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.

Normal sonographic renal length measurements in an Australian pediatric population.

BACKGROUND: Reference charts depicting normal growth are important for the sonographic assessment of the pediatric kidney. Limited charts are available for clinical use in an Australian population. OBJECTIVE: To retrospectively collate sonographic renal length measurements in a cohort of low-risk Australian children aged newborn to 16 years to produce a reference table and comparison with other published charts. MATERIALS AND METHODS: We identified consecutive pediatric patients who were at low risk for renal disease and had renal lengths measured. After exclusions, we included 941 renal lengths (male 490, female 451). We used linear regression to estimate the relationship of renal length with age, gender and side. We calculated percentile values of renal length according to age categories. RESULTS: No statistically significant differences in mean renal length were observed between males and females, or for left and right kidneys. We tabulated reference data and provide them in a reference chart (1-, 2.5-, 5-, 10-, 50-, 90-, 97.5- and 99-percentiles). CONCLUSION: We calculated new reference ranges for pediatric renal length using a larger cohort than previously published, from a population with diverse ethnicity.

Copy-number variation associated with congenital anomalies of the kidney and urinary tract.

BACKGROUND: The most common cause of end-stage renal disease in children can be attributed to congenital anomalies of the kidney and urinary tract (CAKUT). Despite this high incidence of disease, the genetic mutations responsible for the majority of CAKUT cases remain unknown. METHODS: To identify novel genomic regions associated with CAKUT, we screened 178 children presenting with the entire spectrum of structural anomalies associated with CAKUT for submicroscopic chromosomal imbalances (deletions or duplications) using single-nucleotide polymorphism (SNP) microarrays. RESULTS: Copy-number variation (CNV) was detected in 10.1 % (18/178) of the patients; in 6.2 % of the total cohort, novel duplications or deletions of unknown significance were identified, and the remaining 3.9 % harboured CNV of known pathogenicity. CNVs were inherited in 90 % (9/10) of the families tested. In this cohort, patients diagnosed with multicystic dysplastic kidney (30 %) and posterior urethral valves (24 %) had a higher incidence of CNV. CONCLUSIONS: The genes contained in the altered genomic regions represent novel candidates for CAKUT. This study has demonstrated that a significant proportion of patients with CAKUT harbour submicroscopic chromosomal imbalances, warranting screening in clinics for CNV.

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.

Latitudes and attitudes: A multinational study of laparoscopic pyeloplasty in children.

PURPOSE: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty. MATERIALS AND METHODS: A descriptive, retrospective study was conducted evaluating patients undergoing Anderson-Hynes dismembered laparoscopic pyeloplasty. Centers from four different continents participated. Demographic data, perioperative management, results, and complications are described. RESULTS: Over a 9-year period, 744 laparoscopic pyeloplasties were performed in 743 patients. Mean follow-up was 31 months (6-120m). Mean age at surgery was 82 months (1 w-19 y). Median operative time was 177 min. An internal stent was placed in 648 patients (87%). A catheter was placed for bladder drainage in 702 patients (94%). Conversion to open pyeloplasty was necessary in seven patients. Average length of hospital stay was 2.8 days. Mean time of analgesic requirement was 3.2 days. Complications, according to Clavien-Dindo classification, were observed in 56 patients (7.5%); 10 (1%) were Clavien-Dindo IIIb. Treatment failure occurred in 35 cases with 30 requiring redo pyeloplasty (4%) and 5 cases requiring nephrectomy (0.6%). CONCLUSION: We have described the laparoscopic pyeloplasty experience of institutions with diverse cultural and economic backgrounds. They had very similar outcomes, in agreement with previously published data. Based on these findings, we conclude that laparoscopic pyeloplasty is safe and successful in diverse geographics areas of the world.

Overview of a novel paediatric surgical simulation-based medical education programme in Myanmar.

