Caring for Children With the Exstrophy/Epispadias Complex

Bladder exstrophy is a severe developmental disorder in which the bladder protrudes inferior to umbilical cord insertion and separation of the pubic bones results in divergence of the rectus muscles. In males, epispadias of the urethra, where the meatus is on the top of the penis and creates a gutter from the point of the opening to the tip of the glans, is also part of the condition.

Prenatally, bladder exstrophy is suspected when there is notation of an absent bladder, but normal amniotic fluid volume, in conjunction with a lower midline ventral wall mass, abnormal appearing genitalia and bony pelvis malformation. The key defining ultrasound feature is a lower abdominal wall deformity with a low cord insertion that is cephalad to the exstrophied bladder. The fetal kidneys are usually sonographically normal.

Surgical repair

The Center for Reconstructive Surgery within The Children’s Hospital of Philadelphia’s Division of Urology is a specialized program for children with bladder exstrophy and epispadias. The Center also follows children who have isolated epispadias without exstrophy of the bladder, as they will need reconstructive surgery on the penis and urethra and may have challenges with continence. The Center’s surgical team includes two urologists, an orthopaedic surgeon and an anesthesiologist who work together to do Complete Primary Repair of Bladder Exstrophy (CPRE), meaning the bladder is closed at the same time the urethra is reconstructed. The goal of this surgery, which typically occurs four to six weeks after birth, is to have the bladder fill and empty normally to preserve function.

Osteotomies, where the pubic bones are cut, rotated and set in a more optimal position to support the bladder, are also usually performed at this time. The Center has surgically reconstructed 14 children with bladder exstrophy and 11 children with epispadias since 1996 using the CPRE.

• Of the 14 with bladder exstrophy, 13 are older than toilet-training age. Two have had their bladders augmented with their colon and the bladder neck closed and they now empty the augmented bladder through clean intermittent catheterization. One child is an infant and too young to have continence assessed. The remaining patients are voiding with dry intervals, but have not achieved full continence, meaning they have some daytime dampness and some are also wet at night.
• Of the 11 with isolated epispadias, seven are older than toilet-training age. None has undergone secondary bladder neck reconstruction. All are voiding with dry intervals and five of the seven are completely dry day and night. Having an orthopaedist perform osteotomies at the first surgery makes a difference in long-term continence, especially for these patients.

After the initial surgery, the Center follows these children and their families closely, obtaining regular renal bladder ultrasounds and tracking continence over time. Use of bulking agents to improve resistance at the bladder neck has helped children achieve a social level of dryness without the need for a large augmentation procedure. A pediatric psychologist in the Division of Urology works with children and their parents throughout their care at CHOP to facilitate coping and adjustment to this chronic condition.