Incidence of pathologic postobstructive diuresis after resolution of ureteropelvic junction obstruction with a normal contralateral kidney.

INTRODUCTION: Postobstructive diuresis (POD) after unilateral pyeloplasty or percutaneous nephrostomy (PCN) tube insertion for ureteropelvic junction obstruction (UPJO) in patients with a normal contralateral kidney is not well described. OBJECTIVE: The objective of this study was to determine the incidence and characteristics of POD after relief of unilateral UPJO in patients with a normal contralateral kidney. STUDY DESIGN: Children who underwent a unilateral pyeloplasty or PCN for UPJO from 2010 to 2017 with a normal contralateral kidney were retrospectively reviewed. Postobstructive diuresis was defined as urine output (UO) of >300% of expected UO. Patients with a solitary kidney or those who underwent bilateral pyeloplasty or bilateral PCN tube placement were excluded. RESULTS: Out of 396 children meeting inclusion criteria, seven (1.8%) developed POD (4 after pyeloplasty and 3 after PCN tube placement). Median age at intervention was 1.7 years (range 11 days-18 years); median weight was 11.4 kg (range 3.7-54.2 kg). Postobstructive diuresis was more likely to occur in patients with grade 4 hydronephrosis (3.0%) and larger kidneys and if a PCN tube was placed before pyeloplasty. There was no significant difference in age, gender, kidney laterality, or function between those who developed POD and those who did not. Postobstructive diuresis was managed with additional intravenous fluids and electrolyte monitoring. Median initial postprocedure UO was 5.9 mg/kg/hr (range 3.2-10.0 mg/kg/hr). In five children who underwent PCN in whom UO could be differentiated between kidneys, median initial postprocedure UO was 6.1 mg/kg/hr (range 2.5-9.1 mg/kg/hr) from the affected side and 0.8 mg/kg/hr (range 0.4-0.9 mg/kg/hr) from the unaffected side. The median length of time to resolution of POD was 3 days (range 2-4 days). One patient developed significant acidosis and lethargy that improved with intravenous fluid management. Mild hyponatremia developed in two, hypokalemia in one, hypophosphatemia in one, acidosis in one, and hypoglycemia in 1 patient. DISCUSSION: A low but clinically significant risk of POD occurring after relief of unilateral UPJO in children with a normal contralateral kidney is described. Limitations include retrospective analysis and small sample size due to the rarity of the condition. CONCLUSION: Postobstructive diuresis after decompression of UPJO in patients with a normal contralateral kidney is a rare event (1.8%). However, POD does occur, and patients should be carefully monitored after these procedures given the potential for significant dehydration and electrolyte disturbances.

What about my daughter's future? Parental concerns when considering female genital restoration surgery in girls with congenital adrenal hyperplasia.

PURPOSE: The parental decision-making process regarding female genital restoration surgery (FGRS) for girls with congenital adrenal hyperplasia (CAH) is controversial and poorly understood. The aim of the study aim was to evaluate parental concerns related to their child's future and parental plans about disclosure prior to FGRS. MATERIALS AND METHODS: The authors performed an online survey of consecutive parents presenting at a tertiary referral center for consultation regarding FGRS for their daughter with CAH before 3 years of age (2016-2018). Twenty issues initially identified by three families and six clinicians were rated on a 6-point Likert scale of importance ('not at all' to 'extremely'). RESULTS: Sixteen consecutive families participated (Prader 3/4/5: 43.8%/43.8%/12.5%). Fourteen girls (87.5%) subsequently underwent FGRS at a median age of 8 months. Most issues (19/20, 95.0%) were ranked 'quite a bit' to 'extremely' important (Table). Top issues were not surgical: Normal physical/mental development, adrenal crisis and side-effects of medications. Surgery-related and self-image concerns followed in importance. Least prioritized issues were multiple genital exams ('quite a bit' important) and the child not being involved in the decision to proceed with FGRS ('somewhat' important). On average, no issues were considered 'not at all' or 'a little' important. Disclosure of FGRS to their daughter was the 15th prioritized issues. Almost all families (93.8%, 1 unsure) planned to disclose the surgery to their daughter, although many were unsure when and how to do it (33.3% and 37.5%, respectively). COMMENT: Initial efforts to understand the complex process of parental decision-making regarding FGRS in the context of CAH, a complex, multifactorial disease, are presented. Parents of infant girls with CAH simultaneously weigh multiple life-threatening concerns with a decision about FGRS. While issues of genital ambiguity and surgery are important, they are not overriding concerns for parents of girls with CAH. Parents report significant uncertainty about appropriate timing and approach to disclosing FGRS to their daughters. Unfortunately, best practice guidelines for this process are lacking. The findings are not based on actual history of disclosure but on parents' anticipated behavior. Further data are need from parents, children, and women with CAH about successful disclosure. Being a single-center series, these data may not correspond to the wider CAH community. CONCLUSIONS: Parental decision-making regarding FGRS is multifactorial. Even when considering FGRS, parents' largest concerns remain focused on the life-threatening and developmental effects of CAH and side-effects of its medical treatment. The disclosure process deserves further attention.

