Validation of the modified Bosniak classification system to risk stratify pediatric cystic renal masses: An international, multi-site study from the pediatric urologic oncology working group of the societies for pediatric urology.

BACKGROUND: Pediatric cystic renal lesions are challenging to manage as little is known about their natural course. A modified Bosniak (mBosniak) classification system has been proposed for risk stratification in pediatric patients that takes ultrasound (US) and/or computed tomogram (CT) characteristics into account. However, literature validating this system remains limited. OBJECTIVE: To determine if the mBosniak classification system correlates with pathologic diagnoses. The hypothesis is that mBosniak classification can stratify the risk of malignancy in children with renal cysts. STUDY DESIGN: Patients treated for cystic renal masses with available imaging and pathology between 2000 and 2019 from five institutions were identified. Clinical characteristics and pathology were obtained retrospectively. Characteristics from the most recent US, CT, and/or magnetic resonance imaging (MRI) were recorded. Reviewers assigned a mBosniak classification to each scan. mBosniak scores 1/2 were considered low-risk and 3/4 high-risk. These groups were compared with pathology (classified as benign, intermediate, malignant). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated to assess this categorization as a screening tool to guide surgical intervention. Agreement between imaging modalities was also explored. RESULTS: 99 patients were identified. High-risk imaging findings were correlated with malignant or intermediate pathology with a sensitivity of 88.3%, specificity of 84.6%, PPV of 89.8%, NPV of 82.5%, +LR of 5.7, and -LR of 0.14. The sensitivity for detecting malignant lesions only was 100%. There was substantial agreement between US/CT (n = 55; κ = 0.66) and moderate agreement between US/MRI (n = 20; κ = 0.52) and CT/MRI (n = 13; κ = 0.47). DISCUSSION: The mBos classification system is a useful tool in predicting the likelihood of benign vs. intermediate or malignant pathology. The relatively high sensitivity and specificity of the system for prediction of high-risk lesions makes this classification applicable to clinical decision making. In addition, all malignant lesions were accurately identified as mBosniak 4 on imaging. This study adds substantial data to the relatively small body of literature validating the mBosniak system for risk stratifying pediatric cystic renal lesions. CONCLUSIONS: Pediatric cystic renal lesions assigned mBosniak class 1/2 are mostly benign, whereas class 3/4 lesions are likely intermediate or malignant pathology. We observed that the mBosniak system correctly identified pathology appropriate for surgical management in 88% of cases and did not miss malignant pathologies. There is substantial agreement between CT and US scans concerning mBos classification.

Surgical outcomes are equivalent after pure laparoscopic and robotic-assisted pyeloplasty for ureteropelvic junction obstruction.

INTRODUCTION: Minimally invasive (robotic and pure laparoscopic) pyeloplasty has been increasingly used for treatment of ureteropelvic junction obstruction (UPJO). However, few large-scale studies have compared these two modalities directly. METHODS: We performed a retrospective single-center review of all patients who underwent pure laparoscopic (LP) or robotic pyeloplasty (RALP) between 2007 and 2018. Patients were excluded if the initial surgery at our institution was a redo pyeloplasty or if they lacked follow-up information. Outcomes of interest included operative time, length of stay, and complication rates, including rates of secondary procedures. We compared these outcomes between groups using Student's t test for continuous variables and a Chi-square for categorical variables. RESULTS: A total of 282 patients were identified. Forty-eight were excluded based on study criteria; therefore, our total study cohort was 234 patients: 119 RALP and 115 LP cases. Overall mean postoperative follow-up time was 20.8 months, with no significant differences between groups. Mean operative time was shorter in the LP group when compared the RALP group (3 h 7 min vs. 3 h 41 min, p < 0.001). There were no significant differences between groups in length of stay (1.22 days vs 1.50 days, p = 0.095). Complications occurred in 52 patients (22.2% of overall cohort) with no difference in incidence between groups. Twenty-five patients (14 in the RALP group and 11 in the LP group) underwent unplanned secondary procedures; 19 of these patients (9 in the RALP group and 10 in the LP group) needed a procedure to address secondary obstruction. CONCLUSION: We demonstrated no significant differences between RALP and LP in regards to complication rates. Surgeons performing RALP and LP have the potential to offer the same level of care for the surgical management of UPJO, especially in countries where robotic technology may not be readily available.

