Prevalence and Clustering of Congenital Heart Defects Among Boys With Hypospadias.

Importance: Hypospadias is a common birth defect of the male urinary tract that may be isolated or may co-occur with other structural malformations, including congenital heart defects (CHDs). The risk for co-occurring CHDs among boys with hypospadias remains unknown, which limits screening and genetic testing strategies. Objective: To characterize the risk of major CHDs among boys born with hypospadias. Design, Setting, and Participants: This retrospective cohort study used data from population-based birth defect surveillance programs on all male infants born in 11 US states from January 1, 1995, to December 31, 2014. Statistical analysis was performed from September 2, 2020, to March 25, 2022. Exposure: Hypospadias. Main Outcomes and Measures: Demographic and diagnostic data were obtained from 2 active state-based birth defect surveillance programs for primary analyses, the Texas Birth Defects Registry and the Arkansas Reproductive Health Monitoring System, with validation among 9 additional states in the National Birth Defects Prevention Network (NBDPN). Birth defect diagnoses were identified using the British Pediatric Association coding for hypospadias (exposure) and major CHDs (primary outcomes). Maternal covariates and birth year were also abstracted from the vital records. Poisson regression was used to estimate adjusted prevalence ratios and 95% CIs for major CHDs within Texas and Arkansas and combined using inverse variance-weighted meta-analysis. Findings were validated using the NBDPN. Results: Among 3.7 million pregnancies in Texas and Arkansas, 1485 boys had hypospadias and a co-occurring CHD. Boys with hypospadias were 5.8 times (95% CI, 5.5-6.1) more likely to have a co-occurring CHD compared with boys without hypospadias. Associations were observed for every specific CHD analyzed among boys with hypospadias, occurred outside of chromosomal anomalies, and were validated in the NBDPN. An estimated 7.024% (95% CI, 7.020%-7.028%) of boys with hypospadias in Texas and 5.503% (95% CI, 5.495%-5.511%) of boys with hypospadias in Arkansas have a co-occurring CHD. In addition, hypospadias severity and maternal race and ethnicity were independently associated with the likelihood for hypospadias to co-occur with a CHD; boys in Texas with third-degree (ie, more severe) hypospadias were 2.7 times (95% CI, 2.2-3.4) more likely than boys with first-degree hypospadias to have a co-occurring CHD, with consistent estimates in Arkansas (odds ratio, 2.7; 95% CI, 1.4-5.3), and boys with hypospadias born to Hispanic mothers in Texas were 1.5 times (95% CI, 1.3-1.8) more likely to have a co-occurring CHD than boys with hypospadias born to non-Hispanic White mothers. Conclusions and Relevance: In this cohort study, boys with hypospadias had a higher prevalence of CHDs than boys without hypospadias. These findings support the need for consideration of additional CHD screening programs for boys born with hypospadias.

Urological Emergencies and Diseases in Wilderness Expeditions.

When considering medical emergencies that might affect an expedition, urologic emergencies are typically not included. However, the reality is that manageable and prevalent urologic disease processes can pose significant challenges for the wilderness medicine physician and warrant consideration. The purpose of this review is to identify and discuss the most commonly encountered urologic emergencies and diseases in the wilderness setting and to prepare the expedition medicine physician for management of these urgent conditions. A PubMed and Internet search for urologic emergencies and diseases in wilderness conditions was conducted. We also searched bibliographies for useful supplemental literature and material from leading mountain medicine and wilderness medicine societies as well as population-based studies for common urologic diseases. Urologic emergencies and diseases on expeditions and in wilderness conditions have been reported primarily with retrospective case series and case reports. The most commonly reported urologic emergencies in this setting include urologic trauma, renal calculi, and urinary retention. Parasitic infections in the urinary tract also have been reported to cause urinary symptoms and urinary retention in wilderness conditions. Although urologic diseases in such conditions are uncommon, significant morbidity and even potentially life-threatening sequelae to urologic emergencies were found to occur. Major genitourinary emergencies in expedition medicine are uncommon but involve both potentially manageable urgent conditions and serious life-threatening conditions best treated with urgent stabilization and occasionally medical evacuation. The opportunity exists for increased awareness for management strategies for urologic conditions in the often remote or extreme environments of an expedition.

Hydronephrosis and Hydroureter Improvement Rates in Robotic-Assisted Laparoscopic Uretero-Ureterostomies: Does Anastomotic Site Matter?

