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.

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.

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.

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.

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.

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.

ACGME core competency training, mentorship, and research in surgical subspecialty fellowship programs.

OBJECTIVE: To determine the perceived effectiveness of surgical subspecialty training programs in teaching and assessing the 6 ACGME core competencies including research. DESIGN: Cross-sectional survey. SETTING: ACGME approved training programs in pediatric urology and colorectal surgery. PARTICIPANTS: Program Directors and recent trainees (2007-2009). RESULTS: A total of 39 program directors (60%) and 57 trainees (64%) responded. Both program directors and recent trainees reported a higher degree of training and mentorship (75%) in patient care and medical knowledge than the other core competencies (p<0.0001). Practice based learning and improvement, interpersonal and communication, and professionalism training were perceived effective to a lesser degree. Specifically, in the areas of teaching residents and medical students and team building, program directors, compared with recent trainees, perceived training to be more effective, (p = 0.004, p = 0.04). Responses to questions assessing training in systems based practice ubiquitously identified a lack of training, particularly in financial matters of running a practice. Although effective training in research was perceived as lacking by recent trainees, 81% reported mentorship in this area. According to program directors and recent trainees, the most effective method of teaching was faculty supervision and feedback. Only 50% or less of the recent trainees reported mentorship in career planning, work-life balance, and job satisfaction. CONCLUSIONS: Not all 6 core competencies and research are effectively being taught in surgery subspecialty training programs and mentorship in areas outside of patient care and research is lacking. Emphasis should be placed on faculty supervision and feedback when designing methods to better incorporate all 6 core competencies, research, and mentorship.

Pediatric urology fellowship training: are we teaching what they need to learn?

OBJECTIVE: Pediatric urology training has traditionally been based on an apprenticeship model. As part of our curriculum re-development, we surveyed recent graduates (2007-2009) regarding the teaching of clinical/surgical skills and medical knowledge during their training. METHODS: 44 pediatric urologists who completed 2 years of ACGME (Accreditation Council for Graduate Medical Education)-accredited programs and had been practicing for at least 18 months were anonymously surveyed. An IRB-approved survey was developed by a team of educators at the Johns Hopkins School of Medicine and Bloomberg School of Public Health. RESULTS: 31 of 44 responded to 100% of the questions; 90% of the respondents felt their fellowship successfully prepared them for discussing surgical options and performing the procedures that they are now doing; 74% felt well trained to manage perioperative complications and 65% felt well trained to manage non-surgical problems. Faculty feedback/supervision, independent reading, and conferences were rated as a very effective method of teaching (87%). Top three procedures they wished they had learned: laparoscopic/robotic surgery, hypospadias repair, and augmentation/Mitrofanoff. Top three non-surgical topics: urinary tract infection, voiding dysfunction, and billing/coding. CONCLUSION: It is reassuring that ACGME fellowship-trained pediatric urologists feel prepared in commonly performed procedures and perioperative care. Faculty supervision/feedback is highly valued.

Prenatal diagnosis of a paraurethral cyst.

Paraurethral cysts arising from Skene's gland are a rare cause of urogenital masses in the neonate. We report the case of a pelvic mass noted at the vaginal introitus on prenatal ultrasound that following delivery was found to be a paraurethral cyst. On prenatal ultrasound, there was no evidence of involvement of the urinary, gastrointestinal, or upper genital tract. Serial ultrasounds demonstrated slight enlargement of the cyst without other changes. The patient delivered at 33 weeks and postnatal evaluation demonstrated a paraurethral cyst. The cyst was managed expectantly and drained spontaneously on the second day of life with complete resolution.

Teaching and assessing technical proficiency in surgical subspecialty fellowships.

OBJECTIVES: To determine how programs are teaching and assessing procedural skills, and their perceived success. DESIGN: Cross-sectional survey. SETTING: Accreditation Council for Graduate Medical Education (ACGME) approved training programs in pediatric urology and colorectal surgery. PARTICIPANTS: Program directors and recent graduates (2007-2009). RESULTS: Thirty-nine program directors (60%), and 57 graduates (64%) responded; 89.5% of graduates and 94.9% of program directors felt training occurred successfully for the procedures that trainees were performing in their present practice. Nearly 90% of trainees and all program directors reported that there was no formal assessment of procedural competency at the beginning of training, although 66.7% of program directors reported that trainees were assessed "informally." Both program directors and trainees reported dialogue with faculty was the most frequent method used in preparing for operative procedures. Other methods (textbook/atlas, journals, web-based programs, videos) were used less frequently. Program directors with shorter tenure were more likely to use web-based and video methods; younger trainees were less likely to use textbooks/atlases. Faculty feedback on clinical decision-making and postprocedural review were perceived by both program directors and trainees as the most effective assessment methods for improving performance; however, trainees were more likely than program directors to report that postprocedure reviews were not included in their training (15.8% vs 9%, p = 0.045). Patient outcomes, written feedback from peers, and self-assessment were included in most programs, but valued less. Simulation was used in only about half the programs and was valued more highly by trainees than program directors (p = 0.011). CONCLUSIONS: Training in procedural proficiency was viewed as successful by both program directors and graduates. Dialogue with, assessment by, and feedback from faculty were frequently used and most valued; stressing the importance of the facilitator role of faculty in the education of the trainee. These findings provide guidance for the development of newer methods of teaching and assessment.

Urinary diversion in early childhood: indications and outcomes in the exstrophy patients.

