The rationale for bladder washouts in children with neurogenic bladder.

Children with a neurogenic bladder are at risk of developing recurrent urinary tract infections and long-term kidney failure. Due to an altered lower urinary tract, children may be overtreated for simple bacteriuria or undertreated for a potentially severe urinary tract infection. This group of patients represent high users of healthcare, and are at risk of colonization and development of antibiotic resistance. Bladder washouts with non-antibiotic electrochemically activated solutions are a potential new prophylactic option for patients with bladder dysfunction when clean intermittent catheterization has resulted in chronic bacteriuria.

The rationale for nephron-sparing surgery in unilateral non-syndromic Wilms tumour.

The central question of nephron-sparing surgery in unilateral non-syndromic Wilms tumour sits at a crossroads between surgery, oncology, and nephrology. There has been a significant paradigm shift in paediatric oncology towards reducing toxicity and addressing long-term treatment-related sequalae amongst childhood cancer survivors. After paediatric nephrectomy and 30-50 years of follow-up, 40% of patients will have chronic kidney disease, including 22% with hypertension and 23% with albuminuria. It is difficult to predict which patients will progress to develop hypertension, reduced glomerular filtration rate, albuminuria, and a higher cardiovascular risk. For these reasons, nephron-sparing surgery when it is technically feasible must be considered. To decrease the incidence of positive surgical margins (viable tumour present at a resection margin), incomplete lymph node sampling, and complications, these procedures should be performed at specialist and experienced reference centres. Based on the impacts of individual treatment pathways, survivors of childhood WT need to be followed through adulthood for early detection of chronic kidney disease, hypertension, and prevention of cardiovascular events.

Influence of foetal position on the degree of isolated antenatal hydronephrosis.

BACKGROUNDS: The effect of foetal position on the degree of antenatal hydronephrosis (ANH) is unknown. We hypothesized that foetal position is an important contextual factor in ANH, with consequences on prenatal counselling and postnatal management. The current study aimed to investigate the effect of foetal position on the degree of ANH. METHODS: A retrospective study was carried out on consecutive pregnancies with isolated ANH over a 10-year period. Gestational age, foetal presentation (cephalic vs. breech), and degree of ANH (as measured by the AP diameter) were retrieved. Foetuses with other ultrasound findings of the renal tract (renal parenchymal abnormality, ureteric dilatation, or bladder abnormalities) were excluded. Logistic regression analysis was conducted to examine the relationship between the anterior-posterior diameter (APD) and the mode of presentation (cephalic or breech). A P-value of <0.05 was considered significant. RESULTS: Initially 745 scans were identified, with 436 included in the final analysis. Overall, there was an association between foetal presentation and APD regardless of trimester, with cephalic presentations having a greater APD (OR 1.07, 95% CI 1.02-1.11, P = 0.04). CONCLUSION: Cephalic presentations are associated with a significantly greater APD indicating that foetal position should be considered when stratifying ANH. Furthermore, foetal position may be a contributing factor in the mechanism of 'physiological' hydronephrosis.

The burden of radiation exposure in children requiring percutaneous nephrolithotomy.

BACKGROUND: Children undergoing investigation and management for complex upper tract urolithiasis often require multimodal imaging. The significance of related radiation exposure in stone care pathways has received little attention in the published literature. STUDY DESIGN: Medical records of paediatric patients undergoing percutaneous nephrolithotomy were retrospectively analysed to ascertain the modalities used and determine extent of radiation exposure occurring during each care pathway. Radiation dose simulation and calculation was performed a priori. The cumulative effective dose (mSv) and cumulative organ dose (mGy) for radiosensitive organs was calculated. RESULTS: A total of 140 imaging studies were included from the care pathways of 15 children with complex upper tract urolithiasis. Median follow-up was 9.6 years (range: 6.7-16.8 years). The average number of imaging studies with ionising radiation per patient was nine, with a cumulative effective dose of 18.3 mSv across all modalities. The most common modalities were: mobile fluoroscopy (43%), x-ray (24%), and computed tomography (18%). The cumulative effective dose per study type was greatest for CT (4.09 mSv), followed by fixed and mobile fluoroscopy (2.79 mSv and 1.82 mSv, respectively). CONCLUSION: There is high general awareness of radiation exposure involved in CT scanning with resultant caution in employing this modality in paediatric patients. However, the significant radiation exposure relating to fluoroscopy (whether fixed or mobile) is less well documented in children. We recommend implementing steps to minimise radiation exposure by optimisation and avoidance of certain modalities where possible. Paediatrics urologists must employ strategies to minimise radiation exposure in children with urolithiasis, given the significant exposures encountered.

