Diffuse large B-cell lymphoma in an adolescent male presenting as ureteral stricture.

Lymphoma may affect the ureter in cases of retroperitoneal involvement. We present a case of an adolescent male found to have non-Hodgkin lymphoma initially presenting as ureteral stricture evident on imaging. He was treated and responded to multiagent chemotherapy with resolution of both the lymphoma and the ureteral stricture. Although rare, non-Hodgkin lymphoma should be included in the differential diagnosis of pediatric patients with noncalculous, idiopathic ureteral strictures.

Febrile urinary tract infections after ureteroneocystostomy and subureteral injection of dextranomer/hyaluronic acid for vesicoureteral reflux--do choice of procedure and success matter?

PURPOSE: Despite success rates favoring ureteroneocystostomy over subureteral injection of dextranomer/hyaluronic acid for correction of vesicoureteral reflux, the reported incidence of postoperative febrile urinary tract infection favors the latter. We evaluated contemporary treatment cohorts for an association between correction of vesicoureteral reflux and risk of postoperative febrile urinary tract infection. MATERIALS AND METHODS: We retrospectively reviewed the records of 396 consecutive patients who underwent ureteroneocystostomy or subureteral injection of dextranomer/hyaluronic acid between 1994 and 2008. Time to event multivariate analyses included preoperative grade of vesicoureteral reflux and bladder/bowel dysfunction. RESULTS: Of 316 patients meeting study criteria 210 underwent ureteroneocystostomy (356 ureters) and 106 underwent subureteral injection of dextranomer/hyaluronic acid (167). Median patient age was 5.7 years (IQR 3.4 to 8.3). Median followup was 28 months (IQR 8 to 61). Ureteral success was significantly greater after ureteroneocystostomy (88%, 314 of 356 cases) vs subureteral injection of dextranomer/hyaluronic acid (74%, 124 of 167, p = 0.0001). When controlling for preoperative grade of vesicoureteral reflux and bladder/bowel dysfunction, the risk of persistent reflux was 2.8 times greater after subureteral injection of dextranomer/hyaluronic acid (95% CI 1.7-4.7, p <0.0001). The incidence of febrile urinary tract infection did not significantly differ between ureteroneocystostomy (8%, 16 of 210 cases) and subureteral injection of dextranomer/hyaluronic acid (4%, 4 of 106; HR 1.96, 95% CI 0.64-5.9, p = 0.24) even when controlling for preoperative grade of vesicoureteral reflux, a predictor of postoperative febrile urinary tract infection on multivariate analysis (HR 2.2 per increase in grade, 95% CI 1.3-3.6, p = 0.0022). Persistent reflux was not a predictor of postoperative febrile urinary tract infection (HR 0.81, 95% CI 0.22-2.9, p = 0.75 for ureteroneocystostomy vs HR 1.8, 95% CI 0.2-17.3, p = 0.6 for subureteral injection of dextranomer/hyaluronic acid and HR 1.8, 95% CI 0.3-3.3, p = 0.6 for both). CONCLUSIONS: The incidence of postoperative febrile urinary tract infection may be independent of radiographic procedural success.

Vesicoureteral reflux in children with urachal anomalies.

OBJECTIVE: We report the largest known series of vesicoureteral reflux (VUR) in children with urachal anomalies (UA). METHODS: Two institutions' records were reviewed for children with UA (1951‒2007). RESULTS: Of 30 girls and 36 boys with UA (34 urachal cysts, 14 patent urachus, 10 urachal diverticula, 7 urachal sinuses, and 1 unknown), 57 (86%) underwent surgical resection or drainage. A voiding cystourethrogram was obtained in 22 (33%). VUR was demonstrated in 14 of the 22 children (64%), and rates were similar among the various types of UA. The median age with versus without VUR was not different (1.3 vs 1.7 years, P=0.97). Of 24 refluxing renal units, classification was grade≤3 in 71%, 4‒5 in 12%, and unspecified in 17%. Four children (26%) underwent ureteroneocystostomy and 10 observed patients resolved spontaneously. CONCLUSION: To our knowledge, this is the first series of VUR associated with UA. The increased incidence of VUR (64%) in this small subset of patients warrants prospective studies to determine if there is a positive correlation with UA. We believe thorough genitourinary and family histories are important when evaluating children with UA to help detect clinically significant VUR.

