Symptoms of epispadias and bladder exstrophy

Classical symptoms of bladder exstrophy

Unlike patients with cloacal exstrophy and its variants, the symptoms of classic bladder exstrophy are combined with minor and minor concomitant abnormalities of the abdomen, perineum, upper urinary tract, genitalia, spine and pelvic bones.

[1]

Belly and perineum

The largest anatomical changes are subjected to the rhomboid zone, bounded by the navel, the anal opening, the rectus abdominis and the skin covering the puborectal loop. The distance from the navel to the anus in bladder exstrophy is shortened, since the navel is located lower than normal, and the anus is anterior. The area between the distended muscles of the anterior abdominal wall and the edge of the exstrophy bladder is covered with a thin fibrous tissue. Widely placed bony bones shift the rectus laterally. The straight muscle on each side is attached to the lobule. The laterally displaced rectus muscles widen and shorten the inguinal canal, which increases the likelihood of an oblique inguinal hernia.

Inguinal hernia occurs in 56% of boys and in 15% of girls with classic bladder exstrophy. Atresia of the anus during bladder exstrophy is rare. Fusel forms of atresia of the anus are more common, opening on the perineum, into the vagina or into the bladder exstrophy. Unoperated patients with bladder exstrophy often suffer from prolapse of the rectum, which is facilitated by the weakness of the pelvic floor muscles and the anal mechanism characteristic of such patients. Prolapse almost never occurs in patients who underwent plastic surgery during the neonatal period. If prolapse all the same develops. Then surgical treatment does not require, since it passes after the plasticization of the bladder.

[2], [3], [4], [5]

Urinary system

The bladder is extruded in the form of a dome under extrofia and increases in size with the child's anxiety. Extruded sites vary in size: from small (2-3 cm) to a large prolabating mass. The mucous membrane immediately after birth is clean, smooth, pink in color. If the bladder remains open, its surface quickly undergoes polypoid rebirth, which significantly worsens its condition. Irritation of the bladder is facilitated by the contact of the mucous membrane with napkins or clothing.

The lack of treatment leads to flaky or adenomatous metaplasia, and subsequently, in adult patients, can develop into squamous cell carcinoma or adenocarcinoma. Before a radical operation to protect the mucous membrane from damage it is advisable to cover with a perforated cellophane film and avoid direct contact of the mucous membrane with napkins and diapers. There are cases of doubling, when together with the exstrophy of the bladder there is also a normal, shifted to the side.

Researchers believe that bladder exstrophy is characterized by normal blood supply and normal neuromuscular apparatus and, consequently, immediately after plastic surgery, the bladder has a full detrusor function. However, there are isolated reports of a poor detrusor function even after a successful surgical intervention.

Kidney anomalies in patients with bladder exstrophy are rare. If the bladder remains uncovered for a long time and the mucous membrane in the mouth area undergoes a marked fibrous degeneration, sometimes ureterohydronephrosis develops. However, this problem rarely occurs in infancy. Ureters with bladder exstrophy run into it at right angles and practically do not have a submucosal tunnel, because of this, almost all children (more than 90%) have bladder ureter reflux after the primary bladder. Male sexual organs.

In patients with bladder exstrophy, the length of the penis is significantly shorter due to the divergence of the bones, which prevents the connection of the cavernous bodies along the midline, as is normal. As a result, the length of the free protruding "hanging" part of the penis decreases. The size of the penis is also affected by its curvature. Upon examination, it is found that the penis of the child with epispadia has a characteristic forced position, pressed against the anterior abdominal wall. This is due to the pronounced dorsal deformation of the cavernous bodies, which is clearly visible during erection. It has been established that the length of the ventral surface of cavernous bodies with exstrophy of the bladder is greater than the dorsal portion of the bladder due to fibrous changes in the tissue of the gall bladder.

With age, these differences only increase, and the deformation of the penis increases. To achieve complete cavernous body expansion and increase the length of the penis, it is possible to plasticize the dorsal surface of the cavernous bodies with loose patches of de-epithelialized skin. The urethra in patients with bladder exstrophy is completely cleft and opened up to the head. The urethral pad is usually shortened. In rare cases (3-5%), aplasia of one of the cavernous bodies is observed. Perhaps doubling of the penis or its complete absence (aplasia), especially with cloacal exstrophy.

The innervation of the penis is normal. In patients with bladder exstrophy, the superficial neurovascular bundle providing an erection is laterally displaced and is represented by two separate bundles. Despite the massive secretion of tissues during surgery, most patients retain their sexual potency after the reconstruction of the penis.

Wolves structures in boys are normal. In patients with dysfunction of the neck of the bladder or open neck after surgery, there is often a problem of retrograde ejaculation. However, if the reconstruction is performed adequately, then patients with exstrophy may later have children.

Cryptorchidism in patients with exstrophy is 10 times more common, but experience shows that many children who are diagnosed with cryptorchidism are in fact retracted testicles. They can be reduced without special difficulty to the scrotum after the bladder has been reconstructed by the female genital organs.

