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We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup.We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000).A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%.Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.

We assessed the probability of bladder augmentation/diversion and clean intermittent catheterization in classic bladder exstrophy in a multi-institutional cohort.We included children born from 1980 to 2016 with bladder exstrophy and treated across 5 centers (exclusion criteria less than 1 year followup after birth, isolated epispadias, bladder exstrophy variants etc). Outcomes were probability of bladder augmentation/diversion after bladder closure and proportion of patients performing clean intermittent catheterization at last followup. Survival analysis was used.Of 216 patients 63.4% were male (median followup 14.4 years). Overall 4 patients (1.9%) underwent primary diversion and 212 underwent primary closure (72.6% in first week of life). After primary closure 50.9% underwent augmentation, 4.7% diversion and 44.8% neither. By age 18 years 88.5% underwent a bladder neck procedure (synchronous augmentation 27.3%). On survival analysis the probability of bladder augmentation/diversion was 14.9% by age 5 years, 50.7% by 10 years and 70.1% by 18 years. Probability of bladder augmentation/diversion varied significantly between centers (p=0.01). Probability of bladder augmentation/diversion was 60.7% 10 years after bladder neck procedure. At last followup of the entire cohort 67.4% performed clean intermittent catheterization. Among 95 patients with intact native bladders 30.5% performed clean intermittent catheterization (channel 72.4%). Among 76 adults without a diversion 85.5% performed clean intermittent catheterization (augmented bladder 100.0% clean intermittent catheterization, native bladder 31.3%). Fifteen patients underwent diversion (continent 8, ureterosigmoidostomy 5, incontinent 2).On long-term followup probability of bladder augmentation/diversion increased with age, with 1 in 2 patients by age 10 years and the majority in adulthood. Probability of bladder augmentation/diversion differed among institutions. Almost a third of patients, including adults, with a closed native bladder performed clean intermittent catheterization. Considering all adults only 14% did not perform clean intermittent catheterization.

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.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).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, p0.0001) 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, p0.0001). 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.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.

Screening for vesicoureteral reflux in asymptomatic infants with low grade hydronephrosis is unjustified if this condition is benign. We present a cohort with prenatally detected hydronephrosis, some observed without voiding cystourethrography, and compare outcomes to a classic, screened group.A total of 206 consecutive children presenting with postnatally confirmed prenatally detected hydronephrosis (47% bilateral) were included in the study. Cases with associated renal or bladder anomalies were excluded. Children with low grade hydronephrosis either underwent screening voiding cystourethrography or did not, largely based on whether care was managed by a urologist or a nephrologist. Patients with high grade hydronephrosis routinely underwent voiding cystourethrography. Primary outcome was a symptomatic or febrile urinary tract infection. We used Mantel-Haenszel analysis to determine urinary tract infection risk factors during the first 2 years of life.No urinary tract infection was observed in patients with grade I hydronephrosis. Urinary tract infections in low grade hydronephrosis were only seen in the voiding cystourethrogram group (7 patients), including 1 infection following voiding cystourethrogram. Urinary tract infection rate was 3.52 infections per 100 patient-years in children with low grade hydronephrosis and 11.1 infections per 100 patient-years in those with high grade hydronephrosis (p = 0.02). This increased risk of urinary tract infection in high grade hydronephrosis persisted after correcting for gender and circumcision status (IRR 3.17, p = 0.01). The association remained strong (IRR 2.48, 95% CI 0.96-6.44) but was not statistically significant (p = 0.053) after additionally correcting for vesicoureteral reflux status.Our data suggest that children with low grade hydronephrosis and otherwise normal kidneys and bladder do not benefit from voiding cystourethrographic screening. Interestingly high grade hydronephrosis appears to carry a threefold greater risk of urinary tract infection compared to low grade hydronephrosis.

Purpose: The literature on minimally invasive nephrectomy in adults and children on peritoneal dialysis is sparse. Case reports suggest that the transperitoneal approach is effective. We present our experience with retroperitoneoscopic nephrectomy in children on peritoneal dialysis. Materials and Methods: At 11 consecutive retroperitoneoscopic nephrectomies a total of 14 kidneys were removed from 10 children with a mean age of 12 years. We used a 3-port lateral retroperitoneoscopic nephrectomy technique with active trainee participation. Preoperative and postoperative biochemistry results within 3 months of surgery were compared with the Wilcoxon signed rank test. Results: Three bilateral synchronous, 1 bilateral staged and 6 unilateral retroperitoneoscopic nephrectomies were done. Mean operative time was 174 minutes for unilateral and 458 minutes for bilateral nephrectomy, including 1 simultaneous peritoneal dialysis insertion and 1 umbilical hernia repair. No open conversion, blood transfusion or postoperative surgical complication was noted. Peritoneal dialysis was initiated at a median of 9 hours postoperatively and dialysate volume was titrated to target within a median of 60 hours. One patient with a small peritoneotomy needed temporary hemodialysis despite intraoperative airtight repair. After surgery median serum albumin increased from 30.0 to 34.3 gm/l. Conclusions: Retroperitoneoscopic nephrectomy for end stage renal disease is a safe, effective technique that preserves peritoneal integrity in children who require immediate postoperative peritoneal dialysis. Avoiding post-nephrectomy hemodialysis decreases patient morbidity, preserving vessels for future vascular access. Compared to the literature on laparoscopy in this setting, retroperitoneoscopic nephrectomy can be considered the ideal approach for minimally invasive nephrectomy in patients on peritoneal dialysis.