Benjamin Whittam

Mortality after Bladder Augmentation in Children with Spina Bifida

PURPOSE Renal failure has been a leading cause of death for children with spina bifida. Although improvements in management have increased survival, current data on mortality are sparse. Bladder augmentation, a modern intervention to preserve renal function, carries risks of morbidity and mortality. We determined long-term mortality and causes of death in patients with spina bifida treated with bladder augmentation. MATERIALS AND METHODS We retrospectively reviewed the records of patients with spina bifida who underwent bladder augmentation between 1979 and 2013. Those born before 1972 or older than 21 years at augmentation were excluded. Demographic and surgical data were collected. Outcomes were obtained from medical records, death records and the Social Security Death Index. Fisher exact and Wilcoxon rank-sum tests and Kaplan-Meier plots were used for analysis. RESULTS Of 888 patients in our bladder reconstruction database 369 with spina bifida met inclusion criteria. Median followup was 10.8 years. A total of 28 deaths (7.6%) occurred. The leading causes of mortality were nonurological infections (ventriculoperitoneal shunt related, decubitus ulcer fasciitis, etc) and pulmonary disease. Two patients (0.5%) died of renal failure. No patient died of malignancy or bladder perforation. Patients with a ventriculoperitoneal shunt had a higher mortality rate than those without a shunt (8.9% vs 1.5%, p = 0.04). CONCLUSIONS Previously reported mortality rates of 50% to 60% in patients with spina bifida do not appear to apply in children who have undergone bladder augmentation. On long-term followup leading causes of death in patients with spina bifida after bladder augmentation were nonurological infections rather than complications associated with augmentation or renal failure.

Long-term outcomes of catheterizable continent urinary channels: What do you use, where you put it, and does it matter?

INTRODUCTION Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are commonly used catheterizable channels with similar outcomes on short-term follow-up. Their relative long-term results have not been previously published. OBJECTIVE Our goal was to assess long-term durability of APV and Monti channels in a large patient cohort. STUDY DESIGN In this retrospective cohort study, we retrospectively reviewed consecutive patients ≤21 years old undergoing APV and Monti surgery at our institution (1990-2013). We collected data on demographics, channel type, location, continence and stomal and subfascial revisions. Kaplan-Meier survival and Cox proportional hazards analysis were used. RESULTS Of 510 patients meeting inclusion criteria, 214 patients had an APV and 296 had a Monti (50.5% spiral Monti). Median age at surgery was 7.4 years for APV (median follow-up: 5.7 years) and 8.7 years for Monti (follow-up: 7.7 years). Stomal stenosis, overall stomal revisions and channel continence were similar for APV and Monti (p ≥ 0.26). Fourteen APVs (6.5%) had subfascial revisions compared to 49 Montis (16.6%, p = 0.001). On survival analysis, subfascial revision risk at 10 years for APV was 8.6%, Monti channels excluding spiral umbilical Monti: 15.5% and spiral umbilical Monti: 32.3% (p < 0.0001, Figure). On multivariate regression, Monti was 2.09 times more likely than APV to undergo revision (p = 0.03). The spiral Monti to the umbilicus, in particular, was 4.23 times more likely than APV to undergo revision (p < 0.001). Concomitant surgery, gender, age and surgery date were not significant predictors of subfascial revision (p ≥ 0.17). Stomal location was significant only for spiral Montis. DISCUSSION Our study has several limitations. Although controlling for surgery date was a limited way of adjusting for changing surgical techniques, residual confounding by surgical technique is unlikely, as channel implantation technique was typically unrelated to channel type. We did not include complications managed conservatively or endoscopically. In addition, while we did not capture patients who were lost to follow-up, we attempted to control for this through survival analysis. CONCLUSIONS We demonstrate, durable long-term results with the APV and Monti techniques. The risk of channel complications continues over the channels lifetime, with no difference in stomal complications between channels. At 10 years after initial surgery, Monti channels were twice as likely to undergo a subfascial revision (1 in 6) than APV (1 in 12). The risk is even higher in for the spiral umbilical Monti (1 in 3).

