Giant Congenital Melanocytic Nevus of the Male External Genitalia: A Pediatric Case Report of Diagnosis, Evaluation and Management.

Congenital melanocytic nevi are present at birth or develop within the first few months of life. Giant congenital melanocytic nevi are a rare variant and may involve the external genitalia with a confluent "bathing trunk" distribution. Rapid growth of proliferative nodules of melanocytic cells may cause disfigurement and anatomical distortion resulting in psychological distress and loss of functionality. We report the case of a neglected 17-year-old nonverbal male who received a resection of a Giant Congenital Melanocytic Nevi (GMN) engulfing the penis and scrotum with final resected dimensions of 36.0×20.0×8.0 cm.

Infantile versus childhood posterior urethral valve diagnosis: management patterns and clinical outcomes at opposite ends of the spectrum.

BACKGROUND: Management patterns and outcomes are poorly defined in cases of late PUV diagnosis. OBJECTIVE: To compare post-ablation management and clinical outcomes of patients with infantile (<1 year) versus childhood (>5 year) PUV diagnosis to gain insight into the pathologies at opposite ends of the PUV spectrum. STUDY DESIGN: A multicenter retrospective cohort study was conducted using the TriNetX research network between 2006 and 2022. TriNetX synthesizes insurance claims and electronic medical record data for over 110 million patients from 92 healthcare organizations. We defined two cohorts: 1) The <1 year arm had an index diagnosis of PUV and cystoscopy with valve ablation within 1 year of life, 2) the >5 year arm had an index diagnosis of PUV and valve ablation after age 5. We report rates and time-to-first use of antispasmodics, alpha-blockers, CIC, bladder botox, enterocystoplasty or Mitrofanoff or secondary cutaneous vesicostomy, and CKD. RESULTS: We identified 569 patients (323 <1 year; 246 >5 year). Median age at diagnosis was 1 month (median follow-up 8 years) and 9 years (median follow-up 10 years) for the <1 year and >5 year cohorts, respectively. Following ablation, both arms were primarily managed with antispasmodics, with no difference between groups. The >5 year arm was significantly more likely to receive alpha-blockers or bladder botox. The <1 year arm was significantly more likely to be started on CIC, undergo enterocystoplasty, Mitrofanoff or secondary cutaneous vesicostomy, or renal transplantation. The <1 year arm had significantly higher rates and shorter time-to-progression to all stages of CKD. DISCUSSION: Despite higher utilization of conservative strategies among patients with a late PUV diagnosis, these patients had superior renal outcomes and low rates of progression to invasive treatments. Limitations include potential inaccuracies in medical coding as well as variations in thresholds to initiate CIC, perform surgical reconstruction, or proceed with renal transplantation at participating centers. CONCLUSIONS: These findings provide evidence that a late PUV diagnosis reflects an overall milder disease process.

Use of Perirectal Hyaluronic Acid Spacer Prior to Radiotherapy in a Pediatric Patient With Bladder Rhabdomyosarcoma: A Case Report.

Rhabdomyosarcoma (RMS) treatment involves surgery, chemotherapy, and radiotherapy. A radioprotective space between the bladder/prostate and rectum reduces postradiation complications, as reported in adult patients. Describe pediatric preradiotherapy perirectal hyaluronic acid (HA) spacer injection for bladder/prostate RMS. We present a case of a 17-month-old male with bladder/prostate RMS. Before radiotherapy, an HA spacer was injected peri-rectally. Under general anesthesia, a transrectal ultrasound was positioned and 1mL of HA spacer was injected into the perirectal space. No complications were reported at 6-month follow-up. This is the first report of pre-radiation therapy spacer injection for pediatric bladder/prostate RMS.

Role of progressive urethral dilation and primary valve ablation in the long-term renal outcomes of small, preterm infants with posterior urethral valve.