Do anterior catheterizable urinary channels have fewer complications than posterior channels? An international cohort study.

OBJECTIVE: Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are durable catheterizable channels. While subfascial revision rates vary by channel type, a channel implanted in the anterior (vs posterior) aspect of the bladder may have a lower subfascial revision risk, due to decreased channel mobility and better fascial fixation. The present study aimed to compare long-term durability of anteriorly compared to posteriorly implanted APV and Monti channels in a large international cohort. MATERIALS AND METHODS: A retrospective cohort study was conducted on patients aged ≤21 years and who underwent APV or Monti surgery with an open technique at three high-volume centers (1990-2015). The following were noted: patient demographics, stomal and subfascial revisions, stomal location, channel placement (anterior/posterior), and channel type - APV, spiral Monti to umbilicus (SMU), other Monti channels. Survival analysis and Cox proportional hazards regression were used to separately examine the three channel groups. RESULTS: Of the 675 patients who met inclusion criteria, 387 had an APV (71.3% anterior), 53 had an SMU (13.2% anterior) and 235 had other Monti channels (42.1% anterior). Median age at surgery was 8.8 years for APV (median follow-up: 5.5 years), 9.2 years for other Monti (follow-up: 6.6 years) and 7.9 years for SMU (follow-up: 9.0 years). Patients originated from the USA (67.9%), Argentina (26.4%) and Chile (5.8%). Overall, 76 stomal and 77 subfascial revisions occurred. Risk of stomal revision was 9.3-12.0% at 5 years of follow-up, and was similar between channel types or location (P = 0.57). Risk of subfascial revision at 5 years was 7.4% for APV, 12.7% for all other Monti channels and 25.9% for SMU (P = 0.001). On survival analysis, stomal and subfascial revision rates were similar between anterior and posterior channels for APV (P ≥ 0.16), other Monti channels (P ≥ 0.62) and SMU (P ≥ 0.43) (Summary Fig.). On multivariate regression, channel configuration was not associated with stomal or subfascial revision for APV (P ≥ 0.18) or other Monti channels (P ≥ 0.64). Sex, age, diagnosis, country and stomal location were not associated with revision risk (P ≥ 0.06). DISCUSSION: Contrary to the hypothesis, subfascial revision rates were no different between anterior and posterior channels. Given that many reported outcomes related to genitourinary reconstruction occur rarely and require prolonged follow-up, collaborative research in this area should be encouraged. CONCLUSIONS: The study demonstrated durable long-term results with the APV and Monti techniques in an international cohort. Risks of stomal and subfascial complications were not significantly different between anteriorly and posteriorly implanted channels. As previously reported, Monti channels, particularly SMU, were more prone to undergoing subfascial revisions.

A case of base rate bias, or are adolescents at a higher risk of developing complications after catheterizable urinary channel surgery?

