Need for botulinum toxin injection and bladder augmentation after isolated bladder outlet procedure in pediatric patients with myelomeningocele.

INTRODUCTION: In patients with neurogenic bladder outlet incompetence, a bladder outlet procedure (BOP) may be required to achieve urinary continence. However, when performed in isolation, a BOP can be associated with bladder deterioration and upper-tract injury. In the event of bladder deterioration, additional procedures such as bladder augmentation (BA) or botulinum toxin injection (BTI) may be pursued. OBJECTIVE: The aim of this study was to assess long-term outcomes after isolated BOP in a pediatric myelomeningocele (MMC) population, including the need for additional surgical intervention in the form of BTI or BA. MATERIALS AND METHODS: A retrospective cohort study was performed for patients with MMC who underwent an isolated BOP between 2004 and 2017. Primary outcomes included the need for postoperative BTI or BA. Secondary outcomes included the association between preoperative urodynamic parameters and need for BTI or BA. RESULTS: BTI or BA was performed in 18 of 36 (50%) patients at a median of 17.8 months (IQR 11.2-29.3) after an isolated BOP. A median of 1 (IQR 1-3) BTI was performed in 11 (30.6%) patients. BA was performed in 9 (25%) patients, including 2 patients who previously underwent BTI. Patients who did not undergo BTI or BA after BOP had a slightly increased percentage estimated bladder capacity at the end of follow-up (107% versus 95%, p=0.42). By contrast, patients who underwent BTI or BA had a post-BOP percentage estimated bladder capacity that decreased from 112 to 70% (p < 0.001), increased maximum detrusor leak point pressure from 43 to 67 cm H2O (p = 0.01), and higher rate of de novo upper-tract changes. Unfortunately, no preoperative clinical, radiographic, or urodynamic factors predicted the need for BTI or BA. DISCUSSION: On time-to-event analysis, the risk of BTI or BA was 53% at 5 years in our cohort. Risk of these procedures was highest in the first two years after BOP. 9 of 11 (82%) patients who underwent BTI had improvement in bladder dynamics and BA was not pursued. These findings suggest that BTI provides a less-morbid alternative to BA in patients with MMC and de novo adverse bladder storage changes after an isolated BOP. CONCLUSION: The need for BTI or BA after an isolated BOP is significant in patients with MMC. BTI offers a less-invasive alternative to BA in this population.

Epididymitis in patients with anorectal malformations: a cause for urologic concern.

INTRODUCTION: Epididymitis in patients with anorectal malformation (ARM) represents a unique problem because unlike the general population, an underlying urinary tract problem is frequently identified. We review our experience with epididymitis in ARM population with an emphasis on examining urologic outcomes. MATERIALS AND METHODS: We performed a retrospective review of male patients with ARM cared for from 1980 to 2010. Clinical and pathologic variables recorded included age at presentation, recurrence, associated urologic anomalies, incidence of ureteral fusion with mesonephric ductal structures, glomerular filtration rate and urodynamic parameters. RESULTS: Twenty-six patients were identified with documented episodes of epididymitis. Renal injury was noted in five patients (19%), all of whom were diagnosed with neurogenic bladder (NGB) several years after anorectoplasty. NGB was found in ten patients (38%) in our series. Ectopic insertion of ureter into a mesonephric ductal structure was discovered in five patients (19%). Twelve patients (46%) had recurrent episodes of epididymitis, with seven of these patients (58%) being diagnosed with NGB. Two patients in the pubertal group presented with a history of epididymitis and complained of ejaculatory pain. CONCLUSION: Epididymitis in a patient with ARM warrants a comprehensive urologic investigation, particularly in recurrent episodes. Attempts at surgical intervention (e.g. vasectomy) should be avoided until functional assessment of the urinary tract has occurred. Failure to recognize this association may lead to potentially avoidable complications and morbidity. Long term urological follow up of these patients is warranted to identify at risk patients and minimize renal deterioration.

Epididymitis in Patients with Anorectal Malformations: A Cause for Urologic Concern

Introduction Epididymitis in patients with anorectal malformation (ARM) represents a unique problem because unlike the general population, an underlying urinary tract problem is frequently identified. We review our experience with epididymitis in ARM population with an emphasis on examining urologic outcomes. Materials and Methods We performed a retrospective review of male patients with ARM cared for from 1980 to 2010. Clinical and pathologic variables recorded included age at presentation, recurrence, associated urologic anomalies, incidence of ureteral fusion with mesonephric ductal structures, glomerular filtration rate and urodynamic parameters. Results Twenty-six patients were identified with documented episodes of epididymitis. Renal injury was noted in five patients (19%), all of whom were diagnosed with neurogenic bladder (NGB) several years after anorectoplasty. NGB was found in ten patients (38%) in our series. Ectopic insertion of ureter into a mesonephric ductal structure was discovered in five patients (19%). Twelve patients (46%) had recurrent episodes of epididymitis, with seven of these patients (58%) being diagnosed with NGB. Two patients in the pubertal group presented with a history of epididymitis and complained of ejaculatory pain. Conclusion Epididymitis in a patient with ARM warrants a comprehensive urologic investigation, particularly in recurrent episodes. Attempts at surgical intervention (e.g. vasectomy) should be avoided until functional assessment of the urinary tract has occurred. Failure to recognize this association may lead to potentially avoidable complications and morbidity. Long term urological follow up of these patients is warranted to identify at risk patients and minimize renal deterioration .

