A Curious Case of Continuous Incontinence.

Obstructed hemivagina, ipsilateral renal anomaly (OHVIRA) is a rare Müllerian duct abnormality with renal manifestations. Classical presentation is an adolescent female with abdominal pain following menarche. Because of its rarity, diagnosis is often delayed. There have been <20 reported cases of prepubertal OHVIRA. We present a case of missed OHVIRA diagnosis that presented with continuous incontinence following incision of the obstructed hemivagina and discuss the current medical literature on this subject.

Trends in Toilet Training and Voiding Habits among Children with Down Syndrome.

PURPOSE: Children with Down syndrome are at risk for lower urinary tract dysfunction and delayed toilet training. Comparative studies regarding voiding function in the Down syndrome population are lacking. We assessed urinary continence and voiding function in patients with Down syndrome and a control group. MATERIALS AND METHODS: A questionnaire designed to assess toilet training, continence status, symptoms of lower urinary tract dysfunction and prior evaluation of urological complaints was sent to parents of 326 children with Down syndrome who had been seen at our institution previously. The same survey was administered to parents of patients without Down syndrome. Data were compiled, and descriptive and comparative statistical analyses were performed. RESULTS: A total of 77 patients comprised the Down syndrome group and 78 patients without Down syndrome comprised the control group. Average age of reported toilet training completion was 5.5 years in children with Down syndrome and 2.2 years in controls. Of children 5 years or older 79% with Down syndrome were toilet trained, compared to 100% of those without Down syndrome. Incontinence was reported in 46% of previously toilet trained children with Down syndrome and 24.5% of controls. These findings were statistically significant. No significant difference was observed in the rate of urinary tract infection, symptoms of lower urinary tract dysfunction or evaluation for urological complaints. CONCLUSIONS: Children with Down syndrome can experience marked delay in toilet training and are more likely to suffer incontinence afterward. This study was ineffective in determining whether symptoms of lower urinary tract dysfunction could be related to decreased continence rates.

Multimodal management of a pediatric cervical yolk sac tumor.

Even though vaginal bleeding is an unusual clinical presentation in infants and young children, thorough evaluation by the pediatric urologist requires the recognition and knowledge of less-common conditions, including malignancy. Extragonadal germ cell tumors are rare in children aged <15 years, representing approximately 1% of all cancers. Because of the close collaboration between pediatric oncologists and pediatric urologists, a multidisciplinary approach to the management and treatment of these tumors includes chemotherapy and surgical resection, aiming for fertility preservation when possible. We present a 10-month-old infant with a cervical or uterine germ cell tumor and the challenges found during her treatment.

The role of laparoscopic nephrectomy in pediatric xanthogranulomatous pyelonephritis: a case report.

Xanthogranulomatous pyelonephritis (XGP) is a rare chronic renal infection characterized by the destruction of renal parenchyma. Traditional treatment involves open radical nephrectomy, which is challenging due to the inflammatory process associated with XGP. More recently, laparoscopic nephrectomy has been employed successfully in adult XGP. We present a case of a six-year-old female child with XGP who was successfully treated by laparoscopic nephrectomy with minor complications. Our case demonstrates the safety and feasibility of laparoscopic nephrectomy for childhood XGP, indicating that it should be considered a management option in such cases.

Projected financial impact of noncoverage of elective circumcision by Louisiana medicaid in boys 0 to 5 years old.