BACKGROUND: Simulation-based medical education (SBME) is an integral part of undergraduate and postgraduate training in high-income countries (HICs). Despite potential benefits to low- and middle-income countries (LMICs), it has not been widely applied. Our aim was to use SBME to address some essential paediatric surgery learning needs in a LMIC. METHODS: Eleven SBME courses were designed, implemented and evaluated over a 4-year period in partnership with local paediatric surgeons and the University of Medicine 1 in Yangon, Myanmar. All courses were simulation-based and different major SBME modalities were utilized. Evaluation included pre- and post-course questionnaires, other evaluation assessments including Likert scale self-rated confidence in different domains, as well as, mixed method evaluation and Kirkpatrick's hierarchy of evaluation. RESULTS: Over 4 years, a multidisciplinary team consisting of surgical consultants, fellows, and educational specialists delivered 11 courses at a tertiary LMIC paediatric surgical centre. Attendance varied between 23 and 50 healthcare professionals, with some participants attending all of the educational activities. SBME modalities were utilized to meet each courses' learning objectives. All educational courses scored highly and showed statistically significant differences in all the self-rated pre and post-course confidence Likert scale domains. SBME was accepted and embraced by local participants and faculty, and transition to local delivery of educational content has begun. Level 4 of Kirkpatrick's hierarchy of evaluation was demonstrated. CONCLUSION: SBME can be used to meet essential learning objectives of local staff in a LMIC. Through various modalities, it offers a reliable, proven and affordable means of teaching multiple aspects of paediatric surgical clinical practice. By employing innovative simulation-based solutions, it can be adapted by local faculty to continue meeting ongoing learning needs.

Clinical Impact of the Introduction of Pediatric Intussusception Air Enema Reduction Technology in a Low- to Middle-Income Country Using Low-Cost Simulation-Based Medical Education.

INTRODUCTION: Pediatric intussusception is a common cause of bowel obstruction in infants. Air enema (AE) reduction is routine first-line management in many countries; however, there is a high rate of operative intervention in low- and middle-income countries. The aims of the study were to use simulation-based medical education with an intussusception simulator to introduce AE reduction to Myanmar and to assess its effect on provider behaviors and the resulting clinical care. METHODS: Clinical evaluation was conducted by comparing clinical outcomes data for children with intussusception 12 months before implementation with that from 12 months subsequent to implementation. These included the following: AE success rates, recurrence rates, length of stay, intestinal resection, and operative intervention rates. An educational workshop was developed that used a low-cost mannequin to facilitate practice at the reduction of intussusception using AE. Curriculum evaluation was performed through 5-point rating scale self-assessment in several domains. Data analysis was performed with Mann-Whitney U test, Student t test, or Wilcoxon signed-ranks test as appropriate; a P value of less than 0.05 was considered to be significant. RESULTS: After implementation, there was a significant reduction in the overall operative intervention rates [82.5% (85/103) vs. 58.7% (44/75), P = 0.006]. Intestinal resection rates increased [15.3% (13/85) vs. 35.9% (14/39), P = 0.02]. The success rate with attempted AE reduction was 94.4% (34/36), with a recurrence rate of 5.6% (2/36). The simulation-based medical education workshop was completed by 25 local participants. There was a significant difference in the confidence of performing (1.9 vs. 3.6, P ≤ 0.0001) or assisting (2.8 vs. 3.7, P = 0.018) an AE reduction before and after the workshop. CONCLUSIONS: Simulation-based educational techniques can be successfully applied in a low- and middle-income country to facilitate the safe introduction of new equipment and techniques with significant beneficial impact on provider behaviors and the resulting clinical care.

Establishing content validity and fidelity of a novel paediatric intussusception air enema reduction simulator.

