It is a severe anomaly that occurs as a result of the incomplete closure of the anterior abdominal wall below the navel.
It is the failure of the anterior abdominal wall to close below the umbilicus due to abnormal development of the cloacal membrane and consequent inhibition of the migration of mesenchymal tissue.
5% of the cases are in the form of bladder exstrophy.
Exstrophy complex:
• Pubis diastasis (pseudoexstrophy)
• Covered exstrophy
• isolated epispadias
• Superior vesical fissure
• Duplex exstrophy
• Cloacal exstrophy
Exstrophy vesica occurs at a frequency of 3.3/100000 births, is common in males and is common in children of young mothers.
Contrary to exstrophy cloaca anomaly, other system anomalies are rare in bladder exstrophy.
Exstrophy vesica occurs in the weeks 4-10. The principal defect is excessive thickening of the cloacal membrane.
Classical bladder exstrophy: rupture of the cloacal membrane after the urorectal septum has completed its descent; a previous rupture also results in cloacal exstrophy, as both the bladder and hindgut are exposed.
Exstrophy-epispadias complex
• They are usually term babies, their birth weights are normal and there are no significant additional anomalies.
• The umbilicus is just above the bladder, there may be an umbilical hernia together.
• It is common in bilateral indirect inguinal hernia and undescended testis.
• Rarely, omphalocele is also present (exstrophy is more common in the cloaca).
• In the exstrophy-epispadias complex, the defect in the bladder and anterior abdominal wall is closed, the symphysis pubis is joined to the midline, urinary continence is maintained by preserving the renal functions, and the external genital organs are corrected functionally and cosmetically.
Bladder exstrophy
• The part of the anterior abdominal wall below the navel is the bladder, external genitalia, pelvic bones, rectum and anus defect.
• In these, it is seen that the posterior wall of the bladder, which is open under the umbilicus, becomes evident with crying, and urine leaks continuously from the ureteral openings onto the bladder mucosa.
• Bladder mucosa is normal at birth; however, it soon becomes covered with edematous and polypoid structures.
• If untreated, this metaplasia ends with SCC and adenocarcinoma.
• Bladder size, elasticity and neuromuscular function are the most effective factors in primary repair.
• Pubic arms are open and externally rotated. The lower extremities also participate in this external rotation (shaking).
• The more open the pubis, the larger the extrophic bladder.
• The pelvic floor is weak and the external sphinter anatomy is defective. Rectal prolapse and anal incontinence are common. Watery stool cannot be controlled.
• Perineum is short and anus is located anteriorly.
• Anal atresia; There may be rectovaginal and rectoperineal fistulas.
• The penis is short and curved upwards due to the "dorsal cord". Most of them are infertile.
• In girls; urethra and vagina are short in length. The labia are separated in the midline, the vaginal orifice is narrow. Internal genitalia are normal, uterus may prolapse. The clitoris is symmetrical structures on both sides of the urethral plate.
• Genital organ reconstruction in girls is much easier than in boys, and the formed organ is more functional.
• Surgery is required in more than one session in exstrophy vesicle.
Isolated epispadias
• The urethra is located proximal to varying degrees on the dorsal aspect of the penis.
• In isolated cases without exstrophy, the urethral meatus may be in the glans, penile body or peno-pubic (complete) region and is most commonly located in the peno-pubic region.
• While most penile and penopubic cases are incontinent, all of the granular type are continent.
• Pubic arms are open, penis is short.
• Almost all cases have dorsal chordee.
• Most of the patients with penopubic epispadias are infertile due to retrograde ejaculation and incontinent due to bladder neck failure despite surgical repair.
• Upper urinary system anomalies such as renal agenesis, renal ectopia, ureterovesical obstruction and VUR may also be seen.
• In the treatment, functional and cosmetic external genitalia is created in order to maintain continence by protecting the upper urinary system.
Exstrophy cloaca
• It is the most serious of the congenital anterior abdominal wall defects.
• It is seen with a frequency of 1/2000000-400000 and is 2 times more common in men.
• Beneath the omphalocele sac, there are intestinal mucosa, terminal ileum, and orifices of the large intestine that are curved outwards in the middle.
• The large intestine is very short and the terminal ileum has prolapsed.
• There are right and left parts of the bladder on both sides of the intestinal tissue in the middle.
• Below, there are two parts of the penis or labia and clitoris.
• There is anal atresia.
• Symphysis pubis did not join in the midline.
• Concomitant spinal dysraphism and vertebral anomalies (most commonly lumbar), urinary system anomalies, vaginal duplication, vaginal atresia, uterus duplication, ovarian cyst, undescended testicle, omphalocele, rotation anomalies, intestinal duplication, Meckel's diverticulum, short bowel syndrome are common.
• The aim of treatment is surgical repair in stages or in a single session.