Epispadias and bladder exstrophy - Overview of information

Anomalies of the "epispadias exstrophy" group are a combination of a number of defects united by a single etiology and a defect on the ventral surface of at least part of the bladder and urethra. This definition was proposed by Gerhardt and Jaffe in 1996. Manifestations of the anomaly vary from cloacal exstrophy to capitate epispadias. Classical exstrophy of the bladder is expressed in the absence of the anterior wall of the lower half of the abdomen and the anterior wall. This disease is combined with epispadias and pubic symphysis divergence, and often with simultaneous underdevelopment of the testicles, with cryptorchidism, aplasia of one or two cavernous bodies, prostate hypoplasia in boys and various anomalies of the genitals in girls. Most often, this anomaly is defined as the "epispadias exstrophy" complex, which includes the following pathological conditions:

• epispadias;
• partial exstrophy;
• classic exstrophy;
• cloacal exstrophy;
• exstrophy variants.

Formally, exstrophy was first described in detail as a congenital defect in 1597 by Grafenberg, although references to this anomaly were found on Assyrian tablets made 2 thousand years BC. The first attempts at surgical treatment of the defect were made in the mid-19th century. Surgeons created a conventional anastomosis between the bladder and the sigmoid colon, but only in a few patients were they able to effectively direct urine into the rectum and hide the defect of the anterior abdominal wall. One of the first successful operations was performed by Ayres: he managed to cover the exposed mucous membrane and reduce the pain associated with its irritation. In 1906, Trendelenburg attempted to close the bladder by performing a cystectomy in combination with antireflux ureterosigmoidostomy.

1942 Young reported the first success - urinary continence with bladder plastic surgery. In the second stage, the bladder sheikh was formed into a tube, and the girl remained dry for 3 hours. However, until the 1950s, most surgeons preferred to use bladder excision and bowel diversion to correct exstrophy.

The use of bilateral iliac osteotomy in combination with bladder diversion was reported by Schultz in 1954. A week after the removal of the bladder catheter, the girl became continent. Thus, the approximation of the pubic bones by bilateral iliac osteotomy provides a better function of the continence mechanism involving the muscles of the urogenital diaphragm. Bladder diversion has subsequently become more common, especially after numerous reports of recurrent pyelonephritis and progressive chronic renal failure after bladder diversion. However, establishing an acceptable continence mechanism in patients with bladder exstrophy remains a very difficult task.

In recent decades, staged reconstruction has become widely used. Plastic surgery in the first days after birth facilitates the reduction of the pubic bones without osteotomy and reduces dysplastic changes in the mucous membrane. In 1-2-year-old children, plastic surgery of the urethra and straightening of the penis are performed during the correction of epispadias. These procedures ensure the growth of the bladder, which is necessary for plastic surgery of its neck. Reconstruction of the neck in combination with antireflux transplantation of the ureters into the fundus of the detrusor increases the volume of the bladder, and at the end of staged treatment, patients usually retain urine.

In 1999, Grady and Mitchell proposed combining primary plastic surgery with plastic surgery of the penis and urethra in a newborn. However, due to a significant increase in the volume and duration of the operation, this method has not become widespread.

Thus, the modern stage of treatment of the “epispadias exstrophy” complex is associated with the desire of surgeons to ensure acceptable urinary continence and the formation of normal-looking genitals without compromising renal and sexual functions.

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Epidemiology

The incidence of exstrophy ranges from 1 in 10,000 to 1 in 50,000 births. Having 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 in 70 newborns).

According to other data, none of the 102 patients had parents or subsequently had children of their own 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 is apparently approximately 3%. The ratio of classic exstrophy in boys and girls is 2.7:1, respectively.

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