Joao L. Pippi Salle

Imaging of Ambiguous Genitalia: Classification and Diagnostic Approach

Disorders of sex development (DSDs) are congenital conditions in which the development of chromosomal, gonadal, or anatomic sex is atypical. DSDs can be classified broadly into four categories on the basis of gonadal histologic features: female pseudohermaphroditism (46,XX with two ovaries); male pseudohermaphroditism (46,XY with two testes); true hermaphroditism (ovotesticular DSD) (both ovarian and testicular tissues); and gonadal dysgenesis, either mixed (a testis and a streak gonad) or pure (bilateral streak gonads). Imaging plays an important role in demonstrating the anatomy and associated anomalies. Ultrasonography is the primary modality for demonstrating internal organs; genitography is used to assess the urethra, vagina, and any fistulas or complex tracts; and magnetic resonance imaging is used as an adjunct modality to assess for internal gonads and genitalia. Early and appropriate gender assignment is necessary for healthy physical and psychologic development of children with ambiguous genitalia. Gender assignment can be facilitated with a team approach that involves a pediatric endocrinologist, geneticist, urologist, psychiatrist, social worker, neonatologist, nurse, and radiologist, allowing timely diagnosis and proper management.

Pediatric Renal Cell Carcinoma: Single Institution 25-Year Case Series and Initial Experience With Partial Nephrectomy

PURPOSE RCC represents less than 2% to 6% of pediatric renal tumors. Few reports of long-term outcomes exist. We sought to determine the presentation, treatment and outcome of patients at our institution. MATERIALS AND METHODS We retrospectively reviewed the age, mode of presentation, mode of treatment, histological subtype, tumor grade, stage and survival of all patients with RCC from 1980 to 2005. RESULTS A total of 15 patients were identified. Mean age at presentation was 7.9 years. Symptomatic presentations in nearly 75% of patients included gross hematuria, abdominal pain and polycythemia. The remaining 25% of cases were asymptomatic, and were identified by physical examination or incidentally on imaging. Surgical resection consisted of radical nephrectomy in 10 patients and partial nephrectomy in 5. Pathological analysis revealed papillary RCC in 8 patients and clear cell RCC in 7. Six patients had high stage disease. One patient with stage IV disease died 8 months postoperatively. The remaining 14 patients were alive at a mean followup of 4.9 years. All but 1 patient remain recurrence-free, including all of those who underwent nephron sparing surgery. CONCLUSIONS To our knowledge this single institution series is the first to include children treated with partial nephrectomy. Pediatric patients with RCC tend to be older and more likely to present symptomatically compared to the typical patient with Wilms tumor. Hematuria and abdominal pain were the most common presentations, and papillary RCC was proportionately more common in this series. Our initial experience suggests that equivalent cure rates can be expected from a nephron sparing approach in appropriately selected cases.

Long-Term Followup and Time to Event Outcome Analysis of Continent Catheterizable Channels

PURPOSE Creation of a continent catheterizable channel has facilitated the treatment of patients undergoing lower urinary tract reconstruction. We present outcomes and complications of a single center series of continent catheterizable channels followed out to 15 years. MATERIALS AND METHODS We retrospectively reviewed medical records of all children who underwent continent catheterizable channel (Mitrofanoff and Monti) between 1992 and 2007. Collected data included age, underlying diagnosis, associated procedures, stoma site, conduit type (appendix or reconfigured bowel), time to complications and need for subsequent surgical revisions. RESULTS We identified 71 girls and 98 boys who underwent surgery at a mean age of 7.5 years (range 6 months to 22 years) and were subsequently followed for a mean of 5.8 years (8 months to 15 years). Underlying diagnoses included neurogenic bladder (36% of patients), bladder exstrophy (25%), epispadias (6%) and posterior urethral valves (6%). Concurrent procedures were conducted in 71% of cases, including augmentation (35%) and bladder neck plasty (22%) or closure (8%). Surgical revision was performed in 39% of patients, including stomal revision (18%), redo operation (8%), bulking agent injection (8%) and prolapse correction (4%). Although an initial peak was followed by a relatively stable complication-free period, delayed problems were detected on long-term followup. No statistically significant differences in complication rates were noted when comparing use of appendix and reconfigured bowel or different stoma locations. CONCLUSIONS Despite an initial decrease in complications soon after continent catheterizable channel creation, late problems appeared on long-term evaluation. In our experience no specific factor predicted the likelihood of complications. Nevertheless, despite the need for surgical revision, good functional outcomes were evidenced in this series.

