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.

Telemedicine Versus Traditional for Follow-Up Evaluation of Enuresis.

Introduction: Telemedicine allows health care professionals to diagnose and treat patients remotely. Enuresis is one of the most common chronic problems in childhood and specialized care can be limited. Utilization of telemedicine in this setting has not been previously analyzed. Materials and Methods: This study's aim is to evaluate the feasibility and effectiveness of telemedicine follow-up treatment of enuresis compared with traditional follow-up at our institution. A retrospective review of patients treated for nocturnal enuresis with either telemedicine (Group 1) or traditional (Group2) follow-up care was conducted. Patients, aged 5-18 years, treated for enuresis between July 2016 and December 2017 were included. Patients with confounding disease were excluded. Resolution of enuresis was the primary outcome as categorized by the International Children's Continence Society standards. Results: Seventy-seven (n = 77) patients met inclusion criteria with 23 patients in Group 1 and 54 patients in Group 2. Two patients in each group were lost to follow-up and 61.9% in Group 1 and 48.1% in Group 2 responded to treatment. The average age for both groups was 9.2 years. Patients in Group 1 averaged four appointments per patient and patients in Group 2 averaged 3.04 appointments per patient. Telemedicine follow-up patients missed fewer appointments (0.14) than patients with traditional follow-up (0.5) (p-value = 0.016). Thirteen of 21 patients (61.9%) responded to treatment in Group 1 (7 partial and 6 complete responders) as compared with 25 of 52 patients (48.1%) responding to treatment in Group 2 (8 partial and 17 complete responders) (p = 0.22). Of patients in Group 1, 87% reported they would use telemedicine again. Conclusions: Telemedicine follow-up of patients with enuresis demonstrated comparable effectiveness. Most patient families demonstrate a favorable opinion of using telemedicine again for this problem. Further research to understand the efficacy and benefits of telemedicine in this setting is needed.

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.

Altered mechanisms of genital development identified through integration of DNA methylation and genomic measures in hypospadias.

Hypospadias is a common birth defect where the urethral opening forms on the ventral side of the penis. We performed integrative methylomic, genomic, and transcriptomic analyses to characterize sites of DNA methylation that influence genital development. In case-control and case-only epigenome-wide association studies (EWAS) of preputial tissue we identified 25 CpGs associated with hypospadias characteristics and used one-sample two stage least squares Mendelian randomization (2SLS MR) to show a causal relationship for 21 of the CpGs. The largest difference was 15.7% lower beta-value at cg14436889 among hypospadias cases than controls (EWAS P = 5.4e-7) and is likely causal (2SLS MR P = 9.8e-15). Integrative annotation using two-sample Mendelian randomization of these methylation regions highlight potentially causal roles of genes involved in germ layer differentiation (WDHD1, DNM1L, TULP3), beta-catenin signaling (PKP2, UBE2R2, TNKS), androgens (CYP4A11, CYP4A22, CYP4B1, CYP4X1, CYP4Z2P, EPHX1, CD33/SIGLEC3, SIGLEC5, SIGLEC7, KLK5, KLK7, KLK10, KLK13, KLK14), and reproductive traits (ACAA1, PLCD1, EFCAB4B, GMCL1, MKRN2, DNM1L, TEAD4, TSPAN9, KLK family). This study identified CpGs that remained differentially methylated after urogenital development and used the most relevant tissue sample available to study hypospadias. We identified multiple methylation sites and candidate genes that can be further evaluated for their roles in regulating urogenital development.

The role of genetic variation in DGKK on moderate and severe hypospadias.

BACKGROUND: Recent genome-wide association studies of hypospadias have implicated the role of genetic variants in or near the diacylglycerol kinase kappa (DGKK) gene. However, these variants are largely identified among samples of mild and moderate hypospadias cases. Therefore, we evaluated previously identified DGKK variants among second- and third-degree hypospadias cases and controls recruited in Arkansas, a state characterized by a high birth prevalence of hypospadias. METHODS: Second- and third-degree hypospadias non-Hispanic white cases (n = 36 and n = 9, respectively) and controls (n = 45) were recruited at Arkansas Children's Hospital. Preputial tissue was collected on cases and controls between 2013 and 2017. Cases and controls were genotyped using the Illumina Infinium Global Screening Array. We used logistic regression models to assess the association of genotyped and imputed genetic variants mapped to the DGKK region with second- and third-degree hypospadias. RESULTS: All families self-reported as non-Hispanic white and genetic principal component analyses did not demonstrate evidence of population stratification. Five DGKK variants previously reported as associated with hypospadias were identified in the genotype data. None of the variants were associated with second- or third-degree hypospadias (range of odds ratios = 0.7-0.9, all p > .05). CONCLUSIONS: In our analyses, genetic variation in DGKK does not play a role in the development of moderate and severe hypospadias. Our findings provide support to the etiologic heterogeneity of hypospadias by all classifications of severity.