INTRODUCTION: PUV patients managed with primary vesicostomy instead of primary valve ablation (PVA) historically are preterm, low-birth-weight (LBW) infants with inadequate urethral size. We previously described progressive urethral dilation (PUD) as an effective method of enhancing the likelihood of PVA in these infants, allowing equal access to PVA as an initial management method. OBJECTIVE: We aim to characterize renal outcomes in patients managed with PUD + PVA and compare this to outcomes with PVA alone. We also re-examine the effect of LBW and gestational age on renal outcomes in PUV with a cohort treated uniformly by PVA. METHODS: We performed retrospective review of 78 neonates with PUV treated with PVA prior to 10 weeks of age with >1 year of follow up. Before valve ablation, boys either underwent PUD (serial upsizing of a smaller bore urethral catheter to an 8Fr catheter; PUD + PVA) or non-dilation (smaller bore catheter was maintained; PVA-only). PUD + PVA versus PVA-only was compared using chi-square and t-test. Logistic regression was performed to assess the effect of PUD, preterm (<37 weeks), LBW (<2.5 kg), and other predictors on the final outcomes of CKD3+ and ESRD. RESULTS: 31 of 78 patients underwent PUD + PVA. Mean follow up was 5.2 years (SD 3.4), with no significant difference between PUD + PVA and PVA-only. The PUD + PVA group included significantly lower gestational age infants with lower birth weight and ablation weight. There was no significant effect of PUD on final CKD3+ or ESRD outcome on univariable or multivariable analysis. When adjusted for other variables, only Cr nadir >0.5 remained an independent predictor of CKD3+ (OR 41.2; p < 0.001) and ESRD (OR 18.9; p = 0.015). DISCUSSION: We previously demonstrated that PUD is an effective means to achieve PVA in small neonates who might otherwise require vesicostomy. The data herein demonstrates no significant effect of PUD on renal outcomes. In this unique cohort of newborns treated with PVA, only creatinine nadir and not gestational age or an independent predictor of outcomes. CONCLUSION: In small preterm infants who would have been excluded from PVA due to limited urethral size, PUD + PVA confers the same renal outcomes as PVA alone in larger infants. This novel data will assist in the risk-benefit analysis of using PUD before PVA in newborns diagnosed with PUV. When primary intervention is uniformly PVA, preterm birth and LBW are not independent predictors of renal outcomes.

Posterior urethral morphology on initial voiding cystourethrogram correlates to early renal outcomes in infants with posterior urethral valves.

INTRODUCTION: Posterior urethral valves (PUV) is a leading cause of chronic renal failure in childhood. Bladder and posterior urethral deformity in infants with PUV are highly variable on initial voiding cystourethrogram (VCUG). Some types of deformity may be more important than others in determining the severity of the condition. Identification of specific VCUG features may allow for a simple, early screening assessment for patients. OBJECTIVE: Determine whether morphologic features on the initial VCUG correlate to renal outcomes as measured by the creatinine nadir in the first year after ablation. METHODS: Children with PUV treated by primary valve ablation before 12 months old and followed >1 year were identified. Initial diagnostic VCUG was evaluated for the bladder height to width ratio (HW-B), posterior urethral height to width ratio (HW-PU), and posterior-anterior urethral ratio (PA-UR). A trabeculation grade was assigned by three pediatric urologists, and reflux was noted. Univariable analyses with chi-square and t-test were used to compare bladder and posterior urethral morphology factors among those who obtained a creatinine nadir <0.8 or ≥0.8 mg/dL in the first year after ablation. Linear regression was performed to correlate morphology values with true creatinine nadir. RESULTS: A total of 120 boys were ablated at mean age of 40.5 days (range 0-342) and followed for 5.9 years (± 3.85). Among these, 21 (17.5%) had a creatinine nadir ≥0.8 mg/dL. Mean overall HW-B and mean PA-UR were not significantly different between those with creatinine nadir <0.8 versus ≥0.8 mg/dL. Bladder trabeculation grade was not associated with creatinine nadir. For the entire cohort, only the difference in HW-PU was statistically significant between creatinine nadir groups with a much higher ratio among those with a nadir ≥0.8 (p < 0.001). Linear regression demonstrated a significant positive correlation between the HW-PU and creatinine nadir (R 2 = 0.097, p = 0.002). The presence of bilateral reflux is significantly associated with creatinine nadir ≥0.8 mg/dL (p = 0.001). DISCUSSION: We investigate for the first time the association of morphology features on the initial VCUG with renal outcome in PUV patients. Posterior urethral deformity as quantified by a higher HW-PU ratio is significantly correlated with a higher creatinine nadir, whereas measured bladder morphology metrics are not. HW-PU may be an indirect measure of the severity of obstruction in posterior urethral valves. CONCLUSION: The HW-PU appears to be a meaningful early morphologic metric for renal outcome.

