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.

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.

Elevated plasma miR-210 expression is associated with atypical genitalia in patients with 46,XY differences in sex development.

Background: Differences of sex development (DSD) is a term used for conditions in which the chromosomal, gonadal or phenotypical sex is atypical. 46,XY DSD patients frequently present undervirilized external genitalia. The expression of different miRNAs in many organs of the male genital system has been reported, and these miRNAs have been associated with testicular function and its disorders, but no description has been related to DSD conditions.

Scrotal flap phalloplasty as temporary neophallus in infants and children with penile agenesis: Multi-institutional experience and long-term follow-up.

Introduction: Aphallia is a rare congenital disorder pertaining to genotypic males. Early surgical creation of a neophallus is recommended to reinforce the child's male gender-identity, favoring proper psychosexual development. Modern microsurgical techniques used to create a neophallus in adults are not recommended in children due to the invasiveness and complexity of the procedures, along with high complication rates.

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.

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.

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.

Management of 46,XY Differences/Disorders of Sex Development (DSD) Throughout Life.

Differences/disorders of sex development (DSD) are a heterogeneous group of congenital conditions that result in discordance between an individual's sex chromosomes, gonads, and/or anatomic sex. Advances in the clinical care of patients and families affected by 46,XY DSD have been achieved since publication of the original Consensus meeting in 2006. The aims of this paper are to review what is known about morbidity and mortality, diagnostic tools and timing, sex of rearing, endocrine and surgical treatment, fertility and sexual function, and quality of life in people with 46,XY DSD.

Single-stage Abdominoplasty Using Groin Flaps Without Osteotomies: Management of Exstrophy-epispadias Complex.

Background And Objective: The optimal treatment for children born with exstrophy-epispadia complex is still a matter of debate. We demonstrate the Single-Stage Abdominoplasty using Groin Flap technique to close the abdominal wall of children with classic bladder exstrophy (CBE) without osteotomy neither radical soft tissue mobilization. Advantages over current techniques are less risk of penile tissue loss and avoidance of osteotomies.

Diagnostic accuracy of Onen's Alternative Grading System combined with Doppler evaluation of ureteral jets as an alternative in the diagnosis of obstructive hydronephrosis in children.

Introduction: Ureteropelvic junction obstruction (UPJO) is a common congenital anomaly leading to varying degrees of hydronephrosis (HN), ranging from no apparent effect on the renal function to atrophy. Evaluation of these children is based on Diuretic Renal Scintigraphy (DRS) and Ultrasonography (US). Recent studies have suggested that new parameters of conventional and color Doppler ultrasonography (CDUS) may be useful in discriminating which kidneys are obstructed.

Videolaparoscopic radical nephrectomy after chemotherapy in the treatment of Wilms' tumor: Long-term results of a pioneer group.

Introduction: A high cure rate for Wilms' tumor has been achieved using a multidisciplinary approach. The natural step forward is to offer the benefits of a minimally invasive technique for surgery, which is an obligatory part of treatment. Nevertheless, some authors resist using videolaparoscopic radical nephrectomy (VRN) because of concerns about reducing the cure index.

Urethral duplication type influences on the complications rate and number of surgical procedures.

Introduction: Urethral duplication is rare. Characterized by the presence of two urethral channels. This anomaly presents a great variety of clinical findings that depend on the type of duplication that often is associated with other anomalies.

Surgical Treatment after Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia: Are Good Results Possible?

Purpose: Genital reconstruction in female patients with virilizing congenital adrenal hyperplasia (CAH) is very challenging. Our aim was to evaluate the techniques employed to treat complications after failure of primary urogenital sinus (UGS) surgery, as well as the result of these reoperations.

Patients And Methods: Twenty girls with virilizing CAH who were previously submitted to genitoplasty in our service and elsewhere had recurrent UGS stenosis and vaginal introitus stenosis that required surgical treatment.