Kelly procedure for exstrophy or epispadias patients: Anatomical description of the pudendal neurovasculature.

INTRODUCTION: Adequate penile length in males with bladder exstrophy or epispadias is a major challenge. Kelly previously described a surgical technique of a single stage reconstruction for patients with exstrophy or epispadias that potentially achieves significant penile lengthening by completely detaching the insertion of the corpora cavernosa from the ischiopubic rami. However, because of the possibility of damage to the pudendal neurovascular supply that may lead to partial or complete penile loss, this technique has not gained popularity. The aim of this study is to describe the surgical anatomic relationship of the pudendal neurovascular bundle (NVB) to the ischiopubic rami and to determine a safer approach to dissection during the Kelly procedure. METHODS: We performed meticulous dissection in three formalin-fixed and one fresh adult male cadavers to demonstrate the anatomical relationships between the pudendal neurovascular supply of the penis and the cavernosal insertion to the ischiopubic ramus. RESULTS AND DISCUSSION: We demonstrated the relationships and distance between the NVB and the area of separation between the crus and the ischiopubic ramus at the level of the periosteum. The insertion of the crus to the ischiopubic ramus is inferior lateral, whereas the NVB lies at a superior medial position. This anatomical relationship is best visualized when the dissection is carried out starting from the distal portion of the NVB and proceeding proximally. This area of the periosteum is avascular and the NVB can be preserved safely as long as the dissection is conducted at that subperiosteal level. Based on this cadaver dissection study, we suppose that detaching the corporal cavernosa from the pubic bones at the subperiosteal level allows for a safe distance to be maintained from the pudendal nerve at all times. We believe that if a surgeon performs the dissection inferiorly and laterally, the corpora cavernosa can be safely detached from the ischiopubic ramus and injury to the pudendal vessels and nerve can be avoided. However, it must be noted that there are limitations to applying the results from this study of normal, adult cadavers to the anatomy of children and adolescents with exstrophy or epispadias, who form the largest proportion of patients who are candidates for this procedure. CONCLUSION: This anatomical study demonstrates the relationship between the pudendal NVB, the crus, and the ischiopubic ramus. We demonstrated how the separation of the crus from the ischiopubic periosteum might be performed more safely.

Autologous cell seeded biodegradable scaffold for augmentation cystoplasty: phase II study in children and adolescents with spina bifida.

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.

Changing stone composition profile of children with nephrolithiasis.

OBJECTIVE: To determine if this trend toward calcium phosphate stone formation exists in children. METHODS: This is a retrospective study of medical records of 179 children managed at our medical center from 1992-2010 for whom stone analysis and other pertinent laboratory data were available. A comparison of patients managed from 1992-2000 (P1) and 2001-2010 (P2) was undertaken. Statistical analysis included nonparametric tests. RESULTS: There were no significant differences in the mean age of the 2 cohorts. During both periods, boys comprised a significantly higher proportion during the first decade of life, whereas girls comprised a significantly higher proportion during the second decade. A higher percentage of patients had calcium oxalate (CaOx) stones in P1 compared to P2 (60% vs 47%, P = .0019). There was a significant increase in the percentage of patients having calcium phosphate stones in P2 compared to P1 (27% vs 18.5%, P = .008). Twenty-seven patients had recurrent stones. A comparison of the compositions of the first and last stones of patients within this group demonstrated an increasing proportion of brushite stones (3.7% vs 11.1%, P = .04). Twenty-four hour urine testing results were similar for those with CaOx and calcium phosphate stones. CONCLUSION: An increasing proportion of children have calcium phosphate calculi. Brushite stones are more prevalent in children with recurrent stone events. The impetus of these shifts is not readily apparent.

Cold knife valvulotomy for posterior urethral valves using novel optical urethrotome.

OBJECTIVES: To present our results after valve ablation using a novel cold knife urethrotome. METHODS: Eleven consecutive male patients with posterior urethral valves underwent cold knife valvulotomy using a modified optical pediatric urethrotome. Patients were assessed both pre- and postoperatively using serum creatinine, voiding cystourethrography, and renal/bladder ultrasonography. RESULTS: From August 2003 to August 2005, 11 patients underwent cold knife valvulotomy, of whom 7 returned for postoperative follow-up (mean follow-up 17.4 months). At surgery, the patients ranged in age from 5 days to 9 years. At presentation, 5 of the 7 patients had an elevated serum creatinine (mean 2.5 mg/dL, range 0.3-6.5), all had bilateral hydronephrosis of at least grade 3, and 6 of 7 had at least grade 3 reflux on 1 side. Intraoperatively, 1 complication (minor urethral laceration) occurred. Postoperatively, all 6 patients with serum creatinine levels measured showed improvement in renal function (mean creatinine 0.47 mg/dL, range 0.2-0.9). For the 6 patients who underwent postoperative ultrasonography, 4 had either complete resolution or significant improvement in their hydronephrosis, and none showed worsening. Six patients underwent postoperative voiding cystourethrography, with 5 showing either marked improvement or complete resolution of their reflux and 1 showing stable, unilateral reflux. CONCLUSIONS: Valvulotomy using our modified urethrotome is a safe and effective technique for valve ablation.

