Pediatric Bladder Tumors: A Ten-Year Retrospective Analysis.

OBJECTIVE: To present our experience in a single pediatric urology practice over a 10-year period with bladder tumors in the pediatric population in an effort to add to the relatively small amount of existing data. We hope to expand the community's knowledge of presentations, management and natural history of pediatric bladder tumors. METHODS: We retrospectively queried our electronic medical records for International Classification of Diseases, Tenth Revision (ICD-10) and Current Procedural Terminology (CPT) codes relevant for bladder tumors. Patients with underlying bladder pathology, such as neurogenic bladder, history of bladder exstrophy, and history of bladder augmentation, were excluded. RESULTS: We identified 30 patients with bladder tumors from 2011 to 2021. There were 21 males and 9 females. Age at diagnosis ranged from 16 months to 19 years. Tumors identified were: 11 of various inflammatory subtypes; 4 papillomas; 4 rhabdomyosarcomas; 3 papillary urothelial neoplasms of low malignant potential and 8 of other types. Treatment included transurethral resection of bladder tumor, chemoradiation and laparoscopic partial cystectomy. Twenty nine patients had disease limited to the bladder and 1 had disease outside the bladder. Follow-up ranged from 2 weeks to 13 years (median 19 months). All patients had no evidence of disease at most recent follow-up. CONCLUSION: Pediatric bladder tumors range from aggressive rhabdomyosarcomas to more benign urothelial lesions. Fortunately, the latter type of tumor is the more prevalent lesion. Knowledge of the treatment options and natural history of these tumors will hopefully be of benefit to clinicians and parents alike.

Degree of twisting and duration of symptoms are prognostic factors of testis salvage during episodes of testicular torsion.

BACKGROUND: Testicular torsion is surgical emergency. Prompt diagnosis and treatment of testicular torsion is essential for testicular viability. At surgical exploration, the spermatic cord is seen twisted a variable number of times around its longitudinal axis. There is scant data regarding the degree of twisting and its association with testis outcomes. The purpose of our study is to explore how the degree of torsion factors into testicular outcome using follow-up data. METHODS: We retrospectively reviewed the records of adolescent males who presented with testicular torsion to our institution, looking at duration of pain symptoms, degree of torsion documented in the operative note, procedure performed (orchiopexy versus orchiectomy), and follow-up clinic data for whether testicular atrophy after orchiopexy was present. A non-salvageable testis was defined as orchiectomy or atrophy. Receiver operator characteristics (ROC), multivariate, and logistic regression analyses were performed to determine the probability of a non-salvageable torsed testis based on time and degree of twisting. RESULTS: Eighty-one patients met our study criteria, with 55 testes deemed viable and 26 non-salvageable. We found a 25.7% atrophy rate after orchiopexy. Cut-off values of 8.5 h and 495 degrees of torsion would provide sensitivities of 73% and 53%, respectively, with specificity of 80% for both. Only duration and age were correlated with the risk of non-salvage on multivariate analysis. Logistic regression generated linear probability formulas of 4 + (3 ¡Á hours) and 7 + (0.05 ¡Á degrees) in calculating the probability of non-salvage with strong correlation. CONCLUSIONS: We were able to derive separate formulas to determine the viability of the torsed testis based on symptom duration and degrees of twisting. Fifteen h of symptoms and 860 degrees of torsion gives testes a 50% salvage rate. Interestingly, we also found that about 1 out of every 4 testes undergoes atrophy after orchiopexy.

Evaluating compensatory hypertrophy: a growth curve specific for solitary functioning kidneys.

