A novel approach to calculating expected total fetal lung volume in fetuses with isolated congenital diaphragmatic hernia and fetal growth restriction: a theoretical computational simulation.

Objectives: Congenital Diaphragmatic Hernia (CDH) often coexists with fetal growth restriction (FGR). The observed-to-expected (O/E) total fetal lung volume (TFLV) is used to assess CDH severity, predict outcomes, and direct fetal interventions. Expected TFLV measurements traditionally rely only on gestation age (GA).

Risk of missed diagnoses when choosing non-operative management of pediatric uncomplicated appendicitis.

Purpose: An antibiotic-only approach to managing uncomplicated appendicitis in children confers the risk of missed diagnoses of alternate pathologies. We aimed to quantify this risk in patients presenting with uncomplicated acute appendicitis at a large tertiary children's hospital and to assess contemporary perioperative clinical outcomes.

Methods: A retrospective review of patients who underwent appendectomy for uncomplicated appendicitis between January 1, 2019 and December 31, 2023 was conducted.

Advanced practice providers in pediatric general surgery: A single-institution analysis of impact on clinical and surgical productivity.

Introduction: Advanced practice providers (APPs) were initially introduced to the healthcare system due to primary care provider shortages but have since broadened their scope of practice across surgical and non-surgical specialties. There is a paucity of literature regarding how APPs can improve divisional productivity within pediatric general surgery. This study aims to explore the impact of increased APP implementation within the division of pediatric general surgery at a tertiary children's hospital.

Increasing surgical healthcare utilization for infants with congenital anomalies in Texas.

Introduction: Congenital anomalies (CAs) impact 3% of live births and account for disproportionately high healthcare costs. While many CAs require multidisciplinary care and surgical intervention, the overall financial impact of infants diagnosed with CA with surgical needs is unknown. We aim to evaluate and characterize the charges of care in infants with CA and surgical needs in Texas.

Shear-Induced Macrophage Secretome Promotes Endothelial Permeability.

Background: Discrete subaortic stenosis is a pediatric cardiovascular disease marked by fibrotic growth within the left ventricular outflow tract (LVOT), leading to severe complications, including left ventricular hypertrophy, aortic regurgitation, and arrhythmias. Despite surgical intervention, a 20-30% recurrence rate suggests a complex underlying pathophysiology. Elevated flow and resultant hemodynamic shear stress within the LVOT are key factors in DSS development.

Shear Stress Conditioning Promotes a Pro-Inflammatory Response in Porcine Endocardial Endothelial Cells.

Purpose: Discrete subaortic stenosis (DSS) is a congenital heart disease characterized by a narrowing of the passage below the aortic valve in the left ventricular outflow tract (LVOT). While endocardial endothelial cells (EECs) are known to play a role in DSS, the response of these cells to shear stress is not known. In this study, we hypothesize that the response of EECs to shear stress in the LVOT is a mediator of DSS.

The nitrofen/bisdiamine murine model of congenital diaphragmatic hernia has a pulmonary hypertension vascular phenotype consistent with human CDH.

Congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension (CDH-PH) has severe implications for the survival of patients with CDH; however, CDH-PH is often refractory to pulmonary vasodilators, rendering it difficult to treat. As such, models are necessary to study the etiology, mechanism, onset, and progression of pulmonary vascular remodeling in CDH. Despite several established murine models of CDH, no characterized CDH-PH or CDH-associated pulmonary vascular remodeling murine model exists.

Shear Stress Conditioning Promotes a Pro-Inflammatory Response in Porcine Endocardial Endothelial Cells.

Purpose: Discrete subaortic stenosis (DSS) is a congenital heart disease characterized by a narrowing of the passage below the aortic valve in the left ventricular outflow tract (LVOT) [1]. While endocardial endothelial cells (EECs) are known to play a role in DSS, the response of these cells to shear stress is not known. In this study, we hypothesize that the response of EECs to shear stress in the LVOT is a mediator of DSS.

Standardized Note Template Expedites Completion of Consults for Surgical Fetal Anomalies.

Introduction: We developed standardized electronic medical record templates (EMR-temp) for use in ambulatory prenatal surgical consultations for surgical fetal anomalies (SFAs). Our aim was to evaluate EMR-temp impact in provider documentation in prenatal care of SFA.

Methods: Prenatal consultations for SFAs at a single institution were retrospectively reviewed (2019-2022).

Activation of mechanoreceptor Piezo1 inhibits enteric neuronal growth and migration .

Introduction: Dysfunction of the enteric nervous system (ENS) is linked to a myriad of gastrointestinal (GI) disorders. Piezo1 is a mechanosensitive ion channel found throughout the GI tract, but its role in the ENS is largely unknown. We hypothesize that Piezo1 plays an important role in the growth and development of the ENS.

Development of Novel 3D Spheroids for Discrete Subaortic Stenosis.