PURPOSE: Several states, including Louisiana since 2005, no longer cover elective circumcision under Medicaid programs. The recent AAP (American Academy of Pediatrics) policy statement recognizes the medical benefits of circumcision and recommends the removal of financial barriers to this procedure. Cost savings are a factor in the limitation of circumcision coverage, although to our knowledge the actual cost savings to Medicaid programs have not been reported. We analyzed the number of circumcisions performed before and after the policy change to determine an accurate cost of such procedures and whether the increased procedure expense mitigates the initial savings. MATERIALS AND METHODS: We analyzed the number of neonatal and nonneonatal circumcisions in boys 0 to 5 years old to determine trends during the selected period. A cost model for each procedure was created. Neonatal procedure cost was based on professional fees. Nonneonatal procedure cost was based on professional (surgeon and anesthesia) plus facility fees. The number and cost of procedures were compared before (2002 to 2004) and after (2006 to 2010) the policy change. Linear regression was used to predict future costs. RESULTS: The average annual number and expense of neonatal circumcisions were significantly decreased after the policy change. There was no significant decrease in nonneonatal procedures and expense. Cost per procedure ranged from $88.34 for neonatal to $486.76 for nonneonatal circumcision. Secondary to the increasing number of more costly nonneonatal procedures, the annual expense was predicted to exceed pre-policy levels by 2015. CONCLUSIONS: The number of nonneonatal circumcisions is increasing and such procedures place a higher financial burden on the health care system. As a result, the financial benefits of noncoverage of elective circumcision are decreasing.

Salvage of bilateral asynchronous perinatal testicular torsion.

PURPOSE: Current management strategies for prenatal torsion include observation alone, delayed contralateral orchiopexy and emergent contralateral orchiopexy. Bilateral torsion is now being reported with more frequency and approximately a third of these patients have asynchronous torsion. We highlight the role of scrotal exploration in neonates diagnosed with bilateral asynchronous testicular torsion. MATERIALS AND METHODS: We reviewed the clinical experience from 2000 to present of 2 senior pediatric urologists to identify cases of bilateral perinatal testicular torsion. Asynchronous torsion was identified by the varying physical findings of each testicle at exploration. A literature review was done to evaluate the evolving management of perinatal testicular torsion. RESULTS: Six cases of bilateral perinatal torsion were identified since 2000. In 3 of the 6 cases extravaginal torsion of the contralateral testis was incidentally identified at surgical exploration. All 3 testes had normal Doppler flow before exploration. Testicular salvage was not successful in the 2 patients with bilaterally absent blood flow on newborn ultrasound. The 3 cases of incidentally diagnosed contralateral torsion and 1 of preoperatively diagnosed contralateral torsion were successfully salvaged. Thus, 4 of 6 patients with bilateral torsion were left with 1 viable testis. CONCLUSIONS: Physical examination findings and radiography can be inaccurate to assess the contralateral testis in males born with prenatal torsion. We have adopted the strategy of emergent surgical exploration in cases of prenatal torsion. Using this approach anorchia was minimized in patients with bilateral asynchronous torsion.

Bladder regeneration in a canine model using hyaluronic acid-poly(lactic-co-glycolic-acid) nanoparticle modified porcine small intestinal submucosa.

OBJECTIVE: • To determine if hyaluronic acid (HA) can be incorporated into porcine small intestinal submucosa (SIS) through poly (lactide-co-glycolide-acid) (PLGA) nanoparticles to improve the consistency of the naturally derived biomaterial and promote bladder tissue regeneration. METHODS: • Beagle dogs were subjected to 40% partial cystectomy followed by bladder augmentation with commercial SIS or HA-PLGA-modified SIS. • Urodynamic testing was performed before and after augmentation to assess bladder volume. • A scoring system was created to evaluate gross and histological presentations of regenerative bladders. RESULTS: • All dogs showed full-thickness bladder regeneration. • Histological assessment showed improved smooth muscle regeneration in the HA-PLGA-modified SIS group. • For both groups of dogs, urodynamics and graft measurements showed an approximate 40% reduction in bladder capacity and graft size from pre-augmentation to post-regeneration measurements. • Application of the scoring system and statistical analysis failed to show a significant difference between the groups. CONCLUSIONS: • SIS can be modified through the addition of HA-PLGA nanoparticles. The modified grafts showed evidence of improved smooth muscle regeneration on histological assessment, although this difference was not evident on a novel grading scale. • The volume loss and graft shrinkage experienced are consistent with previous models of SIS bladder regeneration at the 10-week time point. • Additional research into the delivery of HA and the long-term benefits of HA on bladder regeneration is needed to determine the full benefit of HA-PLGA-modified SIS. In addition, a more objective biochemical characterization will be needed to evaluate the quality of regeneration.