PURPOSE: While compensatory hypertrophy is expected in solitary kidneys, the definition of appropriate hypertrophy remains unclear. The normal renal growth rate in children age 1 to 18 years with 2 kidneys has been defined as 0.28 × age (years) + 6.1. Solitary kidneys appear to grow faster and larger and, thus, require a separate growth curve. MATERIALS AND METHODS: The records of all patients 18 years old or younger with solitary functioning kidneys were reviewed from 2001 to 2011. Exclusion criteria were greater than SFU (Society for Fetal Urology) grade 2 hydronephrosis, posterior urethral valves, vesicoureteral reflux or any ipsilateral obstruction. Ordinary least squares regression modeled the renal length as a function of age by using only the initial sonogram per subject. The distribution of mean kidney length by age was plotted and compared to published normal values. RESULTS: A total of 91 subjects were included in the study. Patients were evenly split by laterality and gender. Multicystic dysplastic kidney comprised 34% and solitary kidneys 66% of subjects. Of these subjects 55% underwent their first sonogram at younger than 1 year old. There were 61 subjects with multiple sonograms but the initial 91 were included in the study. Age was a better predictor of renal length for subjects age 1 year or older (r(2) = 0.7312) than for those younger than 1 year old (r(2) = 0.6138). For children age 1 to 18 years we used the equation, length = 0.38 × age + 7.2. Solitary kidney values were approximately 2 standard deviations greater than normal values. CONCLUSIONS: The equation 0.4 × age (years) + 7 can be used to accurately estimate expected renal length in children (age 1 to 18 years) with solitary kidneys and can be used as a quick reference to evaluate for renal compensatory hypertrophy.

Familial testicular torsion.

PURPOSE: Isolated reported cases of familial torsion yield modest information on incidence, genetics or clinical features. We present what is to our knowledge the largest series of familial torsion, including the first 3 generation case and a review of the literature. MATERIALS AND METHODS: Since 2006, we have questioned the presence of a positive family history in all patients with torsion at the initial consultation. We compiled data on familial relationship, laterality, age and outcomes of the proband and affected relatives. We collected previously published cases to better understand clinical features and genetics. RESULTS: Eight of 70 boys (11.4%) with torsion had affected family members. Another 2 families were included from a historical perspective. One relative was affected in 7 families, 2 were affected in 2 and 3 were affected in 1. First degree relatives were most commonly affected. In 1 family torsion occurred in 3 consecutive generations. Despite a family history 50% of patients experienced testicular loss. Brothers were affected in each of the 10 previously reported cases. In 3 families fathers were also affected. There were 3 sets of monozygotic twins. We noted laterality concordance 5 times and discordance 6 times. Age at torsion in probands was adolescence except in 2 with neonatal torsion. No clear inheritance mode was found. CONCLUSIONS: Familial torsion occurs in about 10% of probands and can affect multiple relatives and generations. A positive family history may be useful for torsion diagnosis and management. Relatives of affected patients need education on the signs and symptoms of torsion, and the importance of early presentation to improve outcome.

Parenchymal echo texture predicts testicular salvage after torsion: potential impact on the need for emergent exploration.

