Exstrophy is a disorder that affects the normal development of the bladder, urethra (the tube that carries urine from the bladder), and sometimes the intestines. It is part of a group of related disorders called the classic bladder exstrophy-epispadias-cloacal exstrophy complex.
In addition to causing problems with the bladder and the intestines, these rare disorders of newborns also affect the genitals, as well as the bones and muscles of the pelvis and belly.
Exstrophy Surgical Options
The only treatment available for exstrophy and epispadias is surgery. Shortly after birth, a pediatrician and surgeon will examine the newborn and decide the best course of treatment.
The two main surgery options are:
- Total reconstruction: all repairs are made during one procedure
- Staged reconstruction: repairs are made during multiple surgeries over the course of several years (from newborn to 4 or 5 years, with some surgeries done later in life).
The surgical option chosen depends upon the extent of the defects and the preference of the surgeon. While the staged approach allows the newborn to recover in between each surgery, the safety and effectiveness of both options are similar when the procedure is done by experienced surgeons.
The main objectives of surgery are to:
- Close the bladder, pelvis and urethra
- Rebuild the penis (males) or external genitals (female) so they work properly and are cosmetically pleasing
- Allow the child to hold urine and urinate properly (continence)
- Ensure that the kidneys are working properly
Bladder Exstrophy Surgery
Bladder exstrophy involves the bladder and urethra, with occasional spinal cord abnormalities. The procedures done during modern staged reconstruction for bladder exstrophy are identical to those done during a total reconstruction, except they are spaced out over a period of time.
In general, surgery includes:
- Closure of the bladder and the pelvis
- Rebuilding of the urethra to increase its resistance to the flow of urine, which improves the capacity of the bladder
- Surgery on the neck of the bladder to improve its function
- Procedures to increase urine control and improve the appearance of the external genitals.
Depending upon the extent of the defects, some of these stages may be combined. Also, when the bladder is too small to be closed properly, the flow of urine may be rerouted using a procedure called a urinary diversion.
Epispadias affects only the urethra and external genitals, so surgery for this involves only repair of those two parts of the body.
Cloacal Exstrophy Surgery
As with bladder exstrophy, reconstruction for cloacal exstrophy can either be done at the same time (total) or staged. Since cloacal exstrophy also involves the intestines and sometimes the spinal cord, more extensive surgeries may be needed.
The general stages for this condition are:
- Separating the intestines from the urinary and genital tracts, closing the colon, creating an opening for the colon to the outside, and closing the hole in the abdominal wall (omphalocele)
- Further work on the intestines to allow the child to control the bowels
- Closure of the bladder (as with bladder exstrophy)
- Repair of the genitals (as with bladder exstrophy)
Exstrophy Surgery Complications
Since surgery for exstrophy can be extensive, one common complication is a severe allergic reaction (anaphylaxis) to latex found in surgical gloves and tools. Latex allergies occur in 30 percent of newborns undergoing surgery for exstrophy, with 70 percent showing signs of partial sensitivity to latex.
Other surgical complications include:
- Incomplete closure of the bladder, which can require additional surgeries or rerouting of the flow of urine (urinary diversion)
- Hole in the urethra, which may close on its own or require surgery
- Abnormal bladder function, such as incomplete emptying, increased risk of bladder stones, rupture of the bladder or bladder infections.
Exstrophy Surgery Outcomes
Survival rates for exstrophy surgeries are excellent. By adolescence, most children will have continence and cosmetically acceptable external genitals. For bladder exstrophy, staged reconstruction surgery results in 75 to 90 percent of newborns being able to hold and control their urine (continence).
The outcome of surgery depends upon the quality of the bladder at birth, the initial closure of the bladder done on the newborn, the type of reconstruction used, and the experience of the surgeon.
Surgery for cloacal exstrophy is more involved, but new techniques have improved the outcomes. Less than 25 percent of newborns who undergo staged reconstruction will have full control of their urine, due to problems with the nerves that control the function of the bladder.
Other types of bladder reconstruction (using rectal reservoirs or augmenting the bladder with the intestines) have success rates of 90 to 95 percent.
Most males who have had surgery for exstrophy are able to achieve erections during adulthood. However, some may have a smaller or curved penis. They may also have lower quality semen, caused by blockage of the tubes that carry sperm from the testicles. Problems with fertility can be improved using assisted fertility techniques.
Females should have normal sexual abilities and some have even successfully had children. Doctors recommend that females undergo cesarean section during childbirth to prevent injuries to the organs that help them control their urine.
References
Elder JS. (2011). Anomalies of the bladder. Nelson Textbook of Pediatrics. 19th ed.
Gambhir L, Höller T, Müller M, et al. (2008). Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol.
Gearhart JP, Mathews R. (2011). Exstrophy-epispadias complex. Campbell-Walsh Urology. 10th ed.
Jayachandran D, Bythell M, Platt MW, et al. (2011). Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol.
Stein R, Hohenfellner K, Fisch M, et al. (1996). Social integration, sexual behavior and fertility in patients with bladder exstrophy--a long-term follow up. Eur J Pediatr.