OBJECTIVE: Adolescents are considered to be at high risk of developing complications after lower genitourinary tract reconstruction. This perception may be due to base rate bias, where clinicians favor specific information (adolescents with complications), while ignoring more general information (number of total adolescents being followed). The goal of this study was to assess whether age was a true risk factor for subfascial and stomal revisions after continent catheterizable urinary (CCU) channel procedures. MATERIALS AND METHODS: Consecutive patients aged <21 years and who underwent appendicovesicostomy and Monti surgery at the present institution were retrospectively reviewed; demographic and surgical data were collected. Time to subfascial or stomal revision was stratified by age at initial surgery (child: <8, preteen: 8-12, adolescent: 13-17, adult: ≥18 years old) and analyzed with Cox proportional-hazards regression. Secondary analyses included: different age categories at initial surgery (<8, 8-11, 12-15, 16-19, ≥20 years), analyzing age as a continuous and a time-varying covariate. RESULTS: Of the 510 patients with CCU channels (median age at surgery: 7.9 years), 63 (12.4%) had subfascial and 53 (10.4%) had stomal revision (median follow-up: 6.8 years). Median age at subfascial and stomal revision was 11.3 and 10.3 years, respectively. Preteens contributed 33.0% and adolescents contributed 29.3% of the total follow-up time (3263.9 person-years). Over 80% of revisions occurred within 5 years of surgery, regardless of age at initial surgery (P ≥ 0.57) (Summary table). On multivariate analysis, age at initial surgery was not associated with undergoing subfascial (P ≥ 0.62) or stomal revisions (P ≥ 0.69). Montis were 2.1 times more likely than appendicovesicostomies to undergo a subfascial revision (P = 0.03). No other variables were associated with the risk of subfascial or stomal revision (P ≥ 0.11). Secondary analyses provided similar results. DISCUSSION: Since the median age at surgery was 8 years old and most complications occurred within the first 5 years of follow-up, it is not surprising that most revisions occurred in 8-13 year olds. Pediatric urologists appear to base their impression of adolescents being "high risk" on specific information (adolescents having complications), while subconsciously ignoring more general information (adolescents represent a large proportion of patients in follow-up). This study had several limitations: channel complications treated non-surgically (e.g. prolonged catheterization) were not included. The findings may not be generalizable to other genitourinary reconstructive procedures or clinical settings. CONCLUSIONS: While complications were twice as high in Monti channels than appendicovesicostomies, no single age group was at increased risk. The impression that adolescents are a high-risk group appears to represent a base rate bias.

Ambulatory patients with spina bifida are 50% more likely to be fecally continent than non-ambulatory patients, particularly after a MACE procedure.

INTRODUCTION: While fecal incontinence (FI) affects many patients with spina bifida (SB), it is unclear if it is associated with ambulatory status. OBJECTIVE: To determine if ambulatory status is associated with FI, and a potential confounding variable, in patients with and without a Malone antegrade continence enema (MACE). STUDY DESIGN: This study retrospectively reviewed of patients aged ≥8 years with SB who were enrolled in an international quality of life study at outpatient visits (January 2013 to September 2015). Patients reported FI over the last 4 weeks (strict criteria: any FI/accidents vs no FI). Patients unable to self-report FI due to developmental delay were excluded. Those who were ambulating outdoors with/without braces/crutches were considered community ambulators. Non-parametric tests and logistic regression were used for analysis. RESULTS: A total of 115 patients with a MACE and 57 without a MACE were similar in gender (P = 0.99), ventriculoperitoneal status (P = 0.15) and age (16.0 vs 15.4 years, P = 0.11). Median ages at MACE procedure and follow-up were 7.0 and 8.2 years, respectively, and all used the MACE ≥3x/week. They were less likely to be ambulators (54.8 vs 71.9%, P = 0.03). In patients with a MACE, 64 (55.7%) had total fecal continence, compared with 29 (50.9%) without a MACE (P = 0.62). In the MACE group, ambulators were more likely to be continent compared with non-ambulatory patients (65.1 vs 44.2%, P = 0.04) (Table). Although not statistically significant, a similar difference was observed in the non-MACE group (56.1 vs 37.5%, P = 0.25). In the MACE group, continent and incontinent patients, regardless of ambulatory status, had similar rates of MACE use, additive use and time for MACE completion (P ≥ 0.43). MACE ambulators were more likely to be continent than MACE non-ambulators on multivariate analysis (OR 3.26, P = 0.01). DISCUSSION: This study reported higher than typical FI rates since: (1) it used a stringent definition of total fecal continence; (2) patients without FI were perhaps less likely to participate; and (3) it relied on patient-reported rather than clinician-reported outcomes. This cross-sectional study should not be interpreted as "MACE procedure is ineffective;" this would require a longitudinal study. The present findings may not apply to young children or those with significant developmental delay (patients excluded from the study). CONCLUSIONS: Ambulatory patients with SB are 50% more likely to have total fecal continence on long-term follow-up, particularly after a MACE procedure. Ambulatory status is a significant confounder of FI and should be considered in future analyses.

