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Purpose: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes., Materials and Methods: A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded., Results: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals., Conclusions: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

Purpose: ERAS (enhanced recovery after surgery) protocols are designed to optimize perioperative care and expedite recovery. Historically, complete primary repair of bladder exstrophy has included postoperative recovery in the intensive care unit and extended length of stay. We hypothesized that instituting ERAS principles would benefit children undergoing complete primary repair of bladder exstrophy, decreasing length of stay. We describe implementation of a complete primary repair of bladder exstrophy-ERAS pathway at a single, freestanding children's hospital., Materials and Methods: A multidisciplinary team developed an ERAS pathway for complete primary repair of bladder exstrophy, which launched in June 2020 and included a new surgical approach that divided the lengthy procedure into 2 consecutive operative days. The complete primary repair of bladder exstrophy-ERAS pathway was continuously refined, and the final pathway went into effect in May 2021. Post-ERAS patient outcomes were compared with a pre-ERAS historical cohort (2013-2020)., Results: A total of 30 historical and 10 post-ERAS patients were included. All post-ERAS patients had immediate extubation ( P = .04) and 90% received early feeding ( P < .001). The median intensive care unit and overall length of stay decreased from 2.5 to 1 days ( P = .005) and from 14.5 to 7.5 days ( P < .001), respectively. After final pathway implementation, there was no intensive care unit use (n=4). Postoperatively, no ERAS patient required escalation of care, and there was no difference in emergency department visits or readmissions., Conclusions: Applying ERAS principles to complete primary repair of bladder exstrophy was associated with decreased variations in care, improved patient outcomes, and effective resource utilization. Although ERAS has typically been utilized for high-volume procedures, our study highlights that an enhanced recovery pathway is both feasible and adaptable to less common urological surgeries.