Predicting childhood chronic kidney disease severity in infants with posterior urethral valve: a critical analysis of creatinine values in the first year of life.

BACKGROUND: Posterior urethral valve (PUV) is a leading cause of chronic kidney failure in children. Studies have shown that a creatinine nadir above historical cutoff values of 0.8 or 1.0 mg/dL correlates with worse kidney outcomes. The ability to use nadir creatinine more discriminately as a test of kidney outcomes is otherwise limited. METHODS: We performed a retrospective review of 102 infants treated with primary valve ablation prior to 1 year of age. Patient factors including creatinine at presentation (Cr-P), nadir within 6 weeks after ablation (Cr-6 W), and nadir within 1 year after ablation (Cr-1Y) were assessed as predictors of final chronic kidney disease (CKD) severity. An optimal threshold for four CKD levels was defined in incremental fashion using binary outcome with receiver operating characteristic (ROC). Multivariable logistic regression models compared Cr-P, Cr-6 W, and Cr-1Y while adjusting for patient factors. RESULTS: Boys were ablated at mean age of 36.3 days and followed for 6.6 years (± 3.7). When compared to other demographics, only creatinine remained independently predictive of CKD outcomes on multivariable analysis. ROC analysis demonstrated excellent diagnostic accuracy for Cr-6 W and Cr-1Y (p < 0.001) and acceptable accuracy for Cr-P (p < 0.005). Using the Cr-6 W and Cr-1Y models, high sensitivity and specificity creatinine nadir cutoffs were determined to predict each CKD outcome. CONCLUSIONS: The severity of childhood CKD can be predicted with high accuracy using the creatinine nadir within 6 weeks of ablation. The cutoff values described can be incorporated into a clinical setting for patient counseling and individual risk stratification.

Inflammatory Myofibroblastic Tumor Presenting as Gross Hematuria in a Pediatric Patient With VACTERL Syndrome Following Bladder Augmentation.

Inflammatory myofibroblastic tumors (IMT) are rare and poorly understood inflammatory neoplasms. Most commonly occurring in the liver and gastrointestinal tract, cases of bladder involvement have been rarely reported. Bladder IMT generally presents with gross hematuria and can be differentiated from other bladder tumors by expression of anaplastic lymphoma kinase. We report the occurrence of an Bladder IMT detected following lower urinary tract reconstruction with bladder augmentation.

Role of a Preoperative Catheter Regimen in Achieving Early Primary Endoscopic Valve Ablation in Neonates with Posterior Urethral Valves.

