Parental decision regret among Australian parents after consenting to or refusing hypospadias repair for their son: Results of a survey with controls.

INTRODUCTION: Parental decision regret in hypospadias surgery is a recognised source of long-lasting psycho-social morbidity. High parental decision regret after their child's hypospadias repair is reported. The aim of this study is to report on decision regret in Australian parents, who accepted and declined surgery for their son and explore underlying factors for decision-making, satisfaction, and regret. MATERIALS AND METHODS: An online anonymous survey was administered to three groups of parents: 1) parents who consented for hypospadias repair, 2) parents who declined repair and 3) a control group who requested circumcision for their child. Operations occurred between 2010 and 2020 in two paediatric hospitals in New South Wales, Australia. The survey included a validated decision regret assessment tool and additional questions to explore the possible basis of the opinions. RESULTS: One hundred and eighteen parents (invited - 381, completed - 116, response rate - 31%) participated in the survey. Decision regret was present in group 1 (n = 89) - 55% (moderate-to-severe 15%), in group 2 (n = 14) - 71% (moderate-to-severe 57%), and in the control group (n = 15) - 15% (moderate-to-severe 8%) of parents. There was a significant difference in the median decision regret score between all three groups. Parents who chose hypospadias repair were mostly concerned about function. CONCLUSIONS: The prevalence of decision regret among Australian parents who consented for their son's hypospadias repair was lower compared with the mean decision regret reported in the literature to date (55% vs 65%). Decision regret and its severity were highest among parents who declined hypospadias repair. New strategies are needed to reduce decision regret in parents whether they elect for surgery or not.

Practice variation on use of antibiotics: An international survey among pediatric urologists.

INTRODUCTION AND BACKGROUND: Although there is abundance in literature focusing on the use of prophylactic antibiotics for adult urological procedures, the evidence for using antibiotics following common pediatric urological procedures is limited with no specific guidelines for use. Consequently, current practices on antibiotic usage for common interventions may be variable among practicing pediatric urologists, lacking evidence-based support. OBJECTIVE: The aim was to evaluate the current practice pattern on antibiotic usage for common interventions amongst pediatric urologists (PU) practicing in four English-speaking sectors of the world. MATERIALS AND METHODS: An anonymous survey of five scenarios with multiple choice options was disseminated to all active practicing members of the Pediatric Urologist of Canada (PUC) and Society of Pediatric Urology of Australia and New Zealand (SPUNZA), as well as all those attending the 2016 British Association of Pediatric Urology (BAPU) and 2017 American Association of Pediatric Urology (AAPU) meetings. The response for each scenario was summarized for overall practice pattern variation and the pattern for each sector was compared using the Fisher exact test. RESULTS: A total of 126 respondents completed the survey (68.5% response rate) with at least a 65% response rate for each of the four sectors. The majority of respondents do not use antibiotics for indwelling urethral (46.8%) and suprapubic catheters (53.4%); however, they do give antibiotics for J-J stent placement (65.1%) and hypospadias surgery (84.9%), and use antibiotics after hypospadias surgery where catheters or stents are left indwelling (80.9%, 84.2%, respectively). Among those surveyed, the PUC members and AAPU PU demonstrated similar practice patterns which often significantly differed from that of SPUNZA members and BAPU attendees. Specifically, a significantly larger proportion of the North American pediatric urologists do not use antibiotics for common procedures compared with Australia, New Zealand, and the UK (Table). DISCUSSION: In the absence of prospective studies in antibiotic use for pediatric patients to guide clinicians, there is a clear variability among sectors in the use of antibiotics for most clinical scenarios investigated. With increasing resistance patterns and possible adverse effects of antibiotics, it is important that the international pediatric urology community engage in discussions and collaborations to address this issue. CONCLUSION: Practice patterns in antibiotic usage amongst PU varies widely, some of which may be associated with their local "culture." There is a need to understand these differences and begin to standardize treatment in the hopes of increasing appropriate use of antibiotics internationally.