Timing of posterior urethral valve diagnosis and its impact on clinical outcome.

OBJECTIVE: Our goals were to assess long-term outcome for patients with posterior urethral valves (PUV) and determine the impact of the timing of diagnosis. PATIENTS AND METHODS: We reviewed the hospital records for patients with PUV treated in 1994-2008. Only those patients diagnosed, treated surgically, and followed clinically at our center were included. RESULTS: There were 52 patients with PUV. Thirty-nine were diagnosed by 1 year of age (early diagnosis cohort), while 13 were diagnosed after 1 year (late diagnosis cohort). Mean follow up after valve operation was 7.2 years (range 15 months-14 years). Chronic renal failure rates were not statistically different between the early and late diagnosis groups after surgical intervention: 48% (14/29) vs 25% (3/12), P = 0.30. Among the early diagnosis cases, 10% (3/29) eventually required renal transplant, while no child in the late diagnosis group has developed end-stage renal disease to date (P = 0.55). CONCLUSIONS: Gestational age at diagnosis is an important predictor of postnatal renal outcome. Our results suggest that diagnosis after 1 year of age is associated with a lower risk of developing renal insufficiency on long-term follow up.

Evaluation of need for salvage continence procedures after failed modern staged repair.

OBJECTIVES: To assess our experience with salvage continence procedures in patients with bladder exstrophy. METHODS: We retrospectively reviewed our surgical records of patients with bladder exstrophy who had undergone failed modern staged repair elsewhere and underwent salvage continence operations at our institute from 1996 to 2008. RESULTS: A total of 16 patients had undergone salvage continence operations. Bladder augmentation was performed in 11 patients (68.8%); 14 children (87.5%) received a continent, catheterizable stoma. In 6 children (37.5%) bladder neck injections were done. Three patients (18.8%) underwent bladder neck closure combined with other reconstructive procedures. A total of 49 continence procedures were performed, with an average of 3.06 per patient. We included all 16 children >5 years. After these operations, 13 of 16 (81.3%) became continent. Two children were able to void spontaneously and were dry day and night (12.5%). Of the remaining 14 patients, all of whom used clean intermittent catheterization, 11 (68.7%) were continent during the day and night with augmentation and/or a catheterizable stoma. Three patients were incontinent (18.7%). CONCLUSIONS: Although the need to perform salvage continence procedures after failed bladder modern staged repair is high, when patients are appropriately selected, continence can be achieved eventually.

Enhanced angiogenesis of modified porcine small intestinal submucosa with hyaluronic acid-poly(lactide-co-glycolide) nanoparticles: from fabrication to preclinical validation.

Hyaluronic acid-poly(de-co-glycolide) nanoparticles (HA-PLGA NPs) were synthesized to stabilize the porous structure of porcine small intestinal submucosa (SIS), to improve surface biocompatibility and to enhance performance in tissue regeneration. HA-PLGA NPs were characterized for size, zeta potential, surface morphology, and HA loading. Human microvascular endothelial cells responded to HA-PLGA NPs and HA-PLGA modified SIS (HA-PLGA-SIS) with elevated cell proliferation. HA-PLGA-SIS significantly enhanced neo-vascularization in an in ovo chorioallantoic membrane angiogenesis model. The angiogenic capability of the newly fabricated HA-PLGA-SIS was tested in a canine bladder augmentation model. Urinary bladder augmentation was performed in beagle dogs following hemi-cystectomy using HA-PLGA-SIS. The regenerated bladder was harvested at 10 weeks post augmentation and vascularization was evaluated using CD31 immunohistochemical staining. Bladder regenerated with HA-PLGA-SIS had significantly higher vascular ingrowth compared to unmodified SIS. This study shows that HA-PLGA NPs may represent a new approach for modifying naturally derived SIS biomaterials in regenerative medicine.

Urologic tissue engineering in pediatrics: from nanostructures to bladders.