Purpose: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy., Materials and Methods: Our 3 institutions alternatively served as the host site for scheduled surgeries with observation, commentary and critique by visitors from the other sites. The technique was complete primary repair with bilateral iliac osteotomy. The timing of complete primary repair at age 1 to 3 months facilitated collaboration. We recorded patient demographics and outcomes, and the impact of this collaboration on our technique and experience. Video recording was used for real-time observation and teaching, and future analysis, editing and review., Results: A total of 16 site visits occurred from February 2013 through May 2014. Complete primary repair was performed in 9 males and 7 females with bladder exstrophy. Median age at complete primary repair was 2 months (range 0.1 to 28.8). Median followup was 8.9 months (range 2.8 to 18.2). All closures were successful with no dehiscence. Complications included urethrocutaneous fistula in 2 patients, 1 episode of pyelonephritis in 3 each and urethral obstruction in 2 females, of whom 1 required clean intermittent catheterization., Conclusions: We report a multi-institutional collaboration to standardize the surgical management of bladder exstrophy. This effort increased the annual experience of each institution involved from threefold to ninefold and it has accelerated the physician knowledge base to ultimately benefit patient care., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: We evaluated the relationship between testicular volume differential, total testis volume and total motile sperm count in adolescents with varicocele. Prior reports have been inconclusive regarding the association of testis volume with semen analysis parameters., Materials and Methods: We retrospectively reviewed a single institution database of Tanner 5 adolescents with nonoperated left varicocele with at least 1 semen analysis available. Patients were included in analysis if they had clinical left varicocele, ultrasound measurement of testis volume and no prior inguinal or scrotal surgery., Results: We analyzed the records of 100 patients. Mean ± SD age at presentation was 15.2 ± 3.5 years. The mean testicular volume differential was 8% ± 23% and 31 patients (31%) had a greater than 20% testicular volume differential. Mean total testis volume was 30.4 ± 13.3 cc. Varicocele was grade 3 in 39 patients, grade 2 in 50, grade 1 in 8 and ungraded in 3. Median total motile sperm count in the cohort was 42.0 (IQR 9.2, 105.8). ROC analysis with the Youden index indicated an optimal total testis volume cutoff of 30 cc to predict the total motile sperm count. In a multivariable logistic model accounting for age and varicocele grade a testicular volume differential of greater than 20% was associated with twice the odds of a total motile sperm count of less than 20 million per cc (OR 2.1, 95% CI 1.02-4.12, p = 0.04). Total testis volume less than 30 cc more than quadrupled the odds of a total motile sperm count of less than 20 million per cc (OR 4.2, 95% CI 1.8-9.7, p <0.001)., Conclusions: Total testis volume and the testicular volume differential are associated with semen analysis outcomes in adolescents with varicocele. A testicular volume differential of greater than 20% doubles the odds of a low total motile sperm count. A total testis volume of less than 30 cc quadruples the odds of a low total motile sperm count., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Augmentation cystoplasty using gastrointestinal segments in children/adolescents with medically refractory neurogenic bladder is associated with significant complications. We evaluated an autologous cell seeded biodegradable scaffold (Tengion®) for bladder augmentation as an alternative to traditional enterocystoplasty in this population., Materials and Methods: A phase II prospective study was performed in children with neurogenic bladder due to spina bifida requiring enterocystoplasty for detrusor pressure 40 cm H2O or greater despite maximum antimuscarinic medication. Following open bladder biopsy, urothelial and smooth muscle cells were grown ex vivo and seeded onto a biodegradable scaffold to form a regenerative augment as the foundation for bladder tissue regeneration. Bladder neck sling was the only concomitant surgical procedure permitted. Bladders were cycled postoperatively to promote regeneration. Primary and secondary outcomes at 12 months included change in bladder compliance, bladder capacity and safety. Long-term assessment was done with similar outcomes at 36 months., Results: Compliance improved in 4 patients at 12 months and in 5 patients at 36 months, although the difference was not clinically or statistically significant. There was no clinical or statistical improvement in bladder capacity at 12 or 36 months in any patient. Adverse events occurred in all patients, and most were easily treated. Two patients had low cell growth following bladder biopsy, of whom 1 withdrew from the study and 1 underwent a second biopsy. Serious adverse events of bowel obstruction and/or bladder rupture occurred in 4 patients., Conclusions: Our autologous cell seeded biodegradable scaffold did not improve bladder compliance or capacity, and our serious adverse events surpassed an acceptable safety standard., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Concern in patients with bladder exstrophy after reconstruction regarding potential injury to pelvic neurourological anatomy and a resultant functional deficit prompted combined (simultaneous) cystometrography and electromyography after complete primary repair of bladder exstrophy. We determined whether complete primary repair of bladder exstrophy would adversely affect the innervation controlling bladder and external urethral sphincter function., Materials and Methods: Complete primary repair of bladder exstrophy was performed via a modified Mitchell technique in newborns without osteotomy. Postoperative evaluation included combined cystometrography and needle electrode electromyography via the perineum, approximating the external urethral sphincter muscle complex. Electromyography was done to evaluate the external urethral sphincter response to sacral reflex stimulation and during voiding., Results: Nine boys and 4 girls underwent combined cystometrography/electromyography after complete primary repair of bladder exstrophy. Age at study and time after complete primary repair of bladder exstrophy was 3 months to 10 years (median 11.5 months). Cystometrography revealed absent detrusor overactivity and the presence of a sustained detrusor voiding contraction in all cases. Electromyography showed universally normal individual motor unit action potentials of biphasic pattern, amplitude and duration. The external urethral sphincter sacral reflex response was intact with a normal caliber with respect to Valsalva, Credé, bulbocavernosus and anocutaneous (bilateral) stimulation. Synergy was documented by abrupt silencing of external urethral sphincter electromyography activity during voiding., Conclusions: After complete primary repair of bladder exstrophy combined cystometrography/electromyography in patients with bladder exstrophy showed normal neurourological findings, including sacral reflex responses, sustained detrusor voiding contraction and synergic voiding, in all patients postoperatively. These findings confirm the safety of complete primary repair of bladder exstrophy. Based on our results we have discontinued routine electromyography in these patients., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Research on health related quality of life in patients with bladder exstrophy has demonstrated mixed results compared to population samples. Few studies have had a comparison group with a urological disorder and none correlated body image to health related quality of life. We compared health related quality of life in patients with bladder exstrophy to that in patients with kidney stones and correlated body image to health related quality of life in the bladder exstrophy population., Materials and Methods: Participants included 24 patients with kidney stones and 24 with bladder exstrophy. Patient age was 8 to 25 years. Participants completed the PedsQL (Pediatric Quality of Life Inventory) and the 25-item multiple choice UBIQ (Urological Body Image Questionnaire), which was created for the current study., Results: Compared to patients with kidney stones those with bladder exstrophy indicated significantly higher scores on physical functioning (p = 0.0005) and overall health related quality of life (p = 0.02) in the 8 to 17-year-old cohort. When health related quality of life was compared to body image in patients with bladder exstrophy, lower social scores were associated with those who worried about friends finding out about the condition (p = 0.01) and about having romantic relationships (p = 0.003). Lower social (p = 0.006) and emotional (p = 0.009) functioning scores were associated with patients who thought that they were less likely to have romantic relationships., Conclusions: The finding that patients with bladder exstrophy had a better physical functioning score than those with kidney stones is not surprising, given the morbidity of active stone disease. The negative correlations with body image in the bladder exstrophy population may be due to the possibility of others discovering the condition and the perceived likelihood of not having romantic relationships., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Substantial variability exists in bladder exstrophy care, and little is known about costs associated with the condition. We define the care patterns and first year cost for patients with bladder exstrophy at select freestanding pediatric hospitals in the United States., Materials and Methods: We used the Pediatric Health Information System database to identify patients with bladder exstrophy born between January 1999 and December 2010 who underwent primary closure in the first 120 days of life. Demographic, surgical, postoperative and cost data for all encounters were assessed. Multivariate linear regression was used to examine the association between patient, surgeon and hospital characteristics and costs., Results: Of the 381 patients who underwent primary closure within the first 120 days of life 279 (73%) did so within the first 3 days of life. A total of 119 patients (31%) underwent pelvic osteotomy, including 51 of 279 (18%) who underwent closure within the first 3 days of life, 38 of 67 (56%) who underwent closure between 4 and 30 days of life, and 30 of 35 (86%) who underwent closure between 31 and 120 days of life (p = 0.0017). Median inflation adjusted, first year cost in United States dollars per patient was $66,577 (IQR $45,335 to $102,398). Presence of nonrenal comorbidity and completion of primary closure after 30 days of life increased first year costs by 24% and 53%, respectively. Increased post-closure length of stay was associated with greater costs., Conclusions: At select freestanding United States pediatric hospitals the majority of bladder exstrophy closures are performed within the first 3 days of life. Most, but not all, patients undergoing closure after the neonatal period undergo osteotomy. The presence of nonrenal comorbidity and increased postoperative length of stay are associated with greater costs., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Although research in patients with urological conditions indicates that functional limitations, pain, limited mobility and social restrictions can impact self-image, to our knowledge the influence of the visibility of the condition has not been examined. We assessed body image and self-image at school age through young adulthood in patients with bladder exstrophy compared to patients with kidney stones., Materials and Methods: Patients diagnosed with bladder exstrophy and kidney stones completed a 25-item multiple choice Urological Body Image Questionnaire for ages 13 to 25 years, which was created for the current study. It consists of questions on physical appearance, avoidance behaviors, urological condition disclosure, romantic relationships and future expectations. A modified Urological Body Image Questionnaire for patients 8 to 12 years old was created by removing questions on relationships and future expectations., Results: A total of 24 patients with bladder exstrophy and 24 with kidney stones 8 to 25 years old completed the questionnaire. Compared to patients with kidney stones, patients with bladder exstrophy were significantly more likely to avoid changing clothes in front of peers and to not tell friends about the condition (each p = 0.0001). Patients with bladder exstrophy were also significantly more likely to think that the condition would prevent having future romantic relationships (p = 0.002) and children (p = 0.003)., Conclusions: Differences in the visibility and privacy of the 2 conditions likely explain the discrepancy between the groups. Findings may call for an individual approach to assessing issues related to self-esteem and identity in patients with bladder exstrophy and may highlight the importance of receiving psychosocial support., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Ureteral complications of renal transplantation can dramatically impact renal outcomes. We studied whether complications are associated with preexisting genitourinary pathology or transplant using a deceased donor allograft., Materials and Methods: We retrospectively reviewed all patients undergoing renal transplantation at our institution between 2000 and 2010. We abstracted patient demographic details, donor type (living vs deceased), end-stage renal disease etiology, reimplant technique, stent use, preoperative and postoperative imaging, history of lower genitourinary pathology and postoperative complication management., Results: A total of 211 kidneys were transplanted into 206 patients (mean age 13.7 years, mean followup 4.6 years). Most patients (89%) underwent extravesical ureteroneocystostomy without stenting (97%), with roughly half (47%) of transplants being from living donors. Preexisting urological pathology was present in 34% of cases. Postoperative obstruction or extravasation occurred in 16 cases (7.6%), of which 15 were acute. Complications were not associated with donor type, preexisting urological pathology other than posterior urethral valves, surgical technique, etiology of end-stage renal disease or patient age. However, posterior urethral valves or other preexisting genitourinary pathology was not associated with an increased likelihood of genitourinary complications. Posterior urethral valves were associated with development of postoperative vesicoureteral reflux (OR 6.7, p = 0.004) but were not associated with stent placement, surgical technique, donor type or etiology of end-stage renal disease., Conclusions: Patients with posterior urethral valves undergoing renal transplantation are at increased risk for postoperative vesicoureteral reflux but not for other acute surgical complications. There is no association between donor type, etiology of end-stage renal disease, surgical technique or patient age and increased complications., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Currarino syndrome is an inherited disorder consisting of a triad of anorectal anomaly, sacrococcygeal defect and presacral mass. We evaluated the urological issues in patients with Currarino syndrome and sought to determine whether spinal cord detethering improves urinary tract function., Materials and Methods: We retrospectively reviewed 14 patients diagnosed with Currarino syndrome. We evaluated urinary signs/symptoms and urodynamic findings before and after spinal cord detethering., Results: All patients with Currarino syndrome having a sacral defect and presacral mass were diagnosed between birth and 6 years. Of the patients 86% had a tethered spinal cord that was surgically detethered between 8 months and 6 years (average 3 years). Overall 10 of 12 children who underwent surgery had voiding complaints postoperatively, including urgency, frequency and incontinence. Five patients had recurrent urinary tract infections, of whom 3 had vesicoureteral reflux that resolved spontaneously. Three patients had mild unilateral hydronephrosis without reflux. Ten of 12 patients who underwent spinal cord detethering underwent comprehensive urodynamic evaluation. Of the 5 patients who underwent preoperative and postoperative urodynamic evaluation 3 showed improvement with resolution of detrusor overactivity or dyssynergia postoperatively, and 2 demonstrated no change. Of the 5 patients who underwent only postoperative urodynamic evaluation 4 had abnormal findings, including small capacity, poor compliance, detrusor overactivity, detrusor sphincter dyssynergia and/or high voiding pressure. No progressive denervation was seen on electromyography preoperatively or postoperatively., Conclusions: Currarino syndrome is a rare congenital disorder with few published reports regarding the long-term implications. Although no solid conclusions could be drawn regarding urodynamic improvement postoperatively due to our small sample size, spinal cord detethering did not lessen ongoing voiding complaints in the study patients., (Copyright © 2012 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Augmentation cystoplasty is the mainstay of surgical treatment for medically refractory neurogenic bladder in patients with spina bifida. Concerns regarding an increased risk of malignancy have prompted many centers to consider routine postoperative screening. We examine the potential cost-effectiveness of such screening., Materials and Methods: A Markov model was used to compare 2 screening strategies among patients with spina bifida after cystoplasty, namely annual screening cystoscopy and cytology and usual care. Model parameters were informed via a systematic review of post-augmentation malignancy and cost estimates from published reports or government sources., Results: In a hypothetical cohort the individual increase in life expectancy for the entire cohort was 2.3 months with an average lifetime cost of $55,200 per capita, for an incremental cost-effectiveness ratio of $273,718 per life-year gained. One-way and two-way sensitivity analyses suggest the screening strategy could be cost effective if the annual rate of cancer development were more than 0.26% (12.8% lifetime risk) or there were a greater than 50% increase in screening effectiveness and cancer risk after augmentation. After adjusting for multiple levels of uncertainty the screening strategy had only an 11% chance of being cost effective at a $100,000 per life-year threshold or a less than 3% chance of being cost effective at $100,000 per quality adjusted life-year., Conclusions: Annual screening for malignancy among patients with spina bifida with cystoplasty using cystoscopy and cytology is unlikely to be cost effective at commonly accepted willingness to pay thresholds. This conclusion is sensitive to a higher than expected risk of malignancy and to highly optimistic estimates of screening effectiveness., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: A major goal of bladder exstrophy management is urinary continence, often using bladder neck reconstruction. We report our experience with bladder neck reconstruction after complete primary repair of exstrophy., Materials and Methods: Patient history, ultrasound, voiding cystourethrogram, examination using anesthesia and urodynamics were performed during a prospective evaluation. Continence was assessed using the International Children's Continence Society classification and the dry interval. Bladder capacity was measured by examination using anesthesia, voiding cystourethrogram and/or urodynamics. Urodynamics were also done to assess bladder compliance and detrusor muscle function., Results: From 1994 to 2010 we treated 31 male and 15 female patients with bladder exstrophy after complete primary repair of exstrophy. Of patients 5 years old or older bladder neck reconstruction was performed after complete primary repair in 9 of 21 males (43%) and in 3 of 11 females (27%) at a mean age of 6.3 and 8.1 years, respectively. By the International Children's Continence Society classification 6 of 12 patients (50%) were continent less than 1.5 years after bladder neck reconstruction and 2 of 9 (23%) were evaluable 1.5 years or greater after reconstruction. Median bladder capacity was 100 ml before, 50 ml less than 1.5 years after and 123 ml 1.5 years or greater after bladder neck reconstruction. Three males and 2 females emptied via an appendicovesicostomy. Two boys underwent augmentation., Conclusions: In our experience most patients with bladder exstrophy require bladder neck reconstruction after complete primary repair of exstrophy. The need for reconstruction is more common in males. Our rates of bladder neck reconstruction after complete primary repair of exstrophy and of continence after bladder neck reconstruction are similar to those in other reports., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: We investigated associated urodynamic abnormalities in toilet trained children with vesicoureteral reflux., Materials and Methods: A total of 298 toilet trained children with primary vesicoureteral reflux underwent urodynamic evaluation. Urodynamic parameters were reviewed and correlated with age, gender, presence of lower urinary tract symptoms and reflux severity., Results: Symptomatic lower urinary tract symptoms were present in 111 children (37.2%, group 1). Children with lower urinary tract symptoms had significantly decreased severity of vesicoureteral reflux compared to children without these symptoms (187 patients, group 2). The majority of the patients had normal early bladder compliance regardless of presence of lower urinary tract symptoms or reflux grade. On the other hand, decreased late bladder compliance was more common in group 1 vs group 2. Ratio of cystometric bladder capacity to expected bladder capacity was higher in group 2. Detrusor overactivity was observed in 28.5% of the children, and the incidence was significantly higher in group 1 vs group 2, and in mild vs moderate or severe reflux. Dysfunctional voiding from bladder sphincter dyscoordination was seen in 32% of children 2.5 to 4 years old with vesicoureteral reflux and lower urinary tract symptoms, compared to 8% in children 5 to 16 years old., Conclusions: The presence of lower urinary tract symptoms in children with vesicoureteral reflux correlated well with some urodynamic findings suggestive of overactive bladder and negatively correlated with reflux severity. In contrast, dysfunctional voiding was more common in younger children with reflux and lower urinary tract symptoms. These findings suggest that treatment of voiding dysfunction should be directed toward the specific type of abnormality in children with vesicoureteral reflux., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Laparoscopic pyeloplasty is one of the more common robotic assisted procedures performed in children. However, data regarding long-term experience and clinical outcomes for this procedure are limited. We evaluated the long-term outcomes in a large series of patients undergoing robotic assisted laparoscopic pyeloplasty at a teaching institution, and the effect of a collaborative program between the robotic surgeons, surgical nurses and anesthesiologists on overall operative time., Materials and Methods: We retrospectively reviewed 155 patients who underwent robotic assisted laparoscopic pyeloplasty between 2002 and 2009. Operative data, including surgical approach, type of procedure, total and specific operative times and placement of ureteral stents, were determined. Postoperative outcome measurements, including duration of hospital stay, duration of Foley catheter drainage, radiological findings and any subsequent complications, were assessed., Results: Mean operative time and length of hospitalization decreased significantly by the end of the study. At a mean followup of 31.7 months the primary success rate was 96% (hydronephrosis was improved in 85% of patients and stable in 11%). The complication rate was 11%, and recurrent obstruction requiring redo robotic assisted laparoscopic pyeloplasty or open pyeloplasty developed in 3% of patients. Success rate and complication rate were unchanged during the study period., Conclusions: This study confirms that even within the confines of a pediatric urology training program successful collaboration between robotic surgeons, surgical nurses and anesthesiologists can lead to shorter operative times and hospital stays. Long-term surgical success and complication rates were comparable to open surgery., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Urinary tract infection will develop in 40% of children who undergo renal transplantation. Post-transplant urinary tract infection is associated with earlier graft loss in adults. However, the impact on graft function in the pediatric population is less well-known. Additionally the risk factors for post-transplant urinary tract infection in children have not been well elucidated. The purpose of this study was to assess the relationship between pre-transplant and post-transplant urinary tract infections on graft outcome, and the risk factors for post-transplant urinary tract infection., Materials and Methods: A total of 87 patients underwent renal transplantation between July 2001 and July 2006. Patient demographics, cause of renal failure, graft outcome, and presence of pre-transplant and post-transplant urinary tract infections were recorded. Graft outcome was based on last creatinine and nephrological assessment., Results: Median followup was 3.12 years. Of the patients 15% had pre-transplant and 32% had post-transplant urinary tract infections. Good graft function was seen in 60% of the patients and 21% had failed function. Graft function did not correlate with a history of pre-transplant or post-transplant urinary tract infection (p >0.2). Of transplanted patients with urological causes of renal failure 57% had post-transplant urinary tract infection, compared to only 20% of those with a medical etiology of renal failure (p <0.001)., Conclusions: In this study there was no correlation between a history of urinary tract infection (either before or after transplant) and decreased graft function. History of pre-transplant urinary tract infection was suggestive of urinary tract infection after transplant. Patients with urological causes of renal failure may be at increased risk for post-transplant urinary tract infection., (Copyright © 2010 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Optimal management for bulbous urethral stricture in children is poorly defined. We compared our long-term experience with direct vision internal urethrotomy and open repair to define the optimal surgical strategy., Materials and Methods: We reviewed the records of 63 patients who underwent direct vision internal urethrotomy or open repair. A total of 46 patients (73%) were treated with 1 or more urethrotomies. Of the patients 17 (27%) underwent urethroplasty, 13 underwent end-to-end repair and 4 received a patch graft or tube. Eight of 17 cases required urethroplasty only, whereas in 9 combined open repair and urethrotomy were done. Mean patient age was 14.1 years (range 5 months to 21 years). Followup included voiding cystourethrogram, retrograde urethrogram and/or cystoscopy, or flow rate. Mean followup was 30 months for urethrotomy and 16 months for open urethroplasty., Results: When direct vision internal urethrotomy was the initial approach, 1 procedure was successful in 28 of 53 cases (53%). Multiple urethrotomies increased the success rate to 59% (43 of 73 cases). The 53 patients with urethrotomy required a total of 84 procedures (mean 1.6 each). When open repair was the initial approach, 1 procedure was successful in 8 of 10 cases (80%). A total of 12 procedures (mean 1.2 each) were required in those 10 cases. A combined urethrotomy/open approach with 2 procedures was successful in 78% of cases (7 of 9)., Conclusions: Open reconstruction is more successful than direct vision internal urethrotomy as the initial approach to bulbous urethral strictures. Although aggressive, end-to-end repair usually provides a definitive solution. Initial direct vision internal urethrotomy is successful in half of the cases and repeat urethrotomy adds little to success. The success of the combined urethrotomy/open approach approximates that of initial open reconstruction. If initial direct vision internal urethrotomy is elected, we advocate only 1 attempt, followed by open end-to-end urethroplasty if necessary.

