EXSTROPHY OF THE BLADDER

Exstrophy of the bladder, which occurs once per 30,000 live births, represents one of the most difficult challenges to the urologic surgeon. For the family, however, during the past decade, with experience, understanding of the abnormality, and persistence, the overall outlook has become brighter.

At birth, the diagnosis is generally immediately apparent. These are usually healthy babies with rare anomalies unrelated to the genitourinary and orthopedic defects. The exposed bladder is variable in size and generally has a soft, pliable membrane at birth. The kidneys are almost always normal. The umbilicus is low set and located just above the bladder. The bones of the pelvis (pubic rami) are widely separated. Boys will have a short penis with an upward bend (chordee). In the female, the clitoris is bifid. The vagina is usually normal, although sometimes may be short with a narrow opening. Oftentimes, the rectum is somewhat closer to the genitalia than usual.

The surgical treatment of bladder exstrophy is complex and constantly evolving. Goals of therapy include a cosmetically acceptable appearance of the abdominal wall and genitalia, preservation of kidney and sexual function, and urinary continence. Optimum treatment of children with exstrophy of the bladder requires immediate care by a surgeon with expertise and interest in this problem. Our bias in the treatment of exstrophy is primary bladder closure if at all possible and staged reconstruction. A great deal of time must be spent with parents of children with exstrophy early on so that the parents begin to understand the implications of the malformation and the complexity of the surgical reconstruction. This is the beginning of a lifelong commitment to these patients and their families.

Closure of the exstrophied bladder should be performed, ideally, within the first 48 hours of life, if at all possible. Within this time frame, the bones are pliable, the changes in the bladder lining have not occurred yet and the bladder and abdominal wall can usually be closed without disrupting the bony pelvis (iliac osteotomies). Closure after that time usually does require iliac osteotomies. At the initial operation in both boys and girls, an attempt is made to more normalize the appearance of the external genitalia. Boys actually have normal sized organs which are splayed out on the bones and when the bones are brought together, the corporal bodies of the penis are as well, and the penis has more length. In girls, the halves of the clitoris are also put together. Patients are usually in traction for approximately three weeks following the operative procedure.

After an interval of observation, which lasts usually for several years, surgery is then done for continence and to correct reflux (urine backing up to the kidneys). In boys whose bladder capacity is somewhat small, the operation for continence is sometimes preceded by surgery on the opened penis (epispadias) to increase resistance, and this oftentimes increases bladder capacity.

The goal of this staged reconstruction is to have patients with a normal urinary tract, satisfactory external genitalia, and adequate dry intervals. Some children may require clean intermittent catheterization to adequately empty their bladders. Some children need "fine tuning" of their repairs and continence. Recently, the Federal Drug Administration released for general use a collagen substance which can now be injected at the neck of the bladder to increase resistance and continence. I believe that this may just be the beginning of the use of various substances (many of them natural) to improve urinary control.

Urinary control is often further improved at puberty with the growth of the prostate. Adolescence is sometimes a difficult time for boys. Some may need further surgery on the external genitalia to correct any further bends that may develop with growth. Both boys and girls may require surgery on the mons pubis to recreate the normal hair distribution. Most males and females with exstrophy of the bladder can and do have a satisfactory sexual life, and can become parents.

Some patients may have bladders so small that they cannot be satisfactorily closed. There are a number of options available for these patients to lead a very satisfactory life.

I think that the future is bright for patients with exstrophy of the bladder. There are many more options available now and the methods of treatment continue to improve.

This article originally appeared as "Exstrophy of the Bladder - Advances in Management" by Dr. Alan B. Retik, M.D. in The Inside Edition

The information provided in this section should in no way serve as medical advice. Readers are encouraged to confirm the information contained here with other sources and seek medical advice from a physician. Neither the authors nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete and they are not responsible for any errors or omissions or for the results obtained for the use of such information.

Information provided by the Division of Urology, Children's Hospital, Boston, MA.

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