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Purpose: Tumor registries, urological textbooks and literature surveys all assert that yolk sac tumors are the most common primary testicular tumors in boys 12 years and younger. In contrast, several individual institutions have reported that benign tumors are more common than malignant tumors. To clarify these discordant findings, we surveyed the primary pathology records from 4 major pediatric centers., Materials and Methods: The pathology records of the contributing centers were culled for primary testicular masses in boys 12 years and younger. Older boys and those with either paratesticular tumors or leukemia were excluded. The prevalence of each histological subtype was calculated from the pooled cases., Results: A total of 98 patients met our criteria. Only 15% had yolk sac tumors. Teratomas comprised 48% of the tumors (mature 44%, immature 4%). Epidermoid cysts were found in another 14% of patients. Gonadal stromal cell tumors represented 13% of the total, divided among granulosa cell (5%), Leydig cell (4%), Sertoli cell (3%) and mixed gonadal stromal cell (1%). Other pathology, including cystic dysplasia (2), lymphoma (4), inflammatory pseudotumor (1) and gonadoblastoma (2), made up 9% of the total number of cases., Conclusions: We found that benign lesions represent the majority of primary testis tumors (74%), with the most common histological type being teratoma (48%). The reported high prevalence rates of prepubertal yolk sac tumors probably results from a reporting bias, since benign tumors are less likely to be submitted to tumor registries. Therefore, the primary operative approach to the majority of testis tumors in boys 12 years and younger should entail testis sparing surgery. Orchiectomy should be reserved for histologically confirmed malignancy based on increased preoperative alpha-fetoprotein and/or frozen section analysis of the tumor.

Purpose: Congenital giant diverticulum of the bladder is a consequence of deficiency in the detrusor musculature and has been reported in male infants as a rare cause of bladder outlet obstruction., Materials and Methods: A 10-year retrospective review revealed 4 patients (3 boys and 1 girl) with bladder outlet obstruction due to a giant bladder diverticulum. Prenatal and postnatal clinical and imaging records were reviewed., Results: Prenatal sonography was unremarkable in all patients. Three males (ages 4 months, 10 months and 3 years) had no medical history of voiding dysfunction, and 1 female (11 years) had the Ehlers-Danlos syndrome. While the girl presented with urinary tract infection, all patients presented with progressively decreasing urinary stream and urinary retention. Each patient underwent voiding cystourethrography (VCUG) and ultrasound. In each patient VCUG showed a giant (greater than 7 cm) bladder diverticulum that descended below the bladder neck and compressed the urethra during voiding. Vesicoureteral reflux was seen in 2 patients. Ultrasonography demonstrated moderate unilateral hydronephrosis in 2 patients. Bladder diverticulectomy was successfully performed in all patients, with ureteral reimplantation in 3., Conclusions: A giant congenital bladder diverticulum, when noted on VCUG to descend below the bladder neck, may lead to bladder outlet obstruction. To our knowledge we report the first case of a female presenting with bladder outlet obstruction due to a giant bladder diverticulum. Children with connective tissue disorders may be predisposed to this disorder, which must be excluded, regardless of gender, in all patients presenting with voiding abnormalities. Surgical diverticulectomy, often with ureteral reimplantation, is the preferred treatment, with excellent long-term results.

Prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD) implies a dire prognosis. Neonates affected by the more severe variants of ARPKD suffer respiratory failure caused by massive kidneys that restrict diaphragmatic expansion and result in pulmonary hypoplasia. Afflicted infants who survive the neonatal period and gain adequate respiratory function may subsequently suffer from an inability to tolerate enteral nutrition due to abdominal compression from the massive kidney and the systemic effects of renal compromise. Palliative unilateral or bilateral nephrectomy may be considered in rare instances to facilitate pulmonary expansion and gastrointestinal function. We report on an infant with severe ARPKD who was able to tolerate enteral nutrition only after left nephrectomy.

Purpose: We have used an inner preputial flap as a transverse island tube (IT) for the 1-stage repair of proximal hypospadias when the urethral plate could not be preserved and as an island onlay flap (IO) when the urethral plate could be preserved for more than 20 years at our institution. We report long-term followup and an outcome comparison of these 2 techniques., Materials and Methods: We retrospectively reviewed our records for all patients who underwent proximal hypospadias repair with either the IT or IO procedure between 1981 and 1992 by 1 surgeon (HMS). We randomly contacted these former patients to undergo a long-term followup examination, consisting of postoperative history, physical examination and uroflowmetry with post-void residual measurement. A review of patient charts was completed for perioperative complications or the need for secondary procedures., Results: Patient information could be retrieved for 73 of 125 patients who underwent penoscrotal or more severe hypospadias repair with either the IT or IO procedure during the defined interval. We were able to contact 49 of these former patients following a minimum of 10 years and 30 patients agreed to return for a long-term followup examination. The IT and IO repairs were performed on 14 and 16 boys, respectively, at a mean age of 16.8 months (range 8 to 74). At a mean followup of 14.2 years for both groups (range 144 to 253 months) 2 boys in the IT group (14.2%) and none in the IO group had a fistula requiring repair. Distal stenosis requiring meatoplasty occurred in 1 and 2 patients in the IT and IO groups, respectively. There were no urethral strictures and uroflowmetry was available for 11 patients in the IT group and 14 in the IO groups with mean maximal flow rates of 17.3 and 21.8 ml per second (p = 0.343) and mean post-void residuals of 5.0 and 2.36 ml per second, respectively (p = 0.249)., Conclusions: Unlike other forms of substitution urethroplasty, vascularized flaps based on preputial skin appear to be unique in that they do not have the long-term stricture rate seen with substitution urethroplasty using nongenital skin. The IT and IO repairs provided excellent long-term cosmetic and functional results. As the IT does have a higher incidence of postoperative complications, we have continued to extend our application of the IO to more proximal hypospadias repairs with continued success.

