Clinical Manifestations

Anomalies of the bladder are hypothesized to result when the mesoderm fails to invade the cephalad extension of the cloacal membrane; the extent of this failure determines the degree of the anomaly. In classic bladder exstrophy (Fig. 535-1), the bladder protrudes from the abdominal wall and its mucosa is exposed. The umbilicus is displaced downward, the pubic rami are widely separated in the midline, and the rectus muscles are separated. In boys, there is complete epispadias with dorsal chordee, and the overall penile length is approximately half that of unaffected boys. The scrotum typically is separated slightly from the penis and is wide and shallow. Undescended testes and inguinal hernias are common. Girls also have epispadias, with separation of the two halves of the clitoris and wide separation of the labia. The anus is displaced anteriorly in both sexes, and there may be rectal prolapse. The pubic rami are widely separated. Persons with exstrophy tend to be shorter than normal.

Figure 535-1 Classic bladder exstrophy in a newborn boy. The bladder is exposed in the midline, the umbilical cord is displaced caudad, the penis is epispadiac, and the scrotum is broad.

The consequences of untreated bladder exstrophy are total urinary incontinence and an increased incidence of bladder cancer, usually adenocarcinoma. The genital deformities can produce sexual disability in both sexes, particularly in males. The wide separation of the pubic rami causes a characteristic broad-based gait but no significant disability. In classic bladder exstrophy, the upper urinary tracts usually are normal.

Treatment

Management of bladder exstrophy should start at birth. The bladder should be covered with plastic wrap to keep the bladder mucosa moist. Application of gauze or petroleum-gauze to the bladder mucosa should be avoided, because significant inflammation will result. The infant should be transferred promptly to a center with pediatric urologic and anesthetic support for the treatment of such anomalies. These children are prone to latex allergy, so latex precautions should be practiced in their care.

There are 2 surgical approaches: staged reconstruction and total single-stage reconstruction. In a staged reconstruction, the initial stage is bladder closure, the second (in boys) is epispadias repair, and the final stage is bladder neck reconstruction. The single stage reconstruction attempts to reconstruct the entire malformation in a single procedure. When this operation is performed in the newborn, there is an increased risk of intraoperative penile injury and postoperative hydronephrosis, compared with the staged reconstruction. The complication rate is high with both approaches and there is no consensus on which is better.

Prompt closure of the exstrophic bladder in the newborn is preferred. During this procedure the abdominal wall is mobilized and the pubic rami are brought together in the midline. In most cases the pelvic bones must be broken (pelvic osteotomy) to allow the pubic rami to be brought together and create a pubic symphysis to support the bladder closure. Early bladder closure can be performed in almost all neonates with classic bladder exstrophy. Treatment should be deferred in selected situations when surgical therapy would be excessively risky or complex, such as in a premature baby or when it would have to be performed by inexperienced surgeons. In the staged approach, in boys, epispadias repair usually is performed at 1-2 yr of age. At this point the child has total urinary incontinence because there is no functional external urinary sphincter. At 3-6 yr, if the bladder is sufficiently large, bladder neck reconstruction is performed to try to create a functional sphincter.