In this study, we propose a new method for bioprinting 3D Spheroids to study complex congenital heart disease known as discrete subaortic stenosis (DSS). The bioprinter allows us to manipulate the extrusion pressure to change the size of the spheroids, and the alginate porosity increases in size over time. The spheroids are composed of human umbilical vein endothelial cells (HUVECs), and we demonstrated that pressure and time during the bioprinting process can modulate the diameter of the spheroids.

Ex Utero Intrapartum Treatment for Prenatally Diagnosed Cervicofacial Lymphatic Malformations.

Introduction: Cervicofacial lymphatic malformations (cf-LM) may be identified on prenatal ultrasound, prompting consideration of ex utero intrapartum treatment (EXIT) to secure the fetal airway. Furthermore, the recent shift in postnatal management of cf-LM from resection alone toward a multimodal approach including sirolimus and sclerotherapy may impact the neonatal outcomes of cf-LM. This study aims to characterize the neonatal outcomes of patients with prenatally diagnosed cf-LM who underwent EXIT-to-airway.

Shear Stress Induces a Time-Dependent Inflammatory Response in Human Monocyte-Derived Macrophages.

Macrophages are innate immune cells that are known for their extreme plasticity, enabling diverse phenotypes that lie on a continuum. In a simplified model, they switch between pro-inflammatory (M1) and anti-inflammatory (M2) phenotypes depending on surrounding microenvironmental cues, which have been implicated in disease outcomes. Although considerable research has been focused on macrophage response to biochemical cues and mechanical signals, there is a scarcity of knowledge surrounding their behavior in response to shear stress.

Characteristics and Outcomes of Infants in Texas by Facility Children's Surgery Verification Status.

Introduction: The American College of Surgeons Children's Surgery Verification (CSV) status recognizes hospitals that deliver high-quality pediatric surgical care. Texas has 5 CSV centers in three cities, which may limit equitable access to care. We explored the characteristics and outcomes of infants admitted in Texas as a function of facility CSV status.

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics.

Introduction: Congenital lung malformations (CLMs) are diverse and readily diagnosed on prenatal ultrasound. Postnatal computed tomography (CT) characteristics, including volume, are used in centers for the clinical decision-making of asymptomatic CLM. We aim to evaluate the relationship of prenatal CLM volume ratio (CVR) to postnatal CT characteristics by suspected prenatal diagnosis and postnatal radiological diagnosis.

Extracorporeal Membrane Oxygenation Before 34 Wks' Gestation: A Single-Center Experience.

Introduction: Traditionally, gestational age <34 wk and weight <2 kg are considered relative contraindications to extracorporeal membrane oxygenation (ECMO). There is a paucity of information that explains the outcomes in this unique population of premature neonates. The purpose of this study is to examine outcomes of patients who undergo ECMO at <34 wk at a single institution.

Development of a novel 3D perfusable vascular graft model to elucidate the mechanisms for congenital heart disorders.

Pediatric heart transplantation is hampered by a chronic shortage of donor organs. This problem is further confounded by graft rejection. Identification of earlier indicators of pediatric graft rejection and development of subsequent strategies to counteract these effects will increase the longevity of transplanted pediatric hearts.

Treatment Facility Case Volume and Disparities in Outcomes of Congenital Diaphragmatic Hernia Cases.

Introduction: Congenital diaphragmatic hernia (CDH) is a life-threatening, prenatally diagnosed congenital anomaly. We aim to characterize care and outcomes of infants with CDH in Texas and the impact of treating facilities volume of care.

Methods: Retrospective cohort study using a state-wide Hospital Inpatient Discharge Public Use Data File was conducted (2013-2021).

Surfactant Therapy in Congenital Diaphragmatic Hernia and Fetoscopic Endoluminal Tracheal Occlusion.

Introduction: Congenital diaphragmatic hernia (CDH) is a complex pathology with severe pulmonary morbidity. Administration of surfactant in CDH is controversial, and the advent of fetoscopic endoluminal tracheal occlusion (FETO) has added further complexity. While FETO has been shown to improve survival outcomes, there are risks of prematurity and potential surfactant deficiency.

Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations.

Introduction: Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality.

Molecular insights using spatial transcriptomics of the distal lung in congenital diaphragmatic hernia.

Abnormal pulmonary vascular development and function in congenital diaphragmatic hernia (CDH) is a significant factor leading to pulmonary hypertension. The lung is a very heterogenous organ and has marked cellular diversity that is differentially responsive to injury and therapeutic agents. Spatial transcriptomics provides the unmatched capability of discerning the differences in the transcriptional signature of these distinct cell subpopulations in the lung with regional specificity.

Approach and Technique for Cesarean Section to Immediate Resection for High-Risk Sacrococcygeal Teratomas.

Introduction: Ex-utero intrapartum treatment has been established as an option for fetal and perinatal surgeons to deliver patients with sacrococcygeal teratomas (SCTs) which are causing significant fetal distress and possible in-utero fetal demise. However, ex-utero intrapartum treatment procedures carry significant maternal risk and morbidity. Herein, we report an alternative technique of Cesarean section to immediate resection (CSIR) for managing high-risk SCTs.