, adrenogenital syndrome in boys is characterized by active growth, but it does not last long and as a result, the person still remains low, stocky.

Girls

Pathology in girls is often expressed immediately at birth in a virilized form. False female hermaphroditism, characteristic of AGS, is characterized by an increased size of the clitoris, while the opening of the urethra is located directly under its base. The labia in this case resembles a split male scrotum in shape (the urogenital sinus is not divided into the vagina and urethra, but stops developing and opens under the penis-shaped clitoris).

Not infrequently, the adrenogenital syndrome in girls is so pronounced that at the birth of a baby it is difficult to immediately establish its gender. In the period of 3-6 years, the child actively grows hair on the legs, pubis, back, and the girl looks very much like a boy. Children with AGS grow much faster than their healthy peers, but their sexual development soon stops completely. At the same time, the mammary glands remain small, and menstruation is either completely absent, or appear irregularly due to the fact that underdeveloped ovaries cannot fully perform their functions.

Diagnosis of adrenogenital syndrome

It is possible to identify the disease with the help of modern studies of the hormonal background and during a visual examination. At the same time, the doctor takes into account the anamnestic and phenotypic data, for example, hair growth in places atypical for a woman, development of the mammary glands, male body type, general appearance/health of the skin, etc. monitor the level of hormones DEA-C and DEA, which are precursors of testosterone.

Diagnosis also includes a urinalysis for the determination of the 17-KS indicator. A biochemical blood test allows you to establish the level of hormones 17-ONP and DEA-C in the patient's body. Complex diagnostics, in addition, involves the study of the symptoms of hyperandrogenism and other disorders of the endocrine system. At the same time, the indicators are checked twice - before and after the test with glucocorticosteroids. If during the analysis the level of hormones is reduced to 75% or more percent, this indicates the production of androgens exclusively by the adrenal cortex.

In addition to hormone tests, the diagnosis of adrenogenital syndrome includes an ultrasound of the ovaries, in which the doctor determines anovulation (it can be detected if follicles of different levels of maturity are observed that do not exceed preovulatory volumes). In such cases, the ovaries are enlarged, but the volume of the stroma is normal and there are no follicles under the organ capsule. Only after a detailed examination and confirmation of the diagnosis, the treatment of adrenogenital syndrome begins.

Adrenogenital syndrome - treatment

ABC is not a fatal pathology with a fatal outcome, so the likelihood of developing irreversible changes in the patient's body is extremely small. Nevertheless, modern treatment of adrenogenital syndrome cannot boast of its effectiveness and efficiency. Patients with this diagnosis are forced to take hormonal drugs for life in order to make up for the deficiency of hormones of the glucocorticosteroid group and fight the feeling of inferiority.

So far, the prospects for such therapy remain unexplored, but there are data that indicate a high probability of the development of concomitant AGS pathologies of the heart, bones, blood vessels, gastrointestinal organs, and oncological diseases. This explains the need to conduct regular examinations for people with dysfunction of the adrenal cortex - to do an x-ray of the bones, an electrocardiogram, an ultrasound of the peritoneum, etc.

Video

The excretory system begins to develop from the 3rd week of pregnancy. The kidneys begin to function from about the ninth week of the intrauterine life of the baby. Nephrons - the structural units of the kidneys develop from 20-22 weeks. They, like all organs of the genitourinary system, are very sensitive to various damaging (teratogenic) factors. The most dangerous in relation to the development of congenital kidney diseases are the first 2-3 months of pregnancy, and the earlier the teratogenic effect was, the more severe the malformations, possibly incompatible with life, will be. If the harmful factor acts at 3-4 weeks of gestation, then the fetus may not develop kidneys (renal agenesis ...

Discussion

Pelvic dystopia is the case when one of the paired organs is located in the area of the uterus and intestines in a female patient, and between the urinary tract and the male rectum, more details in [link-1] The ureter is reduced, the vascular circulatory system branches off from the internal iliac artery .

May 31, 2018 04:45:27 PM Jojo

I ask everyone who has ever "digged" this topic - share links. It is possible in English. Preferably with photos. There was a thread about this a month ago, but I can't find it in the archive. Links can; but send in the mail: [email protected] Help! Thanks in advance!

Discussion

All links to English sites. I could translate some articles, but I need to know which ones are important to you.
And I also gave emails to Russian doctors. They work in the US with adapted children and invite doctors from Russia and Ukraine. I thought that maybe they would advise you on a FAS specialist in Russia. I do not want to give their emails openly, if you need, I will send you in private.

articles for parents of children with FAS
http://www.faslink.org/katoc.htm#PArenting

strategy of behavior with a child with FAS
http://www.come-over.to/FAS/BEAM.htm

Features of infants with FAS
http://come-over.to/FASCRC/

Girls! Reassure me please with examples (if you find). I'm in my 6th obstetric week. Not only is there no toxicosis, but there are really no signs: ((((A little bit the nipples became more sensitive and that's it! There is no heaviness in the chest, no pigmentation - nothing. But in the past, the chest "was." On ultrasound only in a week. And I'm already slowly going crazy. Perhaps, morning vomiting would greatly brighten up my existence :) The fact that there is no toxicosis, I rarely understood, but it happens. And it happens that almost ...

Discussion

I am also 6 weeks old. A couple of times the stomach twisted with gshaz and that's it (ttt).
It's also not very noticeable to me. Now, if it weren’t already cut down to sleep in the middle of the day ...
By the way, I had an ultrasound a week ago. Why wait so long? Everything is visible there.

05/17/2005 19:35:09, Nastasia

Yes, there were no signs ... if the stomach did not pull, there would be nothing at all ... Well, the waist began to spread by 8 weeks and all ....