The impact of hiring a full-time fertility navigator on fertility-related care and fertility preservation at a pediatric institution.

Fertility navigators (FNs) are important in communicating infertility risk and fertility preservation (FP) options to patients receiving gonadotoxic therapies. This retrospective study examined electronic medical records of patients with fertility consults at a large pediatric institution (2017-2019), before and after hiring a full-time FN. Of 738 patient encounters, 173 consults were performed pre-navigator and 565 post-navigator. Fertility consults for long-term follow-up cancer survivors increased most substantially: pre-navigator (n = 7) and post-navigator (n = 387). Across diagnoses, females had a larger increase in consults compared to males (χ2 [3, N = 738] = 8.17, p < .05). Findings highlight FNs' impact on counseling rates, particularly in survivorship.

Improvement in bladder function in children with functional constipation after a bowel management program.

PURPOSE: We sought to determine if children with functional constipation (FC) would have an improvement in bladder function with treatment of constipation with a bowel management program (BMP). METHODS: A single-institution review was performed in children aged 3-18 with FC who underwent a BMP from 2014 to 2020. Clinical characteristics, bowel management details, and the Vancouver Symptom Score for Dysfunctional Elimination Syndrome (VSS), Baylor Continence Scale (BCS), and Cleveland Clinic Constipation Score (CCCS) were collected. Data were analyzed using linear mixed effect modeling with random intercept. RESULTS: 241 patients were included with a median age of 9 years. Most were White 81 and 47% were female. Univariate tests showed improvement in VSS (- 3.6, P < 0.0001), BCS (- 11.96, P < 0.0001), and CCCS (- 1.9, P < 0.0001) among patients having undergone one BMP. Improvement was noted in VSS and CCCS among those with more than one BMP (VSS: - 1.66, P = 0.023; CCCS: - 2.69, P < 0.0001). Multivariate tests indicated undergoing a BMP does result in significant improvement in VSS, BCS, and CCCS (P < 0.0001). CONCLUSIONS: There is significant improvement in bladder function in children with FC who undergo a BMP. For patients with bowel and bladder dysfunction and FC, a BMP is a reasonable treatment strategy for lower urinary tract symptoms.

Robotics in Pediatric Urology.

Robotic surgery has been slow to be fully accepted in the world of pediatric urology largely because of its initial application directed towards adult use and because of the inherent high cost associated with it. However, as previously shown, it has now become the gold standard for adolescent pyeloplasty in The United States. As the adoption of robotic surgery in children has become more widespread, its use has been applied to a broader spectrum of procedures with similar success rates to standard laparoscopy. These procedures include nephrectomy, heminephrectomy, ureteral reimplantation, and ureteroureterostomy. However, it has also shown feasibility and comparable success when compared to open surgery in procedures that were previously deemed too complex to be done by standard laparoscopy. For example, bladder neck reconstruction with Mitrofanoff and Malone procedure as well as bladder augmentation. This review objective is to provide an overview of robotic surgery in pediatric urology, with a focus on the more common cases such as pyeloplasty and reimplantation as well as more complex bladder reconstruction procedures.

Complex Robotic Lower Urinary Tract Surgery in Patients with History of Open Surgery.

PURPOSE: We describe our experience with robot-assisted complex lower urinary tract reconstruction in patients with a history of open abdominal surgery. MATERIALS AND METHODS: Patients with any previous open abdominal surgery undergoing robot-assisted complex lower urinary tract reconstruction were included. Complex lower urinary tract reconstruction was defined as bladder neck reconstruction or continent catheterizable conduits or both, redo surgery at the bladder neck for persistent incontinence or any of these procedures with creation of a Malone antegrade continence enema. Ureteral and renal surgeries were excluded. Patient demographics, surgery performed, operative techniques, operative times and outcomes were assessed. RESULTS: A total of 36 patients met inclusion criteria, of whom 21 had undergone multiple laparotomies for ventriculoperitoneal shunt revision, 14 had undergone laparotomy with other adjunct procedures and 1 had undergone laparotomy with colostomy. No access injury occurred and there were 5 conversions. Mean operative time was 8.2 hours (range 4 to 12) and mean length of hospital stay was 74.9 hours (23 to 216). The first 18 cases took longer than the last 18 cases (mean 9.1 vs 7.5 hours, p = 0.002). Patients with multiple ventriculoperitoneal shunt revisions had higher conversion rates (p = 0.01) and longer mean operative times (p = 0.002). Patients with a history of multiple ventriculoperitoneal shunt revisions also had longer hospital stays (p = 0.02). CONCLUSIONS: Robot-assisted complex lower urinary tract reconstruction in patients with previous open abdominal surgery is safe and feasible. Longer operative times should be expected early in the experience of a surgeon. Patients with multiple prior ventriculoperitoneal shunt revisions had higher conversion rates and longer operative times compared to those with other indications for prior surgery.