Urethral Lengthening With Anterior Bladder Wall Flap (Pippi Salle Procedure): Modifications and Extended Indications of the Technique

PURPOSE We report a clinical case series of an innovative method of urethral reconstruction for the treatment of urinary incontinence. Modifications of our original technique are presented. MATERIALS AND METHODS Bladder neck repair was done in 17 patients, mean age 9.3, with neurogenic incontinence (13) or exstrophy (4). Average followup is 25.6 months. Of the patients 9 with neurogenic bladder underwent the original procedure using a midline anterior bladder wall flap. In the 4 patients with exstrophy a modified procedure was done using an anterolateral bladder wall flap. In another 4 patients an extended flap of distal mucosa was used to avoid ureteral reimplantation. Augmentation was performed in 13 of the 17 cases (10 detubularized ileum and 3 detubularized colon). RESULTS Continence (greater than 4 hours) was obtained in 12 of the 17 patients (70%), 2 are dry for 1 to 2 hours and 3 are incontinent. A urethrovesical fistula developed in 2 patients (1 closed successfully), and 3 patients have problems with catheterization. CONCLUSIONS Urethral lengthening with anterior bladder wall flap is a versatile alternative in the surgical treatment of urinary incontinence. Variations of the original technique resulted in an improved vascular supply and decreased the formation of fistula at the base of the flap. The modified technique was successful in patients with exstrophy, including those who had failed bladder neck surgery.

Urethral Lengthening with Anterior Bladder Wall Flap for Urinary Incontinence: A New Approach

Urethral irregularity (impeding catheterization) and failure to achieve continence are common complications in surgery for urinary incontinence. We describe a surgical technique using an anterior bladder wall flap that is sutured to the posterior wall in an onlay fashion creating a flap valve mechanism. Experimental work in dogs demonstrated a significant increase in the leak point pressure in the surgical group when compared to controls (p = 0.019). Voiding cystourethrography and bladder inspection demonstrated an anterior flap valve with no fistula formation in all animals. Histological examination showed a viable anterior bladder wall flap in all cases. This technique was then applied to 6 patients with neurogenic bladder and low urethral resistance that failed to resolve with medical treatment. Urinary continence was achieved in 4 patients. In 1 patient a vesicourethral fistula developed 3 months postoperatively, since the mother failed to catheterize for 12 hours. This technique is a useful alternative in the treatment of urinary incontinence.

Outcome Analysis and Cost Comparison Between Externalized Pyeloureteral and Standard Stents in 470 Consecutive Open Pyeloplasties