BACKGROUND: Intussusception is a common, potentially life-threatening paediatric condition. Non-operative treatment with an air enema has been established as the clinical gold standard. There is no validated model for the training of this procedure. Our aim was to produce a novel air enema reduction simulator and validate its use as a training tool. METHODS: A low-cost paediatric intussusception air enema simulator was created. It was designed to include essential key clinical procedural steps. Participants included both procedural experts and novices from the Departments of Paediatric Radiology and Surgery. The simulator was assessed for face and content validity and its physical, conceptual and experiential fidelity by a structured questionnaire using a 5-point Likert's scale. Statistical analysis included a t-test, and a P-value of <0.05 was considered significant. RESULTS: Twenty-four clinicians completed the simulation activity (expert: 13 and novices: 11). All experts had performed a minimum of 40 clinical procedures, and 46% had performed >50 procedures. All scores were favourable in all domains for face and content validity: 3.5 (physical appearance), 3.3 (insertion of the tube and taping), 3.1 (holding of the buttocks) and 3.5 (performing the air enema). The simulator also scored highly with fidelity assessment; visual 3.5, conceptual 3.4. There was no difference in procedural confidence with experts (3.8 versus 3.6, P = 0.28), but there was for novices (1.0 versus 2.9, P = 0.0002). CONCLUSIONS: This low-cost air enema reduction simulator for intussusception has an excellent educational potential for use in a training program in a tertiary centre, as well as, resource-constrained environments.

Simulation in paediatric urology and surgery. Part 1: An overview of educational theory.

Surgical training has changed radically in the last few decades. The traditional Halstedian model of time-bound apprenticeship has been replaced with competency-based training. Advanced understanding of mastery learning principles has vastly altered educational methodology in surgical training, in terms of instructional design, delivery of educational content, assessment of learning, and programmatic evaluation. As part of this educational revolution, fundamentals of simulation-based education have been adopted into all levels and aspects of surgical training, requiring an understanding of concepts of fidelity and realism and the impact they have on learning. There are many educational principles and theories that can help clinical teachers understand the way that their trainees learn. In the acquisition of surgical expertise, concepts of mastery learning, deliberate practice, and experiential learning are particularly important. Furthermore, surgical teachers need to understand the principles of effective feedback, which is essential to all forms of skills learning. This article, the first of two papers, presents an overview of relevant learning theory for the busy paediatric surgeon and urologist. Seeking to introduce the concepts underpinning current changes in surgical education and training, providing practical tips to optimise teaching endeavours.

Disorders of sex development: insights from targeted gene sequencing of a large international patient cohort.

BACKGROUND: Disorders of sex development (DSD) are congenital conditions in which chromosomal, gonadal, or phenotypic sex is atypical. Clinical management of DSD is often difficult and currently only 13% of patients receive an accurate clinical genetic diagnosis. To address this we have developed a massively parallel sequencing targeted DSD gene panel which allows us to sequence all 64 known diagnostic DSD genes and candidate genes simultaneously. RESULTS: We analyzed DNA from the largest reported international cohort of patients with DSD (278 patients with 46,XY DSD and 48 with 46,XX DSD). Our targeted gene panel compares favorably with other sequencing platforms. We found a total of 28 diagnostic genes that are implicated in DSD, highlighting the genetic spectrum of this disorder. Sequencing revealed 93 previously unreported DSD gene variants. Overall, we identified a likely genetic diagnosis in 43% of patients with 46,XY DSD. In patients with 46,XY disorders of androgen synthesis and action the genetic diagnosis rate reached 60%. Surprisingly, little difference in diagnostic rate was observed between singletons and trios. In many cases our findings are informative as to the likely cause of the DSD, which will facilitate clinical management. CONCLUSIONS: Our massively parallel sequencing targeted DSD gene panel represents an economical means of improving the genetic diagnostic capability for patients affected by DSD. Implementation of this panel in a large cohort of patients has expanded our understanding of the underlying genetic etiology of DSD. The inclusion of research candidate genes also provides an invaluable resource for future identification of novel genes.

Transitional cell carcinoma in the paediatric population: be aware of unusual aetiologies.

This is a case report of a 12 year old girl who presented with a rare condition in paediatrics, transitional cell carcinoma of the bladder. It is important because it is readily treated by endoscopic means if diagnosed early. Potential aetiologies for this unusual condition are explored.