Patterns of spinal cord malformation in cloacal exstrophy.

Objective: The objective of this study was to assess the prevalence and spectrum of spinal dysraphism in a cohort of children with cloacal exstrophy (CEX) using MRI.

Methods: Children with CEX presenting between 1999 and 2019 with baseline spinal MRI were included. The images were reviewed in consensus to assess the type of dysraphism.

Antenatal biological models in the characterization and research of congenital lower urinary tract disorders.

Congenital lower urinary tract disorders are a family of diseases affecting both urinary storage and voiding as well as upstream kidney function. Current treatments include surgical reconstruction but many children still fail to achieve urethral continence or progress to chronic kidney disease. New therapies can only be achieved through undertaking research studies to enhance our understanding of congenital lower urinary tract disorders.

Contractile function of detrusor smooth muscle from children with posterior urethral valves - The role of fibrosis.

Introduction: Posterior urethral valves (PUV) is the most common cause of congenital bladder outflow obstruction with persistent lower urinary tract and renal morbidities. There is a spectrum of functional bladder disorders ranging from hypertonia to bladder underactivity, but the aetiology of these clinical conditions remains unclear.

Aims And Objectives: We tested the hypothesis that replacement of detrusor muscle with non-muscle cells and excessive deposition of connective tissue is an important factor in bladder dysfunction with PUV.

Outcomes of urinary tract abnormalities diagnosed by the routine third trimester scan.

Objective: The aim of this study is to determine the incidence of congenital abnormalities of the kidneys and urinary tract (CAKUT) detected for the first time in an unselected population undergoing a routine third trimester scan between 30-34 week's gestation.

Methods: This was a retrospective analysis of 8562 routine third trimester ultrasound scans during which the fetal anatomy was evaluated, and, any structural abnormalities detected, recorded onto a fetal database which was subsequently analysed for CAKUT. All postnatal records of antenatally diagnosed CAKUT were obtained and analysed for diagnosis and management.

The value of the routine third trimester ultrasound scan in antenatal care: Problems with guidance and outdated data in a highly technological field.

Ultrasound scanning is a cornerstone of modern antenatal care, enabling the detection of fetal abnormalities such as congenital disease, chromosomal defects and growth-disorders as well as maternal diseases such as pre-eclampsia. As technology and techniques evolve, care paradigms and clinical guidance must be re-evaluated with current literature so as to provide expectant parents with the highest quality of care. We present here the current state of the literature, the conflict between guidance and research endeavours and present suggestions for future study.

Meeting the challenges of reconstructive urology - Where are we now?

This is based on the 2018 Storz Urology Lecture at the BAPS Conference and is a personal review of three reconstructive paediatric urological conditions: hypospadias, congenital adrenal hyperplasia, and bladder exstrophy from the perspective of changing expectations and outcomes. LEVEL OF EVIDENCE: V (Expert Opinion).

Dual Pathology Causing Congenital Bladder Outlet Obstruction.

Anterior urethral syringocele is an uncommon congenital deformity characterized by cystic dilatation of bulbourethral gland ducts and is usually asymptomatic. We present a case on 4-day-old male neonate who presented with bilateral antenatal hydroureteronephrosis and renal impairment and found to have urethral syringocele and posterior urethral valves.

Isolated bilateral simplex ureteric ectopia: Bladder capacity as an indicator of continence outcome.

Introduction: Isolated bilateral simplex ectopic ureters (BSEUs) are rare but pose a therapeutic challenge: ureteric reimplantation alone does not accomplish continence in all. Identifying the patients needing additional procedures for continence early could prevent multiple operations.

Objective: Potential preoperative indicators for postoperative continence are explored in eight BSEU girls without cloacal, anorectal, or spinal anomalies.

Cloacal Exstrophy Repair with Primary Closure of Bladder Exstrophy: A Case Report and Review of Literature.

Cloacal exstrophy is the most complex congenital, ventral, abdominal wall defect. Traditionally surgery consists of a staged approach to repair which takes place on many separate theatre visits. In this case a primary approach was undertaken resulting in a relatively short inpatient stay and a reduced risk from multiple surgical procedures under general anaesthesia.

5 years after an ACE: what happens then?

Purpose: Antegrade continence enema (ACE) revolutionised the lives of children with chronic constipation and soiling. Parents often ask how long the ACE will be required. We looked at our patients 5 years after ACE formation to answer the question.

Urological management.

The pediatric urologists role is confined to ischio-, para-, and pygopagus conjoined twins. The aim is to preserve renal function, to address problems with urinary continence, and to optimize genital issues with particular reference to sexual and reproductive function. Full urological imaging is essential prior to separation for planning and for the operation itself.

A Case of Embryonal Rhabdomyosarcoma Presenting as a Lobulated Protrusion From the Urethral Meatus at Birth.

