Candace F. Granberg

Augmentation Cystoplasty and Risk of Neoplasia: Fact, Fiction and Controversy

PURPOSE We determined if ileal/colonic bladder augmentation performed in patients with congenital bladder abnormalities is an independent risk factor for bladder malignancy. MATERIALS AND METHODS We reviewed a registry of patients with bladder dysfunction due to neurological abnormalities, exstrophy and posterior urethral valves. Individuals treated with augmentation cystoplasty were matched (1:1) to a control group treated with intermittent catheterization based on etiology of bladder dysfunction, gender and age (±2 years). RESULTS We evaluated 153 patients with an ileal/colonic cystoplasty and a matched control population. There was no difference (p=0.54) in the incidence of bladder cancer in patients with augmentation cystoplasty (7 patients [4.6%]) vs controls (4 [2.6%]). In addition, there was no difference between the 2 groups regarding age at diagnosis (51 vs 49.5 years, p>0.7), stage (3.4 vs 3.8, p>0.5), mortality rate (5 of 7 [71%] vs 4 of 4 [100%], p>0.4) or median survival (18 vs 17 months, p>0.8). Irrespective of augmentation status patients with a history of renal transplant on chronic immunosuppression had a significantly higher incidence of bladder cancer (3 of 20 [15%]), compared to patients who were not immunosuppressed (8 of 286 [2.8%], p=0.03). CONCLUSIONS In patients with congenital bladder dysfunction ileal/colonic bladder augmentation does not appear to increase the risk of bladder malignancy over the inherent cancer risk associated with the underlying congenital abnormality. In addition, immunosuppression irrespective of bladder treatment is an independent risk factor for malignancy in this patient population.

Functional and oncological outcomes after orthotopic neobladder reconstruction in women

To review our experience with orthotopic neobladder reconstruction (ONR) in women, highlighting functional and oncological outcomes, as ONR has been used increasingly for urinary diversion in women after radical cystectomy (RC), largely due to a better understanding of the natural history of urothelial cell carcinoma (UCC) and of the anatomy underlying the female continence mechanism, but defining the safety and long‐term efficacy of ONR remain important to expanding its use.

Surgical Management, Complications, and Outcome of Radical Nephrectomy with Inferior Vena Cava Tumor Thrombectomy Facilitated by Vascular Bypass

OBJECTIVES To describe the technique, complications, and outcomes of vascular bypass during radical nephrectomy and tumor thrombectomy for patients with renal cell carcinoma and venous tumor thrombus. The indications and results for venovenous bypass (VVB) versus cardiopulmonary bypass (CPB) were reviewed as well. METHODS We identified 41 patients who had undergone radical nephrectomy and thrombectomy requiring VVB (n = 13) or CPB (n = 28) at our institution from 1970 to 2005 for renal cell carcinoma with venous tumor thrombus. The clinicopathologic variables and complication rates were compared between the VVB and CPB patients. The postoperative cancer-specific survival was estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS The patients undergoing VVB experienced significantly shorter median bypass times (P = 0.015), operative times (P <0.001), and anesthesia times (P <0.001) compared with those treated with CPB. In addition, VVB was associated with trends toward decreased median intraoperative blood loss (1200 mL versus 2725 mL, P = 0.336), decreased blood/blood products transfused (median 2300 mL versus 4275 mL, P = 0.256), and decreased length of hospitalization (median 7 days versus 9 days, P = 0.078). The 5-year cancer-specific survival rate was not significantly different for patients undergoing VVB (29.8%) versus those treated with CPB (36.4%; P = 0.989). CONCLUSIONS VVB was associated with significantly shorter bypass, operative, and anesthesia times, as well as trends toward decreased blood loss and hospital stay. Although the choice of bypass technique must be individualized according to the assessment of the bulk of thrombus to be resected, our results support the continued use of VVB in the management of renal cell carcinoma with extensive venous tumor thrombus, when appropriate.