Purpose: Biofeedback therapy is a valuable modality in children with dysfunctional voiding. However, it is unclear what factors contribute to the outcome. To define who may or may not benefit from biofeedback therapy we reviewed our experience with this treatment., Materials and Methods: We retrospectively reviewed the charts of 77 children referred between July 2005 and September 2008 for biofeedback therapy. An MR 20 Synergy trainer (Prometheus Group, Dover, New Hampshire) provided nonanimated and animated biofeedback. Uroflowmetry was performed at the start and end of each session. A total of 67 females and 10 males with a mean age of 9.0 years (range 4.8 to 18.2) comprised the cohort group. The primary referral diagnosis was nonfebrile urinary tract infection in 52 patients (67.5%), daytime and nighttime wetting in 47 (61%), voiding postponement in 14 (18.2%) and daytime incontinence in 10 (13%). Children were categorized by an outcome of success, improvement or failure. Results were analyzed using the chi-square, Fisher exact probability and Student t tests., Results: Success, improvement and failure were achieved in 22 (26.8%), 29 (37.7%) and 26 cases (33.7%), respectively. Age and gender were not statistically significant predictors of outcome. A median of 3.0 sessions (range 1 to 8) was administered. Children with 3 or greater sessions were more likely to succeed (p <0.005). The improvement in urinary tract infections was statistically significant (p <0.001). Of 37 children 20 (54%) transformed a staccato voiding pattern to a normal one on uroflowmetry., Conclusions: Biofeedback therapy can be effective in children with dysfunctional voiding and urinary tract infection. Children with a staccato voiding pattern may require a minimum of 3 visits to improve the voiding pattern. Children who complete 3 sessions are more likely to succeed.

