Palliative Care for Pediatric Urology.

Palliative care in the field of urology has largely been limited to adult oncologic conditions. Although there is a plethora of established literature suggesting the advantageous impact of palliative care, there is limited integration of palliative care in adult urology. This underutilization is further exacerbated in pediatric urology, and palliative care in pediatric urology remains an underexplored area despite the prevalence of several life-limiting conditions in this patient population. This paper highlights the potential need for palliative care intervention in a variety of urologic conditions in the pediatric population, including congenital lower urinary tract obstruction, neurogenic bladder dysfunction, exstrophy-epispadias complex, and congenital bilateral renal agenesis. Each condition poses unique challenges that can be addressed with the inclusion of a palliative care team, including decision-making spanning prenatal-neonatal-pediatric periods, acute and chronic symptom management, family relations, body image issues, risk of recurrent hospitalizations and surgeries, and potentially fatal complications. Alongside standard urologic interventions, palliative care can serve as an additional means of addressing physical and psychosocial symptoms experienced by pediatric urology patients to enhance the quality of life of patients and their families.

Effectiveness of Liposomal Bupivacaine With Bupivacaine Hydrochloride vs Bupivacaine Hydrochloride Alone as a Local Anesthetic for Children Undergoing Ambulatory Urologic Surgery: The Baby ORIOLES Randomized Clinical Trial.

PURPOSE: We sought to determine if the addition of liposomal bupivacaine to bupivacaine hydrochloride improves opioid-free rate and postoperative pain scores among children undergoing ambulatory urologic surgery. MATERIALS AND METHODS: A prospective, phase 3, single-blinded, single-center randomized trial with superiority design was conducted in children 6 to 18 years undergoing ambulatory urologic procedures between October 2021 and April 2023. Patients were randomized 1:1 to receive dorsal penile nerve block (penile procedures) or incisional infiltration with spermatic cord block (inguinal/scrotal procedures) with weight-based liposomal bupivacaine plus bupivacaine hydrochloride or bupivacaine hydrochloride alone. The primary outcome was opioid-free rate at 48 hours. Secondary outcomes included parents' postoperative pain measure scores, numerical pain scale scores, and weight-based opioid utilization at 48 hours and 10 to 14 days. RESULTS: We randomized 104 participants, with > 98% (102/104) with complete follow-up data at 48 hours and 10 to 14 days. At interim analysis, there was no significant difference in opioid-free rate at 48 hours between arms (60% in the intervention vs 62% in the control group; estimated difference in proportion -1.9% [95% CI, -20%-16%]; P = .8). We observed no increased odds of patients being opioid-free at 48 hours with the intervention compared to the control group (OR 0.96 [95% CI 0.41-2.3]; P = .9). The trial met the predetermined futility threshold for early stopping. There was no difference in parents' postoperative pain measure scores, numerical pain scale scores, or opioid utilization at 48 hours or 10 to 14 days. No difference in adverse events was observed. CONCLUSIONS: The addition of liposomal bupivacaine to bupivacaine hydrochloride did not significantly improve opioid-sparing effect or postoperative pain compared with bupivacaine hydrochloride alone among children ≥ 6 years undergoing ambulatory urologic surgery.

When to address form and when to address function: Timing of surgical reconstruction for a patient with 46 XY DSD.

Differences of sexual development (DSD) refers to congenital conditions characterized by discordant appearances of external genitalia with respect to sex chromosomes. We present a case of a 46 XY DSD adolescent with bilateral undescended testes and severe scrotolabial anomalies who was lost to follow-up for several years who recently presented with "recurrent UTIs." Although the patient desired immediate reconstruction to void while standing, shared-decision making was used to first address his bilateral cryptorchidism, with plans to delay other reconstruction until the patient is older. Pediatric patients with DSD have complicated medical and surgical problems and require a collaborative multidisciplinary team.

Does prophylactic antibiotics post pediatric pyeloplasty reduce the incidence of febrile UTIs?

