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.

Lack of consensus in atypical congenital obstructive urethral lesions in children: Snapshot of the web-based ObsCUre (obstruction to the child urethra) study.

Background: Atypical Congenital Obstructive Urethral Lesions (ACOUL) are uncommon causes of urethral obstruction in children. They include Cobb's collar or Moorman's ring, Type III posterior urethral valve (PUV), congenital urethral narrowing and anterior urethral valves. This study is aimed to evaluate the knowledge and current practice amongst clinicians attending to ACOUL. An international online case based questionnaire was performed. Materials and methods: A survey was administered to members of international urological societies. It included 22 clinical questions on cases with ACOUL (14 questions suitable for statistical analysis) using cases of Type I PUV as controls. Two sets of paired questions evaluated change in opinion(s) after additional information was provided. Results: One hundred twenty-one participants responded with 71% reporting exposure of less than 5 cases per annum. In questions regarding diagnosis between 11.6% (14/121) and 21.5% (26/121) of participants identified the ACOUL as PUV. Among them, 66% of respondents agreed on ACOUL's causative role in urethral obstruction. Gini coefficient was consistently lower for ACOUL compared to PUV: diagnosis (mean 0.33 vs. 0.44) and prognosis (0.23 vs. 0.43). High intra-rater concordance (kappa 0.420.57) was observed for paired questions-a mean of 5.79% (7.44% and 4.13% for questions 10 and 12, 16 and 17, respectively) of participants changed their answers from an alternate diagnosis to the correct diagnosis of ACOUL after viewing endoscopic images. High variation in management of ACOUL was noted (Gini 0.51). Conclusions: This global snapshot survey identified substantial inconsistency among clinicians dealing with ACOUL. Although rarely encountered in clinical practice, better overall education of ACOUL is warranted.

Reduction of Urinary Tract Infection in Pediatric Surgical Patients Using NSQIP-P and Quality Improvement Methodology.

BACKGROUND: Hospital acquired urinary tract infections (UTIs) have a detrimental effect on patients, families and hospital resources. The Sydney Children's Hospital Network (SCHN) participates in the National Surgical Quality Improvement Program - Pediatric (NSQIP-P) to monitor post operative complications. NSQIP-P data revealed that the median UTI rate at SCHN was 1.75% in 2019, 3.5 times higher than the NSQIP-P target rate of 0.5%. Over three quarters of the NSQIP-P identified UTI cases also had a urinary catheterization performed intra-operatively. A quality improvement project was conducted between mid-2018 to 2021 to minimise catheter associated UTIs (CAUTIs) at SCHN. STUDY DESIGN: NSQIP-P samples pediatric (<18 years) surgical cases from an 8-day cycle operative log. NSQIP-P data is statistically analysed by the American College of Surgeons and provides biannual internationally benchmarked reports. The project utilized Clinical Redesign Methodology with a six-phase process for quality improvement projects. The project utilized Clinical Redesign Methodology with a six-phase process for quality improvement projects. RESULTS: The objectives of the project were to reduce urinary catheter duration of use, educate parents/carers and improve catheter care and insertion technique by health staff. The duration of a urinary catheter in situ reduced from a median of 4.5 days to 3 days from 2017 to 2021. The median NSQIP-P UTI rate at SCHN was reduced by 47.4% from 1.75% in 2019 to 0.9% in 2022. CONCLUSION: A multifactorial approach in quality improvement has been shown to be an effective strategy to reduce UTI rates at SCHN and patient outcomes were improved within a three-year time frame. Whilst this project has reduced UTI rates at SCHN, there remain opportunities for further improvement.

Laparoscopic versus open pyeloplasty in paediatric pelvi-ureteric junction obstruction.