Comparison of antegrade continence enema treatment and sacral nerve stimulation for children with severe functional constipation and fecal incontinence.

BACKGROUND: To compare antegrade continence enema (ACE) treatment and sacral nerve stimulation (SNS) in children with intractable functional constipation (FC) and fecal incontinence (FI). METHODS: We performed a retrospective review of children 6-18 years old with FC and FI treated with either ACE or SNS at our institution. We recorded symptoms at baseline, 6 months, 12 months, 24 months, and their most recent visit after starting treatment. We compared improvement in FI, bowel movement (BM) frequency, abdominal pain, laxative use, and complications. Patients were contacted to evaluate perceived benefit using the Glasgow Children's Benefit Inventory. KEY RESULTS: We included 23 patients treated with ACE (52% female, median age 10 years) and 19 patients treated with SNS (74% female, median age 10 years). Improvement in FI was greater with SNS than ACE at 12 months (92.9% vs 57.1%, P = .03) and 24 months (100% vs 57.1%, P = .02). Improvement in BM frequency was greater with ACE, and children were more likely to discontinue laxatives at all follow-up time points (all P < .05). Improvement in abdominal pain was greater with ACE at the most recent visit (P < .05). Rate of complications requiring surgery was similar between groups (26.3% vs 21.7%). Benefit was reported in 83.3% and 100% of ACE and SNS groups, respectively (NS). CONCLUSIONS AND INFERENCES: Although both ACE and SNS can lead to durable improvement in children with FC and FI, SNS appears more effective for FI and ACE more effective in improving BM frequency and abdominal pain and in discontinuation of laxatives.

Free-floating Paratesticular Calcifying Fibrous Pseudotumors in a Pediatric Patient.

Paratesticular masses are a relatively common finding in males. The majority are benign, as opposed to testis masses, which tend to be malignant. Fibrous pseudotumors are rare, but are the third most common paratesticular tumor after adenomatoid and lipoma. The exact cause is unclear but likely from a fibroinflammatory reaction. Because of the non-specific findings on physical exam and scrotal ultrasound, patients may undergo scrotal exploration and occasionally orchiectomy, in spite of the benign nature of this lesion. Here we report the rare case of free-floating paratesticular calcifying fibrous pseudotumors in a prepubertal patient.

Spinal anesthesia for pediatric urological surgery: Reducing the theoretic neurotoxic effects of general anesthesia.

BACKGROUND: Spinal anesthesia (SA) is an effective technique that has been used in children for years. With growing concern with regard to the risks of general anesthesia (GA), we developed a SA program to provide an alternative option. We present our initial experience with this program. OBJECTIVE: To implement a SA program at a large tertiary care pediatric center and assess the safety and efficacy of the technique as an alternative to GA for urologic surgery. STUDY DESIGN/METHODS: We prospectively collected data on all children undergoing SA at our institution. We recorded demographics, procedure, time required for placement of the SA, length of surgery, success of lumbar puncture, success of attaining adequate surgical anesthesia, need for supplemental systemic sedation, conversion to GA, and perioperative complications. RESULTS: SA was attempted in 105 consecutive children (104 boys, 1 girl) with a mean age of 7.4 ± 4.3 months (range 19 days-24 months) and mean weight of 8.3 ± 1.7 kg (range 3.5-13.7). Placement of the SA was successful in 93/105 children (89%). Inability to achieve lumbar puncture (cerebrospinal fluid was not obtained) meant that SA was abandoned in seven (7%) patients and GA was administered. In five patients in whom SA was successful and surgery was begun, 5/93 (5%) required conversion to GA: two because of evisceration of intestine through large hernia defects related to coughing and abdominal irritation, two because of lack of motor blockade despite an adequate sensory block, and one because of an inability to place an intravenous catheter in the lower extremities (required per SA protocol). If necessary, an intravenous catheter can be placed in the upper extremity, but this must be weighed against the fact that the block has already been placed and is of limited duration. Overall, SA was successful (SA was placed and surgery was completed without conversion to GA) in 88/105 children (84%). No additional sedation and no systemic anesthetic agents were required in 75/88 children (85%). The average time required to place the SA was 3.8 ± 2.7 min (range 1-12). The average time for the surgical procedure was 38.3 ± 23.1 min (range 10-122). No patient required conversion to GA because of recession of block. There were no surgical complications. DISCUSSION/CONCLUSIONS: SA is a safe and efficacious technique for routine pediatric urological procedures. SA should be considered for cases such as neonatal torsion or patients with significant cardiac or pulmonary comorbidities when the risks of GA are often weighed against the risks of non-intervention.