OBJECTIVE: To compare improvement/change of hydronephrosis and hydroureter in patients with complete ureteral duplications that underwent upper and lower robotic-assisted laparoscopic uretero-ureterostomies. The hypothesis being that improvement of hydronephrosis and hydroureter between the two groups was similar. METHODS: 35 subjects met inclusion criteria and were reviewed retrospectively. 'Upper' anastomoses were defined as those being done below the lower pole of the kidney (Group 1), while 'lower' anastomoses were those done below the iliac vessels (Group 2). Primary variables analyzed were antero-posterior and diameter measurements of the renal pelvis and ureter, respectively, before and after surgery. Secondary variables included operative time, length of hospital stay, and complication rates. RESULTS: Group 1 consisted of 20 subjects while Group 2 consisted of 15 subjects. Presenting diagnoses were hydronephrosis in 31 subjects and incontinence in 4 subjects. Group 1 mean AP renal diameters decreased by 62.9% (P<.05), while Group 2 decreased by 65.4% (P<.05). Group 1 mean hydroureter diameter measurements decreased by 80.3% (P<.05), while Group 2 decreased by 83% (P<.05). The improvement in hydronephrosis and hydroureter between the two groups was not statistically different. Group 1 median operative time (271 minutes) was longer than Group 2 (201 minutes) (P<.05). There was no significant difference in hospital stay between the groups and there were no significant complications within the cohort. CONCLUSION: The improvement rate of hydronephrosis and hydroureter is similar in upper versus lower RAL UU. Operative time was significantly shorter in the lower anastomosis group.

Comparative analysis of perioperative prophylactic antibiotics in prevention of surgical site infections in stented, distal hypospadias repair.

PURPOSE: There is limited evidence that prophylactic antibiotics prevent surgical site infection in stented, distal hypospadias repair. Our hypothesis is that the use of prophylactic antibiotics does not affect the rate of surgical site infection in this setting. METHODS: We conducted a retrospective study of consecutive patients over a 6-year period with distal penile hypospadias treated with urethral stenting. Variables analyzed include age, type of repair, usage of preoperative and/or postoperative antibiotics, and length of follow-up. Patients with a history of proximal or re-operative hypospadias repair were excluded. Surgical site infection was defined by the presence of postoperative penile erythema and/or purulent drainage treated with therapeutic antibiotics. Secondary outcome analysis included the presence of other hypospadias complications. RESULTS: 441 consecutive subjects met our inclusion criteria with a mean age of 13.3 months. Patients were categorized into groups: Group 1 - Preoperative antibiotics (n = 64), Group 2 - Both Preoperative & Postoperative antibiotics (n = 159), Group 3 - Postoperative antibiotics (n = 122), Group 4 - No Preoperative or Postoperative antibiotics (n = 96). Two surgical site infections were reported out of the 441 patients: 1 in Group 3 and 1 in Group 4 (p = 0.513). There was no significant difference in the total patients with a hypospadias complication between groups. In the table below, Groups 1-3 were combined (345 patients) for comparison to Group 4 (No antibiotics, 96 patients) for further analysis with no difference in SSIs (p = 0.388) or respective hypospadias complications. CONCLUSIONS: The use of perioperative prophylactic antibiotics, both before and after surgery for distal, stented hypospadias repair, have not been shown to reduce the rate of surgical site infections nor hypospadias complications. Consequently, the benefit of prophylactic antibiotics in this setting is unclear.

2-octyl cyanoacrylate hypospadias repair dressing: a retrospective, controlled comparison.

INTRODUCTION: 2-octyl cyanoacrylate (OC) has been shown to be a viable option for usage following standard circumcision but data on its utilization following hypospadias repair is limited. Both OC and a standard waterproof transparent dressing (WD) are used following hypospadias repair at our children's hospital. Our hypothesis is that patients with distal hypospadias repair using OC for surgical dressing have similar outcomes as compared to patients with WD. MATERIALS AND METHODS: A retrospective study was performed evaluating all patients with distal hypospadias repair during a 2 year period. OC was primarily used by one of the three physicians in the practice with the other two primarily used WD for surgical dressing. The primary endpoints evaluated include hematoma requiring surgical drainage, infection, meatal stenosis, urethrocutaneous fistula, dehiscence, and diverticulum. Standard follow up after hypospadias repair includes a 1 week follow up for patients requiring urethral stent removal and reevaluation for all patients 3-4 months after surgery. REDCap was used in order to compile the database used in this study. RESULTS: A total of 280 patients underwent distal hypospadias repair during this interval. One hundred twenty-two patients had OC used with 3 (2.4%) having complications: 2 fistulas and 1 with both meatal stenosis and fistula. One hundred fifty-eight patients were dressed with WD with 5 (3.2%) complications: 4 fistulas and 1 meatal stenosis. No patients had hematoma, wound dehiscence, diverticulum, or infection. CONCLUSION: A low rate of complication was observed following distal hypospadias repair using both 2-octyl cyanoacrylate and a standard waterproof transparent dressing. 2-octyl cyanoacrylate is a safe option for surgical dressing following distal hypospadias repair but its utilization in this setting is surgeon dependent.