Vesicoureteral reflux: where have we been, where are we now, and where are we going?

We present a retrospective review of the scientific and clinical advances, extending over four decades, which have linked vesicoureteral reflux, with renal injury, and urinary tract infection. We have traced the original studies, coupled with advances in technology which led to the awareness, and ability to detect and diagnose the problems early in childhood. These advances progressed through clinical studies which defined the epidemiology of both reflux and urinary tract infection. Along with these diagnostic advances, there were numerous surgical developments, which allowed progressive improvements in the outcomes and effectiveness of a variety of treatment modalities. All of this literature leads us to the current era, when several clinical trials are currently underway in an effort to more fully define the most efficacious and safe methods to treat vesicoureteral reflux and associated urinary tract infection.

The bear paw: a radiologic sign for congenital ureteropelvic junction obstruction.

This article defines a new radiographic descriptor of high-grade hydronephrosis suggestive of ureteropelvic junction obstruction that is easily recognized by urologists and non-urologists alike. This is useful information for medical personnel participating in the management of children with hydronephrosis.

Comparison of traditional enterocystoplasty and seromuscular colocystoplasty lined with urothelium.

OBJECTIVE: To evaluate long-term outcomes between various methods of augmentation cystoplasty. METHODS: A retrospective analysis was performed of patients undergoing seromuscular colocystoplasty lined with urothelium (SCLU, n=26), and their outcomes compared to a similar population of patients in the same institution who had received traditional forms of bladder augmentation (colocystoplasty and ileocystoplasty, n=32). Measurements included efficacy of the procedure in increasing bladder capacity and achieving urinary continence, and the need of subsequent surgery for complications. RESULTS: There was no statistically significant difference in achieved bladder capacity, subjective urinary continence and the rates of subsequent surgery for stones, vesicoureteral reflux, augment failure, bladder neck continence and catheterizable channel. None of the patients in the SCLU group had spontaneous perforation or small bowel obstruction. CONCLUSION: Patients with SCLU are at decreased risk for bowel obstruction and spontaneous perforation, but are not devoid of other long-term complications including bladder stones, vesicoureteral reflux and augment failure. Most of the risks and benefits of augmentation cystoplasty performed using ileum, colon, or SCLU appear similar.

Management of the valve-bladder syndrome and congenital bladder obstruction: the role of nocturnal bladder drainage.

Valve-bladder syndrome often develops after the resolution of posterior urethral valves, but is also found after the resolution of congenital bladder obstruction. The features of this syndrome include the persistent dilation of the upper urinary tracts, a thick-walled, noncompliant urinary bladder, urinary incontinence, and polyuria secondary to nephrogenic diabetes insipidus. Nocturnal bladder management, which involves timed emptying of the bladder or continuous drainage, has been recommended in conjunction with diurnal timed voiding therapy as an adjunct to the treatment of valve-bladder syndrome. This treatment is derived from the hypothesis that valve-bladder syndrome is caused by congenital obstruction, and that the resultant changes in detrusor muscle are associated with a persistent bladder dysfunction characterized by chronic overdistention of the urinary bladder. Such overdistention is exacerbated by polyuria, and can be a cause of secondary hydronephrosis. Bladder dysfunction and overdistention is usually treated during waking time, but occasionally this is not effective on its own, and nocturnal therapy is used as well. To date, there are a few sets of data that suggest overnight bladder drainage can bring about profound improvements in the degree of upper-tract hydronephrosis, renal function, or bladder function. Nocturnal bladder drainage seems, in these initial reports, to be a simple and safe therapeutic maneuver. This review discusses the etiology of valve-bladder syndrome and examines each of the studies which have investigated nocturnal bladder drainage in its treatment.

Testicular infarction in a 12-year-old boy with Wegener's granulomatosis.

We present the case of a 12-year-old boy with testicular infarction secondary to Wegener's granulomatosis. Scrotal exploration revealed no evidence of testicular torsion. The testis was left in place, and, after systemic therapy, partial reperfusion was present.

The development of laparoscopic surgical skills in pediatric urologists: longterm outcome of a mentorship-training model.