Prolonged urinary retention can and does occur after any type of ureteral reimplantantion.

OBJECTIVE: Transient urinary retention has been recognized as a complication of bilateral ureteroneocystostomy (UNC), when performed extravesically. The objective of this study was to review a collective surgeons' experiences of unilateral extra- and unilateral and/or bilateral intra-vesical ureteral reimplanation, where urinary retention greater than 6 weeks, or what we have termed, "prolonged urinary retention" (PUR), occurred. MATERIALS AND METHODS: We retrospectively reviewed charts to identify PUR after any open or robotic reimplant, other than bilateral extravesical, between 1998 and 2015 as reported by five surgeons. RESULTS: During the review period, ten cases were documented where PUR was encountered. Bilateral Cohen reimplants (5), unilateral extravesical open reimplant with ureteral tapering (3), unilateral Cohen reimplant (1) and unilateral extravesical robotic reimplant with tapering (1) were associated with PUR. Younger males predominated (70%). The mean age at operation of the patients was 3.1 years. Eventually 7/10 patients were able to void normally, with periods ranging from 6 weeks to 8 years. The remaining three patients are still unable to void more than 5 years after UNC. A majority of the samples (6/10) were suspected to have bowel and bladder dysfunction (BBD), but neurologically all were normal. CONCLUSION: PUR can occur as a potential complication following any type of UNC and is associated with the risk of significant morbidity, including permanent urinary retention. Patients and caregivers should be counseled accordingly.

Early mercaptoacetyltriglycine(MAG-3) diuretic renography results after pyeloplasty.

OBJECTIVE: To describe the drainage and functional outcome of paediatric pyeloplasty, 1 week after stent removal 7-9 weeks after pyeloplasty using diuretic renography. PATIENTS AND METHODS: Between 2009 and 2014, we assessed the functional and drainage outcomes according to mercaptoacetyltriglycine MAG-3 diuretic renograms from 66 children (69 kidneys) who underwent modified dismembered Anderson-Hynes pyeloplasty for pelvi-ureteric junction (PUJ) obstruction. Stents were left in place for 6-8 weeks and postoperative renal units were evaluated with MAG-3 renogram 1 week after stent removal. Surgical success was defined by improvement of drainage (half clearance time [T/2] < 20 min), stable or improved function on the postoperative MAG-3 renogram and by decreased pyelocaliceal dilatation on ultrasonography (US) at 1 year. RESULTS: Of the 69 kidneys with a preoperative median range T/2 of 33.4 (7.6-200) min, 60 (87%) had improved drainage curves, with a median (range) T/2 of 6.9 (1.6-19) min. Thirteen percent (9/69) had persistent impaired drainage, with a median (range) T/2 of 36 (24-108) min. Of these nine children, one girl was found to have a persistent obstructive pattern (T/2 = 30 min) associated with a decreased split renal function (SRF; from 42 to 33%) and persistent hydronephrosis (at 28 mm). Redo pyeloplasty was performed 2 months after the initial procedure (and 18 days after stent removal) and renal function recovered to 47%. The remaining eight patients were free of symptoms; hydronephrosis improved at 1 year (anteroposterior diameter decreased from 28 to 18.5 mm; P = 1.94) and SRF remained stable (44.5 vs 48.5% after repair; P = nonsignificant). In the 29% of kidneys (20/69) that had preoperative impaired SRF, postoperative renal function improved in 75% (from 27.5 to 43%; P < 0.001), remained unchanged in 2% and one kidney (0.2%) deteriorated. The median (range) postoperative follow-up was 18 (12-90) months. CONCLUSIONS: There is no agreement regarding the 'gold standard' investigation to use after pyeloplasty for PUJ obstruction. Improvement in hydronephrosis on US is slow and often takes > 12 months. Based on animal studies, it is possible that missed recurrent obstruction will cause irreversible loss of renal function after 6 weeks; therefore, early postoperative assessment is desirable, but there have been few reports on urinary drainage changes with early diuretic renography after pyeloplasty. Most of the renal units had improved drainage on diuretic renography 7 weeks after pyeloplasty and 1 week after stent removal. An early diuretic renogram is a reliable method of documenting surgical success after pyeloplasty.

