Novel minimally invasive abdominoplasty for selected cases of prune belly syndrome: Step-by-step technique description and clinical indications.

Background: Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, bilateral undescended testicles and genitourinary tract anomalies. A variable spectrum of abdominal wall laxity is observed in PBS. We present the first case of a novel technique using a minimally invasive abdominoplasty to specifically address patients with localized abdominal wall weakness in PBS.

Case Study: Rosai-Dorfman Disease and Its Multifaceted Aspects.

Rosai-Dorfman Disease (RDD) is a rare non-Langerhans histiocytosis, usually self-limited and presenting with massive, painless, bilateral cervical lymphadenopathy, with or without constitutional symptoms. Extranodal disease is frequently present, and may happen in the absence of lymph node involvement, symptomatology and differential diagnosis will depend on the site affected and fatal cases may occur. The authors present two cases of Rosai-Dorfman disease (RDD), diagnosed through immunohistochemistry, with different progressions, one with complete remission and one culminating in death, highlighting the variety of presentations and the diagnostic difficulty.

Vesicoureteral reflux by contrast ultrasound, comparison with voiding and retrograde urethrocystography: A prospective accuracy study.

Introduction: Diagnosis of vesicoureteral reflux (VUR) plays an important role in the management and follow-up of children presenting with urinary tract infection. This study compared voiding uronosography (VUS) as a diagnostic method for the diagnosis of VUR with the "gold standard", voiding or micturating cystourethrography (MCU). The objective of the study is not only to compare the effectiveness of both methods, but also to assess the feasibility of implementing VUS technically and economically in a large tertiary radiology center in South America.

Retrospective Analysis of Prognostic Factors in Pediatric Patients with Adrenocortical Tumor from Unique Tertiary Center with Long-Term Follow-Up.

Pediatric adrenocortical tumors (PACTs) represent rare causes of malignancies. However, the south/southeast regions of Brazil are known to have a high incidence of PACTs because of the founder effect associated with a germline pathogenic variant of tumor suppressor gene TP53. We aimed to retrospectively analyze the types of variables among hormone production, radiological imaging, tumor staging, histological and genetic features that were associated with the occurrence of malignancy in 95 patients (71% females) with PACTs from a unique center.

Brazilian Society of Surgical Oncology consensus on fertility-sparing surgery for cervical cancer.

Objective: Several controversies remain on conservative management of cervical cancer. Our aim was to develop a consensus recommendation on important and novel topics of fertility-sparing treatment of cervical cancer.

Methods: The consensus was sponsored by the Brazilian Society of Surgical Oncology (BSSO) from March 2020 to September 2020 and included a multidisciplinary team of 55 specialists.

Bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure.

Background: We report an exceedingly rare case of bilateral external iliac artery pseudoaneurysms causing urinary obstruction and acute renal failure.

Case Presentation: A young man presented with acute severe bilateral testicular pain radiating to the back. Clinical and radiological workup showed bilateral external iliac artery pseudoaneurysms, which caused bilateral ureterohydronephrosis due to urinary obstruction with subsequent renal failure.

Evaluating TIMP-2 and IGFBP-7 as a predictive tool for kidney injury in ureteropelvic junction obstruction.

Unlabelled: A major challenge in the management of ureteropelvic junction obstruction (UPJO) is the selection of patients who would benefit from surgical treatment. Tissue inhibitor metalloproteinase-2 (TIMP-2) and insulin-like growth factor-binding protein 7 (IGFBP7) indicate renal cell stress and are associated with cell cycle arrest. The [TIMP-2] [IGFBP7] ratio (Nephrocheck®) has been recently applied in patients in intensive care units patients to predict the development of acute kidney injury.

Use of contrast-enhanced ultrasound in pediatrics.

Although contrast-enhanced ultrasound has been shown to provide considerable benefits, particularly in pediatric patients, it is still used relatively rarely in Brazil. It has proven to be a safe technique, and adverse effects are rare. In this review, we address the technique and main applications of contrast-enhanced ultrasound in the pediatric population, including the evaluation of focal liver lesions, abdominal trauma, kidney grafts, liver grafts, bowel loops, and vesicoureteral reflux.

Cost Effectiveness and Impact in Quality of Care of a Pediatric Multidisciplinary Stone Clinic.

Unlabelled: Herein, we assess the cost-effectiveness of a multidisciplinary clinic for children with urinary stones. The clinic's primary goals were to decrease unnecessary visits, imaging, and costs while optimizing the quality of care.

Methods: Between October 2012 and January 2016, children with complex stone disease, previously treated in urology and/or nephrology clinics, were seen at a triannual pediatric combined stone clinic.

Modern management of and update on prune belly syndrome.

Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling.