Sacral nerve stimulation allows for decreased antegrade continence enema use in children with severe constipation.

BACKGROUND: Sacral nerve stimulation (SNS) can be beneficial for children with constipation, but no studies have focused on children with constipation severe enough to require antegrade continence enemas (ACEs). Our objective was to evaluate the efficacy of SNS in children with constipation treated with ACE. METHODS: Using a prospective patient registry, we identified patients <21years old who were receiving ACE prior to SNS placement. We compared ACE/laxative usage, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), Fecal Incontinence Severity Index (FISI), and Vancouver Dysfunctional Elimination Syndrome Score (DES) at baseline and progressive follow-up time intervals. RESULTS: Twenty-two patients (55% male, median 12years) were included. Median ACE frequency decreased from 7 per week at baseline to 1 per week at 12months (p<0.0001). Ten children (45%) had their cecostomy/appendicostomy closed. Laxative use, GSS, FIQL, and DES did not change. FISI improved over the first 12months with statistical significance reached only at 6months (p=0.02). Six (27%) children experienced complications after SNS that required further surgery. CONCLUSIONS: In children with severe constipation dependent on ACE, SNS led to a steady decrease in ACE usage with nearly half of patients receiving cecostomy/appendicostomy closure within 2years. LEVEL OF EVIDENCE: IV.

Uroepithelial thickening improves detection of vesicoureteral reflux in infants with prenatal hydronephrosis.

INTRODUCTION: Postnatal evaluation of prenatal hydronephrosis (PNH) often includes a voiding cystourethrogram (VCUG) for VUR assessment. Despite limited supporting data, VCUG is currently recommended if postnatal renal and bladder ultrasound (RBUS) reveals moderate/severe hydronephrosis (HN) or hydroureter (HU). Recent studies have shown VUR is more accurately diagnosed by using certain sonographic findings as criteria for obtaining VCUG. Uroepithelial thickening (UET) of the renal pelvis is a finding associated with high-grade vesicoureteral reflux (HGVUR); however, the clinical significance of UET with PNH has not been studied. OBJECTIVE: We sought to determine if the presence of UET implies increased risk for VUR, and to investigate whether UET can improve the test characteristics of RBUS for VUR. STUDY DESIGN: We retrospectively analyzed postnatal RBUS and VCUG findings in infants ≤30 days undergoing evaluation for "prenatal hydronephrosis" over an 11-year period. We used logistic regression to identify factors associated with VUR. Test characteristics of RBUS for HGVUR were compared based on the presence of UET and two criteria sets to define abnormal RBUS. Criteria set 1 consisted of HN SFU grade 3-4 and/or HU; criteria set 2 was defined by the presence of two of following: UET, HU, duplication, and/or renal dysmorphia. RESULTS: Of 135 patients, 39 (29%) had VUR, of whom 16 (41%) had HGVUR. UET was significantly associated with VUR (p < 0.001), and the sensitivity for HGVUR based on UET alone was 94%. On multivariable analysis, UET, HU, duplication, and renal dysmorphia remained significant independent predictors of HGVUR. Compared to criteria 1, using criteria 2 resulted in 43 fewer VCUGs, and significant improvement in sensitivity and specificity for HGVUR (Table). DISCUSSION: Consistent with previous studies, HN alone on postnatal RBUS has little value in predicting the presence or severity of VUR. This study is the largest known series to evaluate UET in the setting of PNH, and our results demonstrate that UET, as well as HU, duplication and renal dysmorphia, are independent sonographic findings predicting HGVUR. Using our proposed criteria, the probability of HGVUR is fourfold more than the prevalence described in the literature, and importantly, when compared to the criteria recommended by the SFU and AUA, would have resulted in 53% fewer VCUGs while missing zero cases of HGVUR. CONCLUSION: In infants with PNH, the sonographic findings of UET, HU, duplication and renal dysmorphia independently indicate greater risk of HGVUR, and the sensitivity and specificity of RBUS for HGVUR is markedly improved when at least two of the four are present.

