Bladder/prostate rhabdomyosarcoma: miles to go before we sleep.
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Child↗
Biomedical subjects
Howard M Snyder
Publications and source records attributed to Howard M Snyder.
Urethral dilation as treatment for neurogenic bladder.
PURPOSE: We documented the change in bladder physiology and clinical outcomes after urethral dilation for managing neurogenic bladder. MATERIALS AND METHODS: Female patients with neurogenic bladder were identified who underwent urethral dilation due to failure to empty at low pressure or upper tract changes. Urethral dilation was performed using curved sounds to 36Fr (range 18Fr to 36Fr). Clinical charts were reviewed to document urodynamics, infection rates, symptomatology, vesicoureteral reflux and upper tract dilatation before and after dilation. RESULTS: A total of 19 children underwent urethral dilation a mean of 2 times. Mean age at first dilation was 19.3 months. Mean followup was 34.9 months. The indication for dilation was high detrusor leak point pressure in 16 children. The remaining children had recurrent infections (1), poor compliance (1) and bladder spasms (1). Clinical manifestations of increased detrusor leak point pressure in 16 children were vesicoureteral reflux in 11, upper tract dilatation in 9 and recurrent infections in 3. Long-standing improvement occurred in 12 of 16 patients with increased detrusor leak point pressure, in 0 of 1 with infection, in 0 of 1 with poor compliance and in 1 of 1 with bladder spasms. Overall improvement was noted in 13 of 19 cases (68%). Vesicoureteral reflux was identified in 14 of 19 patients. Improvement was seen in 6 of 14 cases, including complete resolution in 5 and improvement in 1. Hydronephrosis was seen in 11 of 19 patients with improvement in 7, including resolution in 1 and improvement in 6. Only 6 children required further surgical intervention, that is vesicostomy in 2 and reimplantation in 4. CONCLUSIONS: Urethral dilation is effective for lowering detrusor leak point pressure in children with neurogenic bladder and for improving upper tract dilatation and vesicoureteral reflux. This procedure may prevent the need for more complicated surgical intervention. It should be investigated further in well designed, prospective studies.
Child, Preschool↗
Prenatally detected primary megaureter: a role for extended followup.
PURPOSE: A high rate of spontaneous resolution of or decrease in urinary tract dilatation is expected for most cases of primary megaureter (PM). We analyzed our results with extended long-term nonsurgical treatment in a group of children diagnosed with prenatally detected PM. We also sought to determine the long-term safety of this approach and the clinical significance of residual hydroureteronephrosis (HUN) when complete resolution does not occur. MATERIALS AND METHODS: We reviewed our experience with 40 infants with prenatally detected hydronephrosis diagnosed as PM who were initially treated nonoperatively between 1986 and 1999. Patients were followed with ultrasonography, nuclear renography and, in some instances, excretory urogram until stable improvement or complete resolution of HUN was noted. Records for patients who specifically returned for long-term followup history and radiological imaging were also reviewed. RESULTS: Of 40 patients with 57 megaureters (17 with bilateral PMs) who were followed 4 infants (10%) with severe HUN were diagnosed with diminished renal function by nuclear renography and underwent surgical repair. A total of 27 infants (67.5%, 40 megaureters) with PM who were followed without surgical intervention returned for followup examination and imaging for a minimum of 24 months after initial diagnosis, for a mean of 6.8 years (range 24 to 210 months). Ultrasonography in these patients revealed complete resolution (Society for Fetal Urology grade 1 or less hydronephrosis) in 21 megaureters (52.5%) at a mean of 2.9 years and improved or stable HUN in 19 megaureters (47.5%). Antibiotic prophylaxis was administered to 12 patients beyond the first month of life and discontinued at a mean age of 19.9 months. A subgroup of 10 patients (16 megaureters) from this cohort returned for extended followup imaging at a mean of 13.4 years (range 84 to 210 months) following initial diagnosis. Four patients with documented resolution of HUN had no recurrence during long-term followup. One male with bilateral PMs diagnosed at birth that had been stable but were not resolved at 8-year followup suffered significant worsening in the degree of HUN, diminished renal function on 1 side and a renal stone on the contralateral renal unit at 14-year followup, despite remaining asymptomatic. CONCLUSIONS: Long-term followup of children with prenatally diagnosed PM with mild to moderate hydronephrosis confirms a high incidence of resolution and improvement. As we have documented 1 case of worsening HUN at 14-year followup, we believe that ultrasonography should be periodically continued until the child reaches adulthood, at least in instances where complete resolution of HUN is not documented.
Adolescent↗
Testicular microlithiasis and antisperm antibodies following testicular biopsy in boys with cryptorchidism.