Female genital organs

When bladder exstrophy in girls, the clitoris is split into two parts. In this case, half of the clitoris is located on each side of the genital gash along with the labia. The vagina is more often united, but it can also be bifurcate. The outer orifice of the vagina is sometimes narrowed and often displaced anteriorly. The degree of displacement corresponds to the degree of shortening of the distance between the navel and the anal opening. The uterus can be bifurcated. Ovaries and fallopian tubes are usually normal. Loss of the uterus and vagina is possible in women with bladder exstrophy at an older age due to a defect in the muscles of the pelvic floor, which creates serious difficulties for this group of patients during pregnancy. Primary plastic of the bladder with reduction of the frontal bones reduces the risk of these complications. Operations to fix the uterus, such as sacrocolpopexy, help to solve these problems.

[6]

Anomalies of pelvic bones

Diastasis of the lateral bones is one of the components of the "exstrophy-epispadia" complex. This is the result of external rotation and underdevelopment of the bones and sciatic bones in the area of their connection in combination with underdevelopment of the bones of the sacroiliac joint. In patients with bladder exstrophy, external rotation and a shortening of 30% of the anterior branch of the ilium are detected in combination with external rotation of the posterior segment of the ilium. Visually, the hips in children with exstrophy are rotated outward, but problems associated with hip joint function and gait disturbance usually do not arise, especially if at an early age the fusion of the bones was performed.

Very many children have a broken gait in their early childhood, they walk around with their feet turned upside down ("duck walk"), but in the future the gait is corrected. Congenital dislocation of the femur in children with this anomaly is extremely rare, in the literature there are only reports of two such cases.

Only in single studies it was noted that in patients with bladder exstrophy more often than in the general population, vertebral anomalies occur.

Various variants of osteotomy of the pelvic bones are proposed to provide information and fixation of the bones. First of all, the operations are aimed not at solving orthopedic problems, but at increasing the chances of reliable performance of the primary plastic of the bladder and greatly improving the functions of the pelvic floor muscles involved in the mechanism of urine retention.

[7], [8], [9], [10]

Extract of cloaca

Exacerbation of cloaca is also called a vesicoucial cleft, an ectopic cloaca, a visceral ectopia complicated by bladder exstrophy and an abdominal wall cleft. This most severe form of an abnormality abdominal wall occurs in 1 out of 400,000 newborns. The complex of anatomical changes in the classical version of the cloacal exstrophy includes a hernia of the umbilical cord at the top, the outward-directed intestinal loops and the bladder split into two parts.

Between the two halves of the bladder there is a gut region that histologically represents the ileocecal region and has up to four holes, the uppermost of which corresponds to the eventuated small intestine. In appearance, cloacal exstrophy is similar to the "face of an elephant", and the outwardly turned intestine resembles its "trunk". At the bottom there is an opening leading to the blindly terminating distal intestine, which is combined with the atresia of the anus. Between the upper (proximal) and lower (distal) openings, one or two "appendicular" openings can be located.

In all cases, patients have pronounced defects of the genital organs. In boys it is cryptorchidism and a penis split into two separate cavernous bodies with signs of epispadias. It is during exstrophy of cloaca that hypoplasia or aplasia of one of the cavernous bodies often occurs. Girls have a split clitoris, a doubling of the vagina and a bicornic uterus.

[11], [12], [13], [14], [15]

Epispadia

Isolated epispadia is considered a less serious problem among the diseases of the complex "ecstasy epispadia." Patients have no anomalies of the bladder and a defect in the anterior abdominal wall, however, as with exstrophy, the urethra is represented by a split area located along the dorsal surface of the penis. In boys, shortening of the penis and dorsal chord formation are revealed, and in girls - cleavage of the clitoris, with the vagina displaced anteriorly or located in a typical place. In addition to the above, diastase of the bones is observed. The cervix of the bladder in epispadias is often incorrectly formed: it is wide and does not retain urine. Urinary retention can be preserved in boys with distal epispadia, in which the neck of the bladder is formed correctly. Female epispadia is almost always accompanied by urinary incontinence to varying degrees.

[16], [17], [18]

Options of exstrophy

Among patients with the "epispadia exstrophy" complex, there are many variants of pathology development. Depending on the size of the vesicle pad, it can be partially closed and submerged in a small pelvis; such forms are called partial, or partial, exstrophy. Incomplete closure of the bladder in the process of its formation manifests itself in the form of various developmental defects - from the introduction of a bladder into the vagina of the rectus abdominis until the bladder is completely protruded through a small defect above the bosom. Described patients with divergence of the bones and a defect in the anterior abdominal wall, but without any defects of the urinary system.

Such cases were diagnosed as a variant of closed exstrophy. Patients with an upper cystic cleft have the same bone and muscle defects as bladder exstrophy, but changes occur only in the upper part. The neck of the bladder, urethra and genitalia undergo less changes: for example, with this pathology a normal or almost normal penis develops. Some children needed minimal corrective intervention to repair the defect, the operations were effective and fully provided a normal urinary retention function.

Doubling of the bladder is possible, when a child has one closed bladder with a normal urinary retention function and the other with an exstrophy. The size of the penis and the degree of separation of the corpus cavernosum in patients vary. There are cases of hypospadias in children with cloacal exstrophy. The description and discussion of various variants of exstrophy are necessary for an estimation of a variety of displays of a pathology. Comparison of the observed variants with the existing classical forms of the complex "exstrophy-epispadia" helps to choose the optimal treatment tactics.