Fetal bladder morphology as a predictor of outcome in fetal lower urinary tract obstruction.

OBJECTIVE: We evaluate survival of fetuses with severe Lower Urinary Tract Obstruction (LUTO) based on bladder morphology. We hypothesize that fetuses with a "floppy" appearing bladder on initial prenatal ultrasound will have worse infant outcomes than fetuses with full/rounded bladders. METHOD: We retrospectively reviewed all cases of LUTO evaluated in our fetal center between January 2013 and December 2021. Ultrasonographic assessment, renal biochemistry, and bladder refilling contributed to a "favorable" or "unfavorable" evaluation. Bladder morphology on initial ultrasound was classified as "floppy" or "full/rounded." Vesicoamniotic shunting was offered for favorably evaluated fetuses. Baseline demographics, ultrasound parameters, prenatal evaluations of fetal renal function, and infant outcomes were collected. Fetuses diagnosed with severe LUTO were included in analysis using descriptive statistics. The primary outcome measured was survival at 6 months of life. RESULTS: 104 LUTO patients were evaluated; 24 were included in analysis. Infant survival rate at 6 months was 60% for rounded bladders and 0% for floppy bladders (p = 0.003). Bladder refill adequacy was lower in fetuses with floppy bladders compared with rounded bladders (p value < 0.00001). CONCLUSION: We propose that bladder morphology in fetuses with severe LUTO may be a prognostication factor for predicting infant outcomes and provides a valuable, noninvasive assessment tool.

Novel scoring system for determining fetal candidacy for prenatal intervention for severe congenital lower urinary tract obstruction.

OBJECTIVES: To evaluate a novel scoring system that combines several prenatal parameters for selecting ideal candidates for fetal intervention, and for predicting postnatal survival in patients with severe fetal lower urinary tract obstruction (LUTO). METHODS: We retrospectively reviewed all cases of severe LUTO evaluated for fetal intervention in a single large fetal center between January 2013 and December 2017. A scoring system for determining fetal candidacy for intervention was retrospectively developed based on postnatal outcomes. The proposed scoring system included fetal urinary biochemistry, renal ultrasound parameters, initial bladder volume, and degree of bladder refill. Relevant demographic characteristics, ultrasound reports and laboratory results were reviewed. Receiver operating characteristic (ROC) curves were used to select the cut-off values for initial bladder volume and degree of bladder refill and to evaluate the performance of the scoring system in predicting postnatal death. RESULTS: Of the 79 LUTO patients evaluated, 31 were eligible for the study. The overall 6-month postnatal survival was 64.5 % (20/31). A scoring system (0-8) was suggested with 2 points for unfavorable biochemistry, 4 points for ultrasound evidence of dysplastic kidneys, 1 point for inadequate initial bladder volume and 1 point for inadequate bladder refill. Scores>3 (N = 7) were associated with 0 % 6-month survival. The ROC curve for predicting postnatal mortality showed area under curve (AUC) of 0.82 (95 % CI 0.65-0.99). Subgroup analysis within subjects who underwent fetal intervention (N = 22) also confirmed the significance of the distribution of the scoring system between groups who survived and those who did not after adjustment for GA at delivery (p = 0.01). CONCLUSION: We propose a novel scoring system for antenatal evaluation of patients with severe LUTO which may be useful in selecting those candidates most appropriate for intervention and in counseling parents about predicted postnatal outcome.

Neurodevelopmental Outcome in Infants with Lower Urinary Tract Obstruction Based on Different Degrees of Severity.