The use of tissue engineering to develop new urinary bladder could have direct impact on children suffering from congenital bladder dysfunction. Current bladder tissue engineering protocols rely on scaffolds to support the ingrowth of new cells and the subsequent development of new tissues. The interaction between cells and scaffolds is mediated on the molecular level and nanotechnology is being investigated as a means to improve the biocompatibility of these scaffolds. In this review, we will discuss the current applications of nanotechnology for bladder tissue engineering; specifically, the use of selective degradation techniques and self-assembly molecules to create nanofeatures on existing scaffolds, the use of electrospinning to create de novo scaffolds, and the use of nanoparticles as a drug delivery system will be discussed. The use of nanotechnology has resulted in novel tissue engineering protocols and is likely to be an important factor in the eventual clinical application of tissue engineering for the urinary bladder.

Regional variations in small intestinal submucosa evoke differences in inflammation with subsequent impact on tissue regeneration in the rat bladder augmentation model.

OBJECTIVE: To examine the histological differences in the inflammatory response and regenerative outcomes of distal vs proximal porcine small intestinal submucosa (SIS) grafts in the rat bladder, as SIS from distal small intestine yields reliable and reproducible bladder regeneration, while SIS from proximal portions of small intestine does not provide similar results. MATERIALS AND METHODS: In all, 30 Sprague-Dawley rats underwent hemi-cystectomy followed by anastomosis of a bladder patch of SIS prepared from either distal or proximal small intestine. After bladder harvest, immunohistochemistry was used to quantify mast cells, eosinophils, macrophages, and neutrophils (PMNs). Total cell count per unit area was compared across the time course in univariate and logistic regression modelling. RESULTS: There were more eosinophils and mast cells in proximal SIS grafts, while there were more macrophages and PMNs in distal SIS grafts (all P < 0.05). Trichrome analysis showed increased collagen deposition in proximal SIS grafts and little smooth muscle regeneration. There was also significant graft contracture in proximal SIS grafts compared with distal SIS grafts (P < 0.05). CONCLUSIONS: We conclude that the location of SIS origin may evoke different inflammatory responses, which results in altered bladder tissue regeneration.

Application of a continent fecal reservoir in patients with cloacal exstrophy.

PURPOSE: Initial enteric diversion in cloacal exstrophy is achieved by ileostomy or end colostomy with formal reconstruction in the form of hindgut pull-through performed in select patients. Those who are not candidates for a pull-through procedure are often left with a permanent incontinent stoma. Additionally due to congenitally deficient intestinal length, some patients with cloacal exstrophy experience short bowel syndrome. We present our surgical technique and clinical experience in 2 patients who were successfully treated with a novel continent cutaneous fecal reservoir. MATERIALS AND METHODS: We devised a fecal reservoir in 2 patients with end ileostomies who were deemed poor candidates for pull-through of the hindgut segment. One of the patients exhibited short bowel syndrome before undergoing reconstruction. A continent cutaneous fecal reservoir was created from all available hindgut and a segment of ileum. A flap valve mechanism was used to create a continent catheterizable channel. RESULTS: Seven years postoperatively both patients are continent of stool and neither has experienced complications attributable to enteric diversion. The patient with short bowel syndrome demonstrated significant improvement in nutritional status as evidenced by rapid weight gain and improvement in serum albumin level. Both patients and their families are highly satisfied with the surgical outcome. CONCLUSIONS: Hindgut and ileum can be combined to create a novel continent fecal reservoir. As an alternative to diverting ileostomy or colostomy, continent diversion offers potential metabolic and social advantages. Select patients with cloacal exstrophy will benefit from this form of enteric reconstruction.

Successful removal of a penile constriction ring in a 14-year-old male.

Penile strangulation is a rarely described medical emergency, especially in the adolescent population. This case demonstrates the successful removal of a constricting metal ring from the penis of a 14-year-old male with a diamond blade equipped orthopedic oscillating saw while under ketamine anesthesia in the emergency department.

Leukocyte inflammatory response in a rat urinary bladder regeneration model using porcine small intestinal submucosa scaffold.