Cutaneous calicostomy for salvage urinary diversion.

OBJECTIVE: We describe a salvage technique for urinary diversion in the face of an obstructed, undilated upper urinary tract utilized in the management of a premature newborn infant with bilateral renal candidiasis, namely cutaneous calicostomy. METHODS: An 850-g 24 weeks' gestation premature male infant developed obstructive nephropathy as a result of renal candidiasis. Systemic therapy with bilateral nephrostomy drainage and amphotericin B irrigations was repeatedly unsuccessful in eradicating the candiduria and fungal balls. Underlying bilateral proximal collecting system obstructions complicated the situation by rendering ureterostomy or pyelostomy impossible. Subsequent flank exploration was performed bilaterally, and bilateral cutaneous calicostomies were performed. RESULTS: The patient's urinary tract was successfully diverted and the fungal balls eradicated. Renal ultrasounds and antegrade nephrostograms through the cutaneous calicostomy stomas revealed the procedure to be successful. Unfortunately, the patient eventually died from overwhelming non-urologic sepsis approximately 3 months postoperatively. CONCLUSIONS: Although systemic antifungal therapy with bilateral nephrostomy drainage has been shown to be successful in the treatment of obstructive uropathy due to renal candidiasis, surgical intervention is necessary when recurrent sepsis with candiduria persists. In the event that underlying collecting system obstruction is also present, cutaneous calicostomy is an option to consider in the treatment of this disease.

Laparoscopic reconstructive options for obstruction in children with duplex renal anomalies.

OBJECTIVE: To present our initial experience of laparoscopic reconstructive surgery in children with upper urinary tract obstruction associated with duplex anomalies, as although there is much information on ablative procedures such as laparoscopic heminephrectomy, there is little available about minimally invasive reconstructive options for duplex renal anomalies in children. PATIENTS AND METHODS: We retrospectively reviewed four consecutive patients (aged 6-11 years) with duplex anomalies and laparoscopic reconstruction for obstructed, dilated segments treated at our institution. The port placement and surgical exposure were analogous to that for transperitoneal laparoscopic pyeloplasty. A JJ stent was placed retrogradely into the ureter immediately before each procedure. The procedures performed were pyelo-ureterostomy for incomplete duplication and lower pole pelvi-ureteric junction (PUJ) obstruction, lower pole pyeloplasty for lower pole PUJ obstruction and complete duplication, and ipsilateral uretero-ureterostomy and distal ureterectomy for an obstructed, ectopic upper pole. Foley catheters were left indwelling for 36-48 h and stents were removed at 4-6 weeks. Postoperative imaging included either ultrasonography or intravenous urography. RESULTS: Three children presented with intermittent flank pain due to lower pole PUJ obstruction. The other child presented with pyonephrosis and purulent drainage from her vagina due to an ectopic ureter associated with a functioning upper pole segment. All procedures were successfully completed. The only complication was in the first patient (pyelo-ureterostomy) who had transient urinary extravasation that resolved with bladder decompression for 10 days. With a follow-up of 6-18 months, all had resolution of symptoms with improvement in radiographic variables. CONCLUSIONS: This series shows that children with duplex anomalies and obstruction can undergo successful reconstruction using techniques learned with laparoscopic pyeloplasty.

Which is better--retroperitoneoscopic or laparoscopic dismembered pyeloplasty in children?