OBJECTIVE: To determine postnatal neurodevelopmental (ND) outcomes for children with congenital lower urinary tract obstruction (LUTO) based on disease severity. METHODS: Twenty male infants with LUTO were classified prenatally as Stage 1 (normal amniotic fluid and renal function; n = 5), Stage 2 (signs of obstruction with preserved renal function; n = 13), and Stage 3 (signs of severe renal damage; n = 2). ND status was assessed using the Developmental Profile-3 test in 5 developmental domains (physical, adaptive behavior, social-emotional, cognitive, and communication). Each domain was considered to be delayed if standard scores were 2 or more SD below the mean. ND outcomes were compared between cases with an expected normal renal function (LUTO Stage 1) and those with impaired renal function (LUTO Stages 2 and 3). Results from cases with Stage 2 were also compared to those from Stage 3. ORs were calculated to predict risk for adverse ND outcome for each domain considering prenatal and postnatal factors. RESULTS: Gestational age (GA) at the diagnosis of LUTO was similar between both groups (Stage 1: 24.85 ± 7.87 vs. Stages 2 and 3: 21.4 ± 4.31 weeks; p = 0.24). Twelve of 15 cases with Stage 2 or 3 underwent vesicoamniotic shunt placement compared to none of Stage 1 fetuses (p < 0.01). No differences in GA at delivery were detected between the groups (37.9 ± 1.6 vs. 35.1 ± 3.6 weeks; p = 0.1). One of the infants in the Stage 2 and 3 groups received a kidney transplant during follow-up. One case (20%) from Stage 1 group required dialysis during the first 6 months of life, and 1 case from Stage 2 to 3 group required it during the first 6 months (p = 1.0), whereas 3 additional cases needed dialysis from 6 to 24 months (p = 0.6). Mean age at Developmental Profile 3 (DP-3) testing was 20.3 ± 12.3 months (Stage 1: 11.2 ± 8.6 vs. Stages 2 and 3: 23.4 ± 13.4 months; p = 0.07). Fifteen of the 20 patients (75%) had no ND delays. Of the 5 patients with ND delays, 4 received dialysis. No differences in ND outcomes between infants with LUTO Stage 1 and those with Stages 2 and 3 were detected except for a trend toward better physical development in Stage 1 (102.6 ± 11.6 vs. 80.7 ± 34.9; p = 0.05). Infants diagnosed with LUTO Stage 3 showed significantly lower adaptive scores than those diagnosed with Stage 2 (Stage 2: 101.9 ± 22.3 vs. Stage 3: 41.5 ± 30.4; p = 0.04) and a nonsignificant trend for lower results in physical (85.8 ± 33.0 vs. 47.5 ± 38.9; p = 0.1) and socio-emotional (94.7 ±17.9 vs. 73.5 ± 13.4; p = 0.1) domains. Infants who received dialysis showed 15-fold increased risk (95% CI 0.89-251) for delayed socio-emotional development (p = 0.06). Diagnosis of fetal renal failure increased the risk for delays in the adaptive domain 30-fold (95% CI 1.29-93.1; p = 0.03). Infants with abnormal renal function had 19 times (95% CI 1.95-292) increased risk for delays in the physical domain (p = 0.03). CONCLUSIONS: While most patients with LUTO do not exhibiting ND delays, our results support the importance of ND monitoring, especially in severe forms of LUTO, as increased severity of this condition may be associated with poorer ND outcomes.

Open versus robot-assisted laparoscopic ureteral reimplantation: Hospital charges analysis and outcomes at a single institution.

PURPOSE: Robot-assisted laparoscopic extravesical ureteral reimplantation has previously been described as a viable minimally invasive option to open surgery. However, concerns for robotic surgery have been raised owing to assumed higher costs and heterogeneous clinical outcomes. We hypothesized that similar hospital charges and clinical outcomes occur when comparing open and robotic cases in matched cohorts. MATERIALS AND METHODS: Open and robotic reimplantation cases from 2013 to 2015 for primary vesicoureteral reflux were matched by age using 1:1 nearest neighbor matching. The matched cohorts were analyzed and compared for their direct itemized hospital charges per surgical case, complications, and clinical outcomes. RESULTS: There were 38 patients in each group after age-matching the 135 patients. Operating room charges were higher for the robotic group compared to the open group (p=0.002), whereas pharmacy and laboratory costs were lower for the robotic group. However, there were no significant differences in total overall charges between the open and robotic groups with cystoscopy or without cystoscopy (p=0.345, p=0.533), since the median hospital stay length was shorter for the robotic group (p<0.001). Clinical success rates were identical for the two groups (open 94.8% vs robotic 94.8%). There were also no significant differences in number of complications between the two cohorts. CONCLUSIONS: This is the first age-matched study comparing hospital charges and clinical outcomes of pediatric open and robotic reimplantation. While operating room charges were higher for the robotic cohort, lower hospitalization charges led to comparable overall hospital charges, as well as equivalent clinical outcomes for both cohorts. LEVEL OF STUDY: Level III (Retrospective comparative study) TYPE OF STUDY: Retrospective Study.