Small intestinal submucosa (SIS) is a biodegradable scaffold that supports bladder regeneration after partial cystectomy. We sought to define the inflammatory response present in a rat bladder augmentation model using distal ileal SIS. Fifteen Sprague-Dawley rats underwent hemi-cystectomy followed by anastomosis of a bladder patch of SIS. Bladders were excised after days 2, 7, 14, 28, and 56. Tissue regeneration was evaluated by standard hematoxylin and eosin. Immunohistochemical staining was used to quantify neutrophils, macrophages, eosinophils, and mast cells. Total cell counts per unit area were compared between native and graft portions of the bladder for each cell type across the entire time course. Statistical analyses were conducted with the Wilcoxon Rank-Sum and Kruskal-Wallis tests. All tests were two-sided with significance set at p < 0.05. These inflammatory responses evolved consistently across all bladders over time. Neutrophil and eosinophil numbers were highest at day 2 and decreased over the 56-day period. In contrast, macrophage and mast cell numbers were low at days 2, 7, and 14; peaked at day 28; and decreased once again at day 56. Cell counts at native and graft sites were equivalent for all cell types, except neutrophils, which were more commonly found in the graft (124 vs. 24 cells/mm(2), p = 0.009). Thus, the inflammatory processes change over time during SIS-mediated bladder regeneration. Similar cell densities are present between the native and SIS portions of the bladder, but neutrophils predominate in the graft areas.

Our initial experience with the technique of complete primary repair for bladder exstrophy.

OBJECTIVE: We reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures. PATIENTS AND METHODS: We performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center. RESULTS: Sixteen children were closed successfully. Six patients (37.5%) experienced complications: umbilical hernias in two, transient penopubic fistula in three, and subcoronal fistula due to meatal stenosis in one. Of the 12 males, seven (58.3%) were left with a hypospadias at the time of primary closure. Two (22.2%) children required a formal bladder neck reconstruction to achieve continence. Bladder augmentation and continent catheterizable stoma was performed in four cases (44.4%), and bladder neck injection in one case (11.1%). Bladder neck closure was also performed in another child following primary closure. Three of these children are continent and void spontaneously (33.3%). The remaining six require clean intermittent catheterization four to six times a day, resulting in four (44.4%) being continent. The number of continence procedures and mean number per patient were 15 and 1.66, respectively. CONCLUSION: Our early experience with this technique has been encouraging, with few major complications, a highly successful closure rate and a cosmetically normal result.

Body mass index as a predictive value for complications associated with reconstructive surgery in patients with myelodysplasia.

PURPOSE: Patients with myelodysplasia often have urological pathology, with 25% to 40% requiring reconstructive procedures to achieve urinary and/or fecal continence. Complication rates from these major reconstructive procedures range between 10% and 50%. Additionally many of these patients have significant comorbidities, including a nonambulatory status that leads to an increased body mass index. It is currently unknown whether a high body mass index is associated with increased surgical complications. In this study we compare body mass index and postoperative complications. MATERIALS AND METHODS: We retrospectively reviewed the charts of all patients with myelodysplasia undergoing urinary or fecal reconstructive procedures. We analyzed data for body mass index and any documented complication occurring during hospitalization or at any time during followup. Patients were categorized based on body mass index as normal weight (less than 85th percentile), overweight (85th to 95th percentile) or obese (greater than 95th percentile). Statistical analyses using chi-square and Fisher's exact tests were then performed. RESULTS: Reconstructive procedures were carried out in 66 patients with myelodysplasia between 1997 and 2005. A total of 48 bladder augmentations were performed with a total of 101 stomas created. Mean followup was 39 months. Height and weight were available for body mass index calculation in 60 patients. Obesity was common in our patients with myelodysplasia, affecting 33% of the population (20 of 60 patients). We found a total of 53 complications in 31 patients (52%). There was a significant association between presence of complications and weight category, with complications occurring in 40% of normal weight, 40% of overweight and 75% of obese patients (p = 0.0380). An association between stomal stenosis and weight category was also found (p = 0.0373). In addition, multiple complications were more prevalent in obese patients. Of the 15 patients (25%) with 2 or more complications 10 (67%) were obese (p = 0.0066). CONCLUSIONS: Patients with myelodysplasia have a high incidence of obesity. Since obesity is associated with a higher complication rate, weight loss programs are highly recommended for obese patients with myelodysplasia before and after any reconstructive surgery.