Day Surgery in Children Undergoing Retroperitoneal Robot-assisted Laparoscopic Pyeloplasty: Is It Safe and Feasible?

Background: Robot-assisted pyeloplasty is the most frequently performed robotic procedure in children. A retroperitoneal approach limits surgical trauma and avoids peritoneal irritation. This led to the establishment of the criteria for day surgery (DS) and a related clinical care pathway. Objective: To assess the feasibility and safety of DS in children undergoing retroperitoneal robot-assisted laparoscopic pyeloplasty (R-RALP). Design setting and participants: We performed a bicentric prospective study (NCT03274050) over 2 yr involving the two major paediatric urology teaching hospitals in Paris. A clinical pathway and a prospective research protocol were specifically established. Intervention: DS in selected children undergoing R-RALP. Outcome measurements and statistical analysis: The primary outcomes were DS failure, 30-d complications, and readmission rates. The secondary outcomes included preoperative characteristics, perioperative parameters, and surgical outcomes. Quantitative variables were expressed as medians with interquartile ranges. Results and limitations: Thirty-two children fulfilled specific inclusion criteria and were consecutively selected for DS following R-RALP. The median patient age was 7.6 yr (4.1-11.8) and weight 25 kg (14-45). The median console time was 137 min (108-167). There were no intraoperative complications or conversions. Six children were kept under observation overnight and discharged the following day due to persistent pain (n = 3), parental anxiety (n = 2), or a prolonged procedure (n = 1). The median duration of hospital stay of the 26 children in the DS setting was 12.7 h (12.2-13.2). During the 30-d period, there were four emergency room visits (15%) resulting in two patients requiring readmission (8%): one for febrile urinary tract infection (Clavien-Dindo II) and one child with no JJ stent for urinoma (Clavien-Dindo IIIb). Radiological studies confirmed improvement in dilatation for all cases with no recurrence (median follow-up: 15 mo). Conclusions: This prospective case series is the first to demonstrate the feasibility and safety of DS in children undergoing R-RALP, obviating the need for routine inpatient care. Excellent results can be achieved by careful patient selection, a clear clinical pathway, and a dedicated team. Further evaluation is warranted to assess the cost effectiveness. Patient summary: This study shows that day surgery after robotic pyeloplasty is both safe and effective in selected children.

Medullary pyramid thickness: The optimal cut-off value associated with the need for pyeloplasty in infants.

INTRODUCTION: The medullary pyramid compresses during the early phases of severe hydronephrosis and represents a promising ultrasound metric for the diagnosis and surveillance of PUJ obstruction. The aim of this study was to define the optimal cut-off value and utility of medullary pyramid thickness (MPT) associated with the need for pyeloplasty in infants being followed up for hydronephrosis. METHODS: A retrospective review was performed over a five-year period to identify patients that were under surveillance for hydronephrosis during infancy and underwent a MAG3 to monitor the possible need for pyeloplasty. Ultrasound images were retrospectively reviewed to measure the MPT of the affected kidney in a blinded fashion. The primary outcome measure was subsequent requirement for pyeloplasty before three years of age. The Mann-Whitney U Test was used to determine statistically significant differences in the minimum MPT between the infant group requiring pyeloplasty and the non-operative group. Receiver operating characteristic analysis was performed to determine the optimal cut-off value associated with the requirement for pyeloplasty. RESULTS: A total of 63 patient cases were included, of which 45 underwent pyeloplasty (70%). A significant difference was found in the median MPT measurement between the pyeloplasty and non-operative groups (1.7 mm vs. 3.8 mm, p < 0.001). The optimal cut-off value of MPT associated with pyeloplasty was 3.4 mm. An MPT threshold of ≤3.4 mm conferred a sensitivity of 98%, specificity of 63%, positive predictive value of 86%, and negative predictive value of 92%. CONCLUSION: Thinning of the medullary pyramid is an important ultrasound sign of parenchymal deterioration in high-grade hydronephrosis. An optimal MPT cut-off value of ≤3.4 mm is associated with subsequent pyeloplasty in infants. MPT should be considered in future studies addressing the diagnosis and surveillance of PUJ obstruction.