Epidural pressure changes following caudal blockade: a prospective, observational study.

PURPOSE: Recent case reports raise the question as to whether anesthetic agents injected into the epidural space could lead to a 'compartment syndrome' and neurovascular sequelae. Single-shot caudal epidural anesthesia has been established as a safe technique, but changes in pressure in the caudal epidural space have not been described. Our aim was to study pressure changes to provide preliminary information for future studies design. METHODS: We prospectively measured the pressure changes in the caudal epidural space in 31 pediatric patients. The pressures were measured at loss of resistance, immediately after the bolus dose of local anesthetic (1 ml/kg), and at 15-s intervals up to 3 min. RESULTS: The pressure at loss of resistance was 35.6 ± 27.8 mmHg. A pulsatile waveform was observed once the epidural space was accessed. The pressure after administration of the local anesthetic bolus (1 ml/kg 0.2 % ropivacaine/bupivacaine with 1:200,000 epinephrine) was 192.5 ± 93.3 mmHg. The pressure decreased to 51.5 ± 39.0 mmHg at 15 s, 26.9 ± 9.9 mmHg after 2 min, and 24.7 ± 11.7 after 3 min. The return to baseline occurred at approximately 45-60 s. CONCLUSIONS: Following the administration of the local anesthetic into the caudal epidural space, there was a marked, but transient, increase in the pressure within the epidural space. It appears unlikely that a slow epidural catheter infusion could lead to a sustained increase in epidural pressure.

Uroepithelial Thickening on Sonography Improves Detection of Vesicoureteral Reflux in Children with First Febrile Urinary Tract Infection.

PURPOSE: The 2011 American Academy of Pediatrics clinical practice guideline for childhood febrile urinary tract infection recommends voiding cystourethrography if renal and bladder ultrasound reveals hydronephrosis, scarring or "other findings" that suggest high grade vesicoureteral reflux. We sought to determine if the finding of uroepithelial thickening indicates greater risk of high grade vesicoureteral reflux and whether uroepithelial thickening improves the screening value of renal and bladder ultrasound. MATERIALS AND METHODS: We retrospectively analyzed renal and bladder ultrasound and voiding cystourethrogram findings in children 2 to 24 months old with first febrile urinary tract infection during an 11-year period. Patients with uroepithelial thickening were compared to an age and gender matched sample without uroepithelial thickening. Logistic regression was used to identify factors associated with high grade vesicoureteral reflux. Test characteristics of renal and bladder ultrasound for high grade reflux were compared based on different criteria to define an abnormal renal and bladder ultrasound. RESULTS: Of 226 patients 143 (63%) had vesicoureteral reflux, of whom 37 (26%) had high grade reflux. On multivariable analysis uroepithelial thickening was a significant independent predictor of high grade vesicoureteral reflux (OR 5.41, 95% CI 1.74-16.79, p = 0.004). When hydronephrosis and hydroureter were considered the only abnormal renal and bladder ultrasound findings warranting voiding cystourethrography, sensitivity of renal and bladder ultrasound for high grade reflux was 84%, and 6 children with high grade and 82 with low grade reflux would have been missed. When uroepithelial thickening was also considered an abnormal finding, the sensitivity increased to 97%, and only 1 child with high grade and 57 with low grade reflux would have been missed. CONCLUSIONS: Uroepithelial thickening is associated with an increased risk of high grade vesicoureteral reflux and is an abnormal finding warranting voiding cystourethrography. Sensitivity of renal and bladder ultrasound as a screening test for high grade vesicoureteral reflux is markedly improved when uroepithelial thickening is considered.

Sacral nerve stimulation: a promising therapy for fecal and urinary incontinence and constipation in children.