INTRODUCTION AND OBJECTIVES: We previously reported the successful attainment of laparoscopic skills in a group of practicing pediatric urologists without previous formal laparoscopic training. During the mentorship period, the four urologists (trainees A, B, C, and D) performed a number of renal retroperitoneal laparoscopic procedures (RRLP) under the tutelage of an expert mentor. Specifically, trainee A performed or assisted in 8 RRLP while trainees B, C, and D performed/assisted in 10, 7, or 18 RRLP, respectively. Herein we assessed the outcome of this training program and practice pattern of this same group of urologists. METHODS: Following the completion of the mentorship period, we reviewed the outcomes of all of the consecutive RRLP performed from September 2001 to March 2005 with respect to operative time, conversion rate, perioperative complications and length of hospital stay (LOS). Furthermore, we attempted to correlate the number of procedures each surgeon performed both during and subsequent to the mentorship period. RESULTS: Fifty-two ablative RRLP including nephrectomy (n=38), partial nephrectomy (n=12), or synchronous bilateral nephrectomy (n=2), were performed on 50 patients (19 males, 31 females) with a mean age of 5.5 years (range 4 months-14 years). Trainee A performed 16/40 procedures, trainees B and C each performed 2/40, while trainee D performed 20/40 procedures. Mean operative time was 2.4 hours (range 1.5-6.3 hours). Five patients required open conversion due to inability to obtain retroperitoneal access (n=3) or failure to progress (n=2). Two patients (one nephrectomy, one partial nephrectomy) developed retroperitoneal urinomas requiring temporary urinary diversion. There were no other perioperative complications and mean LOS was 1.2 days (range 1-4 days). More advanced reconstructive procedures have since been performed with the aid of laparoscopic exposure; trainee D has thus far successfully performed 12 laparoscopically assisted pyeloplasties. CONCLUSIONS: This series demonstrates the effectiveness of the mentorship-training model to introduce RRLP to a pediatric urology training program. It is evident that the post-mentorship practice is affected by the number of cases initially performed during the training period. The development of an "expert" laparoscopist is dependent not only on initial training experience, but continued education through ongoing case exposure.

Psoas abscess complicated by vesical fistula in a child managed by non-surgical therapy.

We report an unusual case of a 2-year-old child with a psoas abscess fistulizing to the bladder, managed by non-surgical therapy including urethral catheter drainage, percutaneous abscess drainage and intravenous antibiotics.

Continent urinary diversion with a urachal remnant.

The Mitrofanoff procedure, first described in 1980, has been widely adopted for pediatric continent urinary diversion to increase patient independence and continence, decrease morbidity and infections, and preserve renal function. Although this principle employs the appendix primarily, numerous other tissue conduits have been described. We report a unique use of a urachal remnant as a continent conduit resulting in less morbidity by maintaining the integrity of the native urinary bladder, while maintaining patient continence and allowing easy and successful catheterization.

Efficacy of polydimethylsiloxane injection to the bladder neck and leaking diverting stoma for urinary continence.

PURPOSE: Achieving urinary continence is a major goal in the treatment of patients with bladder exstrophy/epispadias, spinal dysraphism and other urological disorders. Endoscopic injection of bulking materials is an evolving, minimally invasive procedure that provides an attractive alternative to open bladder surgery to increase outlet resistance. We evaluated the efficacy of bladder outlet (bladder neck and diverting stoma) polydimethylsiloxane injection (BOMI) to achieve continence in children. MATERIALS AND METHODS: We retrospectively reviewed the continence status of 33 patients prior to and following BOMI for urinary incontinence. The variables evaluated as predictors of success were underlying disease, ambulatory status, voiding status, bladder capacity, type and time from previous bladder neck reconstruction, anticholinergic treatment, injection site, amount of polydimethylsiloxane used, detrusor leak point pressure and immediate or delayed clean intermittent catheterization. Improvement was considered a 2-fold increase in the dry interval or 4 hours between bladder emptying in patients on clean intermittent catheterization and a 2-fold decrease in the number of absorbent pads needed. RESULTS: Between 1998 and 2002, 25 male and 8 female patients 4 to 19 years old (mean age 12.4 +/- 3.9) underwent 42 BOMIs for incontinence. Mean followup was 13 +/- 9 months (range 3 to 42). Incontinence was via the urethra in 28 cases and via a diverting stoma in the remaining 5. Of the 28 patients with leakage via the urethra none were cured, 12 (42%) were improved and 16 (58%) had no change in continence status. However, 3 of the 5 inpatients (60%) with leakage via a diverting stoma were cured. No perioperative complications were recorded. Injecting to a diverting stoma was the only factor found to be predictive of success. Extrusion of polydimethylsiloxane during the procedure was the only factor predictive of failure (3 of 3 cases or 100%). New onset dilatation of the collecting system was noted in 2 patients and all had improved continence following injection. CONCLUSIONS: Injection to the bladder neck did not result in any cure and had a low improvement rate of 42%. BOMI is a good therapeutic option for patients with leakage from a diverting stoma. Although no perioperative complications were recorded, 2 patients had new dilatation of the upper tract on ultrasound. Hence, long-term followup is warranted.