Examining predictors and outcomes of fertility consults among children, adolescents, and young adults with cancer.

Infertility has a negative impact on quality of life among cancer survivors. Studies show establishing a fertility team results in improved patient satisfaction. A review of electronic medical records was performed to examine predictors of fertility referrals, interventions, and the impact of an opt-out consult mechanism. Findings show many patients, particularly those that are younger, are still not receiving fertility counseling despite the presence of a fertility team. Notably, patients were 3.6 times more likely to receive a consult after the opt-out. Strategies are needed to improve access to fertility related care, particularly in groups where consults are underutilized.

Transscrotal Near Infrared Spectroscopy as a Diagnostic Test for Testis Torsion in Pediatric Acute Scrotum: A Prospective Comparison to Gold Standard Diagnostic Test Study.

PURPOSE: A rapid test for testicular torsion in children may obviate the delay for testicular ultrasound. In this study we assessed testicular tissue percent oxygen saturation (%StO2) measured by transscrotal near infrared spectroscopy as a diagnostic test for pediatric testicular torsion. MATERIALS AND METHODS: This was a prospective comparison to a gold standard diagnostic test study that evaluated near infrared spectroscopy %StO2 readings to diagnose testicular torsion. The gold standard for torsion diagnosis was standard clinical care. From 2013 to 2015 males with acute scrotum for more than 1 month and who were less than 18 years old were recruited. Near infrared spectroscopy %StO2 readings were obtained for affected and unaffected testes. Near infrared spectroscopy Δ%StO2 was calculated as unaffected minus affected reading. The utility of near infrared spectroscopy Δ%StO2 to diagnose testis torsion was described with ROC curves. RESULTS: Of 154 eligible patients 121 had near infrared spectroscopy readings. Median near infrared spectroscopy Δ%StO2 in the 36 patients with torsion was 2.0 (IQR -4.2 to 9.8) vs -1.7 (IQR -8.7 to 2.0) in the 85 without torsion (p=0.004). AUC for near infrared spectroscopy as a diagnostic test was 0.66 (95% CI 0.55-0.78). Near infrared spectroscopy Δ%StO2 of 20 or greater had a positive predictive value of 100% and a sensitivity of 22.2%. Tanner stage 3-5 cases without scrotal edema or with pain for 12 hours or less had an AUC of 0.91 (95% CI 0.86-1.0) and 0.80 (95% CI 0.62-0.99), respectively. CONCLUSIONS: In all children near infrared spectroscopy readings had limited utility in diagnosing torsion. However, in Tanner 3-5 cases without scrotal edema or with pain 12 hours or less, near infrared spectroscopy discriminated well between torsion and nontorsion.

Diagnosing Testicular Torsion before Urological Consultation and Imaging: Validation of the TWIST Score.

PURPOSE: The TWIST (Testicular Workup for Ischemia and Suspected Torsion) score uses urological history and physical examination to assess risk of testis torsion. Parameters include testis swelling (2 points), hard testis (2), absent cremasteric reflex (1), nausea/vomiting (1) and high riding testis (1). While TWIST has been validated when scored by urologists, its diagnostic accuracy among nonurological providers is unknown. We assessed the usefulness of the TWIST score when determined by nonurological nonphysician providers, mirroring emergency room evaluation of acute scrotal pain. MATERIALS AND METHODS: Children with unilateral acute scrotum were prospectively enrolled in a National Institutes of Health clinical trial. After undergoing basic history and physical examination training, emergency medical technicians calculated TWIST score and determined Tanner stage per pictorial diagram. Clinical torsion was confirmed by surgical exploration. All data were captured into REDCap™ and ROC curves were used to evaluate the diagnostic usefulness of TWIST. RESULTS: Of 128 patients (mean age 11.3 years) 44 (13.0 years) had torsion. TWIST score cutoff values of 0 and 6 derived from ROC analysis identified 31 high, 57 intermediate and 40 low risk cases (positive predictive value 93.5%, negative predictive value 100%). CONCLUSIONS: TWIST score assessed by nonurologists, such as emergency medical technicians, is accurate. Low risk patients do not require ultrasound to rule out torsion. High risk patients can proceed directly to surgery, with more than 50% avoiding ultrasound. In the future emergency medical technicians and/or emergency room triage personnel may be able to calculate TWIST score to guide radiological evaluation and immediate surgical intervention at initial assessment long before urological consultation.