PURPOSE: Testicular biopsy (TBx) performed during orchiopexy in boys with cryptorchidism can help to predict future semen analyses and possibly identify patients at risk for testicular cancer. It has been theorized that TBx can be detrimental to the long-term health of the testis. We examined testicular microlithiasis (TM) and antisperm antibody (ASA) production in cryptorchid TBx. MATERIALS AND METHODS: A total of 112 males underwent fertility evaluation. These patients had previously undergone orchiopexy and bilateral TBx (mean age 8.6 years) for unilateral or bilateral undescended testis. At a mean age of 19.6 years all patients underwent physical examination and scrotal ultrasound for evaluation of testis size, echotexture and abnormalities. Of 112 patients 57 also underwent direct Immunobead(R) assay with positive controls. Moving sperm free and/or bound to IgG bead complex were counted in the preparation. RESULTS: A total of 29 patients had bilateral and 83 had unilateral undescended testis. Of the 112 patients 26 were black and 86 were white. Four black patients (15.4%) and 4 white patients (4.7%) had testicular microlithiasis. Three patients had bilateral and 5 had unilateral diffuse TM. No tunica albuginea scars or testis masses were noted. In the 57 patients who underwent direct Immunobead assay no semen sample demonstrated evidence of forming bead-sperm complex, ie none had direct ASA. CONCLUSIONS: No patient exhibited evidence of direct ASA. Prepubertal open TBx does not increase the rate of TM. We found no evidence of additive testicular damage associated with TBx at the time of orchiopexy.
Adolescent↗
Wilms' tumor management.
PURPOSE OF REVIEW: The management of Wilms' tumor continues to evolve with two different approaches being taken by the National Wilms Tumor Study in North America and the International Society of Pediatric Oncology in Europe in regards to preoperative chemotherapy. Limiting the duration, dosage, and number of chemotherapeutic agents and the dosage of radiotherapy are common goals in both trials. RECENT FINDINGS: Contralateral exploration of unilateral tumors will no longer be recommended in future National Wilms Tumor Studies. Percutaneous biopsy for tissue diagnosis is quite accurate, but there are concerning complications with its use. Partial nephrectomy is successful for low risk unilateral Wilms tumor, but its indications remain controversial. The surgical complication rate was similar between the National Wilms Tumor Study and the International Society of Pediatric Oncology, but intraoperative tumor spill was higher in the North American trials. Doxorubicin decreased the risk of recurrence in stage III tumors by 50%, and its current dose is not associated with late congestive heart failure. For selected patients, shorter courses of vincristine/dactinomycin or vincristine alone show equivalent results compared to current regimens. A longer course of chemotherapy (including doxorubicin) for clear cell sarcoma improves recurrence-free survival. Patients with Wilms' tumor, aniridia, major genitourinary malformations, and mental retardation, the WAGR syndrome, have a 50% chance of unexplained end-stage renal disease 20 years after treatment. SUMMARY: Less aggressive means of diagnosis and treatment for Wilms' tumor are continuing to achieve very good cure rates while lowering long term morbidity for low risk patients. High-risk patients with unfavorable histology or the WAGR syndrome benefit from more intensive treatment and long-term follow-up.
Antineoplastic Combined Chemotherapy Protocols↗
Pediatric urologic oncology: bladder, prostate, testis.
Although treatment for bladder, prostate, and testis cancer comprises a large part of adult urologic practice, the tumors that affect these organs in children are rare. Rhabdomyosarcoma,which affects the bladder, prostate, vaginal, and paratesticular areas,is treated with a combination of surgery, chemotherapy, and radiation. Most transitional cell carcinomas of the bladder and prepubertal testis tumors are managed surgically owing to the low stage at presentation. Application of the technical advances learned in adults with tumors of the bladder, prostate, and testis, combined with an understanding of the difference in tumor biology, helps urologists improve the treatment of these tumors in children.
Child↗
Experience with testis sparing surgery for testicular teratoma.
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.
Child↗
Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis.
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.
Adolescent↗
The island tube and island onlay hypospadias repairs offer excellent long-term outcomes: a 14-year followup.
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.
Child↗
Giant bladder diverticula causing bladder outlet obstruction in children.
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.
Child↗
The physical characteristics of young males with varicocele.
OBJECTIVE: To determine if there is an association with habitus in young males with varicocele, as adolescent boys with varicoceles appear to be mostly taller and leaner than age-matched controls. PATIENTS AND METHODS: Retrospectively reviewing our records we obtained the height and weight of 43 consecutive males (mean age 14.3 years, range 11-19) under long-term follow-up for varicocele. The body mass index (BMI), heights and weights were compared with values from the respective growth charts for boys aged 2-20 years (Center for Disease Control and Prevention), and the statistical significance of differences determined using the chi-square test. RESULTS: The height and weight distributions of patients with varicocele indicated a significant deviation from normal in the 25-95th percentiles for stature and in the 25-75th for weight (P < 0.05). Deviations in BMI were insignificantly different from normal at each percentile. CONCLUSION: These results indicate that patients with varicocele are significantly taller and heavier than age-matched controls. Future studies to address the key areas identified in this study will help to further assess the distribution of the incidence of varicocele in closely defined subsets of adolescent growth and development, which may provide some insight into the cause of varicoceles.