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.

Prophylactic Antibiotics After Stented, Distal Hypospadias Repair: Randomized Pilot Study.

The usage of prophylactic oral antibiotics following distal hypospadias repair with stenting has been recently challenged. This study evaluated the incidence of symptomatic urinary tract infections (UTIs) following stented, distal hypospadias repair and the impact of prophylactic antibiotic therapy. Subjects 0 to 5 years of age with distal hypospadias were randomized to either Group 1 (antibiotics) or Group 2 (no prophylactic therapy). Urinalysis/urine culture was obtained intraoperatively with no preoperative antibiotics given. Phone interviews at 1 month and 3 months after surgery were done. Forty-eight patients were successfully randomized to either Group 1 (24) or Group 2 (24). The incidence of symptomatic UTI in this pilot study is low, and prophylactic antibiotic therapy does not appear to lower the incidence of symptomatic UTI. A larger, randomized, multicenter trial is needed to determine whether antibiotic prophylaxis reduces the risk of symptomatic UTIs following stented, distal hypospadias repair.

Analysis of preoperative antibiotic prophylaxis in stented, distal hypospadias repair.

INTRODUCTION: Surgical site infection [SSI] is a risk for any surgical procedure, including hypospadias repair. Prophylactic antibiotic therapy for patients having surgery is often effective in preventing SSIs, but with increasing rates of antibiotic resistance, this practice has been questioned. The objectives of this study are 1) to assess the incidence of SSIs in patients following stented, distal hypospadias repair and 2) to observe for any potential difference in the incidence of SSIs for patients with and without preoperative antibiotic utilization in this setting. MATERIALS AND METHODS: We retrospectively reviewed consecutive patients treated with stented, distal hypospadias repair from 2011 to 2014 by three surgeons and compared two groups: patients who received preoperative antibiotics and patients who did not. Patients with a history of previous hypospadias repair were excluded from the study. RESULTS: Two hundred twenty-four subjects were identified. Group 1 (135) received preoperative antibiotic and Group 2 (89) did not receive preoperative antibiotics. There was no statistically significant difference in SSI prevalence with 0 patients in Group 1 and 1 patient in Group 2 having a SSI. CONCLUSION: Although prophylactic antibiotics prior to hypospadias repair are most often used by pediatric urologists, this study demonstrates further evidence that antibiotics prior to this procedure do not appear to lower the rate of SSI. This study is limited by its retrospective nature and disparate mean follow up in the two cohorts. Surgical site infection does not appear to be decreased by prophylactic antibiotic therapy before distal hypospadias repair.

To Finish the Cut or Not.

We retrospectively evaluated the management of patients with unrecognized glanular hypospadias and a completed (group 1) or aborted (group 2) neonatal circumcision. The rate and type of subsequent surgeries performed were analyzed. Penile curvature, urinary stream deviation, and their impact on management were evaluated. Surgery was done in 55% of patients-40% of group 1 and 86% of group 2. Completion of the circumcision was done in 63% of group 2. Hypospadias repair was performed in 56% of group 1 and in 34% of group 2. Penile curvature rate did not affect the rate or type of surgery performed. Urinary stream deviation did not affect the rate of repair, but was a significant factor leading to hypospadias repair. We concluded that providers performing neonatal circumcisions do not have to abort the procedure when a glanular hypospadias is noticed. Most patients will require circumcision completion only.

Shortened operative time for pediatric robotic versus laparoscopic dismembered pyeloplasty.

INTRODUCTION: Robotic-assisted laparoscopic pyeloplasty (RALP) is increasingly becoming the standard procedure for management of ureteropelvic junction obstruction (UPJO) in the pediatric population, but few studies have shown a clear advantage over the more technically demanding laparoscopic pyeloplasty (LP) in children. The objective was to study the patients treated with RALP or LP at our institution and the associated outcomes for each minimally invasive approach for the correction of UPJO. MATERIALS AND METHODS: Our laparoscopic and robotic database was queried to identify all patients with a history of primary robotic-assisted or laparoscopic dismembered pyeloplasty for the correction of UPJO performed at our institution from January 2010 through November 2013 and were retrospectively reviewed. We analyzed age, surgical time, blood loss, hospital stay length, postoperative complications, and success rate. RESULTS: Seventy-three total patients were identified as having RALP or LP during this time period with five patients excluded from the analysis. We identified 55 patients with RALP and 13 patients with LP. No differences in success rate or postoperative complications were found for the two cohorts. The length of procedure was significantly shorter for the RALP group compared to the LP group.