Bladder Management and Continence in Girls With Cloacal Malformation After 3 Years of Age.

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes. METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet. RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes. LEVEL OF EVIDENCE: IV.

Robotic-Assisted Surgery in Patients Less than 15 kg: A Single Center Review.

Introduction: Robotic-assisted surgery (RAS) is an increasingly utilized tool in children. However, utilization of RAS among infants and small children has not been well established. The purpose of this study was to review and characterize RAS procedures for children ≤15 kg. Methods: We performed a single institution retrospective descriptive analysis including all patients ≤15 kg undergoing RAS between January 2013 and July 2021. Data collection included procedure type, age, weight, gender, and surgical complications. Cases were further categorized according to surgical specialty: pediatric urology (PU), pediatric surgery (PS), and multiple specialties (MS). t-Tests were used for statistical analyses. Results: Since 2013, a total of 976 RAS were identified: 492 (50.4%) were performed by PU, 466 (47.8%) by PS, and 18 (1.8%) by MS. One hundred eighteen (12.1%) were performed on children ≤15 kg, consisting of 110 (93.2%) PU cases, 6 (5.1%) PS cases, and 2 (1.7%) MS cases. Procedures were significantly more common in the PU subgroup, mean of 12 cases/year, compared to PS subgroup, mean of 0.63 cases/year, (P < .01). The mean weight of PU patients (10.5 kg) was significantly less than PS patients (13.9 kg) (P < .01). Mean age was also significantly lower among PU patients (18.6 months) compared to PS (34.2 months) (P < .01). Conclusion: RAS among patients ≤15 kg is safe and feasible across pediatric surgical subspecialties. RAS was performed significantly more frequently by pediatric urologists in younger and smaller patients compared to pediatric surgeons. Further refinement of robotic technology and instrumentation should enhance the applicability of these procedures in this young group.

Following a strict renal protection protocol in cloacal malformations: A descriptive analysis.

BACKGROUND: In children with cloacal malformations, renal dysfunction is a constant concern, with reported incidence as high as 50%. Multiple factors exist that may impair renal function. Our institution follows a strict renal protection protocol in this population. Incidence of renal dysfunction in these patients is unknown. OBJECTIVE: We aimed to evaluate incidence of renal dysfunction while implementing this protocol in a cohort of children with cloacal malformation. STUDY DESIGN: We reviewed a prospectively collected database of children with cloacal malformations managed at a single institution since implementation of a renal protection protocol. This involves regular laboratory evaluation, appropriate selection of total urogenital mobilization or urogenital separation, proactive imaging in patients with signs of impending renal dysfunction or urinary retention, and early catheterization teaching and implementation if necessary. Glomerular filtration rate (GFR) was calculated with the Schwartz formula and CKD grades assigned per standard definitions. Renal dysfunction was defined as CKD grade 3b or higher, need for renal replacement therapy (RRT) or transplantation. Descriptive statistics were computed. RESULTS: A total of 105 children were managed under this protocol with a median follow-up of 4.2 years [IQR: 2.0-5.9]. Six children (5.7%) had renal dysfunction at most recent follow-up; of these children, only three (2.9%) progressed from normal renal function at initial evaluation to renal dysfunction (Table). No child with normal presenting renal function thus far has progressed to require dialysis or transplantation. DISCUSSION: Previous literature estimated rates of renal dysfunction in cloaca patients as high as 50%; in contrast, we demonstrate a rate of progression to renal dysfunction of 2.9% in girls following a strict renal protection protocol. Most children who developed renal dysfunction had dysfunctional kidneys on presentation. This suggests that preservation of renal function may be possible in early childhood with a strict, multi-disciplinary renal protection protocol. CONCLUSION: In our cohort of patients with cloacal malformations following a strict renal protection protocol, incidence of progressive renal dysfunction is low at 2.9%. Most who go on to renal dysfunction present with impaired renal function.

Robotic-assisted Pediatric Thoracic and Abdominal Tumor Resection: An Initial Multi-center Review.