PURPOSE: The use of postoperative prophylactic antibiotics in pediatric upper urinary tract reconstruction remains controversial. In this study, we examined whether low dose antibiotics administered following pediatric pyeloplasty reduce the incidence of febrile urinary tract infections at our institution. As a secondary outcome, in those patients with infection, additional analysis was performed to better quantify which patient population benefits the most from low dose prophylactic antibiotics. METHODS: Institutional review board approval (IRB) was obtained. All methods were carried out in accordance with relevant guidelines and regulations. A retrospective study was performed in patients who underwent pyeloplasty (2011-2017) at our institution. Surgical approach (laparoscopic versus robotic assisted versus open, with or without internal JJ ureteral stent) were based on surgeon preference. Patients of 8 fellowship trained pediatric urologists were included in the study period. Patients with prior history of urologic interventions or other congenital genitourinary tract abnormalities were excluded. Demographics (age, gender, ethnicity, insurance status), prior history of culture proven urinary tract infection, surgical details (administration of perioperative antibiotics), and postoperative outcomes including; 1) re-admission 30 days post-surgery, 2) any urine cultures collected due to suspected urinary tract infection. RESULTS: A total of 209 patients (149 boys, 60 girls) met our inclusion criteria with 55/209 (26%) receiving postoperative prophylactic antibiotics. The average age was 6 years (range: 2 months-18 years). Indwelling ureteral stent was used in 176 (84%) patients. Eleven patients (5%) had a culture-proven urinary tract infection within 30-days postoperatively. No significant differences were seen in postoperative complications or incidence of urinary tract infection when comparing surgical approaches, +/- ureteral stent, or the use of antibiotics. Secondary analysis noted statistically significant increase in post-operative urinary tract infection in younger children (2.8 v. 6.2 years, p = 0.02), those patients who had a positive preoperative urine culture (8/11, p = 0.01) and those with public health insurance (p = 0.038). CONCLUSION: The incidence of postoperative urinary tract infection following pyeloplasty in our cohort was relatively low. There was a higher incidence of urinary tract infection in patients less than 3 years old. The use of antibiotics in patients post pyeloplasty did not appear to affect the incidence of post-operative urinary tract infection, however, they may have a role in children who have not yet potty trained and in patients with positive preoperative urine culture.

Safety and Efficacy of Long-Acting Liposomal Bupivacaine Plus Bupivacaine Hydrochloride for Dorsal Penile Block During Ambulatory Pediatric Urologic Surgery.

This study evaluates the tolerability and efficacy of preoperative dorsal penile nerve block with Exparel plus bupivacaine hydrochloride in children>6 years old undergoing ambulatory urologic surgery. We demonstrate that the drug combination is well-tolerated, with appropriate analgesic efficacy in the recovery room as well as at 48-hour and 10-14 day follow-up periods. These preliminary data justify the need to perform a prospective, randomized trial comparing Exparel plus bupivacaine hydrochloride to other common local anesthetic regimens used in pediatric urologic surgery.

A Prospective Study of the Urinary and Gastrointestinal Microbiome in Prepubertal Males.

OBJECTIVE: To determine if urinary microbial communities similar to those described in adults exist in children and to profile the urinary and gastrointestinal microbiome in children presenting to urology for both routine and complex urologic procedures. METHODS: Prepubertal boys (n = 20, ages 3 months-8 years; median age 15 months) who required elective urologic procedures were eligible. Urine samples were collected via sterile catheterization and fecal samples were obtained by rectal swabs. DNA was extracted from urine pellet and fecal samples and subjected to bacterial profiling via 16S rDNA Illumina sequencing and 16S rDNA quantitative polymerase chain reaction. We assessed within and between sample diversity and differential species abundance between samples. RESULTS: Urine samples had low bacterial biomass that reflected the presence of bacterial populations. The most abundant genera detected in urine samples are not common to skin microbiota and several of the genera have been previously identified in the urinary microbiome of adults. We report presumably atypical compositional differences in both the urinary and gastrointestinal microbiome in children with prior antibiotic exposure and highlight an important case of a child who had undergone lifelong antibiotic treatment as prophylaxis for congenital abnormalities. CONCLUSION: This study provides one of the first characterizations of the urinary microbiome in prepubertal males. Defining the baseline healthy microbiome in children may lay the foundation for understanding the long-term impact of factors such as antibiotic use in the development of a healthy microbiome as well as the development of future urologic and gastrointestinal diseases.

