Published onUrology Update
Bladder exstrophy (BE) is one of the rarest and most complex congenital urologic disorders, affecting around 1 in every 30,000 to 50,000 births. In bladder exstrophy, the lower abdominal wall fails to develop and the baby is born with the bladder outside the body. In addition, the urethra and external genitalia are open as well, and the entire pelvis is widened and does not close into a full ring. As a result, the child is unable to store urine. Bladder exstrophy occurs on a spectrum called the Exstrophy-Epispadias Complex, which ranges from a milder defect, epispadias, where only the urethra is not fully formed, to cloacal exstrophy, where the defect is much more severe and includes gastrointestinal and spinal defects as well.
The repair of bladder exstrophy has evolved over the last century, often requiring multiple surgeries to ensure that the bladder can be closed while protecting kidney function and creating cosmetically acceptable genitalia. However, restoration of normal urinary continence with the ability to void is a persistent challenge. Historically, fewer than 20% of children born with bladder exstrophy would achieve the ability to volitionally void and maintain continence after primary repair.
A multidisciplinary approach to care
The care of bladder exstrophy at Children’s Hospital of Philadelphia (CHOP) has become a truly multidisciplinary endeavor. From the preoperative evaluation where radiology has developed an ultra-low-dose CT scan to evaluate the pubic bones; to the day of surgery, when urology teams up with orthopaedic surgery and anesthesiology to complete the reconstruction, the teamwork continues.
This multidisciplinary approach continues during the healing phase after surgery, as well as during later growth and development.
Focusing on core pelvic muscle function
We have learned that kids who develop a strong pelvic floor can be better able to achieve continence than other kids. This had been suspected, but a few children who were horseback riders really showed us that this was true. This led us to hypothesize that there are musculoskeletal deficits in children with bladder exstrophy that may contribute to limitations in functional mobility and core pelvic muscle function.
We began working closely with our pelvic floor physical therapy team to see how regimented physical therapy could aid school-age children in becoming continent. Our physical therapists began identifying impairments inhibiting continence in school-age children that were most likely present immediately postoperatively or even pre-operatively.
It was hypothesized that addressing these concerns at peak development would assist in minimizing their impact as the child continued to grow. Crucial pelvic floor muscle development occurs with developmental milestones including rolling, sitting, and crawling. These developmental skills are also essential for the development of the hip and core musculature that stabilize the pelvic floor and allow it to properly function. Because of this, the quality of a BE child’s developmental skills and the use of specific muscles are more important than just achieving the milestone.
Thus, we have partnered closely with our pelvic floor physical therapy team to provide early evaluation and intervention starting only 6 to 8 weeks after surgery. This evaluation helps to ensure that these babies, who have undergone a major reconstruction of both their lower abdomen and pelvic bones, are guided to develop in a similar way as a baby without exstrophy.
Benefits of early intervention
We have found that after exstrophy surgery, babies are rolling over later, are sitting up on their own later, and overall have decreased hip and core strength. By intervening on these impairments early, we can get them on track to develop these fundamental muscles that are imperative to later pelvic floor strength and the development of continence.
In addition, for older children, working on good breathing patterns to facilitate the use of the diaphragm, postural stability, and core and hip strengthening can all assist in the development and function of the pelvic floor.
In addition to this work postoperatively, we have begun to examine the pelvic floor diaphragm and the urethral sphincter muscle during the surgery itself using a muscle stimulator to identify contraction in the muscle (a Peña stimulator). We have demonstrated that these muscles are indeed present, and when we close the urethra and bladder neck, we are bringing those sphincter muscles together into a ring around the urethra—and we need to do everything we can to promote the development and function of these muscles.
Recommendations for evaluation by a pelvic health specialist
Currently, we recommend that every child receive an evaluation by a pelvic health specialist around 6 to 8 weeks postoperatively (which we coordinate with a routine outpatient urology follow-up visit), and then at least annually. Based on the impairments that are found, physical therapy can be recommended at any time. These therapies may include early intervention services, outpatient services with a developmental specialist, school services, or outpatient services with a pelvic health specialist.
We are now in the process of collecting data to verify our hypothesis on the effects of pelvic floor physical therapy, as we continue to get positive feedback from families who participate in the program. We have evaluated 47 children with bladder exstrophy, and are in the process of developing standardized evaluations and therapies. The typical impairments identified by physical therapy evaluation include:
- Dysfunctional breathing patterns
- Scar immobility/pain
- Poor strength and coordination of the pelvic floor
- Core and hip musculature
- Impaired range of motion of the hips
- Impaired toileting posture and positioning
- Limited sensation/awareness of the urge to void and defecate
- Impaired developmental gross motor skills including gait impairments