Prenatal Spina Bifida Repair: A Survey of Current Practice in the United States.

Background: Prenatal myelomeningocele (MMC) repair offers significant benefits over traditional postnatal repair, as demonstrated by the Management of Myelomeningocele Study trial. We characterize the current specialist involvement in prenatal and postnatal MMC repair.

Methods: The top 50 US News Children's Hospitals for Neonatology and Neurology/Neurosurgery were queried, resulting in 67 unique hospitals.

Anorectal manometry and urodynamics in children with spina bifida: can we predict the colonic dysmotility from bladder dysfunction?

Introduction: Spina bifida is a condition that impacts the development of the neural tube leading to urological and gastrointestinal symptoms. Both systems are influenced together due to their shared innervation and embryological origin. Despite its impact on health and well-being there has been limited research on the relationship between manometry results and urodynamic tests, in this patient population.

Postmarketing Safety Surveillance of Topiramate: A Signal Detection and Analysis Study Based on the FDA Adverse Event Reporting System Database.

Objective: This study aims to investigate the occurrence of adverse events associated with topiramate by analyzing data from the FDA Adverse Event Reporting System. The goal is to provide a basis for the safe clinical use of topiramate.

Methods: Adverse event data from the FDA Adverse Event Reporting System, from its inception through the first quarter of 2024, were extracted.

Altered placental phenotype and increased risk of placental pathology in fetal spina bifida: A matched case-control study.

Introduction: Spina bifida (SB) remains one of the most common congenital anomalies and associates with significant comorbidities in the fetus, which may, in part, be driven by placental maldevelopment. We hypothesised that placental pathologies would be more prevalent in fetuses with SB compared to fetuses without congenital anomalies.

Methods: Placental pathology and transcriptome were evaluated for fetuses with isolated open SB born preterm (cases; n = 12) and control fetuses without congenital anomalies (n = 22) born at full term (FT) or preterm (PT).

Having more resilience factors only gets you so far in the context of neighborhood disadvantage: understanding differential predictors of internalizing symptoms and body mass index in youth with spina bifida.

Objective: The primary aim of this study was to determine if neighborhood disadvantage predicted internalizing symptoms and body mass index (BMI) in youth with spina bifida (SB), while accounting for several sociodemographic factors. We also explored whether resilience factors helped explain associations between neighborhood disadvantage and internalizing symptoms or neighborhood disadvantage and BMI.

Methods: Participants (n = 69, Mage=14.

Integrative computational analyses implicate regulatory genomic elements contributing to spina bifida.

Purpose: Spina bifida (SB) arises from complex genetic interactions that converge to interfere with neural tube closure. Understanding the precise patterns conferring SB risk requires a deep exploration of the genomic networks and molecular pathways that govern neurulation. This study aims to delineate genome-wide regulatory signatures underlying SB pathophysiology.

Adaptation of the German language version of the QUAlity of life assessment of spina bifida for adults (QUALAS-A-G).

Introduction: Spina bifida (SB) affects almost all activities in daily life and therefore also health-related quality of life (HRQOL). To assess the HRQOL of adults with SB, a self-reported QUAlity of Life Assessment of Spina bifida in Adults (QUALAS-A) was validated in English. The purpose of this study was to develop and validate a German version of QUALAS-A.

The Intrauterine Treatment of Open Spinal Dysraphism.

Background: Open spinal dysraphism is a congenital malformation that causes major morbidity. Its consequences include sensory and motor impairment as well as bladder- and bowel dysfunction. It is often also associated with prenatal ventriculomegaly, which, in turn, necessitates postnatal treatment with a ventriculoperitoneal shunt in approximately 80% of cases.

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.

Factors associated with transition readiness among adolescents and young adults with spina bifida in South Korea.