AIM: Compared to open pyeloplasty (OP), we hypothesised that laparoscopic pyeloplasty (LP) is associated with early recovery, a shorter length of stay (LOS) and less analgesia requirement. METHODS: Between 2011 and 2016, 146 dismembered pyeloplasty cases were reviewed, of which 113 were in the OP group and 33 were in the LP group. We evaluated both groups regarding operative time, LOS, success rate, complications rate and analgesia requirement. Subgroup analysis was done for patients above the age of 5 years, and within the OP group (dorsal lumbotomy (DL) vs. loin incision (LI)). RESULTS: The success rate was 96% in the open group and 97% in the laparoscopic group. The median operative time was significantly shorter in the open group for the entire cohort (127 vs. 200 min; P < 0.05), and in children older than 5 years (n = 41, 134 vs. 225 min; P < 0.05). Other parameters were similar in both groups. The median LOS was significantly shorter (2 vs. 4 days; P < 0.05), and the median analgesia requirement was less (0.44 vs. 0.64 mg/kg morphine; P < 0.05) in the DL (n = 60) compared to LI (n = 53). CONCLUSION: Both OP and LP dismembered approaches are equally effective in treating pelvi-ureteric junction obstruction. Overall, the LOS, complications rate and analgesia requirement were not significantly different; however, the operative time was significantly longer in LP.

Optimising the management of children with concomitant bladder dysfunction and behavioural disorders.

Bladder dysfunction and behavioural disorders in children are commonly concomitant; hence, it is difficult to treat each in isolation. Pharmacotherapy is common treatment for behavioural disorders, and these medications may have intended or unintended positive or negative bladder sequelae. This review identifies the literature regarding the effects of behavioural pharmacotherapy on bladder functioning and possible bladder management strategies in children with concomitant behaviour and bladder disorders to enable clinicians to better manage both conditions. A PROSPERO registered PRISMA-guided review of three major databases was performed. After an initial scoping study revealed significant heterogeneity, a narrative approach was undertaken to discuss the results of all relevant cases relating to children being treated with pharmacotherapy for behaviour disorders and outcomes related to bladder function. Studies were screened to identify those that described effects of commonly prescribed medications in children with behavioural disorders such as stimulants, alpha 2 agonists, tricyclic antidepressants (TCA), serotonin and noradrenergic reuptake inhibitors (SNRI), selective serotonin reuptake inhibitors (SSRI) and antipsychotics, and the findings and implications were summarised. The review identified 46 studies relevant to behavioural pharmacotherapy and bladder function (stimulants (n = 9), alpha 2 agonists (n = 2), TCAs (n = 7), SNRIs (n = 8), SSRIs (n = 8) and antipsychotics (n = 6). Six studies focused specifically on bladder management in children with behavioural disorders with concurrent behavioural pharmacotherapy. This review identifies useful factors that may assist clinicians with predicting unintended bladder effects following initiation of behavioural pharmacotherapy to facilitate the best approach to the treatment of bladder dysfunction in children with behavioural disorders. With this evidence, we have provided a useful decision-making algorithm to aide clinicians in the management of these dual pathologies.

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.

Peritesticular scarring: An unusual presentation of hemophilia B.

Hemophilia is an inherited clotting disorder that typically presents as spontaneous hemorrhage or prolonged, excessive bleeding following minor trauma. Abnormalities of the genitourinary tract are a rare manifestation of the disease. Here, we report a case of undiagnosed hemophilia B in a teenage boy presenting with worsening testicular pain and a dumbbell-shaped testis. Scrotal ultrasound identified the presence of a hypoechogenic band constricting the left testicle. Surgical exploration of the left testis was performed with release and sampling of the fibrous tunica vaginalis tissue responsible for the testicular deformity. Histopathological analysis revealed evidence of chronic interstitial inflammation with hypocellular keloid-like fiber formation suggestive of old hemorrhage. The procedure was complicated by a delayed scrotal hematoma. Coagulation studies confirmed a prolonged activated partial thromboplastin time and factor IX assay of 5%. Scrotal evacuation with the administration of intraoperative tranexamic acid achieved effective hemostasis. The patient is doing well with ongoing hematology review and prophylactic recombinant factor IX administration.

