Cloacal Dysgenesis Sequence in a Preterm Neonate.

BACKGROUND Cloaca malformations result from a disproportion of apoptosis, cell growth, and maturation. The range of cloacal malformations is extensive and diverse, with a lack of a straightforward classification system. Cloacal dysgenesis sequence (CDS), also known as urorectal septum malformation sequence, is a rare cloaca variant described as the absence of a perineal orifice. Prenatal magnetic resonance imaging and antenatal ultrasounds may reveal a cloacal malformation; however, many patients are not diagnosed with cloacal malformation until birth. CASE REPORT We present a case of a female neonate delivered by a 23-year-old G2P1T1A0L0 mother who had received comprehensive prenatal care. During pregnancy, bilateral multicystic dysplastic kidneys were identified prenatally, leading to the in utero placement of a vesicoamniotic shunt. The physical exam revealed a distended abdomen with reduced abdominal musculature and laxity, ascites, a vesicoamniotic shunt in place, absent urethra, ambiguous genitalia with no vaginal opening, no perineal opening, and clubfoot. Abdominal radiograph showed findings consistent with significant abdominal ascites. An exploratory laparotomy was performed that included diverting colostomy, mucous fistula creation, tube vaginostomy, removal of the vesicoamniotic shunt, and suprapubic tube placement. The patient recovered well from this operation with no complications. CONCLUSIONS CDS is an uncommon condition in pediatric patients, and although sonographic findings can reveal urinary tract abnormalities, prenatal imaging might not always identify CDS. Our case underscores the uniqueness of the case and the significance of early detection and immediate medical and surgical intervention.

Endoscopic treatment of primary obstructive megaureter with high pressure balloon dilation in infants.

INTRODUCTION AND OBJECTIVE: Ureteral reimplantation of the dilated ureter in infants is challenging; however, some patients with primary obstructive megaureter (POM) in this age group require intervention due to clinical or radiological progression. We sought to determine if high pressure balloon dilation (HPBD) can serve as a definitive treatment for POM in children under one year of age, or as a temporizing measure until later reimplantation. MATERIALS AND METHODS: All patients from a single institution who underwent HPBD between October 2009 and May 2022 were retrospectively reviewed. Patients were excluded if older than 12 months or diagnosed with neurogenic bladder, posterior urethral valves, or obstructed refluxing megaureter. Patients with prior surgical intervention at the ureterovesical junction were excluded. Indications for surgery included progressive hydroureteronephrosis or urinary tract infection (UTI). Balloon dilation was performed via cystoscopy with fluoroscopic guidance, followed by placement of two temporary ureteral stents. Primary outcomes were improvement or resolution of megaureter and rates of subsequent reimplantation. Secondary outcomes included total number of anesthetics and postoperative UTIs. RESULTS: Fifteen infants with median age of 7.6 months (IQR 3.8-9.7) underwent HPBD. Twelve (80%) patients were detected prenatally and 3 (20%) after a UTI. Indication for surgery was progressive hydroureteronephrosis in 10 patients (67%) and UTI in five (33%). All had SFU grade 3 or 4 hydronephrosis on preoperative ultrasound and median distal ureteral diameter was 13 mm. Median follow up was 2.9 years. Twelve (80%) succeeded with endoscopic treatment: 7 patients had an undetectable distal ureter on ultrasound at last follow-up, 5 were improved with median distal ureteral diameter of 7 mm. Three patients (20%) required ureteral reimplantation due to progressive dilation, all with grade 4 hydronephrosis and distal ureteral diameters were 11, 15, and 21 mm. Six patients (40%) required two anesthetics to complete endoscopic treatment. Among those, 4 patients required initial stent placement for passive dilation followed by a second anesthetic for HPBD weeks later. Two patients underwent repeat HPBD following postoperative proximal migration of the ureteral stents. All 15 patients had an additional anesthetic for removal of stents. Five patients (33%) were treated for a symptomatic UTI (4 febrile, 1 afebrile) with the stents indwelling but there were no UTIs in the group following stent removal. CONCLUSION: Balloon dilation is a practical option for treatment of POM in infants, and in most cases (80%) avoids subsequent open surgery (over median 2.9 years of follow-up).