INTRODUCTION: Robotic-assisted minimally invasive surgery (RA-MIS) for tumor resection is an emerging technology in the pediatric population with significant promise but unproven safety and feasibility. METHODS: A multi-center retrospective review of patients ≤18 years undergoing RA-MIS tumor resection from December 2015-March 2023 was performed. Patient demographics, perioperative variables, and complication rates were analyzed. RESULTS: Thirty-nine procedures were performed on 38 patients (17 thoracic, 22 abdominal); 37% female and 68% non-Hispanic White. Median age at surgery was 8.3 years (IQR 5.7, 15.7); the youngest was 1.7 years-old. Thoracic operations included resections of neuroblastic tumors (n = 16) and a single paraganglioma. The most common abdominal operations included resections of neuroblastic tumors (n = 5), pheochromocytomas (n = 3), and angiomyolipomas (n = 3). Six patients underwent retroperitoneal lymph node dissection (RPLND) for paratesticular tumors. Median operating time for the cohort was 2:52 h (IQR 2:04, 4:31). Two thoracic cases required open conversion due to poor visualization and lack of working domain. All patients underwent complete tumor resection; one had tumor spillage from a positive margin (Wilms tumor). Median LOS was 1.5 days (IQR 1.1, 3.0). Postoperatively, one patient developed a chyle leak requiring interventional radiology drainage, but none required a return to the operating room. CONCLUSIONS: Robotic-assisted surgery is safe and feasible for tumor resection in carefully selected pediatric patients, achieving complete resection with minimal morbidity and short LOS. Resection should be performed by those with robotic expertise for optimal outcomes. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Original Clinical Research.

Genetic abnormalities in FOXP1 are associated with congenital heart defects.

The etiology for the majority of congenital heart defects (CHD) is unknown. We identified a patient with unbalanced atrioventricular septal defect (AVSD) and hypoplastic left ventricle who harbored an ~0.3 Mb monoallelic deletion on chromosome 3p14.1. The deletion encompassed the first four exons of FOXP1, a gene critical for normal heart development that represses cardiomyocyte proliferation and expression of Nkx2.5. To determine whether FOXP1 mutations are found in patients with CHD, we sequenced FOXP1 in 82 patients with AVSD or hypoplastic left heart syndrome. We discovered two patients who harbored a heterozygous c.1702C>T variant in FOXP1 that predicted a potentially deleterious substitution of a highly conserved proline (p.Pro568Ser). This variant was not found in 287 controls but is present in dbSNP at a 0.2% frequency. The orthologous murine Foxp1 p.Pro596Ser mutant protein displayed deficits in luciferase reporter assays and resulted in increased proliferation and Nkx2.5 expression in cardiomyoblasts. Our data suggest that haploinsufficiency of FOXP1 is associated with human CHD.

Tunica Vaginalis Graft Use for Repair of Traumatic Testicular Rupture: A Case Report.

Testicular rupture is a rare but serious condition which requires immediate and emergent surgical intervention in order to preserve fertility and maintain gonadal hormone function. We present here a case of a 16-year-old male who suffered a shattered right testicle following gunshot wound. Additionally, the left cord structures were also hit with possible compromise of the left testicle. He underwent scrotal exploration with reconstruction of the right tunica albuginea with a tunica vaginalis graft. The right testicle was found to be viable within 2months postoperatively with normal arterial and venous flow seen on Doppler scrotal ultrasound. We propose that tunica vaginalis can be used successfully as a graft to manage testicular rupture.

Outcomes after dextranomer/hyaluronic acid bladder neck injection in patients with urethral incontinence following bladder neck repair.