Precocious puberty in a 7-year-old boy: a novel case.

A 7-year-old boy was referred for evaluation of precocious puberty, evidenced by penile enlargement and pubic hair formation. His testicular size was prepubertal bilaterally. A comprehensive hormonal evaluation showed an elevated serum testosterone value (4.0 nmol/L) and a prepubertal gonadotropin value. A 0.9-cm heterogenous left testicular mass was detected on scrotal ultrasonography. Inguinal exploration was performed with ultrasound-guided open testicular biopsy and orchiectomy. Pathologic evaluation of the orchiectomy specimen showed the unclassified type of a mixed germ cell sex cord stromal tumor (MGCSCST), composed of neoplastic Sertoli cells and seminoma-like germ cells. Isolated previous reports of unclassified MGCSCSTs of the testis are now thought to be reports of sex cord stromal tumors with entrapped non-neoplastic germ cells. In our patient, the germ cells appeared to be neoplastic with aberrant expression of c-kit and placental alkaline phosphatase, a high proliferative rate, and DNA aneuploidy. Postoperatively, the patient's serum testosterone concentrations returned to prepubertal values (<0.2 nmol/L) and puberty was halted. This case represents a novel cause of precocious puberty.

Increasing prevalence and associated risk factors for methicillin resistant Staphylococcus aureus bacteriuria.

PURPOSE: Infections due to methicillin resistant Staphylococcus aureus are becoming increasingly prevalent in hospitals and in the community. We reviewed our institutional experience to determine whether methicillin resistant S. aureus is becoming a more common cause of bacteriuria and to determine if there are specific risk factors that may predict the development of methicillin resistant S. aureus bacteriuria. MATERIALS AND METHODS: We reviewed all urine cultures with a pure growth of a single organism obtained at our institution from 1997 and 2007. Patients with urine cultures positive for methicillin resistant S. aureus were compared to a cohort with cultures positive for methicillin sensitive S. aureus, and to a third cohort with cultures positive for Escherichia coli to determine patient characteristics and associated risk factors. RESULTS: We identified 7,100 and 9,985 positive urine cultures performed in 1997 and 2007, respectively. The most common urinary organism was E. coli. The number of patients with methicillin resistant S. aureus bacteriuria increased from 18 (0.3%) to 74 (0.8%) (p <0.001). On multivariate analysis older age (p = 0.004), catheter use (p = 0.004), hospital exposure (p <0.001) and patient comorbidity (p <0.001) were associated with methicillin resistant S. aureus bacteriuria compared with E. coli bacteriuria. CONCLUSIONS: Methicillin resistant S. aureus remains rare as a cause of bacteriuria but its incidence has increased during the last decade. Risk factors for methicillin resistant S. aureus bacteriuria include increased age, patient comorbidity, hospital exposure and catheter use. For patients with these risk factors and new onset urinary symptoms, methicillin resistant S. aureus should be considered a possible cause of urinary tract infection.

Superficial genitourinary abscesses in children: emergence of methicillin resistant Staphylococcus aureus.