Echidna Splenule: A Case Study of Continuous Splenogonadal Fusion.

Splenogonadal fusion (SGF) is a rare congenital anomaly of an aberrant accessory spleen-gonad connection. We present a rare case of continuous splenogonadal fusion in a full-term male with a left undescended testis, multiple congenital limb anomalies, and syndromic facies. Diagnostic laparoscopy revealed the "Echidna Splenule," a snake-like intraperitoneal splenule coursing from the spleen along the left paracolic region and engulfing an atrophic intra-abdominal testis preventing spontaneous descent and distally herniating into the left open internal inguinal ring. The atrophic testis and Echidna Splenule were resected. Splenogonadal fusion should be considered in children with left undescended testis and concomitant limb and facial anomalies.

The double-face onlay-tube-onlay transverse preputial flap: An advantageous alternative to the two-stage hypospadias repair?

OBJECTIVE: To compare the surgical outcomes and complications of boys who underwent double-face onlay-tube-onlay transverse preputial island flap (DFOTO) one-stage repair vs. two-stage repair for proximal hypospadias. STUDY DESIGN: Males with proximal hypospadias who underwent DFOTO or two-stage repair at a single institution from 2008 to 2021 were identified. Patients who had prior hypospadias surgery were excluded. Outcomes were surgical complications, number of surgical procedures, operative time, and post-operative uroflowmetry results. RESULTS: Fifty-three males who underwent DFOTO and 39 who underwent two-stage repair were included. Median age at surgery was 1.1 years (IQR 0.83-1.6) and median follow-up was 3.0 years (IQR 1.2-6.8). Although not statistically significant, the DFOTO group had higher rates of urethrocutaneous fistula (30% vs. 15%, p = 0.10), urethral stricture (15% vs. 3%, p = 0.07) and urethral diverticulum (8% vs. 3%, p = 0.39). Although the unplanned re-operation rate was higher in DFOTO (58% vs. 33%, p = 0.02), the mean number of procedures and median total surgical time were lower in DFOTO (1.8 ± 0.9 vs. 2.4 ± 0.8, p = 0.0004; 337 min [IQR 278-460] vs. 468 min [IQR 400-563], p = 0.008). There were no significant differences between groups for mean peak flow rates and post void residuals. CONCLUSIONS: In males who underwent DFOTO, 42% achieved completion of their proximal hypospadias repair with one operation, while the remainder had largely minor complications. Accounting for reoperation rates, the mean number of procedures per patient was lower in the DFOTO group. Comparable results can be achieved with both techniques; the risks of higher unplanned operation rates in the DFOTO group should be considered with the benefit of fewer total procedures.

The Pseudo-Bladder: A Case of the Prostatic Urethra Masquerading as the Bladder.

Congenital bladder underdevelopment has detrimental genitourinary effects. We present a male infant with declining renal function, recurrent urinary tract infections, and epididymo-orchitis, in the setting of bilateral high grade hydronephrosis and vesicoureteral reflux. Cystoscopic evaluation revealed the presumed "bladder" to be a massively dilated prostatic urethra. The small dysplastic "true bladder" was found at the anterior surface of the prostatic urethra with massively dilated ureteral orifices. Temporizing diversion was performed in the form of bilateral cutaneous ureterostomies, with an ultimate plan for reconstructive bladder surgery. This case presents diagnostic challenges utilizing current modalities due to a massively dilated prostatic urethra in the rare case of bladder dysplasia.

Home bladder pressure measurements correlate with urodynamic storage pressures and high-grade hydronephrosis in children with spina bifida.