PURPOSE: This study describes our series of children with bowel and bladder dysfunction (BDD) treated with sacral nerve stimulation in order to begin to identify characteristics associated with better outcomes and guide future therapies. METHODS: Between May 2012 and February 2014, 29 patients were evaluated before and after sacral nerve stimulator (SNS) placement. A prospective data registry was developed that contains clinical information and patient-reported measures: Fecal Incontinence Qualify of Life Scale, Fecal Incontinence Severity Scale, PedsQL Gastrointestinal Symptom Scale, and Vancouver DES Symptom Scale. RESULTS: The median age of patients was 12.1 (interquartile range: 9.4, 14.3) years and the median follow-up period was 17.7 (12.9, 36.4) weeks. 93% had GI complaints and 65.5% had urinary symptoms while 7% had urologic symptoms only. The most common etiologies of BBD were idiopathic (66%) and imperforate anus (27%). Five patients required reoperation due to a complication with battery placement. Six of 11 patients (55%) with a pre-SNS cecostomy tube no longer require an antegrade bowel regimen as they now have voluntary bowel movements. Ten of eleven patients (91%) no longer require anticholinergic medications for bladder overactivity after receiving SNS. Significant improvements have been demonstrated in all four patient-reported instruments for the overall cohort. CONCLUSIONS: Early results have demonstrated improvements in both GI and urinary function after SNS placement in pediatric patients with bowel and bladder dysfunction.

Spontaneous clitoral hood epidermal inclusion cyst mimicking clitoromegaly in a pediatric patient.

Epidermal inclusion cysts are benign lesions that can be found in many parts of the body. They are rarely seen in the clitoral region in pediatric patients but when these are found, they are most commonly seen with a history of trauma. We report an uncommon case of a spontaneous nontraumatic epidermal inclusion cyst in the clitoral hood of a female child. This presentation mimicked clitoromegaly but was ultimately found to be a large epidermal cyst that was successfully excised surgically. We present the important pathologic findings and review the relevant literature.

Double-V scrotoplasty for repair of congenital penoscrotal webbing: a hidden scar technique.

OBJECTIVE: Penoscrotal webbing (PSW) is a common reason for deferral of neonatal circumcision. Reports of successful procedures and outcomes in the literature are sparse. We have performed double-V scrotoplasty (DVS), a modification of a V-Y technique, in 138 patients with excellent results. PATIENTS AND METHODS: We retrospectively reviewed the charts of boys who had undergone DVS for PSW since January 2009 by a single surgeon (S.A.A.). The indications, intraoperative findings, concomitant procedures, outcomes, and complications were recorded. RESULTS: A total of 138 DVSs were performed. Concomitant genital surgeries included 81 hidden penis repairs and 10 other (hernia, hypospadias, chordee, orchidopexy). The median age at the time of surgery was 9.6 months (6.1 months-9.8 years). Patients were evaluated about 1 month postoperatively. In seven cases (5%), minor skin separation occurred at the penoscrotal junction but all healed completely. Superficial skin infection occurred in one patient. None required reoperation and cosmetic results were subjectively excellent. CONCLUSIONS: PSW has been corrected in 138 patients without significant complications and with excellent results. This is the largest known peer-reviewed series evaluating a surgical technique for congenital PSW repair. We believe our technique is simple, reproducible, and, with no diverging suture lines lateral to the median raphe, improves cosmesis.

Is VCUG still indicated following the first episode of urinary tract infection in boys?

OBJECTIVES: To determine if voiding cystourethrogram (VCUG) following first episode of urinary tract infection (UTI) offers any incremental diagnostic advantage in boys if a comprehensive renal and bladder ultrasonogram (RBUS) revealed no abnormalities. METHODS: All boys less than 10 years of age whose first evaluation for UTI included RBUS and VCUG were retrospectively studied over a 10-year period. Those with a disorder of the urinary tract known before imaging were excluded. RBUS and VCUG results were analyzed. RESULTS: Of the 77 who met the inclusion criteria, 58 (77%) were <1 year old. 45 (58%) boys had normal RBUS and VCUG. In 16 (21%) both studies were abnormal: 15 had vesicoureteral reflux (VUR) and one had posterior urethral valves. The remaining 16 (21%) had one abnormal study: 10 had pelvicaliectasis on RBUS without VUR; 6 had normal RBUS with VUR. No urethral abnormality was diagnosed on VCUG when RBUS was normal. Of the six who had VUR and normal RBUS, the one who required surgical intervention had recurrent febrile UTI. CONCLUSIONS: If a well-performed RBUS is normal in a boy with first UTI, the likelihood of a significant finding in VCUG is low. A VCUG is likewise of no apparent screening benefit for obstructive uropathy. With the uncertainties surrounding the benefit of chemoprophylaxis, omitting a VCUG when a RBUS is normal in boys with a first UTI avoids the morbidity without missing important pathologies or altering evolving management protocols.

