Reoperation for Channel Complications in Children With Continent Cutaneous Catheterizable Channels: The Test of Time.

OBJECTIVES: To examine the durability of continent cutaneous catheterizable urinary channels (CCCC) in children and assess whether channel complications continue to arise with extended follow-up. Previous studies demonstrated that complications of CCCC cluster in the early years following surgery. METHODS: The database of a tertiary center was queried for patients≤21 years who underwent CCCC. Patients with <6 years of follow-up were excluded. Patients were invited for follow-up to assess continence. Clinic visits and hospital admissions were reviewed for channel complications requiring reoperation. Complications were analyzed against patient and channel characteristics and time since initial surgery. RESULTS: Between 1993 and 2012, a total of 120 patients underwent CCCC at a median age of 6.8(0.4-21) years and a median follow-up of 11.4(6.6-27) years. CCCC were created using the appendix, Monti channels and tapered ileal segments in 74(61.7%), 33(27.5%) and 13(10.8%), respectively. Continence relied on the extra-mural serous lined principle in 85.8% and the stoma was anastomosed to the umbilicus in 90%. Dryness with catheterization intervals of 3 hours or longer was eventually achieved in 90.8% with similar rates among different channel types (P=.149). 26(21.7%) required 42 interventions to treat channel complications with 32.5% occurring >5 years following initial surgery irrespective of the channel type (P=.978). On multivariate analysis, ileal channels had 3.372 higher odds of needing reoperation compared to appendicovesicostomy (95%CI=1.240-9.166; P = .037). CONCLUSION: A high reoperation rate is anticipated throughout the lifetime of CCCC. Appendicovesicostomy has a low complication risk relative to ileal channels.

Durability of oncological outcomes of combination chemotherapy as a monotherapy for a select patient subset with non-metastatic non-alveolar bladder/prostate rhabdomyosarcoma.

INTRODUCTION AND OBJECTIVES: We aim to assess the long-term oncological outcomes of children with bladder/prostate rhabdomyosarcoma (B/P RMS) treated with multiagent chemotherapy as a monotherapy. We hypothesize that a highly select patient subset can be treated with multiagent chemotherapy as a monotherapy and spared the morbidity of local treatment with similar oncological outcomes. METHODS: Patients (≤21-year-old) treated for non-metastatic non-alveolar B/P RMS at a tertiary center and followed for>one year, were retrospectively reviewed. After pathological confirmation, patients received 12 weeks of induction VAC chemotherapy (IC) followed by second-look biopsies. Between 1996 and 2006 (group A), patients with>50% tumor size reduction and negative second-look biopsies following IC were spared local treatment and followed-up closely. Between 2007 and 2020 (group B), local treatment was routinely given at 12 weeks according to the COG protocols, irrespective of IC response. For all patients, consolidation chemotherapy was administered for additional 12-18 months. RESULTS: Between 1996 and 2020, 27 patients (10 stage II, 17 stage III) with a median age of 3(1-21) years were included. Median follow-up was 87.5(15.3-247.1) months. Among 15 patients in group A, 3 were ineligible for the monotherapy protocol and received local treatment. The remaining 12 patients [9 complete (CR) and 3 incomplete response (IR) to IC] were treated exclusively with chemotherapy, of whom 9 were alive free of relapse at last follow-up. Two patients with IR to IC had disease relapse: one had pulmonary relapse at 8.2 months and one had local relapse at 35 months. The 5-year OS and EFS of group A were 86.7% and 80%, respectively. Analyzing survival according to IC response, CR to IC was achieved in 10 patients (9 group A and one group B) and was associated with significantly better OS and EFS than IR(p = 0.026 and 0.004, respectively) (Summary figure). All patients with CR to IC were alive free of relapse at last follow-up. DISCUSSION: Treatment of RMS is traditionally multimodal. Local treatment of B/P RMS is associated with significant patient morbidity. In this study, CR to IC predicted better OS and EFS. Patients who achieved CR (radiological and pathological) to IC remained alive free of relapse irrespective of local treatment. CONCLUSIONS: Our results suggest that patients with non-metastatic non-alveolar B/P RMS who achieve CR to IC can be treated with combination chemotherapy as a monotherapy and spared the morbidity of local treatment with durable survival outcomes. Prospective validation in a larger patient cohort is needed to support our hypothesis.