BACKGROUND: Patients with spina bifida are at risk for developing bladder and renal deterioration secondary to increased bladder storage pressures. OBJECTIVES: To determine the association of home bladder volume and pressure measurements (home manometry) to: 1) detrusor storage pressures on urodynamics (UDS); and 2) the presence of Society of Fetal Urology (SFU) grades 3-4 hydronephrosis on renal bladder ultrasound in patients with spina bifida. METHODS: Data were prospectively collected on patients with spina bifida and neurogenic bladder requiring clean intermittent catheterization. Patients used a ruler and typical catheterization equipment to measure bladder pressures and volumes at home. Home measurements were compared to UDS detrusor pressures and SFU hydronephrosis grade. Detrusor pressure <20 cm H2O at 50% maximal cystometric capacity (MCC) on UDS was used as a measure of safe storage pressures on UDS; conversely, detrusor pressure >20 cm H2O was used a measure to capture both unsafe storage pressures and those with potential for unsafe storage pressures. Receiver-operator characteristic curves and area under curve (AUC) were calculated to depict the association between home manometry variables with detrusor pressures on UDS and SFU grades 3-4 hydronephrosis. RESULTS: Included were 52 patients with a median age of 10.3 years (interquartile range 6.3-14.4 years). Three home manometry measurements (maximum bladder pressure, bladder pressure at maximum catheterized volume, and mean bladder pressure) > 20 cm H2O were sensitive for Pdet >20 cm H2O at 50% MCC. Maximal bladder pressure >20 cm H2O was the most sensitive among home manometry measures (sensitivity 100%, specificity 70%, AUC 0.92 for Pdet >20 cm H2O at 50% MCC on UDS; sensitivity 100%, specificity 62%, AUC 0.89 for SFU grade 3-4 hydronephrosis). None of the patients who had maximum home bladder pressure <20 cm H2O had SFU grades 3-4 hydronephrosis; conversely, individuals with maximal home bladder pressure >20 cm had a wide range of hydronephrosis grades. CONCLUSION: None of the patients with maximal home bladder pressure <20 cm H2O had grade 3-4 hydronephrosis. Home measurements of maximal bladder pressure, bladder pressure at maximum catheterized volume and mean bladder pressure of >20 cm H2O were all sensitive for Pdet >20 cm H2O at 50% MCC on UDS. Home manometry is an inexpensive and simple technique to identify patients at risk for and to monitor individuals at high risk of upper tract dilation, without incurring significant cost or morbidity.

Continuous antibiotic prophylaxis in isolated prenatal hydronephrosis.

BACKGROUND: Prenatal hydronephrosis (PNH) is one of the most common congenital anomalies and can increase the risk of developing a urinary tract infection (UTI) in the first two years of life. Continuous antibiotic prophylaxis (CAP) has been recommended empirically to prevent UTI in children with PNH, but its use has been controversial. OBJECTIVE: We describe the incidence of UTI in children with isolated PNH of the renal pelvis without ureteral dilation. Our objective was to compare patients receiving and not receiving CAP and determine whether CAP is beneficial at preventing UTI in children with isolated PNH. STUDY DESIGN: Children with confirmed PNH were enrolled between 2008 and 2020 into the Society for Fetal Urology Hydronephrosis Registry. Children with isolated dilation of the renal pelvis without ureteral or bladder abnormality were included. The primary outcome was development of a UTI, comparing patients who were prescribed and not prescribed CAP. RESULTS: In this cohort of 801 children, 76% were male, and 35% had high grade hydronephrosis (SFU grades 3-4). CAP was prescribed in 34% of children. The UTI rate among all children with isolated PNH was 4.2%. Independent predictors of UTI were female sex (HR = 13, 95% CI: 3.8-40, p = 0.0001), intact prepuce (HR = 5.1, 95% CI: 1.4-18, p = 0.01) and high grade hydronephrosis (HR = 2.0, 95% CI: 0.99-4.0, p = 0.05; Table) on multivariable analysis. For patients on CAP, the UTI rate was 4.0% compared to 4.3% without CAP (p = 0.76). The risk of UTI during follow-up was not significantly different between patients who received CAP and patients who were not exposed to CAP; adjusting for sex, circumcision status and hydronephrosis grade (HR = 0.72, 95% CI: 0.34-1.5, p = 0.38). In sub-group analysis of patients at higher risk of UTI (uncircumcised males, females and high grade hydronephrosis), CAP use was not associated with a statistically significant reduction in UTI. CONCLUSIONS: The overall UTI rate in children with isolated PNH is very low at 4.2%. In the overall population of patients with isolated PNH, CAP was not associated with reduction in UTI risk, although the limitations in our study make characterizing CAP effectiveness difficult. Clinicians should consider risk factors prior to placing all patients with isolated PNH on CAP.