Anderson-Hynes pyeloplasty: are we all really on the same page?

BACKGROUND: The Anderson-Hynes pyeloplasty (AHP) is a surgical technique used in the management of pelvi-ureteric junction obstruction. This operation has been performed for over six decades and has etched its name in the annals of urology. But are we all really referring to the same procedure? METHODS: A systematic review of the Ovid MEDLINE(R) and Ovid OLDMEDLINE(R) database from 1946 to 20 June 2014 was performed for the terms 'Anderson and Hynes' and 'Anderson-Hynes', with later restriction to the title of journal article. Each respective author's descriptive images (figures/photographic) of technique performed was compared with the original procedure. Non-English figure legends were translated using online translational tools. RESULTS: In total, 242 articles were retrieved. Streamlining this search to articles with the above search terms restricted to article title revealed 58 (34 English/24 non-English) articles. Only 29/58 papers had referenced the original procedure. Operative images were present in 17/58 of the articles claiming to have performed the AHP. Within these papers, only 7/17 articles depicted both the L-shaped cut and pelvic flap. Diverse variations were observed in the remainder 10/17 articles. This was a worldwide phenomenon, observed more commonly in recent decades and when minimally invasive techniques were used. CONCLUSION: The AHP is often not performed as was originally described. Over the decades, surgeons have unknowingly attributed variations to this procedure. Based on this review, a novel 'geometric classification' system has been proposed to better define the dismembered pyeloplasty.

Uncommon causes of anterior urethral diverticula in children: Two cases and review of literature.

Anterior urethral diverticula are rare in children. Anterior urethral valves and associated diverticulum is the commonly discussed pathological entity in children. There is a lack of awareness among clinicians regarding less common presentations of anterior urethral diverticula in children; which can have a diverse involvement of the urinary tract. This report describes two uncommon presentations of anterior urethral diverticula in children, their diagnoses and management. A systematic differential diagnosis and review of anterior urethral diverticula in children is also presented.

Testicular microlithiasis: the importance of self-examination.

AIM: To explore the issue of appropriate management of testicular microlithiasis. We report the third ever case of tumour arising from a testis previously known to have microlithiasis in childhood and review the literature to provide an evidence-based approach to management of testicular microlithiasis. METHODS: Case report and review of previous literature. RESULTS AND CONCLUSIONS: Although there is a strong association between testicular microlithiasis and testicular malignancy at diagnosis, there are only three reported cases of subsequent tumour development in childhood. Testicular microlithiasis is an increasingly recognised entity. There is insufficient evidence in the current literature to support any regime of clinical surveillance. Self-examination is the most important factor in the early detection of testicular malignancy.

Antibiotic prophylaxis for transurethral urological surgeries: Systematic review.

The use of antibiotic prophylaxis to prevent urinary tract infection and bacteremia (sepsis) following endoscopic urologic procedures is a controversial topic. Evidence in the literature revealed that urological instrumentation is associated with increased incidence of urinary tract infection and bacteremia. The aim of this review is to evaluate the effectiveness of antibiotic prophylaxis in reducing the risk of urinary tract infection in patients who had transurethral urological surgeries. We have selected all RCTs of adult population who underwent all different types of transurethral urological surgery, including cystoscopy, transurethral resection of prostate and transurethral resection of bladder tumor, and received prophylactic antibiotics or placebo/no treatment. At first, more than 3000 references were identified and reviewed; of which 42 studies with a total of 7496 patients were included in the final analysis. All those trials were analyzing antibiotic prophylaxis versus placebo/no treatment, and they were significantly favoring antibiotic use in reducing all outcomes, including bacteriuria (RR 0.36, 95% CI 0.29 to 0.46, P < 0.0001) with moderate heterogeneity detected (I(2) 48%), symptomatic UTI (RR 0.38, 95% CI 0.28 to 0.51, P < 0.0001) with no significant heterogeneity was detected (I(2)= 17%), bacteremia (RR 0.43, 95% CI 0.23 to 0.82, P < 0.0001) with no noted heterogeneity (I(2) = 0%), and fever ≥38.5 Celsius (RR 0.41, 95% CI 0.23 to 0.73, P = 0.003); also, there was no noted heterogeneity (I(2) = 0%). However, using antibiotic prophylaxis did not reduce the incidence of low grade temperature (RR 0.82, 95% CI 0.61 to 1.11, P = 0.20) or in moderate grade temperature (RR 1.03, 95% CI 0.71 to 1.48, P = 0.89). Antibiotic prophylaxis appears to be an effective intervention in preventing urinary tract infections and its sequels following transurethral urological surgeries in patients with preoperative sterile urine.