Purpose: We determined the resolution rate of vesicoureteral reflux and the factors that influence it to formulate nomograms to predict the probability of annual resolution for individual cases of reflux., Materials and Methods: We studied 2,462 children with primary vesicoureteral reflux diagnosed between 1998 and 2006. Cox proportional hazards regression was used to model time to resolution as a function of statistically significant demographic and clinical variables. The resulting model was used to construct nomograms predicting the annual cumulative probability of reflux resolution., Results: Multivariate analysis showed that all cases of unilateral reflux resolved earlier than female bilateral reflux (HR 1.42, p <0.001). Additionally age less than 1 year at presentation (HR 1.31, p <0.001), lower reflux grade (2.96, p <0.001 for grade I; 2.28, p <0.001 for grade II; 1.63, p <0.001 for grade III), reflux diagnosed on postnatal evaluation for prenatal hydronephrosis or sibling screening (1.24, p = 0.002) and single ureter (1.55, p <0.001) were associated with significantly earlier resolution of reflux. Specific predicted cumulative probabilities of reflux resolution at annual intervals from diagnosis (1 to 5 years) were calculated for every possible combination of the significant variables., Conclusions: Our analyses demonstrate that resolution of vesicoureteral reflux is dependent on age at presentation, gender, grade, laterality, mode of clinical presentation and ureteral anatomy. We constructed nomogram tables containing estimates of annual reflux resolution rate as a function of these variables. This information is valuable for clinical counseling and management decisions.

Purpose: We prospectively evaluated the precision of ultrasound and computerized tomography to diagnose urinary stones in children and determined whether these differences in radiological findings have any impact on clinical management., Materials and Methods: A total of 50 consecutive patients with suspected urolithiasis underwent computerized tomography and ultrasound. Two radiologists reviewed each study independently in blinded fashion. When a difference in findings was detected, 8 pediatric urologists reviewed the case. Clinical management was based on the results of each radiological test independently. Statistical analysis was performed using Fisher's exact test., Results: Compared to computerized tomography ultrasound had 76% sensitivity and 100% specificity. In 8 patients stone(s) seen on computerized tomography was not seen on ultrasound. The average size of missed stones was 2.3 mm. In 7 patients computerized tomography showed stones bilaterally but stone was seen on only 1 side on ultrasound. When evaluating the clinical impact, the ultrasound/computerized tomography discrepancy did not result in any significant change in clinical management except in 4 cases. In these cases ultrasound findings suggested that additional imaging was required and, thus, stone(s) in the distal ureter would have been identified on subsequent imaging., Conclusions: Although computerized tomography is more sensitive for detecting urolithiasis than ultrasound, the difference in usefulness between the 2 radiological tests may not be clinically significant. Given concerns for the potentially harmful cumulative long-term effect of radiation, ultrasound should be considered the first imaging test in children with suspected urolithiasis.

Purpose: The safety, benefits and usefulness of laparoscopic partial nephrectomy have been demonstrated in the pediatric population. We describe our technique, and determine the safety and feasibility of robot assisted laparoscopic partial nephrectomy based on our initial experience., Materials and Methods: We retrospectively reviewed robot assisted laparoscopic partial nephrectomy performed at our institution between 2002 and 2005. The technique was conducted via a transperitoneal approach with the da Vinci Surgical System using standard laparoscopic procedural steps. Clinical indicators of outcomes included estimated blood loss, complications, in hospital narcotic use and length of stay., Results: Robot assisted laparoscopic partial nephrectomy was completed successfully in 9 cases. Mean patient age was 7.2 years and mean followup was 6 months. Mean operative time was 275 minutes and mean estimated blood loss was 49 ml. Operative times improved significantly with experience. Overall patients had a mean hospitalization of 2.9 days and required 1.3 mg morphine per kg. All patients had a normal remaining renal moiety confirmed on Doppler ultrasound. The only complication was an asymptomatic urinoma discovered on ultrasound, which was treated with percutaneous drainage and ultimately resolved., Conclusions: Our initial experience shows the safety and feasibility of robot assisted laparoscopic partial nephrectomy in children. Operative time decreases with experience. The enhanced visualization and dexterity of a robotic system potentially offer improved efficiency and safety over standard laparoscopy. Robot assisted laparoscopy is an option for partial nephrectomy and may become the minimally invasive treatment of choice.

