Birth Defects Genital Urinary Tract Bladder Exstrophy

Bladder exstrophy is a rare congenital defect of the urinary system in which part of the bladder forms essentially inside out, and protrudes outside the body.

The urethra is the tube by which urine passes from the bladder out of the body. As the fetus develops, the bladder and urethra are normally contained by the abdominal wall. However, in bladder exstrophy, the urethra is incompletely formed, and the bladder is not contained in the abdominal cavity.

Causes and Symptoms of Bladder Exstrophy

Reasons for exstrophy of bladder are not fully understood. One theory suggests that overdevelopment of the cloacal membrane, a temporary membrane present during fetal development, can prevent the muscle and skin of the abdominal wall from developing properly. This can lead to herniation of the bladder, so that it is protruding outside the abdominal wall. In more severe cases of bladder exstrophy, the bowel can also be affected.

Bladder exstrophy can be associated with other defects of the urinary system:

  • Abnormal development of abdominal muscles
  • Shortened urethra and vagina or penis
  • Widened pubic bone.

Bladder exstrophy is also associated with epispadias, a condition in which the opening of the urethra is abnormally located. In addition, the urethra may not have complete closure. This condition can occur in both males and females. The penis or clitoris may be split because of incomplete urethral closure. The severity of bladder exstrophy can vary widely, as can the occurrence of related conditions.

Treatment Options for Bladder Exstrophy

Bladder exstrophy may be diagnosed by fetal ultrasound; if not, it is diagnosed immediately at birth. Decisions can then be made about appropriate courses of treatment, which always involve surgery. Several options exist for the course of bladder exstrophy repair.

The staged procedure uses multiple surgeries. These occur as the child develops:

  • Newborn: closure of the bladder, posterior urethra and abdominal wall
  • Six Months to one year of age: repair of the epispadias
  • Between the ages of four and five: repair of the bladder neck (allows for bladder control).

Complete primary repair of exstrophy (CPRE) completes all surgical repairs at once, with the goal of promoting optimal and natural growth and development of the bladder. This surgery allows for normal bladder function, including urinary continence, to begin sooner.

Bladder exstrophy repair generally results in a high level of success in urinary continence. However, in some cases, repeat surgery may be necessary. Careful post-surgery monitoring is critical, as urinary tract infections may develop after the procedure.

Resources

Association for the Bladder Exstrophy Community. (n.d.). Frequently asked questions.Retrieved April 18, 2010, from: http://www.bladderexstrophy.com/learning_center_faq.htm.

Children’s Hospital Boston. (n.d.). About bladder exstrophy.Retrieved April 18, 2010, from: http://www.childrenshospital.org/clinicalservices/Site1697/mainpageS1697P7.html.

Children’s Hospital Boston. (n.d.). Exstrophy of the bladder and epispadias.Retrieved April 18, 2010, from: http://www.childrenshospital.org/az/Site856/mainpageS856P0.html.

Medline Plus. (n.d.). Epispadias.Retrieved April 18, 2010, from: http://www.nlm.nih.gov/medlineplus/ency/article/001285.htm.