Ambulatory urodynamic studies (UDS) in children using a Bluetooth-enabled device.

OBJECTIVES: • To report the early observations of using ambulatory urodynamic studies (UDS) using a Bluetooth-enabled device in children • To evaluate the incremental value of ambulatory over conventional UDS. PATIENTS AND METHODS: • Ambulatory UDS were performed in selected children with voiding dysfunction between August 2009 and October 2010. • Conventional UDS were concurrently performed wherever possible. • The test results and treatment consequences of the two tests were compared. RESULTS: • In all, 12 ambulatory and seven conventional UDS were performed on 10 children (five boys, median [range] age 7 [4-16] years). • Six of the seven children had a normal conventional UDS. Ambulatory UDS detected phasic detrusor overactivity (DO) in five children and generalised DO in one. • Direct correlation of symptoms to DO was possible in two children during ambulatory UDS. Pressure rise during filling, seen in two children on conventional UDS, was not seen during ambulatory UDS. • Five children showed clinical improvement when therapy was guided by ambulatory UDS results. • Ambulatory UDS was generally well tolerated in eight children, with two complaining of discomfort. Inadequate information was obtained in two children who underwent ambulatory UDS due to technical problems in one and distress induced by the UDS in the other. CONCLUSIONS: • Ambulatory UDS provides useful additional information over conventional UDS and can be used to guide further therapy in selected children with voiding dysfunction. • It is safe and well tolerated in children. • There is a need for explicit guidance for the technical delivery and interpretation of ambulatory UDS in children.

Treatment-responsive pandysautonomia in an adolescent with ganglionic α3-AChR antibodies.

Autoimmune autonomic ganglionopathy (AAG) is a rare disorder that presents with pandysautonomia typically in middle age and elderly patients. AAG is typically associated with serum autoantibodies that bind to the alpha-3 subunit of the ganglionic acetylcholine receptor (α3-AChR Ab). We report a 13 year old girl who presented with gut pseudo-obstruction, bladder dysfunction and dilated pupils unresponsive to pilocarpine. She had positive α3-AChR Ab plus other autoantibodies suggesting an autoimmune diathesis. Our patient was initially resistant to steroid therapy but responded to the addition of azathioprine resulting in a near complete clinical remission. We conclude that pandysautonomia associated with α3-AChR Ab can occur in children and has multi-organ involvement.

Management of daytime urinary incontinence and lower urinary tract symptoms in children.

Lower urinary tract symptoms, particularly urgency, frequency and incontinence are common in school-aged children but are often overlooked. They may cause considerable physical, social and psychological difficulties to children and their families, and usually are manifestations of underlying non-neurogenic voiding disorders. The differential diagnoses include overactive bladder syndrome, dysfunctional voiding and vaginal reflux as well as less common conditions like giggle incontinence, voiding postponement, pollakiuria and diabetes insipidus. In this paper, we discuss an evidence-based approach to the management of conditions causing daytime urinary incontinence and lower urinary tract symptoms in children from a general paediatrician's perspective.