This study aimed to examine the factors associated with transition readiness based on the healthcare transition model among adolescents and young adults (AYAs) with spina bifida (SB) in the individual (self-management and self-efficacy), family or social support (family function and social support), healthcare system, and environmental (transition environment) domains. Using face-to-face and online surveys, we conducted a cross-sectional study on AYAs with SB in South Korea. The participants were aged 13-25 years.

Role of Myofibroblasts in the Repair of Iatrogenic Preterm Membranes Subjected to Mechanical Stimulation.

Objective: We examined the role of myofibroblasts in regulating Cx43 and collagen structure in iatrogenic preterm amniotic membrane (AM) defects subjected to mechanical stimulation.

Method: Preterm AM specimens were collected from women undergoing planned preterm caesarean section after in utero intervention for correction of spina bifida by open fetal surgery (n = 4 patients; preterm delivery at 34 + 0 weeks to 35 + 0 weeks). Control specimens taken 5 cm away from the open fetal surgery defect site were compared with wound edge AM.

MACHINE LEARNING ALGORITHMS FOR PREDICTION OF AMBULATION AND WHEELCHAIR TRANSFER ABILITY IN SPINA BIFIDA.

Objective: To determine which statistical techniques enhance our ability to predict ambulation and transfer ability in people with spina bifida (SB).

Design: Retrospective cohort study SETTING: 35 United States outpatient SB clinic sites PARTICIPANTS: individuals (n=4,589) with SB ages 5-73 (median age =13.59) INTERVENTION: not applicable MAIN OUTCOME MEASURE: ambulation ability, which consisted of the following categories: community ambulators, household ambulators, therapeutic ambulators, and non-ambulators SECONDARY OUTCOME: wheelchair transfer ability, as defined by the ability to transfer in and out of a wheelchair unassisted RESULTS: A Recurrent Neural Network (RNN) utilizing a multilayer perceptron discarded 76 cases during case processing, resulting in 4513 that were run through the RNN.

Clinical profile of Korean children with spina bifida: a single-center prospective cohort study.

Background: Spina bifida (SB) is a chronic condition requiring lifelong self-management, underscoring the need to establish a dedicated cohort for longitudinal monitoring of health outcomes. The purpose of this study was to describe the development and initial implementation of a single-center prospective cohort study of children with SB and their parents living in South Korea and to describe demographics, clinical outcomes, psychosocial characteristics, and family data for this cohort.

Methods: This cohort was established through expert panel formation, identification of health indicators based on the Life Course Model for Spina Bifida, creation of a cohort database system, and quality control planning.

Presentation of polymelia in conjugation with spinal dysraphism: A case report.

Introduction And Importance: Polymelia is a rare congenital deformity characterized by an extra limb connected to a different part of the body. The Incidence of this condition in humans is scant but relatively higher in animals. Hereditary and genetic factors are linked to the pathogenesis of this condition, but the relationship is not clearly understood.

Intraoperative neuromonitoring and mapping during spinal cord untethering surgery; a single-centre paediatric neurosurgery unit experience.

Purpose: A review of intraoperative neuromonitoring (IONM) and mapping (IONMa) utility during paediatric tethered cord surgery with particular attention to feasibility, measures to prevent injury, and postoperative outcome.

Methods: A retrospective analysis of spinal cord untethering surgery between 2015 and 2022 was carried out. Cohort demographics, IONM and IONMa data, and procedural details were summarised and associations between variables explored.

Myelomeningocele repair in Latin America: a systematic review.

Objective: Myelomeningocele (MMC) carries high morbidity and mortality. The Management of Myelomeningocele Study (MOMS) showed improved outcomes after prenatal compared to postnatal repair. However, it is unclear how the MOMS trial affected practice and outcomes in the Latin American region.

Status of prevention of neural tube defects post-folic acid fortification of cereal grains in South Africa.

Objective: Neural tube defects (NTD) are serious, life-threatening birth defects. Staple food fortification with folic acid (vitamin B) is a proven, effective intervention to reduce NTD birth prevalence. Mandatory food fortification with folic acid was implemented in South Africa (SA) in 2003.