Pelvic floor magnetic resonance imaging after neonatal single stage reconstruction in male patients with classic bladder exstrophy.

PURPOSE: We evaluate a magnetic resonance imaging (MRI) protocol used to study the pelvic floor anatomy in male patients following neonatal single stage complete bladder exstrophy and epispadias repair with osteotomies. MATERIALS AND METHODS: From 1996 to 2002, 9 males underwent surgical correction of bladder exstrophy and epispadias with osteotomies within 1 to 12 days of birth. Pelvic floor MRI was conducted comparing this group to 5 aged matched male patients with no pelvic anatomical abnormality who underwent MRI for other illness. We compared various measurement of pelvic musculature by unpaired Wilcoxon test. RESULTS: Median followup was 3 years (range 0.5 to 5.3). All surgical procedures succeeded in closure of abdominal wall and genitalia defects. MRI data showed that in the exstrophy group symphyseal distance was significantly wider than that in controls (median 3.8 vs 1.1 cm). In addition, the levator ani fibers diverted more laterally (42 vs 22 degrees), the pelvic floor in coronal view was more flat (103 vs 80 degrees) and the anus was more anteriorly displaced (2.8 vs 4.4 cm). We also documented shorter anterior corporeal bodies in the exstrophy group (1 vs 2 cm). No statistical difference between the 2 groups was found in the dimensions of the levator and obturator muscles, sagittal angle of the pelvic floor, and the dimensions and angle of the posterior corporal bodies. Two patients achieved some degree of continence around the age of 4 years. They had the shortest symphyseal distance and sharpest angle of levator ani fiber divergence, and the bladder neck was more deeply located in the pelvic. CONCLUSIONS: We applied novel MRI parameters to the pelvic floor anatomy providing a new quantifiable approach. Our protocol is feasible and reproducible, allowing for future comparison of the impact of different surgical modalities, and correlation between anatomical findings and continence.

Hemodynamic and respiratory effect of pediatric urological laparoscopic surgery: a retrospective study.

PURPOSE: We investigate the impact of extraperitoneal and intraperitoneal CO2 insufflation on cardiopulmonary variables in children undergoing laparoscopic surgery. MATERIALS AND METHODS: The records of 73 patients who underwent laparoscopic urological surgery between December 2000 and April 2002 were retrospectively reviewed. Data collection included respiratory rate (RR), peak airway pressure (PAP), O2 saturation, end tidal CO2 (ETCO2), heart rate, systolic and diastolic blood pressure, electrocardiogram and insufflation pressure. All variables were recorded before and after CO2 insufflation. Only patients with complete records were included in the analysis. RESULTS: The study included 62 participants. Of the patients 16 boys and 13 girls with a mean age +/- SD of 7.2 +/- 5.1 years underwent extraperitoneal surgeries, 14 partial or total nephrectomy and 5 pyeloplasty. Mean retroperitoneal CO2 insufflation pressure was 12.1 +/- 1.5 mm Hg and mean operative time was 3.6 +/- 1 hours. We operated on 13 children on the right and 16 on the left decubitus lateral position. Significant increase in ETCO2, RR and PAP was recorded after CO2 insufflation in the extraperitoneal group. Use of the left lateral position resulted in a significant increase in ETCO2 (37.1 +/- 3.6 vs 40 +/- 3.8, p = 0.04) after CO2 insufflation compared to the right decubitus lateral position. Transperitoneal surgery was performed in 32 boys and 1 girl with a mean age of 3.8 +/- 4.1 years for cryptorchidism (32) and attempted pyeloplasty (1). Mean intraabdominal CO2 insufflation pressure was 11 +/- 2.4 mm Hg and mean operative time was 1.7 +/- 0.8 hours. A significant increase in RR (16.5 +/- 3.1 vs 17.9 +/- 3.4, p = 0.0002) and PAP (13.2 +/- 4.8 vs 16.1 +/- 5.7, p <0.0001), and a decrease in O2 saturation (99.6 +/- 0.6 vs 98.7 +/- 7.1, p = 0.0003) and heart rate (116 +/- 19 vs 113 +/- 18, p = 0.019) were recorded after CO2 insufflation. CONCLUSIONS: Our study documented significant hemodynamic and respiratory changes during pediatric laparoscopic surgeries. A similar effect on the respiratory parameters was observed in both groups. Although there were no apparent complications associated with either approach, further prospective studies are warranted to confirm the effect of laparoscopic urological surgery on cardiopulmonary function in children.