The prenatal diagnosis of Beckwith-Wiedemann syndrome using ultrasound and magnetic resonance imaging.

Beckwith-Wiedemann syndrome is a rare congenital overgrowth syndrome. It carries important connotations for the pediatric urologist because it is associated with an increased risk for developing certain embryonal tumors. Until recently, these patients were not diagnosed until after birth; however, with newer imaging modalities, this syndrome may be diagnosed prenatally. We present a case of Beckwith-Wiedemann syndrome, depicting the prenatal modalities that may be used to diagnose this unusual condition.

A review of pediatric glans malformations: a handy clinical reference.

PURPOSE: Lesions of the pediatric glans penis are an uncommon but important aspect of pediatric urological care. We reviewed the available literature on this topic and did not identify a comprehensive reference resource. We compiled our multi-institutional experience with such conditions to prepare a handy clinical reference source. MATERIALS AND METHODS: The diversity of pediatric glans penis lesions was documented by searching the English and nonEnglish literature as well as the archives at our institutions. Cases were included in the study if the patient(s) identified were younger than 18 years at initial presentation or documented to have first had a glans penis lesion when younger than 18 years. Hypospadiac-epispadiac anomalies of the glans were excluded. RESULTS: From our institutions we identified 6 new cases of various pediatric glans lesions. Altogether the literature describes 137 distinct nondiphallia glans lesions and more than 100 cases of diphallia, including glans duplication. The nondiphallia reports consist of a total of 61 cystic lesions (44%), 33 vascular malformations (24%), 20 dermatological lesions (15%), 20 infectious lesions (15%) and 3 neurogenic lesions (2%). We did not identify a compact resource to compare these anomalies. Visual comparison permits structuring a differential diagnosis and determining urological treatment, which is typically excisional biopsy, laser treatment, sclerotherapy or topical steroid administration. CONCLUSIONS: We present a visual reference of varied lesions of the pediatric glans penis, including 6 new cases, with urological significance. These lesions are always benign but they typically require surgical excision for a definitive pathological diagnosis.

Multi-institutional experience with PIC cystography--incidence of occult vesicoureteral reflux in children with febrile urinary tract infections.

OBJECTIVES: It has been reported that all children with febrile urinary tract infections (FUTIs) and normal voiding cystourethrogram (VCUG) findings show occult reflux (vesicoureteral reflux [VUR]) when examined by cystography performed with positioning of the instillation of contrast at the ureteral orifice (PIC cystography). In this study, we evaluated the reproducibility of this finding using a prospective multi-institutional collaborative examination to determine whether PIC-VUR is as common in such cases as previously shown. METHODS: From March 2002 to October 2003, four institutions performed PIC cystography consecutively and prospectively in pediatric cases of FUTI (greater than 101 degrees F) who had not shown VUR by conventional VCUG. We correlated the ureteral orifice (UO) configuration and position with the status of PIC-VUR. RESULTS: PIC cystography was performed in 39 children (31 girls and 8 boys; 75 FUTIs). We identified PIC-VUR in 32 (82%) of 39 children, involving 58 (74%) of 78 ureters. The likelihood of demonstrating PIC-VUR was increased 37-fold if the UO showed a patulous configuration and was increased 9-fold if the UO showed a lateral configuration. PIC-VUR was noted uniformly in UOs showing hydrodistension. CONCLUSIONS: This multi-institutional registry showed that the incidence of PIC-VUR (82%) in children with FUTI and normal standard VCUG findings is similar to that previously reported. We consider that PIC-VUR likely accounts for the FUTIs.