Diagnostic Test Characteristics of Ultrasound-Based Hydronephrosis for Chronic Kidney Disease in Children and Adolescents With Myelomeningocele: Results From the UMPIRE and NSBPR Cohort Studies.

Purpose: Renal ultrasounds are performed in patients with myelomeningocele to screen for markers of kidney health, including hydronephrosis. We evaluated the diagnostic accuracy of hydronephrosis to screen for low kidney function defined by estimated glomerular filtration rate (eGFR).

Materials And Methods: We performed a retrospective cross-sectional study using data from 2 cohorts of children and youth with myelomeningocele.

Mental health, psychosocial functioning, and health-related quality of life of children and adolescents with bladder exstrophy, cloacal exstrophy, and epispadias: A scoping review.

Background: The exstrophy-epispadias complex (EEC) is a spectrum of rare congenital defects affecting the genitourinary system that includes epispadias, bladder exstrophy, and cloacal exstrophy. EEC patients may require several surgeries throughout their lifetime and experience a number of difficulties that may impact their quality of life (QoL), mental health, and psychosocial functioning.

Objective: The purpose of this scoping review is to systematically map existing literature that explores the mental health, psychosocial functioning, and QoL of children and adolescents with EEC.

Urogenital Sinus Diagnosed During Workup of Recurrent Urinary Tract Infections: A Case Report.

Urogenital sinus (UGS) is a rare anomaly characterized by a common cavity involving the reproductive and urinary tracts. We describe a patient with VACTERL syndrome who presented for urologic care at 11 years of age due to supposed "recurrent urinary tract infections" and was subsequently found to have UGS in which the vagina connected directly to the bladder. She underwent robotic UGS mobilization to disconnect the vagina from the bladder and vaginoplasty to mature the vagina to the perineum.

Imaging characteristics predicting elevated detrusor pressures in patients with spina bifida.

Purpose: Patients with spina bifida require urologic follow up with assessments of bladder and renal function. The ideal screening protocol for bladder compliance remains controversial. This study assessed associations between bladder end filling pressure (EFP) and detrusor leak point pressure (DLPP) on urodynamic studies (UDS) and findings from renal bladder ultrasounds (RBUS) and voiding cystourethrograms (VCUG).

False positive pregnancy tests in pediatric patients with augmentation enterocystoplasty.

Purpose: Our primary aim was to determine the prevalence of positive over-the-counter (OTC) pregnancy tests among pediatric female and male patients who had undergone augmentation enterocystoplasty. A secondary aim was to determine whether patient factors or urinalysis (UA) results were associated with false positive OTC pregnancy test results in patients with a history of augmentation enterocystoplasty.

Methods: Patients at a tertiary pediatric hospital who had previously undergone augmentation enterocystoplasty, Mitrofanoff appendicovesicostomy only, and age- and sex-matched controls were prospectively recruited.

Neuropilin 2 Is a Novel Regulator of Distal Colon Contractility.

Appropriate coordination of smooth muscle contraction and relaxation is essential for normal colonic motility. The impact of perturbed motility ranges from moderate, in conditions such as colitis, to potentially fatal in the case of pseudo-obstruction. The mechanisms underlying aberrant motility and the extent to which they can be targeted pharmacologically are incompletely understood.

Utility of renal scintigraphy in diagnosis of multicystic dysplastic kidney.

Purpose: To compare the prevalence of vesicoureteral reflux (VUR), febrile urinary tract infection (fUTI), and chronic kidney disease (CKD) among patients with multicystic dysplastic kidney (MCDK) diagnosed by renal scintigraphy (RS) versus follow-up renal ultrasound (RUS) alone.

Methods: This was a retrospective review of patients seen at a tertiary care center from 2010 to 2020 with MCDK diagnosed by RS or follow-up RUS. Differences in the prevalence of VUR, fUTI, and CKD by cohort were assessed using logistic regression analysis, Pearson X , and Fisher's Exact tests.

Imaging of congenital genitourinary anomalies.

Congenital genitourinary anomalies are among the most frequent types of birth defects in neonates. Some anomalies can be a significant cause of morbidity in infancy, while others remain asymptomatic even until adulthood and can be at times the only manifestation of a complex systemic disease. The spectrum of these anomalies results from the developmental insults that can occur at various embryologic stages, and an understanding of the formation of the genitourinary system is helpful in the evaluation and treatment of a child with a congenital genitourinary anomaly.

Urodynamic characteristics of neurogenic bladder in newborns with myelomeningocele and refinement of the definition of bladder hostility: Findings from the UMPIRE multi-center study.

Introduction: Infants with myelomeningocele are at risk for chronic kidney disease caused by neurogenic bladder dysfunction. Urodynamic evaluation plays a key role to risk stratify individuals for renal deterioration.

Objective: To present baseline urodynamic findings from the Urologic Management to Preserve Initial Renal function for young children with spina bifida (UMPIRE) protocol, to present the process that showed inadequacies of our original classification scheme, and to propose a refined definition of bladder hostility and categorization.

Association between severity of prenatally diagnosed hydronephrosis and receipt of surgical intervention postnatally among patients seen at a fetal-maternal center.

Background: Hydronephrosis (HN) is the most common abnormality detected on prenatal ultrasound. This study sought to stratify outcomes of patients by severity of prenatal HN with postnatal outcomes.