Ileal Neobladder With A Continent Cutaneous Catheterizable Channel Using the Extramural Serous Lined (Mansoura) Technique in A Bladder Exstrophy Patient.

INTRODUCTION AND OBJECTIVE: Continent cutaneous diversion is an appealing option when orthotopic diversion is not feasible and continence preservation is desired. We describe the surgical technique of creating an ileal w-neobladder with a continent catheterizable channel using the extramural serous lined (Mansoura) principle in a bladder exstrophy patient. MATERIALS AND METHODS: A 9-year-old boy born with classic bladder exstrophy, underwent cystectomy and a sigma rectosigmoid pouch after multiple unsuccessful attempts of exstrophy reconstruction. Colonic diversion was complicated by refractory metabolic acidosis, ureteral obstruction and recurrent pyelonephritis. Ileal neobladder with a continent stoma was recommended after family counseling. RESULTS: A 50-cm segment of the terminal ileum is isolated. The proximal 7 cm of the isolated segment are partly tailored and used as an afferent limb. The appendix is used for creating the catheterizable channel. The remainder of the ileal segment is fashioned in a w-configuration. Extramural serous-lined tunnels are created between the lateral limbs of the pouch. The appendix and afferent ileal segment are embedded in the serous lined tunnels to achieve continence and antireflux mechanisms, respectively. The ureters are, thereafter, anastomosed to the afferent ileal segment in an end-to-side fashion. Umbilicoplasty was done using the VQZ-plasty technique. CONCLUSION: Conversion to an ileal neobladder is an option in patients who suffer the complications of colonic diversion. The extramural serous-lined technique is a versatile technique that can be used for creating antireflux and continence mechanisms.

Are Prophylactic Measures Necessary to Prevent Recurrence of Penile Keloids?

Keloid scars result from excess collagen deposition in the dermis or subcutaneous tissue in response to surgery or tissue trauma. The penis is a rare site for keloid formation, even in predisposed individuals. Only few cases of penile keloids have been reported so far. In this report, we present penile keloids complicating neonatal circumcision in twin brothers. Risk of recurrence in previous reports and measures to guard against its occurrence are also discussed.

Effect of Early Oxybutynin Treatment on Posterior Urethral Valve Outcomes in Infants: A Randomized Controlled Trial.

PURPOSE: We studied the effect of oxybutynin on bladder and upper urinary tract outcomes in infants following posterior urethral valve ablation. MATERIALS AND METHODS: Patients younger than 12 months old who had undergone primary endoscopic valve ablation for posterior urethral valves were screened for eligibility. Patients who had undergone urinary diversion or had other conditions that could affect lower urinary tract function were excluded. Study patients were randomized to either oxybutynin (0.2 mg/kg 3 times daily) until toilet training or active observation. The study end points were serum creatinine, estimated glomerular filtration rate, hydronephrosis improvement, vesicoureteral reflux resolution, febrile urinary tract infection and toilet training. RESULTS: A total of 49 infants (24 receiving oxybutynin and 25 undergoing observation) were enrolled between December 2013 and September 2015 and completed at least 1 year of followup. Oxybutynin was discontinued before toilet training in 5 patients due to facial flushing in 2, bladder and upper tract dilatation in 2, and cognitive changes in 1. After a median followup of 44.2 months (range 12 to 57.6) median serum creatinine and estimated glomerular filtration rate were not significantly different between the groups (p=0.823 and p=0.722, respectively). Renal units in the oxybutynin group had a greater likelihood of hydronephrosis improvement (61.9% vs 34.8%, p=0.011) and resolution of vesicoureteral reflux (62.5% vs 25%, p=0.023). Febrile urinary tract infection (29.2% vs 40%, p=0.404), completion of toilet training (70.8% vs 76%, p=0.748) and age at toilet training (p=0.247) did not differ significantly between the oxybutynin and observation groups. CONCLUSIONS: Oxybutynin enhances hydronephrosis improvement and vesicoureteral reflux resolution following primary endoscopic valve ablation in infants but periodic monitoring is warranted.