Evaluating Clinical Outcomes of an Advanced Practice Provider-Led Newborn Circumcision Clinic.

OBJECTIVE: To determine the safety and efficacy of advanced practice provider (APP)-performed newborn circumcisions (NBCs), we reviewed outcomes of NBCs performed by pediatric urologists and APPs. We hypothesize comparable clinical outcomes between the groups. METHODS: All urology performed NBCs during a 5-year period were reviewed, including time surrounding implementation of the APP-led clinic. Return to emergency department (ED) rates, return to operating room (OR) rates, and intraprocedure bleeding requiring intervention were reviewed. Fisher exact and Mann-Whitney testing were utilized. RESULTS: There were no statistically significant differences in rates of intraprocedure bleeding, return to ED in 30days, return to OR for revision or other related penile surgery, or the overall number of patients with complications between the groups. Thirteen patients had complications in the APP cohort, compared to 8 in the urologist cohort. There was a difference in age and weight, with urologists performing NBCs on older and heavier patients. There was no difference in clinical outcomes between children over and under 10 pounds (4.5 kg). There was a significant difference in the need for revision circumcision when comparing children older vs younger than 30days (1.9% vs 0%, P = 0.034). CONCLUSION: An APP-led NBC clinic is both safe and feasible. The widely used age and weight cutoffs for NBC need to be further evaluated, as there was no significant difference in clinical outcomes. This practice design provides pediatric urologists more time to focus on the most complex patients, both in the clinic and OR.

Surgical interventions and anesthesia in the 1st year of life for lower urinary tract obstruction.

BACKGROUND: Patients with a prenatal diagnosis of lower urinary tract obstruction (LUTO) may undergo prenatal interventions, such as vesicoamniotic shunt (VAS) placement, as a temporary solution for relieving urinary tract obstruction. A recent FDA communication has raised awareness of the potential neurocognitive adverse effects of anesthesia in children. We hypothesized as to whether a prenatal LUTO staging system was predictive of the number of anesthesia events for prenatally diagnosed LUTO patients. METHODS: We retrospectively reviewed the prenatal and postnatal clinical records for patients with prenatally diagnosed LUTO from 2012 to 2015. Patients were stratified by prenatal VAS status and by LUTO disease severity according to Ruano et al. (Ultrasound Obstet Gynecol. 2016). RESULTS: 31 patients were identified with a prenatal LUTO diagnosis, and postnatal records were available for 21 patients (seven patients in each stage). When combining prenatal and postnatal anesthesia, there was a significant difference in the number of anesthesia encounters by stage (1.6, 3.7, and 6.7 for Stage I, II, and III respectively, p = .034). Upon univariate analysis, higher gestational age (GA) at birth was associated with a decreased number of anesthesia events in the first year (p = .031). CONCLUSIONS: The majority of infants with prenatally diagnosed LUTO will undergo postnatal procedures with general anesthesia exposure in the first year of life. Patients with higher prenatal LUTO severity experienced a higher number of both prenatal and postnatal anesthesia encounters. In addition, higher GA at birth was associated with fewer anesthesia encounters in the first year. LEVEL OF EVIDENCE: This is a prognostic study with Level IV evidence.

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.

Evaluating Natural History and Follow Up Strategies for Non-obstructive Urolithiasis in Pediatric Population.