Purpose: The repair of penoscrotal or perineal hypospadias with severe chordee may require division of the urethral plate to facilitate repair. While a 2-stage approach to these difficult cases has been advocated, we have used a modified tubularized transverse preputial island flap (TPIF) repair as a 1-stage procedure. We compare our results with both procedures., Materials and Methods: Between 1997 and 2001, 1 surgeon (DAC) performed 22 penoscrotal or scrotal hypospadias repairs that required transection of the urethral plate. A TPIF or planned 2-stage repair was done in 12 and 10 boys, respectively. The TPIF was modified by suturing the medial edge of the island flap along the length of the corpora recreating a urethral plate and then tubularizing a neourethra. The 2-stage approach consisted first of mobilizing skin to the ventrum of the penis followed 6 to 12 months later by a urethroplasty. At followup cosmetic and functional results were assessed., Results: The TPIF and staged repair groups have been followed for a mean 24.5 and 43.5 months following the first repair, respectively. Of the 12 boys undergoing a TPIF 2 (16.7%) required a fistula repair or meatoplasty while 7 of 10 (70%) required at least 1 additional operation after the stage 2 urethroplasty (p <0.05). A mean of 1.6 additional repairs following the stage 2 urethroplasty was required. At last followup physical examination demonstrated a straight penis with distal meatus in 19 of 22 boys. Persistent complications include a concealed penis and recurrent fistula in 2 boys following a staged repair and glanular separation in 1 boy following TPIF., Conclusions: In our experience the staged approach to the repair of severe hypospadias requires significantly more cumulative surgical attempts than the modified TPIF. Indeed, in the majority of our cases the so-called "2-stage hypospadias repair" was a misnomer. The TPIF offers a reliable 1-stage repair. We prefer this approach to staged repairs even for severe hypospadias.

The importance of diet on the development and progression of prostate cancer was initially suggested by epidemiological studies. Since then, there has been a vast amount of research in this field. Compelling evidence now provides hope that evidence-based dietary alterations may markedly alter the natural history of this disease. Is there enough evidence for clinicians to be able to advise dietary modifications? The preliminary results no doubt are encouraging, but at present there seems to be no evidence to justify the widespread use of these proposed dietary interventions. However, as public awareness increases, all physicians involved with the care of patients with cancer of the prostate will need to be better armed with the current updates and advice on this issue.

Hypospadias results from abnormal development of the penis that leaves the urethral meatus proximal to its normal glanular position anywhere along the penile shaft,scrotum, or perineum. Hypospadiology is an evolving and expanding discipline that remains at the forefront of pediatric surgical innovation. Although modern experiments have begun to yield a deeper understanding of the genetic, hormonal, and environmental basis of hypospadias, the quest for a surgical procedure that consistently results in a straight penis with a normally placed glanular meatus has challenged surgeons for over two centuries. This article focuses on the advances in the understanding of the etiology of hypospadias and the current approaches to the correction of hypospadias.

Purpose: The thin filament associated protein h-caldesmon (h-CaD) modulates actin myosin interaction and contraction. Bladder outlet obstruction and detrusor hypertrophy are associated with the over expression of the nonmuscle CaD isoform l-CaD. It implies a poorly differentiated state of bladder myocytes and cytoskeletal remodeling in detrusor hypertrophy. We determined if h-CaD expression can be increased in a unique bladder smooth muscle (BSM) cell line derived from obstructed rabbit bladder smooth muscle that over expresses l-CaD. We examined whether the genetic restoration of h-caldesmon is possible in bladder smooth muscle cells by transfection or by agonist mediated contraction and whether this manipulation would alter cellular morphology., Materials and Methods: BSM cells were transfected with chicken h-CaD cDNA inserted into a mammalian vector. In another experiment BSM cells underwent intermittent bethanechol induced stimulation. h-CaD mRNA and protein were quantified with reverse transcriptase-polymerase chain reaction and Western blot analyses. Cell morphology was assessed using phase, video and confocal microscopy after double immunostaining with antibodies against alpha-actin and caldesmon., Results: Reverse transcriptase-polymerase chain reaction using primers specific for the transfected vector and h-CaD cDNA confirmed stable transfection of cells and increased content of h-CaD mRNA. Following bethanechol induced intermittent contraction Western blotting revealed 80% relative over expression of h-CaD in treated transfected cell lines (p <0.05) and 74% (not significant) in treated nontransfected controls. Confocal immunofluorescence microscopy revealed CaD in the cytoplasmic filaments co-localized to alpha-actin in the main cell body and perinuclear region in transfected cells, in contrast to the diffuse, irregular distribution of these filaments in control cells., Conclusions: A unique bladder myocyte cell line was successfully and stably transfected with h-CaD cDNA. We show that agonist induced intermittent contraction preferentially increases h-CaD expression, the predominant CaD in nonobstructed bladder smooth muscle, and the restoration of h-CaD alters cell morphology and the organization of cytoplasmic filaments in cells derived from obstructed rabbit detrusor musculature.