Evaluating Outcomes of Differential Surgical Management of Nonfunctioning Upper Pole Renal Moieties in Duplex Collecting Systems.

OBJECTIVE: To evaluate the management and clinical outcomes of nonfunctioning upper pole moieties treated with either upper pole heminephrectomy or upper pole preservation with lower ureteral reconstruction at a single tertiary institution. METHODS: After Institutional Review Board (IRB) approval, patients with duplicated systems undergoing upper pole heminephrectomy, ureteroureterostomy, or common sheath ureteral reimplantation from 2012-2017 were identified. Only patients with a nonfunctioning upper pole moiety on ultrasound or renal scan were included. Patients undergoing upper pole heminephrectomy were compared to those undergoing upper pole preservation with respect to demographics, anatomic variations preoperatively, and postoperative outcomes. RESULTS: Twenty-seven (57%) patients underwent upper pole preservation with lower ureteral reconstruction; 20 (43%) patients underwent upper pole heminephrectomy. Patients undergoing lower ureteral reconstruction were older (1.63 vs 2.76 years, P = .018) and more commonly presented with lower pole vesicoureteral reflux (67% vs 25%, P = .008). No significant difference in postoperative complications was seen between the two groups. After ureteroureterostomy, one patient developed new onset symptomatic reflux to the upper pole requiring intravesical reimplantation. In the heminephrectomy group, 4 of 11 patients with ureteroceles had ureterocelectomy with concomitant lower pole reimplantation. After heminephrectomy, two additional patients required further interventions: ureterocele excision and transurethral polyp excision. CONCLUSION: For patients with nonfunctional upper poles, lower tract reconstruction is a safe alternative to upper pole heminephrectomy. No significant difference in outcomes was seen. Considering that nearly 1 of 3 of patients with upper pole heminephrectomy required additional lower urinary tract procedures, pursuing upper pole preservation with lower urinary tract reconstruction may be favorable.

Cytotoxic Necrotizing Factor-1 (CNF1) does not promote E. coli infection in a murine model of ascending pyelonephritis.

BACKGROUND: Urinary tract infections (UTI) are among the most common and costly infections in both hospitalized and ambulatory patients. Uropathogenic E. coli (UPEC) represent the majority of UTI isolates and are a diverse group of bacteria that utilize a variety of virulence factors to establish infection of the genitourinary tract. The virulence factor cytotoxic necrotizing factor-1 (CNF1) is frequently expressed in clinical UPEC isolates. To date, there have been conflicting reports on the role of CNF1 in the pathogenesis of E. coli urinary tract infections. RESULTS: We examined the importance of CNF1 in a murine ascending kidney infection/ pyelonephritis model by performing comparative studies between a clinical UPEC isolate strain and a CNF1-deletion mutant. We found no alterations in bacterial burden with the loss of CNF1, whereas loss of the virulence factor fimH decreased bacterial burdens. In addition, we found no evidence that CNF1 contributed to the recruitment of inflammatory infiltrates in the kidney or bladder in vivo. CONCLUSIONS: While further examination of CNF-1 may reveal a role in UTI pathogenesis, our data casts doubt on the role of CNF-1 in the pathogenesis of UPEC UTI. As with other infections, different models and approaches are needed to elucidate the contribution of CNF1 to E. coli UTI.

Cost and radiation exposure in the workup of febrile pediatric urinary tract infections.