Long-term follow-up of composite bladder augmentation incorporating stomach in a multi-institutional cohort of patients with cloacal exstrophy.

INTRODUCTION: Composite bladder augmentation, incorporating gastric and bowel segments, has the theoretical advantage of metabolic neutrality while potentially avoiding the morbidities of gastrocystoplasty, such as hematuria-dysuria syndrome. The most common indication for this operation is a paucity of bowel, such as in cloacal exstrophy. Despite several early descriptive studies of this technique, there are no reports, to date, of long-term follow-up in this population. OBJECTIVE: To describe the outcomes of composite bladder augmentation utilizing stomach in a cohort of cloacal exstrophy patients. MATERIALS AND METHODS: A retrospective review of cloacal exstrophy patients who underwent composite bladder augmentation from 1984 to 2006 at two institutions was performed. The incidence of mortality and morbidities related to augmentation was evaluated. RESULTS: Eleven patients with cloacal exstrophy underwent composite bladder augmentation. Median age at initial augmentation was 6.4 years (interquartile range (IQR) 4.4-9.1). Median follow-up was 13.2 years (IQR 11.2-24.6). The Summary table describes the types of composite bladder augmentations. Of the three patients with pre-operative metabolic acidosis, two improved with composite bladder augmentation and one developed metabolic alkalosis. Three developed hematuria-dysuria syndrome: one improved with staged ileocystoplasty, and two had persistent symptoms successfully treated with H2 receptor blockers. Two of 11 developed symptomatic bladder stones. There were no reported bladder perforations, bladder malignancies, conversions to incontinent urinary diversions, or deaths. CONCLUSION: With long-term follow-up, very few patients developed metabolic acidosis/alkalosis after composite bladder augmentation. The composite bladder augmentation will continue to be used in patients with cloacal exstrophy, in order to minimize the impact on the pre-existing short gut in these patients.

Long-term outcomes of the Kropp and Salle urethral lengthening bladder neck reconstruction procedures.

INTRODUCTION: The Kropp and Salle procedures have shown good short-term outcomes for managing neuropathic urinary incontinence. However, few studies have assessed their long-term results. OBJECTIVE: This study aimed to evaluate the long-term outcomes of Kropp and Salle procedures, including: dryness, secondary interventions for incontinence or complications, upper urinary tract changes, and use of urethral catheterizations. STUDY DESIGN: Consecutive patients undergoing Kropp and Salle procedures at the present institution (1983-2012) were retrospectively reviewed. Patients with <1-year follow-up or prior bladder neck (BN) continence procedures were excluded. Data were collected on postoperative dryness per urethra at 4-hourly and 3-hourly intervals, secondary interventions, lab tests, and imaging. Non-parametric tests were used for statistical analysis. RESULTS: Thirty-eight patients had Kropp (K: 30 boys) and 12 had Salle procedures (S: 8 boys). Patients underwent surgery at similar median ages (K: 7.4 vs S: 8.7 years old, P = 0.51) and had similar median follow-up (6.9 vs 10.3 years, P = 0.10). Most patients had myelomeningocele, a prior/concomitant bladder augmentation (K: 73.7%, S: 58.3%) and catheterizable channel (K: 81.6%, S: 50.0%). Differences in all outcomes between Kropp and Salle procedures were statistically non-significant. The majority of patients did not have additional BN procedures for dryness (K: 84.2%, S: 66.7%). Of this group, K: 81.3% and S: 75.0% were dry for ≥4 h, K: 93.8% and S: 87.5% were dry for ≥3 h between catheterizations (Table). Of the minority of patients who underwent additional BN procedures for dryness (K: 15.8%, S: 33.3%), most achieved dryness for ≥4 h (K: 66.7%, S: 100%) and ≥3 h (100% for both). Among patients without an initial catheterizable channel, 57.1% had one subsequently created after a Kropp procedure, and 33.3% after a Salle. Among patients without bladder augmentation, approximately half underwent delayed augmentation (K: 50.0%, S: 40.0%). Ultimately, most patients required a secondary intervention under anesthesia for incontinence or complications (K: 79.0%, S: 66.7%). Few patients developed worsening hydronephrosis, vesicoureteral reflux or renal function (K: 2.6%, S: 0.0%). At the end of follow-up, few patients catheterized per urethra (K: 10.5%, S: 33.3%). DISCUSSION: This was a retrospective study without urodynamic data. Originating from a tertiary center, the results may not apply to other clinical settings. CONCLUSIONS: At the present institution the Kropp and Salle procedures attained similar dryness in 75-81% at 4-hourly intervals and 88-94% at 3-hourly intervals without additional BN procedures. Few patients required subsequent BN procedures to achieve dryness. However, the overall need for secondary procedures during long-term follow-up was high for both procedures.