INTRODUCTION: Many patients who undergo surgery for bladder neck (BN) incompetence may still experience incontinence postoperatively. Dextranomer/hyaluronic acid (Dx/HA) is widely used for endoscopic treatment of vesicoureteral reflux in children; however, few studies have reported its use in treating incontinence after BN surgery. OBJECTIVE: The aim of this study was to evaluate outcomes after Dx/HA bladder neck injection in patients with persistent outlet incompetency following BN Repair. STUDY DESIGN: We retrospectively reviewed patients at a single pediatric tertiary care center with history of prior bladder neck surgery and reported persistent incontinence who then underwent endoscopic bladder neck Dx/HA injection from 2013 to 2018 and had subsequent follow-up post-injection. We described primary outcomes of reported incontinence as "wet" (leakage similar to before injection), "improved" (wet but leakage improved), and "dry" (no leakage). Our secondary outcome was need for a secondary procedure after Dx/HA injection, including Dx/HA injection or bladder neck closure (BNC). RESULTS: At first follow-up (median 2.3 months post-op), 7/19 were wet, 6/19 were improved, and 6/19 were dry. At last follow-up (median of 34.7 months), only three patients (16%) were dry. Only one patient who received a single surgery for Dx/HA injection was "dry", though nearly 2/3rds (12) were initially "improved" in their continence. Overall, seven patients had another intervention or surgery after first injection. Five patients had multiple Dx/HA injections after first procedure, which resulted in dryness by last assessment in two of these. Four patients (21%) in the overall cohort required subsequent BNC. DISCUSSION: Longer-term follow-up in our study demonstrated that only one patient who received a single procedure of Dx/HA injection remained dry. Several studies had follow up for greater than 1 year after initial bladder neck Dx/HA injection; rates of dryness after a single surgery for injection were variable and reported between 20 and 40% over follow-up times ranging from 1.5 to 7 years (Alova et al., 2012; DaJusta et al., 2013; Lottmann et al., 2006a; Lottmann et al., 2006b; Kitchens et al., 2007). Our st udy does have inherent limitations. This study was performed at a single institution in a retrospective manner, with a single surgeon reviewing the medical record to determine operative techniques and continence outcomes. The patient population is small, although relatively comparable to other previously reported studies. Outcomes were based on documentation of patient reports and are therefore lacking in objectivity. CONCLUSION: In patients with prior unsuccessful BN repair, long-lasting dryness after single Dx/HA BN injection is unlikely, although one-third may demonstrate relatively durable improvement in incontinence.

Reducing Opioid Prescriptions after Common Outpatient Pediatric Urologic Surgeries: A Quality Improvement Assessment.

Pediatric patients undergoing outpatient surgeries often receive prescriptions for postoperative pain, including opioid medications. As a result, the American Academy of Pediatrics formally challenged all pediatric surgeons to decrease opioid prescribing for common specialty-specific outpatient procedures at discharge. To meet this challenge, we designed a quality improvement project to decrease the average number of opioid doses administered to pediatric patients undergoing 3 common outpatient urologic surgeries: circumcision, orchiopexy, and inguinal hernia repair (IHR). Methods: We formally challenged providers at our institution to reduce opioid doses per prescription and administration to patients overall. We performed a retrospective chart review at our single pediatric institution to establish baseline opioid prescribing values from July 2017 to March 2018. We aimed to reduce this value by 50% in 6 months and sustain this decrease throughout the project duration. Results: We performed 1,518 orchiopexies, 1,505 circumcisions, and 531 IHRs. The percent change in the average number of opioid doses prescribed per patient from baseline values assessed to 2021 was statistically significant for orchiopexies (P < 0.0001), IHRs (P < 0.0001), and circumcisions (P < 0.0001). In addition, the change in the percentage of patients prescribed opioids from baseline was statistically significant for all 3 procedures (P < 0.001). Conclusions: This project demonstrated that through an organized quality improvement initiative, the average number of opioid medications prescribed and the total percentage of patients prescribed opioids following common outpatient pediatric urologic procedures can be decreased by at least 50% and sustained through project duration.

Robotic retroperitoneal lymph node dissection for paratesticular rhabdomyosarcoma in adolescents: a case series.

Robotic assisted (RA) retroperitoneal lymph node dissection (RPLND) has grown in popularity as it offers decreased morbidity and faster recovery compared to the open technique. Proponents of open surgery raised concerns about the oncological fidelity of the RA approach for testicular tumors where complete resection is needed. In boys > 10 years with paratesticular rhabdomyosarcoma (RMS), RPLND is indicated for staging purposes only. In this population, the RA technique should provide its benefits without concerns for oncological compromise. We present an analysis of RA-RPLND for boys with paratesticular RMS. We queried our institution's prospectively collected database of pediatric robotic cases for patients undergoing RA-RPLND post-radical orchiectomy for paratesticular mass, confirmed by pathology as RMS. Demographic, surgical, follow-up, and oncological outcomes were evaluated between 2017 and 2023. Five patients underwent RA-RPLND for paratesticular RMS. The median age was 16.1 years (15-17), with median OR time of 456 min (357-508). No conversions to open occurred. Inpatient median total opioid use was 1.8 (0.4-2.7) morphine equivalent/kg. The median lymph node yield was 27 (8-44) and post-op length of stay was 3 days (2-5). The median time to initiating adjuvant chemotherapy was 10.5 days (7-13). One patient had complications: pneumothorax attributed to central line placement and chyle leak that resolved in 1 week with dietary restriction. Our series demonstrates the feasibility, safety, and efficacy of the RA approach for RPLND in pediatric patients with paratesticular RMS. This is the most extensive case series currently in the literature and the only one exclusively done for paratesticular RMS.