Genetic Basis of Prune Belly Syndrome: Screening for HNF1β Gene

PURPOSE Although the cause of prune belly syndrome is unknown, familial evidence suggests a genetic component. Recently 2 nonfamilial cases of prune belly syndrome with chromosome 17q12 deletions encompassing the HNF1β gene have made this a candidate gene for prune belly syndrome. To date, there has been no large-scale screening of patients with prune belly syndrome for HNF1β mutations. We assessed the role of HNF1β in prune belly syndrome by screening for genomic mutations with functional characterization of any detected mutations. MATERIALS AND METHODS We studied patients with prune belly syndrome who were prospectively enrolled in our Pediatric Genitourinary DNA Repository since 2001. DNA from patient samples was amplified by polymerase chain reaction, sequenced for coding and splice regions of the HNF1β gene, and compared to control databases. We performed functional assay testing of the ability of mutant HNF1β to activate a luciferase construct with an HNF1β DNA binding site. RESULTS From 32 prune belly syndrome probands (30 males, 2 females) HNF1β sequencing detected a missense mutation (V61G) in 1 child with prune belly syndrome. Absent in control databases, V61G was previously reported in 2 patients without prune belly syndrome who had congenital genitourinary anomalies. Functional testing showed similar luciferase activity compared to wild-type HNF1β, suggesting the V61G substitution does not disturb HNF1β function. CONCLUSIONS One genomic HNF1β mutation was detected in 3% of patients with prune belly syndrome but found to be functionally normal. Thus, functionally significant HNF1β mutations are uncommon in prune belly syndrome, despite case reports of HNF1β deletions. Further genetic study is necessary, as identification of the genetic basis of prune belly syndrome may ultimately lead to prevention and improved treatments for this rare but severe syndrome.

Sacral Neuromodulation in Children With Dysfunctional Elimination Syndrome: Description of Incisionless First Stage and Second Stage Without Fluoroscopy

OBJECTIVES To detail a percutaneous technique of sacral nerve neuromodulation (SN) that eliminates the first-stage incisions and the need for second-stage fluoroscopy. Our group has previously described the results of SN in children with medically refractory dysfunctional elimination syndrome. The drawbacks to SN include the use of fluoroscopy and the need to reopen recent skin incisions during the second stage. This results in increased radiation exposure, poor cosmesis, and possible wound infection. METHODS The incisionless first stage consisted of percutaneously tunneling the temporary external appliance to the contralateral axillary line at the buttock after localization of the S3 nerve root and placement of a quadripolar tined lead under fluoroscopic guidance. A subcutaneous bolus of methylene blue marked the lead connector site, obviating the need for later fluoroscopic localization to place the implantable pulse generator at the second stage. RESULTS A total of 27 children with refractory dysfunctional elimination syndrome underwent SN using the InterStim device. Of the 27 patients, 19 underwent our modified technique. The operative time for our modified tunneling and placement technique was < or = 2 minutes. The mean hospital stay was 0.6 day, with no patient requiring postoperative intravenous narcotics. At a mean follow-up of 35.9 months, no wound infections had occurred in the incisionless cohort compared with 1 postoperative wound infection requiring device explantation in the conventional lead placement group. CONCLUSIONS The incisionless technique of SN device implantation is technically simple, quick to perform, and results in decreased radiation exposure, excellent pain control, and improved cosmesis without compromising the outcomes.

Tamsulosin and Spontaneous Passage of Ureteral Stones in Children: A Multi-Institutional Cohort Study