Is renal scintigraphy necessary after heminephrectomy in children?

INTRODUCTION: Heminephrectomy remains an excellent option for a poorly functioning moiety in a duplicated collecting system. A primary concern during heminephrectomy is the potential for a significant functional loss in the remaining ipsilateral moiety. As the gold standard for the assessment of differential renal function, renal scintigraphy is often used in the postoperative evaluation of children undergoing heminephrectomy. However, this imaging modality is costly, invasive, and associated with exposure to radiation. Doppler renal ultrasound (RUS) avoids these concerns and is able to evaluate for structural and functional abnormalities. OBJECTIVE: The present study sought to compare Doppler RUS to renal scintigraphy in determining the viability of the remaining ipsilateral moiety in children who underwent heminephrectomy for a poorly functioning moiety in a duplicated collecting system. MATERIALS AND METHODS: The institutional database of children who underwent open heminephrectomy for a poorly functioning moiety in a duplicated collecting system between 2006 and 2013 was reviewed. Only children who underwent both a postoperative Doppler RUS and renal scan were included. A blinded pediatric radiologist independently reviewed all Doppler RUS. Vascular flow on Doppler RUS was correlated with the preservation of renal function in the remaining ipsilateral moiety on renal scintigraphy. RESULTS: A total of 29 children were identified for inclusion. Demographic and operative data are provided in Table. The average pre-operative and postoperative differential renal function in the ipsilateral kidney was 41.6% and 38% on renal scintigraphy, respectively, for an average decrease of 3.6% (-18% to +12%). Doppler RUS demonstrated the presence of vascular flow to the remaining ipsilateral moieties of all children after heminephrectomy. Renal scintigraphy confirmed the viability of these moieties in all children. DISCUSSION: The first study comparing Doppler RUS to renal scintigraphy was performed to determine the viability of the remaining ipsilateral moiety after heminephrectomy. While no cases of complete functional loss were observed, an average decrease of 3.6% in the ipsilateral renal function favorably compared with other series of children undergoing open heminephrectomy. The limitations of the study included its retrospective design at a single institution. The interpretation of Doppler RUS by an individual pediatric radiologist may also have lead to interobserver variability and impacted the reproducibility of the study, while the absence of any cases of complete functional loss may have impacted its generalizability. CONCLUSIONS: Doppler RUS is an accurate imaging modality for determining the viability of the remaining ipsilateral moiety after heminephrectomy and may obviate the need for renal scintigraphy.

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 classification of solitary renal cysts in children.