Purpose: The new technique of complete primary repair of bladder exstrophy has offered the promise of improved bladder functional outcomes and yet longitudinal followup in patients with exstrophy who have undergone this form of closure is sparse. We present our median 5-year data on patients who have undergone complete primary repair of bladder exstrophy., Materials and Methods: The records of patients who underwent complete primary repair of bladder exstrophy were analyzed retrospectively. Patients were followed prospectively using case report forms, radiological and clinical data, and semistructured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status, and episodes of urinary tract infection., Results: From 1994 to 2007 complete primary repair of bladder exstrophy was performed in 32 patients. Of these patients 28 underwent closure within 72 hours of life, including 20 males and 8 females, and 4 underwent it after 72 hours at ages 7 days to 12 months. One patient underwent complete primary repair of bladder exstrophy elsewhere. Median followup was 5 years (range 1 to 13). In 32 patients a total of 193 surgical procedures were done, of which 40% were minor endoscopic cases. Six of 23 males (26%) underwent bladder neck reconstruction at a median age of 4.5 years (range 4 to 10). Two boys and 1 girl received Deflux injection to the bladder neck at ages 3 to 5 years. To date no patient has undergone bladder augmentation. Nine patients (28%) had 1 to 4 episodes of pyelonephritis and 6 had cortical defects on renal scan. A total of 30 patients were voiding or incontinent via the urethra. One patient performed clean intermittent catheterization via the urethra and 1 performed it via appendicovesicostomy. All 4 children who were at least 6 months from bladder neck reconstruction after complete primary repair of bladder exstrophy had urinary continence periods of 2 to 3 hours or greater. Parents did not report any fecal incontinence or soiling in children older than 4 years. Relative to males the females had better urinary continence and a decreased need for bladder neck reconstruction., Conclusions: Complete primary repair of bladder exstrophy has been shown to be safe and efficacious. Prospective followup in this small number of evaluable patients reveals that continence periods of more than 2 hours are possible in patients after bladder neck reconstruction. Long-term followup in patients after complete primary repair of bladder exstrophy continues to be necessary to establish the long-term effects of this procedure.

Purpose: Surgical indications for correcting adolescent varicocele include high varicocele grade and testicular hypotrophy. To our knowledge these findings have not been correlated with semen parameters to date. We examined the relationship between unilateral left varicocele grade or the testicular volume differential and semen parameters in adolescents., Materials and Methods: Semen analyses were done in 57 Tanner stage V adolescent males at ages 14 to 20 years (mean+/-SD 18 +/- 1.6). Varicocele grade was determined by the attending urologist. Testicular volumes were determined by scrotal ultrasound performed by an attending sonologist. Data analysis was performed using nonparametric statistical methods., Results: Boys with testicular volume differentials greater than 10% vs those with differentials less than 10% had significantly lower sperm concentration and total motile sperm counts. For differentials greater than 20% these decreases were more dramatic. Percent motile sperm was significantly lower for boys with volume differentials greater than 20% vs those in the less than 10% and 10% to 20% categories. No significant differences were detected in percent normal morphology among the volume differentials. No significant differences were detected for any semen analysis parameter as a function of varicocele grade., Conclusions: Sonographically derived volume differentials greater than 10% between normal and affected testes correlate with a significantly decreased sperm concentration and total motile sperm count. This finding may serve as a marker to identify adolescents with unilateral left varicocele who are at greatest risk for future infertility. At least semen analysis and close annual followup of these adolescents seem warranted.

Purpose: The treatment of large stone burdens in children is difficult and often requires multiple procedures using a combination of therapies. Recently, laparoscopy has been shown to be effective in the management of larger stone burdens. We report our experience with robot assisted laparoscopic lithotomy in adolescents, and describe our technique., Materials and Methods: We retrospectively reviewed our experience with robot assisted laparoscopic pyelolithotomy in 5 patients operated on between 2002 and 2005. Mean patient age at surgery was 16.6 years, and mean followup was 15.4 months., Results: Cystine was the etiology in 4 patients with staghorn stones. The remaining patient had calcium oxalate stones and concurrent ureteropelvic junction obstruction. After pyelotomy stones were removed by a robotic grasper or by a flexible cystoscope introduced through a robotic port. One of the patients had an indwelling ureteral stent placed preoperatively, while 4 had stents placed robotically intraoperatively. Mean operative time was 315.4 minutes (range 165.0 to 462.0), and mean estimated blood loss was 19.0 ml (0.0 to 50.0). Mean hospital stay was 3.8 days (range 2.3 to 5.7), and mean narcotic usage was 2.1 mg/kg morphine (1.5 to 3.5). One patient with a cystine staghorn calculus required conversion to an open procedure because of inability to remove the stone. Of the 4 cases completed robotically 3 were rendered stone-free and 1 had a residual 6 mm lower pole stone., Conclusions: The early results of robot assisted laparoscopic lithotomy reveal that the procedure is safe and efficacious. Further prospective studies comparing other minimally invasive procedures used for similar stone burdens are needed to determine the benefits of this procedure and its role in stone management.

Purpose: We compared urodynamic findings in patients who underwent prenatal closure of myelomeningocele with those of patients who underwent postnatal closure, and equivalent lower urinary tract evaluations., Materials and Methods: Urodynamic studies of 5 patients (2 boys, 3 girls) who underwent prenatal closure of myelomeningocele were compared to those of 88 patients with similar level lesions who underwent repair postnatally between 1979 and 2002., Results: All 5 patients in the prenatally treated cohort had lower lumbosacral lesions on neurological examination. These patients displayed no evidence of electromyographic activity, indicating complete denervation of the external sphincter. In comparison 34 of the 88 patients in the postnatal cohort (39%) lacked sphincter activity at newborn examination, with similar findings noted at 1-year evaluation. In terms of bladder function all 5 patients in the prenatal cohort exhibited detrusor overactivity, compared to 33 of the 88 patients (38%) in the postnatal cohort at the newborn examination, with similar findings at 1-year evaluation., Conclusions: Fetal closure of myelomeningocele is associated with a higher incidence of complete denervation of the external urethral sphincter and detrusor overactivity compared to postnatal closure. Patients who undergo this novel procedure should undergo urodynamic studies in the immediate newborn period and should be under close postnatal surveillance to document possible tethering of the spinal cord, urinary incontinence and increased detrusor pressures.

Purpose: We assessed the usefulness of RALP in children and compared an age matched cohort undergoing OPN to RALP for safety, efficacy, operative time, blood loss, in-hospital narcotic use and LOS., Materials and Methods: We performed a retrospective case-control study from 2000 to 2004 of 33 patients undergoing RALP and 33 undergoing OPN. Average age of each group was not significantly different (RALP 7.8 years vs OPN 7.6 years, p = 0.75). Mean followup of RALP and OPN groups was 10 and 21 months, respectively., Results: Etiology of the obstruction was not significantly different. Mean operative time was significantly less for OPN (181 minutes vs 219 minutes for RALP, p = 0.031). As RALP experience increased, operative times improved and approached the OPN experience. RALP complications included 1 patient requiring reoperative surgery vs no complications in the OPN group (p = 0.15). Patients undergoing RALP had a mean LOS of 2.3 days compared to 3.5 days for OPN (p <0.001). Total narcotic requirements were significantly less in the RALP group (p = 0.001). All patients in the OPN and 31 in the RALP group had either resolution of hydronephrosis, improvement in drainage or relief of symptoms., Conclusions: We documented the safety and efficacy of RALP in children. RALP showed advantages of decreased hospital stay, decreased narcotic use and operative times approaching those of open surgery. RALP is an option for pyeloplasty, and as robotic technology improves, this method of repair may become the minimally invasive treatment of choice.