Febrile Urinary Tract Infection After Ureteroneocystostomy: A Contemporary Assessment at a Single Institution

PURPOSE Febrile urinary tract infection represents significant morbidity in patients with vesicoureteral reflux, especially following open surgical or endoscopic treatment. The reported incidence of febrile urinary tract infection after ureteroneocystostomy varies from 10% to 24%. We investigated the incidence of febrile urinary tract infection following ureteroneocystostomy in a contemporary, single institution series. MATERIALS AND METHODS We retrospectively reviewed medical records of 395 consecutive patients undergoing ureteroneocystostomy for primary vesicoureteral reflux at our institution between 2002 and 2007. We examined demographic, diagnostic and operative data, including presence of postoperative febrile urinary tract infection. A Cox proportional hazards model was performed to assess predictors of febrile urinary tract infection following ureteroneocystostomy. RESULTS Ureteroneocystostomy was performed in 395 patients (673 ureters) at a mean age of 58 months. The most common reflux grade was III (41%). The incidence of postoperative febrile urinary tract infection was 4.6% at a mean followup of 15 months. Postoperative dysfunctional elimination syndrome was a significant predictor of febrile urinary tract infection (HR 3.8, 95% CI 1.2-12, p = 0.02), and was identified in 58 of 340 toilet trained children (15% overall). Age at diagnosis, initial presentation, age at surgery, indication for surgery, reflux grade, laterality, surgical technique and preoperative dysfunctional elimination syndrome were not predictive of postoperative febrile urinary tract infection. CONCLUSIONS The incidence of febrile urinary tract infection following ureteroneocystostomy may be lower than previously reported. The presence of postoperative dysfunctional elimination syndrome is a significant predictor of postoperative febrile urinary tract infection.

Cutting for Stone in Augmented Bladders—What is the Risk of Recurrence and is it Impacted by Treatment Modality?

PURPOSE Bladder stones are common after bladder augmentation, often resulting in numerous procedures for recurrence. We sought to determine whether surgical technique and stone fragmentation are significant predictors of bladder stone recurrence after bladder augmentation. MATERIALS AND METHODS We retrospectively reviewed 107 patients treated for first bladder stones at our institution. Patient demographics, details of surgeries, stone therapy and recurrence were reviewed. Kaplan-Meier survival and Cox proportional hazards analysis were used to determine predictors of time to first stone recurrence. RESULTS Of 107 patients 55.1% were female and 79.4% had neuropathic bladder. Patients underwent augmentation at a median age of 8.0 years (range 2.4 to 22.8) and were followed for a median of 12.4 years (1.8 to 34). Segments used for augmentation included ileum (72.9% of cases), sigmoid (16.8%), cecum/ileocecum (9.4%) and other (ureter, stomach/ileum, 1.8%). Bladder neck procedures were performed in 63.6% of patients and catheterizable channels in 75.7%. First stone surgery occurred at a median of 3.1 years after augmentation (range 5 months to 21.8 years). Endoscopy was used in 66.4% of cases and open cystolithotomy in 33.6%. Overall 47.7% of stones were fragmented. Bladder stones recurred in 47.7% of patients (median recurrence time 9.5 years, range 3 months to 14.7 years). Recurrence risk was greatest in the first 2 years postoperatively (12.1% per patient per year, p = 0.03). Recurrence risk did not change with technique (endoscopic vs open) or fragmentation, even after controlling for surgical and clinical variables. CONCLUSIONS Bladder stones recurred in almost half of the patients at 9 years postoperatively independent of treatment technique and patient characteristics. As a high risk group, yearly x-ray of the kidneys, ureters and bladder, and ultrasound of the kidneys and bladder are recommended in these patients.