INTRODUCTION: Solitary renal cysts are typically incidentally found in children who have undergone renal ultrasound (US). The main concern is a cystic tumor. There is no US-based grading system for children to guide management. OBJECTIVE: To evaluate a US-based, modified Bosniak grading system in order to differentiate between simple (grade I or II) and complex (grade II or IV) renal cysts and guide management in children. STUDY DESIGN: This was a retrospective (2003-2011) study of 212 children (114 females), age range one day to 17 years (mean 8.4 years), with solitary renal cysts diagnosed by US. Two radiologists, who were independent and blinded to clinical information, graded the cysts using the modified Bosniak classification system. In children with more than one year of follow-up US, the change (>10%) in cyst diameter was evaluated. Inter-observer variability (Kappa) was calculated. RESULTS: Radiologists one and two saw simple renal cysts in 96.2-96.6% (204-205/212) of the children. Ten children had complex renal cysts, as rated by either of the radiologists. There was good inter-observer agreement (kappa = 0.65) for simple versus complex cysts. In 20.2% (18/89) of the children, the cysts increased in size. A definitive diagnosis was obtained in 8.5% (18/212) of the children. A cystic tumor (multilocular cystic nephroma) was found in one child (Figure) with a complex cyst (graded III by both radiologists). DISCUSSION: The use of a modified Bosniak classification system to grade renal cysts was found to have good inter-observer variability (kappa = 0.65) in differentiating between simple and complex renal cysts. Using this classification, few (<4%) renal cysts were classified as complex. Cystic tumors are rare and the only cystic tumor (multilocular cystic nephroma) was classified as complex renal cysts by the two radiologists. Growth of simple, solitary renal cyst is common (20.2%) and, therefore, if not associated with other imaging findings, is not an indication for a cystic tumor. There were limitations inherent in the retrospective nature of the study and because only one child had a cystic tumor. CONCLUSION: The modified Bosniak classification system demonstrated good inter-observer agreement, and identified the single tumor as a complex cyst. The vast majority of solitary renal cysts in children are simple and if asymptomatic, they require no other imaging evaluation. Complex renal cysts are uncommon and should be evaluated with a pre-intravenous and postintravenous contrast CT scan to exclude a tumor.

Use of the mobilized sinus with total urogenital mobilization.

PURPOSE: The surgical treatment of urogenital sinus anomalies has undergone significant advances in recent years. Total urogenital mobilization, which mobilizes the urogenital sinus, vagina and urethra en bloc toward the perineum, represents one of these advances. MATERIALS AND METHODS: We have improved our results with total urogenital mobilization by incorporating the mobilized urogenital sinus tissue into the repair rather than discarding it, as described originally. We have found this a readily available, easily manipulated and well vascularized flap that is a significant aid to reconstruction. RESULTS: We present our 3 favored means of using the mobilized sinus tissue to create a mucosa lined vestibule, a posterior vaginal wall flap and an anterior vaginal wall flap. CONCLUSIONS: We believe that our techniques result in a further advancement in the cosmetic and surgical outcomes in these patients, and are beneficial in the reconstructive surgery armamentarium.

Total continence reconstruction: a comparison to staged reconstruction of neuropathic bowel and bladder.

PURPOSE: Surgical treatment for neuropathic bowel and bladder has become an essential tool in maximizing the quality of life in patients with myelomeningocele. We present our results comparing results in patients who underwent total continence reconstruction of the urinary and gastrointestinal tracts to patients who underwent a separate or single operation. MATERIALS AND METHODS: We performed a retrospective chart review of all patients with myelomeningocele at our institution who underwent reconstruction with a cutaneous catheterizable urinary channel or Malone antegrade continence enema. We compared outcomes with regard to surgical revisions of the channel between patients who underwent the construction of each simultaneously, that is total continence reconstruction, to outcomes in those with a single channel or who underwent reconstruction at 2 or more operations. RESULTS: Most of our patients underwent genitourinary and gastrointestinal reconstruction, and few desired surgical intervention for only a single system. We were unable to find any differences in the continence rate or stomal complications. However, patients who underwent staged reconstruction usually had significant secondary reasons for repeat surgery. CONCLUSIONS: Surgical success for urinary and fecal continence can be safely and effectively achieved through single or multiple procedures. However, because of shared pathophysiology, we believe that most patients benefit from intervention in the gastrointestinal and the genitourinary tract. Therefore, a major advantage of total continence reconstruction is avoidance of the morbidity of a second major surgical procedure.