Objective: While small non-obstructive stones in the adult population are usually observed with minimal follow-up, the same guidelines for management in the pediatric population have not been well-studied. We evaluate the clinical outcomes of small non-obstructing kidney stones in the pediatric population to better define the natural history of the disease. Methods: In this IRB-approved retrospective study, patients with a diagnosis of kidney stones from January 2011 to March 2017 were identified using ICD9 and ICD10 codes. Patients with ureteral stones, obstruction, or stones >5 mm in size were excluded. Patients with no follow-up after initial imaging were also excluded. Patients with a history of stones or prior stone interventions were included in our population. Frequency of follow-up ultrasounds while on observation were noted and any ER visits, stone passage episodes, infections, and surgical interventions were documented. Results: Over the 6-year study period, 106 patients with non-obstructing kidney stones were identified. The average age at diagnosis was 12.5 years and the average stone size was 3.6 mm. Average follow-up was 17 months. About half of the patients had spontaneous passage of stones (54/106) at an average time of 13 months after diagnosis. Stone location did not correlate with spontaneous passage rates. Only 6/106 (5.7%) patients required stone surgery with ureteroscopy and/or PCNL at an average time of 12 months after initial diagnosis. The indication for surgery in all 6 cases was pain. 17/106 (16%) patients developed febrile UTIs and a total of 43 ER visits for stone-related issues were noted, but no patients required urgent intervention for an infected obstructing stone. Median interval for follow-up was every 6 months with renal ultrasounds, which then was prolonged to annual follow up in most cases. Conclusions: The observation of pediatric patients with small non-obstructing stones is safe with no episodes of acute obstructive pyelonephritis occurring in these patients. The sole indication for intervention in our patient population was pain, which suggests that routine follow-up ultrasounds may not be necessary for the follow-up of pediatric non-obstructive renal stones ≤5 mm in size.

Corrigendum: Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

[This corrects the article DOI: 10.3389/fped.2018.00180.].

Does the use of 5 mm instruments affect the outcomes of robot-assisted laparoscopic pyeloplasty in smaller working spaces? A comparative analysis of infants and older children.

INTRODUCTION: Pediatric robot-assisted laparoscopic (RAL) pyeloplasty has become a viable minimally invasive surgical option for ureteropelvic junction obstruction (UPJO) based on its efficacy and safety. However, RAL pyeloplasty in infants can be a challenging procedure because of the smaller working spaces. The use of the larger 8 mm instruments for these patients instead of the 5 mm instruments is common because of the shorter wrist lengths. OBJECTIVE: We hypothesized that the use of 5 mm instruments for RAL pyeloplasty in infants with smaller working spaces will have comparable perioperative parameters and surgical outcomes in comparison with older children with larger working spaces. STUDY DESIGN: We compared the perioperative parameters and surgical outcomes of RAL pyeloplasties performed by a single surgeon in infants and non-infant pediatric patients over a 2 year period. All of the procedures were performed using an 8.5 mm camera and 5 mm robotic instruments. Patient demographics, operative times, perioperative complications, hospital pain medication usage, hospital length of stay, and treatment success rates were compared between the two groups. RESULTS: A total of 65 pediatric RAL pyeloplasties were included in the study (16 infants and 49 non-infants, Table). There were no significant differences in gender, laterality, proportion of re-do pyeloplasty, or preoperative hydronephrosis grade between the two groups. All procedures were performed without conversion to open surgery or significant perioperative complications. There were no differences in segmental operative times (total operative time, console time, port placement time, time for dissection to UPJO, and anastomosis time), hospital pain medication usage, and hospital length of stay between the two groups (p > 0.05 for all comparisons). The treatment success rates were 93.8% (15/16) and 100% (49/49), respectively (p = 0.08). DISCUSSION: We present the first comparative study of infant and non-infant pediatric RAL pyeloplasty using 5 mm robotic instruments. An advantage of the current study is the use of a single surgeon's experience to compare RAL pyeloplasty outcomes in infants with those of older children, a group in which RAL pyeloplasty has already been shown to be efficacious and safe. Operative tips for infant RAL pyeloplasty are also provided. CONCLUSIONS: RAL pyeloplasty is a safe and effective surgical modality even in infants, with comparable perioperative parameters and outcomes as those in older children. The use of 5 mm instruments in infants does not affect outcomes and offers the potential for improved cosmesis.

Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

Objective: Review outcomes of Prune Belly Syndrome (PBS) with the hypothesis that contemporary management improves mortality. Methods: A retrospective chart review of inpatient and outpatient PBS patients referred between 2000 and 2018 was conducted to assess outcomes at our institution. Data collected included age at diagnosis, concomitant medical conditions, imaging, operative management, length of follow-up, and renal function. Results: Forty-five PBS patients presented during these 18 years. Prenatal diagnoses were made in 17 (39%); 65% of these patients underwent prenatal intervention. The remaining patients were diagnosed in the infant period (20, 44%) or after 1 year of age (8, 18%). Twelve patients died from cardiopulmonary complications in the neonatal period; the neonatal mortality rate was 27%. The mean follow-up among patients surviving the neonatal period was 84 months. Forty-two patients had at least one renal ultrasound (RUS); of the 30 patients with NICU RUSs, 26 (89%) had hydronephrosis and/or ureterectasis. Of the 39 patients who underwent voiding cystourethrogram (VCUG), 28 (62%) demonstrated VUR. Fifty-nine percent had respiratory distress. Nine patients (20%) were oxygen-dependent by completion of follow up. Thirty-eight patients (84%) had other congenital malformations including genitourinary (GU) 67%, gastrointestinal (GI) 52%, and cardiac 48%. Sixteen patients (36%) had chronic kidney disease (CKD) of at least stage 3; three patients (7%) had received renal transplants. Eighty-four percent of patients had at least one surgery (mean 3.4, range 0-6). The most common was orchiopexy (71%). The next most common surgeries were vesicostomy (39%), ureteral reimplants (32%), abdominoplasty (29%), nephrectomy (25%), and appendicovesicostomy (21%). After stratifying patients according to Woodard classification, a trend for 12% improvement in mortality after VAS was noted in the Woodard Classification 1 cohort. Conclusions: PBS patients frequently have multiple congenital anomalies. Pulmonary complications are prevalent in the neonate while CKD (36%) is prevalent during late childhood. The risk of CKD increased significantly with the presence of other congenital anomalies in our cohort. Mortality in childhood is most common in infancy and may be as low as 27%. Contemporary management of PBS, including prenatal interventions, reduced the neonatal mortality rate in a subset of our cohort.

Quantifying the Additional Difficulty of Pediatric Robot-Assisted Laparoscopic Re-Do Pyeloplasty: A Comparison of Primary and Re-Do Procedures.

BACKGROUND: Re-do pyeloplasty after failed open or laparoscopic ureteropelvic junction (UPJ) obstruction correction can be a challenging procedure because of scar formation at the previous anastomosis site and decreased vascularity of the ureter. This study compared the perioperative parameters for pediatric robot-assisted laparoscopic (RAL) primary and re-do pyeloplasties with an emphasis on the intra-operative parameters. MATERIALS AND METHODS: We compared the perioperative parameters of pediatric RAL procedures performed by a single surgeon at a tertiary care children's hospital for both primary ureteropelvic junction obstruction (UPJO) and recurrent UPJO after a previous open or laparoscopic procedure over 2013-2015. The operative time was subdivided as total operative time, console time, port placement time, dissection time to UPJ, and anastomosis time. RESULTS: A total of 65 pediatric RAL pyeloplasty procedures for UPJO were performed (55 primary and 10 re-do pyeloplasties) during the study period. The console times were 43.3% longer for re-do pyeloplasties than for primary pyeloplasties (133.0 ± 30.7 versus 92.8 ± 24.0 minutes, respectively, P < .01). The re-do cases had longer operative times, especially for UPJ exposure (52.2 ± 21.0 versus 28.0 ± 14.0 minutes, P < .01). There were no conversions to open surgery or significant perioperative complications. There was no difference in hospital pain medication usage and hospital length of stay between the 2 groups. The treatment success rates were 98.2% (54/55) and 100% (10/10), respectively. CONCLUSIONS: RAL re-do pyeloplasty is associated with significantly longer operative times as compared with primary pyeloplasties, especially during the exposure of the UPJ, but it is overall a safe and effective surgical modality for persistent/recurrent UPJO in children. As surgeons are increasingly asked for more accurate predictions of operative time lengths when scheduling cases, this information can be helpful for surgeons when scheduling these cases and with counseling families.

Surgeon Burnout among American Pediatric Urologists.