All Incontinence is Not Created Equal: Impact of Urinary and Fecal Incontinence on Quality of Life in Adults with Spina Bifida

Purpose: We previously reported that the self‐reported amount of urinary incontinence is the main predictor of lower health related quality of life in adults with spina bifida. In this study we sought to determine the impact of fecal incontinence on health related quality of life after correcting for urinary incontinence. Materials and Methods: An international sample of adults with spina bifida was surveyed online in 2013 to 2014. We evaluated fecal incontinence in the last 4 weeks using clean intervals (less than 1 day, 1 to 6 days, 1 week or longer, or no fecal incontinence), amount (a lot, medium, a little or none), number of protective undergarments worn daily and similar variables for urinary incontinence. Validated instruments were used, including QUALAS‐A (Quality of Life Assessment in Spina bifida for Adults) for spina bifida specific health related quality of life and the generic WHOQOL‐BREF (WHO Quality of Life, short form). Linear regression was used (all outcomes 0 to 100). Results: Mean age of the 518 participants was 32 years and 33.0% were male. Overall, 55.4% of participants had fecal incontinence, 76.3% had urinary incontinence and 46.9% had both types. On multivariate analysis fecal incontinence was associated with lower bowel and bladder health related quality of life across all amounts (−16.2 for a lot, −20.9 for medium and −18.5 for little vs none, p <0.0001) but clean intervals were not significant (−4.0 to −3.4, p ≥0.18). Conversely, health related quality of life was lower with increased amounts of urinary incontinence (−27.6 for a lot, −18.3 for medium and −13.4 for little vs none, p <0.0001). Dry intervals less than 4 hours were not associated with lower health related quality of life (−4.6, p = 0.053) but the use of undergarments was associated with it (−7.5 to −7.4, p ≤0.01). Fecal incontinence and urinary incontinence were associated with lower WHOQOL‐BREF scores. Conclusions: Fecal incontinence and urinary incontinence are independent predictors of lower health related quality of life in adults with spina bifida. Health related quality of life is lower with an increasing amount of urinary incontinence. Fecal incontinence has a more uniform impact on health related quality of life regardless of frequency or amount.

A comparison of the Monti and spiral Monti procedures: A long-term analysis

INTRODUCTION/BACKGROUND The Monti ileovesicostomy provides an excellent substitution for an appendicovesicostomy when the appendix is unavailable or suitable for use. The spiral Monti is a useful modification to the traditional Monti as it allows creation of a longer channel when needed. In 2007, the short-term outcomes were reported; they compared traditional and spiral Monti in 188 patients with an average follow-up of 43 months. In the present population, a total of 25 subfasical revisions were performed in 21 patients: nine (8.3%) subfascial revisions in the traditional Monti (TM) patients and 12 (15.2%) subfascial revisions in the spiral Monti (SM) patients. The study found an increased risk of subfascial revisions of either TM or SM when the stoma was located at the umbilicus versus right lower quadrant (16.8% vs 6.3%, P < 0.05). On subgroup analysis, this increased subfascial revision rate appeared to be driven by SM channels to the umbilicus rather than other stomal locations, but this trend was not statistically significant. OBJECTIVE It was hypothesized that with longer follow-up, the spiral Monti would require more subfascial revisions due to progressive lengthening of the channel. STUDY DESIGN A retrospective chart review was performed for all patients undergoing a traditional Monti (TM) or spiral Monti (SM) procedure at the present institution (1997-2013). Patient demographics, bowel segment used, stomal location, channel or stomal revisions, number of anesthetic endoscopic procedures performed, and indications for revision were reviewed. Kaplan-Meier analysis and Cox proportional hazards modeling was used for analysis. RESULTS Of the 296 patients identified, 146 had Monti procedures and 150 had spiral Monti procedures (median follow-up 7.7 years). Median age at surgery was 10.6 years. Myelomeningocele was the most common underlying cause of neuropathic bladder, totaling 169 (57.1%) patients. Stomas were located at the umbilicus (106, 35.8%), right lower quadrant (183, 61.8%) and left lower quadrant (seven, 2.4%). Median follow-up for the entire cohort was 7.7 years (range: 1 month-15.7 years). Stomal stenosis rate was 7.4%, and 96.6% of the channels were continent. A total of 87 revisions were performed in 74 patients (25.0%). Of these, 55 were subfascial revisions in 49 patients (16.6%). The umbilical spiral Monti on univariate and multivariate analysis was found to be over twice as likely to undergo subfascial revision. DISCUSSION The majority of patients with a Monti channel had durable results and did not require further channel surgery with long-term follow-up. Spiral Monti channels to the umbilicus were more than twice as likely to undergo subfascial revision compared to all other Monti channels. Overall, one in three umbilical SM channels required a subfascial revision at 10 years after the initial surgery, compared to one in six of all other Monti channels. The study was limited by being a retrospective, single-center series; however, it does represent the largest series of pure SM and TM patients. It focused only on surgical interventions, thus was likely to underestimate the overall risk of complications, as some complications were managed conservatively. As in all studies, some patients were lost to follow-up and inevitably some of these may have had complications. Correction for this was attempted through survival analysis. CONCLUSION The present study reported durable and reliable long-term results with Monti and spiral Monti procedures based on a large patient cohort. Spiral Monti to the umbilicus was more than twice as likely to require a subfascial revision.