PURPOSE: The prevalence of methicillin resistant Staphylococcus aureus is increasing. However, little is known about methicillin resistant S. aureus in the genitourinary tract, particularly in children. We assessed the incidence of pediatric genitourinary methicillin resistant S. aureus superficial abscess requiring surgical intervention. MATERIALS AND METHODS: We reviewed the records of all children undergoing surgical debridement of superficial abscess at a single institution between 1995 and 2007. We assessed surgical site, organism identity, patient comorbidity, methicillin resistant S. aureus risk factors, number of procedures and patient outcome. RESULTS: Surgical debridement of a superficial genitourinary abscess was performed in 60 children. Patient age ranged from 29 days to 17 years (median 3 years). A single debridement was generally curative, with only 5 patients (8.3%) requiring more than 1 procedure. One patient (1.7%) died of sepsis postoperatively due to Pseudomonas infection. One patient had myelomeningocele, 1 had undergone renal transplant and 2 were undergoing chemotherapy at the time of debridement. None of these 3 patients had a methicillin resistant S. aureus infection. Methicillin resistant S. aureus was more common in the groin/genitalia and less common in the perineum (p = 0.007). The incidence of methicillin resistant S. aureus increased during the study period, accounting for none of 40 infections between 1995 and 2003, and 8 of 20 (40%) from 2004 to 2007 (p <0.001). CONCLUSIONS: Methicillin resistant S. aureus has become the predominant organism causing pediatric superficial genitourinary abscesses at our institution, accounting for three-quarters of all surgically managed infections in the last 2 years. Methicillin resistant S. aureus was more common at the groin and genitalia. One debridement was generally curative, and patient morbidity was low with aggressive treatment.

Unilateral vesicoureteral reflux: does endoscopic injection based on the cystoscopic appearance of the ureteral orifice decrease the incidence of de-novo contralateral reflux?

OBJECTIVE: In patients with unilateral vesicoureteral reflux (VUR), it has been suggested that injection of a non-refluxing but cystoscopically abnormal contralateral ureteral orifice (UO) with dextranomer/hyaluronic acid (Dx/HA) should be performed to prevent the development of de-novo contralateral VUR. We evaluate the effectiveness of this practice. PATIENTS AND METHODS: Patients with primary unilateral VUR undergoing injection of Dx/HA from 2002 to 2005 at two institutions were eligible. Patients with unilateral VUR with cystoscopically abnormal contralateral UOs were injected with Dx/HA, while patients with normal appearing UOs received no treatment. Multivariate logistic regression models were used to estimate the impact of prophylactic injection on the development of de-novo contralateral VUR. RESULTS: In total, 101 patients with unilateral VUR and an abnormal appearing contralateral UO underwent prophylactic injection of Dx/HA while 45 patients with a normal appearing contralateral UO were untreated. In patients receiving prophylactic Dx/HA, 9% (9/101) of the previously non-refluxing ureters developed de-novo VUR. Similarly, 13% (6/45) of patients with a normal appearing UO treated by observation alone developed de-novo VUR (P=0.55). The overall incidence of 10% (15/146) de-novo contralateral VUR matches published results where this protocol was not followed. CONCLUSIONS: Our findings suggest that cystoscopic assessment and prophylactic treatment of an abnormal appearing, non-refluxing contralateral UO with Dx/HA is of little clinical benefit and should be abandoned.

B7-H1 expression in Wilms tumor: correlation with tumor biology and disease recurrence.

PURPOSE: Despite tremendous gains in improving prognosis, 10% of patients with Wilms tumor will ultimately experience disease recurrence. The identification of novel prognostic markers and tumor associated targets for patients at risk could enable clinicians to treat recurrences more aggressively and, thus, optimize outcomes. We have previously shown that tumor expression of the T cell coregulatory ligand B7-H1 portends a poor prognosis for adults with renal cell carcinoma and represents a promising target to improve therapy. We hypothesize that this finding may be true for Wilms tumor. MATERIALS AND METHODS: We identified 81 patients with Wilms tumor treated at 1 institution between 1968 and 2004. Histopathological features, including Wilms tumor B7-H1 expression, were correlated with clinical observations and outcome. RESULTS: Tumor recurrences were noted in 22% of patients with Wilms tumor and 14% died. B7-H1 was expressed in 11 tumors (14%) and was more likely to occur in anaplastic Wilms tumor (p = 0.03). Tumor B7-H1 expression was associated with a 2.7-fold increased risk of recurrence, although this difference did not achieve statistical significance (p = 0.06). However, in favorable histology tumors B7-H1 expression was associated with a 3.7-fold increased risk of recurrence (p = 0.03). CONCLUSIONS: B7-H1 is expressed by Wilms tumor, correlates with tumor biology and is associated with an increased risk of recurrence in patients with favorable histology tumors. B7-H1 may prove useful in identifying high risk patients who could benefit from more aggressive initial treatment regimens, and may represent a promising therapeutic target. Multi-institutional studies to elucidate the role of B7-H1 in the treatment of Wilms tumor are warranted.

