Multi-staged vs Single-staged Pelvic Osteotomy in the Modern Treatment of Cloacal Exstrophy: Bridging the Gap.

Purpose: Staged pelvic osteotomy has been shown in the past to be an effective tool in the closure of the extreme pubic diastasis of cloacal exstrophy. The authors sought to compare orthopedic complications between non-staged pelvic osteotomies and staged pelvic osteotomies in cloacal exstrophy.

Methods: A prospectively maintained exstrophy-epispadias complex database of 1510 patients was reviewed for cloacal exstrophy bladder closure events performed with osteotomy at the authors' institution.

Classic Bladder Exstrophy Closure Without Osteotomy or Immobilization: An Exercise in Futility?

Objective: To review the outcomes of classic bladder exstrophy (CBE) closure without the use of osteotomy or lower extremity/pelvic immobilization.

Methods: A prospectively maintained institutional approved exstrophy-epispadias complex database of 1487 patients was reviewed for patients with CBE who had undergone closure without osteotomy nor immobilization. All patients were referred to the authors' institution for reconstruction later in life or for failed closure.

Gastrointestinal Dysfunction in Genetically Defined Neurodevelopmental Disorders.

Gastrointestinal symptoms are common in most forms of neurodevelopment disorders (NDDs) such as in autism spectrum disorders (ASD). The current patient-reported outcome measures with validated questionnaires used in the general population of children without NDDS cannot be used in the autistic individuals. We explore here the multifactorial pathophysiology of ASD and the role of genetics and the environment in this disease spectrum and focus instead on possible diagnostics that could provide future objective insight into the connection of the gut-brain-microbiome in this disease entity.

Mucosal Violations and Their Effect on Successful Bladder Neck Closure in Cloacal Exstrophy.

Background: Cloacal exstrophy (CE) is rare and challenging to reconstruct. In the majority of CE patients voided continence cannot be achieved and so patients often undergo bladder neck closure (BNC). Prior mucosal violations (MVs), a surgical event when the bladder mucosa was opened or closed, significantly predicted failed BNC in classic bladder exstrophy with an increased likelihood of failure after 3 or more MVs.

Achieving goal capacity for continence surgery: A cumulative event analysis of bladder exstrophy patients.

Background: Following successful closure of patients with classic bladder exstrophy (CBE), the next major milestone is the establishment of urinary continence. Prior to determining the most appropriate continence surgery, it is imperative to reach an adequate bladder capacity minimum of 100 cc in order to make the decision between bladder neck reconstruction (BNR) or continent stoma, with or without augmentation cystoplasty (AC).

Objective: To examine the timing of when patients achieve threshold bladder capacity for BNR eligibility.

Bladder capacity and growth in classic bladder exstrophy: A novel predictive tool.

Introduction: Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE). BC is frequently used to determine eligibility for surgical continence procedures, such as bladder neck reconstruction (BNR), and is associated with the likelihood of achieving urinary continence.

Objective: To use readily available parameters to develop a nomogram that could be used by patients and pediatric urologists to predict BC in patients with CBE.

A Single-Institutional Experience With Prenatal Diagnosis of Cloacal Exstrophy: Room for Improvement.

Introduction: A single institutional study characterizes the rate of prenatal diagnosis of cloacal exstrophy (CE) and examines its role on successful primary closures.

Materials And Methods: An institutional database of 1485 exstrophy-epispadias patients was reviewed retrospectively for CE patients with confirmed presence/absence of prenatal diagnostics, primary exstrophy closure since 2000, institution of closure, and at least 1 year of follow up following closure.

Results: The cohort included 56 domestic patients and 9 international patients.

Prevalence of opioid and benzodiazepine use in adult patients with the exstrophy-epispadias complex.

Introduction: Bladder exstrophy (BE), cloacal exstrophy (CE), and epispadias (E) are variants of the exstrophy-epispadias complex (EEC). These children require opioids and benzodiazepines to achieve pain management and immobilization for a lifetime of surgeries. It is hypothesized that these children would be sensitized to opiates and benzodiazepines as adults.

Bladder exstrophy-epispadias complex related litigation: A legal database review.

Objective: To review the nature and extent of bladder exstrophy-epispadias related malpractice litigation in the United States.