Complete Primary Repair of Bladder Exstrophy: Critical Analysis of the Long-term Outcome.

OBJECTIVE: To demonstrate the long-term outcome of a contemporary series of 64 children who underwent complete primary repair of bladder exstrophy (CPRE) in a single tertiary referral center. MATERIALS AND METHODS: Between 1998 and 2012, 64 children, 47 boys and 17 girls, were identified. Only 60 of the 64 cases were available for follow-up. The follow-up was done by renal bladder ultrasound and serum creatinine every 3 months and voiding cystourethrogram from 6 to 12 months postoperatively. Continence was defined as dryness ≥3 hours. RESULTS: Median (range) follow-up is 14 years (from 5 to 19 years). Voided continence was achieved in 14 children (23%) after CPRE only. Additionally, 6 children were continent after bladder neck reconstruction (BNR) and 2 after bladder neck injection (BNI), raising the percentage of voided continence to 36%. The remaining 38 (64%) patients were using clean intermittent catheterization. All cases were continent at last assessment. The results of BNR or BNI were better in de novo than in redo cases (P <.05). The percentage of cases that needed augmentation ileocystoplasty in combination with multiple bladder neck procedures was lower in both female and de novo cases (P <.05). CONCLUSION: The percentage of children with classic bladder exstrophy who underwent CPRE who will achieve continence with volitional voiding via the urethra is 36%. The continence results after BNR and BNI are better in de novo cases than in redo ones. Continence in female and de novo cases is more likely to be achieved with lower number of continence procedures.

Cloacal Duplication: Single-center Experience in the Management of a Rare Anomaly.

Cloacal duplication is an exceedingly rare group of anomalies with a limited number of cases reported so far. The anomaly may be confined to partial bladder duplication or it may involve complete duplication of the urogenital tract, hindgut, spine, lower limbs, and vascular structures. Every case is unique and ought to be approached individually. By means of imaging studies and endoscopy, anatomic details should be carefully defined before endorsing surgical correction. A satisfactory outcome can be achieved in the majority of cases. In this report, we describe 3 girls with cloacal duplication, and review pertinent imaging and surgical management.

Perineal Hernia Is an Unusual Complication Post Perineal Bladder Neck Closure: A Case Report.

Bladder neck closure (BNC) is the ultimate bladder neck reconstruction. If reconstruction fails, closure must be considered as it gives the highest continence rate. The vast majority of BNCs are performed through an abdominal approach (either transvesical or extravesical approach), but perineal approach remains an option for BNC with considerable success rate. Perineal hernia, which is defined as protrusion of abdominal contents through the perineal defect, is a very rare complication after urologic procedures. We report a case of perineal hernia post perineal BNC.

Retropubic bulbourethral sling in incontinence post-exstrophy repair: 2-year minimal follow up of a salvage procedure.

AIMS: Post-exstrophy incontinence is a challenge because continence is difficult to achieve and more difficult to maintain. Feasibility and outcomes of a bulbourethral sling to treat post-exstrophy incontinence is shown in this report. METHODS: A retropubic bulbourethral sling was applied to male patients with incontinence post-exstrophy-epispadius repair. The study included children with total (continuous) incontinence who underwent multiple previous anti-incontinence procedures, ranging from bladder neck injection to bladder neck reconstruction. Preoperative assessment includes urinalysis, renal US, VCUG, 1-hr pad test and urodynamics. The bulbourethral sling applied is made of polypropylene and is suspended by 4 pairs of nylon sutures, to support the bulbar urethra within its covering muscles with the sutures tied on the rectus muscles. Continence was evaluated as well as adverse events. RESULTS: Seventeen children, (median age 8.7 years) completed 24-month of follow up. All had CPRE. Five children (29.27%) were dry. Four micturated through the urethra and one by catheterizing his cutaneous stoma every 3-4 hr. In none, PVR exceeded 10% of expected capacity. Four children underwent re-tightening 1-4 weeks after removal of urethral catheter. Perineal wound dehiscence occurred in one, perineal/suprapubic pain in seven and epididymo-orchitis in one child. CONCLUSION: The current technique is promising for difficult cases of incontinence after CPRE. It is safe, as no serious adverse events occurred during follow up period. It is economic and re-tightening is easy to perform. Neurourol. Urodynam. 35:497-502, 2016. © 2015 Wiley Periodicals, Inc.