The posterior sagittal approach for recurrent genitourinary pathology.

PURPOSE: The posterior sagittal transanorectal approach offers the surgeon excellent exposure to the lower genitourinary system. The approach has been successfully used for revisional surgery involving the urinary tract and rectum. We have used this approach to treat müllerian remnants, acquired or congenital fistulas and acquired urethral strictures. In the setting of revisional surgery abdominal or perineal approaches may not provide the same level of exposure to the lower urinary tract. MATERIALS AND METHODS: Of 911 males who underwent a posterior sagittal approach 12 were identified in whom this approach was used to manage lower urinary tract problems without associated anorectal malformation (group 1). An additional 58 patients with associated anorectal malformations were included in analysis (group 2). RESULTS: All cases were approached with a posterior sagittal incision to facilitate exposure after failed prior surgical attempts to treat traumatic strictures of the proximal urethra in 4 and müllerian remnants in 8 (group 1). In group 2 there were 19 urethral diverticula, 8 strictures and 31 persistent rectourethral fistulas. Followup was 6 months to 15 years. There were no recurrences identified in group 1. In group 2 there were 5 postoperative urethrocutaneous fistulas. Data on potency were available on 4 patients in group 1 and 14 in group 2. All 14 patients in group 2 reported intact potency after surgery. One patient in group 1 was impotent before and after surgery. CONCLUSIONS: The posterior sagittal approach is helpful for addressing lower urinary tract pathology when exposure is inadequate from a perineal or abdominal approach. We propose that better exposure with this approach may limit inadvertent injury to nerves and result in more complete treatment of the presenting pathological condition. This approach provides an alternative that warrants careful consideration when dealing with the lower genitourinary tract in pediatric patients, especially in the setting of revisional surgery.

Incontinence following bladder neck reconstruction--is there a role for endoscopic management?

PURPOSE: Little has been reported concerning the efficacy of endoscopic injection of dextranomer/hyaluronic acid for the treatment of residual incontinence following bladder neck reconstruction. We present the experience of 2 institutions using endoscopic submucosal injection of dextranomer/hyaluronic acid to correct incontinence in patients who had previously undergone bladder neck reconstruction with or without concomitant enterocystoplasty. MATERIALS AND METHODS: A retrospective chart review was performed with patient demographics, indications for treatment and outcomes recorded. All patients had adequate bladder capacity and compliance on maximized medical therapy before injection. Continence was defined as at least a 3-hour daytime dry interval, while improvement was defined as an increase in the daytime dry interval to at least 2 hours. RESULTS: A total of 14 patients (10 females and 4 males) underwent 21 injections. At a median followup of 17 months 10 patients had successful results (6 continent, 4 improved). CONCLUSIONS: Endoscopic injection of dextranomer/hyaluronic acid to correct incontinence following bladder neck reconstruction appears safe and can increase the daytime dry interval in more than 70% of carefully selected patients. Continued followup is necessary to evaluate the long-term effectiveness of this treatment.

The role of ureteroscopy in pediatric urology.

PURPOSE OF REVIEW: Endoscopic evaluation and management of the diverse conditions involving the upper urinary tract using rigid or flexible endoscopes is now readily feasible and has been shown to be safe and efficacious even in the smallest children. RECENT FINDINGS: Reduction in the size of the endoscopes, improvements in electronic imaging systems, proliferation of ancillary equipment, and improvement in endourologic skills among pediatric urologists make endoscopic treatment of pediatric urolithiasis the treatment of choice. The safety and efficacy of holmium:YAG laser lithotripsy make it the intracorporeal lithotriptor of choice. Successful outcomes can be obtained for ureteral and renal calculi that are similar to the adult population. Endoscopic treatment can be effective in highly selected children with intraluminal ureteral obstruction in the hands of a very experienced endoscopic pediatric urologist. These conditions include renal hemangiomas or arteriovenous malformations, ureteropelvic junction obstruction, ureteral strictures, or ureteral polyps. SUMMARY: Pediatric ureteroscopic procedures are similar to their adult counterparts, in that basic endoscopic principles should be observed. Nevertheless, children pose specific technical challenges that require planning before endoscopy and that affect the risks and outcomes of these procedures. Future improvements in ureteroscopy will rely on the continued application of the new technology.