Recent advances in urologic tissue engineering.

Tissue engineering provides exciting therapeutic potential in urology. Urinary bladder regeneration is the most heavily researched aspect of tissue engineering in urology. Recent clinical research demonstrated the feasibility of bladder regeneration in children with neurogenic bladders. Despite this success, the ideal materials and methods for tissue engineering in urology have not been identified. We review the advances that have been made in tissue engineering in urology over the past 3 years. We particularly emphasize advances in our understanding of the raw materials and the methodology currently employed in urologic tissue engineering protocols.

Endoscopic injection of submucosal bulking agents for the management of incontinent catheterizable channels.

BACKGROUND: Urinary and fecal continence can be achieved by constructing catheterizable continent channels that provide access to the bladder and bowel. Some patients develop persistent stomal leakage. A minimally invasive method of injection with a bulking agent for treatment of stomal incontinence was evaluated. METHODS: A retrospective review identified patients with incontinence of their catheterizable continent urinary channel (CUC) and/or antegrade continence enema (ACE). All patients underwent circumferential endoscopic sub-mucosal injection of the channel with a bulking agent, performed at the level of the continence mechanism. The type of injected material, number of procedures required, and success rates were evaluated. RESULTS: Out of 157 patients with a CUC and/or ACE (total of 164 stomas), eight patients underwent the minimally invasive therapy (total of nine stomas). The initial reconstructive procedure was appendicovesicostomy in one patient, ileovesicostomy (Monti) in seven patients, and ACE in two patients. Amount of bulking agent injected varied from 1.4 to 7 cc (mean 3.72 cc). Follow up ranged from 1 to 39 months (median 15 months). Two patients received multiple injections. One patient had injection of both a CUC and ACE. At the time of final follow up, 6/7 (86%) patients with a CUC and 1/2 (50%) with an ACE were continent per catheterizable channel. CONCLUSION: Injection of a bulking agent provides an excellent minimally invasive treatment alternative for incontinence of a catheterizable channel.

Use of second look nephroscopy in children undergoing percutaneous nephrolithotomy.

PURPOSE: We determined the rate of stone clearance in children following percutaneous nephrolithotomy, endoscopic assessment of residual stone and the judicious use of second look nephroscopy. MATERIALS AND METHODS: We retrospectively reviewed the charts of all children undergoing percutaneous nephrolithotomy from 1996 to 2007. Cases were reviewed for pertinent details including preoperative and postoperative imaging, specifics of the procedure and followup. On completion of percutaneous nephrolithotomy the collecting system was assessed via combined nephrostogram and direct nephroscopy. If the collecting system could not be completely visualized or all stone fragments could not be cleared, the patient was referred for second look nephroscopy. Any residual stone present on initial followup imaging constituted a treatment failure. RESULTS: A total of 24 patients underwent percutaneous nephrolithotomy for 30 separate renal calculi. Mean patient age was 9.6 years, and mean stone burden was 2.53 cm. The success rate for percutaneous nephrolithotomy (inclusive of second look nephroscopy) was 87%. Of 30 cases 16 were managed by second look nephroscopy, with findings of residual fragments in all 16. Treatment failure was noted in 1 patient without second look nephroscopy and in 3 patients with second look nephroscopy. Of the 4 patients with treatment failure all were eventually rendered stone-free. CONCLUSIONS: Second look nephroscopy based on endoscopic findings during initial percutaneous nephrolithotomy is a reliable method of detecting and clearing residual stone fragments. Using such a protocol we achieved a high success rate of stone clearance in a population of patients with a significant stone burden.