Effectiveness of standard urotherapy (basic bladder advice) and combination therapies in managing bladder dysfunction in children with treated behavioral disorders: Results of a prospective cohort (DABBED) study.

AIMS: We evaluated the efficacy of standard urotherapy and combination therapies in treatment of bladder dysfunction in children with treated behavioral disorders. METHODS: Prospective study of children (6-16 years) with bladder dysfunction and behavioral disorders was conducted between March 2018-2020. Eligible children were initially offered standard urotherapy and those with no response at 3 months were offered combination therapies. Symptomatic response, changes in Akbal score and PinQ score were reported at 6 months and outcomes were correlated to behavioral diagnoses and medications. RESULTS: Thirty-nine consecutive children (male = 27, mean age [SD] 10.3 [±2.0] years) were recruited, of whom 29 completed the study (five lost to follow-up, three non-compliant to treatment, two excluded). Thirty-four (87%) children had attention deficit/hyperactivity disorder. Monosymptomatic nocturnal enuresis (n = 11) and non-monosymptomatic enuresis (n = 17) were the commonest diagnoses. Following 3-month review, 14 (38%) children continued to receive standard urotherapy, while 15 (41%) children were transitioned to combination therapy. At 6-month review, complete/partial response was seen in 62% (23/37) and no response in 16% (6/37); with 32% (12/37) responding to standard urotherapy alone. Akbal symptom scores (15.9-11.5; P < 0.01) and PinQ scores (26.0-19.5; P = 0.008) improved significantly at 6-month follow-up. Type of underlying behavioral disorder(s) or medications for behavioral disorder did not influence the outcomes. CONCLUSION: This study confirms that children with underlying behavioral disorders are able to have a good response to the appropriate therapy for their bladder dysfunction with a third of children responding to standard urotherapy alone.

The microbiome and host mucosal interactions in urinary tract diseases.

The urinary tract consists of the bladder, ureters, and kidneys, and is an essential organ system for filtration and excretion of waste products and maintaining systemic homeostasis. In this capacity, the urinary tract is impacted by its interactions with other mucosal sites, including the genitourinary and gastrointestinal systems. Each of these sites harbors diverse ecosystems of microbes termed the microbiota, that regulates complex interactions with the local and systemic immune system. It remains unclear whether changes in the microbiota and associated metabolites may be a consequence or a driver of urinary tract diseases. Here, we review the current literature, investigating the impact of the microbiota on the urinary tract in homeostasis and disease including urinary stones, acute kidney injury, chronic kidney disease, and urinary tract infection. We propose new avenues for exploration of the urinary microbiome using emerging technology and discuss the potential of microbiome-based medicine for urinary tract conditions.

Parental decision regret in childhood hypospadias surgery: A systematic review.

We conducted a systematic review of the literature to establish the prevalence of and predictive factors for parental decision regret in hypospadias surgery. A search strategy without language restrictions was developed with expert help, and two reviewers undertook independent study selection. Five studies were included in this review (four for quantitative analysis) with a total of 783 participants. The mean overall prevalence of parental decision regret was 65.2% (moderate to severe - 20.3%). Although significant predictors of regret were identified (post-operative complications, small size glans, meatal location, decision conflict between parents, parental educational level and others), they had unexplained discordance between studies. Parental decision regret after proximal hypospadias surgery and refusing surgery was inadequately reported. In conclusion, even though the prevalence of parental decision regret after consenting for the hypospadias repair appears to be high, risk factors associated with it were discordant suggesting imprecision in estimates due to unknown confounders.

Group 2 Innate Lymphoid Cells Are Redundant in Experimental Renal Ischemia-Reperfusion Injury.