Prenatal diagnosis and telemedicine consultation of fetal urologic disorders.

In Arkansas, telemedicine is used commonly in obstetrics through Antenatal and Neonatal Guidelines, Education and Learning System (ANGELS), the existing statewide telemedicine network. This network is used primarily for tele-ultrasound and maternal-fetal medicine consultation. This study is a retrospective case series, describing all the patients who had a prenatally diagnosed urologic anomaly that required prenatal urologic consultation. From 2009-2013, approximately 1300 anomalies were recorded in the Arkansas Fetal Diagnosis and Management (AFDM) database, 14% of which were urologic anomalies. Twenty-six cases required prenatal urologic consultation, 25 of which were conducted via telemedicine. Teleconsultation allowed patients to combine maternal-fetal medicine and urologic consultations in one visit, saving time and effort and ultimately, for most patients, providing reassurance that delivery could be accomplished locally with postnatal follow-up already arranged. While there are several studies reporting the use of telemedicine for various subspecialty consultations, to our knowledge, this is the first to describe the use of telemedicine for prenatal urology consultation. Future research could randomize patients prospectively to allow comparison of both the outcomes as well as the patient experience.

A pilot study of telemedicine for post-operative urological care in children.

We conducted a retrospective study of paediatric urological surgery patients over a 12-month period. We compared patients followed up by telemedicine with those who had post-operative follow-up on site at the Arkansas Children's Hospital (ACH) in Little Rock. All pre-operative patients living in northwest Arkansas were given the opportunity to use telemedicine from a satellite clinic at Lowell, 328 km from the hospital. Of 61 patients, 10 chose telemedicine and 51 chose to be evaluated at the ACH clinic. All telemedicine visits were completed successfully, but in four cases, the video clarity of the telemedicine images was not sufficient for decision-making, and a digital photograph was sent by email to the physician at the ACH. There were no post-operative surgical complications in either patient group. In the telemedicine group, the median distance to the ACH was 330 km, and the median distance to the remote clinic was 35 km. In the on-site group, the median distance to the ACH was 293 km, which was significantly less (P=0.03). In the on-site group, the median travel time to the ACH was 174 min. If the telemedicine group had driven to the hospital, the median travel time would have been 192 min. Logistic regression showed that for every 37 km increase in distance to ACH, patients had a 111% increase in the odds of receiving telemedicine compared to receiving on-site care (OR=2.1, 95% CI: 1.0, 4.4). The pilot study supports the use of telemedicine for the post-operative evaluation of paediatric urology surgery patients and suggests that substantial travel distance and time savings can be made.

Can we rely on the presence of dextranomer-hyaluronic acid copolymer mounds on ultrasound to predict vesicoureteral reflux resolution after injection therapy?

PURPOSE: We determined whether the presence or absence of dextranomer-hyaluronic acid copolymer mounds on bladder ultrasound coincides with voiding cystourethrogram results after injection therapy in children with primary vesicoureteral reflux. MATERIALS AND METHODS: We retrospectively reviewed consecutive cases of dextranomer-hyaluronic acid copolymer injection for primary vesicoureteral reflux. The primary outcome investigated was the appearance of dextranomer-hyaluronic acid copolymer mounds on ultrasound and their association with voiding cystourethrogram results postoperatively. An intramural dextranomer-hyaluronic acid copolymer mound on ultrasound was considered a negative test result for vesicoureteral reflux. RESULTS: A total of 187 cases were identified, of which 132 had imaging available for evaluation. Intramural mounds were seen on ultrasound in 86 cases postoperatively, of which 34 (40%) had a positive voiding cystourethrogram. Of 46 cases in which no mound was identified 21 (46%) had a positive voiding cystourethrogram. The sensitivity of ultrasound to determine the presence or absence of vesicoureteral reflux was 38%, specificity was 67%, accuracy was 55%, the positive predictive value was 46% and the negative predictive value was 60%. CONCLUSIONS: Our results indicate poor correlation of dextranomer-hyaluronic acid copolymer mound appearance on ultrasound with voiding cystourethrogram results after injection. To adequately evaluate for the resolution of vesicoureteral reflux a voiding cystogram or nuclear medicine cystogram needs to be performed after injection therapy.