Bricker Conduit for Pediatric Urinary Diversion--Should we Still Offer It?

PURPOSE: We sought to evaluate long-term outcomes of the Bricker conduit urinary diversion in children. MATERIALS AND METHODS: We retrospectively reviewed the database of a single tertiary center for children who had undergone ileal conduit between 1981 and 2011. Patients followed for less than 1 year were excluded. Patient files were reviewed for demographics, diversion indication, preoperative imaging, surgical details, hospital readmissions and followup data. Renal function at baseline and last followup was assessed by estimated glomerular filtration rate, calculated using the modified Schwartz or MDRD (Modified Diet in Renal Disease) formula. Growth charts elucidated patient growth patterns, while an internally designed quality of life questionnaire demonstrated patient and family satisfaction with the procedure. RESULTS: We evaluated 29 children who underwent Bricker conduit at a median age of 10 years (range 2 to 18) and were followed for a median of 91 months (16 to 389). Neuropathic bladder was the underlying diagnosis in 72.4% of cases. Hydronephrosis improved or remained stable in 39 of 55 studied renal units (70.9%). Although no statistically significant difference was observed between mean ± SD baseline (64.5 ± 46 ml/minute/1.73 m(2)) and last followup estimated glomerular filtration rate (54.1 ± 44.9 ml/minute/1.73 m(2)), chronic kidney disease stage had worsened in 13 patients (44.8%), end-stage kidney disease had developed in 11 patients and 9 patients had died. Six patients underwent undiversion after stabilization of renal function. Linear growth was negatively affected in 12 patients (41.4%), and 85% reported poor quality of life. A total of 19 hospital readmissions were required in 14 patients to treat diversion related complications. CONCLUSIONS: The Bricker conduit does not seem to halt renal deterioration in children. Negative impact on growth and quality of life, and the anticipated rate of complications are significant limitations of the procedure in the pediatric population.

Can computerized tomography accurately stage childhood renal tumors?

PURPOSE: Staging of childhood renal tumors is crucial for treatment planning and outcome prediction. We sought to identify whether computerized tomography could accurately predict the local stage of childhood renal tumors. MATERIALS AND METHODS: We retrospectively reviewed our database for patients diagnosed with childhood renal tumors and treated surgically between 1990 and 2013. Inability to retrieve preoperative computerized tomography, intraoperative tumor spillage and nonWilms childhood renal tumors were exclusion criteria. Local computerized tomography stage was assigned by a single experienced pediatric radiologist blinded to the pathological stage, using a consensus similar to the Children's Oncology Group Wilms tumor staging system. Tumors were stratified into up-front surgery and preoperative chemotherapy groups. The radiological stage of each tumor was compared to the pathological stage. RESULTS: A total of 189 tumors in 179 patients met inclusion criteria. Computerized tomography staging matched pathological staging in 68% of up-front surgery (70 of 103), 31.8% of pre-chemotherapy (21 of 66) and 48.8% of post-chemotherapy scans (42 of 86). Computerized tomography over staged 21.4%, 65.2% and 46.5% of tumors in the up-front surgery, pre-chemotherapy and post-chemotherapy scans, respectively, and under staged 10.7%, 3% and 4.7%. Computerized tomography staging was more accurate in tumors managed by up-front surgery (p <0.001) and those without extracapsular extension (p <0.001). CONCLUSIONS: The validity of computerized tomography staging of childhood renal tumors remains doubtful. This staging is more accurate for tumors treated with up-front surgery and those without extracapsular extension. Preoperative computerized tomography can help to exclude capsular breach. Treatment strategy should be based on surgical and pathological staging to avoid the hazards of inaccurate staging.