Urinary extracellular matrix proteins as predictors of the severity of ureteropelvic junction obstruction in children.

Introduction: Ureteropelvic junction obstruction (UPJO) particularly represents a challenge in regard to management, as not all hydronephrosis (HN) represent a kidney-damaging state. Urinary biomarkers have been proposed as noninvasive tools. Extracellular matrix (ECM) proteins are associated with tissue fibrosis in the setting of UPJO and is poorly explored.

Can extended upper pole ureterectomy prevent ureteral stump syndrome after proximal approach for duplex kidneys?

Introduction: Symptomatic duplex kidneys usually present with recurrent urinary tract infection due to ureteral obstruction (megaureter, ureterocele or ectopic ureter) and/or vesicoureteral reflux. Upper-pole nephrectomy is a widely accepted procedure to correct symptomatic duplex systems with poor functioning moieties, also known as upper or proximal approach. The distal ureteral stump syndrome (DUSS) can be a late complication of this approach.

Genetic and clinical aspects of paediatric pheochromocytomas and paragangliomas.

Objective: Few and conflicting reports have characterized the genetics of paediatric pheochromocytomas and paragangliomas (PPGLs). This study aimed to investigate the clinical and genetic features of Brazilian children with PPGL.

Patients And Methods: This study included 25 children (52% girls) with PPGL.

Bladder and Bowel Dysfunction Network: Improving the Management of Pediatric Bladder and Bowel Dysfunction.

Unlabelled: Lower urinary tract symptoms with constipation characterize bladder and bowel dysfunction (BBD). Due to high referral volumes to hospital pediatric urology clinics and time-consuming appointments, wait times are prolonged. Initial management consists of behavioral modification strategies that could be accomplished by community pediatricians.

Patients with encrusted ureteral stents can be treated by a single session combined endourological approach.

Purpose: To describe our experience in the management of retained encrusted ureteral stents using a single session combined endourological approach.

Materials And Methods: Patients with retained encrusted ureteral stents who had been submitted to a single session combined endourological approach from June 2010 to June 2018 were prospectively evaluated. Patients were divided according to the Forgotten-Encrusted-Calcified (FECal) classification.

Laparoscopic hidden incision endoscopic surgery (hides) nephrectomy VS. Traditional laparoscopic nephrectomy: Non-inferior surgical outcomes and better cosmetic results.

Introduction: The benefits of laparoscopic total nephrectomy in pediatric patients are well established. Traditional Ports placement (TPP) still follows Clayman's classic description: except for the umbilical scar, the other laparoscopic scars are exposed in the abdomen. Advances in robotic surgery permitted the development of HidES (hidden incision endoscopic surgery) technique, to obtain a better final cosmetic aspect with the scars located intraumbilically and in the hypogastric region, in an area easily hidden by underwear.

Management of functioning pediatric adrenal tumors.

Aim: The aim of this study is to present our experience in the management of hormonally active adrenal tumors in children.

Material And Methods: We did a retrospective chart review of all children with hormonally active adrenal tumors evaluated at the endocrinology clinic and operated at our institution between 1983 and 2019.

Results: There were 75 patients included in the study, 58 with adrenocortical tumors (ACTs) and 17 with pheochromocytomas (PCCs).

Prune perineum surgical correction - Treatment of a rare syndrome.

Introduction: Prune Perineum Syndrome (PPS) is rare, with only three cases described in the literature. Treatment requires abdominal and pelvic surgeries. Our goal is to provide a video with steps of the surgical corrections of this syndrome.

Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients.

Objective: To gain insight into the causes of infertility in Prune Belly Syndrome (PBS) by evaluating reproductive system anatomy and gonadal function in a cohort of postpubertal PBS patients.

Methods: We contacted all PBS patients 14 years old or older treated and followed at our institution. Age at orchiopexy, type of orchiopexy (with or without ligation of gonadal vessels), testicular volumes and positions were evaluated.

Impact of pulmonary artery reduction during arterial switch operation: 14 years follow-up.

Objectives: Pulmonary artery/aorta (PA/Ao) size discrepancy plays an important role in the development of neoaortic root growth and valve regurgitation. Since 2004, we started using PA reduction to manage severe great vessels root mismatch at the time of arterial switch operation. The purpose of this study is to evaluate the impact of this technique in the mid- and long-term follow-up.

Recent advances in pediatric bladder malignancies.

Urothelial pediatric neoplasms are relatively rare. Papillary urothelial neoplasms of low malignant potential (PUNLMPs) and rhabdomyosarcoma (RMS) are the most common bladder malignancies in the pediatric population. Clinical presentation encompasses macroscopic hematuria or lower urinary tract symptoms (or both) or is detected incidentally at imaging.