BACKGROUND: Technetium-99m dimercaptosuccinic acid (DMSA) scans are often used in the evaluation of pediatric patients with febrile urinary tract infections (UTIs). Given the prevalence of febrile UTIs, we sought to quantify the cost, radiation exposure, and clinical utility of DMSA scans when compared with dedicated pediatric renal ultrasounds (RUSs). MATERIALS AND METHODS: An institutional review board approved retrospective study of children under the age of 18 years evaluated at our institution for febrile UTIs between the years 2004-2013 was conducted. The patients had to meet all of the following inclusion criteria: a diagnosis of vesicoureteral reflux, a fever >38°C, a positive urine culture, and evaluation with a DMSA scan and RUS. A chart review was used to construct a cost analysis of technical and professional fees, radiographic results, and radiation dose equivalents. RESULTS: Overall, 104 children met the inclusion criteria. A total of 122 RUS and 135 DMSA scans were performed. The technical costs of a DMSA scan incurred a 35% cost premium as compared to an RUS. The average effective radiation dose of a single DMSA scan was 2.84 mSv. New radiographic findings were only identified on 7% of those patients who underwent greater than 1 DMSA scan. CONCLUSIONS: The utility of the unique information acquired from a DMSA scan as compared to a RUS in the evaluation of febrile UTI must be evaluated on an individual case-by-case basis given the increased direct costs and radiation exposure to the patient.

Profound Hematuria in a Toddler Yields an Unusual Diagnosis.

Herein we present a rare case of profound recurrent gross hematuria in a young child with no known predisposing event. She was eventually diagnosed with a large lymphovascular malformation of the bladder. She underwent multiple unsuccessful attempts at embolization before eventual curative partial cystectomy.

Presentation of a Clitoral Mass in a Prepubescent Female: A Case Report and Discussion of the Evaluation.

Clitoral masses are rare. We present a case report of a prepubescent female with a periclitoral mass.

Is There Hope for Renal Growth on Imaging Studies Following Ureteral Reimplant for Boys With Fetal Hydronephrosis and Urinary Reflux?

Reflux nephropathy is thought to be the etiology for renal maldevelopment. We present two boys with fetal hydronephrosis and sterile vesicoureteral reflux (VUR). There was lack of renal growth of the refluxing renal units on surveillance renal ultrasound. Parents elected to undergo open ureteral reimplants. Post-surgical ultrasounds demonstrated improved renal growth.

Persistent Urinary Incontinence: A Case Series of Missed Ectopic Ureters.

Ectopic ureter is a rare cause of urinary incontinence in children. Symptomatic cases are unlikely to resolve spontaneously, and incontinence are often associated with significant psychological impact on these children. This case series of 2 older children with missed ectopic ureters by the urologic community outline workup for persistent urinary incontinence, with emphasis on history, physical, embryology review, and radiographic evaluations.

Impact of nephrectomy on long-term renal function in non-syndromic children treated for unifocal Wilms tumor.

OBJECTIVE: The present study is designed to assess the long-term renal function of children who underwent radical nephrectomy for unifocal Wilms tumor. METHODS: A single institution retrospective cohort study of non-syndromic children treated with radical nephrectomy for unifocal Wilms tumor between 1995 and 2011 was performed to identify risk factors for decreased glomerular filtration rate (GFR). The primary endpoint was decrease in age-adjusted GFR below normal published ranges. The secondary endpoint was progression to chronic renal insufficiency (CRI). RESULTS: A total of 55 patients were identified in the cohort. Eight (15%) patients exhibited decreased age-adjusted GFR during the follow-up period, with 2 (4%) progressing to CRI. Increasing time between surgery and the last known GFR follow-up was associated with decreased GFR, with the normal GFR group having median follow-up of 7.32 years versus 11.47 years (p = 0.019) in the decreased GFR group. CONCLUSIONS: A trend toward decline in GFR was detected with longer follow-up. Longer follow-up may reveal that clinically significant decline in renal function occurs years following nephrectomy among a subset of Wilms tumor survivors, even among those who do not progress to end stage renal disease.

Ureteroscopy for treatment of upper urinary tract stones in children: technical considerations.