INTRODUCTION: Burnout is a syndrome characterized by exhaustion, depersonalization and low personal achievement that leads to decreased effectiveness at work. Little to no information is known about the surgeon burnout rate of pediatric urologists. METHODS: An online survey containing a modified Maslach Burnout Inventory and additional demographic information was emailed to practicing pediatric urologists in the United States. The dependent variable was professional burnout measured by 3 subscales from the Maslach Burnout Inventory of 1) emotional exhaustion, 2) depersonalization and 3) low personal achievement. The independent variables included surgeon demographics and practice characteristics. RESULTS: The survey response rate was 66.7%. Of the 41 subjects most were male (82.9%), approximately three-quarters worked at academic centers, more than half had a rank of division chief or full professor and more than half worked more than 60 hours per week. Six (14.6%) pediatric urologists met the criteria for burnout, defined as high scores in emotional exhaustion plus high scores in depersonalization or low personal achievement. When comparing demographic and practice characteristics between pediatric urologists who were burned out to those who were not burned out, there were no statistically significant differences between the 2 groups. Only American Urological Association North Central and Western sections had surgeons with burnout. CONCLUSIONS: Pediatric urologists have a low burnout rate compared to adult urologists and other surgical specialties.

Urologic Considerations in the Separation of Thoracoomphalopagus Conjoined Twins.

Conjoined twins are seen in approximately 1/500,000 live births, and therefore surgical management of urologic anomalies in conjoined twins has not been extensively reported. Various degrees of sharing of the urinary tracts and genitalia can be seen in different types of conjoined twins. Detailed preoperative imaging, including magnetic resonance imaging, computed tomography, and voiding cystourethrogram, is essential to define the anatomy and planning of a successful separation. We describe the urologic presentation, evaluation, and treatment of thoracoomphalopagus conjoined twins.

Whole-exome sequencing in the molecular diagnosis of individuals with congenital anomalies of the kidney and urinary tract and identification of a new causative gene.

PURPOSE: To investigate the utility of whole-exome sequencing (WES) to define a molecular diagnosis for patients clinically diagnosed with congenital anomalies of kidney and urinary tract (CAKUT). METHODS: WES was performed in 62 families with CAKUT. WES data were analyzed for single-nucleotide variants (SNVs) in 35 known CAKUT genes, putatively deleterious sequence changes in new candidate genes, and potentially disease-associated copy-number variants (CNVs). RESULTS: In approximately 5% of families, pathogenic SNVs were identified in PAX2, HNF1B, and EYA1. Observed phenotypes in these families expand the current understanding about the role of these genes in CAKUT. Four pathogenic CNVs were also identified using two CNV detection tools. In addition, we found one deleterious de novo SNV in FOXP1 among the 62 families with CAKUT. The clinical database of the Baylor Miraca Genetics laboratory was queried and seven additional unrelated individuals with novel de novo SNVs in FOXP1 were identified. Six of these eight individuals with FOXP1 SNVs have syndromic urinary tract defects, implicating this gene in urinary tract development. CONCLUSION: We conclude that WES can be used to identify molecular etiology (SNVs, CNVs) in a subset of individuals with CAKUT. WES can also help identify novel CAKUT genes.Genet Med 19 4, 412-420.

Urologic Outcomes of Children With Hemorrhagic Cystitis After Bone Marrow Transplant at a Single Institution.

OBJECTIVE: To analyze clinical outcomes and the risk factors associated with genitourinary (GU) morbidity and mortality in children who present with hemorrhagic cystitis (HC) after bone marrow transplant (BMT). METHODS: A retrospective chart review of patients with HC who had undergone BMT at a single pediatric hospital from 2008 to 2015 was conducted. Demographic data, severity of hematuria, HC management, and mortality were analyzed. Bivariate analysis and binary logistic regression were performed to identify risk factors. RESULTS: Out of 43 patients who met inclusion criteria, 67.4% were male with a median age at BMT of 10.2 years (interquartile range 5.8-14.6). Percutaneous nephrostomy catheters were inserted in 5 patients for urinary diversion. All-cause mortality was 32.6% (N = 14). Intravesical retroviral therapy (P <.001), HC grade (P <.001), total Foley time (P <.001), total gross hematuria time (P <.001), total days hospitalized (P = .012), and days to most improved hematuria (P = .032) were associated with significant GU morbidity on bivariate analysis. On multivariable analysis, days to most improved hematuria was associated with significant GU morbidity odds ratio of 1.177 (1.006-1.376) (P = .042). Status of percutaneous nephrostomy was not associated with increased mortality (P = .472); however, in the multivariate model, BK viremia (P = .023), need for renal dialysis (P = .003), and presence of Foley catheter (P = .005) were associated with increased mortality. CONCLUSION: Children with HC after BMT fall in a very high-risk category with high mortality and significant GU morbidity. The presence of a Foley catheter, need for dialysis, and BK viremia are associated with increased mortality.