Defining the role of pre-operative hormonal therapy in hypospadias.

In hypospadias surgery, pre-operative hormonal therapy (PHT) is primarily used to increase penile dimensions and the vascularity of tissues available for reconstruction, but its use is non-uniform in clinical practice, with no consensus on application or utility. This review aims to summarise: (i) the penile tissue response to hormone therapy, (ii) its impact on hypospadias surgery outcomes, and (iii) the endocrinological considerations and sequelae. PHT is more often indicated for complex cases such as proximal hypospadias, hypospadias with microphallus and hypospadias reoperations. While PHT has clear effects on penile morphometry, and more recent controlled trials suggest improved surgical outcomes, the lack of consistent outcome definitions and generally inadequate follow-up periods continue to consign many of the potential long-term effects of PHT to the unknown. There is currently insufficient robust evidence to allow a clinical guideline to be constructed. The need for a well-powered multi-centre prospective randomised trial to address this question is evident but awaits a unified consensus on issues surrounding the understanding of aetiology, classification of hypospadias morphology, definition of important prognostic variables and uniform application of outcome measures. The effects of PHT may be utilised to improve outcomes in cases of proximal and severe hypospadias, which under the current paradigm represent a significant surgical challenge.

Paediatric kidney transplantation: Towards a framework for pretransplant urological evaluation.

The role of the urologist in paediatric kidney transplantation has evolved alongside advances in management for the various causes of end-stage kidney disease. Improvements in antenatal intervention and postnatal care have seen children with increasingly complex urological anomalies survive until transplant. Once solely responsible for the oversight of a child's surgical care, the paediatric urologist now works within a multidisciplinary transplant team, alongside transplant surgeons, paediatric nephrologists, transplant coordinators, psychologists, social workers, and transitional care specialists. We sought to identify available pretransplant evaluation frameworks to guide urological preparation and decision-making. Drawing from available evidence and reflecting on multi-institutional experience, we propose a streamlined approach to urologic assessment, which recognises that optimal transplant outcomes in this heterogenous cohort require lower urinary tract dysfunction to be carefully defined preoperatively.

Justin H. Kelly and his procedure for bladder exstrophy and epispadias.

The operations involved in the repair of complete bladder exstrophy (CBE), familiarly known as the Kelly procedure, evolved over more than 100 years. Through repeated cycles of trial and error, some of the most prominent urologists in the world developed techniques that addressed each of the formidable surgical challenges presented by CBE and epispadias. A key figure is Justin H. Kelly of the Royal Children's Hospital, Melbourne, who made surgery for CBE his life's work. He took the lessons of his surgical predecessors, giants like: Friedrich Trendelenburg, Hugh Hampton Young, John Dees, and Guy Leadbetter, applied techniques for anorectal anomalies from his contemporary Alberto Peña, and saw his procedures improved by the next generation of leaders in paediatric urology that included Phillip Ransley, Peter Cuckow, Patrick Duffy, and John Gearhart. Over his long career, Mr Kelly modified and perfected his eponymous procedure patient-by-patient through a painstaking process of trial-and-error, bearing with his young patients and their families through every heart-breaking complication, and gradually creating the standard operation for children with CBE and epispadias.

Nomogram of paediatric male urethral size: A systematic review.

INTRODUCTION: Knowledge of the normal size of the urethral meatus in boys is important for safely performing urethral catheterization and fundamental to the diagnosis and treatment of paediatric urological conditions. However, clinicians often rely subjectively on previous experience and clinical judgement, rather than a robust evidence-base. A systematic review of the literature was undertaken to define the calibre of the male urethral meatus in paediatric age-groups. METHODS: The MEDLINE and EMBASE databases were systematically searched from inception to December 2020 for studies measuring meatal calibre in boys up to 16 years of age. Google Scholar searches along with forward and backward citation tracking identified additional studies. Studies of subjects >16 years, females, or patients with urethral diseases were excluded. RESULTS: Nine articles (2084 paediatric subjects) were included in the final review. Mean meatal calibre increases non-linearly with age, with accelerated growth occurring during infancy (mini-puberty) and adolescence (puberty). Meatal calibre can be approximately grouped by age as follows: neonate (6-10Fr); infant (10-12Fr); child (12-14Fr); and adolescent (14-18Fr). There is substantial individual variability for same-aged children. Meatal calibre has been independently related to height, weight, and penile size, but the effects of ethnicity and circumcision remain unclear. CONCLUSIONS: The evidence-base for this fundamental aspect of paediatric urology includes only a few low-quality studies and is largely derived from one early study of circumcised American boys. Further studies are warranted to establish normative urethral size values based on rigorous, contemporary data with known dependent variables also included.

