Bladder bowel dysfunction scoring system (BBDSS): A novel, illustrated questionnaire for evaluation of voiding dysfunction in children.

Introduction: We sought to evaluate the reliability and validity of a new, illustrated questionnaire, the bladder bowel dysfunction symptom score (BBDSS) in the assessment of overactive bladder (OAB) and bladder bowel dysfunction (BBD).

Methods: The BBDSS questionnaire consisted of 12 structured questions. This pilot study was designed with two principal groups of questions: one group to assess bladder symptoms and the other to assess bowel dysfunction during the preceding month.

Voiding efficiency in children is significantly impaired by the presence of the urethral catheter during pressure-flow studies.

Background: Little is known about the effect of catheterization during pressure-flow studies (PFS) on voiding efficiency in children. Our objective was to determine the effect of urethral catheterization on Qmax and flow index (FI) during PFS compared to the free flow of uroflowmetry (UF).

Methods: We retrospectively reviewed 63 consecutive children who underwent UF and PFS at our center on the same day (2019-2022).

How can we measure the renal pelvic anteroposterior diameter in postnatal isolated hydronephrosis?

Introduction: The anteroposterior diameter (APD) of the renal pelvis is a commonly used ultrasound parameter in the evaluation and management of hydronephrosis. It has been established that an APD value associated with pyeloplasty is around 25 mm. Some believe the APD should be measured at the innermost part of the renal pelvis while others suggest that it should be done at the renal contour.

Validation of modified diuretic drainage times criteria in congenital hydronephrosis.

Introduction And Objective: The value of diuretic renography drainage times in congenital hydronephrosis (AHN) decision making is controversial. Recently, a group suggested a modification to the classically described diuretic drainage time cut-off values. They found that a drainage half-time (T) < 5 min was normal whereas a T exceeding 75 min predicted pyeloplasty.

Von Hippel-Lindau disease and rapidly progressing pheochromocytomas in siblings.

Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited condition with a predisposition to the development of a variety of tumors including pheochromocytomas. A number of cancer surveillance protocols for patients with VHL have been developed, all of which are based on expert opinion. We report a case of two brothers with a strong family history of VHL type 2 due to a pathogenic germline VHL variant, specifically, a surface missense substitution, with a rapidly progressive clinical course that both presented with a large adrenal mass.

Total glans amputation after neonatal circumcision.

Complete penile glans amputation is exceedingly rare in children with little known about the outcomes of management. We present a case of a 12-day-old male who underwent ritual circumcision complicated by total amputation of the glans, which was treated with reimplantation of the glans. Our technique was successfully carried out following reconstruction of anatomical planes with loop magnification and fine 7-0 sutures.

Children with vesicoureteric reflux have joint hypermobility and occasional tenascin XB sequence variants.

Introduction: To consider alternative mechanisms that give rise to a refluxing ureterovesical junction (UVJ), we hypothesized that children with a common heritable urinary tract defect, vesicoureteric reflux (VUR), may have a defect in the extracellular matrix composition of the UVJ and other tissues that would be revealed by assessment of the peripheral joints. Hypermobile joints can arise from defects in the extracellular matrix within the joint capsule that affect proteins, including tenascin XB (TNXB).

Methods: We performed an observational study of children with familial and non-familial VUR to determine the prevalence of joint hypermobility, renal scarring, and DNA sequence variants in TNXB.

Does the presence of non-refluxing hydroureter impact the management and outcome of high-grade hydronephrosis?

Introduction: We aimed to evaluate the impact of non-refluxing hydroureter on the initial management of high-grade hydronephrosis (HGH) management. Moreover, we evaluated the occurrence of febrile urinary tract infection (fUTI) and surgical intervention for conservatively managed units.

Methods: Patients' charts with postnatal hydronephrosis from 2008-2014 were retrospectively reviewed.

Influence of postnatal hydroureter in determining the need for voiding cystourethrogram in children with high-grade hydronephrosis.

Objective: To evaluate the utility of hydroureter (HU) to identify high-grade vesico-ureteric reflux (VUR) in patients with high-grade postnatal hydronephrosis (PH).

Patients And Methods: We retrospectively reviewed patients' charts that had antenatal hydronephrosis from 2008 to 2014. Patients were excluded if they presented with febrile urinary tract infection (fUTI), neurogenic bladder, posterior urethral valve, multi-cystic dysplastic kidney, and multiple congenital malformations.

Is the renal pyramidal thickness a good predictor for pyeloplasty in postnatal hydronephrosis?

Objectives: We evaluated the feasibility and value of renal pyramidal thickness (PT) as a predictor of pyeloplasty in high-grade postnatal hydronephrosis.

Patients And Methods: We retrospectively reviewed the charts of patients who presented with postnatal hydronephrosis from 2008 to 2013. Included cases had grade 3 or 4 hydronephrosis.