Urethrorectal fistula repair in children: urologic perspective.

OBJECTIVES: We report our experience in the management of urethrorectal fistulae in children with emphasis on the complexity of this rare disease. PATIENTS AND METHODS: A retrospective review was performed in cases that underwent repair of urethrorectal fistula at our center between 1997 and 2007. Records were reviewed for age, history, presentation, radiologic data, operative data, and condition at last follow-up. RESULTS: Five children were managed for urethrorectal fistula. Their mean age was 6.8 years (range, 2 months-12 years). One case had congenital urethrorectal fistula and four had acquired fistulas including two after abdomino-perineal pull through for imperforate anus, one case post perineal urethroplasty, and another post repair of posttraumatic rectal tear. All patients presented with history of passing urine both through the rectum and the urethral meatus. We have three urinary diverted cases: one case in whom urinary and fecal diversions were performed and the remaining case was operated without diversion. Perineal approach was adopted in four procedures and abdomino-perineal approach in one. One patient required optical internal urethrotomy for anastomotic stricture at 6 months follow-up. CONCLUSIONS: Urethrorectal fistula is a rare complication whether congenital or iatrogenic. Perineal repair is challenging, necessities meticulous dissection, adequate vascularity of the edges, and interposition of vascularized flaps and is potentially successful.

Factors affecting outcome of tubularized incised plate (TIP) urethroplasty: single-center experience with 500 cases.

PURPOSE: To review our experience of tubularized incised plate (TIP) urethroplasty in children with hypospadias defects. METHODS: Of 500 children (mean age 6 years) who received a TIP urethroplasty, 439 (87.8%) had primary hypospadias and 61 had one failed previous repair. The hypospadias defects were coronal in 110 (22%), distal penile in 261 (52.2%), midpenile in 78 (15.6%) and proximal in 51 (10.2%). Chordee was present in 98 (19.6%) patients. Presence of complications requiring re-operation and overall general appearance was recorded. RESULTS: The mean (SD, range) follow-up was 34 (18, 7-77) months. Overall success rate was 81.4%. Re-operation was required in 93 patients (18.6%); for urethrocutaneous fistula in 47 (9.4%), complete disruption of the repair in 32 (6.4%) and meatal stenosis in 14 (2.8%). In univariate analysis, complications were significantly higher in stented repairs, posterior hypospadias, those with no neourethral coverage (spongioplasty), and repairs early in the study. The last three factors were the only significant independent risk factors in multivariate analysis. CONCLUSIONS: TIP is a reliable method for treating both distal and proximal hypospadias and is suitable for both primary and re-operative cases with a low rate of complications. A significantly better outcome is achieved with distal hypospadias, covering the neourethra with the mobilized corpus spongiosum (spongioplasty) or a flap, and experience. Stenting of the repair, patient age, or previous failed repair has no statistically significant impact on outcome.

Surgical management of failed pyeloplasty in children: single-center experience.

PURPOSE: To evaluate the outcome of secondary surgical procedures for the management of failed pyeloplasty in children. MATERIALS AND METHODS: Between 1996 and 2007, 590 cases of primary ureteropelvic junction (UPJ) obstruction underwent open dismembered pyeloplasty at our center. Of these patients, 18 (3%) with recurrent UPJ obstruction (14 males, 4 females; age range: 2-15 years) have undergone management of failed pyeloplasty. Secondary intervention was by open operative procedure in all cases. Clinical and radiological outcomes were assessed. Success was defined as both symptomatic relief and radiographic resolution of obstruction at last follow up. RESULTS: Follow up ranged from 8 to 41 months (mean 28). The overall salvage rate was 89%. Secondary reoperative surgery was successful in 16 patients: dismembered pyeloplasty in 14 patients (78%) and ureterocalyceal anastomosis in 2 (11%). Nephrectomy was necessitated in 2 patients (11%). No perioperative complications were encountered. All patients showed stability of renal function on radiological follow up without evidence of obstruction and with no further symptoms. CONCLUSION: Persistent UPJ obstruction after pyeloplasty is an uncommon complication. Secondary procedures have a very high success rate with excellent functional results. Nephrectomy is indicated in rare cases of severely deteriorated renal function.

