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.

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).

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.

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.

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.

Ureterocystoplasty: long-term functional results.

Purpose: Many institutions have reported varied levels of success with ureterocystoplasty. Recently, there have been concerns regarding the efficacy of ureterocystoplasty as a form of bladder augmentation. We report our long-term functional outcomes with the procedure based on the experience of a single surgeon.

Changes to the contractile function of ureter smooth muscle after partial infravesical obstruction in fetal sheep.

Objective: To measure spontaneous contractile activity, and the responses to agonists using in vitro preparations of sheep ureter after a period of bladder outlet obstruction (BOO) initiated at mid-gestation.

Material And Methods: Date-mated Romney Marsh ewes, bearing fetuses of 70-75 days of gestation (term in this breed is 145 days) were used. Five fetuses underwent urachal obstruction and partial urethral constriction for 30 days.

Renal transplantation or bladder augmentation first? A comparison of complications and outcomes in children.

Objective: To identify whether the order of performing transplant and bladder reconstruction operations in children who need both operations affects outcome of either operation.

Patients And Methods: A retrospective case note review was performed of children identified from our database, who had undergone both renal transplantation and bladder augmentation between 1990 and 2005.

Results: In all, 18 renal transplants (eight live-related) were performed in 16 children with 10 transplants done after bladder augmentation and eight transplants done before augmentation.

An initial experience of 100 paediatric laparoscopic nephrectomies with transperitoneal or posterior prone retroperitoneoscopic approach.

The objective of this study was to evaluate the outcome of the initial 100 consecutive laparoscopic nephrectomies using a transperitoneal or a posterior prone retroperitoneoscopic approach. The medical records of 97 consecutive children who underwent laparoscopic nephrectomy between January 2000 and December 2003 were reviewed. Children having concomitant operative procedures were excluded from the study.

Radiotelemetered urodynamics of obstructed ovine fetal bladders: correlations with ex vivo cystometry and renal histopathology.

Objective: To test the hypothesis that fetal bladder outlet obstruction (BOO) in sheep causes abnormal urodynamics correlating with the initiation of renal pathology, as most boys with posterior urethral valves (PUV) have bladder dysfunction when assessed after birth.

Materials And Methods: Male ovine fetuses at 94 days of gestation were assigned to BOO (four) or sham control (five) groups. In BOO fetuses, radiotelemetry catheters were inserted transurachally and intraperitoneally, and the urethra was partly occluded.

The two-stage repair for severe primary hypospadias.

Objectives: The repair of severe primary hypospadias represents a major surgical challenge. After initial enthusiasm for single-stage procedures, many paediatric urologists have turned to the alternative two-stage approach after experiencing disappointing results. A single surgeon's experience of the two-stage procedure is reported.

Contractile properties of the developing fetal sheep bladder.

Aim: To characterise the developmental changes to the normal bladder by examining the in vitro contractile properties of the fetal sheep detrusor smooth muscle bladder at different gestational ages.

Methods: Three groups of normally developing fetal sheep bladders were investigated: (1) 65-70 days gestation (2nd trimester); (2) 105-110 days (3rd trimester); (3) 135-140 days (term). Isometric contractions in isolated detrusor strips were measured during either electrical field stimulation (EFS) or exposure to agonists: carbachol, alpha-beta methylene-ATP (ABMA) and KCl.

The role of unilateral nephrectomy in the treatment of nephrogenic hypertension in children.

Objectives: To define the efficacy of unilateral nephrectomy in a large series of patients presenting with renal disease and hypertension, as the latter may be a prominent finding in children with nephrourological disease (renal parenchymal disease, renovascular disease, obstruction, renal dysplasia and cancer).

Patients And Methods: We retrospectively reviewed the hospital and outpatient records of 118 children who presented for evaluation with hypertension, and who had a nephrectomy between 1968 and 2003. Patients included in the study were those who had a unilateral nephrectomy for benign renal hypertension with a normal contralateral kidney; in all, 21 had complete records and follow-up were evaluated.

Urinary outflow obstruction increases apoptosis and deregulates Bcl-2 and Bax expression in the fetal ovine bladder.

During organogenesis, net growth of tissues is determined by a balance between proliferation, hypertrophy, and apoptotic death. Human fetal bladder outflow obstruction is a major cause of end-stage renal failure in children and is associated with complex pathology in the kidney and lower urinary tract. Experimental manipulation of the fetal sheep urinary tract has proved informative in understanding the pathobiology of congenital obstructive uropathy.

Antenatal urodynamic studies in the fetal lamb: experimental protocol and preliminary results.

Objectives: To set up a fetal lamb model for intrauterine fetal urodynamic studies.

Methods: Fourteen fetal lambs underwent placement of a bladder catheter at a mean gestational age of 87 days. Three fetuses also had a partial urethral obstruction by the simultaneous placement of a peri-urethral constricting ring.

Effects of in utero bladder outflow obstruction on fetal sheep detrusor contractility, compliance and innervation.

Purpose: Congenital bladder outflow obstruction caused by posterior urethral valves is a common cause of end stage renal failure in boys. We hypothesized that fetal bladder outflow obstruction perturbs detrusor contractility and innervation and bladder storage volume-pressure relationships.

Materials And Methods: Severe bladder outflow obstruction was induced in male fetal sheep by placing a urethral ring and urachal ligation midway through gestation at 75 days.