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Purpose: To review surgical management, tumour stage and clinical outcomes in children with intravascular extension of Wilms tumour (WT) registered in a national clinical study (2012-19)., Methods: WTs with presence/suspicion of tumour thrombus in the renal vein (RV) or beyond on radiology, surgery or pathology case report forms were identified. Only cases where thrombus was confirmed by surgeon and/or reference pathologist were included. Surgical management, disease stage, overall (OS) and event free survival (EFS) were investigated., Results: 69/583 (11.8%) patients met the inclusion criteria. Forty-six (67%) had abdominal stage III due to thrombus-related reasons: 11 had macroscopically incomplete resection, including 8 cases where cavotomy was not performed; 20 had piecemeal complete resection of thrombus; 15 had microscopically positive resection margins at the RV. 66% of tumour thrombi contained viable tumour. There were eight relapses and five deaths. EFS, but not OS, was significantly associated with completeness of surgical resection (P<0.05). OS and EFS were also significantly associated with histological risk group (P<0.05) but not with viability of tumour thrombus (P=0.19; P=0.59)., Conclusions: WTs with intravascular extension have a high risk of local stage III due to thrombus-related reasons. Controlled complete removal of the thrombus should be the aim of surgery., Level of Evidence: Level II., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier Inc.)

Background: Cloacal exstrophy (CE) is the most severe end of the Exstrophy-Epispadias Complex malformations spectrum. Improvements in postnatal management and well-established operative techniques have resulted in survival rates approaching 100%. This systematic review aims to define the prevalence of long-term active medical problems affecting CE patients after the first decade of life., Methods: PubMed/Medline, Embase, Scopus, and ISI Web of Knowledge databases were used for the literature search. Original articles related to medical, surgical, and psychosocial long-term problems in CE patients >10 years of age were included in the study. Quality assessment of the articles was performed through the Newcastle-Ottawa Scale. Prevalence estimates and 95% CI were assessed for each outcome., Results: Twelve studies were included. The most common long-term active problems identified were: urinary incontinence with a prevalence ranging from 9.1% to 85%; sexual function issues related to vaginal anomalies with a prevalence ranging from 8.3% to 71.3%, and uterine anomalies, with a prevalence from 14.3% to 71%; gender identity issues in 46, XY patients raised female had a prevalence from 11.1% to 66.7%. There is no documented history of paternity. Impairment of ambulatory capacity was recorded in 13.8% of patients. Only one paper studied psychological well-being, reporting significantly higher levels of depression among gender reassigned patients., Conclusions: Teenagers and adults born with CE have well defined long-term problems compared to the general population. Recognition and expert management are crucial to improve care and quality of life during and after the transition into adulthood., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background/purpose: Adrenocortical tumors (ACTs) are rare in children and the extent to which histopathological features can predict clinical behavior remains uncertain. The aim of this study was to investigate the relationship between histopathological features (Weiss score), surgical approach, tumor size, underlying genetic predisposition syndrome, and outcome., Methods: Twenty-nine ACTs treated at our institution between 1987 and 2011 were identified from a histopathology database. The histological features were categorized using the Weiss scoring system. For tumor staging, the UKCCSG staging system was utilized., Results: At a median follow-up of 25 months, 19 patients (65.5%) survived without evidence of disease and 10 patients (35.5%) had died. There was a strong association between high Weiss score and both large tumor size (P<0.01) and adverse outcome (P<0.01). Outcome for stage I and IIA disease was significantly better compared to higher stage disease and/or tumor rupture (P<0.01)., Conclusion: There is an association between high Weiss score, large tumor size, underlying genetic predisposition syndrome and an adverse outcome for pediatric ACTs. Regardless of histopathological findings, complete surgical resection, without tumor spillage, is optimal for survival. Genetic evaluation is recommended in patients with ACTs, particularly those with a high Weiss score., (© 2014.)

This case relates to a child with an antenatal diagnosis of severe bilateral hydronephrosis with congenital anomalies of the ureters, bladder, and urethra. We describe the presentation and surgical management, highlighting the complexity of this anomaly and the surgical technique used to reconstruct the lower urinary tract. To our knowledge, there has not been a similar case in the literature and the use of a segment of colon for ureteric substitution in children has not been previously reported in the literature., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Bladder prolapse is extremely rare in children. Reports have been limited to pathologic bladder conditions such as the exstrophy-epispadias complex. We report an interesting presentation of bladder prolapse in a child with Williams syndrome with an apparently normal bladder.