Heterozygous loss-of-function mutation in Odd-skipped related 1 () is associated with vesicoureteric reflux, duplex systems, and hydronephrosis.

Odd-skipped related 1 (Osr1) is a transcriptional repressor that plays critical roles in maintaining the mesenchymal stem cell population within the developing kidney. Here, we report that newborn pups with a heterozygous null mutation in exhibit a 21% incidence of vesicoureteric reflux and have hydronephrosis and urinary tract duplications. Newborn pups have a short intravesical ureter, resulting in a less competent ureterovesical junction which arises from a delay in urinary tract development.

A case of epidermoid median raphe cyst traversing the corpora cavernosa.

Median raphe cysts are congenital lesions that typically have a superficial appearance. We present a very unusual case of a deep perineal mass in a six-year-old boy. The lesion extends into the corpus cavernosum, suggesting that the anomaly was an early embryological event.

Antibiotic prophylaxis for prevention of urinary tract infections in prenatal hydronephrosis: An updated systematic review.

Introduction: While continuous antibiotic prophylaxis (CAP) is currently recommended to prevent urinary tract infections (UTIs) in infants with prenatal hydronephrosis (HN), this recommendation is not evidence-based. The objective of this study was to systematically determine whether CAP reduces UTIs in the HN population.

Methods: Applicable trials were identified through an electronic search of MEDLINE (1946-2015), EMBASE (1980-2016), CINAHL (1982-2016), and CENTRAL (1993-2016) and through a hand search of American Urological Association (AUA) (2012-2015) and European Society for Pediatric Urology (ESPU) (2012-2015) abstracts, as well as reference lists of included trials.

Laparoscopic pyeloplasty: Initial experience with 3D vision laparoscopy and articulating shears.

Introduction: Laparoscopic reconstructive surgery is associated with a steep learning curve related to the use of two-dimensional (2D) vision and rigid instruments. With the advent of robotic surgery, three-dimensional (3D) vision, and articulated instruments, this learning curve has been facilitated. We present a hybrid alternative to robotic surgery, using laparoscopy with 3D vision and articulated shears.

Long-term functional outcomes after bladder exstrophy repair: A single, low-volume centre experience.

Introduction: In this study, we present our experience managing bladder exstrophy (BE) in a low-volume centre over 24 years.

Methods: Charts of patients with BE between 1990 and 2014 were retrospectively reviewed. Patients with BE closure and ≥5 years followup were included.

Comprehensive analysis of the clinical and urodynamic outcomes of primary tethered spinal cord before and after spinal cord untethering.

Introduction: Primary tethered spinal cord (TSC) refers to a group of abnormalities associated with a posterior bony spinal defect that develops beneath an intact dermis and epidermis. There is relative agreement that patients with symptomatic TSC will require surgical intervention. However, it is still debatable as to how to approach asymptomatic patients with primary TSC.

Comprehensive analysis of the clinical and urodynamic outcomes of secondary tethered spinal cord before and after spinal cord untethering.

Objective: Secondary tethering of the spinal cord (TSC) occurs in 3-30% of patients with history of spinal cord dysraphism repair. As many patients with spinal cord dysraphism already have symptoms, it might be difficult to diagnose secondary TSC. Regular clinical/urodynamic (UDS) evaluation is therefore advised to pick-up changes early before they progress.

Testicular hypertrophy as a predictor for contralateral monorchism: Retrospective review of prospectively recorded data.

Objectives: Testicular hypertrophy has previously been evaluated as a predictor of monorchism. However, its implication in clinical practice is not well evaluated. The aim of the present study was to examine its value in planning the operative time.

First case of invasive squamous cell carcinoma in a stoma of a Monti ileovesicostomy.

We report a very rare case of invasive squamous cell carcinoma (SCC) in the abdominal stoma of a Monti ileovesicostomy. Our patient underwent an uncomplicated Monti ileovesicostomy at age 16 for a neurogenic bladder. She presented 10 years later with difficulty catheterizing the stoma.

Outcome of surgical management of non-palpable testes.

Background: We reviewed the success rates of orchidopexies performed for non-palpable testes at our institution and correlated preoperative and intraoperative findings to eventual outcomes.

Materials And Methods: We retrospectively reviewed ninty five medical records of patients who underwent laparoscopic exploration for the assessment of a non-palpable testes between 1996 and 2009. Intra-operative data for one hundrad eight non-palpable testes were collected.

A single-center cohort of Canadian children with VUR reveals renal phenotypes important for genetic studies.

Background: Many genes and loci have been reported in genetic studies of primary vesicoureteral reflux (VUR), but few have been reproduced in independent cohorts, perhaps because of phenotype heterogeneity. We phenotyped children with VUR who attended urology clinics so we could establish criteria to stratify patients based on the presence or absence of a renal malformation.

Methods: History, chart review, and DNA were obtained for 200 children with VUR from 189 families to determine the grade of VUR, the mode of presentation, and the family history for each child.