Acute kidney injury (AKI) can be fatal and is a well-defined risk factor for the development of chronic kidney disease. Group 2 innate lymphoid cells (ILC2s) are innate producers of type-2 cytokines and are critical regulators of homeostasis in peripheral organs. However, our knowledge of their function in the kidney is relatively limited. Recent evidence suggests that increasing ILC2 numbers by systemic administration of recombinant interleukin (IL)-25 or IL-33 protects against renal injury. Whilst ILC2s can be induced to protect against ischemic- or chemical-induced AKI, the impact of ILC2 deficiency or depletion on the severity of renal injury is unknown. Firstly, the phenotype and location of ILC2s in the kidney was assessed under homeostatic conditions. Kidney ILC2s constitutively expressed high levels of IL-5 and were located in close proximity to the renal vasculature. To test the functional role of ILC2s in the kidney, an experimental model of renal ischemia-reperfusion injury (IRI) was used and the severity of injury was assessed in wild-type, ILC2-reduced, ILC2-deficient, and ILC2-depleted mice. Surprisingly, there were no differences in histopathology, collagen deposition or mRNA expression of injury-associated (Lcn2), inflammatory (Cxcl1, Cxcl2, and Tnf) or extracellular matrix (Col1a1, Fn1) factors following IRI in the absence of ILC2s. These data suggest the absence of ILC2s does not alter the severity of renal injury, suggesting possible redundancy. Therefore, other mechanisms of type 2-mediated immune cell activation likely compensate in the absence of ILC2s. Hence, a loss of ILC2s is unlikely to increase susceptibility to, or severity of AKI.

Fatal Exsanguination Following Rupture of an Iliac Artery Aneurysm in an Infant With Menkes Disease.

Menkes disease (MD) usually presents in infancy with respiratory and neurological complications. Severe isolated vasculo-connective tissue involvement in infancy is rare, and hence the precise and timely diagnosis is difficult. We report a case of an 8-week-old male infant who succumbed to acute, severe exsanguination, and hemorrhagic shock secondary to a large retroperitoneal hematoma due to rupture of a right iliac artery aneurysm. Perimortem musculoskeletal findings raised suspicion of nonaccidental injury. However, postmortem review of facial traits raised the suspicion of MD. MD was subsequently confirmed on genetic testing. Child health clinicians must remain aware of MD as a rare cause of infant vasculopathy or atypical skeletal abnormalities.

Emerging therapeutic potential of group 2 innate lymphoid cells in acute kidney injury.

Acute kidney injury (AKI) remains a global challenge and, despite the availability of dialysis and transplantation, can be fatal. Those that survive an AKI are at increased risk of developing chronic kidney disease and end stage renal failure. Understanding the fundamental mechanisms underpinning the pathophysiology of AKI is critical for developing novel strategies for diagnosis and treatment. A growing body of evidence indicates that amplifying type 2 immunity may have therapeutic potential in kidney injury and disease. Of particular interest are the recently described subset of innate immune cells, termed group 2 innate lymphoid cells (ILCs). Group 2 ILCs are crucial tissue-resident immune cells that maintain homeostasis and regulate tissue repair at multiple organ sites, including the kidney. They are critical mediators of type 2 immune responses following infection and injury. The existing literature suggests that activation of group 2 ILCs and production of a local type 2 immune milieu is protective against renal injury and associated pathology. In this review, we describe the emerging role for group 2 ILCs in renal homeostasis and repair. We provide an in-depth discussion of the most recent literature that use preclinical models of AKI and assess the therapeutic effect of modulating group 2 ILC function. We debate the potential for targeting these cells as novel cellular therapies in AKI and discuss the implications for future studies and translation. Copyright © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Traumatic Urethraggia in Adolescence: Ushering in the New Age of "Extreme Sports".

Herein, we describe a case of a 14-year-old boy with straddle injury to the base of the penis, sustained during an indoor rock climbing accident, who presented with severe urethraggia. Urethral injury was confirmed with retrograde urethrography. The patient became hemodynamically unstable from persistent blood loss, and corporal arterial injury was diagnosed on computed tomography angiography. The urethraggia was successfully controlled with angioembolization. The unique aspects of this case were the challenges in establishing the diagnosis on the background of the abnormal presentation of vascular injury of blood loss as opposed to painless priapism.