Spica casting compared to Bryant's traction after complete primary repair of exstrophy: safe and effective in a longitudinal cohort study.

PURPOSE: Bryant's traction is the most commonly used method for immobilization after bladder exstrophy repair. We hypothesized that spica casting is a safe and effective alternative to Bryant's traction after complete primary repair of exstrophy. MATERIALS AND METHODS: Complete primary repair of exstrophy was performed for initial repair in 39 consecutive children by all surgeons at Seattle Children's Hospital since 1998. Three sequential cohorts were evaluated-Bryant's traction without osteotomy (13 patients), spica casting without osteotomy (14) and spica casting with osteotomy. These 3 sequential cohorts represent eras of care and an evolution of practice. Primary outcomes included major complications related to immobilization, dehiscence, urinary incontinence and length of stay. We defined complications of immobilization as nonunion of pelvic osteotomy, femoral nerve palsy, revision of spica cast requiring return to the operating room, infection at the osteotomy site and activity limiting pain at the osteotomy site. Fisher's exact test or t test was used to determine statistical significance. RESULTS: There was no difference in urinary continence (p = 0.09). Use of Bryant's traction was associated with double the length of stay (p >0.001). There was no correlation of major complications to the type of immobilization used. CONCLUSIONS: Spica casting compared to Bryant's traction is associated with shorter hospitalization following complete primary repair of exstrophy and does not have a significant difference in the rate of complications. In our longitudinal cohort study with long-term followup spica cast was safe and effective for patients with bladder exstrophy, and should be considered an acceptable method of immobilization.

Injection volumes of dextranomer/hyaluronic acid are increasing in the endoscopic management of vesicoureteral reflux.

PURPOSE: Dextranomer/hyaluronic acid (Deflux) has been increasingly used for the treatment of vesicoureteral reflux (VUR). Experience has shown that injecting more volume of material is necessary to achieve greater success. We evaluate trends in the number of vials being used to treat VUR using a multi-institutional database and data from patients treated at our own institution. METHODS: Children of age 0-19 years in the Pediatric Health Information System (PHIS) database from 2003 to 2008 were extracted with a VUR diagnosis (ICD-9 593.7x) and subureteric injection procedure code (CPT 52327). We identified children with reflux treated with endoscopic injection at Seattle Children's Hospital from 2005 to 2008. Hospital trends of the number of vials used were evaluated using multivariate linear regression. RESULTS: From 2003 to 2008, we identified 4,078 endoscopic injection procedures in PHIS. There was a 33% increase in the average number of vials used per patient (p < 0.0001) with more than a threefold increase in the number of patients receiving three or more vials per procedure. All institutions increased the average vials used per patient with the most pronounced increase at the highest-volume centers. These trends were also present in the 186 children treated at our own institution. CONCLUSION: Over the study period there was an increase in the number of vials of dextranomer/hyaluronic acid being used per patient to treat children with VUR. This practice may improve success rates but will increase the cost of treatment due to the inherent expense of the material.

The role of the Malone antegrade continence enema (MACE) in the management of myelodeysplatic patients.

Myelodysplasia is a congenital neural tube defect commonly affecting bladder and bowel function. Management of fecal incontinence is paramount to achieve patient independence and self-confidence. When conservative measures fail alternative invasive methods can be successfully applied. The Malone antegrade continence enema (MACE) was introduced in the late 1980's to treat spina bifida patients with fecal incontinence refractory to conservative management. Since its introduction, multiple successful variations have been described and its role has expanded. Indeed, the MACE has revolutionized the care of myelodysplastic patients, their bowel function, and ultimately their self image.

Localized penile bullous pemphigoid of childhood.

Bullous pemphigoid is an acquired autoimmune bullous condition that is uncommon in childhood. Genital involvement is extremely rare. We present a case of a 7-year-old boy with bullous lesions confined to the glans penis. Precise diagnosis is based on clinical presentation and specific histopathologic testing. Oral corticosteroid therapy is the treatment of choice.

Primary renal synovial sarcoma.

Synovial sarcoma of the kidney is a recently reported neoplasm. Only 33 cases have been reported in published studies. It is a difficult neoplasm to differentiate from other renal masses and requires specific molecular/genetic testing. Currently, no treatment guidelines have been established. Accurate diagnosis is imperative to implement effective therapy.