Ultrasound diagnosis of multicystic dysplastic kidney: Is a confirmatory nuclear medicine scan necessary?

OBJECTIVE It is critical to differentiate between a multicystic dysplastic kidney (MCDK) and a kidney with severe hydronephrosis as the treatment varies significantly. We designed a study to compare renal ultrasound (RUS) to nuclear medicine (NM) scan in the diagnosis of MCDK, in order to determine if RUS can be used for the definitive diagnosis of MCKD without use of NM scan. MATERIALS AND METHODS We performed a retrospective review of children with MCDK, who underwent both a RUS and Tc-99m MAG3 or DMSA scan. We planned to calculate the positive predictive value of an RUS diagnosis of MCDK, using NM scan diagnosis of a nonfunctioning kidney as the gold standard. RESULTS The diagnosis of MCDK was made by RUS in 91 patients, 84 of whom had a normal bladder US. NM confirmed the diagnosis of MCDK in all 84 of these patients (100%). CONCLUSION We have demonstrated a high predictive value for RUS in the diagnosis of MCDK. Our data support that in healthy infants with RUS diagnosis of unilateral MCDK and normal bladder US, NM scan may be unnecessary to confirm the diagnosis.

Multi-Institutional Review of Outcomes and Complications of Robot-Assisted Laparoscopic Extravesical Ureteral Reimplantation for Treatment of Primary Vesicoureteral Reflux in Children

Purpose: Robot‐assisted laparoscopic extravesical ureteral reimplantation has been proposed as a minimally invasive alternative to open ureteral reimplantation for correcting primary vesicoureteral reflux in children. However, in the current literature there are conflicting data regarding the safety and efficacy of this approach. Amid ongoing debate we analyzed outcomes and complications from this procedure in a large multi‐institutional cohort. Materials and Methods: We reviewed the records of children who underwent robot‐assisted laparoscopic extravesical ureteral reimplantation at 9 academic centers from 2005 to 2014. Radiographic failure was defined as persistent vesicoureteral reflux on postoperative voiding cystourethrogram or radionuclide cystogram. Complications were graded using the Clavien‐Dindo scale. Results: A total of 260 patients (363 ureters) underwent robot‐assisted laparoscopic extravesical ureteral reimplantation for primary vesicoureteral reflux during the study period. The cohort included 90 patients with either duplex ureter (42), failed endoscopic treatment (40) or concomitant diverticulectomy (8). Of the 280 ureters with postoperative voiding cystourethrogram or radionuclide cystogram available radiographic resolution was seen in 246 (87.9%). There were 25 complications overall (9.6%), with 7 grade 3 complications (2.7%) and no grade 4 or 5 complications. Four patients (3.9%) had transient urinary retention following bilateral reimplantation. Conclusions: Robot‐assisted laparoscopic extravesical ureteral reimplantation has a low complication rate consistent with published series of open ureteral reimplantation. Radiographic success rates are approaching those of the open procedure but continue to fall short of the gold standard approach. These findings necessitate critical thinking regarding potential technique improvements and further prospective investigation into the efficacy of this procedure.

Quality of Life Assessment in Spina Bifida for Children (QUALAS-C): Development and Validation of a Novel Health-related Quality of Life Instrument.