PURPOSE: In the setting of signs and symptoms of testicular torsion the absence of diastolic flow and/or color flow on Doppler ultrasound has traditionally prompted emergent scrotal exploration. This practice emanates largely from the difficulty on ultrasound of distinguishing salvageable torsed testes from those that are not salvageable. We identified ultrasound findings predictive of testicular viability or the lack thereof. MATERIALS AND METHODS: We retrospectively reviewed the charts of all boys who underwent scrotal exploration for signs and symptoms of torsion during a 4-year period. In those who underwent preoperative Doppler ultrasound of the scrotum ultrasound findings were reviewed, as were the operative dictations. In patients who underwent orchiectomy the pathology reports were also reviewed. In patients in whom the torsed testis appeared viable and who underwent orchiopexy followup data were reviewed when available. Emergency room charts were also reviewed to ascertain, when documented, the duration of pain before presentation to the emergency room and the interval between ultrasound and operating room. RESULTS: During this period 55 boys underwent exploration after preoperative scrotal Doppler ultrasound revealed absent diastolic flow and/or color flow Doppler in the symptomatic testis. Assessment of parenchymal echogenicity revealed heterogeneity in 37 testes (67%), of which none were deemed viable at exploration. Orchiectomy was performed in 34 of 37 cases. Pathological examination revealed necrosis in all 34 cases, a finding consistent with late torsion. The remaining 3 testes underwent orchiopexy by parental directive despite nonviability, as confirmed by biopsy and subsequent atrophy. Thus, heterogeneity on preoperative ultrasound was universally predictive of organ loss (chi-square p <0.001). Of the 18 symptomatic testes (33%) demonstrating homogeneity and isoechogenicity on ultrasound 16 (89%) were deemed viable at exploration. Boys in whom the torsed testicle was nonviable on exploration experienced an average of 27.5 hours of pain preoperatively (range 5 to 72), whereas boys in whom the torsed testis was salvaged experienced an average of 20.5 hours of pain (range 2 to 96) (p = 0.073). The nonviable group underwent surgery an average of 49 minutes after ultrasound, whereas the viable group underwent surgery 52 minutes after ultrasound (p = 0.92). None of the 55 patients experienced any surgical or anesthetic complications and no pathological condition was noted intraoperatively in the contralateral asymptomatic testis. CONCLUSIONS: In the setting of Doppler proven testicular torsion heterogeneous parenchymal echo texture indicates late torsion and testicular nonviability. Therefore, the case may not require emergent scrotal exploration. On the other hand, homogeneous echo texture portends extremely well for testicular viability. Thus, such testes should be explored emergently.

Neonatal torsion: a 14-year experience and proposed algorithm for management.

PURPOSE: Management of neonatal torsion is controversial, since the likelihood of testicular salvage and metachronous contralateral torsion must be weighed against the risk of neonatal anesthesia. We reviewed a large series of such cases and stratified neonatal torsion based on time of presentation to determine the potential for testicular salvage. To our knowledge this is the largest series of its kind in the literature. MATERIALS AND METHODS: All cases of neonatal torsion were classified as either prenatal (noted at the time of delivery) or postnatal (noted after birth and before age 1 month). The charts of all patients were reviewed and data were collected on demographic information, pregnancy and birth history, laterality, physical examination findings, radiological imaging, intraoperative findings, anesthetic morbidities, perioperative complications and pathological diagnoses. Followup data were also collected for patients who underwent detorsion and orchiopexy. RESULTS: A total of 16 neonatal torsions (right side 8, left side 6, bilateral 1) were diagnosed in 15 patients at our institution between 1993 and 2007. A total of 13 torsions (81%) were prenatal and 3 (19%) were postnatal. All 13 prenatal torsions (100%) resulted in infarction (right 7, left 4, bilateral 2) confirmed by pathological examination. All patients underwent testicular exploration via an inguinal approach. A total of 11 cases were managed by orchiectomy at an average of 7.6 days (range 0 to 37) following birth. One of the bilaterally torsed testes showed infarction and necrosis on biopsy, and was detorsed and fixed in place. A second prenatally torsed testis was detorsed and pexed but atrophied on followup. Among the 3 postnatal torsions 1 (33%) was deemed viable on exploration and, therefore, salvaged. Of the 10 prenatal torsions with known prenatal history 5 (50%) were associated with at least 1 significant prenatal complication. Nine of the 10 patients with prenatal torsion (90%) were delivered vaginally, and 1 by cesarean section after prolonged failure of descent. CONCLUSIONS: Complicated pregnancies and vaginal deliveries seem to predispose patients to testicular torsion. Contrary to previous series, neonatal torsions do not appear to favor one side or the other. Prenatal torsions are never salvageable, and, therefore, do not warrant emergent intervention. Postnatal torsions are sometimes salvaged, and a judicious approach to surgical exploration should be taken.

Biofeedback therapy expedites the resolution of reflux in older children.