What is the need for additional bladder surgery after bladder augmentation in childhood?

PURPOSE: Bladder augmentation has revolutionized the care of children with a neuropathic bladder but it remains a major surgical procedure. However, the need for subsequent bladder surgery has not been well defined in a large series with long-term followup. MATERIALS AND METHODS: We retrospectively reviewed the records of the first 500 bladder augmentations performed from 1978 to 2003 at our institution. Charts were reviewed for complications requiring additional surgery, including malignancy, bladder perforation, repeat augmentation, bowel obstruction and bladder calculi. Mean and median followup was 13.3 years. RESULTS: Complications occurred in 169 patients (34%) resulting in a total of 254 surgeries. The cumulative risk of further surgery at the bladder level was 0.04 operations per patient per year of augmentation. Three patients (0.6%) had transitional cell carcinoma, of whom all presented with metastatic disease and died. Bladder perforation occurred in 43 patients (8.6%) with a total of 53 events. Of the patients 16 (3.2%) required laparotomy for bowel obstruction and 47 (9.4%) required repeat augmentation. Bladder stones were treated in 75 patients (15%), who required a total of 125 surgeries. CONCLUSIONS: Bladder augmentation provides immeasurable improvements in quality of life but it requires lifelong dedication from the patient, family and health care providers. While the requirements for additional surgery are not trivial, 66% of our patients have not required any further surgery in the augmented bladder.

Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale.

PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. Although several reports of successful surgical treatment exist, controversy remains with respect to patient selection and efficacy. We present a large series with excellent clinical followup, neuroimaging and urodynamic characterization. MATERIALS AND METHODS: We present our experience with 36 patients at a single institution with preoperative clinical findings, neuroimaging and urodynamics available. Postoperative outcomes were assessed clinically and with urodynamics. We determined predictive parameters to improve patient selection. RESULTS: Approximately 0.04% of pediatric urology clinic visits resulted in neurosurgical referral for the potential of an occult tethered cord. They occurred after failure of a mean of 2 years of aggressive medical management. Daytime urinary incontinence was present in 83% of patients and 47% had encopresis. Preoperative urodynamics were markedly abnormal in all patients with mean bladder capacity 55% of expected capacity. Clinical improvement in urinary symptoms was seen in 72% of patients with resolution of incontinence in 42%. Bowel symptoms improved in 88% of cases, including resolution of encopresis in 53% within 3 months of surgery. Urodynamic improvements were demonstrated in 57% of cases. We were unable to determine preoperative factors that were more likely associated with surgical success. CONCLUSIONS: In a highly select population with severe urinary and fecal dysfunction sectioning a normal-appearing filum terminale can result in significant improvement. We were unable to identify factors that may increase the chance of surgical success.

Spontaneous bladder perforations: a report of 500 augmentations in children and analysis of risk.

PURPOSE: The spontaneous perforation of an augmented bladder is an uncommon but serious complication. To our knowledge our institution has the largest reported series of bladder augmentations. We examined our data to determine the incidence of spontaneous bladder perforation and to delineate associated risk factors. MATERIALS AND METHODS: We performed a retrospective chart review of 500 bladder augmentation procedures performed during the preceding 25 years with a minimum followup of 2 years. RESULTS: Spontaneous perforations occurred in 43 patients (8.6%), for a total of 54 events. The calculated risk was 0.0066 perforations per augmentation-year at risk. Approximately a third of the cases had perforated within 2 years of surgery, a third between 2 and 6 years postoperatively, and a third at more than 6 years after augmentation. Patients who underwent augmentation between 1997 and 2003 had a higher rate of perforation within 2 years of surgery than those operated on between 1978 and 1987. Increased risk of perforation was observed with the use of sigmoid colon and bladder neck surgery. A decreased risk was associated with the presence of a continent catheterizable channel. CONCLUSIONS: We believe that this large and comprehensive series gives valuable insight into this serious complication. The delineation of these potential risk factors serves as a guide for further discussion and investigation.

Partial urogenital mobilization: a limited proximal dissection.