Purpose: SCT treatment in newborns consists of surgery and selective chemotherapy. Few reports document urological sequelae using this approach. This review focuses on the urological and neurourological findings following SCT treatment in the newborn period., Materials and Methods: We reviewed the records of all infants with SCT resected in early infancy who underwent urodynamic evaluation for bladder dysfunction between 1986 and 2004. The radiological, neurological and urodynamic findings, and postoperative incontinence management were analyzed., Results: We analyzed UDS of 14 patients who presented with urinary infection or incomplete bladder emptying after SCT resection. At the time of UDS an abnormal neurological examination was noted in 5 patients (36%). Detrusor overactivity was seen in 8 patients, underactivity in 2 and normal activity in 4. Abnormal urethral sphincter EMG potentials were observed in 7 of 13 patients (54%). Five of 13 patients (38%) had sphincter dyssynergia during voiding. Consequently, CIC was needed in 11 of the 14 patients (79%) to empty the bladder, of whom 5 also required anticholinergics to improve detrusor compliance and dryness. Only 3 patients voided spontaneously with normal bladder and sphincter function, of whom 2 were toilet trained. Hydronephrosis was seen in 6 patients and reflux was noted in 7 (including 5 of 6 with hydronephrosis). Antireflux surgery was performed in 6 patients, all of whom had up to grade 4 reflux due to recurrent urinary tract infection. One girl with grade 2 reflux had spontaneous resolution., Conclusions: SCT and its treatment can produce neurourological dysfunction of the lower urinary tract with high grade reflux, and abnormal bladder and urethral function. Complete assessment, including urodynamic studies, is imperative preoperatively and postoperatively. Constant vigilance is required to maintain as near normal bladder function as possible and to prevent upper urinary tract injury.

Purpose: Testicular hypotrophy is the most widely accepted indication for correcting adolescent varicocele. Previous studies in adolescents have shown a relationship between increasing grade of varicocele and the likelihood of testicular hypotrophy. As this relationship has significant clinical implications, we studied the correlation between grade and testicular volume disproportion in our adolescent varicocele population., Materials and Methods: We reviewed the adolescent varicocele database at our institution. A total of 168 patients 8 to 21 years old were studied. We routinely calculated testis volumes using scrotal ultrasound. Testicular disproportion was calculated using the equation [(size of unaffected testis) - (size of affected testis)]/(size of unaffected testis) x 100%. Disproportion was categorized as less than 10%, 10% to 20% and more than 20%. Varicoceles were graded by an attending urologist with the patient standing, using the system of Dubin and Amelar. Analysis of variance and Pearson chi-square indicated no significant differences in volume differential between varicocele grades., Results: Mean +/- SD volume differential was 18% +/- 15% for grade I, 25% +/- 20% for grade II and 19% +/- 14% for grade III. ANOVA revealed no significant difference in mean volume differential between the 3 varicocele grades (p = 0.10). When categorizing patients into 3 levels of volume differential (less than 10%, 10% to 20%, more than 20%) no significant correlation was observed between varicocele and volume differential (p = 0.48, chi-square test)., Conclusions: Grade of varicocele does not correlate with presence or severity of testicular disproportion in adolescent boys with varicocele as measured by scrotal ultrasound.

Purpose: Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE., Materials and Methods: From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections., Results: A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan., Conclusions: We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.

Purpose: Balanitis xerotica obliterans (BXO) is a chronic dermatitis of unknown etiology most often involving the glans and prepuce but sometimes extending into the urethra. We report our 10-year experience with BXO in pediatric patients., Materials and Methods: Our pathology database was queried for all tissue diagnoses of BXO from 1992 to 2002. Available charts were reviewed and patient presentation, clinical and referral history, operative procedure(s) and postoperative course were recorded., Results: A total of 41 patients had a tissue confirmed diagnosis of BXO. Median patient age was 10.6 years. Of the patients 85% were 8 to 13 years old and all had referrals available for review. The most common referral diagnoses were phimosis (52%), balanitis (13%) and buried penis (10%). No patient had the diagnosis of BXO at referral. Of the patients 19 (46%) underwent curative circumcision or redo circumcision and had no recurrence at a mean followup of 12.5 months (range 1 to 57). A total of 11 patients (27%) had BXO involvement of the meatus and underwent circumcision combined with meatotomy or meatoplasty. Nine patients (22%) required extensive plastic operation(s) of the penis, including buccal mucosa grafts in 2., Conclusions: The incidence of BXO in pediatric patients may be higher than previously reported, with the diagnosis rarely made by pediatricians. Our study demonstrates that older patients, those with BXO involvement of the meatus and those with a history of surgery for BXO tend to have a more severe and morbid clinical course.

Purpose: We characterize pelvic soft tissue and bony anatomy of patients before and after complete primary repair of exstrophy (CPRE)., Materials and Methods: We evaluated 15 measurements on pelvic magnetic resonance imaging (MRI) in patients who underwent CPRE without osteotomy at our institution from 1996 to 2004. MRI protocols included axial, sagittal and coronal fast spin echo proton density sequences. Measurements were compared before and after CPRE using a paired t test, and between patients after CPRE, and age and gender matched controls using linear regression adjusting for the matched case-control groups. Patients older than 3 years with continent intervals longer than 3 hours after CPRE were compared to age and gender matched controls using linear regression., Results: A total of 29 MRIs in 18 patients with exstrophy were available for analysis. Median patient age at postoperative MRI was 25 months (range 4 to 36). The configuration of the post-CPRE pelvis was significantly different from that of controls in all parameters measured including wider symphyseal diastasis (34.5 mm vs less than 1 mm) and more obtuse iliac wing (121 degrees vs 98 degrees), puborectalis (94 degrees vs 49 degrees) and ileococcygeus angles (111 degrees vs 98 degrees ). The anatomy of continent patients after CPRE was not significantly different from that of controls in most parameters measured., Conclusions: Comparison of the pelvic anatomy in patients before and after CPRE suggests that after CPRE patients have parameters that more closely approximate, but are still significantly different, from those of control patients. Patients with greater than 3-hour continent intervals after CPRE have anatomic parameters most similar to those of age matched controls.

Purpose: Intermittent testicular torsion (ITT) is a poorly characterized condition but harbors potentially serious implications with regard to testicular viability. We report better characterization of the diagnostic features of ITT., Materials and Methods: We performed a retrospective review of all patients 1 to 18 years old seen from 1997 to 2003 at our institution diagnosed with ITT. Patients with acute scrotal pain and spontaneous resolution who underwent bilateral testicular fixation were included in the study., Results: A total of 50 patients meeting the inclusion criteria were identified with mean age at presentation of 12.2 years (range 4 to 17). The mean number of painful episodes before surgery was 4.3 (range 1 to 30). The most common presenting features were severe pain of rapid onset and resolution. Nausea and/or vomiting was reported in a quarter of the patients. Finding of a horizontal lie of the testes on examination was significantly associated (p <0.05) with the existence of the bell-clapper deformity. All patients underwent surgical fixation of the testes. There were no postoperative complications. Of 38 patients (97%) for whom followup was available 37 had complete resolution of symptoms (mean followup 7.9 months)., Conclusions: ITT should be a diagnostic consideration in patients who present with recurrent acute scrotal pain with rapid spontaneous resolution. Recurrent severe pain with rapid onset and resolution seems to be highly characteristic. Horizontal lie on examination is highly correlated with the bell-clapper deformity at surgical exploration. Surgery may be recommended in these patients as it appears to result in pain relief in the majority, is likely to prevent future testicular infarction and is associated with low morbidity.