PURPOSE: In older children the spontaneous resolution rate of low grade vesicoureteral reflux is low and currently its management is controversial in regard to surgery versus prophylaxis versus observation alone. Bladder dysfunction in children with neurogenic bladders and to a less declarative degree in neurologically intact children has a role in the etiology or persistence of reflux. We determine the impact of biofeedback therapy on neurologically intact children with vesicoureteral reflux and detrusor-sphincter dyssynergia. MATERIALS AND METHODS: Vesicoureteral reflux was detected by voiding cystourethrography in children evaluated for urinary tract infections. Children with breakthrough infections or dysfunctional voiding based on history underwent uroflowmetry with concomitant patch electromyography of the external sphincter. Dyssynergia was defined as increased or steady electromyography activity during micturition. Biofeedback was initially performed weekly and the interval increased as indicated. The goals were to eliminate dyssynergia and reduce or eliminate post-void residual urine. Voiding cystourethrography was performed 1 year later to determine the status of the reflux. Ureteral reimplantation was performed during the period of biofeedback when indicated. RESULTS: From February 1997 to March 2001, 25 children 6 to 10 years old (mean age 9) with vesicoureteral reflux and detrusor-sphincter dyssynergia were treated with biofeedback therapy. There were 31 units (5 bilateral) with reflux, which was grade I in 10, II in 15, III in 5 and IV in 1. Children underwent an average of 7 sessions of biofeedback (range 2 to 20). On followup voiding cystourethrography, vesicoureteral reflux resolved in 17 units (55%), grade improved in 5 (16%) and reflux remained unchanged in 9 (29%). All cured vesicoureteral reflux was grade I (8 cases) or II (9). Four children (5 renal units) underwent reimplantation. In cured children there were no breakthrough infections during or since therapy and post-void residual urine decreased from an average of 40% before to 10% after therapy. Symptoms of urgency, daytime wetting and hoarding of urine improved or were eliminated in all children with resolved vesicoureteral reflux. CONCLUSIONS: Treating external detrusor-sphincter dyssynergia in older children with low grade vesicoureteral reflux, with biofeedback results in 1-year resolution rates that are considerably greater than historical resolution rates. External detrusor-sphincter dyssynergia should be screened for in children when surgery or discontinuation of chemoprophylaxis is considered so that biofeedback can be started.

The "long Snodgrass": applying the tubularized incised plate urethroplasty to penoscrotal hypospadias in 1-stage or 2-stage repairs.

PURPOSE: The technique of tubularized incised plate urethroplasty (Snodgrass modification) has gained wide acceptance for hypospadias repair. The reported experience with this surgical modification has been primarily in cases of distal hypospadias. We applied this technique to cases of penoscrotal hypospadias and incised the urethral plate for its entire distance. MATERIALS AND METHODS: In the last 24 months 21 boys 7 months to 8 years old with penoscrotal hypospadias were treated with a Thiersch-Duplay urethroplasty using the Snodgrass modification. The procedure was performed in 7 patients as a primary repair and in 14 at stage 2 of the 2-stage repair. The entire length of the urethral plate was incised along the midline in primary repairs, and the skin flaps and residual urethral plate were incised in the 2-stage repairs. The neourethra was tubularized over a 5 or 8Fr catheter. A layer of de-epithelialized tissue from the dorsal prepuce was used to cover the neourethra. No patient required skin flap to complete the urethroplasty. The urethral stent was removed in 7 to 10 days postoperatively. RESULTS: Of the 21 patients 19 (90%) required no other surgery as the repair provided a normal appearing penis (straight, terminal meatus, cosmetics) without complications such as meatal stenosis, fistula and diverticula, and voiding with a well directed full stream. One child had dehiscence of the glanular portion of the repair and 1 child had a pinpoint fistula, both of which were repaired successfully at a later date. Followup ranges from 5 to 12 months. CONCLUSIONS: The "long Snodgrass" modification to a Thiersch-Duplay repair is an effective technique for penoscrotal hypospadias as a single and 2-stage procedure. The success and complication rates are excellent in the short term. Longer term complications, such as strictures and diverticula, need to be assessed in the future.