Epispadias and Bladder Exstrophy: A Review of Information

Anomalies of the group "epiphadia exstrophy" are a combination of a number of defects combined by a single etiology, and a defect on the ventral surface of at least part of the bladder and urethra. This definition was suggested by Gerhardt and Jeffe in 1996. Manifestations of anomalies range from cloacal exstrophy to epicadapia. Classical exstrophy of the bladder is expressed in the absence of the anterior wall of the lower half of the abdomen and the front wall. This disease is combined with epispadia and the divergence of the pubic symphysis, and often with the simultaneous underdevelopment of the testicles, with cryptorchidism, aplasia of one or two cavernous bodies, hypoplasia of the prostate in boys and various abnormalities of the genital organs in girls. Most often this anomaly is defined as a complex of "epiphadia exstrophy", including such pathological conditions:

• epispadia;
• partial exstrophy;
• classical exstrophy;
• exstrophy of cloaca;
• variants of exstrophy.

Formally, for the first time in detail described exstrophy as a congenital malformation in 1597 by Grafenberg, although references to this anomaly were found on Assyrian tablets made 2 thousand years BC. The first attempts to promptly treat the defect were undertaken in the middle of the 19th century. Surgeons created a normal anastomosis between the bladder and the sigmoid colon, but only in single patients could effectively direct urine into the rectum and at the same time hide the defect of the anterior abdominal wall. One of the first successful operations was carried out by Eyrs: he managed to hide the naked mucous membrane and reduce the pain associated with her irritation. Trendelenburg in 1906 made an attempt to close the bladder, performing a cystectomy in combination with antireflux Uregerosigmogomie.

1942 Young reported the first success - retention of urine with the help of plasty of the bladder. The second stage of the sheikh of the bladder was formed into a tube, and the girl remained dry for 3 hours. However, until the 50s of the last century, most surgeons preferred to use excision of the bladder and nighting in the intestine for the correction of exstrophy.

About the bilateral iliac osteotomy in combination with the plastic of the bladder, Schulz reported in 1954. A week after the removal of the catheter from the bladder, the girl began to hold urine. Thus, the approaching of the bones with bilateral bilateral iliac osteotomy provides a better function of the urine retention mechanism involving the muscles of the urogenital diaphragm. Later on, the plastic of the bladder became more common, especially after a large number of reports of recurrent pyelonephritis and progressive chronic renal failure appeared after the urine diversion to the intestine. However, creating a mechanism for acceptable retention of urine in patients with bladder exstrophy remains a very difficult task.

In recent decades, stage reconstruction has been widely used. Plastic in the first days after the birth of the child facilitates the reduction of bones without osteotomy and reduces dysplastic changes in the mucosa. In 1-2-year-olds, when correcting epispadias, the urethra is plastic and the penis is straightened. These procedures provide the growth of the bladder, necessary for the plasticity of its neck. Reconstruction of the cervix in combination with antireflux transplantation of the ureters into the detrusor bottom increases the volume of the bladder, and at the end of the treatment, patients usually retain urine.

Grady and Mitchell proposed in 1999 to combine primary plastic with the plastics of the penis and urethra in the newborn. However, due to a significant increase in the volume and duration of the operation, this technique was not widely used.

Thus, the modern stage of treatment of the "epiphadia exstrophy" complex is associated with surgeons' desire to provide acceptable urine retention and the formation of normal genital organs without compromising renal and sexual functions.

[1], [2], [3], [4], [5]

Epidemiology

The frequency of exstrophy ranges from 1 to 10,000 to 1 in 50,000 newborns. The presence of exstrophy in one of the parents increases the likelihood of its occurrence in children. According to summary statistics, 9 cases of exstrophy occur in 2500 siblings, and the risk of having a child with an anomaly is 3.6%. Of the 215 children born to parents with exstrophy, three inherited it (1 child for 70 newborns).

According to other data, out of 102 patients none had parents or, subsequently, their own children with this anomaly. The reasons for the discrepancies in the data are not clear. However, the risk of having a child with exstrophy in families in which someone has this anomaly appears to be about 3%. The ratio of classical exstrophy in boys and girls is 2.7: 1, respectively.

[6], [7], [8], [9], [10]