Risk of urinary tract infection in patients with hydroureter: An analysis from the Society of Fetal Urology Prenatal Hydronephrosis Registry.

BACKGROUND: Prenatal hydronephrosis is one of the most common anomalies detected on prenatal ultrasonography. Patients with prenatal hydronephrosis and ureteral dilation are at increased risk of urinary tract infection (UTI) and continuous antibiotic prophylaxis (CAP) is recommended. However, current guidelines do not define the minimum ureteral diameter that would be considered a dilated ureter in these patients. OBJECTIVE: We evaluate the definition of clinically relevant hydroureter, its association with UTI, and the impact of CAP. STUDY DESIGN: Patients with prenatal hydronephrosis from seven centers were enrolled into the Society for Fetal Urology Prenatal Hydronephrosis Registry from 2008 to 2020. Patients with ureteral measurement on ultrasound were included. Patients with ureterocele, ectopic ureter, neurogenic bladder, posterior urethral valves, horseshoe or solitary kidney, known ureteropelvic junction obstruction, or follow-up less than one month were excluded. Primary outcome was UTI. Analyses were performed using Cox regression. RESULTS: Of the 1406 patients enrolled in the registry, 237 were included. Seventy-six percent were male, ureteral diameter ranged from 1 to 34 mm, and median follow-up was 2.2 years. Patients with ureters 7 mm or greater had nearly three times the risk of UTI adjusting for sex, circumcision status, antibiotic prophylaxis and hydronephrosis grade (HR = 2.7, 95% CI: 1.1-6.5, p = 0.03; Figure). In patients who underwent voiding cystourethrogram (VCUG; 200/237), ureteral dilation of 7 mm or more identified patients at increased UTI risk controlling for sex, circumcision status, vesicoureteral reflux and hydronephrosis grade (HR = 2.3, 95% CI: 0.97-5.6, p = 0.06). CAP was significantly protective against UTI (HR = 0.50 (95% CI: 0.28-0.87), p = 0.01). Among patients who underwent VCUG and did not have vesicoureteral reflux, ureteral dilation 7 mm or greater corresponded with higher UTI risk compared to ureteral diameter less than 7 mm on multivariable analysis (HR = 4.6, 95% CI: 1.1-19.5, p = 0.04). CONCLUSIONS: This is the first prospectively collected, multicenter study to demonstrate that hydroureter 7 mm or greater identifies a high-risk group for UTI who benefit from antibiotic prophylaxis. In contrast, patients with prenatal hydronephrosis and non-refluxing hydroureter less than 7 mm may be managed more conservatively.

Retractable foreskin reduces urinary tract infections in infant boys with vesicoureteral reflux.

BACKGROUND: Uncircumcised males are at higher risk of urinary tract infection (UTI) in the first year of life and circumcision is recommended as an option for males with vesicoureteral reflux (VUR). Uncircumcised males treated successfully with topical corticosteroid cream have decreased risk of UTI but the role of preputial management has not been explored previously in males with VUR. OBJECTIVE: We hypothesized that among uncircumcised boys with VUR, those with retractable foreskin would be at reduced risk of UTI compared to those with non-retractable foreskin. STUDY DESIGN: Males less than one year of age with primary VUR were prospectively enrolled. Patients with concomitant urologic diagnoses or less than one month of follow-up were excluded. Phimosis severity was graded on a 0-5 scale. Primary outcome was UTI during follow-up. Patients were divided into three groups for analysis: circumcised, low grade phimosis (grades 0-3) and high grade phimosis (grades 4-5). Multivariable Cox proportional hazards regression was used to estimate UTI risk adjusting for risk factors. RESULTS: One-hundred and five boys (24 circumcised and 81 uncircumcised) with VUR were included. Median age at enrollment was 4.4 months (IQR 2.2-6.6) and median follow-up was 1.1 years (IQR 0.53-2.9). Males with phimosis grades 4-5 had a higher UTI rate (29%) compared to phimosis grade 0-3 (4%). Based on Kaplan-Meier curves, boys with initial phimosis grades 4-5 were significantly more likely to develop a UTI than boys who were circumcised or had phimosis grades 0-3 (p = 0.005). On multivariable analysis, boys with phimosis grades 4-5 were significantly more likely to develop UTI when compared to boys with grades 0-3 phimosis (HR = 8.4, 95% CI: 1.1-64, p = 0.04). DISCUSSION: Males with a retractable prepuce had a lower UTI risk compared to males with non-retractable prepuce (high grade phimosis) and this remained significant on multivariable analysis. This is concordant with prior studies demonstrating that a retractable prepuce is associated with decreased UTI risk. Limitations of our study include using phimosis grade at time of study enrollment and heterogenous prophylactic antibiotic use in our population. CONCLUSIONS: Retractable foreskin reduces UTI risk in uncircumcised boys less than one year of age with VUR. Medical phimosis treatment to achieve a retractable prepuce offers an alternative and less invasive modality to reduce UTI risk in males with VUR.