Adolescent↗
Molecular response of the bladder to obstruction.
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Animals↗
Spontaneous regression of cystic dysplasia of the testis.
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Child↗
Testis histopathology in boys with cryptorchidism correlates with future fertility potential.
PURPOSE: We studied the ability of testis biopsy to predict eventual fertility potential. MATERIALS AND METHODS: A total of 25 patients with unilateral undescended testis and 11 with bilateral undescended testes who previously underwent orchiopexy at a median age of 9 years were followed until after age 18 years. Semen analysis, testicular volume, follicle-stimulating hormone, luteinizing hormone, testosterone and inhibin B were compared to previously obtained biopsies of each testis. Biopsies were graded as mild, moderate or severe histology based on published data for normal and undescended testis histology. RESULTS: Patients with unilateral undescended testis and moderate histology were more likely to have a sperm density of greater than 20 million per cc than those with severe histology (p = 0.006), although no difference in hormonal parameters was noted. Patients with bilateral undescended testes and moderate histology were more likely to have normal follicle-stimulating hormone and inhibin B than those with severe histology (p = 0.05 and 0.002, respectively), although no difference in semen analysis was observed. Overall 7 of the 9 patients with bilateral undescended testes had less than 20 million sperm per cc. CONCLUSIONS: Testis biopsy provides useful prognostic information about semen analysis in patients with unilateral undescended testis. In the 9 patients with bilateral undescended testes testis biopsy divided them into normal and abnormal groups in terms of hormonal parameters. Testis biopsy was not able to provide additional prognostic information on semen analysis in patients with bilateral undescended testes since they were uniformly poor.
Adolescent↗
The ascending testis and the testis undescended since birth share the same histopathology.
PURPOSE: The etiology of the ascending testis is controversial. We propose that ascending testis, defined as a testis previously thought to be descended and later noted to be out of the scrotum, is due to mild hypogonadotropic hypogonadism affecting both testes. The diagnosis of these low types of true undescended testes is difficult to make clinically in children since they are frequently confused with retractile testes. In this study we compared testicular biopsies in a group of boys with ascending testes with those in boys who had an undescended testis since birth (primary undescended testis). MATERIALS AND METHODS: Between 1985 and 1995, 91 patients with ascending testes underwent orchiopexy and bilateral testis biopsy. The total germ cell count, processus vaginalis status, age at surgery and whether followup was done by a pediatrician or pediatric urologist were compared in patients with ascending and unilateral primary undescended testes. RESULTS: The total germ cell count was similar in the undescended and the contralateral descended testis in patients with ascending and primary undescended testes. The processus vaginalis was more likely to be closed in ascending testes (57% versus 36%, p = 0.0001). Age at surgery and the total germ cell count were similar in patients followed by pediatricians and pediatric urologists. CONCLUSIONS: The ascending testis has the same germ cell count as the primary undescended testis. Yearly followup by the primary care physician is recommended for patients with retractile testes.
Biopsy↗
Spontaneous resolution of vesicoureteral reflux: a 15-year perspective.
PURPOSE: The spontaneous resolution rate of vesicoureteral reflux is helpful for determining the need for surgical intervention and the proper followup schedule in patients on antibiotic prophylaxis. We determined the resolution rate by patient rather than by ureter and analyzed the effects of laterality, gender, age and dysfunctional voiding. MATERIALS AND METHODS: We retrospectively reviewed the records of 179 girls and 35 boys who presented between 1981 and 1984 with urinary tract infection and were diagnosed with primary vesicoureteral reflux. Mean age at presentation was 4.2 years and median followup was 3 years. Of the patients 107 (50%) had bilateral reflux and 60 had dysfunctional voiding. In 146 children (68%) reflux spontaneously resolved during the study. Patients were categorized by the worst grade of reflux, maintained on antibiotic prophylaxis and underwent voiding cystourethrography yearly until reflux resolved. Kaplan-Meier curves were constructed to define the resolution rate. RESULTS: Grades I to III reflux resolved at 13% yearly during the initial 5 years of followup and then at 3.5% yearly during subsequent followup. Grade IV to V reflux resolved at 5% rate yearly. Bilateral reflux resolved more slowly than unilateral reflux and it resolved more rapidly in boys than in girls. Untreated dysfunctional voiding had no effect on overall resolution. CONCLUSIONS: Grades I to III primary vesicoureteral reflux diagnosed after urinary tract infection resolve at identical rates and significantly more rapidly than grades IV to V. Early repair of grade IV to V reflux should be considered after age 18 months.
Adolescent↗