Standardized process to improve patient flow from the Emergency Room to the Operating Room for pediatric patients with testicular torsion.

BACKGROUND: Testicular torsion (TT) remains one of the most common urological emergencies. The length of time from onset of symptoms to detorsion and degree of spermatic cord twisting are usually the most important factors for testicular damage. Therefore early presentation, accurate diagnosis, and prompt treatment are important factors for optimizing the testicular salvage rate. While delay in seeking medical attention is a common cause of testicular loss in pediatric patients with testicular torsion, delays in diagnosis and treatment can be preventable causes of testicular loss. OBJECTIVE: In this study, we aimed to develop a standardized process to improve the patient flow from the Emergency Room (ER) to Operating Room (OR) for TT patients in an academic children's hospital. STUDY DESIGN: Thirty consecutive pediatric patients with acute testicular torsion between November 2013 and July 2014 served as the control group. A scrotal pain checklist was implemented in July 2014, and 30 consecutive patients from July 2014 until April 2015 served as the study group. Perioperative parameters including times, ultrasound (US) findings, and surgical results were reviewed. RESULTS: The mean ages of the control group and the study group were similar (12.3 ± 4.9 years and 11.5 ± 5 years, respectively) (p = 0.575). ER arrival to OR time, triage completion to OR time, and scrotal US to OR time were significantly decreased in the study group (p < 0.001) (Table). Although triage time and ER arrival to scrotal US times were decreased in the study group, the differences were not significant (p = 0.071, p = 0.112, respectively). DISCUSSION: Utilizing scoring tools during the triage of patients with scrotal pain can help identify high-risk patients earlier and prevent unnecessary use of resources in an ER serving a large pediatric population. Limitations of this pilot study include the limited number of patients and the potential for the Hawthorne effect (staff awareness of the study). Additionally, we did not examine scrotal pain checklist scores for other acute scrotal diseases. This study focused on a quality improvement process for TT patients, in order to reduce ER to OR times. CONCLUSION: A standardized process with use of a scrotal pain checklist and prompt communication between the ER, Urology, and Radiology teams led to significantly reduced times from the ER to the OR. Standardized processes for pediatric patients with testicular torsion may help to improve testicular survival rates.

Factors associated with fetal shunt dislodgement in lower urinary tract obstruction.

OBJECTIVE: To identify factors associated with fetal shunt dislodgement in lower urinary tract obstruction (LUTO). METHODS: We conducted a retrospective study of 42 consecutive fetuses with a diagnosis of LUTO in a tertiary fetal center between April 2013 and November 2015. Possible factors associated with prenatal shunt dislodgment were evaluated in fetuses who underwent shunt placement, including gestational age at diagnosis, gestational age at procedure, presence of 'keyhole sign', initial fetal bladder volume and wall thickness, prenatal ultrasonographic renal characteristics, amniotic fluid volume, presence of ascites prior to shunting, and type of fetal shunt. RESULTS: Nineteen (46.3%) fetuses underwent shunt placement at a median gestational age of 19 (range: 16.3-31.1) weeks. Shunt dislodgement occurred in 10 (52.6%) patients. A total of 35 procedures were performed; among which 16 (45.7%) were repeat procedures. The only prenatal factor associated with shunt dislodgement was the type of the shunt; Kaplan-Meier analysis indicated that the Rocket was associated with increased likelihood of remaining orthotopic (p = 0.04). CONCLUSION: Fetal shunt dislodgement occurs in approximately half of the patients and appears to be associated with the type of the shunt. Future research is necessary to develop better shunt systems and to investigate different fetal therapeutic approaches. © 2016 John Wiley & Sons, Ltd.