Purpose: We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach., Materials and Methods: We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction., Results: Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p <0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002)., Conclusions: Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

Purpose: We assessed the clinical usefulness of laparoscopic retroperitoneal partial nephrectomy (LRPN) in children by comparing an age matched cohort of pediatric patients undergoing open partial nephrectomy (OPN) to those undergoing LRPN for safety, efficacy, operative time, blood loss, inpatient narcotic use and length of hospitalization., Materials and Methods: A retrospective case-control study was performed of 1 group undergoing LRPN (treatment arm) and 1 undergoing OPN (controls) between 1997 and 2003. The 14 patients in each group were matched by age (mean 1.9 years in LRPN group vs 2.2 years in OPN group, p = 0.98) and gender., Results: All patients had duplication anomalies. Mean operative time was 194 minutes in the LRPN group and 193 minutes in the OPN group (p = 0.83). The single complication in the LRPN group was a urinoma that did not require intervention. There were no complications in OPN group. LRPN patients had a mean hospitalization of 1.7 days compared to 4.7 days in OPN patients (p = 0.001). Intraoperative narcotic requirements were higher in the LRPN group (0.59 vs 0.22 mg/kg, p = 0.04). Postoperative narcotic requirements were significantly less in the LRPN group (0.44 vs 1.53 mg/kg, p = 0.04). Similar findings were noted in a subanalysis of patients younger than 2 years. All patients had documented postoperative ipsilateral renal growth. Mean followups in the treatment and control groups were 26 and 25 months, respectively., Conclusions: Retroperitoneal laparoscopic partial nephrectomy in small children was shown to be safe and effective. It had equivalent operative times, and decreased postoperative narcotics and hospitalization relative to the open approach, and, therefore, is a potentially advantageous technique.

Purpose: We determined whether there was evidence of progression in either varicocele grade or testicular volume differentials over time in our adolescent varicocele population., Materials and Methods: A total of 41 boys 7.3 to 19 years old (mean age 13.7, SD 2.5) were seen at sequential visits. At each visit varicocele grade was determined by the attending urologist and ultrasound calculated testicular volume differential was determined by an attending ultrasonographer. A senior staff statistician performed statistical analysis of the sequential data., Results: The varicocele grade data were analyzed using the nonparametric Wilcoxon test and no significant evidence of progression on sequential visits was demonstrated. Similarly, the serial volume differential data were assessed using the paired t test and no significant evidence for progression of testicular volume differentials was demonstrated., Conclusions: In our study population neither varicocele grade nor change in testicular volume differentials increased over time in a statistically significant manner. These results argue against the concept that adolescent varicocele is a progressive disease process.

Purpose: Fibroepithelial polyps are benign mucosal projections that can be found throughout the urinary system. We review our experience with fibroepithelial polyps of the ureteropelvic junction in children to define more clearly this entity and its outcome following treatment., Methods: We reviewed the records of all children with fibroepithelial polyps causing ureteropelvic junction obstruction treated at our institution between December 1967 and February 2002., Results: Nine patients 6 weeks to 9 years old had 11 ureteropelvic junction obstructions secondary to fibroepithelial polyps, representing a 0.5% incidence of all ureteropelvic junction obstructions seen during that period. The majority of the patients were male (89%) and had obstruction on the left side (78%). Only 22% of the patients had a diagnosis of obstructing polyps suggested preoperatively. All patients underwent dismembered pyeloplasty but 1 required subsequent nephrectomy due to progressive loss of renal function. All lesions were characterized microscopically by polypoid projections of fibrous tissue covered by epithelium and demonstrating varying degrees of inflammation. No recurrences were seen during a mean followup of 44 months., Conclusions: Fibroepithelial polyps are rare lesions that cause ureteropelvic junction obstruction in children, primarily in males and on the left side. Filling defects were diagnosed preoperatively in 22% of the patients in this series. Excision and dismembered pyeloplasty were curative, and recurrences were not observed. The etiology of this disease remains obscure.

Purpose: We assessed clinical and ultrasound predictors of the spontaneous resolution of prenatally diagnosed, primary nonrefluxing megaureter., Materials and Methods: Primary nonrefluxing megaureter was prenatally diagnosed in 54 neonates from 1993 to 1998, including 15 in whom it was bilateral (69 megaureters). Median followup period from birth to the last radiology examination was 25.8 months (range 2.3 to 72)., Results: Of the patients 10 underwent surgical repair for severe hydroureteronephrosis, including decreasing renal function on diuretic renography in 1, and an episode of bilateral obstruction and acute renal failure in 1. Hydroureteronephrosis resolved in 39 cases (72%) and persisted in 5 at 30 to 72 months of followup. Laterality, gender and retrovesical ureteral diameter had no significant effect on the resolution rate (p >0.05). Mean initial ureteral diameter in patients with resolution was less than in those without resolution and in those who underwent surgery (0.8 versus 1.15 and 1.32 cm., respectively). Presenting hydronephrosis grade was a significant predictor of the resolution rate (p = 0.03). Grades 1 to 3 hydronephrosis resolved at a median age of 12.9, 23.9 and 34.6 months, respectively (range 4.1 to 66). In patients in whom grades 4 and 5 hydronephrosis resolved the median age at resolution was 48.5 months., Conclusions: Most cases of prenatally diagnosed, primary nonrefluxing megaureter resolve spontaneously. Grades 1 to 3 hydronephrosis tend to resolve between ages 12 and 36 months. For these grades followup intervals may be progressively elongated after a pattern of improving hydronephrosis has been established. In children with grade 4 or 5 hydronephrosis, or a retrovesical ureteral diameter of greater than 1 cm. the condition may resolve slowly and require surgery.

Purpose: We evaluated the impact of tubularized incised plate urethroplasty on primary and repeat hypospadias repair., Materials and Methods: We retrospectively reviewed the medical records of all boys who underwent hypospadias repair at our institution during a recent 3-year period. The level of the hypospadias defect, technique of repair, primary repair versus reoperation, age at surgery and complications were recorded., Results: A total of 520 hypospadias repairs were done from May 1996 through June 1999. We began to perform tubularized incised plate urethroplasty in November 1996. During the ensuing consecutive 32 months 181 primary and 25 repeat hypospadias repairs were done using this technique. Mean patient age at surgery was 22 months (range 3 months to 30 years). During the 6 months immediately before we began to use this method the Mathieu flip-flap procedure was the most commonly performed technique, accounting for 38% of all hypospadias repairs. In contrast, during the last 6 months reviewed tubularized incised plate urethroplasty accounted for 63% of all repairs, including 41 of 65 primary operations (63%) and 4 of 6 reoperations (67%), while no Mathieu procedures were performed. Postoperative followup was 6 to 38 months for tubularized incised plate repair. Overall meatal stenosis and a urethrocutaneous fistula developed in 1 and 14 boys, respectively (7% complication rate)., Conclusions: Tubularized incised plate urethroplasty has become the preferred technique of primary and repeat hypospadias repair at our institution. The technique has few complications as well as proved success and versatility that continues to expand its applicability and popularity.