Factors influencing quality of life in children with urinary incontinence.

PURPOSE: We evaluated quality of life in children with urinary incontinence using a disease specific tool (Pediatric Incontinence Questionnaire) and determined factors that decrease quality of life in affected children. MATERIALS AND METHODS: The Pediatric Incontinence Questionnaire was self-administered by children 6 to16 years old with urinary incontinence while attending outpatient clinics at a tertiary pediatric hospital in Australia between October 2009 and May 2010. A weighted summative quality of life score with a range of 1.75 to 7 (7 being lowest quality of life) was generated, and patient characteristics (age, gender, ethnicity, symptom severity) were evaluated as potential predictors. RESULTS: Of 146 children invited to participate 138 consented (response rate 95%). About half of the participants (77) were boys, and mean patient age was 10 years. Girls had a lower quality of life than boys (mean score 3.60 vs 3.31, 95% CI 0.10-0.57, p=0.04), and nonwhite children had a lower quality of life than white children (3.97 vs 3.35, 95% CI 0.23-0.99, p<0.01). Older age (r=0.21, p=0.01) but not increasing symptom severity (r=0.15, p=0.09) or underlying chronic disease (difference 0.12, p=0.91) was correlated to decreased quality of life. Multivariate regression analysis demonstrated that older age, nonwhite ethnicity and female gender were independent predictors of decreased quality of life. CONCLUSIONS: Older age, female gender and nonwhite ethnicity are associated with a lower disease specific quality of life in children with urinary incontinence. Clinicians need to be aware of the differential effect of urinary incontinence in children of different ages and ethnic backgrounds.

High grade primary vesicoureteral reflux in boys: long-term results of a prospective cohort study.

PURPOSE: We evaluated the incidence of new permanent defects in boys with grade 4 or 5 vesicoureteral reflux, identified the risk factors for new permanent defects and reviewed the outcome of different management approaches by assessing the rates of urinary tract infection and new permanent defects. MATERIALS AND METHODS: This prospective cohort study recruited patients from July 1995 to December 2006. Study inclusion criteria were male gender and grade 4 or 5 primary vesicoureteral reflux. Patients were divided into 2 groups by presentation mode, including group 1-prenatal reflux diagnosis and group 2-reflux diagnosed after investigation for urinary tract infection. All patients underwent initial renal (99m)Tc-dimercapto-succinic acid scan evaluation. Continuous antibiotic prophylaxis was given in all patients until at least age 2 years. Surgical correction for reflux was done in 28 patients and 76 were circumcised. Followup included renal (99m)Tc-dimercapto-succinic acid scan with renal ultrasound at age 12 months with repeat (99m)Tc-dimercapto-succinic acid scan at ages 2 and 4 years. RESULTS: Included in our study were 151 patients (206 high grade refluxing renal units) with a median age at diagnosis of 1.9 months (range 1 day to 8.8 years). Median age at first followup was 14 months (range 3 months to 3 years) and at next followup it was 39 months (range 10 months to 11.3 years). There were 52 boys (34%) in group 1 and 99 (66%) in group 2. Baseline perfusion defects on initial renal (99m)Tc-dimercapto-succinic acid scan were identified in 41 of 52 boys (78.8%) in group 1 and in 74 of 99 (74.7%) in group 2. During followup new permanent defects developed in 8 of 52 boys (15%) in group 1 and in 10 of 99 (10%) in group 2. In 18 patients a total of 20 renal units showed new permanent defects, including 13 in kidneys with baseline perfusion defects and 7 in previously normal kidneys (p >0.9). In groups 1 and 2 combined infection developed before and after circumcision in 62 of 137 (45.2%) and 5 of 74 cases (6.7%), respectively (p <0.001). New permanent defects were seen in 4 of 76 circumcised (5.2%) and in 14 of 137 uncircumcised boys (10.2%) (p >0.3). CONCLUSIONS: Baseline perfusion defects were seen on (99m)Tc-dimercapto-succinic acid scan at presentation in 115 of our 151 patients (76%) independent of presentation mode. New permanent defects developed in abnormal and previously normal kidneys, and were associated with urinary tract infection. Being circumcised was associated with fewer urinary tract infections and a lower incidence of observed new permanent defects (5.2% vs 10.2%).

