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Pediatric Urinary Reconstruction: Lessons Learned over 25 Years

By: Konrad M. Szymanski, MD, MPH; Rosalia Misseri, MD | Posted on: 01 Mar 2021

It will be soon be 25 years from the time one of us (RM) graduated from medical school. The impetus to pursue a career in urology was directly related to an interest in pediatric urinary reconstruction. It was a fascination with the ability to protect the kidneys while improving bladder dynamics and function, as well as continence and quality of life.

In addition to personal lessons, the specialty as a group has learned much about the long-term outcomes of surgeries performed in children as they grow to become adults. Many factors will affect the longevity of any reconstruction performed in childhood, including surgical technique, progression or evolution of the disease, changes in body habitus and changes in social structures that occur with aging. Independence among children with congenital urological disease varies, family structure and support systems vary, and the ability to care for oneself varies as well. The ultimate goal of any urinary tract reconstruction remains, and will always remain, protecting the kidneys from progressive injury related to bladder dysfunction. In a recent retrospective review of patients with bladder augmentation with a median followup of 11.2 years, we concluded that bladder augmentation is durable but carries long-term risks of reoperation of about 44% at 10 years.1 Interventions for bladder calculi are the most common and comprise more than half of the surgeries. The 10-year risk of perforation, a more concerning and potentially life-threatening complication, is approximately 10%, and a third of those with 1 perforation will re-perforate in the subsequent 10 years. We found the risk to be greatest in those with nondetubularized bowel segments, a technique rarely used in modern times. While we think bladder augmentation has been a reliable technique to care for the hostile bladder, we found the 10-year risk of incontinent diversion to be 3%. This has been related to recurrent perforation and, perhaps in some cases, poor patient selection. Several studies have reported the risk of developing bladder cancer after bladder augmentation, but it has also become apparent that there is an inherent risk of malignancy in the neuropathic bladder even without augmentation. We have learned of criteria for endoscopy of the augmented bladder by Higuchi et al2 and have adopted these to screen for malignancy.

Despite our best efforts, we have learned that there is no optimal technique to address persistent urethral incontinence. Regardless of the technique employed, we are certain of particular needs associated with embarking on the goal of urinary continence when poor outlet resistance is to blame. The patient undergoing bladder neck reconstruction/closure or artificial urinary sphincter placement must be carefully selected. She/he must be motivated, have a strong support structure, including a person who can help troubleshoot at home and a team of health care professionals who can troubleshoot in the clinical setting, and above all must have an excellent and reliable way to empty the bladder. Despite studies suggesting that bladder neck procedures without augmentation are safe and effective in the short term, we have learned that 50% of carefully selected patients with a seemingly adequate bladder on urodynamic evaluation will require bladder augmentation within 10 years of an isolated bladder neck reconstruction or closure.3 Up to 40% of patients developed renal scarring and/or loss of function after bladder augmentation and bladder neck reconstruction in a series of patients followed longitudinally by Husmann.4 It is sobering that 69% of patients had progression of renal disease associated with poor adherence to catheterization schedules.

In addition, introduction of clean intermittent catheterization almost 50 years ago has revolutionized the fields of reconstructive and pediatric urology. The introduction of the Mitrofanoff principle has allowed us to facilitate catheterizations in children and adults. Time has taught us many tricks to avoid the angst associated with the inability to catheterize through a continent catheterizable channel. As children grow and body habitus changes, stomas may become stenotic or hidden, while channels may kink or become traumatized. To avoid these troubles, we have learned to make channels that are as short and straight as possible, and to fix the channel to the anterior abdominal wall. In a retrospective review of more than 500 continent catheterizable channels, we found that channel continence, stomal stenosis and the overall need for stomal revisions were similar for appendicovesicostomies and Monti channels. Ten years after initial surgery, Monti-Yang channels were twice as likely to require a subfascial revision (1 in 6) compared to appendicovesicostomies (1 in 12), while spiral Monti channels to the umbilicus had the highest risk of requiring a subfascial revision (1 in 3).5

Building on the achievements of the pioneers of genitourinary reconstruction, we continue to gain more insights into the long-term results of genitourinary reconstruction. Evaluating clinically meaningful outcomes has allowed us to improve appropriate patient and family counseling and selection, as well as surgical techniques and postoperative care. This particularly includes the absolute need for long-term urological care of adults who underwent genitourinary reconstruction as children; while most complications occur early after surgery, delayed complications can and do occur even after decades in patients who have done well previously.1,3,5 A critical and honest approach also implies facing the prospect that some of our assumptions may be wrong (teenagers are not at higher risk for problems with catheterizable channels6), and we may not be “as good as we think we are” (as many as half of children with spina bifida may be lost to followup transitioning to adulthood;7 adults do not irrigate their augmented bladders as repeatedly instructed8). We hope that looking at things as they really are, while keeping in mind the aspirations of patients, families and surgeons, will allow us to continue delivering ever more effective and patient-centered care.

  1. Szymanski KM, Misseri R, Whittam B et al: Additional surgeries after bladder augmentation in patients with spina bifida in the 21st century. J Urol 2020; 203: 1207.
  2. Higuchi TT, Granberg CF, Fox JA et al: Augmentation cystoplasty and risk of neoplasia: fact, fiction and controversy. J Urol 2010; 184: 2492.
  3. Whittam B, Szymanski K, Misseri R et al: Long-term fate of the bladder after isolated bladder neck procedure. J Pediatr Urol 2014; 10: 886.
  4. Husmann DA: Long-term complications following bladder augmentations in patients with spina bifida: bladder calculi, perforation of the augmented bladder and upper tract deterioration. Transl Androl Urol 2016; 5: 3.
  5. Szymanski KM, Whittam B, Misseri R et al: Long-term outcomes of catheterizable continent urinary channels: what do you use, where you put it, and does it matter? J Pediatr Urol 2015; 11: 210.e1.
  6. Szymanski KM, Whittam B, Misseri R et al: A case of base rate bias, or are adolescents at a higher risk of developing complications after catheterizable urinary channel surgery? J Pediatr Urol 2017; 13: 184.e1.
  7. Szymanski KM, Cain MP, Hardacker TJ et al: How successful is the transition to adult urology care in spina bifida? A single center 7-year experience. J Pediatr Urol 2017; 13: 40.e1.
  8. Peycelon M, Szymanski KM, Francesca Monn M et al: Adherence with bladder irrigation following augmentation. J Pediatr Urol 2020; 16: 33.e1.

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