Purpose: Although vesicoureteral reflux associated with bacteriuria may cause renal scarring, sterile reflux is thought not to cause renal injury. We determined the incidence and associated characteristics of renal abnormalities using 99mtechnetium(Tc) dimercapto-succinic acid (DMSA) renal scintigraphy in infants with high grade vesicoureteral reflux but no history of urinary tract infection., Materials and Methods: We retrospectively reviewed the results of 99mTc-DMSA renal scintigraphy and renal ultrasonography performed during the first 6 months of life in infants with vesicoureteral reflux detected during the postnatal evaluation of prenatal hydronephrosis or sibling reflux screening. Those with a history of urinary tract infection, or evidence of ureteropelvic junction or bladder outlet obstruction were excluded from study., Results: Of the 28 male and 6 female infants who met study criteria vesicoureteral reflux was bilateral in 25 and unilateral in 9. Reflux grade was IV or V, II or III and I in 38, 18 and 3 of the 59 refluxing renal units, respectively. 99mTc-DMSA renal scintigraphy revealed parenchymal abnormalities in 24 refluxing renal units (41%) in 22 patients (65%), of whom 19 (86%) were male and 15 (68%) had bilateral reflux. We noted differential uptake less than 40% with and without cortical defects in 10 and 7 refluxing units, respectively, and cortical defects only in 7. Of the 24 refluxing units with abnormalities 21 were associated with grade IV or V and 3 with grade II or III reflux. Ultrasound showed evidence of renal injury in only 7 of the 17 patients (41%) in whom 99mTc-DMSA scintigraphy was abnormal., Conclusions: In our study the majority of infants with high grade reflux had decreased differential function and/or cortical defects. Parenchymal defects detected by 99mTc-DMSA renal scintigraphy were often not identified by renal ultrasound. Therefore, 99mTc-DMSA renal scintigraphy is especially useful for initially evaluating infants with high grade, sterile vesicoureteral reflux.

Purpose: We describe several modifications of the retroperitoneoscopic approach to nephrectomy for benign renal disease, including the use of 2 mm. instrumentation and prone patient positioning., Materials and Methods: A total of 14 children underwent retroperitoneoscopic nephrectomy in the prone position. An inflatable dissecting device was inserted into the retroperitoneum after a small muscle splitting incision was made at the lateral border of the sacrospinalis muscle approximately 1 cm. below the costovertebral angle. After inflation the dissecting device was replaced with a 5 mm. cannula and pneumoretroperitoneum was maintained with carbon dioxide insufflation. Two 2 mm. trocars were then placed under endoscopic guidance. Dissection was performed using 2 mm. instrumentation and the specimen was retrieved through the largest port site., Results: Nephrectomy was performed in 9 girls and 5 boys 3 months to 9.8 years old. The preoperative diagnosis included chronic pyelonephritis with minimal renal function, reflux with a nonfunctioning kidney, multicystic dysplastic kidney, an upper pole dysplastic moiety with an associated ureterocele and a dysplastic kidney with a vaginal ectopic ureter. Mean operative time for retroperitoneoscopic nephrectomy was 142 minutes with an estimated blood loss of less than 15 ml. Contralateral ureteral reimplantation was performed after retroperitoneoscopic dissection in 5 patients. Overall average hospital stay was 2 days and there were no complications., Conclusions: Several modifications of the retroperitoneal approach, including the use of prone patient positioning and 2 mm. instrumentation for visualization and dissection, may improve the safety and efficacy of this technique in children.

Purpose: The Beckwith-Wiedemann syndrome is most commonly characterized by macroglossia and abdominal wall defect(s), and it carries a predisposition to embryonal tumors, including Wilms tumor. We report our experience with the character and incidence of renal disease in patients with the Beckwith-Wiedemann syndrome, and discuss the role of radiological followup., Materials and Methods: We reviewed the medical records of all patients diagnosed with the Beckwith-Wiedemann syndrome who were treated at our institution between March 1979 and February 1998. Radiological followup consisted of renal ultrasound at approximately 3 to 6-month intervals with the addition of computerized tomography or magnetic resonance imaging (MRI) in patients with an indeterminate lesion(s) or nephrogenic rest(s)., Results: A total of 29 patients were identified. Of these cases renal ultrasound showed normal kidneys bilaterally in 19 (70%), simple cysts in 5 (19%), indeterminate lesion(s) in 2 (7%) and nephrocalcinosis in 1 (4%). Nephrogenic rests were followed with MRI in 1 patient, and 1 in whom a 2 cm. mass was revealed by followup MRI underwent partial nephrectomy and chemotherapy for stage I Wilms tumor., Conclusions: The 3.7% incidence of Wilms tumor in our patients with the Beckwith-Wiedemann syndrome is similar to that in previously published reports. Aggressive follow-up by a sensitive radiological technique is warranted in cases of the Beckwith-Wiedemann syndrome, and associated hemihypertrophy and/or nephromegaly with or without evidence of a Wilms tumor precursor. The detection of suspected malignant disease at an early stage may permit curative nephron sparing surgery.

Purpose: We determined whether 60 to 79-year-old men with a negative digital rectal examination and a serum prostate specific antigen (PSA) within age specific PSA reference ranges could safely forgo prostate biopsy., Materials and Methods: We reviewed the medical records of all 60 to 79-year-old men at the Brooklyn Veterans Administration Medical Center who had a PSA assay, digital rectal examination and subsequent prostate biopsy for an abnormal rectal examination and/or PSA greater than 4.0 ng./ml. from January 1991 through August 1995. We compared our results using the standard reference range of 0 to 4.0 ng./ml. with those obtained had we used any of 4 different age specific PSA reference ranges., Results: We performed 1,280 prostate biopsies in 1,046 men with available PSA and digital rectal examination data. Using age specific PSA reference ranges 73 of 1,280 biopsies (5.7%) would have been avoided. Of those 73 avoided biopsies 15 (20.5%) had cancer that would have gone undetected and 9 of 15 (60%) undetected cancers had unfavorable histology. Results were not statistically significantly different among the 4 age specific PSA reference ranges. Regarding race, cancer detection rates were significantly higher for black compared with white men but there was no statistically significant difference for missed cancers or missed cancers with unfavorable histology., Conclusions: In contrast to previous reports of unfavorable histological characteristics in only 5% of missed cancers using age specific PSA reference ranges, 60% of missed cancers in our patients exhibited unfavorable histology. We conclude that age specific PSA reference ranges did not safely eliminate the need for prostate biopsy in our study population. In 60 to 79-year-old men with a negative digital rectal examination we continue to use PSA greater than 4.0 ng./ml. as an indication for prostate biopsy.

Purpose: Circumcision remains the most common operation performed on male individuals in the United States. Unfortunately various complications may occur during circumcision ranging from trivial to tragic. We report 7 cases of traumatic amputation of the glans penis and/or urethra during circumcision. In addition, errors in circumcision technique as probable mechanisms of injury, principles of repair and limits of tissue viability are discussed., Materials and Methods: The medical records of 7 patients who underwent traumatic circumcision amputation of the glans penis and/or urethra were reviewed. Glanular amputation occurred in 6, 8-day-old neonates during ritual circumcision and in 1, 5-month-old infant circumcised by a physician., Results: Excised glanular tissue remained viable up to 8 hours after injury. Followup ranged from 8.5 to 108 months. All patients had an acceptable cosmetic result. No long-term complications developed in the 8-day-old group but a distal urethral fistula formed in the 5-month-old patient., Conclusions: Careful selection of technique and device as well as strict attention to detail at circumcision should eliminate most injuries. On the basis of our results we recommend reanastomosis of the glans and/or urethra following distal amputation even when there is a delay in surgical repair of up to 8 hours.

Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism are 2 forms of male pseudohermaphroditism that present with absent müllerian regression and ambiguous genitalia. We present a retrospective analysis of 10 patients with the diagnosis of either mixed gonadal dysgenesis or dysgenetic male pseudohermaphroditism encountered during a 16-year period at our institution. We assigned a female gender to 4 patients and a male gender to 3. Three patients were assigned a male gender before referral (2 for whom we would have preferred female assignment). All intra-abdominal gonads were removed except in 2 patients assigned a male gender who underwent bilateral orchiopexy. Patients underwent hypospadias repair or feminizing genitoplasty depending upon the gender assigned. Although female gender assignment is usually preferred, male assignment is a justifiable alternative in instances of extreme virilization and a descended testis. However, in these male assigned patients, consideration must be given to the role of periodic testicular biopsies. In light of tumor potential, all intra-abdominal gonads should be removed. When necessary, appropriate surgical genitoplasty should be performed at an early patient age.