PURPOSE Tamsulosin is associated with increased passage of ureteral stones in adults but its effectiveness in children is uncertain. We determined the association between tamsulosin and the spontaneous passage of ureteral stones in children. MATERIALS AND METHODS We performed a multi-institutional retrospective cohort study of patients 18 years or younger who presented between 2007 and 2012 with ureteral stones up to 10 mm and who were treated with tamsulosin or oral analgesics alone. The outcome was spontaneous stone passage, defined as radiographic clearance and/or patient report of passage. Subjects prescribed tamsulosin were matched with subjects prescribed analgesics alone, using nearest neighbor propensity score matching to adjust for treatment selection. Conditional logistic regression models were used to estimate the association between tamsulosin and spontaneous passage of ureteral stones, adjusting for stone size and location. RESULTS Of 449 children with ureteral stones 334 were eligible for inclusion, and complete data were available for 274 patients from 4 institutions (99 receiving tamsulosin, 175 receiving analgesics alone). Following case matching, there were no differences in age, gender, weight, height, stone size or stone location between the 99 subjects prescribed tamsulosin and the 99 propensity score matched subjects prescribed analgesics alone. In the tamsulosin cohort 55% of ureteral stones passed, compared to 44% in the analgesics alone cohort (p=0.03). In multivariate analysis adjusting for stone size and location tamsulosin was associated with spontaneous passage of ureteral stones (OR 3.31, 95% CI 1.49-7.34). CONCLUSIONS The odds of spontaneous passage of ureteral stones were greater in children prescribed tamsulosin vs analgesics alone.

Contemporary review of testicular torsion: New concepts, emerging technologies and potential therapeutics

Testicular torsion is one of the few emergencies in pediatric urology which requires an accurate and timely diagnosis in order to avoid testis loss. It is not an uncommon event affecting a young male population. In fact, testicular torsion is more common than testicular tumors for this same age group, yet testicular torsion has not been given the public attention it deserves as a male health risk. In this review we highlight the new information published over the past four years regarding testicular torsion. We will discuss a variety of topics associated with torsion including: medical legal issues, etiology and genetics, imaging diagnostics, innovative surgical techniques, management controversies, fertility, and new drug therapies.

Objective use of testosterone reveals androgen insensitivity in patients with proximal hypospadias.

OBJECTIVE We report preoperative testosterone stimulation based on glans width measurements in patients with midshaft and proximal hypospadias, revealing androgen resistance in those with proximal hypospadias. METHODS Patients had maximum glans width measured preoperatively. Those <14 mm initially received 2 mg/kg testosterone cypionate intramuscularly for two to three doses, with the aim of increasing glans width ≥ 15 mm. Not all patients achieved targeted growth, and some were subsequently treated with escalating doses of testosterone. RESULTS 5/15 midshaft patients had two to three doses of 2 mg/kg testosterone, with all increasing glans width to ≥ 15 mm. 29/47 proximal patients had testosterone, with 13 (57%) not reaching desired glans width. Six of these and another six patients had escalating doses from 4 to 32 mg/kg testosterone, with 11 then achieving targeted glans width. Relative androgen resistance was found in 19/29 (66%) proximal cases, including all treated patients with perineal hypospadias. CONCLUSIONS 39/62 (63%) patients met objective criteria for preoperative testosterone stimulation based on glans width <14 mm, which is less than the average normal newborn glans diameter. Evidence of relative androgen resistance was found in 19 (49%), all with proximal hypospadias.

Oncologic outcomes of partial versus radical nephrectomy for unilateral Wilms tumor.

Radical nephrectomy (RN) is the recommended surgical management as part of multi‐modality therapy for unilateral Wilms tumor (UWT). Based on recent data demonstrating that renal preserving surgery decreases the likelihood of chronic renal disease and associated co‐morbidities, we analyzed oncologic outcomes of patients after partial nephrectomy (PN) for UWT.

Robot-assisted laparoscopic retroperitoneal lymph node dissection in an adolescent population.

Abstract Laparoscopic retroperitoneal lymph node dissection (RPLND) has been shown to be safe and effective in appropriately selected pediatric and adolescent patients with paratesticular rhabdomyosarcoma (RMS) and testicular germ-cell tumors (T-GCT). While the use of robot-assisted laparoscopy has expanded rapidly in many areas, there are very limited reports of its use with RPLND. We present two cases of adolescents who were treated using robot-assisted laparoscopic RPLND (R-RPLND)-one with paratesticular RMS (PT-RMS) and one with testicular GCT (T-GCT)-with good outcomes and low morbidity.