Methods: Two legal databases (Nexis Uni, WestLaw) were reviewed for state and federal cases using the terms "bladder exstrophy", "cloacal exstrophy", "epispadias", in combination with "medical malpractice", or "negligence", or "medical error", or "complication", or "malpractice", or "tort". Databases were queried from 1948 to 2022 and reviewed for medical and legal details.

Fixation with lower limb immobilization in primary and secondary exstrophy closure: A saving grace.

Purpose: A pivotal factor in the success of bladder closure in patients with classic bladder exstrophy (CBE) is the postoperative immobilization of the pelvis and lower extremities after pelvic osteotomy. This study investigates the outcomes of closure among patients with lower limb immobilization using many techniques. The authors hypothesize that the addition of external fixation (pelvic immobilization) in patients with any form of limb immobilization will be associated with improved outcomes.

Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex.

Objective: To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair.

Methods: A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate.

Orthopedic complications after osteotomy in patients with classic bladder exstrophy and cloacal exstrophy: a comparative study.

Background: The addition of pelvic osteotomy to the armamentarium of tools for correction of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) has undeniably served as one of the most effective advancements in improving the likelihood of successful primary bladder closure. Osteotomy-related complications have been studied and documented extensively in patients with CBE, yet evaluation remains limited in CE concordant with its relative rarity.

Objective: To compare orthopedic complications in patients with CBE and CE who underwent primary bladder closure with osteotomy.

Batch effects removal for microbiome data via conditional quantile regression.

Batch effects in microbiome data arise from differential processing of specimens and can lead to spurious findings and obscure true signals. Strategies designed for genomic data to mitigate batch effects usually fail to address the zero-inflated and over-dispersed microbiome data. Most strategies tailored for microbiome data are restricted to association testing or specialized study designs, failing to allow other analytic goals or general designs.

Prognostic and Goals-of-Care Communication in the PICU: A Systematic Review.

Objective: Admission to the PICU may result in substantial short- and long-term morbidity for survivors and their families. Engaging caregivers in discussion of prognosis is challenging for PICU clinicians. We sought to summarize the literature on prognostic, goals-of-care conversations (PGOCCs) in the PICU in order to establish current evidence-based practice, highlight knowledge gaps, and identify future directions.

Pelvic osteotomy in cloacal exstrophy: A changing perspective.

Background: The type of osteotomy and pelvic fixation in the management of primary cloacal exstrophy (CE) closure is variable. The purpose of this study was to evaluate primary CE closure outcomes with osteotomy, immobilization, and multi-staging procedure trends over time.

Methods: An institutional database was retrospectively reviewed for patients who underwent primary CE closure from 1960 to 2020.

Development of a non-pharmacologic delirium management bundle in paediatric intensive care units.

Background: Non-pharmacologic interventions might be effective to reduce the incidence of delirium in pediatric intensive care units (PICU).

Aim: To explore expert opinions and generate informed consensus decisions regarding the content of a non-pharmacologic delirium bundle to manage delirium in PICU patients.

Study Design: A two-round online Delphi study was conducted from February to April 2021.

Longitudinal changes in uric acid concentration and their relationship with chronic kidney disease progression in children and adolescents.

Background: Elevated serum uric acid concentration is a risk factor for CKD progression. Its change over time and association with CKD etiology and concomitant changes in estimated glomerular filtration rate (eGFR) in children and adolescents are unknown.

Methods: Longitudinal study of 153 children/adolescents with glomerular (G) and 540 with non-glomerular (NG) etiology from the CKD in Children (CKiD) study.

Video tools in pediatric goals of care communication: A systematic review.

•Goals of Care resources are primarily in written format and for adult patients.•Video tools can support families of pediatric patients facing prognostic uncertainty.•Videos represent an effective but underutilized Goals of Care communication tool.

Measures of Intracranial Injury Size Do Not Improve Clinical Decision Making for Children With Mild Traumatic Brain Injuries and Intracranial Injuries.

Background: When evaluating children with mild traumatic brain injuries (mTBIs) and intracranial injuries (ICIs), neurosurgeons intuitively consider injury size. However, the extent to which such measures (eg, hematoma size) improve risk prediction compared with the kids intracranial injury decision support tool for traumatic brain injury (KIIDS-TBI) model, which only includes the presence/absence of imaging findings, remains unknown.

Objective: To determine the extent to which measures of injury size improve risk prediction for children with mild traumatic brain injuries and ICIs.