Methods: This was a retrospective review of patients referred to a tertiary care fetal-maternal clinic with diagnosis of prenatal HN from 2004 to 2019.

Timing of inguinal hernia following complete primary repair of bladder exstrophy.

Introduction/background: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood.

Missed anterior sacral meningomyelocele presenting with obstructive uropathy.

A 2-month-old full-term female presented with a large anterior sacral meningomyelocele resulting in transient obstructive uropathy with bilateral hydronephrosis and acute kidney injury. After initial bladder decompression and surgical resection of the meningomyelocele, there was spontaneous resolution of bladder function confirmed with urodynamics. Anterior spinal meningomyelocele (ASM) is a rare neural tube defect that may present with urinary dysfunction secondary to compression of the bladder and sacral nerve roots or congenital defects to the bladder nervous supply.

Robotic adrenalectomy in the pediatric population: initial experience case series from a tertiary center.

Background: Laparoscopic resection is the most well described minimally-invasive approach for adrenalectomy. While it allows for improved cosmesis, faster recovery and decreased length of hospital stay compared with the open approach, instrument articulation limitations can hamper surgical dexterity in pediatric patients. Use of robotic assistance can greatly enhance operative field visualization and instrument control, and is in the early stages of adoption in academic centers for pediatric populations.

Mature Ovarian Cystic Teratoma Mimicking a Bladder Diverticulum in a 16-year-old Female.

A 16-year-old female was incidentally found to have an abnormal pelvic ultrasound while undergoing evaluation for mild scoliosis. A large, thick-walled, lobular, fluid-filled structure was found at midline in the pelvis that initially resembled bladder diverticula due to its anatomical position and sonographic appearance. Voiding cystourethrogram and computed tomography later revealed a large cystic mass originating from the right ovary.

Testicular Torsion From Bell-clapper Deformity.

A 12-year-old otherwise healthy boy presents to a local emergency room with sudden onset, sharp, continuous left testicular pain for 9 hours, associated with nausea and vomiting. Exam and ultrasound is consistent with testicular torsion. During scrotal exploration, a bell-clapper deformity of the left testicle is appreciated, with a normal gubernacular attachment of the right testicle.

Severe ureteropelvic junction obstruction with massive hydronephrosis causing compression of the inferior vena cava.

A 7-week-old girl presented with tachypnea and abdominal distension. Abdominal ultrasonography demonstrated a 16 cm cystic mass in the right abdomen. Contrast-enhanced T2-weighted magnetic resonance imaging revealed severe hydronephrosis of the right kidney with thinning of the renal parenchyma consistent with a massive ureteropelvic junction (UPJ) obstruction.

Forniceal Rupture in Neonate Masquerading as a Cystic Renal Mass.

A 5-week-old full-term female with high-risk right hydronephrosis was transferred to the emergency department (ED) for tachypnea. A contrast-enhanced computed tomography (CT) study demonstrated a 9.0 cm cystic lesion in the region of the previously seen hydronephrosis (Fig.

Concordant miRNA and mRNA expression profiles in humans and mice with bladder outlet obstruction.

Bladder outlet obstruction (BOO) leads to lower urinary tract symptoms (LUTS) and urodynamic changes of the bladder function. Previously we identified microRNA (miRNA) and mRNA expression profiles associated with different states of BOO-induced LUTD in human patients. Bladder wall remodeling resulting from obstruction is widely studied in animal models of experimentally-induced partial BOO (pBOO).

Pelvic floor laxity: A not so rare but unrecognized form of daytime urinary incontinence in peripubertal and adolescent girls.

Introduction: Stress urinary incontinence (SUI) is common among older multiparous females but rarely reported in active, young girls.

Objectives: Our hypothesis is: physically active adolescent females develop pelvic floor laxity demonstrable on upright VCUG. Our objectives are to (1) increase awareness of SUI in young females, (2) test our hypothesis with an upright VCUG, and (3) report effectiveness of step-wise management.

Deletion of neuropilin 2 enhances detrusor contractility following bladder outlet obstruction.

Chronic urethral obstruction and the ensuing bladder wall remodeling can lead to diminished bladder smooth muscle (BSM) contractility and debilitating lower urinary tract symptoms. No effective pharmacotherapy exists to restore BSM contractile function. Neuropilin 2 (Nrp2) is a transmembrane protein that is highly expressed in BSM.

An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care.

Purpose: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy.

Where do we place the sacrocolpopexy stitch? A magnetic resonance imaging investigation.

Objectives: Sacrocolpopexy presacral sutures are placed at or slightly above the sacral promontory without knowledge of the location of intervertebral discs. We used magnetic resonance imaging to assess the anatomic relationship of the sacral promontory to intervertebral discs.

Methods: We reviewed spinal magnetic resonance images of women imaged at Loyola University Medical Center between January 2010 and February 2012.

Definition of reliable, objective criteria by abdominal radiography to identify occult constipation in children with lower urinary tract symptoms.

Purpose: Treating constipation in children with voiding dysfunction may improve or resolve urinary symptoms. A clinical diagnosis of constipation may not identify all patients. Abdominal radiographs (plain x-ray of the kidneys, ureters and bladder) are often used to assess constipation but no objective definition of constipation based on abdominal radiographs exists.