Purpose: Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis., Materials and Methods: We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years. We specifically reviewed our experience with JGCT in terms of presentation, surgical treatment and long-term outcome. We also reviewed the microscopic findings and histochemical techniques used to confirm the diagnosis., Results: We identified 77 tumors during the defined interval, of which 3 (3.9%) were JGCTs. All 3 patients with JGCT were first noted to have a testis mass soon after birth. All presented with a firm, unilateral testicular mass. Ultrasonographic findings were consistent with a complex, multiseptated, hypoechoic mass. Two of the 3 patients underwent radical orchiectomy. Testis sparing mass excision was performed in 1 patient. Grossly the tumors were partially cystic masses. Histologically positive immunostaining with inhibin-alpha and negative staining for alpha-fetoprotein (AFP) reliably differentiated JGCTs from yolk sac tumors. At a mean followup of 8.5 years (range 5 to 14) no metastases or local tumor recurrences have been diagnosed., Conclusions: To our knowledge we report the first case of testis sparing enucleation of a JGCT with a 5-year recurrence-free followup. Testis sparing enucleation is now our procedure of choice for tumors in neonates and prepubertal children with serum AFP in the normal range for age. JGCT should be suspected in neonates presenting at birth with a complex, cystic mass of the testis. Positive immunostaining for inhibin-alpha and a lack of AFP staining have consistently corroborated the pathological diagnosis in our experience and they should be applied for pediatric testis tumors that may mimic yolk sac tumor pathology.

Purpose: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery., Materials and Methods: We reviewed the pathology records at our institution for all testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We specifically examined the prepubertal incidence of teratoma, including epidermoid cysts, and our experience with testis preserving surgery. Preoperative and postoperative ultrasonography images were used to calculate the atrophy index following surgery. Patients were contacted for long-term followup., Results: Of 77 primary testicular and paratesticular tumors 38 were diagnosed in prepubertal boys (age younger than 13 years) including 11 mature teratomas and 5 epidermoid cysts. Mean patient age at treatment was 34.4 months (range 4 months to 10 years). All boys presented with a painless scrotal mass, cystic foci within an intratesticular mass on ultrasound and a normal alpha-fetoprotein level. Of the 16 boys with benign teratomas 13 (81%) were treated with a testis sparing procedure. At a mean 7-year followup no patient has presented with recurrent tumor in the ipsilateral or contralateral testicle. Postoperative physical examination and scrotal ultrasound were obtained in 9 patients at a median followup of 10.2 months, and there was no evidence of testicular atrophy or persistent discomfort., Conclusions: Unlike previously published series based on tumor registries, benign teratoma was the most common pediatric testicular tumor treated at our institution. Our single institution experience with testis preservation and long-term followup confirms the role and safety of this technique. Testis sparing surgery remains our technique of choice for testicular teratoma.

Objectives: To evaluate the clinical and urodynamic results of a tapered-cecal wrap (TCW) versus a tapered-plicated ileal (TPI) anti-incontinence mechanism., Methods: Of 54 consecutive patients who had undergone continent urinary diversions, 33 (17 with TCW and 16 with TPI) were evaluated. The primary disease that prompted diversion included bladder cancer (84%), neurogenic bladder (12%), and interstitial cystitis (3%). All patients were evaluated using a telephone questionnaire regarding ease of catheterization, degree of continence, occurrence of postoperative complications, and overall satisfaction in relation to their stoma. In addition, 6 patients in the TPI group and 5 in the TCW group underwent enterocystometry and outlet pressure recording. The mean follow-up was 30 months for the TCW group and 48 months for the TPI group., Results: The overall functional continence rate was 100% for the TCW group and 81.3% for the TPI group. Transient difficulty with catheterization occurred in 35.3% of the TCW group and 18.7% of the TPI group. No differences were observed in the occurrence of postoperative complications. Urodynamics demonstrated a statistically significant increase in maximal outlet pressure with the reservoir full in the TCW group that was not noted in the TPI group., Conclusions: The addition of a cecal wrap to the efferent limb results in significantly improved continence. This was supported urodynamically with demonstration of an increase in maximal outlet pressure with the reservoir full in the TCW group. No difference in the surgical complication rate or long-term difficulty with catheterization was observed.