PURPOSE Despite the widespread use of ureteral stents for pyeloplasty by pediatric urologists there is ongoing controversy regarding the most advantageous type of transanastomotic drainage. We compared patients who underwent placement of an externalized pyeloureteral Salle intraoperative pyeloplasty stent (Cook Urological, Spencer, Indiana) to those who had a standard Double-J ureteral stent placed to assess the benefits, drawbacks and costs of each modality during open pyeloplasty. MATERIALS AND METHODS Our study sample comprised 470 age matched children who underwent primary open pyeloplasty in an 11-year period. A total of 242 patients (51.5%) underwent Double-J ureteral stent insertion and 228 (48.5%) underwent placement of a Salle intraoperative pyeloplasty stent at surgery. Operative time, hospital stay, overall complication and success rates, type of complications and hospital costs were compared between the 2 groups. RESULTS Median age was 18 months and median followup was 39 months. Mean hospital stay was 3.0 and 3.1 days in children with a Double-J ureteral and a Salle intraoperative pyeloplasty stent, respectively (p = 0.7). The overall complication rate was 9.9% (24 of 242 patients) for the Double-J ureteral stent vs 8.3% (19 of 228) for the Salle intraoperative pyeloplasty stent (p = 0.6). Complications in patients with a Double-J ureteral stent consisted of urinoma in 3, return visits due to bladder spasms in 7 or catheter obstruction in 6 and readmission due to pyelonephritis in 5. Complications in children with a Salle intraoperative pyeloplasty stent involved urinoma in 1, prolonged drainage through the Penrose drain in 5 and readmission due to pyelonephritis in 1. Recurrent ureteropelvic junction obstruction developed in 12 cases per group. The success rate was 95.0% (230 of 242 cases) and 94.7% (216 of 228) for the Double-J ureteral and the Salle intraoperative pyeloplasty stent, respectively (p = 0.2). Hospital charges, including the surgical procedure, postoperative hospitalization and cystoscopy or a clinical visit for catheter removal, in patients with a Double-J ureteral and a Salle intraoperative pyeloplasty stent were

Learning From the Learning Curve: Factors Associated With Successful Endoscopic Correction of Vesicoureteral Reflux Using Dextranomer/Hyaluronic Acid Copolymer

9,825 and

Timing and nature of reconstructive surgery for disorders of sex development - introduction.

9,260, respectively. CONCLUSIONS The 2 ureteral stents are equivalent in regard to overall complication and success rates after pyeloplasty. However, Salle intraoperative pyeloplasty stent insertion was associated with a Canadian

Ventral Penile Lengthening Versus Dorsal Plication for Severe Ventral Curvature in Children With Proximal Hypospadias

565 cost decrease per patient and most importantly the preclusion of a second general anesthesia for catheter removal.

Hypospadias in Males With Intrauterine Growth Restriction Due To Placental Insufficiency: The Placental Role in the Embryogenesis of Male External Genitalia

PURPOSE Conflicting reports exist regarding the parameters guiding successful correction of vesicoureteral reflux using dextranomer/hyaluronic acid copolymer. We performed logistic regression analysis to evaluate the effect of injected volume while adjusting for other factors potentially associated with success following dextranomer/hyaluronic acid copolymer injection. MATERIALS AND METHODS Between July 2003 and June 2006, 126 consecutive patients (34 boys and 92 girls) with a mean +/- SD age of 6.5 +/- 3.7 years with primary vesicoureteral reflux (196 refluxing ureters) underwent injection for febrile urinary tract infections. Success was defined as complete reflux resolution. Age, gender, laterality, preoperative vesicoureteral reflux grade, surgeon experience, dextranomer/hyaluronic acid copolymer volume, time to surgery from initial presentation and preoperative treatment for lower urinary tract symptoms were analyzed. RESULTS Vesicoureteral reflux grade was I to V in 7 (3.5%), 53 (27%), 91 (46.4%), 30 (15.3%) and 15 renal units (7.6%), respectively. The success rate after 1 injection was 50% by patient and 59.2% by ureter. Mean injected volume was 0.9 +/- 0.27 ml in those who had a successful injection vs 0.67 +/- 0.24 ml in those in whom injection failed (p <0.001). The success rate after 1 injection was 78.9% using 0.8 ml or greater dextranomer/hyaluronic acid copolymer compared to 31.7% with less than 0.8 ml. Multivariate analysis confirmed that higher dextranomer/hyaluronic acid copolymer volume (p = 0.001), lower preoperative grade (p = 0.013), surgeon experience (p = 0.025) and treatment for lower urinary tract symptoms (p = 0.009) were associated with successful correction of vesicoureteral reflux. CONCLUSIONS Our analysis strengthens the previously reported association of surgeon experience and vesicoureteral reflux grade with successful endoscopic vesicoureteral reflux correction. The data also revealed an association between injected volume and vesicoureteral reflux correction even while controlling for other variables, highlighting its importance as a true success modifier.