Defining and predicting 'intrauterine fetal renal failure' in congenital lower urinary tract obstruction.

BACKGROUND: The aim of this study was to identify predictors of 'intrauterine fetal renal failure' in fetuses with severe congenital lower urinary tract obstruction (LUTO). METHODS: We undertook a retrospective study of 31 consecutive fetuses with a diagnosis of LUTO in a tertiary Fetal Center between April 2013 and April 2015. Predictors of 'intrauterine fetal renal failure' were evaluated in those infants with severe LUTO who had either a primary composite outcome measure of neonatal death in the first 24 h of life due to severe pulmonary hypoplasia or a need for renal replacement therapy within 7 days of life. The following variables were analyzed: fetal bladder re-expansion 48 h after vesicocentesis, fetal renal ultrasound characteristics, fetal urinary indices, and amniotic fluid volume. RESULTS: Of the 31 fetuses included in the study, eight met the criteria for 'intrauterine fetal renal failure'. All of the latter had composite poor postnatal outcomes based on death within 24 h of life (n = 6) or need for dialysis within 1 week of life (n = 2). The percentage of fetal bladder refilling after vesicocentesis at time of initial evaluation was the only predictor of 'intrauterine fetal renal failure' (cut-off <27 %, area under the time-concentration curve 0.86, 95 % confidence interval 0.68-0.99; p = 0.009). CONCLUSION: We propose the concept of 'intrauterine fetal renal failure' in fetuses with the most severe forms of LUTO. Fetal bladder refilling can be used to reliably predict 'intrauterine fetal renal failure', which is associated with severe pulmonary hypoplasia or the need for dialysis within a few days of life.

Posterior urethral valves: Risk factors for progression to renal failure.

INTRODUCTION: Posterior urethral valves (PUVs) are the most common etiology for congenital urethral obstruction and congenital bilateral renal obstruction. PUVs produce a spectrum of urologic and renal sequelae. Our aims were to assess outcomes of PUV patients, to determine whether vesicoureteral reflux (VUR) is a risk factor for progression to renal failure, and to identify other risk factors for poor outcomes. MATERIALS AND METHODS: We conducted a retrospective analysis of PUV patients from 2006 to 2014. Data collected included demographics, initial renal ultrasound (RUS) findings, creatinine at presentation and nadir, pre- and postoperative VUR status, presence or absence of recurrent urinary tract infections (UTIs), and surgical intervention(s). Univariate and multivariate analyses were used to determine risk factors for renal failure. RESULTS: Of 104 patients, 42.3% (44/104) were diagnosed prenatally, 31.8% (14/44) of whom underwent prenatal intervention. Postnatally, 90.4% (94/104) initially underwent transurethral resection of PUVs (TUR-PUVs). Vesicostomy was the next most common index surgery (4.8%). Forty-two percent (44/104) required >1 surgery. The predominant second surgery was repeat TUR-PUV in 16 patients. At last follow-up (mean 28.8 months after initial surgery), 20.2% had chronic kidney disease (CKD) of at least stage IIIA, and 8.6% had progressed to end-stage renal disease (ESRD). Antenatal diagnosis, prematurity, abnormal renal cortex, and loss of corticomedullary differentiation (CMD) on initial RUS were associated with CKD and ESRD on univariate analysis, as were elevated creatinine on presentation and at nadir. Presence of pre- or postoperative VUR and recurrent UTIs were associated with the need for multiple surgeries, but not with poor renal outcomes. On multivariate analysis, nadir creatinine was the only independent predictor of final renal function. CONCLUSIONS: Our finding that creatinine is the only independent risk factor for poor renal outcomes in PUV patients is consistent with the literature. The effect of VUR has been controversial, and our finding that VUR is associated with need for multiple surgeries but not with CKD or ESRD is novel. Limitations include biases inherent to retrospective studies and relatively small sample size. The majority of patients with PUVs (56.7%) required one surgery and maintained renal function with CKD II or better (79.8%) up to 2 years after initial surgery. While multiple factors were associated with poor renal outcomes, nadir creatinine was the only independent predictor. VUR and recurrent UTIs were not associated with poor renal outcomes. Longer follow-up is necessary to identify risk factors for delayed progression of renal disease.