Utility of dextranomer/hyaluronic acid injection in setting of bladder and ureteral anomalies.

OBJECTIVES: Previous studies have shown that the cure rates after dextranomer/hyaluronic acid (Dx/HA) injection can be decreased in patients with neurogenic bladder, previous ureteroneocystostomy, duplicated ureters, or periureteral diverticula. We attempted to determine whether these factors reduce the efficacy of Dx/HA injection compared with that in otherwise normal patients. METHODS: All children with vesicoureteral reflux (VUR) undergoing Dx/HA injection from April 2002 to March 2006 at two institutions were eligible for this study. Multivariate logistic regression models were built to assess the effect of bladder/ureteral anomalies on the success of Dx/HA injection. We adjusted for previously described predictors of injection success, including VUR grade, sex, age, surgeon experience, and injection technique. RESULTS: A total of 543 refluxing ureters (373 patients) were included, of which 145 (27%) had persistent VUR on postoperative voiding cystourethrography; 86 ureters (16%) had anatomic anomalies. On univariate analysis, the most important predictors of injection failure were increasing VUR grade, male sex, younger age, subureteral injection, ureteral duplication anomaly, increasing Dx/HA volume, and surgeon experience. On multivariate analysis, however, the only significant predictors of injection failure were increasing VUR grade, subureteral injection technique, and surgeon experience. No anatomic or functional abnormalities, considered individually or grouped, significantly affected the probability of injection failure. CONCLUSIONS: In our experience, children with functional and anatomic bladder/ureteral anomalies were no more likely to have Dx/HA injection fail than were children with uncomplicated VUR. The most important predictors of Dx/HA success remained VUR grade, injection technique, and surgeon experience. Dx/HA injection in patients with complex bladders could be a reasonable therapeutic option.

Multivariate comparison of the efficacy of intraureteral versus subtrigonal techniques of dextranomer/hyaluronic acid injection.

PURPOSE: Numerous factors have been postulated to increase success rates for dextranomer/hyaluronic acid injection for vesicoureteral reflux. Ureteral hydrodistention combined with intraureteral injection reportedly improves injection success rates. We combined the results of 5 pediatric urologists to evaluate the efficacy of this technique compared to that of subtrigonal-only injection in relation to other factors. MATERIALS AND METHODS: Patients with primary vesicoureteral reflux undergoing dextranomer/hyaluronic acid injection from April 2002 to December 2005 at 2 institutions were eligible. Only patients with primary vesicoureteral reflux were included in the study. Injection success was defined as the complete absence of reflux on followup voiding cystourethrogram or radionuclide cystogram. Predictors of a successful outcome were analyzed statistically with logistic regression. Factors included in our analysis were gender, age, vesicoureteral reflux grade, dysfunctional voiding, amount of injected dextranomer/hyaluronic acid, injection technique (intraureteral vs subureteral) and surgeon. RESULTS: A total of 301 patients (453 ureters) with a median age of 5.5 years met inclusion criteria, of whom 199 (66%) were cured at 3 months of followup. Of the patients 145 (48%) underwent subureteral injection and 156 (52%) underwent ureteral hydrodistention combined with intraureteral injection. On multivariate analysis only vesicoureteral reflux grade (p <0.001) and surgeon (p = 0.01) were significantly predictive of injection success. There was a trend toward significance with ureteral hydrodistention combined with intraureteral injection (p = 0.056). CONCLUSIONS: In our multivariate model only vesicoureteral reflux grade and surgeon were independently predictive of injection success in patients with primary, uncomplicated vesicoureteral reflux. There was a trend toward improved results with ureteral hydrodistention combined with intraureteral injection, although this did not achieve statistical significance.

Urachal anomalies: a longitudinal study of urachal remnants in children and adults.