Keys to improving patient satisfaction in the pediatric urology clinic: A starting point.

BACKGROUND: Recent developments in healthcare have placed increased focus on patient satisfaction. Among adult populations, validated patient satisfaction tools have now been incorporated into routine practice. This process has been much slower and is significantly less standardized in pediatric populations. OBJECTIVE: The objective of this analysis was to evaluate various factors that predict patient satisfaction in pediatric urology settings. MATERIALS AND METHODS: Data from the National Research Corporation (NRC) Health Patient Survey were collected from children receiving urological care between 2017 and 2019. Survey data were merged with demographic and visit-related information from electronic health records (EHR). Rating of provider on the NCR Health survey was chosen as the primary outcome. Two multivariable models were analyzed by logistic regression. The first model contained only demographic and clinic-based predictor variables. The second model contained the NRC survey questions. RESULTS: This analysis includes a total of 3232 surveys. Multivariable analysis of EHR demographic and visit data found that low income insurance (Medicaid) (OR = 1.3, p = 0.035), primary language Spanish (OR = 1.5, p = 0.012), and shorter in-office wait time (OR = 0.94, p < 0.001) were predictors for higher patient satisfaction scores. Multivariable analysis of NCR Health survey data identified physician explanation, listening, respect for patient, time spent with patient and communication between physicians and nurses as highly significant predictors of satisfaction (p < 0.001). DISCUSSION: This analysis has identified several modifiable and non-modifiable variables that predict patient satisfaction in a population of children receiving care in a pediatric urology clinic. Limitations of this study include the possibility for response bias and lack of data on other potentially important but unmeasured factors. CONCLUSIONS: Certain patient populations are more satisfied with the outpatient urology clinic experience. Several factors related to physician-patient communication are modifiable areas to improve patient satisfaction. Further intervention studies focusing on the modifiable areas are needed to increase patient satisfaction in pediatric urology.

Testicular Volume Changes in Laparoscopic Staged Fowler-Stephens Orchiopexy: Studying the Impact of Testicular Vessel Division.

OBJECTIVE: To study the effect of testicular vessel division on testicular volume during laparoscopic staged Fowler Stephens orchiopexy (LSFSO). METHODS: Testicular dimensions were prospectively measured intraoperatively at both first (S1) and second stages (S2) of LSFSO, and with scrotal ultrasound 3-12 months postoperatively. Testicular volumes were compared to reference ranges. Volume changes were tracked with a change of >20% considered clinically significant. RESULTS: A total of 52 nonpalpable testes treated with LSFSO between 2008 and 2018 were included in the study. After a median follow-up of 6.8 (3-91.3) months, 46 (88.5%) testes were palpable in a scrotal location without adjunctive procedures and 39 (75%) maintained vascular flow on duplex ultrasound. One testis retracted to an inguinal position and was successfully treated with inguinal orchiopexy for an overall success of 90.4% (47/52). Of 36 testes with intra- and postoperative testicular volume documentation, only 2 (5.6%) had significant volume loss after S1. Both testes had catch-up growth after S2. Eight (22.2%) testes had significant volume loss after S2. At follow-up, 24 (66.7%) testes were smaller than the mean for age, of which 20 (83.3%) were small at baseline. Only 41.7% of testes larger than mean for age at follow-up, were small at baseline (P = .02). CONCLUSION: Significant testicular volume loss does not occur after testicular vessel division at S1, but expected in approximately 1 quarter of testes after S2. We propose that testicular atrophy after LSFSO is primarily due to defective testicular development and rarely due to vascular compromise during S2.