PURPOSE: Primary valve ablation is preferred to vesicostomy in the initial management of posterior urethral valves. However, some neonates have a prohibitively small urethra. We describe our experience with a preoperative urethral catheter regimen to enhance the likelihood of neonatal valve ablation. MATERIALS AND METHODS: We performed a retrospective review of 126 neonates with posterior urethral valves treated between 2003 and 2019 with valve ablation prior to 10 weeks of age. The preoperative indwelling catheter either was gradually upsized to an 8Fr (progressive urethral dilation), was not upsized (nondilated) or was initially larger bore (8Fr only). The primary outcome was the ability to perform primary ablation by neonatal resectoscope. The secondary objective was to establish the parameters for considering progressive urethral dilation as well as its associated risks. RESULTS: Overall 97% could be ablated. The progressive urethral dilation group had the lowest mean weight (p <0.001). Only a larger catheter at the time of ablation was significantly associated with feasible ablation (p <0.001) and not urethral dilation, the infant's weight or his gestational age. Progressive urethral dilation was associated with a longer duration of catheterization as well as double the rate of febrile urinary tract infections (8.5%) over the nondilated group (3.6%). CONCLUSIONS: A much higher rate of primary ablation is feasible (97%) than previously reported (82%). More important than the infant's weight is whether a 6Fr to 8Fr catheter is in place at ablation. If an initial 6Fr to 8Fr catheter cannot be placed, urethral dilation to 8Fr should be performed before attempting ablation. This is both a technique and preoperative assessment that is useful for operative planning.

A Review of Mullerian Anomalies and Their Urologic Associations.

Structural anomalies of the female reproductive tract, known as Mullerian anomalies, can occur in isolation or in association with anomalies of other organ systems. Due to shared embryology, the most common association in up to 40% of patients is with renal, ureteral, and bladder anomalies. Affected girls can have a wide range of genitourinary symptoms with urologists playing an integral role in their diagnosis and treatment. To facilitate the recognition and management of these conditions, we provide a review of Mullerian anomalies including the embryology, classifications, syndromes, evaluation, and treatments with attention to their urologic applicability.

Fate of Pediatric Renal Angiomyolipoma During mTOR Inhibitor Treatment in Tuberous Sclerosis Complex.

OBJECTIVE: To evaluate the clinical and radiographic follow-up of renal angiomyolipoma (AML) in pediatric patients with tuberous sclerosis complex (TSC) on mTOR inhibitors. METHODS: We performed retrospective chart review of children who were diagnosed with TSC between 2000 and 2019 and prescribed everolimus at age ≤18 years. Treatment assessment was performed in patients who were medically-compliant by serum drug trough levels and who had at least a baseline and one subsequent renal imaging study. RESULTS: Nineteen patients were analyzed. Average age of everolimus initiation was 9 years, and indication was neurologic in 17 (90%). Fourteen patients (73.6%) had AML with average size of 1.9 (0.4-5) cm. Medication was discontinued due to side effects in 3 (16%) patients. Treatment assessment was analyzed for 15 patients with median medication exposure 5.1 (0.8-8.5) years. Among 13 with AML, the dominant lesion decreased in size in 9 (69%) and stayed stable in 4 (31%). Greatest absolute size decrease was seen for lesions ≥2 cm. No new AML lesions formed during treatment. CONCLUSION: Although not currently approved for this indication, everolimus appears to be well-tolerated with similar efficacy for pediatric AML as in adult AML. Use may be most warranted in children with AML ≥2 cm.

Assessment of health literacy in adolescents with spina bifida and their caregivers: a multi-institutional study.