This is the first reported case of rhabdomyosarcoma presenting as a mass protruding from the urethral meatus present at birth. A male neonate was transferred to a tertiary pediatric urology center on day 4 of life where the mass was surgically excised. Histology confirmed an embryonal rhabdomyosarcoma and chemotherapy commenced.

What is the risk of urinary tract infection in children with antenatally presenting dilating vesico-ureteric reflux?

Introduction: The incidence of recurrent urinary tract infection (UTI) in children with primary vesico-ureteric reflux (VUR) presenting symptomatically is well documented. The risk of UTI in asymptomatic primary VUR diagnosed on investigation of antenatal hydronephrosis (ANH) is less clear. Paradoxically, several previous studies have suggested a lower risk (1-25%).

Spiral rotational flap for the creation of a new umbilicus in bladder exstrophy.

Objective: A range of surgical techniques have been described to create a new umbilicus in children with bladder exstrophy. We present a detailed video demonstrating our technique for the creation of a new umbilicus.

Method: The position of the planned umbilicus and flap were marked on the abdomen.

Functional properties and connective tissue content of pediatric human detrusor muscle.

The functional properties of human pediatric detrusor smooth muscle are poorly described, in contrast to those of adult tissue. Characterization is necessary for more informed management options of bladder dysfunction in children. We therefore compared the histological, contractile, intracellular Ca2+ concentration responses and biomechanical properties of detrusor biopsy samples from pediatric (3-48 mo) and adults (40-60 yr) patients who had functionally normal bladders and were undergoing open surgery.

Testicular outcome following laparoscopic second stage Fowler-Stephens orchidopexy.

Objective: To assess outcome after laparoscopic second-stage Fowler-Stephens orchidopexy (L2(nd)FSO).

Patients And Methods: Retrospective review of 94 children (aged 0.75-16 years, median 2.

Current practice in feminizing surgery for congenital adrenal hyperplasia; a specialist survey.

Aim: To present the outcome of an online survey of the current practice in feminizing surgery for congenital adrenal hyperplasia (CAH) among the specialists attending the IVth World Congress of the International Society of Hypospadias and Disorders of the Sex Development (ISHID), 2011.

Material And Methods: An online survey covered 13 individual questions regarding the management and surgical techniques for 46XX CAH patients. All delegates attending the conference were invited to complete this anonymous survey.

Current practice in paediatric hypospadias surgery; a specialist survey.

Aim: To undertake an online survey of current hypospadias surgery practice among those specialists attending the IVth World Congress of the International Society for Hypospadias and Disorders of Sex Development (ISHID), 2011.

Materials And Methods: An online survey covering 22 separate questions relating to proximal and distal hypospadias surgery was set up, and all delegates registered for the conference were invited to complete this questionnaire anonymously. The data was analysed by three of the authors.

Incidence of Deflux® calcification masquerading as distal ureteric calculi on ultrasound.

Objective: Dextranomer-hyaluronic acid (Deflux(®)), the most widely used compound in the endoscopic treatment of vesico-ureteric reflux (VUR) today, is believed to provoke only minimal inflammation. Reports of calcification of Deflux(®) are increasing. We ascertain the incidence of Deflux(®) calcification appearing as distal ureteric calculi on ultrasound.

Requirements for a multicentric multidisciplinary registry on patients with disorders of sex development.

Disorders of Sexual Development (DSDs) are a group of rare to very rare congenital anomalies of the genito-urinary tract of genetic and endocrine causes. Recently, an international database I-DSD was successfully implemented to register patients with DSD and to provide the basis for epidemiologic, genetic, and clinical research. This tool needs to be adjusted and supplemented with additional modules in order to better assess the anatomical basis of DSD as well as to monitor risk factors such as gonadal histology.

Utility of diffusion-weighted imaging in the presurgical diagnosis of an infected urachal cyst.

Urachal cysts are one of a spectrum of urachal abnormalities that occur following failure of regression of the allantois and presumptive bladder between 4 weeks and 6 weeks of gestation. Infection is the most common complication of this rare congenital anomaly. The nonspecific presentation may mimic other pathological processes, underlining their clinical and radiological significance.

The predictive value of a repeat micturating cystourethrogram for remnant leaflets after primary endoscopic ablation of posterior urethral valves.

Objective: We routinely perform a cystourethroscopy 3 months after initial ablation of posterior urethral valves. The aim of this study was to determine the predictive value of the urethral appearance on preoperative micturating cystourethrogram (MCUG) for further valve resection at check cystoscopy.

Patients And Methods: We retrospectively reviewed 31 consecutive boys (aged 4-18 months) who underwent check cystoscopy and repeat MCUG between 2006 and 2008.