A Survey of the Current Practice Patterns of Contralateral Testis Fixation in Unilateral Testicular Conditions.

OBJECTIVE: To query the current contralateral testis fixation (CTF) practice patterns among pediatric urologists in different clinical situations that could result in monorchism. METHODS: An online survey was sent to members of the Urology Section of the American Academy of Pediatrics. The survey included questions addressing CTF practice patterns in 14 clinical scenarios. Responses were anonymously submitted, blindly reviewed, and analyzed. RESULTS: Among 53 respondents, 62.3% had academic appointments and 73.6% had an exclusive pediatric urology practice. All participants agreed on CTF necessity in testicular torsion beyond the neonatal period. CTF was advocated by 84.9% in prenatal torsion, 96.2% in postnatal torsion, and 94.3% in delayed torsion presentation. Emergent intervention was favored by 64.4% in prenatal and 98% in postnatal torsion. Only 1 participant (1.9%) preferred CTF with a unilateral testicular tumor and 5 (9.4%) in trauma substantiating an orchiectomy. There was less consensus on CTF in torsed undescended testis (79.3% in prepubertal and 81.13% in postpubertal), testicular nubbin in a child (40.4%), palpable atrophic undescended testis (13.2%), and unilateral bell-clapper anomaly (47.2%). In situations other than torsion, lack of strong evidence was the commonest reason not to perform CTF. CONCLUSION: The majority of responding pediatric urologists currently performs CTF in neonatal torsion. Although there is a general consensus on CTF in testicular torsion outside the neonatal period, CTF remains controversial in other clinical situations, warranting further research. The decision for CTF should involve patients, parents, and treating physicians.

Medical Treatment of a Staghorn Calculus: The Ultimate Noninvasive Therapy.

A 77-year-old female presented with bilateral staghorn calculi. She underwent an uneventful left percutaneous nephrolithotomy (PCNL); the stone analysis revealed a 90% struvite and 10% calcium phosphate stone. Treatment of the right stone was postponed by the patient. During the next 9 months, her family physician gave her multiple courses of culture-directed antibiotics due to breakthrough urinary-tract infections, despite her also being on a prophylactic antibiotic. After 9 months, she agreed to undergo her right PCNL. Preoperatively, a non-contrast CT scan was obtained; it revealed complete resolution of the right staghorn calculi.

Radiation exposure during continuous and pulsed fluoroscopy.

UNLABELLED: Abstract Background and Purpose: Previous studies using pulsed fluoroscopy have shown variable effects on radiation exposure because of the ramp and trail effect in older C-arm systems. This study compares radiation delivered in pulsed and continuous modalities using a modern C-arm system. MATERIALS AND METHODS: Thermoluminescent dosimeters (TLDs) positioned in three body locations directly measured radiation dose during simulated ureteroscopy. Thirty pedal activations were administered using a pulsed or continuous mode to visualize an implanted guidewire and a radiopaque stone. TLD absorbed radiation and image quality were compared between imaging modes. RESULTS: Pulsed fluoroscopy delivered less radiation compared with continuous fluoroscopy at each site: Anterior skin (0.10 vs 0.26 mGy, P<0.001), kidney (0.15 vs 0.40 mGy, P<0.001), and posterior skin (0.92 vs 2.62 mGy, P<0.001). Mean fluoroscopy time differed between continuous and pulsed modes (12.5 vs 3.0 seconds; P<0.001). Fluoroscopy time positively correlated with radiation exposure at all sites: Anterior skin (0.017 mGy/s, R(2)=0.90), left kidney (0.026 mGy/s, R(2)=0.96), and posterior skin (0.18 mGy/sec, R(2)=0.98). When evaluated by blinded urologists, 100% of reviewers felt pulsed images were adequate to identify guidewire position and 90.5% felt pulsed images were adequate for stone localization. CONCLUSION: Pulsed fluoroscopy reduced fluoroscopy time by 76% and radiation dose by 64% compared with continuous fluoroscopy. Pulsed fluoroscopy images were adequate for most tasks of ureteroscopy and should be considered for reduction of radiation during ureteroscopy.