Modulation of germ cell apoptosis with a nitric oxide synthase inhibitor in a murine model of congenital cryptorchidism.

PURPOSE: Apoptosis has been implicated in testicular germ cell loss in experimental models of cryptorchidism. Nitric oxide synthase (NOS) has been shown to have a role in apoptosis in many cell types. The Hoxa 11 knockout mouse has congenital bilateral cryptorchidism and is uniformly sterile. We examined the time course of apoptosis in this model and attempted to attenuate this response in vivo by inhibition of NOS. MATERIALS AND METHODS: The offspring of heterozygous Hoxa 11 knockout mice were genotyped by polymerase chain reaction. Homozygous knockout mice treated with the NOS inhibitor Nomega-nitro-L-arginine methyl ester (L-NAME) and untreated controls were sacrificed at weekly intervals at 3 to 13 weeks of age. Spermatogenesis was evaluated with hematoxylin and eosin staining. Germ cell apoptosis was assessed with a TUNEL assay and DNA staining. Co-localization of NOS activity was measured with a polyclonal antibody to endothelial NOS. RESULTS: Impaired spermatogenesis was observed in Hoxa 11 knockout mice. Testis/body weight ratios were decreased in this group at weeks 6 and 7, while body weights were unchanged. Germ cell apoptosis was significantly higher in the knockout group compared to wild-type controls. Co-localization was observed between endothelial NOS activity and apoptotic cells, while mice treated with L-NAME demonstrated improved spermatogenesis and attenuated apoptosis. CONCLUSIONS: Apoptosis and NOS reactivity appeared to co-localize in the seminiferous tubules in the Hoxa 11 knockout mouse model. Treatment with the NOS inhibitor L-NAME attenuated apoptosis and improved spermatogenesis. This finding suggests that early treatment might serve as an adjunct to early surgical intervention to reduce testicular atrophy, although any impact on long-term fertility remains to be determined.

Early orchiopexy restores fertility in the Hoxa 11 gene knockout mouse.

PURPOSE: We investigated whether infertility could be reversed in cryptorchid mice (with disrupted expression of the homeobox gene Hoxa 11) by orchiopexy and mating such animals with females of proven fertility. MATERIALS AND METHODS: Hoxa 11 mutant and WT male mice were genotyped by polymerase chain reaction. Surgery (orchiopexy or sham operation) was performed at age 18 days and fertility was assessed at ages 6 to 8 weeks. Animals were sacrificed at ages 6 to 9 months and computer assisted semen analysis was performed on fluid obtained by epididymal puncture. RESULTS: Five of 28 mutant mice proved fertile following orchiopexy versus 0 of 22 after sham operation (p <0.05). Values in WT mice were 18 of 35 and 25 of 33, respectively (p <0.01). Mean spermatozoa counts +/- SEM were 21.7 +/- 5.9 x 106/ml in 8 mutant mice with orchiopexy, 2.78 +/- 1.59 x 106/ml in 8 sham operated mutant mice (p <0.002), 15.6 +/- 4.9 x 106/ml in 7 WT mice with orchiopexy and 36.3 +/- 10.5 x 106/ml in 9 sham operated WT mice (p <0.02). CONCLUSIONS: Testicular position following orchiopexy is important to achieve fertility but the surgical procedure was associated with a degree of damage. Since mutant animals did not attain the fertility rates observed in WT animals following orchiopexy, other factors (possibly vaso-epididymal) may be necessary for normal spermatogenesis. Further studies of this model may allow the identification of such factors.

Metabolic evaluation of children with urolithiasis: are adult references for supersaturation appropriate?

PURPOSE: We determined the incidence of urinary stone risk factors in pediatric patients with urolithiasis. MATERIALS AND METHODS: Between 1998 and 2001, 71 children with urolithiasis at 2 pediatric institutions underwent metabolic evaluation. The 24-hour urine samples were analyzed outside central laboratory using adult and known pediatric references. Supersaturation and traditional metabolic parameters were determined and compared. RESULTS: All patients had metabolic abnormalities. Calcium related abnormalities were present in 92% of children, calcium oxalate supersaturation was abnormal in 69%, calcium phosphate supersaturation was elevated in 70% and traditional calcium parameters were abnormal in 80%. While 11% of the patients had abnormal calcium phosphate or oxalate supersaturation with normal traditional calcium parameters, 10% had normal calcium oxalate or phosphate supersaturation with abnormal traditional calcium parameters. Low urinary volume was identified in 75% of the children. CONCLUSIONS: Metabolic abnormalities are extremely common in pediatric patients with urolithiasis. Calcium related abnormalities are the most common abnormality. Urinary supersaturation values are complementary to traditional metabolic parameters and may be more sensitive predictors of recurrent stone risk. It is important to establish pediatric reference ranges to interpret these data more accurately.

