Bladder exstrophy (BE) is widely recognized as one of the most challenging congenital urological conditions for the treating urologist, both because of its rarity as well as the technical challenges associated with surgical reconstruction. With a prevalence of approximately 1:40,000 live births, most pediatric urologists even at academic centers can expect to see only a handful of cases in a career. Therefore, collaborative models of care have becoming increasingly frequent. Starting with the Multi-Institutional Bladder Exstrophy Consortium (MIBEC) among Boston Children’s Hospital, Children’s Hospital of Philadelphia and Children’s Hospital of Wisconsin in 2012, pediatric urologists are increasingly joining forces to increase surgical volume to share clinical outcomes and to assess, define and evaluate the care and treatment of patients with BE.

Within the past 3 years, multiple recognized academic centers of excellence for bladder exstrophy have released long-term outcomes data detailing volitional voiding, dry intervals and used of clean intermittent catheterization (CIC) among BE patients. In 2019 Szymanski et al reported pooled data from the Pediatric Urology Midwest Alliance (PUMA) demonstrating a CIC rate of 67.4% at last known followup visit with median followup of 14.4 years.¹ Patients in the study underwent primary reconstruction between 1980 and 2016. The majority of older patients in this cohort (70.1%) underwent bladder augmentation or other urinary diversion by age 18. Similarly, Maruf et al from Johns Hopkins recently reported volitional voiding per urethra with at least 3-hour dry intervals in 80 (23%) of their cohort of 350 patients with BE for whom continence could be assessed (median followup 14.8 years).² We have similarly examined our BE cohort before the initiation of our MIBEC partnership, finding that 20.4% of patients met criteria for volitional voiding per urethra with dry intervals of at least 3 hours (median age at followup 12.1 years).³

While volitional voiding rates, dry intervals and use of CIC offer easily reportable metrics, they only hint at the end goal for BE patients and surgeons: urinary continence. There remains no standard definition of continence within the BE population or in the field of pediatric urology at large. Within this vacuum, the most commonly reported outcome is a binary differentiation between those who can be dry for 3 hours at a time and those who cannot. Whether patients utilizing CIC can be considered continent remains debated.⁴

As BE surgeons continue to study and refine BE reconstructive techniques, the need for a more nuanced and universal definition of continence continues to emerge. Our own experience within MIBEC has shown us that there is a significant subset of patients who achieve and are satisfied with a 2-hour to 3-hour dry interval with volitional voiding per urethra.³ Many of these patients express that further reconstructive surgery to achieve a longer dry interval with reliance on CIC would diminish their quality of life. We must listen to these patients when they tell us that dryness and continence are not the same. Additional work by Ellison et al in Seattle suggests that continence can be a process for BE patients after reconstruction with a proportion of patients attaining continence years after their last surgical reconstruction.⁵ Our multi-institutional experience mirrors this finding, and we often celebrate with patients and parents as they slowly achieve longer dry intervals with normal growth and development, often aided by physical therapy. Still, continence cannot be achieved without successful surgery to restore anatomic bladder and pelvic floor anatomy. Even more importantly, if we are impatient and rush to bladder augmentation and diversion we may inadvertently deprive a subset of patients from achieving true continence with volitional voiding per urethra.

While a universal definition of continence remains fleeting, the concept of continence after exstrophy closure continues to evolve as pediatric urologists continue to work collaboratively to examine and report long-term clinical outcomes. We acknowledge that there is great room for improvement in achieving continence after exstrophy closure as continence by current metrics is only achieved by a minority of patients in these studies. Nevertheless, there remains a persistent 20% to 30% of patients who demonstrate durable volitional voiding per urethra with significant dry intervals for years after initial surgical reconstruction regardless of institution or surgical technique. In our MIBEC cohort, analysis of 28 patients with at least 3 years of followup after initial surgical reconstruction demonstrated that 32% of patients were achieving dry intervals of at least 1 hour at a young age, the majority after a single surgery for continence.⁶ Therefore, we are heartened that continued refinement of BE reconstruction along with diligent long-term clinical care and physical therapy can lead to continued improvement in continence both in terms of our understanding of continence as well as the proportion of patients who achieve it.