PURPOSE: Urachal anomalies are often recognized in children but they may persist into adulthood and cause considerable morbidity. We reviewed the clinical findings associated with these embryological structures and evaluated risk factors for cancer to define the optimal timing for intervention. MATERIALS AND METHODS: We examined the medical records of 176 patients diagnosed with a urachal anomaly between 1951 and 2005. Association of clinicopathological features with malignancy for adults was evaluated using univariate and multivariate logistic regression studies. RESULTS: Urachal remnants were identified in 46 children and 130 adults. Of the 46 children 20 (43%) presented with umbilical drainage and 23 (50%) could be diagnosed by physical examination. A total of 34 children (74%) underwent simple excision. In contrast, 65 of the 130 adults (50%) presented with hematuria, 78 (60%) required cystoscopy and 53 (41%) required computerized tomography. A total of 66 adults (51%) required more extensive surgical intervention with partial or radical cystectomy. Of adult urachal anomalies 66 (51%) were malignant. Patient age 55 years or older and hematuria were the strongest predictors of urachal malignancy (each p <0.001). Cancer risk increased with advancing adult age. CONCLUSIONS: Urachal anomalies present and progress differently in pediatric and adult populations. Adults are at high risk for urachal cancer but children usually incur lower morbidity. Urachal lesions noted early in childhood should be excised to prevent problems in adulthood.

Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome.

PURPOSE: Testicular tumor of the adrenogenital syndrome is a rare clinical entity found in young men with endocrine disorders. Histologically it resembles Leydig cell tumor. We 1) reviewed the clinical features of testicular tumor of the adrenogenital syndrome and 2) determined if special histopathological features of the tumor and synaptophysin reactivity could distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor. MATERIALS AND METHODS: We reviewed the medical and pathological records for all patients with testicular tumor of the adrenogenital syndrome seen at our institution from 1978 to 2004. These tumors were examined by histological and immunophenotypic methods for comparison to Leydig cell tumor. RESULTS: A total of 14 males with an endocrine disorder had pathological evidence of testicular tumor of the adrenogenital syndrome. These tumors were often bilateral (93% or 13 of 14 cases), associated with pain (92% or 12 of 13) and refractory to medical management with high dose exogenous steroids (93% or 13 of 14). Testicular tumor of the adrenogenital syndrome was managed by tumor enucleation in 7 patients (54%) and by radical orchiectomy in 6 (46%). All patients had resolution of pain at 3-month followup. Upon histological review features found to be more common to testicular tumor of the adrenogenital syndrome compared with Leydig cell tumor were nuclear pleiomorphism, low mitotic activity, extensive fibrosis, lymphoid aggregates, adipose metaplasia and prominent lipochrome pigment. Synaptophysin (ICN, Costa Mesa, California) reactivity was strong in testicular tumor of the adrenogenital syndrome but rarely observed in Leydig cell tumor. CONCLUSIONS: In our series medical treatment failed in patients with testicular tumor of the adrenogenital syndrome and conservative surgical therapy was possible in select individuals. We identified special histopathological and immunophenotypic features, including synaptophysin staining, which distinguish testicular tumor of the adrenogenital syndrome from Leydig cell tumor.

Management of nephrolithiasis after Cohen cross-trigonal and Glenn-Anderson advancement ureteroneocystostomy.