PURPOSE: Groups at multiple institutions have documented the efficacy of minimally invasive repair of ureteropelvic junction obstruction with a retroperitoneoscopic or laparoscopic approach. To our knowledge no group has compared the 2 operative procedures directly at a single institution. MATERIALS AND METHODS: The records of 49 consecutive patients with a history of retroperitoneoscopic pyeloplasty or transperitoneal laparoscopic pyeloplasty for ureteropelvic junction obstruction were reviewed retrospectively, of whom 29 underwent attempted retroperitoneoscopic pyeloplasty and 20 underwent laparoscopic pyeloplasty. Retroperitoneoscopic pyeloplasty cases were performed first in the series before changing to the laparoscopic pyeloplasty approach. Retroperitoneoscopic pyeloplasty was performed using an anterolateral approach with retroperitoneal balloon distention. Laparoscopic pyeloplasty repair was performed using a transmesenteric approach for left ureteropelvic junction obstruction or after right colon mobilization for right repairs. Dismembered pyeloplasty was performed over a stent using 5-zero polydioxanone suture. Stents were placed antegrade or retrograde based on anatomy and presenting symptoms. Parameters studied were patient age, operative time, postoperative analgesic requirement during hospitalization, hospital stay and success rate. RESULTS: No difference was observed between the 2 groups in patient age, success rate, hospital stay or analgesic narcotic requirement. Average operative time for retroperitoneoscopic pyeloplasty was significantly longer than for laparoscopic pyeloplasty (239.1 vs 184.8 minutes). Overall success rates were also statistically equivalent (25 of 27 retroperitoneoscopic and 19 of 19 laparoscopic pyeloplasties) with incomplete followup in 1 patient in the retroperitoneoscopic pyeloplasty group and 1 in the laparoscopic pyeloplasty group. Three children, including 2 with retroperitoneoscopic and 1 with laparoscopic pyeloplasty, had transient urinary extravasation postoperatively, which was related to poorly positioned stents. Five patients in the retroperitoneoscopic group and 1 in the laparoscopic group underwent balloon dilation for indistinct but persistent postoperative flank pain with equivocal radiological findings. There were no major complications following either technique. CONCLUSIONS: In our experience no major difference exists between the retroperitoneoscopic and laparoscopic approaches for correcting ureteropelvic junction obstruction. The difference in operative time likely reflects the learning curve for laparoscopic suturing and dissection. Currently we prefer the laparoscopic approach because of the larger working space for suturing, the perceived ease of antegrade stent placement and the subjective improvement in cosmetic outcome. The 2 techniques should be considered equal with regard to the successful correction of ureteropelvic junction obstruction.

Vesicoscopic cross-trigonal ureteral reimplantation: a minimally invasive option for repair of vesicoureteral reflux.

PURPOSE: Cross-trigonal ureteral reimplantation is a commonly performed procedure for the correction of vesicoureteral reflux. Most previously described laparoscopic techniques have used an extravesical approach. A "vesicoscopic" technique is analogous to standard open cross-trigonal repair in principle, except that 3 ports with insufflation of the bladder are used to perform the ureteral reimplantation. MATERIALS AND METHODS: A retrospective review was performed of patients treated for primary vesicoureteral reflux with either vesicoscopic or open ureteral reimplantation. For patients with vesicoscopic reimplantation a 5 mm port is placed in the dome of the bladder and 2, 3 mm ports are placed laterally. The ureters are mobilized transvesically, cross-trigonal submucosal tunnels are made and the ureters are sutured in place with intracorporeal suturing. The bladder ports are closed and a urethral catheter is left indwelling for 36 hours. Among the open reimplantation group 38 patients underwent cross-trigonal ureteral reimplantation, 2 underwent extravesical ureteral reimplantation and 2 were excluded due to insufficient records. Followup imaging for both groups included ultrasonography at 1 month and voiding cystography at 3 months postoperatively. RESULTS: A total of 52 consecutive children underwent vesicoscopic ureteral reimplantation with 1 patient converted to open intravesical reimplantation, and 40 consecutive controls underwent open ureteral reimplantation. Postoperative vesicoureteral reflux resolution rates for the vesicoscopic and open groups were 91% (42 of 46 patients) and 97% (31 of 32 patients), respectively. Mean patient age and mean maximal grade of vesicoureteral reflux in the vesicoscopic and open groups were 5.7 and 4.0 years (p=0.001), and 2.8 and 3.2 (p=0.036), respectively. Mean operative times for vesicoscopic and open procedures were 199 and 92 minutes, respectively (p=0.001). While the average length of hospital stay of 2 days was similar between the groups (p=0.122), less oral and intravenous analgesia was needed postoperatively in the vesicoscopic group (p=0.001 and p=0.005, respectively). Complications of vesicoscopic ureteral reimplantation included urinary leakage in 1 child, bladder stones in 1 and bilateral ureteral obstruction in 1. There were no complications in the open group. CONCLUSIONS: There is an ever increasing trend toward minimally invasive surgery. Our preliminary results indicate that vesicoscopic ureteral reimplantation is an effective procedure with minimal morbidity. Although success rates for vesicoureteral reflux resolution were slightly lower in the vesicoscopic group in this study, the favorable results of other series and the improvement in postoperative discomfort observed here suggest that this technique may be a reasonable option in the management of vesicoureteral reflux. Further refinement of the technique and critical analysis of the long-term outcomes are needed to understand fully its place in the treatment of vesicoureteral reflux.