Study of botulinum toxin A in neurogenic bladder due to spina bifida in children.

BACKGROUND: We report results of a pilot study investigating the safety and efficacy of Botulinum A toxin on urinary incontinence and bladder function in children with neurogenic bladder. METHODS: This was a prospective, non-randomized clinical trial. Seven children with median age of 16 years with spina bifida who had high storage pressures, poor bladder compliance and had failed treatment with anticholinergic medications were offered a single intra-detrusor injection of Botulinum A toxin. All subjects were on clean intermittent catheterization before and during the study. Follow-up videourodynamic studies were performed at 1 month, between 3 and 6 months, and at 9 months. Data were collected on safety and on subjective outcomes through validated questionnaires filled out by patients at each visit. RESULTS: In majority of the patients (5/7), the injection produced an increase in bladder compliance (P < 0.05) and an improvement in incontinence (P < 0.05) at 1-month follow-up. However, in two patients whose baseline bladder capacity was markedly reduced (<200 mL), the improvement was very minimal. The beneficial effects in bladder compliance and incontinence dissipated by 9 months. The changes in subjective outcomes (incontinence and satisfaction scores) did not parallel the changes in urodynamics through the study period. No side effects of Botulinum toxin were seen. CONCLUSION: Botulinum A toxin injection produces beneficial urodynamics and clinical effects. These beneficial effects last for approximately 9 months. There is a poor correlation between improvement in the urodynamics and the subjective outcomes. Botulinum A toxin injection is a safe alternative treatment for patients with spina bifida and a neurogenic bladder.

Pelvi-ureteric junction obstruction in duplex kidneys.

OBJECTIVE: To report our experience of 11 children with pelvi-ureteric junction obstruction (PUJO) in a duplex kidney and to review previous reports. PATIENTS AND METHODS: From 1995 to 2005 the second author (G.H.H.S.) performed 145 pyeloplasties in single-system kidneys and in 11 children with PUJO in a duplex kidney. Upper and lower pole obstructions were found in both complete and incomplete duplicated systems. Investigations included renal ultrasonography, renal scintigraphy, micturating cystography, cystoscopy and retrograde pyelography. Ten patients had surgery (eight dismembered pyeloplasties, one upper-pole heminephrectomy, one pyelo-ureterostomy). RESULTS: Three children had obstruction of the upper moiety and eight had obstruction of the lower moiety. Upper and lower pole obstructions were found in both complete and incomplete duplicated systems. CONCLUSION: Upper-pole obstruction in a duplex kidney is rare; the present series suggests that it is more common than previously reported.

EEC syndrome, Arg227Gln TP63 mutation and micturition difficulties: Is there a genotype-phenotype correlation?

We report on two unrelated families with EEC syndrome (ectrodactyly, ectodermal dysplasia, cleft lip/palate), each with an Arg227Gln TP63 gene mutation, where the phenotype overlapped extensively with the allelic disorder, limb-mammary syndrome (LMS). Features common to both families were an ectodermal dysplasia principally affecting tooth, breast and nipple development, dacryostenosis and severe micturition difficulties. Additional findings included post-axial digital hypoplasia, cleft uvula, anal stenosis, hypoplasia of the perineal body and biopsy-proven interstitial cystitis. No individual had cleft lip. Split hand-split foot malformation (SHFM) occurred in one child-born after the molecular diagnosis was established. Unlike previous reports, the urinary symptoms were refractory to treatment with oral Fibrase and persisted into adulthood. Of the six cases/families now reported with EEC syndrome and Arg227Gln TP63 mutation, four have manifested this distinct urological abnormality, indicative of a genotype-phenotype correlation.