Current strategies to predict and manage sequelae of posterior urethral valves in children.

Posterior urethral valves (PUV) constitute a significant urological cause of chronic kidney disease (CKD) in children. The condition is characterised by the unique pathophysiology of the evolution of bladder dysfunction after relief of obstruction, which contributes to CKD. Improvements in prenatal diagnosis followed by selective foetal intervention have not yet produced improvement in long-term renal outcomes, although better patient selection may alter this in the future. Proactive management with surveillance, pharmacotherapy, timed voiding, double voiding, and/or assisted bladder-emptying, is being increasingly offered to those with severe bladder dysfunction and has the potential of reducing the burden of renal disease. Clinicians are currently able to counsel regarding the prognosis using serum creatinine and other emerging markers. However, much of this work remains to be validated. Satisfactory graft survival rates are now reported with aggressive management of bladder dysfunction in children who are candidates for renal transplantation. Knowledge gaps exist in identifying early markers of renal injury, risk stratification, and in understanding patient and carer perspectives in PUV.

Laparoscopic repair of intra-abdominal bladder perforation in preschool children.

Intraperitoneal bladder rupture is uncommon in very young children, but its incidence may increase with increasing use of seat and lap belts. To the best of our knowledge, there are no prior reports of laparoscopic repair of this injury in children. We describe two recent cases and discuss useful technical points that facilitate a successful laparoscopic repair. Both our patients were preschool age girls who sustained seat and lap belt injuries. Contrast computed tomography scan suggested a large amount of free peritoneal fluid and cystogram confirmed intraperitoneal bladder perforation (isolated injury). The injury was repaired using delayed absorbable sutures and intracorporeal suturing (continuous in 1, interrupted in 1) using a 3 port laparoscopic technique. Meticulous peritoneal lavage was carried out to minimise urinary peritonitis and the bladder as well as the peritoneal cavity were drained. Check cystograms (day 7) revealed no leaks. Young girls appear to be at risk of intraperitoneal bladder injuries following lap belt injuries. After exclusion of life-threatening injuries and concurrent abdominal injuries which need rapid control or preclude pneumoperitoneum, a laparoscopic repair can be safely performed.

Adjunct ultrasonography in children with suspected acute appendicitis: identifying the optimal target group.

BACKGROUND: Role of ultrasonography in diagnosis of acute appendicitis in children is being debated. This study was conducted ahead of a clinical trial and aimed to evaluate the adjunctive role of ultrasonography in this group. METHODS: Retrospective chart review of consecutive children undergoing appendicectomy in an Australian tertiary children's hospital over 24 months. Two study groups were: clinical assessment and clinical assessment plus ultrasonography. The accuracy of diagnosis was compared by generating estimates for sensitivity and positive predictive value using histopathology of the appendix as the reference standard. The incremental value of ultrasonography was evaluated in subgroups of gender, age and clinical severity. RESULTS: Two hundred ninety-five children (170 boys, median age 10 years) were included in the study. Negative appendicectomy rate was 17.3%; 10.4% in the clinical assessment group (n = 134) and 23% in the ultrasonography group (n = 161). Overall sensitivity for diagnosis by clinical assessment (71.4%, 95% confidence interval (CI) 63.3-79.5) was not significantly different to that using adjunct ultrasonography (77.7%, 95% CI 69.7-85.7; P = 0.29). Adjunct ultrasonography was more sensitive between 7 and 11 years (81% versus 68%, P = 0.002) and possibly in girls (86% versus 73%, P = 0.1), but did not increase the sensitivity of the diagnosis in children who presented with milder symptoms and signs. CONCLUSIONS: Adjunct ultrasonography may not increase the overall accuracy of diagnosis of acute appendicitis in children but holds promise in certain subgroups viz. between 7 and 11 years and in girls. There is an increase in negative appendicectomies following a 'non-targeted' use of ultrasonography. Future prospective trials should take cognizance of these findings in designing the subgroup analysis.