The incidence of pediatric urolithiasis is increasing. While many smaller stones may pass spontaneously, surgical therapy is sometimes warranted. Surgical options include shock wave lithotripsy, ureteroscopy, percutaneous nephrolithotomy, and open surgery. Ureteroscopy represents a minimally invasive approach, and it is increasingly being used to treat pediatric upper tract calculi. Ureteroscopy is performed under anesthesia and fluoroscopic guidance, with basket extraction or lithotripsy of the calculi. Technical considerations include active or passive ureteral dilatation, the use of ureteral access sheaths for larger stone burdens, and post-operative stent placement. The current pediatric literature suggests high success rates (equal to or surpassing shock wave lithotripsy) and low complication rates. However, concerns remain regarding feasibility in patients with variant anatomies and risk due to intra-operative radiation exposure.

Cytotoxic necrotizing factor 1 contributes to Escherichia coli meningitis.

E. coli is the most common Gram-negative bacteria causing neonatal meningitis, and E. coli meningitis continues to be an important cause of mortality and morbidity throughout the world. Recent reports of E. coli meningitis caused by antimicrobial resistant strains are a particular concern. These findings indicate that a novel strategy is needed to identify new targets for prevention and therapy of E. coli meningitis. Cytotoxic necrotizing factor 1 (CNF1) is a bacterial virulence factor associated principally with E. coli strains causing urinary tract infection and meningitis. We have shown that CNF1 contributes to E. coli invasion of the blood-brain barrier and penetration into the brain, the essential step in the development of E. coli meningitis, and identified the host receptor for CNF1, 37-kDa laminin receptor precursor (37LRP). CNF1, however, is a cytoplasmic protein and its contribution to E. coli invasion of the blood-brain barrier requires its secretion from the bacterial cytoplasm. No signal peptide is found in the CNF1 sequence. CNF1 secretion is, therefore, a strategy utilized by meningitis-causing E. coli to invade the blood-brain barrier. Elucidation of the mechanisms involved in CNF1 secretion, as shown in this report with the involvement of Fdx and YgfZ provides the novel information on potential targets for prevention and therapy of E. coli meningitis by virtue of targeting the secretion of CNF1.

Utility of screening ultrasound after first febrile UTI among patients with clinically significant vesicoureteral reflux.

OBJECTIVE: To assess the sensitivity and negative predictive value (NPV) of screening renal and bladder ultrasound (RBUS) after initial febrile urinary tract infection (UTI) among patients with clinically significant vesicoureteral reflux (VUR). METHODS: A retrospective review was performed of all children <2 years of age who presented with a febrile UTI between 2004 and 2011. The sensitivity and NPV of initial RBUS was calculated among patients who were found to have high-grade (IV-V) VUR. Additionally, initial RBUS among patients with evidence of photopenia on dimercaptosuccinic acid (DMSA) scan or who underwent surgical intervention were reviewed. RESULTS: One hundred forty-four patients with febrile UTI were identified; available RBUS, voiding cystourethrogram (VCUG), and DMSA results for each kidney were reviewed. One hundred fifty-eight kidneys had evidence of VUR on VCUG, and initial RBUS demonstrated abnormality in 25 (sensitivity 0.17). Forty-five kidneys had high-grade VUR and RBUS revealed abnormality in 16 (sensitivity 0.36). One hundred seventy-eight kidneys had no evidence of abnormality on initial RBUS, and 136 (76%) were found to have VUR (NPV 0.24), of which 31 had high-grade VUR (NPV 0.83). Seven kidneys had scarring on DMSA and initial RBUS was normal in 4 (57%). Twelve of 19 patients (63%) who eventually underwent surgical intervention had a normal initial RBUS. CONCLUSION: RBUS has poor sensitivity and NPV for detecting high-grade VUR in patients <2 years who present with a febrile UTI. A significant number of patients who were diagnosed with high-grade VUR, renal scarring, or underwent surgical correction of VUR had a negative screening RBUS.