INTRODUCTION: and Objective: Health literacy is defined as the ability to obtain, integrate, and appraise health-related knowledge. It is known to correlate with disparities in clinical outcomes in adults with chronic disease. Patients with spina bifida represent a potentially vulnerable cohort as they often have multiple comorbidities. The authors aimed to characterize health literacy in adolescent patients with spina bifida and their caregivers. STUDY DESIGN: The Newest Vital Sign (NVS), a validated assessment of health literacy and numeracy, was administered to patients (aged ≥10 years) and caregivers in outpatient pediatric urology and multidisciplinary spina bifida clinics. Subjects not fluent in English or Spanish and those with cognitive delay were excluded. Survey responses and demographic information were analyzed and compared between the spina bifida and control groups. RESULTS: Three hundred eleven caregivers (caregivers for patients with spina bifida: 185, caregivers for the controls: 126) and 84 adolescents (those with spina bifida: 46, controls: 38) completed the NVS. Although there was no difference in health literacy between caregivers (p = 0.98), adolescents with spina bifida demonstrated lower NVS scores (spina bifida 1 [0-3] vs. control 2 [2-4]; p = 0.02) (Figure). Health literacy of patients with spina bifida and controls increased with age (p = 0.002). Adjusting for age and gender, the odds of having limited literacy were 5.5 times higher in patients with spina bifida than in the controls (p = 0.004). Inadequate caregiver health literacy was associated with a lower education level (p < 0.001). DISCUSSION: Spina bifida is among the most complex birth defects compatible with life and affects a multitude of systems. Although it is well established that limited health literacy in adults with chronic disease is associated with adverse outcomes, there are considerably fewer data in the pediatric population. The authors found that caregivers of patients with spina bifida exhibited health literacy and numeracy comparable with parents in the control group, despite lower education levels. In contrast, adolescents with spina bifida demonstrated poorer health literacy than the controls. Multidisciplinary care is necessary, given the medical complexity of spina bifida; therefore, impaired understanding of adults' own needs may pose a barrier to successful transition to their care and subsequent outcomes. CONCLUSIONS: The majority of surveyed caregivers for patients with spina bifida exhibited adequate health literacy, especially those with some college education; however inadequate health literacy was more likely among adolescents with spina bifida when compared with the controls. Screening for health literacy may be useful to assess readiness for transition to more independent self-care among patients with spina bifida.

Opioid utilization is minimal after outpatient pediatric urologic surgery.

INTRODUCTION: There are no guidelines for opioid use after pediatric urologic surgery, and it is unknown to what extent prescriptions written for these patients may be contributing to the opioid epidemic in the United States. We sought to characterize opioid utilization in a prospective fashion following outpatient pediatric urologic surgery at our institution. MATERIALS AND METHODS: After obtainingapproval from the Institutional Review Board, we prospectively recruited pediatric patients undergoing outpatient urologic surgery. All patients and families were counseled regarding appropriate use of over-the-counter pain medications as first-line agents, with opioids for breakthrough pain only. All patients received an opioid prescription (ORx), which we attempted to standardize to 10 doses. Parents were provided with a log for keeping track of pain medication administration. Postoperative surveys were sent at various time points after surgery to assess utilization of pain medications at home. We quantified unused opioids prescribed and evaluated factors potentially associated with opioid use. RESULTS: Two hundred and two patients were recruited. All patients were male, with a median age of 2.7 years (interquartile range (IQR) 5.5, range 0.5-17.9 years). One hundred and fifty-four children underwent penile surgery, 22 underwent scrotal surgery, and 27 underwent inguinal surgery. Nearly half of our study patients were black, 33.2% were white, 12.9% were Latino, and 4.0% were Asian. The median number of doses prescribed was 10 (IQR 0, range 4.0-20.8). Postoperative surveys were completed by 80.7% of study patients. The median number of opioid doses used was 0 (IQR 2), whereas the mean was 1.28 (standard deviation (SD) 1.98). None of the factors evaluated (including patient age, surgery type, perioperative pain management techniques, length of surgery, and insurance type) were associated with the amount of opioid used at home after surgery, as utilization was equally low across all groups. DISCUSSION AND CONCLUSIONS: Ensuring adequate postoperative pain control for children is critical, yet it is also important to minimize excess ORx. We found that the majority of pediatric patients used 0-2 doses of prescription pain medication after discharge following outpatient urologic surgery, representing a small percentage of the total prescribed amount. Low utilization was seen irrespective of patient age, procedure, and perioperative factors. These data can be used to guide perioperative patient and family counseling and to guide future efforts to standardize ORx following outpatient pediatric urologic surgery.

Abdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness.