Prospective study of the long-term effects of shock wave lithotripsy on renal function and blood pressure.

PURPOSE: We clarified the long-term effects of extracorporeal shock wave lithotripsy on renal function and blood pressure, and its relation to body mass index and type of lithotriptor. MATERIALS AND METHODS: A total of 100 patients with a single radiopaque renal stone 2 cm or less in length were followed for 18 to 57 months (mean 43.6 +/- 13.8) after being stone-free by extracorporeal shock wave lithotripsy monotherapy using 2 lithotriptors. Mean patient age was 47.9 +/- 9.1 years (range 23 to 66). Nuclear scintigraphy using (99m)technetium-mercaptoacetyltriglycine was done for all patients before extracorporeal shock wave lithotripsy, as well as at the last followup visit to estimate glomerular filtration rate, clearance and split renal function. Blood pressure measurements were recorded at admission and at each followup visit. RESULTS: Before extracorporeal shock wave lithotripsy treated side mean (99m)technetium-mercaptoacetyltriglycine clearance, glomerular filtration rate and split function were 146.22 +/- 59.48, 52.66 +/- 13.69 and 49.7 +/- 7.31, respectively. At the last followup visit they were 145.1 +/- 58.82 (p = 0.842), 54.85 +/- 15.75 (p = 0.114) and 49.96 +/- 8.68 (p = 0.577), respectively. Of 100 patients 18 were hypertensive before extracorporeal shock wave lithotripsy and 21 were hypertensive at the last followup visit. Mean diastolic blood pressure before extracorporeal shock wave lithotripsy was 80.2 +/- 6.2 vs 80.6 +/- 7.8 mm Hg (p = 0.674) at the end of followup. Mean systolic blood pressure before extracorporeal shock wave lithotripsy was 121.2 +/- 9 vs 121.55 +/- 10.2 mm Hg by the end of the study (p = 0.748). There were no statistically significant differences among body mass index groups or lithotriptor groups. CONCLUSIONS: Extracorporeal shock wave lithotripsy is a safe procedure and has no significant long-term effects on renal function or blood pressure regardless of the type of machine used or body mass index.

Evaluation of a synchronous twin-pulse technique for shock wave lithotripsy: a prospective randomized study of effectiveness and safety in comparison to standard single-pulse technique.

OBJECTIVE: To asses the efficacy and safety of bidirectional synchronous twin-pulse extracorporeal shock wave lithotripsy (ESWL) compared with standard ESWL. PATIENTS AND METHODS: Between March 2003 and December 2006, 240 patients with a radio-opaque single renal stone of 10 mm the rate for the failure of disintegration was 13.3% for the DLS vs 1.4% for the TH (P = 0.009). For stones of 10 mm it was 78.1% and 66.7%, respectively (P = 0.14). The median (range) number of sessions in both groups was 2 (1-5). After ESWL urinary NAG levels were increased significantly in both groups; in the TH group it declined below the level before ESWL after 2 days, while in the DLS group it remained high after 7 days. In the DLS group four patients developed subcapsular or parenchymal haematoma after ESWL, vs none in the TH group. There was loss of corticomedullary differentiation after ESWL in three patients in the DLS group and only one in the TH group. In the DLS group there was a statistically significantly decrease in bilateral renal perfusion after ESWL, but no changes in the TH group. CONCLUSIONS: Synchronous twin-pulse ESWL has clinical advantages over standard ESWL in terms of safety and efficacy.