Access to emergency paediatric surgery for testicular torsion or intestinal volvulus in New Zealand: A system perspective.

AIM: The risk of organ loss is increased in children with testicular torsion or intestinal volvulus if surgical management is not expedient. The current retrospective study aims to review the time-course from first symptom to 'knife to skin' in these conditions, to determine where delays occur and facilitate a systems approach to better manage these children. METHODS: One hundred consecutive paediatric cases of scrotal exploration for presumed testicular torsion, and 100 neonatal cases presenting with possible malrotation/volvulus were analysed to evaluate the exact time-course of events from admission to surgery. RESULTS: (i) Scrotal exploration: the median time from onset of symptoms to presentation was 12 h (interquartile range (IQR): 5-48 h). In children over 5 years of age, 36% (33/93) were transferred from an external district service area. (ii) Malrotation/volvulus: the median duration of symptoms prior to arrival/assessment was 12 h (IQR: 4-24 h). The median cumulative in-hospital time was over 6 h (368 min, IQR: 247-634 min). CONCLUSIONS: Time to presentation contributes significantly to testicular ischaemic time. This delay to timely surgical intervention is multi-factorial, and must be addressed at a public health level. Support and training in the management of testicular torsion should be provided to all adult surgeons/trainees that may care for these children. In general, this condition is best managed at the presenting hospital whenever appropriate expertise is available. Novel pathways that streamline care may improve efficiency at an institutional level. Addressing issues of access to specialised neonatal surgery is more vexed on account of the tyranny of distance, and the pre-requisite level of surgical expertise required.

Complication profile of augmentation cystoplasty in contemporary paediatric urology: a 20-year review.

BACKGROUND: The aim of this study was to describe the complication profile of augmentation cystoplasty in contemporary paediatric urology as well as its effect on bladder metrics. METHODS: Consecutive operative cases were retrospectively reviewed at a single institution over 20 years (1999-2019). Short- and long-term outcomes and complications following augmentation cystoplasty were defined. RESULTS: Of the 71 operative cases; the most common underlying diagnoses were neurogenic bladder (34%), exstrophy-epispadias complex (30%) and posterior urethral valves (23%). The most common tissue-type utilized was ileal (58%) and ureteric (30%). Peri-operative urine leak affected nine (13%) children but reservoir perforations were less common (4%). Mean end-of-study detrusor pressure improved significantly following bladder augmentation (38-17 cmH2 O, P < 0.001). Bladder capacity improved significantly (67-89%, P = 0.041). The median follow-up was 4.5 years (interquartile range: 1.9-10 years). Bladder urolithiasis affected 13 (18%) patients, and symptomatic urinary tract infections 36 (51%) patients. Formation of a continent catheterisable channel contributed a number of complications relating predominantly to stenosis (50%). Repeat augmentation cystoplasty was necessary in three (4%) cases. CONCLUSION: Augmentation cystoplasty is a surgical intervention that improves bladder metrics. Given the potential complications, careful patient selection and appropriate pre-operative counselling are essential. Furthermore, pro-active post-operative management and transitional care are vital in the surgical care of children following augmentation cystoplasty.

The bell-clapper deformity of the testis: The definitive pathological anatomy.