INTRODUCTION: Abdominoplasty is an important component of the management of children with prune belly syndrome (PBS). While there are features of the abdominal defect in PBS which are common to all patients, there will be differences unique to each patient that should be taken into consideration in surgical planning. Specifically, we have come to realize that although the Monfort procedure assumes a symmetric pattern of abdominal wall laxity, this symmetry is rarely present. OBJECTIVE: The aim of this report is to describe our modifications and review our outcomes for the Monfort procedure which more completely address correction of the abdominal wall laxity including both common and uncommon features while positioning the umbilicus to a more anatomically correct position (Figure). STUDY DESIGN: Sixteen male patients with PBS and one female pseudoprune belly syndrome patient, aged 2-9 years, were treated at our institution between 2003 and 2014. Modifications incorporated into the abdominoplasty procedure for PBS applied to this study group included: 1) use of diagnostic laparoscopy to define the topography of the abdominal wall defect, 2) initial midline rather than elliptical skin incision to defer retailoring of the skin coverage until the final step of the procedure, 3) varying the width of the central plate to correct side to side asymmetry in redundancy, 4) plication of the central plate to reduce vertical redundancy and reposition the umbilicus, and 5) plication of focal areas of fascial weakness, most often in the flank region. RESULTS: All patients have improved abdominal wall contour with a more uniform correction of areas of weakness at a mean follow-up of 5.5 years (range 18 months-11.5 years). All patients and parents indicate that they are very satisfied with the outcome of their procedures without any revisions being performed. This study is descriptive in nature and retrospective, with the patient population treated in a relatively uniform fashion that does not allow direct comparison with other techniques. CONCLUSIONS: The modified Monfort procedure recognizes the pattern of abdominal muscular deficiency unique to each patient and incorporates this information into the surgical design.

Outcomes of complex robot-assisted extravesical ureteral reimplantation in the pediatric population.

INTRODUCTION AND OBJECTIVE: While open ureteral reimplantation remains the gold standard for surgical treatment of vesicoureteral reflux (VUR), minimally invasive approaches offer potential benefits. This study evaluated the outcomes of children undergoing complex robot-assisted laparoscopic ureteral reimplantation (RALUR) for failed previous anti-reflux surgery, complex anatomy, or ureterovesical junction obstruction (UVJO), and compared them with patients undergoing open extravesical repair. STUDY DESIGN: Children undergoing complex RALUR or open extravesical ureteral reimplantation (OUR) were identified. Reimplantation was classified as complex if ureters: 1) had previous anti-reflux surgery, 2) required tapering and/or dismembering, or 3) had associated duplication or diverticulum. RESULTS: Seventeen children underwent complex RALUR during a 24-month period, compared with 41 OUR. The mean follow-up was 16.6 ± 6.5 months. The RALUR children were significantly older (9.3 ± 3.7 years) than the OUR patients (3.1 ± 2.7 years; P < 0.001). All RALUR patients were discharged on postoperative day one, while 24.4% of children in the open group required longer hospitalization (mean 1.3 ± 0.7 days; P = 0.03). Adjusting for age, there was no significant difference in inpatient analgesic usage between the two cohorts. Three OUR patients (7.3%) developed postoperative febrile urinary tract infection compared with a single child (5.9%) undergoing RALUR (P = 1.00). There was no significant difference in complication rate between the two groups (12.2% OUR versus 11.8% RALUR; P = 1.00). A postoperative cystogram was performed in the majority of RALUR patients, with no persistent VUR detected, and one child (6.7%) was diagnosed with contralateral reflux. DISCUSSION: Reported VUR resolution rates following robot-assisted ureteral reimplantation are varied. In the present series, children undergoing RALUR following failed previous anti-reflux surgery, with complex anatomy, or UVJO experienced a shorter length of stay but had similar analgesic requirements to those undergoing open repair. Radiographic, clinical success rates and complication risk were comparable. This study had several limitations, aside from lack of randomization. Analgesic use was limited to an inpatient setting, and pain scores were not assessed. Not all children underwent a postoperative VCUG, so the true radiographic success rate is unknown. A larger patient cohort with longer follow-up is necessary to determine predictors of radiographic and clinical failure. CONCLUSION: Older children with a previous history of anti-reflux surgery were more likely to undergo RALUR. These children had success and complication rates comparable to younger patients following complex open extravesical reimplantation, which underscores the expanding role of robot-assisted lower urinary tract reconstructive surgery in the pediatric population.