PURPOSE: Ureteroneocystotomy is frequently performed for ureteral injury or vesicoureteral reflux. The Glenn-Anderson technique advances the ureteral orifice distal to its native position, while the Cohen technique crosses the orifice to the opposite trigone. Each treatment can alter access to the upper genitourinary tracts. We report our experience with subsequent nephrolithiasis in these patients. MATERIALS AND METHODS: We performed a retrospective chart review of all patients treated with ureteroneocystotomy since 1980 who had nephrolithiasis. RESULTS: Nephrolithiasis developed in 9 patients with prior Cohen ureteroneocystotomy and in 15 with prior Glenn-Anderson ureteroneocystotomy. Stones size was 2 to 20 mm (mean 6.4). In the Cohen group ureteroscopy was attempted and failed in 2 patients, requiring percutaneous nephrolithotomy. Attempted shock wave lithotripsy failed in 2 patients, of whom 1 required percutaneous nephrolithotomy and 1 required observation. Primary percutaneous nephrolithotomy was performed in 1 patient. One patient required nephrectomy for chronic pyelonephritis related to nephrolithiasis. Two patients had active stone disease and were awaiting further treatment, while 1 passed the stone. In the Glenn-Anderson group ureteroscopy was successful in all 4 attempts. Attempted shock wave lithotripsy in 2 patients was successful in 1. The other patient required subsequent percutaneous nephrolithotomy. Primary percutaneous nephrolithotomy was required in 2 patients. All other patients were asymptomatic and under observation. CONCLUSIONS: Treatment for upper tract nephrolithiasis is effected by prior ureteroneocystotomy. Minimally invasive treatments were less successful after Cohen ureteroneocystotomy than after Glenn-Anderson ureteroneocystotomy. In this study patients with prior cross-trigonal ureteroneocystotomy required more invasive therapies for symptomatic nephrolithiasis.

Clinical manifestations and functional outcomes in children with eosinophilic cystitis.

PURPOSE: Eosinophilic cystitis is a rare disorder, with fewer than 30 pediatric cases reported in the literature. We describe our experience with pediatric eosinophilic cystitis during a 20-year period. MATERIALS AND METHODS: Four children referred to our institution were subsequently diagnosed with eosinophilic cystitis between 1984 and 2004. A retrospective chart review was performed to assess clinical presentation, diagnosis, treatment and outcomes. RESULTS: Mean patient age at presentation was 10.8 years (range 5 to 18) and male-to-female ratio was 3:1. All 4 patients presented with irritative urinary symptoms, including 3 with dysuria and/or gross hematuria and 2 with urinary frequency, lower abdominal pain and/or a concomitant urinary tract infection. Allergic diseases (asthma, allergic rhinitis, etc) were present in 3 patients, and a formal allergen skin test was positive in 2 of those tested. A bladder mass mimicking malignancy was documented in 2 patients. Three patients had symptom resolution with conservative treatment, while 1 had development of an unremitting tumefactive process that eventually required partial cystectomy and bladder augmentation. CONCLUSIONS: Eosinophilic cystitis is a rare condition with a wide range of clinical manifestations. Children can present with a bladder mass mimicking sarcoma, underscoring the need for biopsy before diagnosis and treatment of a presumed oncological process. The condition usually follows a benign course, although unremitting progression remains a possibility.

Split-thickness skin graft for the management of concealed penis.

PURPOSE: We review the use of split-thickness skin grafting in children with concealed penis. MATERIALS AND METHODS: Medical records were retrospectively reviewed for all patients younger than 20 years seen at our institution from 1995 to 2003 with a diagnosis of concealed penis. Patients were separated into "primary" and "secondary" groups based on the cause of concealment. Primary factors were prominent prepubic fat pad, dysgenetic dartos fascia or both. Secondary factors were post-circumcision phimosis and overzealous circumcision. RESULTS: A total of 26 patients 1 month to 19 years old were treated. In the primary group of 23 patients 11 underwent lysis of dartos fascia. Four of these 11 patients had insufficient skin, and split-thickness skin grafting was necessary to resurface the penile shaft. Five of the patients underwent excision of the fat pad only, and 2 underwent excision of the fat pad and lysis of fascia. Five patients are being observed. Of the 3 patients in the secondary group 1 underwent manual reduction of post-circumcision phimosis, 1 underwent scrotal flaps and 1 is being observed. Followup ranged from 2 weeks to 46 months (mean 13 months). Of 20 surgically repaired patients 19 (95%) had an excellent cosmetic result, were satisfied with penile length and reported no voiding complaints. CONCLUSIONS: The surgical approach for correcting concealed penis varies, depending on the cause. Of our 26 patients 4 (15%) had insufficient penile skin to resurface the penile shaft. In these select children split-thickness skin grafting provided a good cosmetic appearance and functional result.