OBJECTIVE: To evaluate indications and applicability of continent and incontinent urinary diversion (CUD and IUD, respectively) in early childhood in patients with classic bladder exstrophy (CBE). METHODS: Using an institutionally approved exstrophy database, patients with CBE born after 1980 who underwent CUD or IUD by 2 surgeons within the first 5 years of life were identified. All aspects of their care and clinical outcomes were studied. RESULTS: In the CUD group (n = 14), only 21% had successful primary closure. Indications were desire to be dry (7), persistent hydronephrosis (4), urinary tract infections (UTIs) (1), repeat CUD (1), and inaccessible proper follow-up (1). Three patients had neobladder creation, 10 had bladder augmentation with continent stomas, and 2 underwent ureterosigmoidostomy. Currently, all patients are dry with clean intermittent catheterization (CIC). In the IUD group (n = 5), only 1 had successful primary closure. In addition to small, noncontractile bladders, the indications for IUD were severe hydronephrosis (2), recurrent UTIs (2), and noncompliance with catheterization (1). Four patients were re-diverted to CUD after a mean of 9.4 years and 1 has colon conduit. All are socially dry via catheterization. There was no case of renal function loss or malignant transformation. CONCLUSION: The need for early diversion in CBE is primarily driven by upper tract changes after secondary closure and social factors. Urinary diversion can be safe in younger children with a favorable continence outcome.

Surgical management of meatal stenosis with meatoplasty.

Meatal stenosis is a common urologic complication after circumcision. Children present to their primary care physicians with complaints of deviated urinary stream, difficult-to-aim, painful urination, and urinary frequency. Clinical exam reveals a pinpoint meatus and if the child is asked to urinate, he will usually have an upward, thin, occasionally forceful urinary stream with incomplete bladder emptying. The mainstay of management is meatoplasty (reconstruction of the distal urethra /meatus). This educational video will demonstrate how this is performed.

Ectopic ureteroceles in duplex systems: long-term follow up and 'treatment-free' status.

OBJECTIVE: Definitive treatment of ectopic ureterocele (EU) implies that no further surgery or prophylactic antibiotic is needed. The literature is unclear on which interventions render a child 'treatment free'. MATERIALS AND METHODS: Thirty (23 female, seven male) patients presented between 1984 and 2000. Follow up ranged from 5 to 15 years (mean: 7). Presenting reasons were: urinary tract infection in 18 (16 females, two males; age: 17<6 months, one 2 years), prenatal ultrasound in 11 (seven females, four males), and renal failure in one (male, aged 3 weeks). RESULTS: Treatment was as follows. No intervention, three (10%). Single procedure, eight (27%): five hemi-nephrectomy (HN), two transurethral incisions (TUI), one excision and re-implantation (E&R). Two procedures, 14 (47%): first procedure 10 TUI, 4 HN; second procedure 13 E&R, 1 TUI. Three procedures, three (10%): first 2 TUI, 1 HN; second 3 TUI; third 2 E&R, 1 HN. Four procedures, two (7%): first 2 TUI; second 1 HN, 1 TUI; third 2 TUI; fourth 2 E&R. Eight (27%) remained on prophylaxis: two had no intervention, in 4 the ectopic ureterocele was in situ after HN or TUI, and two had reflux after E&R. Twenty two (73%) came off prophylaxis (16 E&R, 4 HN, 1 TUI, 1 observation). Poorly or non-functioning upper pole moieties were left in place in 14/18 who underwent E&R. CONCLUSION: 'Treatment-free' status most often requires ureterocele excision. HN alone can be definitive, while TUI alone is so rarely. Poor or non-functioning upper pole segments can remain after E&R. Children with collapsed ureteroceles in situ often must remain on antibiotic prophylaxis. A staged approach with initial TUI, followed by E&R, was successful in definitively treating the majority.

Endocrine disruptors, genital development, and hypospadias.

Hypospadias is one of the most common congenital anomalies in the United States, occurring in approximately 1 in 125 live male births. Embryological studies have demonstrated that, depending on where the urethral development arrests, the meatal opening can be anywhere along the shaft of the penis or, in more severe forms, within the scrotum or in the perineum. Currently, the only available treatment is surgery. If left uncorrected, especially in its severe form, there is risk of infertility and psychological effects, such as avoidance of intimate relationships. The cause of hypospadias is largely unknown; however, current epidemiology and laboratory studies have shed new light into the etiology of hypospadias. With recent advancements in molecular biology and microarray technology, it appears that hypospadias is potentially related to disrupted gene expression. Specifically, some of the environmental chemicals are acting as antiandrogens and interfere directly with the action of testosterone-related gene expression. In this paper, we briefly review the normal development of male external genitalia and the prevalence and environmental risk factors related to hypospadias. In addition, we discuss some of the recent laboratory findings that contribute to our current understanding of this disease.

Effects of vitamin C on androgen receptor mediated actions in human prostate adenocarcinoma cell line LAPC-4.

OBJECTIVES: To examine the effects of vitamin C (VC) on androgen receptor (AR)-mediated functions in a human prostate cancer cell line, Los Angeles prostate cancer (LAPC-4). VC is an essential dietary substance in the maintenance and preservation of vital functions in humans. However, the role of VC in prostate cancer remains to be elucidated. METHODS: Cell proliferation and the expression of two well-known androgen regulated proteins, prostate-specific antigen and human glandular kallikrein-2, were studied in the presence of VC. RESULTS: In the presence of androgen and VC, both cell growth and the expression of prostate-specific antigen and human glandular kallikrein-2 proteins were decreased. Moreover, AR-mediated transcription activity of the prostate-specific antigen gene was suppressed with VC, similar to the phenomenon observed when cells were treated with hydrogen peroxide. These effects were reversed with catalase. However, additional studies did not reveal changes in the expression level of AR protein or its androgen-binding activity with the addition of VC. CONCLUSIONS: The results of our study suggest that the pro-oxidant property of VC might be one of the mechanisms by which it modulates AR-mediated function in LAPC-4 cells.