The high intra-abdominal testis: technique and long-term success of laparoscopic testicular autotransplantation.

BACKGROUND AND PURPOSE: Treatment of the high intra-abdominal testis is often a challenging problem. We present our technique of and results with laparoscopically assisted testicular autotransplantation (LATA) for treatment of the high intra-abdominal testis. PATIENTS AND METHODS: We reviewed the medical records of 15 patients with a high intra-abdominal testis who underwent procedures between 1993 and 1999 in which intra-abdominal dissection and testicular mobilization was performed laparoscopically. The LATA technique is described. RESULTS: The 15 patients underwent 17 LATA procedures. Age at surgery ranged from 6 months to 13 years (mean 3.6 years; median 2.5 years). Two of these patients had undergone failed contralateral orchidopexy for an intra-abdominal testis, and two patients underwent metachronous bilateral procedures. The laparoscopic dissection and microvascular anastomoses were successful in all patients. There were no postoperative complications. The involved testis remained viable with adequate growth in 15 of 17 procedures (88%) during a median 42-month (range 7-70 months) follow-up. Successful procedures included those in the two patients with contralateral atrophic testes and the two who underwent bilateral procedures. CONCLUSIONS: The LATA demonstrated a good long-term surgical outcome and may be the technique of choice in patients with bilateral intra-abdominal testes or patients who have contralateral testis atrophy following unsuccessful orchidopexy.

Appendiceal versus ileal segment for antegrade continence enema.

PURPOSE: To assess outcomes after the antegrade continence enema procedure, we present our results with an ileal segment or the appendix in children with severe bowel dysfunction. MATERIALS AND METHODS: A retrospective review of 45 children who had undergone the creation of a continent cecostomy for severe chronic constipation and fecal incontinence was performed. RESULTS: The appendix was used to create the continent cecostomy in 28 patients (group 1) and ileum 17 (group 2). Of 16 patients who underwent simultaneous construction of appendiceal Mitrofanoff neourethra, including continent catheterizable stoma, the appendix was split and used for the cecostomy and neourethra in 11. Overall, acceptable continence was achieved in 39 (87%) patients and total continence 31 (69%). No significant difference was noted in the rate of continence between groups 1 and 2. Nonstomal postoperative complications occurred in 5 patients in group 1 and 3 group 2. Complications that required reoperation related to the continent cecostomy occurred in 10 patients, including stomal stenosis in 8, with 6 group 1 and 2 group 2 (p >0.05), and stricture in 2, with 1 group 1 and 1 group 2. There were 2 patients who had previously undergone colostomy for intractable constipation who were undiverted at the time of the creation of continent cecostomy. Both were continent postoperatively. There were 3 patients, including those 2 who presented with chronic severe constipation of unclear etiology, who underwent colostomy for unrecoverable colonic dys-motility, of whom 1 subsequently required total colectomy. CONCLUSIONS: The creation of a continent cecostomy for antegrade continence enema is a successful management option in children with debilitating fecal incontinence, and may enable undiversion of an existing colostomy. The appendix and ileal segment are viable options for the procedure, with no significant difference noted in continence or complication rates.

Extravesical common sheath detrusorrhaphy (ureteroneocystotomy) and reflux in duplicated collecting systems.

PURPOSE: Various surgical approaches have been used for treatment of vesicoureteral reflux in uncomplicated duplicated collecting systems. We retrospectively evaluated our success with extravesical common sheath detrusorrhaphy in such a setting. MATERIALS AND METHODS: Between 1990 and 1997, unilateral and bilateral extravesical nondismembered detrusorrhaphy was performed in 24 and 19 patients, respectively, with 51 duplicated collecting systems, including 8 bilateral, for management of vesicoureteral reflux. Vesicoureteral reflux was present in the moieties of ipsilateral duplicated kidney in 2 patients, and bilateral reimplantation was performed in 2 who had a history of contralateral vesicoureteral reflux that resolved. Patient age ranged from 1 to 16 years (median 3.67). Followup ranged from 36 to 114 months (median 86), and included cystourethrography and renal ultrasound. RESULTS: Vesicoureteral reflux resolved in all but 1 duplicated renal moiety, which was a success rate of 98%. In this patient the persistent reflux resolved by 16 months postoperatively on conservative treatment. There were no intraoperative complications and all patients resumed spontaneous voiding before discharge home. No postoperative obstruction of the involved kidney or de novo contralateral reflux developed in any patient. CONCLUSIONS: Common sheath ureteral reimplantation with extravesical detrusorrhaphy can be successfully used to treat vesicoureteral reflux in uncomplicated duplicated collecting systems. This approach demonstrates excellent long-term surgical outcome with minimal perioperative morbidity.