INTRODUCTION: The bell-clapper deformity (BCD) predisposes to intravaginal torsion (IVT) and is classically bilateral. The precise pathological definition of what constitutes a BCD is not clear. The current study aims to clarify the specific anatomic details of this anomaly. METHODS: A systematic review was performed utilizing the PRISMA principles. Studies are presented chronologically based on their level of evidence. They are further divided into study types: autopsy and operative studies of acute torsion, intermittent torsion and studies of the contralateral testis in vanishing testis. RESULTS: The bell-clapper deformity is best defined by complete investment of the testis, epididymis and a length of the spermatic cord by the tunica vaginalis. Based on autopsy studies the rate of BCD in scrotal testis varied from 4.9% to 16%; with bilaterality in 66%-100%. In cases of acute IVT bilaterality was noted in 54%-100%. The most disparate results were in cases of testicular regression syndrome where contralateral BCD was noted in 0%-87% of cases. CONCLUSION: We suggest future studies employ the strict anatomical definition above. As there is evidence of age-dependent investment of the testes, it will be important to develop age-standardized measurements of intravaginal length of spermatic cord. This critical morphometric measurement will allow a better understanding of the risk of IVT. LEVEL OF EVIDENCE: Systematic review of diagnostic studies: lowest level of evidence of included manuscripts Level IV (case-control studies with a poor reference standard).

H-type congenital tracheoesophageal fistula: Insights from 70 years of The Royal Children's Hospital experience.

BACKGROUND: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. METHODS: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes. RESULTS: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. CONCLUSIONS: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. LEVEL OF EVIDENCE: IV.

Side predilection in congenital anomalies of the kidney, urinary and genital tracts.

BACKGROUND: There appear to be various patterns of sidedness with relation to the common urogenital malformations observed in pediatric urology. The objective of this statistical review was to synthesize this data and to assess if these patterns are significant. MATERIALS AND METHODS: Eighteen urogenital conditions were investigated and for each condition the five largest studies that noted laterality were included. The sidedness of each condition was then analysed for statistical significance. RESULTS: Three conditions had a statistically significant higher proportion on the right side: palpable undescended testis (63%, p = 0.0002), inguinal hernia (59%, p = 0.0001) and hydrocele (60%, p = 0.003). Three conditions were significantly more common on the left side: impalpable undescended testis (59%, p = 0.0008), renal agenesis (54%, p = 0.02) and vesico-ureteric junction obstruction (71%, p < 0.0001) while both pelvi-ureteric junction obstruction (62%, p = 0.09) and absent vas deferens (61%, p = 0.11) were trending towards significance. CONCLUSIONS: Various urogenital malformations display a predilection for one side. Proximal malformations tend to be more frequently seen on the left side, where as inguinoscrotal malformations are more frequently observed on the right. There is an increasing body of literature regarding aetiological factors for these conditions. However, our current understanding of the pathophysiology of these conditions does not completely explain this pattern of observation.

Contemporary management of Hirschsprung disease in New Zealand.

BACKGROUND: The aim of this study was to report the contemporary management of Hirschsprung disease (HD) in New Zealand. METHODS: We undertook a national multi-centre retrospective review of all newly diagnosed cases of HD during a 16-year period (2000-2015). Demographics, genetic and syndromic associations, family history, radiology and histology results and surgical interventions were analysed. RESULTS: A total of 246 cases (males:females 4:1) were identified, an incidence of 1:3870 live births. Short-segment disease was present in 81.7%, long-segment disease in 8.5%, total colonic aganglionosis in 6.5% and unknown in 3.3%. HD was diagnosed by 4 weeks' corrected gestational age in 67%. Thirty cases (12%) also had Trisomy 21. Fifty-three (21.5%) patients required a repeat rectal biopsy for definitive diagnosis. A contrast enema was performed in 55% and identified the transition zone with 69% accuracy. Primary pull-through surgery was undertaken in 59% (65% of short-segment cases) at a median age of 27 days; others were initially managed by a defunctioning stoma. The commonest definitive procedure was a Soave-Boley endorectal pull-through (79%) (or similar variant). During a median follow-up of 7.4 years, six (2.5%) survivors underwent a redo pull-through, 13 (5.5%) an appendicostomy, 16 (6.8%) a defunctioning stoma and 10 never had a definitive procedure. Total colonic aganglionosis was significantly more likely to be fatal (12.5% versus 0.5%, P < 0.0005) or associated with a permanent end stoma (27.5% versus 4.5%, P < 0.0005). CONCLUSIONS: Most New Zealand born infants with short-segment HD are currently managed by primary pull-through, usually in the first months of life.