OBJECTIVE To develop and validate a self-reported health-related QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C). METHODS We drafted a 27-question pilot instrument using a patient-centered comprehensive item generation and refinement process. It was administered to a sample of children 8-12 years old with spina bifida (SB) recruited online via social media and in person at an outpatient SB clinic (January 2013-September 2014). Healthy controls were recruited at routine pediatrician visits. Validation and final questions were determined based on clinical relevance, high loadings on factor analysis, and domain psychometrics. Children with SB also completed the validated generic Kidscreen-27 instrument. RESULTS Median age of 150 participants was 9.6 years (60.7% male, 72.7% Caucasian), similar to 46 controls (P ≥ .10). There were 97 online and 53 clinic participants (89.0% and 84.2% of eligible, respectively). Face and content validities of the 2-domain, 10-question QUALAS-C were established by patients, parents, and experts. Internal consistency and test-retest reliability was high for the Esteem & Independence and Bladder & Bowel domains (Cronbachs alpha: 0.72-0.76, ICC: 0.74-0.77). Correlations between QUALAS-C domains were low (r = 0.51), indicating that QUALAS-C can differentiate between two distinct health-related quality of life components. Correlations between QUALAS-C and Kidscreen-27 were also low (r ≤ 0.44). QUALAS-C scores were significantly lower in children with SB than without (P < .0001). CONCLUSION QUALAS-C is a short, valid health-related quality of life tool for children with SB. It will be useful in clinical and research settings.

Current opinions regarding care of the mature pediatric urology patient

INTRODUCTION With continued improvements in pediatric urology care of patients with complex congenital genitourinary conditions, many survive into adulthood. This fact has created a challenging situation of transitioning from pediatric to adult care. Establishing long-term follow-up with appropriate specialists is a critical part of a successful transition to adulthood for this population. OBJECTIVE This study sought to elucidate current practices and opinions regarding the management of adult complex genitourinary patients by pediatric urologists, in order to determine if a consensus for adult care exists. STUDY DESIGN An anonymous, 15-question online survey was created to address practice patterns and opinions regarding the transition of care of complex genitourinary patients. An invitation to participate was distributed via email to 200 pediatric urologists who were members of the American Urological Association. Complex genitourinary patients were defined broadly as those with a history of: spina bifida, bladder exstrophy, cloacal exstrophy, cloacal anomalies, posterior urethral valves or disorders of sex development. Fishers exact test was used for analysis. RESULTS The response rate was 31.0% (62/200). Two-thirds (67.7%) cared for adults with complex genitourinary conditions. Overall, 51.6% of pediatric urologists felt that general urologists best follow adult patients, but only 6.5% recommended this for patients with prior complex genitourinary reconstruction (P < 0.001). Instead, the majority (80.6%) felt that a pediatric or adult urologist with an interest and training in adolescent/transitional urology who routinely performs such procedures would provide optimal care. Follow-up by a primary care physician alone was not recommended. Recommendations did not change if patients had developmental delay or lived independently (P = 0.47 and P = 0.72, respectively). Overall, 69.4% would refer mature complex genitourinary patients to a urologist with interest and training in adolescent/transitional urology, if one was available. However, only 45.2% had such an individual available in their practice (P < 0.001). DISCUSSION In the present study, the opinions of pediatric urologists regarding optimal providers of long-term follow-up for mature complex genitourinary patients were presented. While the results may not represent the views of the entire pediatric urology community, responses from motivated individuals with a particular interest in transition care may be especially valuable. Although the present study did not outline a mechanism for improving transitional care, it offered valuable information on prevailing opinions in this area. Finally, the opinions of mostly North American Pediatric Urologists were presented, which may not apply to other healthcare settings. CONCLUSIONS Pediatric urologists appeared to be virtually unanimous in recommending that urologists provide the most appropriate long-term follow-up of patients with congenital genitourinary conditions. Specifically, 80% recommended that patients with prior complex surgical reconstruction be followed by a urologist with specific interest, training and experience in the area of transitional urology. The data suggest that this may be an unmet need of these specialists and may signify the need for specific training in the care of such patients.