Bladder exstrophy is a congenital malformation of the genitourinary system characterized by the absence of the anterior abdominal wall and the anterior wall of the urinary bladder, splitting of the urethra, and separation of the pelvic bones up to 7 centimeters.
Clinical presentation
In affected children, protrusion of the mucosa of the posterior bladder wall is observed in the suprapubic region; it has a bright red appearance. In boys with bladder exstrophy, urine constantly drains to the outside from the ureteral orifices. The skin in this area gradually scars and becomes macerated due to continuous contact with urine. The umbilicus is often absent. The penis is reduced in size and is most often split.
In girls, the urethra is also split, the clitoris is divided into two parts, and the labia may be represented by two separate halves. The vagina is covered by the mucosa of the bladder and urethra. In addition, there is separation of the pelvic bones at the level of the pubic symphysis up to 7 centimeters, which leads to gait disturbance. Constant contact of the bladder mucosa with surrounding tissues and the external environment results in the development of cystitis and pyelonephritis, followed by progressive renal scarring.
In addition to bladder exstrophy, these children may present with cryptorchidism, inguinal hernias, various malformations of the ureters and kidneys, and possible fecal incontinence with prolapse of the rectal mucosa. In girls, malformations of the female reproductive system may be present, including vaginal duplication and a bicornuate uterus.
Children are largely deprived of the ability to communicate and play normally with their peers, and a persistent odor of urine is often present.
Treatment of bladder exstrophy
Bladder exstrophy is treated exclusively by surgical methods. Correction of the defect should be performed early in life. All types of surgical procedures are carried out from birth up to 1–2 years of age, before scar changes develop in the tissues surrounding the bladder. The extent of surgical intervention depends on the size of the exstrophy, the general condition of the child, and the child’s sex.
Surgical management of this condition consists of several stages:
- Reconstruction of the anterior abdominal wall and the anterior wall of the urinary bladder;
- Correction of urethral splitting and reconstruction of the penis;
- Management of urinary incontinence, with possible bladder augmentation.
Primary bladder reconstruction is performed during the first two weeks of life. During this procedure, the bladder is formed using local tissues. This is followed by closure of the pubic bone diastasis with reinforcement of the ligamentous apparatus of the pelvic organs. The anterior abdominal wall is reconstructed, and a channel for urine outflow through the urethra is created. In girls, reconstruction of the external urethral orifice is performed together with reconstruction of the labia majora and minora.
At the age of 1–2 years, boys undergo reconstructive surgery to correct epispadias.
Between the ages of 5 and 7 years, the external urethral sphincter and bladder neck are reconstructed, as these structures play the most important role in urinary continence. If bladder growth is insufficient and its capacity remains small, bladder augmentation is performed by increasing its volume using a segment of the small intestine.
Advantages of treating bladder exstrophy in Belarus
In leading pediatric surgical centers in Belarus, children with bladder exstrophy receive highly professional medical care. Accumulated clinical experience and the use of modern equipment that meets international standards ensure favorable treatment outcomes and help children achieve a better quality of life.
Timely surgical management can prevent the development of severe complications associated with this condition.