INTRODUCTION: The treatment of urogenital sinus malformations is complex and controversial. Despite numerous and significant contemporary surgical advances, the dissection of the urogenital sinus remains technically challenging. METHODS: Based on total urogenital mobilization, we describe a technique whereby this dissection is limited to the pubourethral ligament. Our short-term results with partial urogenital mobilization (PUM) performed on 15 patients are retrospectively reviewed. RESULTS: There were no intraoperative complications and the short-term cosmetic results are excellent. No patients have developed voiding dysfunction or urinary tract complications. CONCLUSIONS: While total urogenital mobilization is a very effective procedure, we believe that the PUM approach limits potential morbidity in the reconstruction of these complex problems.

Mitrofanoff channel survival after pedicle ligation.

In patients who have undergone complex genitourinary reconstruction, additional abdominal surgery is often required. We report 2 cases in which the blood supply to an existing Mitrofanoff channel was divided. In both cases, the conduits appeared to remain well perfused, presumably based on collateral blood supply. Both conduits remained healthy and functioning at more than 1 year of follow-up.

TNF-alpha mediates obstruction-induced renal tubular cell apoptosis and proapoptotic signaling.

Obstruction of the upper urinary tract induces a progressive loss in renal mass through apoptotic renal cell death. Although TNF-alpha has been implicated in ischemia-reperfusion-induced apoptotic renal cell death, its role in obstructive renal cell apoptosis remains unknown. To study this, male Sprague-Dawley rats were subjected to left unilateral ureteral obstruction vs. sham operation. Twenty-four hours before surgery and every 84 h thereafter, rats received either vehicle or a pegylated form of soluble TNF receptor type 1 (PEG-sTNFR1). The kidneys were harvested 1, 3, or 7 days postoperatively, and tissue samples were subsequently analyzed for TNF-alpha (ELISA, RT-PCR), Fas ligand (RT-PCR), apoptosis (TUNEL, ELISA), and caspase 8 and 3 activity (Western blot). Renal obstruction induced increased tissue TNF-alpha and Fas ligand mRNA levels, TNF-alpha protein production, apoptotic renal tubular cell death, and elevated caspase 8 and 3 activity, whereas treatment with PEG-sTNFR1 significantly reduced obstruction-induced TNF-alpha production, renal tubular cell apoptosis, and caspase activity. PEG-sTNFR1 did not significantly alter Fas ligand expression. These results demonstrate that TNF-alpha mediates obstruction-induced renal tubular cell apoptosis and proapoptotic signaling and identify TNF-alpha neutralization as a potential therapeutic option for the amelioration of obstruction-induced renal injury.

Use of the silastic sheath in bladder neck reconstruction.

OBJECTIVE: The study compared two populations of patients undergoing bladder neck reconstruction using the silastic sheath in two major pediatric centers. The success with this technique was markedly different in the two centers. The purpose of the study was to determine factors that might explain the divergent results. PATIENTS AND METHODS: Fifteen patients treated in Indianapolis were compared with 94 patients treated in London with the silastic sheath technique of bladder neck reconstruction. Eighty-seven percent of the Indianapolis patients had myelomeningocele whereas 86% of the London group had exstrophy/epispadias. Median age of the Indianapolis patients was 11 years whereas it was 8.4 years in London. Seventy-three percent of patients in Indianapolis were female and 79% in London were male. Patients were followed for a minimum of eight years in Indianapolis and a mean of seven years in London. Similar surgical technique was employed in the two centers but, over time, the London approach included use of a non-reinforced silastic wrapped loosely around the bladder neck with the interposition of omentum. RESULTS: Both groups achieved continence rates exceeding 90%. Of the Indianapolis patients, two-thirds experienced erosion of the silastic at a mean of 48 months. With modifications in the London technique, the erosion rate of silastic was lowered from 100% to 7%. CONCLUSION: Direct, snug wrap of silastic without omentum around the Young-Dees tube as well as simultaneous bladder augmentation placed patients at increased risk for erosion. The silastic sheath technique may be less applicable to myelomeningocele patients. It seems most applicable to older male patients with exstrophy or epispadias undergoing Young-Dees bladder neck reconstruction who have the ability to void.